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Clinical Vignette and Research Abstract Compendium
ACP Southern California Chapters I and III Scientific Meeting
SGIM California Regional Meeting
October 23-25, 2009
Hyatt Grand Champions Resort and Spa
Indian Wells, California
Table of Contents:
(Within category, abstracts are organized by first letter of first author’s last name)
Clinical Vignette Abstracts: p. 3-77
Research Abstracts: p. 77-91
Dear Readers,
The Program Committee of the first joint ACP Regions 1 and 3 Scientific Meeting/ California
SGIM Regional Meeting congratulates the abstract authors on their excellent submissions. This
year, authors submitted a record number of abstracts. The high quality reflects the diligence, intelligence, integrity, and creativity of the authors and their advisors/mentors.
We thank the abstract reviewers for their difficult task of selecting abstracts for oral presentation.
And we appreciate the oral and poster competition judges for their similarly-difficult task.
Sincerely,
Simerjot K. Jassal, MD, MAS, FACP, Co-Chair
Nader Kamangar, MD, FACP, FCCP, Co-Chair
Alpesh N. Amin, MD, MBA, FACP
Daniel S. Anderson, MD, FACP
Dennis W. Cope, MD, FACP
Joel C. Diamant, MD, FACP
Carla Fox, MD, FACP
Charles G. Goldberg, MD
Mark Richman, MD, MPH
Soma Wali, MD, FACP
Louise Walter, MD
Natalie Kazcur
2
Clinical Vignette Abstracts
Two for One: An Unusual Case of Weakness
Pouya Afshar, MD; Nekhonti Adams; Payam Afshar
Scripps Green Hospital
A sixty-eight year–old female was admitted for a oneweek history of progressive lower extremity weakness
and associated falls. She denied any loss of consciousness, back pain, myalgias, numbness, paresthesias, bowel
or bladder incontinence, recent illnesses, or change in
medications. Immediately preceding her falls, the patient
reported a transient weakness in her thighs that persisted
for approximately thirty minutes and resolved spontaneously. Past medical history was significant for hyperlipidemia, essential tremors, and a seventy-five pack year
history of smoking. Pertinent exam findings included
inspiratory and expiratory wheezing, and bilateral 4/5
strength in the proximal lower extremity muscles with
diminished reflexes. Laboratory evaluation revealed a
sodium of 110 mmol/L, urine osmolality of 279
mOsm/kg, and serum osmolality of 230 mOsm/kg. Serum
creatinine, blood urea nitrogen, random cortisol, thyroid
stimulating hormone, creatine kinase, and erythrocyte
sedimentation rate were all found to be normal. Given the
pulmonary exam and the likely diagnosis of a syndrome
of inappropriate antidiuretic hormone secretion, a chest xray was ordered and showed a mediastinal mass. A subsequent CT of the chest confirmed a mediastinal mass, as
well as secondary compression of the right pulmonary
artery and mainstem bronchus. To avoid further respiratory compromise, an emergent bronchoscopy/biopsy and
radiation therapy were ordered. The pathology confirmed
a diagnosis of limited stage small cell lung carcincoma,
with no evidence of metastasis on further imaging studies. Despite correction of the hyponatremia from 110 to
130 mmol/L, the patient’s weakness did not improve during her hospitalization. Prior to discharge, a voltage-gated
calcium channel (VGCC) antibody test was ordered to
rule out the possibility of another paraneoplastic syndrome (Lambert-Eaton). Within two weeks, the VGCC
was found to be positive, confirming a diagnosis of Lambert-Eaton syndrome as the etiology of her lower extremity weakness. After several rounds of chemotherapy and
radiation, there is no further evidence of active tumor and
the weakness has resolved without any further intervention. This case highlights a patient with two paraneoplastic syndromes, SIADH and Lambert-Eaton Syndrome.
The latter represents a very rare, pre-synaptic defect that
is associated with small cell lung carcinoma in fifty percent of cases. While there have been several documented
cases describing SIADH or Lambert-Eaton Syndrome in
association with small cell lung carcinoma, this is only
the second case report where a patient is afflicted with
both paraneoplastic syndromes.
Intense Pruritus Requiring Hospitalization Secondary
to Acute EBV Hepatitis
Satyanisth Agrawal, MD; Alok Jain, MD; Kevin
Mullen, MD; Neil Kaplowitz, MD
University of Southern California
Pruritus is a complication of chronic liver disease. The
prevalence of pruritus in liver disease in general varies
from 5% in patients with chronic hepatitis C to 70% in
patients with Primary Biliary Cirrhosis. Epstein Barr Virus (EBV) infection causes asymptomatic liver-associated
enzyme abnormalities in 80 to 90% of cases, which are
often unrecognized. Patients with acute EBV infections
may also develop cholestatic hepatitis with associated
jaundice (< 5% of cases) and hepatitis with moderate
elevation in transaminase. We report a case of intense
pruritus in cholestatic jaundice secondary to EBV hepatitis requiting hospital admission for treatment. A 39 year
old Caucasian male was referred to our out-patient clinic
with 3-week history of generalized weakness, jaundice
with worsening LFT’s, & pruritus. The onset of these
symptoms was preceded with 5 days of fever, chills, nausea and vomiting, decreasing appetite, and diarrhea. A
trial of hydroxyzine and SSRIs by his primary physician
had failed to control his pruritus. His PE was remarkable
for icterus, an enlarged right anterior cervical lymph
node, and multiple linear excoriations on his extremities
and trunk from pruritus. Lab tests showed a mixed picture
with increased transaminases (resolving) and increased
bilirubin (worsening). He had a (+) Monospot; EBV serology confirmed acute infection. Initial management
included supportive care and ursodiol in addition to the
antihistamines. However, the patient returned with worsening symptoms and was noted to have pruritus severe
enough to cause significant insomnia and lethargy. During the next few appointments at the liver clinic he was
tried on rifampicin, diphenylhydramine, cholestyramine
and naltrexone, none of which helped him. He stopped
naltraxone after the 1st dose due to development of electric shock like symptoms, shakes including numbness and
paresthesias involving the extremities. Having exhausted
all options and worsening pruritus, he was admitted for
naloxone infusion to introduce an opiate antagonist
slowly to prevent the withdrawal reaction. The patient
was started on an ultra low dose naloxone drip (0.02
µg/kg/min) and over 24 hours titrated up to a rate of 0.8
µg/kg/min. He tolerated the treatment without adverse
effects and reported complete relief from pruritus. The
patient was discharged home on low dose naltrexone tablets. He remained asymptomatic from pruritus during
follow up visits over one month. This report illustrates
successful treatment of severe pruritus with naloxone drip
in the setting of cholestatic liver disease secondary to
acute EBV infection requiring hospitalization after failing
out-patient therapy.
Classic Presentation of Diffuse B-Cell Lymphoma
Kevan Akrami, MD; Seanna Grob, BS
University of California, San Diego
Case: Diffuse Large B cell Lymphoma (DLBCL), the
most common Non-Hodgkins Lymphoma, classically
presents as a rapidly growing mass fatal if untreated.
Here we present a case report of the prototypical presentation and clinical course of DLBCL. A 56 year old man
with three week history of a dental abscess presents to the
emergency department for evaluation of an enlarging
neck mass. His initial symptoms include a severe sore
throat and subjective fevers lasting one week that improved briefly with aspirin and fluids. Concurrent with
this he developed a large minimally tender, hard left
sided neck mass that grew over the course of three weeks
causing a feeling of fullness in his throat. He notes
“choking” sensation preventing him from lying on his
right side or back. He has no myalgias, nausea, vomiting
or night sweats, though reports intentional twenty-five lb
weight loss over three months. No report of recent rash,
arthralgias or changes in urinary or bowel habits. He has
no recent travel or exposure to un-pasteurized milk or
animals. On physical exam, he is comfortably lying in
bed without any visible respiratory distress. Neck exam
reveals a large, well circumscribed, hard mass extending
from angle of the jaw to clavicle and a 2-3 cm left tonsillar mass partially obstructing his posterior oropharynx.
He has no other lymphadenopathy, organomegaly, or skin
lesions. Lungs are clear, and no peripheral edema. On
admission, labs reveal a normal CBC and differential,
negative RPR. Bacterial culture from the mass grew pansensitive Klebsiella pneumoniae with negative acid fast
stains, fungal stains and cultures. Serologies were negative for Brucella, EBV, CMV. CT imaging revealed an
irregular rim enhancing fluid collection in the left lateral
neck surrounded by inflammation concerning for abscess
versus a mass of necrotic masses secondary to neoplasm.
He underwent an excisional biopsy and left tonsillectomy
by surgery. Flow cytometry revealed a monoclonal population of B-lymphocytes and pathology of the biopsy
showed high grade diffuse large B-cell lymphoma.
Workup for possible metastatic disease was initiated including CT chest, abdomen, pelvis followed by PET-CT.
All studies were negative for other involved sites. He
underwent a bone marrow biopsy that revealed no evidence lymphoma and was started on CHOP chemotherapy.
Discussion: This case report of rapid onset, history of
molar abscess and sore throat initially seem consistent
with an infectious. Clues to DLBCL include large, hard,
non-tender mass in the neck and night sweats. An infectious etiology would more likely be associated with
growth that is tender to palpation. DLBCL is known to be
an aggressive subtype of Non-Hodgkin’s lymphoma traditionally treated with chemotherapy regimens including
doxorubicin with rituximab adjuvant therapy to improve
overall survival and 5-year disease free intervals.
Atrial Fibrillation as a Complication of Anomalous
Pulmonary Venous Connection and Atrial Septal Imperfection
Ashkan Amooee, MD; Omar Darwish, DO; Alexander
Stein, MD; Ravi Menghani, MD; Jasminka Criley,
MD, FACP, FHM
St. Mary Medical Center and David Geffen School of
Medicine at UCLA
Case: A 44 year-old Hispanic man was admitted with a
2-day history of atypical chest pain. He was found to
have atrial fibrillation (Afib) with rapid ventricular response. His past medical history included Afib, HTN,
dyslipidemia, DVT and “heart murmur”. Physical exam
revealed an irregularly irregular heart rhythm and elevated JVP. ECG showed Afib with rapid ventricular rate
and right bundle-branch block. A β-blocker and digoxin
were instituted. Patient was ruled-out for myocardial infarction. Repeated cardiac examination detected a fixed
splitting of S2 and 2/6 midsystolic murmur best heard at
the 2nd LICS. Upon obtaining outside records it was discovered that the patient was worked up extensively 2
years prior with right- and left heart catheterization disclosing a sinus venosus atrial septal defect (SVASD) and
an anomalous pulmonary venous connection (APVC) but
“clean” coronary arteries. He had been offered surgical
intervention at that time, but had declined. At this admission, it was felt that patient would benefit from a surgical
repair of the ASD and APVC given impending risk for
worsening heart failure and related symptoms. He underwent repair with an autologous pericardial patch, covering the ASD and redirecting the pulmonary veins to the
left atrium through the septal defect. He also underwent a
modified maze procedure to eliminate Afib. Patient tolerated surgery well. Postoperatively, he required a dualchamber pacemaker due to persistent junctional rhythm
with heart rates ranging from 40-50. He was discharged
home in stable condition.
Discussion: Atrial fibrillation is the most common cardiac arrhythmia. One of the less common causes of Afib
is congenital heart disease. ASD is the most common
congenital cardiac lesion presenting in adults. SVASD
accounts for only 10% of the ASD’s. The more common
Sinus Venosus type defect occurs in the upper atrial septum and is contiguous with the superior vena cava (SVC).
It is almost always associated with anomalous pulmonary
venous drainage of the right upper pulmonary vein into
the SVC. People with congenital ASD can be asymptomatic through their 20’s, but by age 40, most will have
symptoms including dyspnea, Afib and eventually heart
failure. Individuals with small defects can live a normal
life, but larger defects require surgical correction. The
mortality from corrective surgery in people younger than
45 years of age is < 1%.
In the adult population, congenital anomalies are commonly associated with Afib. This is an unusual case of
SVASD with 4 anomalous pulmonary veins, who underwent surgical repair and maze procedure to eliminate
Afib. Untreated ASD’s are associated with a significantly
shortened life expectancy. Operation for SVASD is associated with low morbidity and mortality, and postoperative subjective clinical improvement occurs irrespective
of age at surgery. SVASD repair should be considered
whenever repair may impact survival or symptoms.
4
Innocent Presentation of a Malignant Pheochromocytoma
Gaja Andzel, MD; Silvana M. Giannelli, MD; Mauro
C. Bandeira Janoski, MD
Loma Linda University
Malignant Pheochromocytomas (MPCCs) are rare catecholamine producing tumors derived from chromafin
cells from the adrenal medulla. Their diagnosis can be
challenging and to date, there is no effective cure. We
present a case with atypical clinical picture to illustrate
manifestations, diagnosis and current treatment modalities.
A 71 year-old African-American male was evaluated for
a growing left adrenal incidentaloma. The mass was first
seen by CT in 1991 and had been stable at 4 cm for 15
years. During his initial visit he complained of 20-pound
weight loss in 6 months and fatigue. Past medical history
included chronic hypertension controlled on three agents,
stroke and diabetes. No relevant family history. His blood
pressure was 138/78 mmHg and pulse was 109 bpm. His
exam was only remarkable for cachexia. Twenty-four
hour urine collection showed very high normetanephrine,
slightly high norepinephrine, normal dopamine and normal epinephrine levels. Terazosin was started prior to left
adrenalectomy. Pathology was consistent with pheochromocytoma (PCC). Surgical margins were clear, but
the specimen showed necrosis and limphovascular invasion. Catecholamine levels normalized after surgery. Unfortunately, the patient was lost to the follow-up and returned after 2 years. PET/CT scan showed a recurrent 1.3
cm left adrenal mass and new multifocal liver lesions.
Patient underwent chemoembolization of liver lesions
which was complicated by liver abscess. Methyrosine
was started to control his blood pressure, as catecholamine levels were increased. Radiolabeled metaiodobenzylguanidine scintigraphy (MIBG) showed uptake limited
only to right hepatic lobe, the PET/CT scan was repeated
and showed multiple foci of intense activity in both lobes
of the liver as well as adrenal mass. The bone scan was
negative for metastasis. Based on these results chemotherapy with Cyclophosphamide, Vincristine and Dacarbazine (CVD) was recommended.
Although most MPCCs have a rapid progression they can
have a highly variable clinical course. Our case did not
have any of the classic triad of PCC (episodic headache,
sweating and palpitations). Main symptoms were weight
loss and fatigue, which are described occasionally. He
also had sustained hypertension, which is actually more
frequent than paroxysmal hypertension. Most PCCs produce both norepinephrine and epinephrine. Ours was a
rare case of adrenal PCC secreting norepinephrine alone.
Malignant and benign tumors are indistinguishable clinically; the only reliable criteria for malignancy are distant
metastasis. Alpha blockage and Methyrosine are useful
pharmacological treatments, but surgery is still the mainstay approach. Chemotherapy and MIBG have roughly
the same results, with relapse of most cases within two
years. Although prognosis is difficult to predict most
MPCCs have a 50% five-year survival rate. There are no
published randomized trials evaluating available therapies.
In conclusion, it is important to the clinicians to have a
working knowledge about adrenal incidentalomas, PCCs,
their identification and treatment options.
A Case of Coarctation of the Aorta in a Young Adult
Female
Anthony Arredondo, MD; Janine Vintch, MD
Harbor-UCLA Medical Center
A 19 year old woman with a history of HTN diagnosed 2
months prior to admission complained of a 2 week history of fatigue and light-headedness associated with intermittent palpitations. She denied chest pain, shortness
of breath, nausea, vomiting, or headache. She reported
strict adherence to her BP meds including HCTZ and
clonidine. BP 220/110 mm Hg, HR 89. She was in no
acute distress. PE was notable for no carotid bruits or
elevated JVP, lung fields clear to auscultation, a 2/6 systolic ejection murmur over the left sternal border, and
normal abdominal examination with no bruits. It was
noted that she had non-palpable distal pulses and skin
that was cool to touch in her lower extremities bilaterally
as compared to her upper extremities. Upon noting this
discrepancy in pulses and skin temperature, bilateral brachial and ankle blood pressures were measured. Her brachial blood pressures remained elevated and were equal,
while her ankle blood pressures were equal but measured
100 mg Hg lower systolic BP as compared to the brachial
readings. Electrolytes and renal function were normal.
Tests to evaluate secondary causes of hypertension were
unremarkable. Her chest x-ray was without any findings
including no evidence of rib notching CT angiogram
demonstrated coarctation of the aorta arising distal to the
left subclavian artery with the narrowest point measuring
only 7.5 mm. Several enlarged bronchial and intercostals
arteries were also noted. A transthoracic echocardiogram
confirmed this radiographic finding and measured a peak
transcoarctation velocity of 4.7 m/s and a peak gradient
of 89 mm Hg. Renal ultrasound demonstrated tardusparvus waveforms in blood flow to both kidneys; a renal
captopril scan showed normal perfusion and cortical uptake bilaterally. BP was controlled with three oral medications; she was discharged with follow-up with cardiothoracic surgery.
The presence of HTN requiring medical therapy in young
adults may suggest the presence of a number of conditions including coarctation of the aorta, fibromuscular
dysplasia, or an endocrine disorder such as pheochromocytoma, primary hyperaldosteronism, or Cushing’s Disease. The clinical clue to this patient’s diagnosis was not
only the young age of onset for severe HTN, but also the
presence of non-palpable pulses in her lower extremities
concerning for coarctation of aorta.
5
May-Thurner Syndrome: Coumadin Failure or
Treatable Condition?
Benton Ashlock, MD
Scripps Green Hospital
A 57 year-old female with a history of well controlled
hypertension presented to the ED with acute onset of severe left leg pain, swelling, and discoloration. Three
months prior, she suffered a pulmonary infarction and
was found to have a concurrent 15cm left ilio-femoral
thrombosis. At that time anticoagulation initiated and due
to the size and appearance of the thrombus and inferior
vena cava filter was placed. A CT-venogram performed
at the time of the current admission demonstrated flattening of the left common iliac vein as it passed under the
right common femoral artery with thrombosis extending
down to the popliteal vein. A large embolic-like thrombosis on the inferior vena cava filter was also noted. Lab
records indicated she remained therapeutically anticoagulated during the three months between incidents. She subsequently underwent successful thrombolysis with tissue
plasminogen activator which resulted in complete clearing of the thrombosis. Reduced flow continued to be present despite absence of thrombosis. Based on the CT
venogram and the continued reduced blood flow, a diagnosis of May-Thurner syndrome was made.
May-Thurner syndrome describes the occurrence of deep
vein thrombosis due to significant compression of the left
common iliac vein by the overlapping proximal right
common iliac artery. Anatomically the overlap of theses
two vessels is normal but in some patients, particularly
young women, the elevated arterial pressure and thickness of the vascular wall of the overlying iliac artery produces sufficient venous compression resulting in hemodynamic compromise and subsequent venous thrombosis.
External vascular compression from May-Thurner Syndrome or other external compressive forces should be
seriously entertained in all patients for whom therapeutic
anticoagulation is unsuccessful in preventing thrombosis
recurrence. A small number of case series have reported
excellent long term success with iliac vein stent placement combined with short term anti-coagulation.
If undiagnosed, patients with May-Thurner syndrome
routinely continue to have thrombosis despite therapeutic
anticoagulation. Patel, et. al. demonstrated that these patients can remain symptom free after thrombolysis and
venous stent placement. Early recognition of MayThurner syndrome can prevent the severe morbidity and
mortality associated with recurrent deep venous thrombosis as well as chronic anti-coagulation. CT venography to
evaluate for extrinsic vascular compression should be
considered, particularly in patients with large, proximal
deep vein thrombosis of the left lower extremity and in
all such patients who fail appropriate anticoagulation
therapy.
Steroid-Induced Hepatotoxicity in a Patient with
Transverse Myelitis
Kimberly Atianzar, MD; Cong Nguyen, MD; Mary
El-Masry, MD; John J. Kim, MD; Neil Kaplowitz,
MD
University of Southern California
Hepatotoxicity is an uncommon clinical course in patients requiring steroids. We report the case of a patient
with transverse myelitis in which treatment with a 3-day
course of high dose steroids resulted in acute hepatitis
and cholestatic injury. A 52 year old Hispanic Male with
a history of chronic hepatitis C and hypertension presented to the Emergency Department with jaundice, dark
brown urine, generalized fatigue, fecal incontinence, and
progressively worsening bilateral lower extremity weakness and numbness over the past 3 weeks. He denied any
other systemic symptoms. Pertinent physical findings
included bilateral sclera icterus, jaundice and 3/5 strength
of lower extremities bilaterally. Remaining physical examination was unremarkable. Initial laboratory workup
showed normal basic metabolic panel and complete blood
count. Liver function panel was normal with the exception of aspartate aminotransferase (AST) level of 52 U/L
and alanine aminotransferase (ALT) level of 77 U/L.
MRI of the lumbar spine was unremarkable and showed
no evidence of cord compression. CT scan of the brain
was unremarkable. Lumbar puncture was negative for
evidence of infection. The patient was treated with a 3day course of 1gram IV Methylprednisolone for transverse myelitis in which his symptoms significantly improved, and he was transferred to a rehabilitation facility.
During his physical rehabilitation, the patient again complained of worsening bilateral lower extremity weakness.
He was treated with a second 3-day course of 1gram IV
Methylprednisolone followed by a tapering course of oral
Prednisone. The patient’s symptoms improved after the
pulse course of IV steroids but subsequent AST and ALT
levels elevated to 177U/L and 235U/L respectively. Total
bilirubin level was 11.8mg/dL and direct bilirubin level
was 7.8mg/dL. He was admitted back to the hospital for
acute hepatitis and cholestatic injury. HIV, RPR and
Hepatitis B serology were negative. Abdominal ultrasound showed multiple gallstones, portal hypertension
and no evidence of biliary dilatation. CT scan of the abdomen and pelvis showed gallstones in a non-distended
gallbladder. MRCP showed multiple gallstones, cirrhotic
liver, and no choledocholithiasis. His liver function panel
continued to improve during his hospitalization but remained elevated at discharge with an AST of 114U/L,
ALT of 78U/L, total bilirubin of 8.4mg/dL and direct
bilirubin of 63mg/dL. Two months later during his outpatient visit, the patient’s liver function panel returned to
baseline with an AST of 57U/L, ALT of 81U/L, total
bilirubin of 0.9mg/dL, direct bilirubin of 0.2mg/dL.
While other causes of liver injury must be excluded, steroid-induced hepatotoxicity, although rare, should always
be considered in patients with transaminitis after receiving therapy with high dose IV steroids. Clinical course is
highly variable ranging from asymptomatic transaminitis
6
to fulminant hepatic failure with improvement of symptoms by discontinuation of the offending steroid.
Dasatinib Induces HUS and Pulmonary Hypertension
Ashkan Attaran, MD; Reza Rafie, MD; Siamak Sadaat, MD; Sharmineh Shams, MD
University of Southern California
Introduction: Pulmonary hypertension has been reported
in hemolytic uremic syndrome (HUS). There are several
previous case reports about the development of HUS with
different chemotherapy medications but there is no previous report about the side effects of dasatinib. In this case
report, for the first time we describe the development of
pulmonary HTN in the context of HUS in patient treated
with datasinib.
Clinical presentation: Patient is a 26 yo female with no
pmhx, that initially presented with fibrolamellar cancer of
liver. Patient was initially treated with nexavar and due to
lack of response then was subsequently switched to
dasatinib. Two months into treatment with dasatinib patient developed HUS and at that time treatment was
stopped. Patient was treated by transfusions. Subsequently patient started to develop SOB on exertion, and
eventually SOB at rest. Patient was then admitted, initially suspected of heart failure, vs. PE, both were R/O.
On cardiac Echo patient’s RV pressure was measured to
be greater than 60, and Dx of Pulmonary hypertension
was made. At that time it was decided that the patient
needs to be treated with plasma exchange. Patient received 6 treatment of plasma exchange with no improvement. It was decided that the patient needs to be started
on Eculizumab.
Discussion: Previous studies showed that HUS can result
in pulmonary hypertension. The underlying pathology
that predispose to pulmonary vascular disease in these
patients has not been well defined. Different mechanisms
such as endothelial dysfunction, decreased nitric oxide
bioavailability and enhancement of coagulopathy have
been suggested. Mitomycin C is an alkylating agent that
reported to cause pulmonary hypertension and HUS. In
this report we described the patient with underlying
fibrolamellar CA of liver that was treated with dasatinib
.She developed renal failure along with other signs of
HUS that was subsequently complicated with pulmonary
HTN. There has been no reported case of dasatinib as a
potential cause of HUS, or Pulmonary HTN.
LV Non-Compaction in Family with History of Sudden Cardiac Death
Ashkan Attaran, MD; Reza Rafie, MD; Amir Tahbaz,
MD; Sharmineh Shams, MD; Ali Farvid, MD
University of Southern California
Left ventricular (LV) non-compaction manifests as layering of myocardium of left ventricle with compacted and
non-compacted layers. LV non-compaction is being di-
agnosed if the maximum ratio of non-compacted to compacted layer is > 2.3/1. We present a patient with palpitations, that had family history of sudden cardiac death. His
cardiac MRI showed left ventricular non-compaction.
Case: A 42 yo Caucasian male with no PMH requested a
cardiac work-up. He gave a family history of sudden cardiac death in three family members at a young age. EKG
did not reveal any abnormalities, cardiac Echo was suspicious for LV non-compaction. This was confirmed by
Cardiac MRI showing LV non-compaction to compaction
ratio of 2.88/1.
Discussion: Left ventricular (LV) non-compaction has
been described as arrest in the compaction process of
fetal myocardium that can manifest in adult age as heart
failure, atrial and ventricular arrhythmias and thromboembolic events. Although echo data has been used to
diagnose but recent studies showed increased specificity
with using cardiac MRI. The patient that we described
here had sudden cardiac death in his immediate family
that made us suspicious for structural heart disease. Subsequent echo and cardiac MRI were (+) for left ventricular pathology. Cardiac MRI showed the left ventricular
non-compaction to compaction ratio 2.5/1 that confirmed
our clinical suspicion. LV non-compaction should be
more thoroughly evaluated in patients with unexplained
heart failure and history of sudden cardiac death in first
degree relatives.
Normal BNP in Heart Failure
Ashkan Attaran, MD; John Fan, MD; Leslie Saxon,
MD
University of Southern California
Introduction: Brain natriuretic peptide (BNP) has diagnostic and prognostic significance in patients with heart
failure. BNP has been considered as a sensitive marker
for early diagnosis of heart failure. In this case report we
describe a patient with advanced heart failure that despite
of requirement for cardiac resynchronization therapy always had normal values of BNP. another interesting aspect of this case is that this atient is one of few patients in
the country that their heart failure is being followed by a
left atrial pressure device. This device transmits the left
atrial pressure to a hand held device, and informs the patient if they need to adjust their medications in order to
better control their heart failure.
Case presentation: Pt is 54 yo female with pmhx sig for
dialted cardiomyapathy, that initially presented with
SOB, fatigue, DOE. Echocardiogram showed global hypokenesis, and elevated left atrial pressure. Patient symptomatically was classified as NY class II, however this
patient’s serum BNP levels were low normal level.
Discussion: Previous studies showed the sensitivity of
BNP to diagnose heart failure is 97-99%. Subsequent
studies showed normal level of BNP in minority but quite
significant number of patients with heart failure. Young
7
women with non ischemic cardiomyopathy constitute the
major part of heart failure patients with normal BNP
level. The patient that we described here had history of
non ischemic cardiomyopathy status biventricular pacemaker placement that serial checking of BNP level
showed maximum that was in normal range. Although
BNP is very sensitive marker to diagnose heart failure, it
never should substitute the clinical judgment. Left atrial
pressure device allows the patient to have an active role
in mangment of their heart failure, and could eventually
cut down on frequency of office visit. This device will
transmit left atrial pressure, to a hand hel device, and
based on this information the physician will set two sets
of therapy. First set is of constant regimen of medical
therapy (beta-blockers, ace inhibitors,…) and a second
set of dynamic therapy. Device will check left atrial pressure, and if the left atrial pressure is elevated then the
patient will take a PRN dose of diuretics, or afterload
reducing medications. These data are reviewed and the
physician can make adjustment as needed. This device
could potentially decrease the frequency of office visits
as well as decrease the frequency of CHF exacerbation,
and could cut down the cost of heart failure management.
only evident in V3-V6, however this patient displayed
early repolarization in all leads.
Discussion: ST segment elevation has broad differential
diagnosis such as myocardial infarction, pericarditis,
myocarditis, ventricular hypertrophy , hyperkalemia. One
of the main causes of ST elevation of J point in young
healthy people is normal early repolarization variant.
Although early repolarization involves V3-V6 most of
the time but it can have a diffuse pattern. One of the
methods that can differentiate the acute pericarditis from
early repolarization is the ratio of ST elevation to T wave
amplitude in lead V6 .If the ratio exceeded 0.24 the diagnosis will be most likely acute pericarditis. The patient
that we described here had diffuse ST elevation in all
leads That by using the ST/T ratio in lead V6, the diagnosis would be 0.30 . He had drug seeking behavior and
used his ECG as a tool to get pain medications.
West Nile Viral Myocarditis
Ashkan Attaran, MD; Reza Rafie, MD; Sharmineh
Shams, MD; John Fan, MD; Leslie Saxon, MD
University of Southern California
Pericarditis Versus Early Repolarization
Ashkan Attaran, MD; Reza Rafie, MD; Sharmineh
Shams, MD; Ali Farvid, MD; M. Pashmforoosh, MD
Introduction: Early repolarization manifests as J point
ST elevation that commonly can be detected in leads V3V6. It is a major ECG diagnostic dilemma. Sometimes it
is difficult to differentiate early repolarization from
STEMI or pericarditis. We present a patient with diffuse
ST elevation that was diagnosed as pericarditis but retrospective inspection of his cardiac echo confirmed the
early repolrization.
Case presentation: Patient is a 42 yo aam with PMHx
sig for reported recurrent pericarditis, and CAD (per patient) presented with sever pluritic chest pain. Patient
described the pain as sharp, 10/10 in intensity, without
radiation. Patient stated that his chest pain was worse
when lying in supine position, and was somewhat alleviated when leaning forward. Patient also stated that he has
had hx of NSTEMI, in Minnesota. Patient’s EKG showed
diffuse ST segment elevations in all leads. Patient refused
NSAIDs for treatment, and stated that his chest pain only
was alleviated by IV Dilaudid. Given the significant
CAD hx that the patient was claiming, we contacted the
hospital that he was getting care in, in Minnesota. The
records from outside hospital showed that the patient was
admitted frequently with the same symptoms, and had an
extensive work-up including cardiac ECHO, as well as
cardiac MRI, which showed no evidence of pericarditis,
off interst it was also established that the patient had no
history of CAD(established by cardiac catherization). On
follow-up exam it was established patient had drug seeking behavior, and that his EKG was representive of a
normal variant of early repolariztion. This EKG was interesting because in most cases, early repolarization is
Introduction: West Nile virus (WNV infection usually
present as self limited febrile illness with fever, HA and
myalgia. There are several previous case reports about
pathologically diagnosed myocarditis, with all cases leading to patient’s demise. There is limited data clinically
correlated cardiac dysfunction secondary to WNV infection. In this case report we describe a patient with WNV
cardiomyopathy that showed cardiac function abnormality.
Case: Patient is a 27 yo previously healthy female with
no PMH. Presented with flu like symptoms, and then
suffered a syncopal episode. Patient was admitted and
initial work-up showed normal cardiac function, however
her clinical picture worsen over the following two days.
Patient started to develop dyspnea on exertion initially,
then dyspnea at rest. Patient was placed on telemetry and
was noticed to have episodes of v-tach. Echo showed
EF15%. At this time patient’s WNV titers came back
high. Based on clinical presentation a diagnosis of viral
myocarditis was made patient was started on IV prednisone, as well as for hemodynamic support patient was
supplemented with intra-aortic balloon pump, as well as
an ECHMO device. Cardiac MRI and cardiac biopsy
were also performed. MRI showed pericardial enhancement, as well as delayed enhancement in the subendocardium. Biopsy specimen was then send for immunohistochemical staining, which subsequent result confirmed
diagnosis of west Nile myocarditis. Patient’s condition
improved over the course of one week. Repeat cardiac
echo showed EF 55-60%, and patient’s frequency of
arrythmias decreased. Patient was subsequently discharged with a life vest, and close follow-up.
Discussion: Only 20% of patients with West Nile virus
infection become symptomatic. It can also present with
8
severe neurologic manifestations such as meningitis or
encephalitis. Rare presentation of this infection can be
rhabdomyolysis, hepatitis, and pancreatitis. Myocarditis
is one of the unusual presentation for these patients.
There are 2 previous case reports about cardiac arrhythmia and myocarditis in patient with West Nile virus infection that in both of these case reports, Patients died of
possible fatal arrhythmia. The patient that we described
here showed evidence of myocarditis with rhythm abnormalities (episodes of V-tach, and frequent PVCs). As
the diagnosis for cases with Wet Nile virus increases, we
should pay more attention to unusual but clinically important aspect of this infection such as myocarditis. Future studies may show the necessity of close monitoring
of WNV infected patient with any sign or symptoms of
cardiac involvement.
Aortic Valve Thrombosis Presenting as Non-ST Elevation Myocardial Infarction
Emil Avanes, MD; Grace Nam, MD; Kenneth Narahara, MD
Harbor-UCLA Medical Center
A 73 year old man presented with 9 hours of constant
nonexertional, pressure-like chest pain. He also complained of shortness of breath, a decreased exercise tolerance, and 2 pillow orthopnea, but no PND. He denied
upper respiratory symptoms/fevers. He had a past h/o
diabetes, hypertension, and myocardial infarction 9 years
previously with subsequent coronary bypass grafting and
aortic valve replacement (AVR). On exam, the patient
appeared comfortable with vital signs: T 97, BP 127/58,
HR 62, RR 18, oxygen saturation 96% on 2L oxygen. He
had normal jugular venous pressure but rales ½ up the
back bilaterally with muffled aortic closure sound. A
grade II/VI systolic murmur that radiated to the carotids
was present as was a left-sided S3 gallop. Routine lab
values were notable for INR of 1.2 and troponin I of 0.45
(normal <0.4). The EKG demonstrated sinus rhythm, 1°
AV block and nonspecific ST-T changes. An enlarged
heart and bilateral pleural effusions were seen on X-ray.
A TTE revealed normal RV and LV function with LVH.
A mechanical aortic valve was present but the leaflets
were not well seen. A 49 mmHg gradient was recorded
across the prosthesis. Aortic valve thrombosis from inadequate anticoagulation was suspected. Initially, the
patient was treated with aspirin and a heparin infusion.
Warfarin was restarted. Over the next 4 days, the patient’s heart sounds took on a more normal mechanical
quality, the S3 and rales disappeared. A repeat TTE revealed essentially normal aortic valve function and a
transesophageal echocardiogram (TEE) confirmed the
absence of significant thrombus. The patient was discharged with an INR of 3.2.
Mechanical heart valves were introduced in the 1950s.
Our patient had a St. Jude bileaflet valve, a model in use
since 1977. The recommended INR for a St. Jude valve is
2.5-3.5 although one series recently suggested an INR of
2 for an AVR. The AHA/ACC recommends INRs based
on risk assessment. High risk patients (h/o atrial fibrillation, left ventricular dysfunction, hypercoagulable state,
or history of previous thromboembolism) should maintain
INR from 2.5-3.5 whereas those without these risk factors
should aim for 2.0-3.0. When prosthetic heart valve
thrombosis is diagnosed, there are 3 treatment modalities:
surgery, fibrinolysis, or unfractionated heparin. Thrombus size & prior stroke are independent predictors of
complications with thrombolytic therapy. Traditionally,
primary care physicians & anticoagulation clinics have
managed warfarin dosing for patients with prosthetic
valves, but self-management methods have the potential
for maintaining more accurate and stable INRs.
A Benign Tumor and a Great Imitator
Kendan Baban, MD; D. Rebenson, MD; M. Kyle, MD
Scripps Green Hospital
A 61 year-old female admitted to the hospital with one
day history of slurred speech and left arm weakness. Her
past medical history was unremarkable. Her review of
system was significant for twenty pounds weight loss,
chills, fatigue, myalgia, arthralgia and bilateral lower
extremities painful rash with paresthesias for 3 months.
On examination patient was slightly hypertensive, cardiovascular exam revealed loud S1, normal S2 with regular rate and rhythm and no murmurs noted. Her neurological exam was remarkable for left facial droop and 3/5
left arm muscle strength with discordant finger to nose.
Examination of her extremities revealed blanching papules on the palms of hands and the sole of foot bilaterally.
Initial laboratories revealed no leukocytosis, and normal
electrolytes. Previous workup included skin biopsy,
cryoglobulin level, UPEP, SPEP, ESR, ANCA panel,
ANA titer which were all normal. Brain MRI/MRA on
admission revealed multiple foci of restricted diffusion in
both hemispheres, and all lobes of the brain as well as
both cerebellar but spearing the brainstem. The multiple
vascular distributions suggested multiple small infarcts
from an embolic source. Based on the finding on the MRI
and her constitutional symptoms TTE was performed.
Initial TTE showed EF of 72% and a large highly mobile
mass plopping through the mitral valve orifice suggestive
of left atrial myxoma. A transesophageal echocardiogram
confirmed the presence of left atrial myxoma. The patient
promptly under went cardiac surgery to prevent further
embolization. A 5.2 x 4 x 1.8 cm gelatinous, red brown
aggregate of friable locally bloody tissue was resected
from the left atrium. Pathology indicated benign myxoma
with chronic hemorrhage. Patient tolerated surgery well
and underwent extensive physical rehab. At three months
follow up the patient was completely recovered from her
symptoms of fatigue, rash and weakness. Primary cardiac
neoplasms are rare with reported prevalence in autopsy
series of 0.0017-0.03%. Approximately 80% of primary
cardiac tumors are benign. The most common cardiac
neoplasm is myxoma. Patients with cardiac myxoma present with a wide range of clinical signs and symptoms,
which in part depend on the location and morphology of
the tumor and its tendency to embolize. The most com-
9
mon presenting symptoms being heart failure, but it may
also present with constitutional symptoms, lethargy, arthralgias, and petechiae. This case domenstartes a life
threatning presentation of histologically benign cardiac
tumor. High index of suspicion is fundamental in clinical
approach and correct diagnosis of cardiac myxoma because of the potential life-threatening sequelae of this
tumor. In addition this tumor can mimic not only every
cardiac disease but also, inefective, immounologic and
malignant processes.
Acute Herpes Hepatitis in Pregnancy
symptoms of HSV hepatitis include fever, transaminitis,
abdominal pain, encephalopathy, and coagulopathy.
Vaginal or oral lesions are present in only 50% and jaundice is uncommon. Definitive diagnosis requires liver
biopsy with immunohistochemical staining and PCR
techniques. Treatment is high dose intravenous Acyclovir
although liver transplantation should be considered for
failure of therapy or further clinical deterioration. HSV
hepatitis must be considered in febrile pregnant patients
with anicteric transaminitis as early detection and initiation of antiviral medication is associated with close to
100% survival.
Vidhya Balasubramanian, MD; Sarah Khan, MD;
Erica Palys, MD;
Suzanne Donnovan, MD, MPH; Stanley Dea, MD;
Glenn Mathisen, MD
Olive View-UCLA Medical Center
PCP Pneumonia and Granulomas?
Case: A 17 year old G1P0 female presented at 34 weeks
gestation with three days of fevers, right flank pain and
uterine contractions. Her medical history was significant
for a Chlamydia infection that was treated three months
prior to admission. She denied recent travel and had one
male sexual partner. Exam revealed a fever to 39.2C and
right flank tenderness but no vaginal lesions. Fetal monitoring revealed tachycardia. Ceftriaxone was started for
presumed pyelonephritis as urinalysis had one WBC/hpf.
On hospital day four, antibiotics were broadened and she
underwent emergent cesarean section due to preterm
premature rupture of membranes and concern for
chorioamnionitis. Despite an uncomplicated delivery of
the baby, her fevers persisted so other sources of infection were sought. Serologies and PCR were sent for viral
hepatitis, mycobacterium, coxiella, Herpes Simplex Virus
(HSV), Cytomegalovirus, EBV, Varicella, Cryptococcus,
Coccidiomycosis and HIV. Blood and placental cultures
remained negative. Labs on hospital day seven revealed
AST 2906 units/L, ALT 675 units/L, Alkaline Phosphatase 184 units/L, Total Bilirubin 0.6 mg/dL, INR 1.91
and Albumin 1.7 mg/dL. CT scan of the abdomen and
pelvis showed hepatomegaly and multiple small low attenuation lesions in the liver. Serologies for HSV-1 (IgG
1.34), HSV-2 (IgG >6.0) and HSV 2 PCR were positive.
All other tests were negative. Given the concern for fulminant hepatic failure, the patient was transferred to the
Intensive Care Unit under the medicine service. On hospital day eleven, the patient underwent liver biopsy which
showed patchy necrotizing hepatitis and stains for HSV
were weakly positive. Cultures were later positive for
HSV-2. The patient was started on high dose intravenous
Acyclovir and her abdominal pain, fevers and transaminitis resolved. The baby had a negative evaluation for congenital HSV-2.
Introduction: Hypersenstivity pneumonitis (HP) is an
inflammatory condition caused by immunological reactions to environmental antigens. This case will review the
details of this disease. However, the major learning objective will be for the clinician to remain open to the idea
of having co-existing diagnoses.
Discussion: Hepatitis is a rare presentation of HSV infection in immunocompetent patients. Fulminant hepatic
failure can progress quickly with an untreated mortality
rate of 80-90%. Pregnant patients are at increased risk for
HSV hepatitis and in the 24 cases reported from 19691999, all occurred during the third trimester. Common
Vidhya Balasubramanian, MD; Nader Kamangar,
MD, FACP
Olive View-UCLA Medical Center
Case: A 29 year old male presented after a syncopal episode. On review of systems he endorsed having one
month of dry cough, fevers, and anorexia. His social history was significant for tobacco use and unprotected heterosexual intercourse. The patient was born in Guatemala
and moved to Idaho four years prior to admission where
he worked as an agricultural laborer. He denied any other
travel history or animal exposures. Notable findings on
physical examination included a temperature of 38.6C ,
HR of 50 bpm, but no evidence of respiratory distress.
Laboratory results showed a WBC of 5,200 cells/uL with
lymphopenia. CT scan of the chest revealed diffuse
ground glass opacities and extensive reticulonodular and
centrilobular infiltrates predominant in the bilateral upper
lobes. He was started on Ceftriaxone and Levofloxacin,
as well as on treatment for Tuberculosis given a high
clinical suspicion. Additional laboratory tests revealed a
positive HIV screen with a CD4 count of 1 (0%). Cryptococcal antigen, coccidiomycosis antibody, and urine
histoplasmosis antigen were all negative. On hospital day
3, the patient underwent bronchoscopy. The bronchoalveolar lavage (BAL) AFB smears were negative while
Pneumocystis jirovecii Pneumonia (PCP) DFA stains
were positive. The pathology report on the transbronchial
biopsies showed Pneumocystic organisms, however, it
also showed non necrotizing granulomas. The patient was
started on Bactrim with improvement in symptoms.
However, the radiographic findings and pathology report
were not consistent with PCP. Given his occupational
exposure, the notable centrilobular ground glass nodules
seen on CT, and the non-necrotizing granulomas on
transbronchial biopsy, a diagnosis of concurrent subacute
hypersensitivity pneumonitis was made.
10
Discussion: Diseases characterized by profuse centrilobular nodules includes subacute hypersensitivity pneumonitis, respiratory bronciolitis-interstiial lung disease
(RB-ILD), diffuse panbronchiolitis, endobronchial spread
of tuberculosis, and cryptogeneic organizing pneumonia.
The only one of these entities also associated with noncaseating granulomas is HP. HP encompasses more specific diseases such as bird fancier’lung and farmer’s lung.
This patient likely had farmer’s lung, where aspergillus
has been identified as the major antigen. Clinically these
patients present with acute onset of cough, fevers, and
dyspnea after exposure to the offending agent. Characteristic CT findings in the subacute stage are discussed
above while chronic disease causes fibrotic changes. Diagnosis is based on a combination of clinical history with
known environmental exposure, radiographic findings,
and biopsies showing non-caseating granulomas. Treatment includes removal of the offending antigen and glucocorticoids for those with persistent symptoms. The
majority of patients recover and have no functional limitations.
Transfusion Related Lung Injury and Cardiac Stent
Thrombosis
Raj Balasundaram, MD; Sarah Elsayed, MD
University of Southern California
This case identifies a blood transfusion causing both
TRALI (transfusion related lung injury) and cardiac stent
thrombosis in 3 RCA stents. A 64 year old male with a
past medical history of COPD presented with the chief
complaint of left sided chest pressure, 10/10, radiating to
his back and with nausea/vomiting, shortness of breath,
and diaphoresis. The patient was diagnosed with an
STEMI and taken for cardiac catheterization. He was had
3 vessel disease including total occlusion of the right
coronary artery. Balloon angioplasty of the RCA was
performed. After the cardiac catheterization the patient
was started on Aspirin, Plavix, and Integrellin. He developed AV block with bradycardia and was subsequently
started on a dopamine drip. The patient then developed
hemoptysis and was intubated for airway protection. He
was weaned off pressors and extubated, however, on hospital day #5 the patient again developed AV block and ST
elevation in the inferior leads. Three bare mental stents
were then placed in the RCA. The patient remained stable
until hospital day #12 when he was transfused four units
of packed red blood cells for a progressive decrease in Hb
from 14.0 to 7.8. Four hours after the transfusion the patient developed tachypnea, oxygen saturation decreased
to 75% and he was reintubated. EKG again showed inferior ST elevation. Trans-thoracic echo showed an EF of
65%. The wedge pressure obtained from the Swan Ganz
catheter was 13. CXR showed bilateral patchy infiltrates,
right > left, consistent with ARDS secondary to TRALI.
Cardiac catheterization revealed total RCA occlusion,
including thromboses in all prior RCA bare metal stents.
The patient survived after wire passage and additional
stent placement to the RCA. TRALI is defined as acute
lung injury caused by a transfusion of blood products
occurring within six hours after a transfusion. Known
causes of TRALI include transfusions of platelets,
PRBC’s, whole blood, and stem cells. It is the leading
cause of transfusion related mortality in the United
States. Acute or subacute thrombosis after coronary dilatation is a dramatic event with high mortality. Three cases
by Cornet have described patients who had been treated
with coronary stents and anti platelet therapy. Patients
subsequently suffered GI bleeds requiring platelet transfusions causing stent thrombosis. 2/3 patients died and
have no angiographic evidence of thrombosis. One patient survived after wire passage and placement of another stent. This case represents a transfusion of packed
red blood cells causing both TRALI and stent thrombosis,
raising a new risk for the transfusion of packed red blood
cells following cardiac catheterization.
Diffuse Pulmonary Hemorrhage in an Elderly Patient:
An Uncommon Presentation of Wegener's Granulomatosis
Linda Barkodar, MD; Meika Fang, MD
Cedars-Sinai/ Greater Los Angeles VA Healthcare
System
Wegener's Granulomatosis (WG) is a disease with a
characteristic necrotizing granulomatous vasculitis of
small and medium sized blood vessels, which principally
affects the kidneys and the upper and lower respiratory
tracts. It is an uncommon and diagnostically challenging
disorder that is usually revealed through a combination of
clinical criteria, serum cytoplasmic antineutrophilic cytoplasmic antibody (c-ANCA) and histology. WG usually
presents by the sixth decade, and the typical lesions in the
lung consist of necrotizing granulomatous inflammation
associated with a necrotizing vasculitis. We describe an
unusual case of WG in which massive pulmonary hemorrhage was the initial manifestation in an 87 year old male.
The late age of presentation along with isolated massive
pulmonary hemorrhage without necrotizing nodules is
rare and presents a difficult diagnostic challenge for the
clinician in the acute care setting. The diagnostic criteria
as well as management of this disease will be reviewed
here as well.
Hey Doc, Why Are My Eyes Yellow?
Robert Basseri, MD; Benjamin Kianmahd, BA
Dept. of Internal Medicine, Cedars-Sinai Medical
Center/Greater Los Angeles VA
The patient is a 30-year-old female with a history of depression, who presented to Cedars Sinai Medical Center
after being told by a co-worker, that “her eyes were yellow.” The patient stated that she had been feeling well
until approximately one month prior to presentation when
she noticed the onset of decreased appetite and increased
fatigue. She herself did not note any yellowing of her
eyes or skin. She notes that she drinks alcohol on a social
basis, which includes 5 to 6 alcoholic beverages per
week. She denies associated abdominal pain, abdominal
11
fullness, nausea, vomiting, fever, chills, or sick contacts.
She denies a change in bowel habits. She denies bright
red blood per rectum, hematochezia or melena. She denies any prior notice of intermittent jaundice of the skin
or scleral icterus. She denies a known history of liver
disease or history of liver disease within her family. She
denies heavy use of Tylenol. The patient works as a
bookkeeper, has a 10 pack year history smoking although
she has been tobacco free for over 5 years, drinks five to
six alcoholic beverages per week and denies intravenous
drug use. She has a family history of brain cancer and
alcoholism.
The patient was afebrile and her vitals were within normal limits. In general she was an ill-appearing, cachectic,
and jaundice female appearing older than stated age. She
had obvious scleral icterus. Pertinent abdominal exam
findings include hepatomegaly, ascites and presence of a
fluid wave. Furthermore, mild pitting edema bilaterally
with right greater than left to the level of the ankles. Slit
lamp examination demonstrated an early KayserFleischer ring in the patient’s right eye.
Pertinent workup laboratory values include mildly elevated liver function tests and total bilirubin, and low total
protein, as well as elevated CA 125 (663), CA 19-9
(113), urinary copper and low serum ceruplasmin. Of
note, CEA, AFP, alpha-1 antitrypsin, smooth muscle antibody, EBV, hepatitis B surface antigen, mitochrondial
antibody, and anti-nuclear antibody were all negative on
admission. CBC demonstrated a mild anemia and macrocytosis, however peripheral smear did not demonstrate
polychromasia or signs of hemolysis. Ultrasound imaging
demonstrated hepatomegaly, a heterogeneous appearance
consistent with fatty infiltration of the liver as well as
splenomegaly and ascites. Liver biopsy was performed
and ultimately diagnosed Wilson’s disease.
Discussion:
Wilson’s disease is an autosomal recessive genetic disorder where excessive copper accumulates in the liver and
brain, causing liver failure and neuropsychiatric conditions. This case highlights the importance of a complete
history in diagnosing a rare condition (incidence
1/100,000 patients), with textbook presentation. Recognition of new onset neuropsychiatric symptoms in the context of acute liver failure and the discovery of kayser
flescher rings are key in the diagnosis of Wilson’s disease. Diagnosis is facilitated with serum ceruloplasmin,
urinary copper excretion, and ultimately liver biopsy with
special copper stains.
Hepatosplenic T-Cell Lymphoma in a Young Man
with Crohn’s Disease
Jennifer Bennitt, MD; Hillary Bownik, MD
University of California, Los Angeles
Hepatosplenic T-cell lymphoma (HSTCL) is a rare form
of non-Hodgkin’s lymphoma and comprises only 5% of
peripheral T-cell lymphomas. While the majority of cases
develop de novo, approximately one-third of cases have
been associated with organ transplantation or other conditions that require immune-modifying agents. We present
a case of HSTCL in a 22 year-old male with Crohn’s disease previously treated with infliximab and 6mercaptopurine. Two years after his last course of infliximab, he developed fevers and jaundice, with associated hepatomegaly and marked splenomegaly, and acute
leukemia, which are typical features of HSTCL. The diagnosis was confirmed with flow cytometry and bone
marrow biopsy. Because it is so rare, recognizing the
intersection between the clinical features and those patients at risk of developing HSTCL is paramount to making the diagnosis. The clinical features, treatment options,
and prognosis of HSTCL will be discussed. In addition
the evidence suggesting an association between HSTCL
and inflammatory bowel disease is reviewed.
Neurofibromatosis Presenting as SVC Syndrome
Antonio Bonet, MD; Shefali Samant, MD; Mallory
Witt, MD; Fernando Antelo, MD
Harbor-UCLA Medical Center
Case: A 39 year-old Caucasian female presented to the
emergency department complaining of right neck pain
and swelling of one day’s duration. Her symptoms were
preceded by a sensation of fullness and soreness in the
superior right chest. She did not use tobacco, and denied
shortness of breath, cough, hemoptysis, dysphagia,
changes in her voice, fevers, chills, or weight loss. On
physical examination, multiple café au lait spots were
noted on her back, chest and arms, with axillary freckling
and fleshy nodules. Prominent right-sided neck veins
were noted, along with right sided supraclavicular swelling, facial fullness, and venous dilatation over the chest
wall, consistent with superior vena cava syndrome No
underlying neck mass was palpable. Auscultation of the
lungs revealed decreased breath sounds in the right upper
lung field. Upon further questioning, the patient reported
that she and her son had both been given a diagnosis of
neurofibromatosis by previous physicians. Computed
tomography (CT) imaging of the chest revealed a 9 cm
round, heterogeneous mass in the right upper lobe. The
patient underwent CT-guided biopsy of the lung mass,
and examination of the tissue was consistent with neurofibroma, with positive S-100 stain and a high mitotic
index.
Discussion: This patient presented with features of superior vena cava syndrome (SVC syndrome) secondary to
neurofibromatosis. Neurofibromatosis type 1 is an autosomal dominant disease that manifests as cutaneous café
au lait spots and neurofibromas, with thoracic rib and
spine deformities that result from plexiform neurofibromas. Thoracic neurofibromas most commonly occur in
paravertebral and mediastinal locations, or adjacent to
ribs. Approximately twenty percent of neurofibromatosis
type 1 can be associated with diffuse lung disease, especially upper lobe cystic disease and lower lobe fibrosis, as
well as solid pulmonary masses. Approximately 1-25% of
12
all neurofibromas have the potential for malignant degeneration into neurofibrosarcoma; therefore, treatment of
these masses tends to be surgical resection, with subsequent chemotherapy and/or radiation when malignancy is
diagnosed. To our knowledge, only two other cases of
neurofibromatosis leading to superior vena cava syndrome have been reported. Recommended treatment in
this case was surgical resection.
Abdominal Pain: Expanding the Differential Diagnosis
Hilary Bownik, MD; Nasim Afsar-Manesh, MD; Benjamin Waterman, BA
University of California, Los Angeles
pregnancy, obesity, and coagulation disorders (1,3,6,4).
Inciting factors include: straining, coughing, exercising,
prophylactic subcutaneous heparin injections, or trauma.
Measures should be taken to prevent the occurrence of
RSH as its mortality in patients on anticoagulation
reaches 25% (6,12). Patients with cough or on ventilation
should have prophylactic heparin injections only twice
daily and administration to the thigh (4). Treatment for
RSH is usually conservative. Surgical evacuation and
hemostasis is necessary if the hematoma fails to resolve,
if hemostasis cannot be achieved, or if a secondary infection develops (6,7). Failure to recognize RSH leads to
abdominal compartment syndrome, hemodynamic instability, and multi-organ dysfunction.
Diabetic Myonecrosis: A Case of Less is More
Learning Objectives: 1) Rectal Sheath Hematomas
(RSH) should be included in the differential diagnosis for
acute abdominal pain in all patients on systemic or subcutaneous anticoagulation. 2) Clinicians must maintain a
high suspicion for RSH as only 50% of patients will have
a visible hematoma at the time of presentation. 3) Early
diagnosis and appropriate treatment is necessary to prevent complications including hemodynamic instability,
abdominal compartment syndrome, and even multiorgan
dysfunction.
Case Information: We present a case of acute right
lower abdominal pain in an obese 45 year old female with
past medical history of hypertension, anti-phospholipid
syndrome, and end stage renal disease, who was admitted
for development of both a dvt and SMA clot despite
lovenox therapy. Four days after conversion from subcutaneous to IV heparin drip anticoagulation, the patient
complained of RLQ pain. She had a small 2x3 cm hematoma in her RLQ from previous lovenox and insulin injections, no rebound or guarding. Vital signs were stable
and hemoglobin unchanged. An abdominal series was
negative for obstruction or free air. That evening, she
developed significantly increased RLQ pain and an expanded RLQ mass of approximately 20 x10 cm. She was
tachycardic to the 150s. Abdominal CT showed a 9.7 x
16.1 cm anterior rectal sheath hematoma. Her repeat hemoglobin was now 5.5. The patient underwent CV angiogram with embolization of the right inferior epigastric
artery and required surgical evacuation of the hematoma.
Implications/Discussion: RSH are a rare cause of abdominal pain. They are an uncommon complication of
systemic or subcutaneous anticoagulation, usually developing 4 to 14 days after its initiation (6). Symptoms are
non-specific and include: abdominal pain, palpable mass,
nausea, vomiting, fever, and leukocytosis (3,8). The
Fothergill sign is diagnostic (3,6). This sign is positive if
the palpated mass, which shows lateral mobility, becomes
fixed when a patient contracts the rectus muscle (6, 9,10).
Only 50% of patients will have a visible hematoma at the
time of presentation (7). Diagnosis is confirmed by Abdominal CT. Medical conditions predisposing patients for
RSH development include: hypothyroidism, abdominal
surgery, ascites, hypertension, collagen vascular disease,
Hilary Bownik, MD; Jennifer Bennitt, MD; Benjamin
Waterman, BA
University of California, Los Angeles
Introduction: Diabetic Myonecrosis is a rare complication of diabetes that often goes unrecognized, leading to
delayed diagnosis, unnecessary procedures, and hospital
acquired infections.
Case Description: A 46 year old Hispanic female with
poorly controlled type 2 diabetes for 12 years, obesity,
peripheral neuropathy, and retinopathy presents with a 3
month history of bilateral lower extremity edema. For 2
weeks, she had experienced extreme left leg pain and
worsening of her edema to the point that she could no
longer ambulate. At a local ER, a MRI showed diffuse
quadricep edema consistent with cellulitis, and the patient
was started on multiple broad spectrum antibiotics, with
no improvement of symptoms. Given extreme pain out of
proportion to exam, there was concern for necrotizing
fascitis. The patient refused all recommended surgical
interventions or wound debridement except for a small
2.5 cm left medial thigh incision. 5 Days after a PICC
line was placed, she became febrile and hypotensive. She
was found to have candida glabrata fungemia and pulmonary infiltrates. She refused bilateral lower leg amputation and was transferred for a higher level of care. Upon
arrival, she had extremely painful pitting 2+ edema up to
the groin bilaterally, 2 oozing right inner thigh lesions
measuring 2-3 cm, and one left thigh non healing surgical
incision. MRI of legs revealed profound muscle edema
along the left vastus intermedius muscles and lateral aspect of the vastus medialis, findings consistent with diabetic myonecrosis.
Discussion: Diabetic Myonecrosis is a rare complication
of diabetes with less than 50 case reports in the literature.
Often initially unrecognized, it can lead to delayed diagnosis, unnecessary procedures, hospital acquired infections, and patient suffering. It occurs more often in insulin dependent, poorly controlled, longstanding (>15
years), and type 1 diabetics. The most common muscles
involved are the quadriceps (62%), hip adductors (13%),
hamstrings (2%), or hip flexors (2%). Patients present
13
with acute onset of painful swelling, normal pulses, no
strength nor sensation abnormalities, and no history of
trauma. They are normally afebrile, non toxic appearing,
and have extreme tenderness to palpation. One third of
patients will have bilateral physical findings. Only 8 %
will have leukocyotosis. Muscle biopsy shows muscle
necrosis and edema. MRI with gadolinium is the gold
standard for diagnosis, which shows extensive edema of
the vastus lateralis, vastus medialis, and occasionally the
rectus femoris muscle. Treatment is immobilization, pain
control, and tight glucose control (100-115). With tight
glucose control and aspirin, most patients have spontaneous resolution in a few weeks (average 5). Surgical incision is contraindicated because of poor wound healing
and risk of superinfection (average healing >13 weeks).
Overall 5-year mortality rate after initial diagnosis is high
owing to severe underlying diabetic complications.
left breast mass resulted in the diagnosis of infiltrating
ductal carcinoma that was estrogen receptor positive,
progesterone receptor negative, Her-2/neu negative, and
BCL-2 positive. Patient was treated with radiation therapy, high dose steroids, bisphosphonates, and subsequent
chemotherapy.
From Cat Scratch to Can't Walk
Acute, Reversible Cardiomyopathy in a Patient with
Group A Streptococcal Toxic Shock Syndrome
Leticia Campbell, MSIII; Vivek Ramanathan, MD;
Enma Alvarado, MD; Sassan Farjami, MD; Jasminka
Criley, MD
Charles R. Drew University of Medicine and Health
Sciences David Geffen School of Medicine at UCLA
Case: A 52 year old woman presents to the hospital with
a complaint of lower extremity weakness and difficulty
ambulating. The patient was bed ridden for 3 months,
after experiencing a series of falls. The patient’s history
includes a work injury, which resulted in an L5 disk herniation, otherwise she has been healthy. Two months
prior she presented to an outside hospital with a lesion
above her left breast extending to the left axilla diagnosed
as cellulitis. She believes that sitting for 6 hours in a
wheelchair at that time lead to the difficulty walking. She
was adamant that the lesion started as a bump 5 years ago
after her cat pounce off her left breast. It erupted 1.5
years ago into a fungating lesion, which she believed to
be healing until she became bed ridden. The patient has
experienced a 30lbs weight loss in the last 2 months. Her
mother has been in remission for breast cancer for 12
years; her aunt also had breast cancer. Physical examination revealed decreased sensation in her lower extremities
bilaterally in the L3-S1 dermatomes and left upper extremity in the 3rd and 4th digits to pin prick. Strength: 5/5
upper extremities and left hip flexion and knee extension,
3/5 right lower extremity. Babinski sign was positive
bilaterally. There was no pronator drift. Breast exam: Left
breast 21x22 cm firm mass in the 12 o’clock position
with overlying fungation lesion extending to the axilla
and total breast retraction. Cervical CT scan showed extensive destructive lesions involving C6 and the posterior
region of C5 with extension into the spinal canal and involvement of the skull and clavis; CT chest/ab/pelvis
revealed a large left breast mass with axillary lymphadenopathy, right liver lesions, and lytic lesions at L5 extending to the thecal sac, left sacroiliac joint, right ischium, ilium, and acetabulum. Labs on admission were
significant for increased WBC 14.5, platelets 576, alkaline phosphatase 414, and AST 44. A core biopsy of the
Discussion: This case illustrated the importance recognizing signs and symptoms of metastatic disease as the
initial presentation of underlying malignancy, and emphasizes the importance of health education and access to
screening. Breast cancer is the most common cancer and
the second most common cause of cancer death in U.S.
women; most of which are infiltrating ductal carcinoma.
There is a 3-4 time increase risk if it has affected a primary relative. When detected early, breast cancer has a
increased survivorship and lower morbidity rates.
Steven Carlson, MD; Barbara Natterson, MD; Daniel
Uslan, MD
University of California, Los Angeles
Introduction: Toxic Shock Syndrome (TSS) is a rare
complication of Group A Streptococcal infections. Little
is known on the time course and prognosis specifically in
relation to the cardiac manifestations of this disease.
Case: A 26 year old white male presented with a 4 day
history of sore throat, fevers and myalgias. On examination, vital signs: 39.4° C, HR125, BP 100/56. He was
initially saturating 100% on room air but over the next 24
hours became tachypneic requiring increasing amounts of
oxygen. He was toxic appearing. Exudates were noted on
his tonsils bilaterally. There was cervical lymphadenopathy with tenderness to palpation. Neck was supple without meningismus. Lung exam was notable for bibasilar
crackles and wheezing. A diffuse maculopapular rash was
noted on the patient’s trunk, lower and upper extremities
as well as on the palms and soles, however the face was
spared.
Lab studies disclosed the following significant values:
WBC of 6.9 with a left shift. CSF analysis including
WBC and cultures were negative. On the day of admission the student health center, where the patient had initially been seen 4 days prior called to inform the admitting team that a throat culture from 4 days prior was
growing Group A Streptococcus.
Approximately 24 hours after admission the patient
acutely decompensated becoming hypotensive and requiring mechanical ventilation. He was transferred to the
Medical Intensive Care Unit (MICU) and started on
broad spectrum antibiotics including Meropenem and
Clindamycin for presumed Streptococcal toxic shock
syndrome as well as initiated on pressor support with
Levophed. IVIG infusion was also started at that time.
14
Hemodynamic monitoring via Swan Ganz catheter was
implemented and revealed a cardiac output of 3L/min
corresponding to a cardiac index (CI) of 1.6 for this patient. CVP was 21. Echocardiogram was obtained on the
day of ICU transfer which revealed severe biventricular
dysfunction with a quantitated left ventricular ejection
fraction of 18.5%. He was started on dopamine in addition to levophed for treatment of cardiogenic shock.
Over the next 3 days the patient was weened completely
off of pressor support. Repeat echocardiogram on Day 8
after admission revealed EF of 50%. On Day 16 patient
was walked himself out of the hospital
Discussion: This case demonstrates the unique cardiac
manifestations of Streptococcal TSS. The hemodynamic
pattern seen in these cases is of cardiogenic shock and not
of septic shock, in that cardiac output is decreased and
systemic vascular resistance is not significantly decreased. The likely mechanism of cardiogenic shock seen
in these patients is toxin mediated, thus administration of
IVIG and anti-toxin antibiotics such as clindamycin are
useful. Prompt detection of Streptococcal TSS and administration of the appropriate supportive and therapeutic
measure can lead to a full reversal of even severe cardiomyopathy.
A 29-Year-Old Man with Paraneoplastic Angioedema
Randall Chan, MD; Salima Thobani, MD, Peter
Hyunh, MD
University of Southern California
A 29-year-old Hispanic man with no past medical history
presented with a chief complaint of swelling of his lips
for six months. Over the course of the 6 months, his lips
became progressively swollen and his eyelids too became
swollen over 2 months. He had been treated by multiple
physicians with trials of antihistamines, oral corticosteroids as well as acyclovir. Physical exam revealed marked
swelling of his upper and lower lips with pink discoloration consistent with chronic vascular congestion, as well
as edematous eyebrows bilaterally that were also pink
with vascular congestion. He had a faint violaceous rash
to be nasal bridge, cheeks and forehead without sparing
of his nasolabial folds. Oral examination revealed thrush
on his tongue and ulcerations of his gingiva. Chest auscultation demonstrated crackels throughout and abdominal exam revealed a mild splenomegaly. Petechaie were
scattered diffusely. The remainder of his exam was normal. A complete blood count revealed a profound thrombocytopenia of 8,000/cumm, as well as an absolute lymphopenia. Lactate dehydrogenase was markedly elevated
at 1204 units per liter. Chest x-ray revealed an infiltrative
lung disease and CT scan revealed multiple pulmonary
nodules—the largest of which was nearly 3 centimeters in
size—as well as splenomegaly. A workup for chronic
angioedema was performed. TSH and HIV were noted to
be negative. C1-inhibitor levels were normal, as well as a
C1-inhibitor functional assay. Additionally, C2 and C4
serum levels were normal as well. An autoimmune
workup was unrevealing. Serum protein electrophoresis
did not reveal a monoclonal paraproteinemia. Given the
above findings, lymphoma was suspected and the patient
underwent a bone marrow biopsy, which was unrevealing. However, prior to diagnosis being made, the patient
rapidly developed a large effusion with necrotizing
pneumonia and septic shock. Cytology from fluid confirmed malignancy, however patient expired prior to receiving chemotherapy. Paraneoplastic angioedema is a
rare but known complication of B-cell lymphoma and
lymphoproliferative disorders, due to the production of an
antibody against C1-inhibitor. This can be detected by
decreased levels and function of C1-inhibitor, as well as
decreased levels of C2 and C4 which are consumed in the
classical complement pathway. Additionally, a monoclonal paraprotein may be detected. Cases of paraneoplastic angioedema associated with lymphoma
but with normal levels and function of C1-inhibitor have
been described, but are far rarer. Nonetheless, normal
complement workup does not exclude the possibility of
paraneoplastic angioedema, and it is useful to continue to
consider the possibility of malignancy in this setting.
The Worm That Got Away: Can Hookworm Infection
Cause Frank Hematemesis?
Beverly Chen MD; Tung Nguyen MD
University of Southern California
There are more than 700 million persons in tropical and
subtropical regions infected with hookworms. Most patients have mild, nonspecific symptoms and mild degrees
of anemia. There have been pediatric cases that presented
with occult gastrointestinal bleeding and adult cases that
presented with melena; however, to date, there have been
no cases of hookworm infestation in an adult presenting
as overt acute hematemesis.
A thirty-one year old Hispanic male, with a history of
alcohol abuse, presented with hematemesis and bright red
blood per rectum. His hemoglobin was 7.2 and he was
intubated for airway protection. Emergency EGD revealed small amount of blood in the fundus which was
suctioned but normal esophagus with no varices, and no
evidence of esophageal or gastric ulcer. A large fungating
mass was found in the duodenum with visible purple lesions near the ampulla but there was no evidence of active bleeding. The GI bleed was attributed to the fungating periampullary mass. The patient was placed on intravenous esomprazole and transfused a total of six units of
packed red blood cells and two units of plasma, with repeat hemoglobin stabilizing at 11.
Computed tomography was notable for thickening of the
first and second portions of the duodenum, but no apparent mass. On repeat endoscopy a week later, no masses or
lesions were seen up to the ampulla. Five centimeters
distal to ampulla, an area of thickened mucosa with slight
erythema was noted. When the endoscope was advanced
into this area and biopsies were being obtained, a worm
arose from the abnormal mucosa. Subsequently, three
15
additional worms were seen, two of which were removed
with biopsy forceps.
Infectious disease was consulted and the patient was
started on albendazole on suspicion of hookworm infection. Duodenal biopsy was notable for mild focal increase
in eosinophilic infiltrate. However, no parasites were
identified on biopsy of the duodenum. The patient’s hospital course was complicated by aspiration pneumonia for
which he was started on antibiotics but discharged home
in stable condition with no further episodes of bleeding.
While biopsy results did not confirm the presence of
hookworm infection, stool studies were notable for
hookworm ova. The abnormal pale mucosa and eosinophilia in the duodenum noted on biopsy may have been
secondary to chronic changes from hookworm infestation. Although laboratory, radiographic, and pathologic
work-up negative did not reveal a clear etiology to this
patient’s hematemesis, there is a distinct possibility that
his presentation may have been secondary to a hookworm
infection, although this cannot be definitively proven.
A Case of Acute Interstitial Nephritis Presenting with
Markedly Elevated Erythrocyte Sedimentation Rate
Eric Cheung, MD; H. Madkour, MD
University of Southern California
Acute interstitial nephritis (AIN) is a form of nephritis
most commonly induced by drug therapy. Additional
causes include autoimmune disorders, as well as infectious etiologies. Although a definitive diagnosis can only
be made via kidney biopsy, most cases are diagnosed and
managed through history and laboratory findings. Classically, cases of drug-induced AIN present with a triad of
rash, fever, and eosinophilia, though recent studies have
demonstrated that the presence of all three findings is less
common previously thought.
A 51 year old female who was recently discharged for
atypical chest pain presented with a chief complaint of
bilateral lower extremity swelling, fevers, and fatigue. On
review of her medical records, she had recently been
started on several medications, including omeprazole,
furosemide, and labetolol. She denied any chest pain,
rashes, dizziness, or HA.
Initial physical examination revealed an obese female
with significant pitting edema in bilateral lower extremities. Her lung exam revealed scattered wheezes with
crackles at bilateral bases. The remainder of the exam
was unremarkable.
On admission, the patient had an elevated serum
creatinine of 2.89mg/dL, compared with her creatinine of
1.1mg/dL one week prior. A urinalysis showed trace protein, trace blood, moderate leukocytes, specific gravity of
1.012, and a pH of 6.5. Given her complaint of fevers at
home, a CRP and ESR were checked, which were elevated at 102.9mg/L and 116mm/hr, respectively. Urine
and blood cultures were negative. A repeat urinalysis
with Wright’s stain was obtained the day following admission, and was positive for 1-2 eosinophils per highpowered field. A renal ultrasound showed normal sized
kidneys without evidence of obstruction.
Given the patient’s recent medical history and acute onset
of renal failure, the decision was made to discontinue her
omeprazole secondary to concerns for AIN. Her serum
creatinine subsequently dropped to 2.5-2.7mg/dL over the
following 4 days, but without noticeable improvement in
her lower extremity edema. Corticosteroid therapy was
initiated on day 5 of her hospitalization secondary to lack
of improvement in her renal function. Over the following
5 days, the patient’s serum creatinine decreased from
2.5mg/dL to 1.87mg/dL, with notable diuresis and decreased swelling in her lower extremities.
There has been extensive literature reporting the association of proton-pump inhibitors and acute interstitial nephritis. Though a majority of patients demonstrate significant improvement in their renal function after withdrawal of the proton-pump inhibitor, certain subsets of
patients continue to have chronic renal insufficiency despite prompt intervention. Elevated ESRs have been
noted in several studies, but rarely to the degree this patient exhibited. Recognition of the variety of abnormal
laboratory findings associated with AIN is crucial for
early diagnosis, given that significant improvement has
been demonstrated with prompt intervention.
Angina as a Rare Manifestation of a Rare Disease
Raj Chudasama, MD; Stanley Dea, MD
Olive View-UCLA Medical Center
Case: A 60 year old Armenian male with a history of
hypertension, dyslipidemia, and coronary artery disease
presented with chest pain and shortness of breath for
three days. He has a history of two coronary stents placed
three years ago. In addition, he reported bowel movements that were darker than usual for the past day but
denied hematemesis or bright red blood per rectum. He
noted a ten pound unintentional weight loss over the past
10 days. There were no other significant GI symptoms.
He denied use of tobacco, alcohol or illicit drugs. Medications included aspirin 325mg and meloxicam 7.5mg
daily as well as ibuprofen 400mg which he took often for
pain. Physical exam was unremarkable. Significant lab
data included Hgb 6.5, MCV 69, Iron 13 and TIBC 428
(iron saturation=3%). After being cleared of acute cardiac
disease, the patient was scheduled for esophagogastroduodenoscopy (EGD) the following day to evaluate for
upper GI bleeding with suspicion of NSAID induced ulcers. Although, the EGD showed three small clean-based
antral ulcers, the patient’s melena and anemia was
thought to be from a large ulcerated mass in the first and
second portion of the duodenum. Multiple biopsies were
taken which later confirmed a diagnosis of moderately
differentiated, invasive duodenal adenocarcinoma. Based
16
on the location, this was felt not to originate from the
ampulla.
Discussion: Primary duodenal adenocarcinoma is a rare
malignant neoplasm of the small bowel, representing
0.5% of all gastrointestinal (GI) tract cancers. Although
duodenal cancers make up half of all cancers found in the
small bowel, the majority of these cancers are in the periampullary region. Thus duodenal adenocarcinoma originating from outside this area is relatively rare. A possible
reason for this predilection to the ampullary region is
interactions of carcinogens with pancreaticobiliary secretions. The clinical presentation of duodenal adenocarcinoma may be vague, or even asymptomatic, leading to
late diagnosis with advanced stage at the time of diagnosis. When present, the most common initial symptoms
include abdominal pain and/or obstructive symptoms.
Ulceration of the mucosa is common and may result in
occult GI bleeding or chronic anemia. However, in this
unusual case, the anemia was so profound it caused angina in a patient with underlying cardiac disease. All patients able to tolerate surgery should be given the option
of aggressive resection regardless of tumor size, invasion
or appearance of positive lymph nodes. Pancreaticoduodenectomy is usually necessary if the tumor is localized to the proximal portion of the duodenum. Unresectable tumors should be considered for treatment by
radiotherapy. Many patients with advanced cancer of the
duodenum will become obstructed and therefore require
palliative GI bypass or endoscopically placed stents.
Fabry Disease Masquerading as Hypertrophic Cardiomyopathy
David Cohen, MD; Stephen Hu, MD; Nestor
Mercado, MD; Allen Johnson, MD
Dept. of Internal Medicine/Cardiovascular Disease
Scripps Green Hospital
The patient is a 52 year old gentleman who initially presented for cardiovascular consultation on May 22, 2007
in regards to two previous symptomatic episodes of rapid
atrial fibrillation. The patient sought medical attention
with his second episode in August 2006 and was chemically converted. His resting electrocardiogram during that
hospitalization showed dramatic T-wave inversions in the
lateral precordial leads with small Q-waves and prominent R waves in V1 and V2. Subsequent echocardiography revealed a hypertrophic cardiomyopathy, with apical
cavity obliteration and no outflow tract obstruction or
valvular lesions. He was discharged on Norpace CR and
atenolol.
At the time of his initial consultation in May of 2007 he
was without recurrence of arrhythmia, but was struggling
with intermittent headaches associated with lightheadedness. Norpace was held as a suspected culprit for possible
symptoms of orthostasis. A repeat echocardiogram
showed moderate concentric left ventricular hypertrophy
with an echogenic myocardial texture consistent with an
infiltrative myocardial disorder. A serum protein electro-
phoresis showed no monoclonal proteins. In the meantime, the patient continued to struggle with headaches off
Norpace, now constant. MRI of the brain showed no abnormalities. The patient’s family history was ultimately
most revealing as his brother was diagnosed with Fabry
disease as part of a work-up for renal insufficiency. This
would provide a rare yet unifying diagnosis in this patient
with symmetic hypertrophic cardiomyopathy in the setting of neurological symptoms. Repeat echocardiogram
with optimal views revealed the presence of a binary appearance of the left ventricular endocardial border, with a
hyperechogenic layer along the ventricular endocardium
paralleled by hypoechogenic myocardium. Serum alphagalactosidase A levels were then assessed and found to be
low with an increased ceramide trihexoside in his urinary
sediment. These findings were consistent with Fabry disease.
Although uncommon, atypical Fabry disease can present
with left ventricular hypertrophy and no other obvious
disease manifestations. Our patient had symmetric hypertrophic cardiomyopathy without the neuropathy, skin
lesions, or renal involvement typical of Fabry disease.
His echocardiogram elucidated the binary appearance of
the LV endocardial border seen in patients with Fabry
cardiomyopathy, reflecting the endocardial and subendocardial compartmentalization of glycosphingolipid material. This case demonstrates the importance of screening
for Fabry disease in patients with hypertrophic cardiomyopathy, particularly if symmetric, given the availability of effective therapy.
Metastasis or New Primary?
Dana Copeland, MD
Scripps Green Hospital
Introduction: Biopsy is not considered necessary in patients who are likely to have a brain metastasis, yet alternative diagnoses are more frequent than one might suspect. This may lead to inappropriate treatment and its
associated morbidity, particularly with chemotherapy.
Case Description: A 63 year-old female with a history of
stage I ductal cell carcinoma (T1N0M0) diagnosed
in1998 who was status post lumpectomy, radiation and
chemotherapy presented with nausea and vomiting up to
ten times per day. She also complained of a dull frontal
headache which was 4/10, constant and non-radiating.
For the past 6 months she had noted intermittent nausea,
photosensitivity and a five pound weight loss. Two
months prior, she also began experiencing diplopia, dizziness and word-finding difficulties. In addition, she had
subjective fevers for five days prior to admission. Her
cancer screening was up to date, although her last mammogram 1 month prior was BI-RADS 2. Her family history was significant for a mother and sister with breast
cancer 70 and early 40s, respectively. She was otherwise
healthy and had completed 5 years of hormonal therapy 8
months prior. On neurologic exam, the only finding was a
left sided resting tremor. A non-contrast CT of the head
17
showed extensive left frontal edema with mass effect. A
subsequent MRI showed an isolated neoplasm in the left
mesiofrontal lobe with cystic and hemorrhagic features.
A biopsy confirmed glioblastoma multiforme, WHO
Grade IV.
Discussion: Brain metastases occur in 20-40% of known
cancer patients, of which lung and breast cancers are the
most likely to metastasize. Brain metastases outnumber
primary neoplasms by at least 10 to 1. Therefore, a brain
tumor in a patient with known history of breast cancer is
much more likely to be metastasis than new primary.
Biopsy is not considered necessary in patients who are
likely to have a metastasis; however, in cancer patients
with brain tumors, 11% were found to have a new primary lesion, inflammatory process, or infection rather
than metastasis. This case represents the importance of a
biopsy before beginning treatment for brain malignancies
even when a patient has known history of breast, lung, or
other cancers that commonly metastasize to the brain.
Our clinical suspicion for breast metastasis in this patient
was low considering 70% of metastases have multiple
lesions, and she had only a single lesion. A biopsy proved
to be helpful since the treatment would be different in
each scenario.
A 17 Year Old Female Presenting with
Multi-Vessel ST Elevated
Myocardial Infarction from Systemic Lupus
Erythematous Induced
Coronary Artery Vasculitis
Douglas Crowley, MD; Richard Friedman, MD;
Katherine Nguyen, MD
Scripps Mercy Hospital
CASE: A 17 year old female presented to the ED with
severe chest pain radiating to her left scapula, neck, jaw
and left arm. The pain was constant over 5 hours and was
associated with SOB, nausea, diaphoresis and bilateral
arm numbness. Twelve days prior to this presentation the
patient underwent laparoscopic cholecystectomy for acalculous cholecystitis. Gallbladder pathology showed necrotizing median vessel vasculitis resembling Polyarteritis Nodosum (PAN). She was discharged with the tentative diagnosis of PAN and given 60mg of prednisone
daily. On this presentation, the patient was in moderate
distress and diaphoretic, with vitals significant for a HR
of 140. EKG showed inferior and lateral wall ST elevations and cardiac markers were elevated. Urine toxicology and pregnancy screens were negative. She was taken
for cardiac catheterization which showed a large dominant right coronary with 100% occlusions at the mid posterolateral branch and the distal right. Acute marginals
had multiple beaded areas consistent with thrombus.
Normal left main, however the LAD was 100% obstructed in the mid portion. Large first diagonal and circumflex were both 100% obstructed in the mid portion
with visible thrombus in circ with multiple occluded
small branches. Due to the vast extent of coronary in-
volvement and no evidence of atherosclerotic lesion or
plaque no stents were deployed. The patient was continued on heparin drip, given full dose of aspirin and eptifibatide, loaded with clopidogrel, and started on reteplase.
Echocardiogram showed cardiomyopathy with an EF of
15-20% and depressed right ventricular function. Lab
studies showed a positive ANA and DS DNA, elevated
ESR and CRP and depressed C3. Anti-phospholipid,
HIV, hepatitis, ANCAs, and Anti-Smith labs were negative. The patient was diagnosed with SLE vasculitis of
the coronary arteries and was started on solumedrol 60mg
IV BID and cyclophosphamide 2mg/ kg/ day. For CHF
management lisinopril, metoprolol, atorvastatin, aspirin
and clopidogrel were initiated. Repeat echocardiogram
showed an EF of 40%. The patient was discharged on day
30 on a prednisone taper, the above mentioned cardiovascular medications and was transitioned to a three month
cyclophosphamide pulse regiment.
DISCUSSION: Cardiac involvement in patients with
SLE is not unusual with studies demonstrating a prevalence of 50%; however, SLE induced coronary vessel
vasculitis resulting in an acute MI is rare. Most cases of
acute coronary syndrome and SLE involve accelerated
atherosclerosis secondary to the vasculitis leading to
premature coronary artery disease. The rare cases of SLE
induced coronary artery vasculitis have high morbidity
and mortality and prompt recognition and alterations in
therapy are required.
Hysterical Dysphagia
Matthew Crull, MD; Ian H. Jenkins, MD; Omeed
Saghafi, BS
University of California, San Diego
Once commonplace in the differential diagnosis of dysphagia, Plummer-Vinson Syndrome is nowadays infrequently reported. Currently, the very existence of the
syndrome is challenged. This syndrome may, however,
still have clinical significance. A 41 year-old African
American woman with a history of menorrhagia and no
recent primary care presented complaining of fatigue. She
also noted progressive dysphagia of solids for the past 3
years. Examination revealed koilonychia, cheilosis, atrophic glossitis, and conjunctival pallor. The hemoglobin
was 4.3 g/dl, the MCV was 48.3, the iron was 6 mcg/dl,
and the ferritin was undetectable (<1 ng/ml). A barium
esophagram was done to better characterize the dysphagia, and showed probable esophageal webs. Esophageal webs were confirmed by upper endoscopy; the webs
were successfully dilated with a balloon dilator. She was
transfused 4 units of packed red cells, treated for menorrhagia with oral contraceptives, and started on ferrous
sulfate with ascorbic acid for her iron deficiency anemia.
At the time of discharge, she reported improvement in her
dysphagia.
Plummer-Vinson Syndrome presents with the classic
triad of dysphagia, iron deficiency anemia and esophageal webs. Patterson and Kelly independently described
18
the triad of iron-deficiency anemia, dysphagia, and upper
esophageal webs in 1919 following Plummer (1912) and
Vinson’s (1919) similar but less comprehensive descriptions of “hysterical dysphagia.” Plummer-Vinson syndrome is rare today; exact data about incidence and
prevalence are not available. Whether iron deficiency
truly causes esophageal webs is debated, but iron deficiency is thought to weaken esophageal musculature and
cause epithelial cell atrophy. Autoimmunity and genetic
predisposition are other postulated causes. Dilation of the
webs is usually curative, although their association with
an increased risk of upper alimentary cancers may justify
surveillance endoscopy. Koilonychia, glossitis and angular cheilitis, which are often found in patients with varying degrees of malnourishment, are frequently reported in
those with Plummer-Vinson syndrome in addition to the
classic triad of findings. The proposed mechanisms are
similar to those in the development of esophageal webs,
and include depletion of iron containing enzymes involved epithelial cell turnover. While iron-deficiency
anemia is frequently found in women of childbearing age,
Plummer-Vinson Syndrome is rarely encountered in current times. Not only does this case illustrates unique
physical exam findings, but may still have clinical relevance due to its association with cancer involving the
upper esophagus.
cardiomyopathy. Marked ascites was seen on abdominal
US. She reported that over ten members of her family in
three generations have had cardiac and cerebrovascular
disease at relatively young ages. Her father had atrial
fibrillation and ascites. Her family pedigree was significant for father to daughter transmission with no skip generations, seen typically with an autosomal dominant pattern. The patient was started on multiple anti-arrhythmic
drugs to assist with treatment of her atrial flutter as well
as anticoagulation therapy. She was also started on diuretics to assist with her right heart failure. Familial restrictive cardiomyopathy is an autosomal dominant cardiomyopathy histologically characterized by myocyte
hypertrophy and interstitial fibrosis. Other causes of restrictive cardiomyopathy include endomyocardial fibrosis, infiltrative diseases such as amyloid and sarcoid,
scleroderma, carcinoid heart disease, malignancies, and
medications. Complications seen in familial restrictive
cardiomyopathy include development of bundle branch
blocks with eventual heart block and progressive skeletal
myopathy.
Interrupted Aortic Arch Presenting as Myocardial
Infarction
Daniel Daneshvar, MD; Kiran Philip, MD
Cedars-Sinai Medical Center
Familial Restrictive Cardiomyopathy
Daniel Daneshvar, MD; Gautam Kedia, MD; Robert
J. Siegel, MD
Cedard-Sinai Medical Center
Restrictive cardiomyopathy (CM) is a type of mixed primary CM with a primary abnormality of diastolic dysfunction. Common symptoms with this form of cardiomyopathy are increased jugular venous pressure (JVP),
peripheral edema, and ascites due to right heart failure.
There are many causes of this form of cardiomyopathy,
with a number of reports of idiopathic restrictive cardiomyopathy with familial roots. A 35 year-old female presented with palpitations, shortness of breath, and abdominal bloating. She had always felt that her stamina
was low & recently noticed her heart was beating faster
than usual. The patient has a history of abnormal ECGs,
told she had an abnormal p wave. Family history was
significant for multiple family members dying at an early
age. On exam, the patient was tachycardic, with a split S2
and a grade II/VI systolic murmur heard best at the left
sternal border. JVP was increased to the angle of the jaw.
Her abdomen was distended with a positive fluid wave
and marked hepatomegaly. Her extremities showed dependent cyanosis in both feet. CXR revealed right ventricular enlargement with mild pulmonary vascular congestion. ECG exhibited tachycardia consistent with atrial
flutter. An echocardiogram and cardiac catheterization
revealed normal systolic function, abnormal left ventricular relaxation, marked biatrial enlargement, increased
right atrial pressure, tricuspid regurgitation and increased
end diastolic pressure. Doppler parameters were consistent with restrictive physiology consistent with restrictive
Interrupted aortic arch (IAA) is defined as a loss of luminal continuity between the ascending and descending
portions of the aorta. It is a rare congenital malformation
with an incidence of approximately 3 cases/million live
births and accounts for about 1% of congenital cardiovascular defects. IAA is typically associated with other cardiovascular anomalies and is usually diagnosed and repaired in the neonatal period. IAA in adults is exceedingly uncommon and may be misdiagnosed as primary
HTN. A 29 year-old Hispanic male with a history of hyperlipidemia and HTN presents with chest pain. He reports intermittent shortness of breath and non-exertional
chest pain for 4 days, worsened on the day of admission
while driving. He described the pain as left-sided, heavy
and burning, 10/10 in severity without radiation, nonpleuritic in nature, associated with some nausea without
vomiting or light-headedness. Initial ECG suggested an
acute MI. He was given ASA 325 mg and 2 NTG tablets
sublingually with mild improvement. Patient was active
as a child, playing football. He recently noticed increasing symptoms while standing at work. The patient is noncompliant with his medication regimen of gemfibrozil,
amlodipine, and HCTZ. He smokes one pack of cigarettes
daily, with occasional alcohol use. His family history is
noncontributory. On exam, BP 140/86, other vitals were
stable. Lung exam was clear to auscultation and heart
exam was regular rate and rhythm with a grade III/VI
systolic murmur at the base. Extremities had no edema
and 1+ distal pulses. ECG revealed ST segment elevation
in lead II, III, & aVF with reciprocal ST depressions in
V2-6 & inverted T waves in V4-6 with left ventricular
hypertrophy. Troponin was initially 0.1, then 10.97 with a
CXR showing cardiomegaly. He was taken emergently
19
for cardiac catheterization; the guide wire was unsuccessfully advanced through the aorta, with angiogram and
transthoracic echocardiography findings concerning for
the presence of a aortic dissection vs. aortic coarctation.
He was then taken to the OR for a diagnostic transesophageal echocardiogram which demonstrated a hypoplastic distal aortic arch and inferior wall hypokinesis.
Chest CT angiogram and MRI showed no evidence of
aortic dissection but found a 4.7 cm interruption of the
aortic arch proximal to the expected origin of the left
subclavian artery with enlarged intercostal artery collaterals. The patient was also found to have right coronary
artery occlusion on repeat cardiac catheterization through
the radial artery. He was scheduled for elective aortic
repair after recovery from his MI. The case illustrates an
extremely rare presentation of isolated IAA in adulthood
with an ST elevation MI.
Common Drug Causes Rare Life-Threatening Side
Effect
Omar Darwish, MD; Jasminka Criley, MD
St. Mary Medical Center
Hydrochlorthiazide (HCTZ), an inexpensive and effective
drug, is one of the most common antihypertensive medications prescribed in the United States. Well-known side
effects of the drug include electrolyte imbalances, hypotension, hyerglycemia, and various gastrointestinal
complaints. However, a select few have had a rare lifethreatening side effect—non-cardiogenic pulmonary
edema with shock.
A 46-year-old woman with hypertension developed
dyspnea, lightheadedness and vomiting 20 minutes after
taking one tablet of 25 mg HCTZ. She felt lightheadedness with her first HCTZ ingestion a few months earlier
and had not taken it since then until the day of admission.
She was otherwise a healthy stay-at-home mother. Home
medications included HCTZ 25 mg and Atenolol 50 mg.
Physical examination revealed a lethargic woman with
O2 sat 80% on room air, temperature 98° F, pulse 98,
respiratory rate 24, and a blood pressure 115/88. The
neck veins were flat. Auscultation of the chest revealed
bilateral rhonchi. Heart sounds were normal without gallop. Laboratory data were significant for WBC 5,100/uL,
lactate level 3.5 and BNP 12. Arterial blood gas demonstrated a PaO2 56 mmHg, PaCO2 63 mmHg, and a pH of
7.16 on a non-rebreather mask. The electrocardiogram
showed normal sinus rhythm with right axis deviation.
Chest X-ray showed bilateral diffuse alveolar infiltrates.
Transthoracic echocardiogram revealed normal ejection
fraction, no right-sided heart strain, normal valves and
chamber sizes. Spiral chest CT showed no pulmonary
embolism but diffuse alveolar consolidations. The patient
was intubated and mechanically ventilated. She then became febrile to 104.1º F and blood pressure dropped to
60/48 requiring 5 L of fluids and 20 micrograms/min of
levophed. After thirty-five hours on the ventilator, the
patient continued to require high positive end-expiratory
pressures and pressors. Bronchoscopy was done and the
brochoalveolar lavage revealed 12 percent lymphocytes,
18 percent macrophage, 70 percent granulocytes and no
eosinophils. Alveolar hemorrhage was not seen. Bacterial, AFB smears, fungal, and viral cultures were all negative. Blood cultures were negative as well. In addition to
mechanical ventilation, crystalloids, and pressors, she
was treated empirically with broad-spectrum antibiotics
and a stress dose of steroids for possible septic shock.
The patient was successfully weaned from levophed
within 36 hours and was extubated on day four of her
hospitalization. Since her symptoms developed immediately after taking HCTZ, she was presumed to have developed HCTZ induced pulmonary edema, a recognized
but rare side effect of the drug. She was discharged seven
days after admission with instructions to abstain from
hydrochlorothiazide in the future.
Development of respiratory failure with shock is a rare
consequence of hydrochlorothiazide ingestion. Since this
medication is prescribed to millions of patients, knowledge of not only the common adverse reaction is important, but also the most lethal—pulmonary edema with
shock.
A Mysterious Red Rash: Don't Blame the Mosquitoes
Monya De, MD, MPH; Kalpesh Patel, MD
Olive View-UCLA Medical Center
Case: A 55-year-old Guatemalan female presented with
four days of fevers, chills, bitemporal headache, and itchy
truncal rash. One month earlier, the patient had returned
to the United States from visiting relatives in Guatemala
for two weeks. She reported many mosquito bites during
the trip and no sick contacts or new medications. Initial
PE: normotensive, HR 107, T 39.1. She was flushed appearing, with an erythematous morbilliform rash with
some areas of confluence over the entire body, sparing
the face, lower legs, palms, and soles. Labs: WBC normal, BMP normal, new transaminitis (AST 262, ALT
223) alkaline phosphatase normal. UA: microscopic hematuria and pyuria. CXR: normal Infectious disease was
consulted, and the initial impression was dengue fever.
An extensive workup was ordered, including HHV6, parvovirus, dengue, HSV, VDRL, thick and thin smear,
ASO titer, HIV, and HSV (all negative). Ceftriaxone and
doxycycline were started empirically. The patient’s rash
progressed and spread to the previously spared locations,
with formation of large bullae. She had milia on the scalp
in addition to erythema from the rash. She did not have
any visible lesions in the oropharynx, but stated she had
felt one blister pop. She developed a leukocytosis with
lymphocyte predominance. She became very edematous
and required aggressive fluid resuscitation for persistent
hypotension and tachycardia. A skin biopsy showed bullous dermatitis. During the admission, there was a change
of fellow on the ID service. The new fellow questioned
the patient more directly about new medications taken,
and she finally revealed that, while in Guatemala, her
physician brother had run “routine tests” including serum
uric acid. Despite that she was asymptomatic, he gave her
20
allopurinol for her elevated uric acid, which she took for
approximately 5 weeks until her symptoms began. He
also gave her terbenafine for a fungal infection of the
feet. She had not thought these medications important to
mention on admission as they were already stopped. The
timing was consistent with a drug eruption, and IV steroids were initiated, resulting in dramatic improvement of
the rash, transaminitis, and edema. As both allopurinol
and terbenafine are associated with drug eruption, it is
impossible to define a single offending agent, and both
may have acted synergistically.
Discussion: This case serves to remind us of several important attributes of a successful diagnosis: probing history-taking, “fresh eyes” looking at a complicated patient,
and keeping a wide differential. In particular, the dramatic severity and high incidence in the literature of the
allopurinol drug hypersensitivity reaction and its underemphasis in medical education indicate a need for awareness among clinicians in general and specialty practice
who initiate this very common drug.
Cocaine Induced Peripheral Limp Vasculitis Requiring Amputation
Rahul Dhawan, DO; Joseph Carey, MD; Roger Chan,
MD; Divya Sachdev, BA
University of Southern California Keck School of
Medicine Department of Internal Medicine
We present a case report of a 46-year-old Caucasian female with history significant for cocaine addiction and
bipolar disease who presented to Los Angeles
County+University of Southern California Medical Center with hypotension, hemoptysis, and coagulopathy. She
was a known heavy inhalational and injection cocaine
user. During her acute critical illness, she required intubation and transfusion, and developed full thickness necrosis of skin in the extremities. She demonstrated acute
thrombosis of the distal radial and ulnar arteries, as well
as the Dorsalis pedis and Posterior tibial arteries. She
ultimately required below elbow amputation of her right
arm and a below the knee amputation of her left foot, and
debridement and grafting of the left foot. An acquired
vasculitis was suspected. She had rheumatologic serologies drawn, and was found to be positive for Antineutrophil cytoplasmic antibodies (ANCAs), a group of
mainly IgG antibodies against antigens in the cytoplasm
of neutrophil granulocytes. Small artery biopsies confirmed the diagnosis of acute vasculitis. It should be studied whether individuals with positive ANCA serologies
are more likely to suffer peripheral vasculitis if exposed
to excessive catecholomines such as cocaine.
T Wave Alternans Preceding Cardiac Arrest in Long
QT Syndrome
Jashdeep Dhoot, MD; Holly Middlekauff, MD
University of California, Los Angeles
Case Presentation: A 31 year old male with history of
Goodpasture''s syndrome, two failed renal transplants and
chronic hypocalcemia developed cardiac arrest with polymorphic ventricular tachycardia during a third renal
transplant operation. Preoperative workup included negative cardiac stress testing. Prior to surgery, his ionized
calcium was 0.89 mmol/L and his serum potassium was
3.2 mmol/L. An EKG showed normal sinus rhythm with
old non-specific T-wave inversions and a prolonged corrected QT segment of 573 msec. Vancomycin, methylprednisolone, and anti-thymocyte globulin was administered prior to the procedure. Approximately four hours
into his operation prior to reperfusion of the new renal
allograft, the patient went into ventricular fibrillation
arrest. Cardiopulmonary resuscitation was performed for
20 minutes, which included three defibrillations, before
normal sinus rhythm with palpable pulses was regained.
He was then hemodynamically stable for the rest of the
procedure and the operation was completed without major complications. Post-operative labs were significant for
a magnesium of 1.3 mEq/L. Post-operatively the patient
was monitored on telemetry in the ICU without an event.
He regained full neurological recovery after extubation.
Despite repletion of his electrolytes and avoidance of QTprolonging medications, the patients corrected QT interval remained prolonged. Retrospective analysis of the
patient’s intra-operative telemetry strip revealed beat-tobeat changes in T wave polarity, a phenomenon known as
T wave alternans, for several minutes prior to the sudden
onset of torsades de pointes polymorphic ventricular
tachycardia. Given these unique clinical findings suggesting congenital long QT syndrome (LQTS), the patient
had an automatic implantable cardioverter defibrillator
(AICD) placed prior to discharge. Genetic testing for
congenital LQTS is pending at this time.
Discussion: Long QT syndrome (LQTS) involves
marked prolongation of the electrocardiographic QT interval that predisposes to a distinctive form of ventricular
arrhythmia known as torsades de pointes. Clinically, this
syndrome presents as recurrent dizziness, syncope, or
sudden death. The etiology of this syndrome may be either acquired or congenital. The distinction is important
since therapies for the two forms differ. The congenital
form generally requires long-term beta-blocker therapy
which can be harmful in the acquired form. T-wave alternans has been observed in a wide variety of clinical and
experimental conditions to be associated with ventricular
arrhythmias including acute myocardial infarction, electrolyte imbalances, and LQTS. In association with LQTS,
T-wave alternans immediately preceding the development of torsades de pointes is a finding relatively specific
to the congenital form. Therefore it is helpful in distinguishing it from the acquired form. It also portends a possible impending ventricular arrhythmia. In patients diagnosed with the congenital form of LQTS, genotyping can
give insight into the patient’s potential response to betablocker therapy and prognosis. Also, genotype testing
aids in helping identify family members who may be at
risk.
21
Pregnant Women: The Silent Victims of the H1N1
Influenza Pandemic
Miliary Presentation of Bronchioloalveolar Carcinoma
Mary Elmasri, MD
University of California, San Diego
Elysia Engelage, MD*; Shahriar Pirouz, MD; Nader
Kamangar, MD, FACP, FCCP, FAASM
*University of California, Los Angeles, Olive ViewUCLA Medical Center
Case: A 28-year-old female at 26 weeks gestation presented to an outside hospital with four days of fever,
shortness of breath, and cough as well as myalgias, rhinorrhea and sore throat. Initial chest x-ray at the outside
hospital showed a right basilar opacity and her rapid influenza panel was positive for influenza A. She was initially started on oseltamivir and piperacillin/tazobactam.
Her course was significant for worsening hypoxia requiring BIPAP and acute kidney injury and volume overload
requiring dialysis.
The patient was transferred to our medical center approximately forty-eight hours after initial presentation.
Physical exam upon transfer was notable for tachypnea,
oxygen saturation 100% on 60% BIPAP, mild respiratory
distress, and bibasilar crackles. Labs were significant for
a leukocytosis of 19,000 with a bandemia of 64%. Chest
x-ray upon transfer showed a persistent right basilar
opacity. She was continued on oseltamivir and her antibiotics were broadened to vancomycin, ceftazadime, and
azithromycin. Influenza culture done at the outside hospital was positive for influenza A, which was then confirmed to be H1N1.
On hospital day two at our institution the patient’s respiratory status continued to deteriorate and she was intubated for hypoxemic respiratory failure. Immediately
afterward, the patient underwent emergent cesarean section. Her baby was taken to the neonatal intensive care
unit and did well. The patient subsequently developed
acute respiratory distress syndrome and was ventilated
using lung protective strategies. The patient had a complicated month long hospital course requiring advanced
modes of mechanical ventilation, such as bi-level ventilation, to maintain oxygenation. She ultimately required a
tracheostomy and was discharged off the ventilator to an
acute care facility.
Discussion: The H1N1 influenza virus has caused a pandemic of febrile respiratory illness throughout the world.
According to the CDC as of August 2009, greater than
98% of circulating influenza viruses in the United States
were the H1N1 influenza virus. Initial data found that
30% of all women ages 20-39 who died from H1N1 infection were pregnant or postpartum. This case illustrates
the fact that pregnant women are at higher risk for severe
illness with increased morbidity and mortality from
H1N1 infection and highlights their complicated and
devastating course. During this pandemic it is imperative
that pregnant women receive immediate treatment with
antiviral medication when there is clinical concern for
infection. It will also be essential that pregnant women
receive early immunization when the vaccine becomes
available later this fall.
Case: A 54 year old woman with no significant past
medical history presented to our hospital with one month
of dry cough. After seeing her primary care physician,
she promptly underwent a chest radiograph and was referred to our emergency room for further evaluation. She
denied any fevers, chills, hemoptysis, night sweats,
weight loss, or tobacco use. Her only other complaint was
mild hip pain and decrease in exercise tolerance. She
reports an abnormal chest radiograph approximately 5
years prior to presentation for which no further action
was taken. She emigrated from Korea approximately
25years ago and lives in the San Fernando Valley. Chest
x-ray obtained in the ER showed diffuse reticulonodular
disease with upper lobe predominance. Subsequent CT of
the chest showed confluent peribronchovascular opacities
in bilateral upper lobes and imaging of the abdomen and
pelvis showed nodules within the left adrenal gland, liver
and several bony lesions. She was placed in TB isolation
and started on RIPE therapy while awaiting AFB sputum,
urine, stool, coccidioidomycosis and histoplasma serologies. She was taken for bronchoscopy with pathology
from the biopsies showing bronchioloalveolar carcinoma.
TB isolation and medications were discontinued, Oncology was consulted, she was begun on Tarceva and dexamethasone and discharged home.
Discussion: We present an unusual case of BAC presenting radiographically similar to disseminated tuberculosis.
Bronchioloalveolar carcinoma (BAC), once a rare subtype of pulmonary adenocarcinoma has in recent years
increased its prevalence and now accounts for approximately 24% of all lung cancers. It is known as a cancer of
non-smokers and more frequently affects women and
Asians. The most common signs and symptoms are
cough, chest pain, dyspnea and hemoptysis. BAC most
commonly presents as a solitary nodule, but can also present as multiple synchronous nodules, or a diffuse parenchymal process. Radiographic findings that raise suspicion of BAC include ground-glass opacities, nonresolving consolidation, and satellite nodules. There is evidence
that the presence of any BAC features predicts improved
survival, stage for stage, other types of nonsmall cell lung
carcinoma (NSCLC). A study of patients with IIIB or IV
disease demonstrated a median survival time of 15
months versus 10 months for NSCLC. Our patient reports
an abnormal chest radiograph approximately 5 years prior
to her presenting at our hospital. This underscores the
importance of regular follow up and further investigation
into abnormal radiographs.
Thyrotoxicosis Fails the Right Heart
22
Babak Eshaghian, MD, MS; Roshni Shah, DO; Michael Hochman, MD; Babak Saedi, MD; Grace
Huang, MD; Jooby Babu, MD
University of Southern California
the increased metabolism of intrinsic pulmonary vasodilator. This case raises the awareness that isolated right
heart failure is a complication of thyrotoxicosis.
Chest Pain: The Great Masquerader
Case: A 37 year old female with a past medical history of
hypertension came to the ER complaining of shortness of
breath for 10 days, palpitations and anasarca. She reported 8-9 months of amenorrhea, anxiety, weight loss,
and two months of watery diarrhea. In the ER she was in
atrial fibrillation with right ventricular response and was
started on diltiazem. Physical exam was significant for
bilateral exophthalmos and an enlarged thyroid. Breath
sounds were diminished at the right lung base, the heart
rate was irregularly irregular, the abdomen was distended, and there was hepatomegaly, and anasarca. Thyroid panel revealed low TSH (0.01mIU/L), elevated free
thyroxine (>7.77ng/mL). Significant labs include elevated alkaline phosphatase, total and direct bilirubin, and
prolonged prothrombin time. Upon admission to ICU
diltiazem was discontinued and the patient was started on
propranolol. However, the patient became very hypotensive, bradycardic and hemodynamically unstable. She
was given atropine, glucagon and started on a dopamine
drip. Chest X-ray indicated right pleural effusion and
thorcentesis confirmed a transudative fluid. Ultrasound of
the abdomen showed ascites. Echocardiogram revealed
normal LV function with ejection fraction of 64% and
moderately enlarged right ventricle and right atrium,
moderate to severe tricuspid regurgitation, and elevated
right ventricular systolic pressure 40-50mmHg. Patient
also had elevated central venous pressures ranging from
27-32cmH2O and elevated pulmonary arterial pressures;
systolic readings in the upper 40’s, suggesting a diagnosis
of isolated right heart failure. She had low systemic vascular resistance of 716, and elevated cardiac output pointing towards high-output cardiac failure due to the effects
of high triiodothyronine (T3). Patient was started on potassium iodide, PTU, and decadron per endocrinology.
With aggressive management the patient became hemodynamically stable with decreasing mean pulmonary arterial pressures and liver function tests. A follow-up chest
X-ray demonstrated decreased pleural effusion. The patient was clinically improved and was discharged on methimazole and propranolol.
Discussion: There are several differentials to consider
when evaluating right heart failure; however, thyrotoxicosis is not commonly on that list. Frequent symptoms of
thyrotoxicosis include palpitation, sinus tachycardia,
atrial fibrillation, hypertension, left heart failure, and angina. Right heart failure is a rare manifestation of thyrotoxicosis and there are very few cases reported. Over the
past 15 years there have been very few case reports of
thyrotoxicosis causing isolated right heart failure. Here
we present a case of a patient with severe thyrotoxicosis,
causing elevated pulmonary artery pressures leading to
right heart failure. Possible mechanisms of pulmonary
hypertension induced by hyperthyroidism entail pulmonary vascular endothelial dysfunction or damage due to
the autoimmune process, the high cardiac output state, or
Heidi Fagerlund, MD; Jigar Patel, MD
Case: A 33 year old male with a history of ankylosing
spondylitis and coronary vasospasm presents with polyarthralgias, fever and chest pain. The patient was initially evaluated in an Urgent Care Center for a one week
history of diffuse polyarthralgias and fevers. He was subsequently diagnosed with presumed influenza and discharged home. He returned for evaluation secondary to
continued fever, new onset migraine symptoms, nausea
and substernal chest pain with radiation to the neck. On
evaluation his temperature was 39.5 C, blood pressure
115/72, heart rate 72 and oxygen saturation 99% on room
air. He was slightly diaphoretic with a normal jugular
venous pulsation. Cardiovascular and pulmonary examinations were normal, with no murmurs, rubs or gallops
auscultated. There were no focal deficits or meningeal
signs. Initial electrocardiogram (EKG) displayed diffuse
ST elevations. Chest x-ray did not identify any acute cardiopulmonary process. Transthoracic echocardiogram
was performed and revealed normal left ventricular (LV)
size, normal LV thickness, and normal LV function, with
a preserved LV ejection fraction. There was no evidence
of pericardial effusion or wall motion abnormalities Review of previous coronary angiogram in 2005 was negative for obstructing lesions. Laboratory values were significant for sedimentation rate 6, AST 158, ALT 210.
Serial cardiac markers revealed a peak Troponin-I of
9.08, total CK 484 and CK-MB fraction of 44.3. Cardiac
MR illustrated a pattern of late enhancement consistent
with myocarditis. CMV IgM serology returned positive at
1:10 with a negative IgG antibody. The presence of CMV
was confirmed by PCR. Serologies for other viruses including HIV and EBV were negative. Prior to discharge
our patient was started on Colchicine therapy with improved symptoms.
Discussion: This case was unique in that although CMV
is a ubiquitous organism, CMV myopericarditis is an
unusual and relatively under-studied entity. Of particular
interest is that almost all documented cases of CMV pericarditis have been in immunocompromised or transfused
hosts whom are rarely seropositive. Ankylosing spondylitis portends additional importance, although unlikely
related to acute myopericarditis, there is a higher incidence of Aortic Insufficiency and conduction disturbances in this population. This case highlights the importance of identifying atypical etiologies of a common
complaint in the ever emerging field of science.
Recurrent Pneumothoraces As a Complication in a
Patient with Idiopathic Lipoid Pneumonia
Farbod Farmand, DO; Castro, Robert DO; Chen
Cynthia, MS3
23
Western University of Health Sciences
Lipoid pneumonia is a very rare pulmonary disorder resulting from exogenous inhalation of lipid materials or
endogenous deposition of fat in the lung tissue. Its diagnosis is usually difficult as there are no pathognomonic
imaging studies or serological tests for it. Here we report
the case of a patient with recurrent pneumothoraces and
underlying lipoid pneumonia found on lung biopsy. Since
there was no positive history of lipid exposure or endogenous mechanism, it was diagnosed to be idiopathic.
To our knowledge, this is the first reported case of idiopathic lipoid pneumonia that presented with recurrent
pneumothoraces.
A Rare Cause of Headache and Gait Instability
Charles Feng, MD; Pouya Afshar, MD; Charles
Smith, MD
Scripps Clinic/Green Hospital
Creutzfeldt–Jakob Disease is a prion disorder that causes
severe neurological dysfunction. It commonly presents
with rapid-onset dementia, myoclonus, ataxia, and speech
impairment.
We report a 64-year-old female who came to clinic for a
headache and difficulty ambulating. At the same time,
she stated that her thought processes had "dulled," and
that she had problems recalling facts. The patient had
been well prior to the visit. Physical exam did not reveal
any evidence of dementia. Aside from a mildly unsteady
gait, there were no focal neurologic deficits. Further, a
work-up for dementia, including CT of the head without
contrast, B12, folate, ESR, CRP, and TSH, was negative.
The patient was referred for physical therapy for her gait
disturbance. Two months later, the patient came back for
follow-up, and her condition had noticeably deteriorated.
She had difficulties speaking and could not stand without
the assistance of her husband. Physical exam showed a
saccadic ocular pursuit, and unsustained upbeat nystagmus on upward gaze.
An extensive work-up was initiated. The cerebrospinal
fluid was acellular, with normal glucose and protein levels. A test for the anti-Yo antibody, which is specific for
paraneoplastic cerebellar degeneration, was negative.
However, EEG showed background slowing with periodic sharp waves and bursts of high-voltage delta waves.
In addition, MRI showed bright cortical signaling over
the right frontal lobe and the medial aspect of the right
occipital lobe on diffusion-weighted imaging. Given the
high clinical suspicion for CJD, the 14-3-3 protein, found
in the CSF, was sent off and came back positive. The
patient passed away one month after her last clinic visit.
Our current knowledge of CJD is limited. A diagnostic
test such as EEG has a sensitivity of 64 percent and a
specificity of 91 percent, while an abnormal 14-3-3 protein has sensitivities and specificities ranging from 53 to
88 percent. Thus, definitive diagnosis can only be made
with a brain biopsy. What’s more, there is no effective
treatment for the disease.
CJD occurs with a prevalence of 1 case in one million
people, but the rapidity with which a patient declines
after diagnosis makes CJD a devastating disease to endure. This case illustrates the importance of considering
CJD in the initial differential of a patient with rapid-onset
dementia and ataxia. Even though there are no treatments,
early diagnosis has many practical benefits. The patient
and her family, along with her physician, can properly
manage expectations, and can mentally and physically
prepare for what will happen as the disease progresses.
Moreover, early diagnosis would allow for more clinical
trials to be performed, so that, in time, a cure for CJD
will eventually be found.
Cancer…. Not Always the Answer
Aimee French MD; Nader Kamangar MD
Olive View- UCLA Medical Center
Case: 32 year-old nulligravid heterosexual Hispanic female, diagnosed with HIV/AIDS presented with two
weeks of pressure-like abdominal pain, increasing abdominal girth, nausea and vomiting. patient also endorsed
subjective fevers, chills, and night sweats. She noted a
40lbs unintentional weight loss. She denied cough and
hemoptysis. Her only medical history was HIV/AIDS.
She was taking HAART therapy , TrimethoptimSulfamethoxazole, and Azithromycin. She was febrile to
39.7, BP 116/65, HR 80, RR 18, and oxygen saturation
100% on room air. She was ill-appearing and cachectic.
She had bilateral post-cervical, submandibular and axillary lymphadenopathy all of which were firm, fixed, and
non-tender. Pelvic exam revealed bilateral tenderness of
the adenexa. No adenexal fullness was noted. The remainder of her exam was normal. Laboratory studies revealed a CD4 count of 60 cells/mL and an HIV RNA of
2970 copies/mL. CA-125 72 units/mL (normal 0-34). The
following studies were (-): sputum AFB, fungal stains
and cultures, Pneumocystis jiroveci pneumonia (PCP),
cytomegalovirus (CMV), coccidioides, and cryptococcus;
blood cultures for bacteria and fungus; urine for histoplasmosis and legionella. CT chest revealed scattered
bilateral parenchymal nodular opacities with mediastinal
and hilar lymphadenopathy suspicious for infectious or
metastatic disease. Abdominal CT scan revealed omental
caking, ascites, lymphadenopathy throughout the abdomen including the pelvic side walls, and cystic structures
present on bilateral adenexa suspicious for ovarian malignancy. Fine needle aspiration of enlarged lymph nodes
and interventional-radiology guided biopsies of the
omental wall revealed necrotizing granulomas with extensive necrosis and visible acid fast bacilli. AFB stool
was (+).
Discussion: This case illustrates that without a high clinical suspicion the diagnosis of abdominal tuberculosis
may be missed or delayed. Once thought to be rare in
developed countries, the incidence of abdominal TB is
24
rising. With the recent HIV/AIDs pandemic the incidence
of tuberculosis is growing along with the frequency of
extrapulmonary disease. Abdominal TB is also more
prevalent and may or may not include pulmonary manifestations. Without a high clinical suspicion diagnosis
may be missed or delayed. Multiple case reports and
journal articles site the repeated misdiagnosis of abdominal TB as malignancy specifically ovarian cancer and
lymphoma. As with our patient, tissue biopsy made the
diagnosis in these misdiagnosed cases. Without coexisting work-up for abdominal TB therapy in this patient
therapy may have been delayed.
Amyotrophic Lateral Sclerosis Presenting as Recurrent Pseudomonas Pneumonias
Natalie Garcia, MD
University of California, San Diego
Case: A 56-year-old man was transferred from an outside
hospital with pneumonia and weight loss. Repeat sputum
cultures grew Pseudomonas, and the patient was treated
with three courses of antibiotics during the two months
prior to transfer. Review of the patient’s history revealed
he had been admitted to various institutions a total of five
times in the past two years for recurrent Pseudomonas
pneumonias and had previously been diagnosed with obstructive lung disease based on hyperexpansion on chest
x-ray, though no pulmonary function studies were available. In addition, though he had been obese, the patient
was cachectic on presentation with a 97kg weight loss
over the previous two years. He had a history of depression and aggressive dieting, and the weight loss was initially attributed to a combination of these compounded by
his recent, prolonged illness. During the initial two weeks
after transfer, the patient had recurrent decline in his respiratory status requiring mechanical ventilation. He
would improve gradually with therapeutic bronchoscopy,
aggressive pulmonary toilet and physical therapy only to
worsen again. After two months of hospitalization, the
patient required on-going, night-time mechanical ventilation to avoid hypercapnic respiratory failure. Neurology
was consulted for muscle weakness and atrophy, which
were initially thought to be secondary to his recurrent
pneumonias and prolonged illness in combination with
statin therapy. On repeat evaluations and exams, he was
noted to have the onset of tongue fasciculations and hyperreflexia in his lower limbs. Given the progressive nature and the development of both upper and lower motor
neuron signs, amyotrophic lateral sclerosis was diagnosed.
Discussion: This case reiterates the potential variability
in presentation of amyotrophic lateral sclerosis and emphasizes the need to pursue an underlying diagnosis
rather than to simply treat the complications. Primary
respiratory muscle weakness is the presenting symptom
in only 1-3% of cases of this rare disease, and early diagnosis of hypoventilation followed by pulmonary function
tests, demonstrating low forced vital capacity, and, possibly, phrenic nerve conduction studies may be useful. The
ultimate recognition of this patient’s disease was integral
in formulating a long-term treatment plan and in helping
him cope with his illness.
Thyroid in the Brain
Vikram Garg, MD; Tanya-Sue Winey, MD
Scripps Green Hospital
Case Presentation
An 81 year-old female presented symptoms of left sided
weakness and ataxia for 3 weeks. The weakness was
causing her difficulty with walking. She had a history
significant for papillary thyroid carcinoma which was
initially diagnosed in 2001 and treated with surgery and
radiation. She suffered 2 recurrences in regional lymph
nodes in 2004 and 2005 and she was treated at both times
with surgery and radiation. Her current medications include Lipitor and Synthroid. She is a non-smoker, originally from Mexico, and moved to the United States 5
years ago. Family history was significant for 1 brother
with leukemia. On exam she was afebrile and hemodynamically stable. Pertinent neurological findings included
3 beat nystagmus with leftward gaze, left facial droop,
left sided lower and upper extremity strength 4/5 compared to 5/5 on the right, sensory deficits on the left compared to the right, and ataxia towards the right with ambulation. The remainder of the physical exam was unremarkable. Initial basic metabolic panel and liver function
tests were unremarkable. Her CBC showed a white cell
count of 7.0, hemoglobin of 13.2, hematocrit of 36.7, and
a platelet count of 207. ESR was13 and her INR was 1.0.
An MRI showed a 3.6 cm x 2.6 cm right posterior frontal
lobe mass with surrounding edema and multiple hemorrhagic foci within the mass (Figures 1 and 2). Imaging
evaluating for other possible metastatic sites was unrevealing. Neurosurgical consultation was obtained and the
patient opted to have surgical removal of the mass. Biopsy results from the mass revealed papillary thyroid
carcinoma. The patient recovered from the surgery quite
well and was discharged in stable condition to a neurological rehabilitation
Discussion
Several studies have looked at the incidence of cerebral
metastases of papillary thyroid carcinoma and the results
range from 0.4% to 3% as the first site of distant metastatic involvement (1,3). However, when looking at second or subsequent distant metastatic sites approximately
1/3 involved the brain in one retrospective study (3). Patients with brain metastases almost always have complications with metastases at other sites such as liver, lung,
or bone with the lung being one of the common distant
metastatic sites in one study (3). Cerebral metastases
suggest an aggressive disease and have an unfavorable
prognosis. However, early detection and treatment can
lead to long-term survival. Our patient had local lymph
node recurrence but no distant metastatic organ involvement so this case was unique in that the brain seemed to
be the first site of distant metastasis.
25
ICD Lead Thrombus Causing Multiple Pulmonary
Emboli
Paul Gerczuk, MD
University of Southern California
This is a 36-year-old Hispanic female with a known history of congenital long QT syndrome who was admitted
to the hospital because of an implantable cardioverter
defibrillator (ICD) lead thrombus, which was incidentally
found on routine echocardiography. The patient was diagnosed with long QT syndrome in 1993 after surviving
an episode of sudden cardiac death; an intra-abdominal
ICD was subsequently placed. In 1996, the intraabdominal ICD was exchanged for a subclavian ICD.
Three days prior to admission the patient underwent routine transthoracic echocardiography in preparation for a
generator change that was scheduled for the following
week. The echocardiogram revealed multiple mobile
masses in the right atrium that appeared to be attached to
the ICD lead. The patient was admitted to the hospital for
further workup. She denied any recent difficulty breathing, chest discomfort, palpitations, cough or hemoptysis.
She did admit to initiating oral contraceptive therapy 3
months prior to admission. Cardiopulmonary exam was
unremarkable except for the ICD palpable below the left
clavicle. Bilateral lower extremity exam revealed no
swelling/edema and Homan’s sign was negative. Transesophageal echocardiogram performed on hospital day # 1
showed multiple mobile masses on the ICD lead in the
right atrium, the largest measuring 1.6 cm x 1.0 cm. The
septum was aneurismal and there was likely a patent foramen ovale. Left and right ventricular function appeared
normal. CT pulmonary angiogram was performed on
hospital day #2 and revealed multiple bilateral filling
defects of the pulmonary arteries consistent with pulmonary emboli without filling defects in the lower extremities. Anticoagulation therapy with intravenous heparin
was initiated on hospital day #2. Doppler ultrasound of
the lower extremities and left subclavian and internal
jugular vein were negative for thrombosis. Transesophageal echocardiogram was repeated on hospital day
#12 and showed a decrease in thrombus size from 2.5cm2
to 2.2cm2 . The patient was discharged on hospital day
#14 on oral anticoagulation therapy with coumadin.
Transthoracic echocardiogram was done 1 year later,
which showed complete resolution of all thrombi. The
patient remained asymptomatic and is scheduled to undergo ICD generator change in the near future.
Cytomegalovirus Infection Disguised as an Ulcerative
Colitis Flare
George M. Girgis, MD
University of Southern California
Introduction: Ulcerative colitis is a disease of the intestine characterized by bloody diarrhea and abdominal pain
during periods of flare. On rare occasion, these symptoms
can be caused by infection, subsequently misdiagnosed
and treated as a flare of ulcerative colitis.
Case: A 42 year old woman with a nine year history of
ulcerative colitis presents to the emergency room with
two weeks of progressive abdominal pain and bloody
diarrhea. The pain was characterized as dull and pressurelike, radiating throughout her abdomen, and worse on the
left side. She also complained of 8-10 loose bloody bowel
movements daily. Other symptoms included generalized
fatigue and weakness. Three weeks prior, the patient was
treated for similar symptoms at an outside hospital. A
flexible sigmoidoscopy at that time was consistent with
significant colonic inflammation and ulceration. She was
treated for an ulcerative colitis flare and discharged on
prednisone, oral asacol, and steroid enemas, but the
symptoms progressed. On admission the patient was
afebrile, tachycardic and normotensive. She had pallor,
abdominal pain in all quadrants, and gross blood on rectal
examination. Laboratory values included a WBC of 9800,
Hgb of 10.2 g/dL, platelet count of 502K, and an electrolyte panel within normal limits. Abdominal X-ray demonstrated colonic dilatation but no evidence of toxic
megacolon. She was started on parenteral and topical
steroid therapy, 5-ASA, and bowel rest. Despite aggressive treatment, her symptoms did not improve. A repeat
flexible sigmoidoscopy with biopsy was performed. Biopsy results returned positive for cytomegalovirus infection and she was started on antiviral treatment with parenteral gancyclovir. The symptoms subsequently improved with antiviral therapy and the patient was discharged with Infectious Disease and Gastroenterology
followup.
Discussion: This case represents how CMV colitis can be
mistaken for an ulcerative colitis flare. IBD patients are
commonly immunosuppressed (particularly those on
steroid or immunologic therapy), and therefore presumably at increased risk for opportunistic infections. Clinicians must keep a high index of suspicion for CMV superinfection in cases of IBD refractory to parenteral therapy.
Sarcoid: The Textbook would be Proud!
Thomas Griffiths, MD
University of California, San Diego
Sarcoidosis is a disease of unknown etiology, characterized by noncaseasting granulomas of affected organs.
Known to most to be a cause of large hilar adenopathy,
sarcoid can manifest in many other ways. A 40 year old
African American male seen in a pulmonary clinic after a
chest X-ray for Addiction Day Treatment Program
screening demonstrated radiographic evidence of sarcoidosis. On further questioning the patient described symptoms such as yellowish green sputum in the a.m. for 3-4
years, worsening over the last year. He complained of
skin lesions on his right hand and neck that were non
tender and non pruritic and described a subcuticular mass
the size of a grape in his left axilla. The patient’s physical
exam was pertinent for findings of: stridor and diffuse
wheezing bilaterally, subcuticular nodularity in the axilla,
26
non tender papular lesions that were non erythematous at
the base of penis as well as similar nodules on the extensor surfaces of the hand’s. The insidious nature in which
the disease had progressed was objectively summarized
with flow spirometery. The patient was subjectively asymptomatic of respiratory symptoms however the results
demonstrated otherwise: FEV1/FVC 51.9% of predicted,
consistent with severe obstructive disease. High Resolution CT demonstrated right middle lobe and right lower
lobe atelectasis secondary to hilar adenopathy causing
compression of bronchial tree. Sarcoidosis was confirmed
by skin biopsy from a lesion on the back of the patient’s
neck, which demonstrated classic non caseating granulomas associated with the disease. Treatment was initiated
with a six week tapering course of high dose corticosteroids. The patient’s response to treatment was evaluated
with repeat spirometry which improved dramatically after
the steroid course. Unfortunately the steroid course
proved too much for the patient’s already poor sugar control and he had to be started on oral therapy for type two
diabetes. This case illustrates a classic presentation of a
relatively common disease of which we do not fully understand. The insidious nature in which sarcoid can progress is illustrated by the patient’s subjective asymptomatic state even with his right middle and lower lobes
being completely atelectatic. Further development of
more specific treatment is essential if this disease is to be
treated successfully without other metabolic complications of high steroid therapy.
Making the Diagnosis: Paralyzed Legs and a Cardiac
Mass
Katherine Hartzell, BS; Dana Faw, MD; Rebecca
Liddicoat Yamarik, MD, MPH
University of Calinfornia, Irvine
Case: A 71 y/o Filipino female presents with sudden onset severe burning abdominal pain and paraplegia of bilateral lower extremities. Her vital signs were within
normal limits. PE revealed: mild epigastric pain, 0/5
strength in both lower extremities, absent DTRs, Babinskis neutral, and absent rectal tone. Brian MRI revealed
one thalamic and two cerebellar lesions consistent with
acute infarcts, and T-spine MRI revealed an enhancing
T2 signal abnormality from T8 to T11. The patient’s
presentation was consistent with anterior cord syndrome;
differential diagnosis was transverse myelitis with unclear etiology vs. cord infarction vs. an autoimmune
process.
The patient was initially treated with high dose steroids
and IVIG for 5 days, without change in her symptoms. A
TTE was normal, but a TEE revealed a 1.1cm mass on
the mitral valve. The differential included infective endocarditis manifesting with embolic spinal cord infarction
and embolic strokes. The patient was started on antibiotics. Blood cultures, however, were negative x 5 and the
patient remained afebrile. The location of the embolic
events in the brain and spinal cord were not typical for
emboli originating from the heart. Other etiologies were
considered, including a Rheumatologic disease or a paraneoplastic process from a possible cardiac tumor. ESR
and CRP were normal on admission, but rose to 67 and
18.5 respectively, on day 12. Lupus anticoagulant (antiHex) was positive, and a diagnosis of antiphospholipid
antibody syndrome was entertained. However, the test
was drawn when the patient had a supratherapeutic PTT
secondary to a sensitivity to subcutaneous Heparin and
was thought to be falsely positive. Temporal artery biopsy was also negative, making vasculitis or other
Rheumatologic problem unlikely. Cardiac MRI demonstrated a benign calcification, an unlikely source of emboli. Repeat MRI of the T spine showed expansion of
cord edema consistent with evolution of a previous infarction. Her history of PVD, and evidence of arterial
calcification noted on previous CT made a diagnosis of
anterior spinal cord infarction secondary to diffuse
atherosclerotic disease the most likely cause of her symptoms of paralysis, with simultaneous CVAs due to PVD
seen on brain MRI.
Discussion: This case demonstrates the wide variety of
disease processes that can cause nontraumatic spinal cord
injury, how each of these different processes may manifest, how these conditions are worked up and the importance of keeping a wide differential in order to ultimately
arrive at the correct diagnosis.
An Incidental Finding of Metformin Associated Lactic
Acidosis
Aimee Hechanova MD; Maher Roman, MD, MBA
Loma Linda University
Case: A 76 year-old diabetic male came to clinic to establish care and get medication refills. Physical exam was
unremarkable and the patient appeared well, though on
review of systems endorsed fatigue, nausea, decreased
appetite, and weakness. Per patient, outside labs drawn 3
weeks prior were all normal. He had also gotten a CT
head with contrast one week prior and had resumed metformin immediately afterwards. New baseline labs were
drawn, showing a creatinine of 1.8 and a wide anion gap
acidosis (Na 142, Cl 100, CO2 19.4, AG 22). After reviewing the labs the following day, the patient was contacted immediately, told to stop metformin and come to
the emergency department for further evaluation. Repeat
BMP showed an improvement in renal function (Cr 1.4,
Na 141, Cl 99, CO2 22.8, AG 19), and lactic acid 3.7. The
patient received intravenous fluids, and the next day was
feeling much better with improved mentation, decreased
fatigue, and a good appetite. Repeat labs showed resolution of the patient’s contrast-induced acute kidney injury,
resolution of the anion gap acidosis and normalized lactic
acid levels (Cr 1.2, Na 140, Cl 107, CO2 23.0, Lactic acid
0.8).
Discussion: Metformin associated lactic acidosis
(MALA) is a very rare complication of metformin (3
cases per 100,000) which is crucial to detect because it
carries a mortality rate of 48%. It most frequently occurs
27
after contrast imaging due to contrast induced acute kidney injury. The acute kidney injury allows metformin,
which is renally excreted, to accumulate and cause lactic
acid buildup. This case demonstrates the importance of
having a high index of suspicion for MALA in patients
on metformin with an acute impairment of renal function
and an anion gap acidosis. It also underscores the importance of holding metformin immediately prior to any contrast study and checking the creatinine 2-3 days afterwards to ensure normal renal function before restarting
metformin.
vitamin A. The patient discontinued all nutritional supplementation while in hospital and was discharged home
with the diagnosis of vitamin A induced intrahepatic cholestasis. At 2 months free of nutritional supplementation
the patient’s bilirubin and alkaline phosphatase levels
returned to normal. A review of the literature revealed
one other case of vitamin A toxicity from long term consumption of “therapeutic” vitamin A supplementation. In
intrahepatic cholestasis vitamin A toxicity should be considered even with long term low dose consumption of
vitamin A.
Vitamin A Toxicity from Long Term Herbalife® Dietary Supplement Use
Pheochromocytoma Presenting as Acute Congestive
Heart Failure and Acute Renal Failure
Gary Hensley, MD; Bim Pham
St. Mary Medical Center
Andrew Ho, MD; Run Yu, MD, PhD; Amanda Ewing,
MD
Cedars-Sinai Medical Center
Nutritional supplementation has steadily increased over
the past 20 years, however supplement use is not without
risk. Vitamin A induced liver disease has been recognized for over 100 years. Liver hepatotoxicity as a result
from low “therapeutic” doses of vitamin A has not been
considered clinically significant. Here we present a case
of intrahepatic cholestasis from prolonged ingestion of
vitamin A from Herbalife® dietary supplements. A 46year-old male presented to our ED with complaints of
worsening jaundice and pruritis for 2 weeks. Upon admission to our ED the patient had normal vital signs;
physical exam was significant for scleral icterus, jaundice, palpable liver at 2cm below the costal margin, and
negative hepatitis and autoimmune serologies. An abdominal ultrasound showed a common bile duct of
4.7mm, no evidence of gallstones, no gallbladder wall
thickening, and no intrahepatic biliary dilation. Initial
liver serologies revealed a total bilirubin 10 times the
upper limit of normal, a direct bilirubin 21 times the upper limit of normal, and an alkaline phosphatase 1.5 times
the upper limit of normal. AST and ALT values were
within normal limits. Total and direct bilirubin and alkaline phosphatase continued to increase daily and on hospital day 4 an ERCP was performed and a biliary stent
was placed. A second ERCP was performed on hospital
day 8, due to continued increasing bilirubin and alkaline
phosphatase levels, which demonstrated a biliary stent
that had good bile and sludge flow. Yet the stent was
removed and replaced. On hospital day 13, a liver biopsy
was performed for increasing total bilirubin 20 times the
upper limit of normal, direct bilirubin 40 times the upper
limit of normal and alkaline phosphatase 2.2 times the
upper limit of normal despite 2 normal ERCPs. Pathology
revealed prominent centrilobular cholestasis with activated stellate cells with increased vacuoles, intracellular
necrosis with apoptotic bodies observed in Kupffer’s
cells, dilated bile ducts, and bile thrombi. Based upon
these results a more detailed history was obtained and the
patient had been consuming a Herbalife® Healthy Meal
Nutritional Shake and two Herbalife® Multivitamin
Complex pills daily for the past 12 years. This nutritional
supplementation amounts to approximately 5000IU of
vitamin A which is also the recommended daily dose of
A 41-year-old male with a history of hypertension on no
medications, presented to the emergency room complaining of one week of shortness of breath with exertion. Patient also noticed bilateral lower extremity swelling with a 15 pound weight gain. Pt denied chest pain,
orthopnea, sick contacts, fevers, night sweats, or recent
travel. Pt did report intractable headaches, exacerbated
by occasional episodes of flushing over the past few
years.
While in the ER, patient's vitals were 98˚F, 98
beats/min, blood pressure 201/132 and O2 saturation of
99%. Physical exam revealed an obese man, with elevated jugular venous pressure, bilateral crackles at the
lung bases with dullness to percussion on the right side.
Patient's heart was tachycardic but regular, with bilateral
lower extremity pitting edema.
Labs revealed Cre 1.4, hemoglobin 11.9, BNP 478, troponin 0.03. EKG showed sinus tachycardia, poor R
wave progression, low voltage and QTc472. A chest xray showed congestive heart failure (CHF) and a right
pleural effusion.
CT chest with contrast was performed because of suspicion for PE and was negative, but bilateral adrenal
masses were incidentally found measuring 7cm in the
left and 3cm in the right.
Transthoracic echocardiogram revealed an ejection fraction of 55 to 60%, left atrium and ventricle dilation,
moderate to severe mitral regurgitation, and diastolic
dysfunction.
Management of patient’s heart failure included diuresis
and blood pressure control. Attention was given to not
use beta blockers while waiting lab results given a high
suspicion for pheochromocytoma. The patient’s clinical
condition improved.
Laboratories confirmed pheochromocytoma with urinary catecholamine metabolites markedly elevated
28
(metanephrine level of 12,600 μg/day; normetanephrine
level of 5040 μg/day), and the fractionated plasma free
metanephrines elevated at 22.3 and 17.9 for metanephrine and normetanephrines respectively.
sis, fibrosing interstitial pneumonitis, and perivascular
amyloid deposits consistent with amyloidosis. Tests for
other sites of amyloid involvement, including fat pad
biopsy, echocardiogram, and urine studies, were normal.
Adrenal pheochromocytomas are neuroendocrine tumors
that secrete high levels of both epinephrine and norepinephrine. Patients with a pheochromocytoma classically
present with the triad of headaches, palpitations, and diaphoresis. However, patients can also present either
asymptomatically or with chest pain, shortness of breath,
flushing, or anxiety. It is also known that pheochromocytomas can present with refractory hypertension. It is unusual however, for a patient with a pheochromocytoma to
present in acute decompensated CHF requiring hospitalization like our patient. Some have suggested that patients
with an unknown etiology of CHF be screened for pheochromocytoma, but as of current practice, this is not
widely done. In a patient with CHF where there is a suspicion of pheochromocytoma, caution should be used in
initiating beta blockers in the treatment regimen to avoid
unopposed alpha-adrenergic receptor stimulation and
further blood pressure increases. In summary, we present
a young patient with no history of previous myocardial
damage, who was in florid CHF, found subsequently to
have pheochromocytoma.
The patient’s pulmonary symptoms improved with intravenous ceftriaxone and azithromycin. Her arthralgias
were treated initially with ibuprofen, but after the severity
worsened, she was given intravenous methylprednisolone. At discharge, she was given prednisone, azathioprine, and prophylactic trimethoprim-sulfamethoxazole.
Not Your Average Pneumonia: Primary Pulmonary
Amyloidosis
Siwen Hu-Lieskovan, MD, PhD; Taib Rawi, MD; Andrew Hendifa, MD; Samina Ahmed, MD; Aarti
Chawla, MD; Jose Gonzales, MD; Emanuel Voyiaziakis, MD; Howard Liebman, MD; Bharat Chaudry,
MD
George Holman, MD
University of Southern California
Case: A 61 year-old woman with prior history of
trigeminal neuralgia was brought to our hospital after
being treated for presumed pneumonia at another clinic.
Two months prior to admission, she had acute onset of a
dry cough and pleuritic chest pain. She went to her clinic
after the symptoms persisted for four weeks and was initially given a week-long course of an unknown antibiotic.
She improved while taking the antibiotic, but her symptoms returned after completing the regimen. She also
developed fevers and severe arthralgias, mostly in her
wrists and left hand. She returned to the same clinic and
was given a different antibiotic for another week. Again,
she improved while taking the antibiotic, but her symptoms returned after finishing it.
On physical exam, she was found to be afebrile, tachypneic, and had an oxygen saturation of 94% on room air.
She had bibasilar lung crackles. There was tenderness
and swelling in both wrists and the joints of her left hand,
but no erythema or warmth. Results of routine lab studies
were normal except for albumin 3.1g/dL and magnesium
1.5mg/dL. ESR was 67mm/Hr and high-sensitivity CRP
was 68mg/L. Later, she was found to have positive ANA,
rheumatoid factor, pANCA, and anti-myeloperoxidase
antibody. The chest x-ray showed a reticulonodular pattern in the lung bases, so a high-resolution CT was performed. It found idiopathic pulmonary fibrosis. A bronchoscopy was performed and found peribronchial fibro-
Discussion: This patient’s presentation was unusual for
amyloidosis because she was acutely symptomatic and
amyloid was only found in the lungs. In general, isolated
pulmonary amyloidosis is seen as a lung nodule and
caused by abnormal B cell proliferation. Diffuse pulmonary amyloidosis is associated with systemic involvement
and predominantly affects the kidneys and other organs
before the lungs. As was done in this case, the diagnosis
of amyloidosis requires further studies to determine the
extent of the disease and to rule out inflammation, infection, neoplasm, or genetic disease as secondary causes.
A Case of Warm-Antibody Autoimmune Hemolytic
Anemia without Spherocytosis
University of Southern California, Dept. of Medicine/Hematology
Autoimmune disorders are often associated with secondary autoimmune hemolytic anemia (AIHA), mostly
warm antibody (Ab) AIHA. Patients with this variant
present with varying degrees of anemia, jaundice and
hepatosplenomegaly, with lab values showing increased
LDH, indirect bilirubinemia, decreased haptoglobin &
reticulocytosis. One diagnostic hallmark of warm Ab
AIHA (WAHA) is spherocytes in the peripheral smear,
due to splenic sequestration and monocyte/macrophagemediated partial destruction of Ab-attached RBCs. We
report a case of WAHA without spherocytosis. Patient is
a 61 year-old Hispanic woman with hypothyroidism
treated with Synthyroid for 3 years and interstitial cystitis
treated with Elmiron for 2 years, who presented 2-months
of fatigue, dizziness & jaundice. Hb was 4.5 gm%, with
increased LDH (456), indirect bilirubin (3.5) and decreased haptoglobin (<20). Elmiron was held. Type &
cross showed the patient was (+) for indirect Coomb’s
test with non-specific reactivity (NSR) but (-) for direct
antiglobulin test (DAT). Patient denied past transfusions/pregnancies. Peripheral smear showed reticulocytosis but no spherocytes. Two units of matched pRBCs
were transfused twice during the hospital stay. The patient initially responded, but in both instances Hb
dropped over the next 3 days. Cold hemagglutinin test
29
was (-). Patient was also found to have autoimmune hepatitis with (+) titers for ANA & anti-smooth muscle Ab &
elevated AST/ALT. Abdominal US showed cirrhosis &
enlarged spleen (13cm). Coomb’s-(-) hemolytic anemia
work-up demonstrated a (+) DAT due to IgG (warm
autoantibody of broad specificity) & complement-coated
red cells. The patient's indirect antiglobulin test was also
(+) with NSR. She responded well to Prednisone 50 mg
po daily, with stabilized Hb. This patient has a classic
presentation of secondary AIHA due to autoimmune disease with (+) warm antibodies on RBCs & in the peripheral blood but without peripheral spherocytes. One possible explanation is complete splenic sequestration of Abcoated RBCs by macrophages circulated out of the
spleen. DAT-negative non-spherocytic AIHA is a known
entity but DAT-positive non-spherocytic AIHA has rarely
been reported in the literature (Pubmed search, 9/09).
This case demonstrates that the diagnosis of AIHA can
not be excluded in the absence of spherocytosis.
Indinavir-Induced Nephrolithiasis after Cessation of
Indinavir Therapy
Julie Huynh, MD; John J. Sim, MD; and Aviv Hever,
MD
Kaiser Permanente Southern California
Indinavir sulfate is a protease inhibitor used in the treatment of HIV by inhibiting the cleavage of precursor
polyproteins into functional infectious proteins. Indinavir-induced nephrolithiasis is a well reported side effect of the medication; however, there is little literature
regarding the occurrence of indinavir-induced nephrolithiasis after cessation of therapy. We present a patient
with confirmed indinavir-induced nephrolithiasis 3.5
years after the cessation of indinavir therapy.
A 49-year-old man with HIV was treated with a HAART
regimen that included indinavir. After eight years of therapy, the patient presented with signs and symptoms of
nephrolithiasis and indinavir had to be discontinued. A
CT KUB at that time demonstrated mild bilateral hydronephrosis and right hydroureter consistent with obstruction although no radioopaque calculi were seen. The
patient’s symptoms resolved with the discontinuation of
indinavir and conservative management. A follow up
renal furosemide scan performed showed no evidence of
stone or obstruction.
More than three years after the patient discontinued indinavir therapy, the patient again presented with gross hematuria and right flank pain. A CT KUB showed complete resolution of the previously mentioned left hydronephrosis. However, there was moderate dilatation of
the right intrarenal collecting system and right ureter,
again with no evidence of calculi. The patient eventually
passed multiple stones with resolution of symptoms.
Stone composition analysis revealed indinavir.
This case shows that indinavir-induced nephrolithiasis
can present many years after cessation of therapy. Indi-
navir stones are not easily detectable because they are
radiolucent and not identifiable on CT. In addition, because of their gelatinous consistency, indinavir stones can
cause high grade obstruction and are not amenable to
lithotripsy. Although conservative management may be
effective, some cases may require ureteral stenting.
Therefore, there must be a high clinical suspicion of indinavir-induced nephrolithiasis in patients with symptoms
of flank pain or hematuria who have a history of indinavir
use, no matter how remote.
Lemierre's Syndrome - Forget Me Not
Ngoezi Iroezi, MD; Jenny Shen, MD; Neveen El
Farra, MD
University of California, Los Angeles
Introduction: This case displays the presentation and
clinical features of Lemierre’s syndrome & aids in broadening the differential for a young adult presenting with
non-resolving fevers/pharyngitis.
Case description: 19 year old male presented to the hospital with 2 weeks of persistent high fevers and nonresolving sore throat. In the week prior to admission, he
was examined twice and found to have no lymphadenopathy or tonsillar exudates. He also had 2 (-) strep
throat cultures and a (-) Monospot. He represented to the
ER and in the interim had developed left upper quadrant
abdominal pain with pleurisy, as well as numbness under
his right jaw. Review of systems was notable for a mild
HA, the absence of cough, and no signs of meningismus.
He denied intravenous drug use or unprotected sexual
contact. Physical exam was notable for a temperature of
38.7 Celsius, pulse of 126, BP 142/51, respiratory rate 20,
& 95% oxygen saturation on room air. He had submandibular lymaphadenopathy, an erythematous posterior
pharynx with no ulcerations and exudates, crackles at
bilateral lung bases, and splenomegaly. Labs showed a
mild leukocytosis, a positive Epstein Bar Virus EBNA
antibody, indicating an infection of mononucleosis at
least 6 weeks old and a negative rapid HIV. A CT of the
chest revealed findings consistent with septic emboli.
Echocardiogram showed no vegetations. An MRI of the
neck, however, revealed an abscess in the medial margin
of the right mandibular body. Blood cultures grew Fusobacterium Necrophorum. The patient clinically improved
on meropenem and metronidazole finishing a 14 day
course upon discharge.
Discussion: Lemierre’s syndrome involves a septic
thrombophlebitis of the internal jugular vein caused by
Fusobacterium necrophorum. F. necrophorum is an anerobic gram rod, a normal flora of the oral cavity, gastrointestinal and female genital tract. The pathogenesis is
characterized by hematogenous spread from a peritonsilar
abscess to the jugular vein. Classic signs and symptoms
include neck pain and swelling, septic emboli to the
lungs, fever, leukocytosis, and thrombocytopenia. It has
been hypothesized that immunosuppresion from a preceeding infection such as mononucleosis may predispose
30
individuals to Lemierre’s syndrome. It may lead to lifethreatening erosion of the vein or invasion into the adjacent carotid artery. It can be easily treated with antibiotics
if caught early. It is known to occur in immunocompetent
young adults. Thus a thorough evaluation of a nonresolving pharyngitis that includes an infectious workup
and appropriate imaging will preclude missing the almost
forgotten syndrome- Lemierre's.
Non-Hodgkin Lymphoma Presenting as Pleural Effusion with Dermatomyositis
Farokh Jamalyaria, MD; Michelle Phillips, MD;
Enoch J. Wang, MD
Scripps Green Hospital
Introduction: An 84-year-old man presented with a
three-week history of progressive weakness in his shoulders and hips and a progressively nonproductive cough.
He complained of difficulty brushing his hair, unsteadiness on his feet, and had suffered a mechanical fall. Over
the six months prior to admission, he had unintentionally
lost 30 pounds. He denied pleuritic pain, fever, chills,
rigors, night sweats, lymphadenopathy, or chest pain. He
had a three-month history of the following: erythema and
exfoliation over the forehead consistent with
erythroderma, heliotrope rash under the eyes,
poikilodermatous macules over the nape and shoulders
consistent with a positive shawl sign, patchy redness over
the knuckles bilaterally consistent with Gottron’s sign,
and periungual erythema over the right fifth digit. His left
lung had markedly decreased breath sounds throughout,
with dullness to percussion and decreased tactile fremitus, but without egophony. The patient had coronary artery disease and a smoking history of occasional cigar
and pipe use over approximately twenty years, having
quit ten years prior to admission.
Case: Based upon the history and physical findings, a
diagnosis of dermatomyositis was strongly suspected.
Chest x-ray revealed a large left-sided pleural effusion. A
diagnostic and therapeutic thoracentesis was performed;
pleural fluid analysis revealed an exudative picture:
cloudy yellow color, glucose less than 1 mg/dL, protein 4
g/dL, LDH 3983 U/L, WBC 24800/cumm, RBC
30000/cumm, 200 cells counted (1% segs, 5% lymphocytes, 94% mononuclear cells). A comprehensive rheumatologic panel was positive only for ANA panel with
ANA Hep-2 cell titer of >= 640. The following rheumatologic labs were negative: anti-DS DNA, anti-Jo-1,
Smith, RNP, SSA, SSB, Anti-Scl-70, and rheumatoid
factor. Erythrocyte sedimentation rate, uric acid, Creactive protein, and peripheral LDH were elevated at 40
mm/hr [0-30], 14.9 mg/dL [3.5-7.2], > 90 mg/dL [0-10],
and 484 units/L [100-190], respectively. UPEP revealed
albuminuria; no monoclonal proteins identified. SPEP
revealed hypoalbuminemia without monoclonal proteins.
Pleural fluid cultures and blood cultures were negative.
HIV antibody screen was negative. Flow cytometry of the
pleural fluid revealed an aggressive non-Hodgkin lymphoma. CT of the chest, abdomen, and pelvis for staging
was significant for no lymphadenopathy.
Discussion: Non-Hodgkin lymphomas (NHLs) are a diverse group of lymphoproliferative disorders. Up to forty
percent of patients with NHL present with “B” symptoms
(fever, weight loss, and night sweats). About 20% present
with mediastinal lymphadenopathy, and abdominal / pelvic lymphadenopathy is common in most subtypes. Dermatomyositis is a known paraneoplastic syndrome: approximately 15% of patients with dermatomyositis have a
neoplasm. Roughly 10% of patients with NHL present
with pleural disease. However, it is very unusual for NHL
to present primarily as a pleural effusion in an HIVnegative patient. It is more common in HIV-positive patients (HIV-associated pleural effusion lymphoma).
Zollinger-Ellison Syndrome
Aaron Jeng, MD
University of Southern California
Introduction: Zollinger-Ellison syndrome is characterized by hypersecretion of gastric acid, high serum gastrin
concentrations, and non-beta islet cell tumors of the pancreas. It was initially estimated that the incidence in the
United States ranged from 0.1 to 1 percent of patients
with peptic ulcer disease. Gastrinomas are derived from
multipotential stem cells of endodermal origin. Known as
enteroendocrine cells, they arise mainly in the pancreas
as well as the small intestine.
Case Presentation: A 60 year-old woman presented with
the chief complaint of coffee ground emesis. Pt endorses
11 months of inability to retain ingested food. Gradually
during the course of her disease, pt symptoms worsened
and was seen in the ER multiple times. ER visits consisted of treatment with fluids, antibiotics, and PPI. Pt
finally admitted 1 month prior to admission for dysphagia. During pt’s first admission, EGD done and pt was
found to have multiple esophageal and duodenal ulcers.
Pt subsequently sent home with PPI therapy and GI cocktail. Pt now returns with 2 day history of coffee ground
emesis. Pt exam is significant for conjunctivial palor and
epigastric tenderness, otherwise no other findings. Labs
were significant for H/H of 5.3/ 15.6, gastrin level of 886
pg/ml, and chromogranin level of 341.2. Imaging studies
that were done include small bowel follow through with
findings concerning for intestinal disease and a octreotide
scan with findings consistent with gastrinoma. Pt also had
multiple EGDs with multiple esopheal and duodenal ulcers visualized and an EUS attempted but failed because
of a stricture. Pt subsequently diagnosed with gastrinoma
and Zollinger-Ellison syndrome and started on PPI gtt
and converted to PO PPI. Surgical service was consulted
and decision was to schedule pt for outpatient surgical
intervention.
Discussion: Zollinger-Ellison syndrome, unlike typical
peptic ulcer disease, is often progressive and persistent
and can frequently be life-threatening if not diagnosed or
treated appropriately. With current assays for serum gastrin and current imaging, the number of individuals that
31
are diagnosed with the Zollinger-Ellison syndrome have
increased and the diagnosis is often established before
development of the complications of peptic ulcer disease
or spread of a malignant gastrinoma. Additionally, patient
are given appropriate therapy that alters the course of the
disease.
cal history was significant only for Diabetes Mellitus type
II and hyperlipidemia. Diagnostic paracentesis revealed a
SAAG of 0.8 and atypical cells consistent with adenocarcinoma. In conjunction with CT findings and omental
biopsy, the diagnosis of metastatic pancreatic carcinoma
was made.
It’s an Ulcer, an Outpouching, No a Double Pylorus!
Discussion: Ascites is a harbinger of the final stages of
pancreatic cancer. The incidence of clinically detected
ascites in pancreatic carcinoma is reported to be between
15.6% and 38.7% [10]. It occurs relatively infrequently
as many patients do not survive long enough to manifest
with it. Carcinomas of the body and tail of the pancreas
are more likely to induce ascites rather than those of the
head of the pancreas. Nonetheless, the cases of pancreatic
carcinoma with ascites as the initial sign seem to be extremely rare; a mere handful of case reports have described this presentation in the past [10, 12].
Radha Juneja, MD; Eric Choi
Scripps Green Hospital
A double pylorus is an accessory pyloric channel that can
be classified as congenital or acquired. The most common cause is a complication of an ulcer that leads to intramural erosion between the lesser curvature of the
stomach and duodenal bulb. This case provides a possible
interesting alternative to the etiology of this rare abnormality. A 76-year-old man presented with progressively
worsening RUQ pain that began 2 days prior to admission after eating a large meal. The pain was constant,
non-radiating, sharp, and 8/10 in intensity. He denied
associated symptoms. He attempted to relieve his symptoms with TUMS, and positional changes without avail.
His PMH was significant for osteoarthritis treated with
diclofenac twice daily for 15 years. Abdominal exam
revealed a soft, non-distended abdomen, tender in the
right lower quadrant. The patient’s abdominal CT revealed inflammation stranding along the second portion
of the duodenum. A 2.4 cm round posterior outpoutching
was amidst this inflammation, suggesting the possibility
of duodenal diverticulitis. The patient was admitted and
treated with ciprofloxacin and flagyl. Additionally, the
patient was started on a proton pump inhibitor and advised to stop taking diclofinac. The symptoms resolved
within 48 hours and the patient was discharged in stable
condition. Three weeks later, he underwent a follow up
upper endoscopy. Gastric and duodenal mucosa showed
no evidence of erythema or mucosal erosion. Insufflation
of the stomach revealed 2 separate channels from the
gastric lumen into the duodenal bulb. The endoscope
could be passed through both ducts, and the findings were
verified by infusing water through the ducts and into the
lumen of the duodenum. This case suggests the possibility that a double pylorus may be acquired secondary to
duodenal diverticulitis leading to inflammation, erosion,
and intramural fusion. However, we cannot be sure that
this case, like the majority of other cases of double pylorus, was not a complication of peptic ulcer disease. Although, there was no evidence of gastric ulcers on endoscopy, the patient’s long history of chronic NSAID use
also makes this etiology quite possible.
New Onset Ascites Resulting in Diagnosis of Metastatic Pancreatic Carcinoma
Mary Kamel, MD
Ceadrs-Sinai Medical Center
Lost to Follow-up: Acute Paralysis and Cyanosis
Sarah Kemble, MD; Jamil Aboulhosn, MD
University of California, Los Angeles
Case: A 20-year-old male developed sudden onset of
confusion, slurred speech, and left-sided weakness. On
arrival of paramedics, he was also noted to have perioral
cyanosis. His mother reported he had always been blue,
and had undergone heart surgery in childhood. He had
been followed by a pediatric cardiologist, but at age seventeen, his father lost his job, and he lost the health insurance provided under his father’s plan. He continued to
take aspirin, and seemed to be doing well, working full
time and playing pick-up football on the weekends. However, over the last month, he had become increasingly
short of breath with exertion. He had worsening cyanosis
and was unable to work due to fatigue. His mother had
also noticed snoring at night and pauses in his breathing
which sometimes caused him to awaken from sleep. In
the Emergency Department, a CT scan of the head
showed no intracranial bleed. Oxygen saturation was
80%. Tissue plasminogen activator was administered,
with some improvement in speech and strength. An MRI
of the brain showed a right middle cerebral artery infarct.
A bedside echocardiogram demonstrated a single right
ventricle, D-transposition of the great arteries with likely
Stansel anastamosis, hypoplastic left ventricle, mitral
atresia, lateral tunnel Fontan with fenestration and right
to left shunting, non-obstructive ASD, and non-restrictive
VSD. Right ventricular ejection fraction was reduced at
20%. Cardiac catheterization was performed, and the
patient underwent Amplatzer device closure of the Fontan fenestration. Significant innominate to coronary sinus
venous collateral flow was noted and treated with coil
embolization. The patient demonstrated improved oxygenation following these procedures, and was started on
warfarin anticoagulation and afterload reduction. He was
discharged to an acute rehabilitation facility for further
functional recovery following his stroke.
Case: 64 year old male was transferred to our facility due
to new onset ascites of unknown etiology. His past medi-
32
Discussion: After Fontan repair, patients are at higher
risk for thrombosis and arrhythmias, particularly atrial
fibrillation. Fenestrations in the Fontan conduit, as well
as arteriovenous collaterals or malformations which develop as a result of high Fontan pressures can cause
shunting, chronic hypoxia, and predispose to embolic
stroke. Anticoagulation may be considered routinely
post-Fontan to prevent thrombus formation and reduce
stroke risk. Survivors of congenital heart surgery must
undergo routine follow-up, and should be screened for
comorbid conditions that affect the hemodynamics of
their repair. In this case, obstructive sleep apnea may
have contributed to increased Fontan pressures, further
predisposing to thrombosis and Fontan failure. As more
adolescents age out of health insurance coverage with no
affordable alternatives, inadequate access to care has become a critical comorbidity for survivors of chronic diseases of childhood.
De Novo Epitheloid Angiosarcoma of the Urinary
Bladder
Michael Keng, MD; Aashini Master, DO
University of Southern California
Background: Angiosarcoma of the bladder is exceedingly rare and often fatal. Only 12 cases of primary angiosarcoma have been documented in the literature, and
even fewer de novo cases reported. The following case
presentation is a patient with de novo epithelioid angiosarcoma of the urinary bladder.
Case Report: The patient was a 60-year-old Caucasian
man with coronary artery disease, hypertension, and right
knee arthritis, who presented with bilateral leg pain for
nine months, worsening over the last two months. He also
admitted to occasional fever, sore throat, and dysuria.
He had recently visited Panama in May 2008. After returning, he developed right foot pain and lesions and bilateral lower extremity rash. Leg x-rays were normal.
Biopsies of the thigh and feet revealed keratosis and leukocytoclastic angitis and keratin debris, respectively. The
patient’s feet remained painful, and sudden onset of left
foot numbness and cyanosis prompted hospitalization.
Social history was remarkable for alcohol use, 20-packyear tobacco history, and remote history of heavy LSD
use. He worked in the construction industry and had a
history of unprotected sex.
Initial physical examination revealed normal vital signs,
bilateral leg edema up to the mid calf, and white-brown
verrucous lesions on the plantar surface of the right foot.
The toes of the left foot were cold, purple and insensate
with weeping, scattered bullous lesions, necrosis and
purple discoloration. Significant serological tests revealed: white blood count 17.3K/cumm, LDH
164units/L, ESR 112mm/hr, CRP 260.9mg/dL, and positive lupus anticoagulant. All other routine, infectious, and
rheumatologic serologies were negative.
Leg x-rays showed multiple lesions of both femurs with
fracture of the right femur. MRI of the left foot showed
dry gangrene and right femur showed a pathologic subtrochanteric fracture suspicious for telangiectatic osteosarcoma. CT scan showed scattered nodules in the
lungs, lytic lesions of the right femur, multiple lytic lesions of the pelvis. Biopsy of the right femur revealed
epithelioid angiosarcoma. Ultrasound showed a thrombus
in the right superficial femoral vein.
While in the hospital, he developed gross hematuria,
which was thought to be secondary to anticoagulation.
The patient received bilateral below-the-knee amputations for progressive gangrene. Unfortunately, shortly
after, he expired from cardiopulmonary arrest.
Postmortem examination revealed epithelioid angiosarcoma of the urinary bladder with metastases to the lungs,
skin, prostate, scrotum, and bone. Additionally, three
pulmonary emboli were noted.
Conclusion: Angiosarcomas are rare mesenchymal neoplasms that most often involve the skin and less commonly the deep tissues. The head and neck region is the
most common site of angiosarcomas, but reports have
indicated the presence of tumor throughout the body,
including the heart, thyroid, breast, ovary, retroperitoneum, and other sites. Angiosarcoma of the bladder is
exceedingly rare and epithelioid appearance has been
documented previously in only two cases.
Hyperfibrinolytic Activity in Patients with Liver Cirrhosis
Michael Keng, MD; Yee Lu, MD; Matthias Barden;
Eric Hsieh, MD
University of Southern California
Background: Hyperfibrinolytic activity is a poorly understood complication of liver dysfunction, partially due
to the diverse causes of liver disease, clinical presentations, and laboratory variability amongst studies. Patients
have increased bleeding risks, such as soft tissue hematoma, intracranial bleed after trauma, esophageal variceal
bleeding, and transfusion requirements in the perioperative period for transplant patients. All of which are associated with increased morbidity and mortality. While
hyperfibrinolytic activity is present in thirty-percent of
patients with end stage liver disease, it is still clinically
under-diagnosed and usually diagnosed only after patients develop severe bleeding complications. This presentation is a review of three patients who developed the
complication and the available data for their prognosis
and treatment.
Case Series:
Case I: Fifty-year-old Mexican gentleman with alcoholic
cirrhosis (Child-Pugh-Class-C/MELD-26), current alcohol abuse, and hypertension presented with acute decompensated cirrhosis with fluid retention (due to spontane-
33
ous bacterial peritonitis). Incidentally, patient was anemic
with decreased fibrinogen and platelets and elevated DDimer and INR. Hematology was consulted and disseminated intravascular coagulopathy was ruled out due to a
normal Factor-VIII level. Laboratory evaluation was consistent with hyperfibrinolytic activity and the patient was
subsequently started on chronic epsilon-aminocaproic
acid (EACA).
Case II: Forty-nine-year-old Mexican gentleman with
alcoholic cirrhosis (Child-Pugh-Class-C/MELD-34) and
portal hypertension and history of alcohol hepatitis presented with bilateral lower extremity weakness and
numbness. Admission labs revealed hyperfibrinolytic
activity. Patient was started on EACA. Unfortunately, the
patient developed a spontaneous retroperitoneal bleed and
passed away.
Case III: Fifty-year-old African-American lady with
alcoholic cirrhosis (Child-Pugh-Class-B/MELD-21) presented with two-month history of prolonged epistaxis,
three-week history of increasing ecchymosis without
trauma of the right upper extremity, and two-week history of right wrist drop. Physical exam was consistent
with radial nerve palsy secondary to extensive ecchymosis and hematoma. All laboratory results indicated an
hyperfibrinolytic activity without evidence of acute decompensated cirrhosis.
Conclusion Patients with cirrhosis who develop hyperfibrinolytic activity have worse morbidity and mortality.
However, this conclusion is confounded by the association with higher Child-Pugh scores. More studies are
needed to assess whether patients with early stage cirrhosis, as classified by Child-Pugh and MELD scores,
should be evaluated routinely for this coagulopathy before developing a clinical complication. This would allow
physicians to intervene earlier with treatments to prevent
bleeding events known to be associated with worse outcomes. Current literature only supports the use of antifibrinolytic agents once patients fail conventional treatment
for bleeding complications from hyperfibrinolytic activity. There is no data for treating this coagulopathy prior
to clinically significant presentation or when patients are
non-transplant candidates. Given the impact on patient
survival, this disorder should be considered as an independent predictor of hepatic dysfunction and more studies are warranted to evaluate prophylaxis treatment.
Inflammatory Bowel Disease in the Setting of Ankylosing Spondylitis
Michael Keng, MD; Seint Yee, MD; Meera Malhotra,
MD
University of Southern California
Background: Ankylosing spondylitis (AS) is a spondyloarthropathy, characterized by sacroillitis and chronic
inflammatory lower back pain. The axial skeleton is
mainly affected, but it is commonly associated with peripheral, asymmetric arthritis, enthesitis, and acute ante-
rior uveitis. Inflammatory bowel disease (IBD), is a lesser
known, but significant extra-articular manifestation of
AS. While only occurring between 5 and 10% of AS
cases, subclinical inflammation of the gastrointestinal
tract is much more prevalent (25-60%).
Case Presentation: J.G. is a 36-year-old Hispanic male
who presented with an exacerbation of AS. Onset of his
symptoms started four years prior to admission after a
motor vehicle accident. Since then, the patient never had
complete resolution of his low back pain and he was
clinically diagnosed with AS. His pain is located in the
lower thoracic and lumbar regions and is accompanied by
right leg radiculopathy. His symptoms have been progressive and unresponsive to all conservative measures.
A review of systems revealed concurrent tenesmus and
weekly intermittent diarrhea, but no fevers, chills,
melena, or hematochezia.
Significant findings on physical exam included limited
cervical spine extension, tenderness along the lower thoracic and sacral spinous processes, bilateral hip tenderness with decreased range of motion, positive WrightSchober test, and positive straight leg test on the right
side. Neurologic exam was otherwise unremarkable. An
ano-in-fistula was found on rectal examination.
Significant laboratory findings included: hemoglobin
9.5g/dL, hematocrit 30.2%, platelets 729K/cumm, CRP
93.7mg/dL, ESR 100mm/hr, and RF 20IU/mL. Iron studies corresponded to anemia of chronic inflammation.
Anti-smith antibody, RNP antibody, ANA, ds-DNA,
HIV, and HLA-B27 were all negative. A MRI revealed
sacroillitis, confirming the diagnosis of AS. It further
showed bilateral hip arthritis and a moderate right hip
effusion. The aspirate was negative for infection.
High-dose indomethacin and steroids were initiated with
improvement of back pain, though with concomitant
worsening of rectal pain, requiring a seton placement. A
colonoscopy with biopsy was consistent with Crohn’s
disease.
Conclusion: IBD is an important extra-articular manifestation of AS. The internist should be aware of this significant association during the diagnosis and work-up of
spondyloarthopathies, as there are important implications
for treatment when the diagnoses occur together. There is
evidence of subclinical microscopic inflammation of the
GI tract in up to 60% of AS patients, of which a fraction
evolved into actual IBD over time. Furthermore, remission of joint inflammation is always linked to reduction
of gut inflammation. Case series indicate that there is an
additional decline in quality of life and overall worse
prognosis due to the extra-articular burden of disease.
Modification of treatment, namely avoiding non-steroidal
anti-inflammatory medications, is paramount to avoid
further injury to already inflamed bowel. Treatment
should target the underlying inflammatory processes of
both the AS and extra-articular symptoms.
34
Renal Failure Associated With Silicone-Induced
Granulomas
nificant history as an uncommon but serious cause of
non-parathyroid hormone mediated hypercalcemia.
Omer Khan, MD; John Sim, MD
Kaiser Permanente Los Angeles
Fabry Disease in a Young Active Duty Male with Asymptomatic Proteinuria
The use of liquid silicone for soft tissue augmentation has
been in practice over the last 40 years. Initially thought to
be inert, liquid silicone was extensively used for cosmetic
subcutaneous augmentation. With its rampant and unregulated use however this substance has become implicated in a variety of adverse reactions. We report a case
of a male-to-female transsexual patient who had received
liquid silicone injections into both hips for cosmetic
augmentation. Years after receiving these injections she
developed silicone-induced granulomas causing hypercalcemia and subsequent renal failure. We discuss the
controversy over the use of liquid silicone for soft tissue
augmentation. In addition, the pathophysiology of granulomatous disease causing hypercalcemia, and treatment
options for silicone-induced granulomas is discussed.
Christine Kim, MD; Shagun Chopra-Sonthalia, MD
Naval Medical Center San Diego
Liquid injectable silicone is commonly used to correct or
augment facial and body contours. The proponents for its
use argue that when used appropriately liquid silicone can
provide predictable beneficial long-term results. Conversely, opponents condemn its use altogether because of
its numerous complications including chronic cellulitis,
nodule formation, foreign body reactions, and migration.
The controversy over the use of liquid silicone is yet to
be resolved.
The mechanism for hypercalcemia of granulomatous disease involves activated mononuclear cells, which are
responsible for excessive conversion of calcidiol to calcitriol. Calcitriol in turn promotes hypercalcemia through
various metabolic pathways. One would then expect that
a patient with hypercalcemia due to granulomatous disease would have decreased serum levels of calcidiol and
increased levels of calcitriol. This may not always be the
case as illustrated in our patient who had normal levels of
calcitriol (37 pg/mL; normal: 15-55 pg/mL). However,
even though the calcitriol level was within the normal
range, it was inappropriately elevated in the setting of
hypercalcemia (12.7 mg/dL in our patient; normal: 8.410.2 mg/dL). Thus the notion of having a “normal” level
of calcitriol alone should not rule out hypercalcemia due
to granulomatous disease.
Although the mainstay of treatment for silicone-induced
granulomas is glucocorticoids, there are many other
treatment options currently being utilized. A key point is
that the removal of liquid silicone, which is not encapsulated, is difficult to perform. Surgical excision may result
in incomplete removal, deformities, fistulas, and abscesses. This underscores the potential permanence of
liquid silicone injections with adverse reactions requiring
chronic long-term treatment.
There are many causes of hypercalcemia. We should consider silicone induced granulomas in patients with a sig-
Introduction: Fabry disease is a rare lysosomal storage
disorder caused by a deficiency of alpha-galactosidase A.
Its clinical manifestations result from the accumulation of
glubotriaosylceramide in cells of the brain, heart, and
kidneys. Symtoms are nonspecific which may delay diagnosis and worsen prognosis. We present a case of a
young active duty male who presented with unexplained
proteinuria.
Case: A 33-year-old active duty male with a history of
proteinuria as a reservist five years ago presented to the
ophthalmology clinic with a six month history of binocular diplopia and was found to have a left 4th nerve palsy
and corneal ventricullata suggestive of Fabry disease.
Upon initial evaluation, he was noted to have 3 grams of
proteinuria, low levels of alpha galactosidase, and normal
kidney function. Nephrology evaluation revealed mildly
elevated blood pressure and multiple angiokeratoma on
scrotum. Serologic workup for other etiologies of proteinuria and renal ultrasound were unremarkable. A renal
biopsy was performed with glomeruli demonstrating
large podocytes with clear foamy cytoplasm. Fabry disease was confirmed. The pt was started on therapy with
Fabrazyme every two weeks with reduction of proteinuria, and has maintained preserved kidney function for
past three years.
Discussion: Our case demonstrates the importance of
early diagnosis of Fabry’s disease in the young patient
who presents with proteinuria. Early recognition of this
disease is essential as devastating long-term complications can be reduced with early treatment.
Life without Fibrinogen: An Uncommon Hemophilia
Treated with a Common Blood Product
Jerome Kim, MD; Deepthi Karunasiri, MD; Leland
Powell, MD, PhD
Olive View-UCLA Medical Center
While relatively uncommon, patients with hemophilia A
or B are easy to identify but require specific factor products for treatment. We present a case of a much less
common congenital bleeding disorder whose treatment
requires a readily available blood product.
Case: A 21 yo woman with a history of easy bruising and
bleeding presented with a three day history of increasing
right hip pain. She denied any history of trauma and
stated that the pain increased with hip flexion. There were
no other associated symptoms including menorrhagia or
hematochezia. She was a poor historian, but her past
35
medical history was significant for prior hematomas and
an intra-cranial hemorrhage 5 years ago with no additional details. She had no siblings and stated that her parents were healthy. Her physical exam demonstrated decreased range of motion in the right hip and increased
pain with right hip flexion and rotation. There was no
obvious swelling, purpura, or petechia. Plain films of the
hip were negative for fracture or dislocation. A CT scan
of the pelvis indicated an asymmetric enlargement of the
right obturator internus muscle consistent with a hematoma.
Laboratory studies included platelets = 366,000, PT >
100 seconds, PTT > 201 seconds, fibrinogen level < 20
mg/dL, and d-dimer = 128 ng/mL. A PT mixing study
(1:1 mix) corrected to 14.8 seconds. She was treated with
20 units of cryoprecipitate, and follow up laboratory studies included PT = 14.7 seconds, PTT = 28.6 seconds, and
fibrinogen = 187 mg/dL. Subsequent studies from an
outside reference laboratory indicated normal levels of
Factors II, V, VIII, and IX, but with thrombin and reptilase times both > 150 seconds.
Discussion: The patient was diagnosed with a congenital
hypofibrinogenemia. Given the severity of her disorder
and the lack of symptoms in either of her parents, she
most likely had a mixed heterozygous state. The presence
of a measurable d-dimer on initial presentation, along
with the relatively mild bleeding history, suggested that
she had a level of fibrinogen below the level of detection
of standard laboratory assays. The blood product cryoprecipitate is known to be a rich source of fibrinogen
(250 mg per unit). With correction of her fibrinogen level
with cryoprecipitate, her fibrinogen was measured to decay at a half-life of approximately 2 ½ days, consistent
with published reports and indicating the lack of a circulating inhibitor. She was prescribed to receive 20 units of
cryoprecipitate every two weeks, and remained without
clinically significant bleeding episodes when compliant
with therapy.
Preemptive Prevention of Immune Reconstitution
Inflammatory Syndrome (IRIS) in Cryptococcal Meningitis
Jerome Kim, MD; Catherine Yim, MD; Suzanne
Donovan, MD, MPH
Olive View-UCLA Medical Center
A patient with AIDS and severe cryptococcal meningitis
was initiated on corticosteroids, in conjunction with
highly active antiretroviral therapy (HAART), to prevent
immune reconstitution inflammatory syndrome (IRIS).
IRIS is a frequent and troublesome complication of
HAART in patients with cryptococcal and other opportunistic infections.
Case: A 49-year-old male presents with cryptococcal
meningitis complicated by increased intracranial pressures. He was found to be HIV-infected with a viral load
of >100,000 copies/mL and CD4 of 10/mm3. He was ini-
tially treated with Amphotericin and 5-FC, followed by
high dose fluconazole. MRI demonstrated confluent gelatinous pseudocysts secondary to cryptococcal infection
(Figure 1). Follow-up exams, lumbar punctures and fundoscopic exams demonstrated persistent headache, elevated opening pressures and papilledema despite aggressive antifungal treatment, acetazolamide and serial lumbar punctures to relieve elevated pressures. Results of
serial lumbar punctures are shown in Table 1. Follow-up
CSF fungal cultures were negative and cryptococcal antigen (CRAG) titers on subsequent lumbar punctures demonstrated a significant decrease. Patient refused intraventricular shunt placement. HAART was initiated with high
dose prednisone (60 mg/day), because of concern for
IRIS with potential worsening of his intracranial pressures, with a prednisone taper over 6 weeks. Symptoms
of IRIS (headaches, vomiting and increased ICP) occurred 4 months later after prednisone taper during a time
of immune reconstitution (HIV viral load 422 copies/mL
, CD4 30/mm3). CSF parameters are shown in Table 1.
Patient responded to reinstitution of a longer taper of corticosteroids and short course of acetazolamide. Follow-up
MRI demonstrated resolution of gelatinous pseudocysts.
Results of follow-up lumbar punctures normalized.
Discussion: Immune Reconstitution Inflammatory Syndrome (IRIS) is an inflammatory response to a preexisting infection after initiation of highly active antiretroviral therapy (HAART) in patients with AIDS. It
occurs in up to one third of AIDS patients starting
HAART, and has a tendency to affect patients who have
profound immune deficiency at baseline, and rapid viral
suppression and immune recovery with HAART. The
treatment for cryptococcal IRIS include serial lumbar
punctures to relieve elevated intracranial pressure, decreasing fungal load with antimicrobial therapy (Amphotericin B and flucytosine) and the use of corticosteroids
for anti-inflammatory effects. We present a case of a patient at high risk for IRIS, and at significant risk for neurologic complications, successfully treated with steroids
at HAART initiation.
A Case of Coloboma and Horseshoe Kidney
Paul Korc, MD; Eunice Rios, MD
University of Southern California
Case: A 27 year old woman with no past medical history
presented to our hospital with 4 days of cough, shortness
of breath, and wheezing. In the emergency room she was
hypoxic with an oxygen saturation of 82% on room air.
She was treated for new onset asthma with bronchodilators and systemic steroids. On exam she was noted to
have abnormally shaped and downwardly displaced pupils consistent with coloboma of the iris. She also had a
secondary complaint of lower abdominal pain and underwent a CT abdomen/pelvis, which demonstrated an
ovarian teratoma and, incidentally, horseshoe kidney. She
denied a history of any visual complaints, and had normal
visual acuity. Her renal function is normal as well.
36
Discussion: Coloboma results from a failure of the fetal
or choroidal fissure to close during the 5th to 7th weeks of
fetal life. It can involve almost any ocular structure. The
Renal Coloboma Syndrome (or Papillorenal Syndrome) is
an autosomal dominant disorder characterized by renal
abnormalities and optic nerve defects. The gene that has
been implicated is PAX2, which encodes the PAX2 developmental nuclear transcription factor. Renal abnormalities can include hypoplasia, early renal failure, and
vesico-ureteral reflux. The degree of renal abnormality
lies along a spectrum, and variable phenotypes have been
reported, including rare cases of horseshoe kidney. Given
the constellation of bilateral coloboma and horseshoe
kidney in our patient, she may harbor the PAX2 mutation
and a variant of the Renal Coloboma Syndrome. We are
currently considering genetic testing.
candidate for percutaneous closure of the ASD given his
symptoms. Cardiac catheterization was performed. There
was no evidence of coronary artery disease. Pulmonary
artery pressure was 40/12. QP to QS ratio was 1.26. MRI
of the lumbar spine revealed L4-L5 disk prolapse with
impingement of the L5 nerve root. The patient underwent
neurosurgery for the disc herniation. After recovery, he
had percutaneous closure of the ASD, which improved
his symptoms and oxygenation.
This case illustrates the importance of detecting abnormalities on physical exam as well as clinical studies,
which can clue the internist into completing a further
workup. Congenital heart diseases vary in their age of
presentation. It is important, not only for pediatricians,
but also for internists to be able to diagnose these conditions.
Ebstein's Anomaly Diagnosed in a 33 Year-Old
Roshan Kotha, MD
Scripps Mercy Hospital
Congenital heart diseases vary widely in their severity
and clinical presentations. Certain pathologic findings on
physical exam and clinical studies can provide clues to
the diagnosis.
A 33-year-old Vietnamese male presented to the ED
complaining of shortness of breath and right lower extremity pain. The patient endorsed limitations in physical
activity due to dyspnea since childhood. He had been told
he had a “leaky valve” in childhood, however did not
have further follow up for this. Over a few days prior to
admission, he developed worsening dyspnea with minimal exertion. He also reported intermittent right lower
extremity pain radiating from the gluteal area, to the ankle over the past 3 years. 2-3 days prior to admission, he
developed weakness as well.
On exam, he was a well appearing, well developed, male
in no distress. His vitals were within normal limits with
an oxygen saturation of 96% on room air. There was no
evidence of cyanosis. Lungs were clear to auscultation.
Cardiovascular exam revealed a regular rate and rhythm,
with a fixed and split S1 and S2, and a 3/6 systolic murmur at the left lower sternal border. JVP revealed prominent a and v waves. Strength was 5/5 throughout, except
for the right leg, which had 2-5/5 strength in various
muscles. Sensation was decreased to light touch and pinprick along the L5 dermatome.
Laboratory studies were negative for evidence of myocardial ischemia. EKG revealed sinus rhythm, right atrial
enlargement, and a non-specific intraventricular conduction delay. Chest X ray revealed cardiomegaly, without
pulmonary vascular prominence. A transthoracic echo
revealed Ebstein’s anomaly, with atrialization of the right
ventricle and apical displacement of the tricuspid valve,
along with an atrial septal defect. A transesophageal echo
revealed a bidirectional shunt, which was predominantly
left to right along the ASD. The patient was felt to be a
New Onset Reflux After Acute Gastroenteritis: A
Case of Post-Infectious GERD
David Kunkel, MD; Mark Pimentel, MD
Cedars-Sinai Medical Center
Exposure of the gut to infectious pathogens can alter native bowel flora and motility, triggering disorders such as
Irritable Bowel Syndrome. Here we report the first case
of GERD developing in an adult after an episode of acute
gastroenteritis. A previously healthy 48 year old female
presented to GI clinic for evaluation and treatment of
belching and epigastric bloating. One year prior to presentation, the patient had traveled to a remote area of
China and eaten at a local restaurant of questionable repute, subsequently developing nausea, vomiting, and diarrhea the following morning. While her acute symptoms
resolved, the patient started to experience frequent belching associated with bloating and anorexia. She had resorted to eating small meals because large portions resulted in food regurgitation. She was not having any fevers, sweats, cough, blood per rectum, abdominal pain, or
change in bowel habits. Short courses of probiotics, digestive enzymes, peppermint oil, and herbal medications
had not relieved her symptoms. On physical exam, the
patient had a temperature of 98 degrees. Auscultation of
the abdomen revealed intermittent bursts of hyperactive
bowel sounds over the epigastrium occurring once every
20 seconds. Her abdomen was otherwise soft, non-tender,
with no masses or hepatosplenomegaly. Laboratory studies revealed a normal complete blood count. Her liver
function tests, amylase and lipase were normal. Stool
studies including fecal ova and parasite were negative for
evidence of active infection. Upper endoscopy was performed which revealed normal gastric and duodenal mucosa. Biopsies were negative for H. pylori and celiac disease. Antroduodenal manometry disclosed high amplitude contractions of the pylorus up to 466 mm Hg.
Twenty-four hour pH monitoring revealed markedly elevated acid exposure times in both the upright and recumbent positions of 20% and 14% respectively, for an acid
reflux composite score of 79, consistent with a diagnosis
of GERD. The patient was placed on a PPI and experi-
37
enced complete resolution of her symptoms. Exposure to
infectious pathogens can lead to several complications
ranging in severity from acute gastroenteritis to postinfectious IBS. This case illustrates the first report of
reflux developing after an epsidoe of acute gastroenteritis.
Ruptured Intracranial Mycotic Anuerysm in Infective
Endocarditis
Isabel Kuo; Theodore Long; Bharat Chaudry, MD;
Michael Karp, MD; Nathan Nguygen, MD
University of Southern California
Mycotic aneurysms (MA) are intracranial aneurysms that
develop in the presence of infections such as infective
endocarditis (IE). They account for a small percentage of
all intracranial aneurysms but carry a high mortality rate
when they rupture. A high index of suspicion and early
treatment can reduce the risk of rupture and improve outcome.
Our case is that of a 54-year-old male with history of IE
complicated by cerebrovascular accident 15 years ago
without residual deficit who was in his usual state of
health until 2 days prior to admission when presented to
the emergency department complaining of slurred speech.
His physical exam was significant for a temperature of
102.3 degrees Fahrenheit and a 3/6 holosystolic murmur
most prominent at the left lower sternal border with radiation to the axilla. He also had poor dentition, marked
clubbing of his fingers, left facial weakness, subtle slurring of speech, and diffuse hyperreflexia. The echocardiogram showed a large mobile mass on the mitral valve
leaflet, and multiple sets of blood cultures grew Streptococcus viridans. An MRI of the brain showed an acute
right frontal lobe infarct and old blood products. Intravenous Penicillin G and Gentamicin were started immediately; however, six days into his antibiotic course, he
became obtunded. An emergent CT cerebral angiogram
showed a large ruptured aneurysm at the distal segment
of the anterior cerebral artery with adjacent intraparenchymal and intraventricular hemorrhage. He underwent
emergent craniotomy and aneurysm clipping. His postoperative course was complicated by multiple arterial
vasospasms treated with triple-H therapy.
The patient achieved partial recovery and was able to
follow basic commands at the time of discharge. Valve
replacement surgery was deferred until resolution of his
acute stroke and hemorrhage. After tracheostomy and
gastric tube placement, he was transferred to a skilled
nursing facility for a planned six-week course of IV antibiotics.
This case emphasizes the importance of considering MA
in the differential diagnosis when IE and intracranial septic embolization are present. MA should be investigated
early with vascular angiography, and a low threshold of
suspicion for subarachnoid hemorrhage should be maintained. Once the diagnosis has been made, a neurosurgi-
cal consult should be obtained early to determine the risk
of rupture and the appropriate therapeutic intervention.
Crystal Clear: A “Shock”-ing Case of Gout
Brent W. Lacey, MD; James Prahl, MD; Scott Parrish, MD
Naval Medical Center San Diego, CA
Case: An 80 year-old male with chronic gout presented
with severe left knee and lower back pain. A lumbar MRI
showed findings consistent with L4/5 pyogenic discitis,
and an intervertebral disc biopsy showed minimal granulomatous changes. All cultures were negative. The patient’s condition deteriorated despite broad spectrum antibiotics, and he developed polyarticular inflammation
and delirium. He subsequently required intubation and
vasopressor support. A left knee arthrocentesis revealed
monosodium urate crystals, 32,000 neutrophils, and a
negative gram stain. Repeat lumbar MRI showed changes
consistent with gouty arthritis. After all cultures were
confirmed to be negative, solu-medrol was started to treat
systemic distributive shock presumed to be secondary to
an acute gout flare. The patient’s fever defervesced, and
vasopressor and ventilator support were discontinued the
next day. The patient made a full recovery on a 3-week
steroid taper and allopurinol.
Discussion: 81 cases have been reported where gout was
seen to mimic pyogenic discitis. Only one case has been
associated with distributive shock. This case represents
the first patient reported to have both conditions simultaneously. Gout is a monoarticular inflammatory condition
most commonly affecting the knee or first metatarsophalangeal joints. The definitive diagnosis is incumbent on
identification of either tophi or monosodium urate crystals. Gout affecting the spine, though rare, can pose a
significant health risk, including spinal cord compression
necessitating urgent surgical intervention. Spinal gout
appears to respond to standard acute gout management,
despite the unusual location. The development of distributive shock is the most significant feature of the case.
Distributive shock results from an imbalance between the
pro- and anti-inflammatory components of the host’s
immune system. This patient did not have an obvious
predisposing feature for progression to shock. The pathogenesis of his condition should therefore prompt exploration into genetic or other pathophysiologic factors that
would incline this patient to develop shock.
Profound Lactic Acidosis Due to Hepatic Steatosis
Mark Lee, MD; Carrie Chun, MD
Scripps Green Hospital
Lactic acid is a derivative of anaerobic metabolism and is
the primary cause of metabolic acidosis. Early clearance
of plasma lactate levels has been associated with improved survival in hospitalized patients. Identifying the
source of lactic acidosis is critical so that appropriate
treatment can be tailored to the underlying cause.
38
University of Southern California
A 57 year-old morbidly obese woman with a history of
gastric-banding surgery, right hemicolectomy secondary
to colon cancer, and nephrolithiasis presented with a two
day history of clear emesis and colicky, abdominal pain.
Her husband noted a two month period of depression and
poor oral intake with an associated 50-pound weight loss.
On exam, the patient was normotensive and tachycardic,
with exophthalmos, dry mucous membranes, decreased
skin turgor and an obese but benign abdomen. Labs demonstrated: WBC 15.2 cells/μL, potassium 2.6 mmol/L,
serum creatinine 0.8 mg/dL, serum bicarbonate 9 mEq/L,
anion-gap 28, lactic acid 2.0 mmol/L , VBG pH 7.12,
normal hepatic function and urine ketones. AbdomenPelvis CT revealed small renal stones with mild hydronephrosis. The patient was admitted to the ICU with
starvation ketoacidosis. She received IV-fluid resuscitation and TPN with resolution of the acidosis.
After early improvement, the patient developed hypotension initially responsive to fluid resuscitation, but eventually required vasopressor support. She became febrile
with persistent leukocytosis, and broad-spectrum antibiotics were begun. Given her hemodynamic instability, a
lactic acid was repeated and elevated at 7.8 mmol/L.
Blood and urine cultures were negative. Repeat abdomenpelvis CT showed decreased hydronephrosis with no evidence of mesenteric ischemia or masses. Upper endoscopy revealed only mild gastritis.
On hospital day 5, the patient was intubated for impending respiratory failure. Lactic acid was 14.7 mmol/L.
Multiple blood, urine, and sputum cultures remained
negative. Her medication profile was reconciled with her
husband, and no lactic acid inducing medication was
identified. Despite continuous bicarbonate infusion and
initiation of CRRT, shock and lactic acidosis persisted.
After several days of maximal aggressive care, the family
requested transition to comfort care. The patient expired
on hospital day 7. Autopsy toxicology results were unrevealing, but gross pathology revealed marked macrovesicular hepatic steatosis with near complete fatty replacement of the liver.
This case demonstrates a rare case of hepatic steatosis
induced lactic acidosis. All other workup for hyperlactatemia was negative. Lactic acidosis and hepatic steatosis may result from medications, but there are few reports
of hyperlactatemia from hepatic steatosis alone. In advanced liver disease, lactic acidosis is thought to arise
from tissue hypoxia that results from arteriovenous shunting. This finding is associated with high mortality, and
care is supportive only.
Pulmonary Mucormycosis in a Non-DKA Diabetic
Patient
Than Lin, DO; Kenneth Lue, DO; Sid Anand, MS IV,
MBA; Christine Kim, MD; Shahrod Mokhtari, MD;
Vonny Tunru-Dinh, MD; Michael Karp MD
Introduction: Pulmonary mucormycosis, a subset
of zygomycosis, occurs when spores are inhaled through
the respiratory tract. Though uncommon in healthy individuals, patients with poorly controlled diabetes are at
increased risk for the infection. Diabetic ketoacidosis (DKA) is a common underlying medical condition that is present when mucormycosis is detected. In
those with DKA, the acidic environment of the serum
causes dissociation of free iron from sequestering proteins, stimulating rapid fungal growth.
Case: A 26 year old Hispanic female with newly diagnosed diabetes (HbA1c 13.2) presented with hemoptysis,
night sweat, fever and unintentional weight loss. Chest
radiograph showed right lower lobe consolidation suspicious for abscess formation. Sputum cultures were negative for tuberculosis. CT chest showed a necrotizing
process in the right lower lobe and a second cavitary lesion in the right upper lobe. Bronchoscopy was performed with bronchoalveolar lavage resulting positive for
Rhizopus species and biopsy revealed nonseptate hyphae
consistent with invasive mucormycosis. The patient was
immediately started on Amphotericin B, Deferasirox,
tight glycemic control, and surgical consultation was
sought for lobectomy. After several weeks of antimicrobial therapy, the patient underwent single lobectomy and
was recovering from that procedure as of the writing of
this abstract.
Discussion: Pulmonary mucormycosis is a rapidly invasive pulmonary infection that usually affects immunocompromised patients with hematologic malignancies receiving chemotherapy or HIV. Diabetic patients
have a distinct clinical syndrome as evidenced by the
organism invading the airways and hilar vessels, resulting
in atelectasis, abscess formation, or severe hemorrhage
presenting as massive hemoptysis, usually with a predilection for the right lung. Radiographic features include
nodular infiltrates, mediastinal widening, solitary nodules, cavitation or pleural effusion. Histologic characteristics will show nonseptate hyphae with right angle
branching invading pulmonary tissue. Despite early surgical and medical therapy, literature suggests a poor
prognosis for pulmonary mucormycosis. The mortality
rate of patients solely treated with antifungal medication
is 68% compared to 11% for patients treated with antifungal medication and surgical correction.
Conclusion: Diabetic patients have been linked to Mucormycosis, but most cases presented with rhino-cerebral
infection, and most have DKA. This case is unique in that
our patient did not present with DKA, had pulmonary,
not rhino-cerebral infection, and that she was not immunocompromised with malignancy or HIV. While literature suggests a mechanism for patients with DKA,
there is limited information regarding why non-DKA
diabetes are predisposed to infection. This case suggests
the need for a proposed mechanism of action for nonDKA diabetic patients infected with Mucor. If the
39
mechanism of infection in non-DKA diabetics is not the
creation of a high-iron environment through acidosis,
perhaps there is an additional component of the pathophysiology of the disease that should be explored for optimal management.
28 Year-Old Female Requiring Frequent Therapeutic
Whole-Lung Lavages
Eduardo A. Lopez, MD; Nikhil Barot, MD; Dennis
Yick, MD, FCCP; Nader Kamangar, MD, FACP,
FCCP
Olive View-UCLA Medical Center
Case: 28-year-old African American woman consulted
her doctor because of
progressive dyspnea on exertion, non-exertional chest
pain, and occasional hemoptysis. Her lung examination
was normal despite grossly abnormal chest radiograph.
She did not exhibit any evidence of heart failure, cyanosis, or clubbing. Her pulmonary function tests
revealed a restrictive ventilatory defect and a moderate to
severe reduction of her DLCO. Her arterial oxygen saturation was 94% while she was breathing ambient air and
quickly desaturated after minimal exertion, and her chest
radiograph showed bilateral alveolar and interstitial
opacities. High-resolution computed tomography (CT) of
the chest showed a bilateral ground-glass opacity with
interlobular septal thickening, creating a mosaic, or
"crazy paving," pattern in both lung fields. A very extensive work up failed to demonstrate an infectious origin of
her symptoms. Treatment was initiated with
high-dose corticosteroids and broad spectrum antibiotics.
Despite this, her condition worsened. Bilateral WLL was
performed under general anesthesia and single-lung ventilation. . We used 2 liters of Normal Saline in the left
lung and 8 liters on the right side,1 week apart. The lavage fluid was initially turbid with sediment but gradually
cleared. After the procedure, the symptoms diminished,
with O2 saturation improving to 97% while the patient
was breathing ambient air and 96% with exertion. The
patient was asymptomatic at discharge.
Discussion: Pulmonary alveolar proteinosis (PAP) is a
rare disorder caused by abundant accumulation of surfactant-derived components in the lungs. Historically, there
has been no effective pharmacotherapy for PAP, and sequential whole-lung lavage (WLL) under general anesthesia has become the mainstay of treatment. PAP should
be considered an "orphan" lung disease. There are only
500 reported cases in the literature to date. Secondary
PAP can be seen in hematologic cancers, pharmacologic
immunosuppression, inhalation of toxins, and certain
infections. Crazy-Paving is characteristic for PAP, yet
many other diseases can show this appearance on CT;
Crazy-paving is a nonspecific finding. This case illustrates that many issues remain unclear regarding the optimal therapy of patients with PAP.
The Least Compatible Patient
Dustin Lorentz, MD; Mark Tschanz, DO
Cedars-Sinai Medical Center
Autoimmune hemolytic anemia (AIHA) is caused by the
presence of autoantibodies directed against antigens on
the surface of red blood cells. The incidence is reported at
one per 80,000 people, peaking in the first four years of
life, with males and females affected equally. Most commonly idiopathic, predisposing conditions include infections, drugs, hematologic conditions, and/or immune disorders. Treatment is aimed at reducing the amount of
antibody produced or decreasing the efficiency of the
antibody in lysing cells, most commonly with corticosteroids or immunoglobulin. AIHA can be an emergency
when patients become severely anemic, as they can lose
the ability to sustain sufficient oxygenation to vital organs. In these patients, immediate transfusion of blood
can be life-saving. Our patient is a 62 year-old female
with a history of idiopathic thrombocytopenic purpura
(ITP) who was admitted with generalized weakness and
jaundice. Her baseline hemoglobin was 14.4 g/dl, and
upon admission the hemoglobin of 7.4 g/dl dropped precipitously to 4.8 g/dl within 10 hours of presentation. All
lab values including direct Coomb’s test, haptoglobin,
lactate dehydrogenase, and reticulocyte count were consistent with a diagnosis of AIHA. Orders were made to
crossmatch blood, but all available units were deemed
incompatible. The blood bank was contacted for stat retrieval of “least incompatible” blood, and the patient was
placed on high-dose corticosteroids prior to transfusion of
blood in the intensive care unit. Her hemoglobin eventually stabilized, and she was discharged from the hospital
on prednisone. Given her associated history of ITP, the
patient was diagnosed with Evans Syndrome, a combination of Coombs-positive warm AIHA and ITP. Though
people with this syndrome are historically more resistant
to standard treatment than those with AIHA or ITP alone,
the patient continues to have stable blood counts on follow-up. In AIHA, it is unlikely that fully compatible
blood can be matched, since the autoantibody produced
by the patient reacts with antigens present on cells of
almost all individuals in the donor population. Thus, for
patients in need of emergent transfusion, compatibility
testing should not delay their possibly life-saving treatment. This often requires contacting the blood bank to
minimize delays and avoid misunderstandings concerning
the degree of compatibility of available blood. With appropriate and timely therapy, these incompatible patients
can be successfully treated 95% of the time.
An Incidental Finding of Coccidioidomycosis Osteomyelitis
Monique Mahlum, MD
University of California, San Diego
Case: Mr. S is an 82-year-old African-American man
originally from Arizona with history of diabetes mellitus
who presented with microhematuria in routine screening
labs from clinic. Ureterolithiasis was suspected as the
source of microhematuria. An abdominal x-ray was nega-
40
tive for calcifications. A CT urogram was done and
showed an incidental finding of inflammatory changes in
the L1-2 region. After this finding, a focused history revealed 3 months of consistent low back pain. He denied
fevers, weight loss, night sweats, cough, and shortness of
breath. He had no history of IV drug use, previous back
surgery, or trauma to the low back. On exam, he had no
spinal tenderness or neurological signs. Chest x-ray was
clear. ESR was elevated at 79. Blood cultures were negative. Bacterial, AFB, and mycology bone biopsy cultures
were negative. Pathology showed non-necrotizing granulomas with foreign body type giant cells. GMS and PAS
stains highlighted rare round, encapsulated organisms. A
diagnosis of vertebral coccidioidomycosis was made.
Serology showed cocci IgG positive, IgM negative. The
patient was started on fluconazole 400 mg PO q12 hours
with infectious disease follow up.
Discussion: Coccidioidomycosis, also known as Valley
Fever, is endemic in the southwestern United States, parts
of Mexico and South America. Spores live in the soil and
are spread airborne in dust. Of people who live in an endemic region, about 10-50% will have evidence of exposure to Coccidioides.
The annual incidence of Coccidioidomycosis tripled in
California between 2000 and 2006. Incidence rates in
Arizona are similar to those in California. Arizona annually reports about 60% of all cases in the US. Less than
1% of patients infected with C. immitis progress to extrapulmonary disease, most commonly to the skin, bones,
joints and CNS. Bone infections, specifically the vertebral column, account for about 20-50% of extrapulmonary cocci. An increased risk of disseminated disease is
seen in men, African-Americans and Filipinos. Mechanism of spread is hematogenous after a primary pulmonary infection. By the time patients experience skeletal
involvement, the pulmonary infection has resolved and
findings on CXR are normal. This is the case with Mr. S
as the IgG was positive with a clear CXR and no pulmonary symptoms. In an endemic Cocci region, clinicians
must always have a high level of suspicion for this fungus, even with no overt pulmonary symptoms.
Left Bundle Branch Block Induced by Near Tachycardia in a Patient without Ischemic Disease
Nalin Mallik, MD; Reza Rafie, MD; Meera Malhotra,
MD; Sharmineh Shams, MD
University of Southern California
Rate-related bundle branch block is a well established
phenomenon. These patients have an increased refractoriness of the bundle and the block appears at tachycardic
rates. They usually have underlying coronary disease. In
this case report we present a patient with serial ECGs that
demonstrate the induction of LBBB at near-tachycardia,
with a return to a narrow QRS at slower rates. She has no
underlying CAD.
Case Description: A 65 year-old woman with a history
of hypertension was admitted for chest pain. Her chest
pain started three days prior to admission. She described
the pain as pressure-like with radiation to the back and
neck. The pain lasted for a few minutes, and occurred at
both rest and during exertion. The patient also experienced associated shortness of breath with her chest pain.
She had good exercise capacity and denied orthopnea,
peripheral edema or paroxysmal nocturnal dyspnea. She
was taking losartan for her hypertension. Physical exam
was significant for a BP of 148/96 mmHg and heart rate
of 93 bpm. Heart exam showed regular rhythm without
murmur, rub or gallop. Her troponin levels were checked
three times, all of which were in the normal range. The
first ECG showed normal sinus rhythm with a left bundle
branch block pattern. The QRS duration was 124 msec,
and heart rate was 95 beats/minutes. Subsequent ECGs in
the next few hours showed sinus rhythm with heart rates
of 68 and 76 beats/minute. The QRS durations on these
repeat ECGs were 84 msec and 82 msec, respectively,
with no evidence of conduction abnormality. The patient
was recognized as near tachycardia-induced LBBB. She
subsequently underwent exercise cardiac thallium scan
that showed a normal ejection fraction with normal cardiac perfusion results. She had no further chest pain and
was discharged on losartan and amlodipine.
Discussion: Rate-dependent LBBB uncommonly occurs
in healthy adult patients with normal cardiac perfusion
radionuclide scans, and heart rates exceeding 125
beats/minute. The left bundle branch has dual blood supply involving left anterior descending and posterior descending artery and the LBBB can be due to either coronary vasospasm versus a perfusion defect in the microcirculation. Rarely, exercise-induced LBBB has been associated with dilated cardiomyopathy. However, for the
first time we present a case of near tachycardia-induced
LBBB occurring in a patient with a normal cardiac perfusion radionuclide scan at a heart rate of 95 beats/minutes.
This case report depicts that upper-limits of normal heart
rate can induce LBBB in patients with normal cardiac
perfusion. Although previous studies have shown exercise induced LBBB predicts a higher risk of cardiovascular events, more studies are needed to figure out the outcome in patients with LBBB induced by trivial elevation
in heart rate and normal cardiac thallium scan.
Acromegaly Uncovered by West Nile Meningoencephalitis
Ryan Martin, BS; Nasser Mikhail, MD; Mark
Richman, MD, MPH
Olive View-UCLA Medical Center
Case: A 63 year-old Hispanic male with a past medical
history of hypertension, type II diabetes mellitus, and
bilateral knee osteoarthritis presented with acute onset of
severe headache, fatigue, fever, and light-headedness
with a questionable episode of loss of consciousness one
night prior to admission. Upon presentation, his blood
pressure was 119/84, pulse 140, respiratory rate 23 with
41
normal oxygen saturation, and a temperature of 40o C. He
was obese with oily, diaphoretic skin and coarse facial
features, including frontal bossing, prognathism, and
large, thick hands. He spoke with a deep, resonant voice.
His mental status was intact and there were no focal neurological deficits. Kernig and Brudzinski signs were
negative.
Initial laboratory studies were normal except for an elevated lactic acid level of 2.5 mmol/L. Brain CT showed
an enlarged 12 mm pituitary gland. Pituitary function
tests were normal except for an elevated insulin-like
growth factor-1 (IGF-1) of 650 ng/mL and low total testosterone of 103 ng/dl. A lumbar puncture elicited an
opening pressure of 28 cm H20 with apparently normal
CSF analysis; CSF analysis subsequently proved to be
positive for the West Nile Virus. The patient responded
well to conservative management and was discharged
home. An outpatient MRI of the brain showed a large T1
hypointense, hypo-enhancing intra-sellar mass measuring
12.5 x 16 x 12.2 mm, consistent with a pituitary
macroadenoma. Formal visual field evaluation elicited
bilateral superior hemianopia. A diagnosis of acromegaly
and hypogonadotropic hypogonadism was established
and the patient was referred to neurosurgery for resection
of the pituitary macroadenoma.
Discussion: Acromegaly is a rare endocrine disorder,
with over 90% of cases secondary to a benign monoclonal GH–secreting pituitary adenoma, leading to excessive production of IGF-1 by the liver. This causes excessive cell growth, multiplication, and inhibition of apoptosis in a variety of organ tissues. More than half the
cases are diagnosed by subspecialists after evaluation for
a variety of cardiovascular, endocrine, musculo-skeletal,
neurologic, respiratory, and other manifestations resulting
from either local pituitary mass or systemic growth hormone effects. The diagnosis has historically been made
by combining a high index of suspicion with abnormal
oral glucose tolerance testing. However, the latter has
largely been replaced by direct assays of elevated serum
IGF-1 levels, which correlates better with the signs and
symptoms of acromegaly. Early diagnosis is important, as
the morbidity and 3-fold increased mortality associated
with acromegaly is largely related to the duration of exposure to elevated serum IGF-1 and GH levels; early detection and treatment can limit many of these deficits.
Treatment goals of acromegaly include normalization of
IGF-1 levels which can be obtained through surgical or
external beam radiotherapy debulking of the pituitary
adenoma. The somatostatin analogs, ocreotide and lancreotide, are options for conservative management.
Ornithine Transcarbamylase Deficiency in Patients
with Neuroendocrine Tumors
Aashini Master, MD; Michael Keng, MD; Helen Gu,
MD
University of Southern California
Background Urea cycle deficiencies are extremely rare
disorders of nitrogen metabolism occurring in one out of
eight thousand births. Most affected patients present as
newborns, but those with partial enzyme deficiency may
become symptomatic later in childhood or adulthood. The
following cases presented are those with previously undiagnosed ornithine transcarbamylase deficiency (OTCD)
unmasked by increasing hepatic tumor burden from neuroendocrine cancer.
Case DESCRIPTION The following cases were patients
with newly diagnosed neuroendocrine tumors presenting
with altered mental status.
Case I: A 28-year-old Chinese woman with a history of
chronic Hepatitis B and newly diagnosed neuroendocrine
tumor with liver metastasis presented with six months of
abdominal pain. After consuming a colonoscopy preparation for a scheduled endoscopy to look for the primary
tumor site, the patient presented to the ED with severe
abdominal pain and nausea. In the ED, she became increasingly confused and progressed to becoming unresponsive to verbal and painful stimuli. Upon admission,
she was found to have normal laboratory values other
than a mild transaminitis and elevated ammonia level,
which prompted an amino acid analysis panel since there
was no evidence of portal hypertension or hepatic synthetic dysfunction.
Case II: A 51-year-old Korean woman with a history of
metatastic carcinoid tumor of the pancreatic tail presented
to the ED for altered mentation. The patient had an elevated ammonia level without evidence of portal hypertension or hepatic synthetic dysfunction and was treated
for presumed hepatic encephalopathy during prior hospitalizations. The patient returned with the same chief
complaint despite medication compliance. Chemotherapy
treatment was withheld secondary to her poor performance status. Despite implementation of aggressive treatment for hepatic encephalopathy, the patient’s mentation
continued to decline, resulting in an amino acid evaluation.
Conclusion The two cases discussed both illustrate examples of hyperammonemia in the setting of neuroendocrine tumor. It is important to underscore that urea cycle
genetic disorders can manifest later in life. Although
these disorders may not be the most common causes for
hyperammonemia, it is important to consider their diagnoses during periods of acute stress such as malignancy,
pregnancy, bleeding, sepsis, and fasting. Upon review of
the literature specifically regarding malignancy, there is a
well-known association of hyperammonemia with multiple myeloma, plasma cell leukemia, and leiomyosarcoma,
or in patients who have recently received chemotherapy.
To our knowledge, there has only been one published
case report of hyperammonemia in the setting of neuroendocrine tumor. The cases presented demonstrate a further association between neuroendocrine tumors and urea
cycle deficiencies. This highlights the necessity for a
42
complete urea cycle evaluation for patients diagnosed
with neuroendocrine tumor and elevated ammonia levels.
Spontaneous Rupture of the Spleen in a Patient with
Diffuse Large B-Cell Lymphoma
Aashini Master, MD; Michael Keng, MD; Roberto
Contreras II, MD; Marcia A. Ciccone
University of Southern California
Introduction: Spontaneous splenic rupture (SSR) is a
rare, but known complication of hematologic malignancies. It is defined as splenic rupture without antecedent
trauma. This event is a true emergency, requiring prompt
surgical intervention.
Case Presentation: The patient was a 45-year-old Caucasian woman with no past medical history presenting
with two weeks of fevers and left flank pain. The left
flank pain was described as “throbbing and aching,”
which progressively increased in intensity. The patient
only admitted to having fevers and myalgias. She denied
night sweats, chills, and weight loss. On admission, she
appeared comfortable and in no acute distress. Vital
signs: Temperature 102.3F, HR 103/min, and BP
100/63mmHg. Initial exam was significant for palpable,
non-tender axillary and cervical lymphadenopathy and
splenomegaly extending six-centimeters below the costal
margin. She had diffuse abdominal tenderness to deep
palpation, without rebound or guarding. Significant admission laboratory findings: hemogloblin 8.9g/dL, hematocrit 25.4%, platelets 41K/cumm, white cell count
5K/cumm, metamyelocyte 2%, myelocyte 2%, total protein 4.9g/dL, and albumin 2.6g/dL. The patient received
an abdominal ultrasound in the ED, revealing
splenomegaly approximately at 16cm in length. CT abdomen/pelvis confirmed the ultrasound finding. She was
admitted to the medicine service for fever and
splenomegaly work-up. She was found to be Hepatitis C
positive; but all other infectious work-up including
Brucella, CMV, EBV, Hepatitis B, Histoplasma, HIV,
Parvovrius, West Nile Virus, fungal, and bacterial infections were negative. She was empirically placed on antibiotics for persistent fevers per Infectious-Disease consultation. Further laboratory evaluation revealed an LDH
of 463U/L. Vitamin B12, folate, TSH, iron panel, and
reticulocyte count were all within normal limits. Bone
marrow biopsy subsequently revealed diffuse large B cell
lymphoma (DLBCL). Unfortunately, prior to the results
of the bone marrow biopsy, the patient complained of
sudden increased severity of abdominal pain and was
noted to have new abdominal distention. She quickly
decompensated, and was unable to be resuscitated. Postmortem examination showed marked splenomegaly with
splenic rupture and 3000cc blood within the abdominal
cavity. Changes of DLBCL were seen involving the
spleen, bone, and lymph nodes.
Conclusion: SSR is an infrequent occurrence that is generally seen in the setting of infectious, neoplastic, hematologic diseases, or congenital splenic lesions. Risk fac-
tors associated with SSR are male sex, adulthood and
massive splenomegaly. A review of the literature reveals
approximately 150 cases of SSR in the setting of hematologic malignancy, most frequently seen with nonHodgkin’s lymphoma and acute myeloid leukemia. Splenectomy remains the mainstay of treatment for SSR and
this case emphasizes the importance of prompt surgical
evaluation for massive splenomegaly and the necessity
for further evaluation of the risk factors associated with
splenic rupture in the setting of hematologic malignancy.
Hypercalcemia and Strongyloides Superinfection in a
Patient with Adult T-Cell Lymphoma/leukemia
(ATLL)
Bradley Messenger, MD; James Yeh, MD; Maria
Dungo, MD
Harbor-UCLA Medical Center
A 55 year-old black male from Belize 6 years ago was
admitted for 1 week of alteration in mental status, decreased oral intake, and low back pain. He had 10 lb
weight loss and night sweats for 2 months. 2 months ago
he had abdominal pain and stools were positive for
Strongyloides stercoralis. He was given oral antimicrobials, but was lost to follow-up. He was afebrile, BP
170/100, lethargic, oriented only to self, hypovolemic,
and appeared wasted. He had inguinal lymphadenopathy.
BUN 60 mg/dL, Cr 4.5mg/dL, Ca 18.2mg/dL, total protein 7.1g/dL, albumin 2.7 g/dL, alkaline phosphatase 309.
Urinalysis had 1+ protein. CT scan showed multiple lytic
lesions in ribs, pelvis and spine. Following IV hydration,
furosemide, and calcitonin, the hypercalcemia improved
and the patient became gradually alert. Workup for multiple myeloma, include serum and urine protein electrophoresis and serum light chain, was negative. He was
started on hemodialysis for renal insufficiency, but subsequently developed worsening hypercalcemia and leukocytosis. Bone marrow aspirate revealed hypercellularity with involvement by adult T-cell leukemia and lymphoma, and peripheral blood smear demonstrated marked
lymphocytosis with “flower cells.” Due to refractory hypercalcemia and leukocytosis, patient was initiated on
dexemethasone, but subsequently developed respiratory
distress requiring monitoring in the intensive care unit.
Bronchoscopy revealed Strongyloides hyperinfection.
Stool also showed Strongyloides. Serology for HTLV-1
was positive, and he subsequently received combination
chemotherapy with cyclophosphamide, vincristine,
doxorubicin and prednisone. After an initial hematologic
response with decrease in lymphoma cells in the peripheral blood over several days, patient’s lymphocytosis and
hypercalcemia recurred. He developed fever, lactic acidosis, and myocardial infarction, and expired after being
placed on comfort measures.
Adult T-cell lymphoma/leukemia (ATLL) is a nonHodgkin's T-cell lymphoid neoplasm of varying clinical
manifestations and prognoses. ATLL results from HTLV1 infection and its incidence strongly associates with areas endemic to the virus, such as Japan, the Caribbean,
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western Africa and Peru. HTLV-1 may be transmitted by
blood or sexual contact, but more often transmitted
through breastfeeding, thereby infecting many at a young
age. Pathogenesis of ATLL requires HTLV-1 infection,
but only 4-5% of HTLV-1-infected individuals develop
the malignancy, typically 10-30 years following infection. Diagnosis is made by serologic confirmation of
HTLV-1 infection and identification of morphologically
proven malignant cells with T-cell surface antigens.
ATLL has 4 clinical variants: acute, lymphomatous,
chronic, and smoldering, in descending order of incidence and worsening prognosis. Chronic and smoldering
variants may be monitored without therapy, while acute
and lymphomatous variants benefit from combination
chemotherapy.
Peritoneal Tuberculosis Associated with Tumor Necrosis Factor Alpha Inhibitor Therapy for Psoriasis
LT Russell J Miller, MD; LT James C. Clifford, MD;
LCDR Robert Carpenter, DO; CDR John S.
Hammes, MD
Department of Internal Medicine
Naval Medical Center San Diego
Introduction: Tumor necrosis factor alpha (TNF-α) antagonists are effective tools in the treatment of rheumatic,
dermatologic, and gastrointestinal inflammatory diseases.
However, with increasing use of these drugs, adverse
events have become more prevalent. One increasingly
recognized complication is reactivation or development
of Mycobacterium tuberculosis (TB) infections. We present a previously treated PPD-positive patient who developed TB peritonitis after receiving TNF-α inhibitors.
Case Presentation: A 57 year-old male with a history of
longstanding psoriasis originally managed with topical
agents, was given etanercept due to progressive skin lesions. The patient was a known PPD converter with a
recent PPD in 2007 causing 20mm of induration. He denied history of active pulmonary TB, and reportedly received several months treatment for latent TB in 1975.
During treatment with etanercept, chest radiography
showed no evidence of active pulmonary TB. Three
months after initial etanercept infusion, the patient presented with progressive fatigue, anorexia, and abdominal
distention. A CT scan following admission revealed ascites and diffuse thickening of the greater omentum. Due
to concern for malignancy a subsequent PET scan was
obtained with showed multiple areas of increased uptake
in the abdominal cavity consistent with tumor versus infection. Surgical biopsy was obtained for tissue histology,
which demonstrated numerous non-caseating granulomatous foci in the omental tissue. Special stains were positive for acid-fast bacilli. The patient was started on combination drug therapy for treatment of TB peritonitis.
Discussion: After numerous reports of re-activation and
disseminated tuberculosis, the Food and Drug Administration issued a black box warning describing increased
TB risk and need for screening prior to TNF-α antago-
nists therapy. In addition, other serious adverse events
including disseminated fungal infections and lymphoma
were reported at rates exceeding the general population.
However, much about the safety information of these
agents is still unknown due to the increased rates of infection and malignancy associated with the underlying diseases as well as concomitant use of steroids and chemotherapeutic agents. Many dermatologist argue that the
disease not the drug is responsible, resulting in poor
compliance with universal screening recommendations.
This case represents an increasing number of adverse
events from using TNF-α inhibitors in the treatment of
psoriasis.
Conclusion: Although TNF-α antagonists are effective in
the treatment of multiple inflammatory diseases, there is
a growing recognition that these drugs do raise the risks
of disseminated infection regardless of the underlying
disease process. All physicians should understand the
importance of both screening and surveillance before
prescribing these drugs. Health care providers also must
be aware that myocbaterial infections often present in
atypical fashions in patients treated with these agents.
Further review is necessary to develop a uniform policy
to screen for both infectious and malignant complications
of patients receiving biologic immune modulators. As
well, we recommend initiation of a registry amongst all
deployable personnel receiving these agents.
Subcutaneous Panniculitis Like T-Cell Lymphoma
Presenting as Optic Neuritis
LT Russell J Miller, MD; CAPT Preston Gable, MD;
George Luiken, MD
Depts. of Internal Medicine and Hematology/Oncology Naval Medical Center San Diego
Introduction: Optic neuritis is classically associated with
multiple sclerosis (MS) with nearly half of MS patients
having at least one episode in the course of disease. Despite this association, optic neuritis does not necessarily
equate to a diagnosis of MS and can represent an isolated
phenomenon, infection, connective tissue disease, or
neoplasia. We present the first described case of optic
neuritis as the presenting symptom of subcutaneous panniculitis like T-Cell lymphoma.
Case Presentation: A 27 year old female presented to
ophthalmology clinic with 5 days of right-sided central
vision changes. Ophthalmological exam was consistent
with optic neuritis without associated disk edema and the
patient was admitted for IV steroids and MS work-up.
Previous medical history was only remarkable for an incidental pulmonary nodule found on CT one month prior.
Physical exam was notable for numerous non-tender subcutaneous nodules on all extremities measuring 0.51.5cm that had developed suddenly three weeks earlier. A
brain MRI was unremarkable and CT chest showed resolution of previous pulmonary nodule with interval development of numerous new nodules. Biopsy of skin lesions,
although non-diagnostic, showed features consistent with
44
subcutaneous T-cell lymphoma. Follow-up PET scan and
bone marrow biopsy were negative. The patient chose to
return to Texas to be close to family and for further diagnostic evaluation but was lost to follow-up. Five months
later, the patient re-presented with bilateral visual
changes progressing to complete blindness. Repeat MRI
showed a large frontal brain mass with extensive edema.
Craniotomy was performed for decompression and biopsy. Biopsy showed necrotic tissue with peripheral lymphocytic infiltration. The patient’s disease progressed and
she died one month later.
Discussion: Subcutaneous panniculitis like T-Cell lymphoma. is a rare type of non-Hodgkin’s lymphoma which
typically presents with deep subcutaneous nodules over
the extremities and trunk. Often the diagnosis is missed
because the lesions can resolve without treatment and reappear in other areas. Biopsy findings of skin lesions are
usually non-specific and often have features which mimic
benign panniculitis. We found only one similar case report of a 44 year-old man who presented with subcutaneous and pulmonary nodules initially responsive to steroids. In the course of his disease, he eventually developed
neurological symptoms of mono-neuritis multiplex, but
ocular nerve involvement has not previously been described.
Conclusion: Although optic neuritis is thought to be associated with multiple sclerosis, other systemic processes
should be considered in atypical cases including infectious, connective tissue, and malignant disorders. This
case is the first one describing subcutaneous panniculitis
like T-Cell lymphoma presenting as optic neuritis.
A Caucasian Male with Multiple Syncopal Episodes:
Sometimes the Hoofbeats are Zebras
Laura Morgan, MD; Gregory Price, MD
Naval Medical Center San Diego, CA
Brugada Syndrome, also known as Sudden Unexpected
Nocturnal Death syndrome, is a familial condition caused
by dysfunctional cardiac sodium channels. It is responsible for nearly one-half of sudden deaths in healthly young
individuals without structural heart disease in endemic
areas. A 28-year-old active duty Caucasian male presented to the Emergency Department complaining of 11
days of pleuritic chest pain. EKG was noted to have features consistent with a Type II Brugada pattern (a “saddleback” appearance of the ST segment with an ST elevation of 1 mm and incomplete right bundle branch block).
Cardiology elicited a history of multiple unexplained
syncopal episodes dating back to adolescence without
associated prodrome or presyncope. At least one of these
episodes had occurred during a shipboard deployment
several years earlier resulting in facial trauma necessitating surgical intervention. Previous cardiovascular and
neurological evaluations performed at an outlying facility
revealed a normal echocardiogram, head CT, electrolytes,
and thyroid stimulating hormone. The patient did not
have a family history of sudden death, aborted sudden
death, drownings, or dysrhythmias. He also denied any
history of palpitations. During his initial NMCSD Cardiology consultation, the EKG demonstrated a Type II
Brugada pattern in lead V2. Moving V1 and V2 up intercostal spaces did not result in a conversion to a Type I
pattern. Review of his medical record from three years
prior also demonstrated an EKG with a Type II Brugada
pattern. Five days after consultation a Procainamide challenge was performed. At the conclusion of the 10 minute
infusion (10mg/kg total dose), lead V1 demonstrated 2
mm of J-point elevation with a smooth, down-sloping ST
segment which ended in a terminally negative T-wave
(i.e. a Type I Brugada Pattern). Lead V2 had 2 mm of flat
ST elevation. When these two leads were repositioned
one intercostal space higher, both demonstrated Type I
Brugada patterns, yielding a positive test. Based on his
three presenting EKGs, multiple unexplained syncopal
episodes, and a positive procainamide challenge test,
Brugada syndrome was diagnosed. The patient subsequently underwent implantation of an automated implantable cardioverter defibrillator, in accordance with current
American College of Cardiology guidelines. This case
illustrates the need for clinicians to be familiar with specific EKG patterns which may be suggestive of an increased potential for malignant dysrhythmias, and guide
specific therapeutic interventions. Although Brugada
syndrome is thought to be a common cause of death in
Asian men younger than 50 years, it can also affect males
from other ethnic backgrounds.
Bilateral Inguinal Masses in a 50 Year Old Female
Margaret Moscato, MD; Christina Wang, MD
Harbor-UCLA Medical Center
A 50 y/o woman presented with a L inguinal mass that
enlarged rapidly over the past 6 months. It was barely
palpable and the same size as a mass in her R inguinal
area for over 10 years. PMH: primary amenorrhea & lack
of breast development. There was no facial hair, axillae
were shaved, and there were bilateral breast implants. A 6
x 8 cm discrete, hard mass in the L upper inguinal area
was mildly tender and a palpable mass deep in the R inguinal area was non-tender. There were labia majora &
minora & clitoromegaly (1 cm in diameter at the base and
2 cm in length). The vaginal canal ended blindly (w/o
cervix). CT showed 2 inguinal masses (R was 2.4 x 1 cm)
& no uterus. She had mild anemia (Hgb 11.4 g/dl*), normal chemistry except LDH was slightly increased to 348
units (98-192), B-HCG increased to 54.4 mIU/ml (< 0.52.67), AFP-serum, 1.2 ng/ml (0-9.0). Serum gonadotropins were increased: LH 32.5 U/l (1.9-9.3), FSH 38.2 U/l
(1.6-8.0). Serum steroid showed: increased androstenedione (A) 451 ng/dL (40-190), low testosterone (T)
96 ng/dL (250-1100), dihydrotestosterone (DHT) 20
ng/dL (25-75), increased testrone (E1) 154 pg/ml (< 68),
low estradiol (E2)16 pg/mL (< 29); while DHEA 331
ng/dL (180-1250) DHEA-SO4 84 mcg/dL (25-240), 17
hydroxypregneolone alpha 33 ng/dL (20-450) were normal. Karyotype was 46, XY. Pathology: the L mass was a
seminoma, stage T1NXMX, and the R was a testis with
45
severe tubular atrophy, loss of all germ cells & nodular
hyperplasia of Leydig cells. Further genetic testing is
pending. (*Male reference ranges are in parentheses.)
The patient is a male pseudohermaphrodite (XY genotype, testes, & genital ducts or external genitalia not
completely masculinized). Major categories of 46, XY
disorders of sexual differentiation include: 1) androgen
synthesis defects (affecting corticosteroids & androgens);
2) inability to convert T to DHT (5-alpha reductase 2
deficiency); and 3) androgen insensitivity (due to androgen receptor defects or post receptor signaling problems).
T is synthesized from cholesterol via 5 main enzymatic
steps. In the final step, A is converted to T by 17 betahydroxysteroid dehydrogenase 3 (HSD17B3) in the Leydig cells of the testes. increased levels of A and decreased
levels of T support a Dx of HSD17B3 deficiency. Mean
T:A ratio in one case series of patients with HSD17B3
deficiency was <0.4; this patient’s was 0.2. increased E1
compared to E2 levels further support the diagnosis since
HSD17B3 also converts E1 to E2.
Patients with HSD17B3 deficiency are born with ambiguous or female external genitalia. They may present
because of primary amenorrhea, inguinal masses, or virilization during puberty. Some degree of virilization
develops during puberty due to peripheral conversion of
A to T by 17B-hydroxysteroid dehydrogenase-5. A similar phenotype may be seen with 5-alpha reductase deficiency; however, the T:DHT ratio is high (>20: 1). Males
with complete androgen insensitivity do not virilize at
puberty and develop female secondary characteristics
from conversion of androgens to estrogens. The gene for
HSD17B3 is on chromosome 9q22; 20 mutations have
been reported. The deficiency is autosomal recessive;
females are asymptomatic. Treatments at a younger age
include estrogen therapy and/or corrective surgeries
(clitoroplasty or vaginal dilation). Removal of the cryptorchid testes is done to avoid neoplastic transformation,
as occurred in this patient.
Endobronchial Hodgkin Lymphoma Presenting as a
3rd Cranial Nerve Palsy
Ameer Mousssa, MD; Nadar Kamangar, MD, FACP;
Nikhil Barot, MD
Olive View-UCLA Medical Center
Endobronchial Hodgkin Lymphoma is rare and should be
part of the differential in any patient that presents with
pulmonary mass and associated symptoms. What about
those patients who present with seemingly unrelated
symptoms? It is up to the clinician to determine the relevance of every aspect of the history and physical as they
are presented and act accordingly. A 25 year-old woman
presented to neurology clinic with history of severe headaches, which started 10 days prior & awakened her from
sleep, as well as left eye swelling and diplopia that started
the previous day. She was diagnosed with 3rd nerve cranial palsy and sent to the emergency room for further
evaluation. On exam, there was a noticeable 3rd nerve
cranial palsy with ptosis of the left eye, horizontal nystagmus, decreased air entry on the right lower lung fields
in the mid-axillary region and fine crackles; there was no
miosis, anhydrosis, or JVD. She denied SOB, dizziness,
convulsions, fevers, nausea or vomiting, no recent travels
nor a history of STD’s. An MRI/MRA of the head was
done to rule out any obvious neurologic causes; which
came back negative. A chest x-ray was done which
showed a large mediastinal mass that infiltrated the right
lung. On further investigation she was found to have a
nonproductive cough and night sweats that had occurred
for the past four to five months; which she thought was
irrelevant. CT of the chest was completed and showed an
extensive heterogeneous anterior mediastinal mass extending into the mid-mediastinum, paratracheal area and
the right perihilar region with partial collapse of the RML
and RLL, random lower lobe nodules, bilateral adrenal
and renal masses. Brochoscopy showed a large exophytic
lesion that obstructed the right middle bronchus. Biopsies
were taken showing Hodgkin Lymphoma. Lumbar puncture showed no malignant cells, normal glucose and cell
count. She was then sent home with follow up to the oncology clinic where the appropriate outpatient regimen
would be started to treat the endobronchial Hodgkin lymphoma. This case shows the importance of a proper diagnosis and staging of mediastinal masses; as well as the
importance of proper history taking. The patient was being evaluated for possible causes of a 3rd cranial nerve
palsy with associated ptosis; was found to have pulmonary symptoms and a large mediastinal mass that infiltrated the right lung. Endobronchial Hodgkin lymphoma
was found; which is rare and could be mistaken for more
worrisome lesions that have worse outcomes. With
proper diagnosis, an appropriate treatment plan can be
devised and instituted with good prognosis.
Ribavirin and Peginterferon in a DRESS
Sheila Naghshineh, MD; Habibollah Alamdari, BA;
Pooya Bokhoor, MD; Sammy Saab, MD, MPH; Delphine J. Lee, MD, PhD
University California, Los Angeles
DRESS syndrome (drug reaction with eosinophilia and
systemic symptoms), is a serious idiosyncratic druginduced skin eruption presenting with fever & lifethreatening organ involvement. The delayed onset & severity of the reaction makes its diagnosis critical in preventing patient morbidity & mortality. A 64 year old man
with hepatitis C, HTN, type 2 DM, and stage III CKD
presented with malaise, fevers to 39°C, and a worsening
diffuse pruritic rash for 16 days. He had nonpurulent,
nontender confluent erythematous, scaly plaques with
scattered papules predominantly on the trunk and extremities. Labs revealed 33% peripheral eosinophilia &
serum Cr 5.2 mg/dL (GFR 11, a 63% reduction from
baseline). Skin punch biopsy showed mixed interface &
spongiotic dermatitis accompanied by a superficial and
deep perivascular mixed inflammatory infiltrate with
eosinophils. There was no evidence of vasculitis, malignancy, or infection. His symptoms began 10 weeks after
46
starting ribavirin and peginterferon alfa-2a treatment for
hepatitis C. The patient had no known drug allergies. The
clinical presentation & pathology results supported a diagnosis of DRESS syndrome associated with acute tubular necrosis. Treatment began with a corticosteroid cream
and antihistamines for the rash. The offending drugs had
been discontinued 2 weeks prior to presentation. Since
the rash was not significantly improving, IV corticosteroids were administered. He had a rapid response with the
IV steroids. His rash began to resolve, his peripheral
eosinophilia approached normal levels, and his renal
function improved. IV corticosteroids were discontinued
after 3 days and topical corticosteroid cream and antihistamines were continued. Within 10 days, the rash resolved and his renal function returned to baseline. This
case highlights the potential association of ribavirin and
peginterferon alfa-2a treatment with DRESS syndrome.
Although these adverse effects are rare, early recognition
is essential to formulate definitive therapy and prevent
severe organ damage.
Leather Fever
Sheila Naghshineh, MD; Lobna Shahatto, BA; Suzanne Donovan, MD, MPH
University of California, Los Angeles
Coxiella burnetii, the strictly intracellular, gram-negative
bacterium causing Q fever, is often latent in animals,
primary reservoirs being species of mammals, birds, and
ticks, with persistent shedding of bacteria. Humans are
usually infected by contaminated aerosols from domestic
animals, such as cattle, sheep, goats, and pets.
We present a case of a 60 year-old gentleman, in the
processed leather repair industry, who presented with 3
weeks of generalized malaise, daily fevers up to 40º C,
chills, non-pruritic migrating erythematous rash, nonproductive cough, and a 5 lb weight loss. On physical
examination, the lesions consisted of well-circumscribed
erythematous macules and papules, ranging from 1-10 cm
in diameter, waxing and waning in size and color. Labs
were significant for elevated liver enzymes, with Aspartate Aminotransferase (AST) 155 and Alanine Aminotransferase (ALT) 147. Alkaline Phosphatase and Total
Bilirubin were within normal limits. Studies for Histoplasma, Coccidioides, Cryptococcus, Acute Viral Hepatitis, Rocky Mountain Spotted Fever, Brucella, and HIV
were all negative. Right upper quadrant ultrasound was
unremarkable. The patient was initially placed on vancomycin. His lower extremity rash showed some improvement; however, he continued to have daily fevers. The
patient subsequently underwent a bone marrow biopsy,
which showed no evidence of infection or infiltrative
disease. Rheumatologic work-up for Still's disease, Lupus, and Rheumatoid Arthritis were also negative. Given
his persistent fevers and transaminitis, the patient was
started on doxycycline four days after presentation, and
he subsequently defervesced in two days. Later, his
Coxiella burnetii serology was consistent with an acute
infection, as both the phase I and Phase II titers were
positive, with the Phase II titers being greater than Phase
I. The patient was discharged with a ten day course of
doxycycline. At his three-week follow-up appointment,
he was asymptomatic with resolution of his rash and
normalization of his liver enzymes.
The case illustrates the value of taking a complete employment history and the connection between handling
processed leather and Q fever. His clinical presentation
also highlights the dermatologic manifestations of Q fever, which resemble rheumatologic, malignant and other
infectious etiologies.
Clues of the Brugada Syndrome
Elisa Ng, MD
University of California, San Diego
Brugada syndrome is characterized by a high incidence of
sudden death and one of several electrocardiogram
(EKG) patterns with an incomplete right bundle branch
block and ST-segment elevations in the anterior precordial leads. The typical patient with Brugada syndrome
is a young, otherwise healthy male with normal physical
exam findings.
A 30 year-old Caucasian male with depression on citalopram and attention-deficit hyperactivity disorder presented to the emergency department shortly after an episode of generalized weakness. At the time of the incident,
the patient was pushing shopping carts at work when he
experienced palpitations associated with diffuse involuntary muscle relaxation, followed by 10-15 minutes of
profound weakness. He had experienced similar episodes
on two other occasions in the past 5 months. His presenting vital signs and physical examination were unremarkable. EKG showed normal sinus rhythm at 75 beats per
minute, with a large R’, 1mm ST-segment elevation, and
upright T wave in V2. Labs were unremarkable including
negative cardiac markers. The patient was referred to
Cardiology for further evaluation. Repeat EKGs showed
resolution of the prior changes. Work-up is ongoing, and
the patient is scheduled to undergo a flecainide challenge
and holter monitoring to confirm Brugada syndrome.
This case illustrates helpful clues in recognizing the Brugada syndrome. Although syncope is a common manifestation, some patients are asymptomatic and the diagnosis
is made based on routine EKG. The classic Type 1 pattern consists of an accentuated J wave, coved-type STsegment elevation, and negative T wave in V1-V3. The
Type 2 pattern consists of right precordial ST-segment
elevation ≥1mm and upright T wave forming a saddleback configuration. Type 3 is similar to Type 2 but with
<1mm ST-segment elevation. Our patient’s EKG was
most consistent with the Type 2 pattern. These EKG patterns can be intermittent, making diagnosis more difficult. The pathophysiology of Brugada syndrome involves
a defect in myocyte voltage-gated sodium channels that
alters sodium flux in early repolarization, thereby increasing risk of ventricular arrhythmias. Acquired Brugada
47
syndrome may be triggered by medications such as antiarrhythmics or psychotropic drugs (including selective
serotonin reuptake inhibitors) that inhibit sodium channels.
Suspicion of Brugada syndrome in this patient was based
on his history of weakness and palpitations, SSRI administration for depression, and transient Type 2 EKG
findings. Administration of sodium channel blocking
agents can help induce Brugada patterns and arrhythmias
on cardiac monitoring. Because Brugada syndrome is one
of the leading causes of sudden death in men under age
40, patients found with a Brugada EKG pattern should be
referred immediately for further electrophysiology studies and considered for an implantable cardiac defibrillator
when the diagnosis is confirmed.
Gurgling in the Chest: An Unexpected Finding of
Bochdalek’s Hernia
Visal Nga, DO; James Keefe, MD; Ann Marie Levan,
MD; Patrick Cahill, MD; Jasminka Criley, MD
St. Mary Medical Center and David Geffen SOM at
UCLA
Case Description: A 70 year old Caucasian female with
past medical history of hypertension, and GERD presented with coffee ground emesis. She was on her annual
cruise ship enjoying breakfast when she started having
nausea and vomited blood. She denied abdominal pain,
difficulty or pain with swallowing, shortness of breath,
chest pain, or cough. On physical examination, T 98oF,
RR 20, Blood pressure 102/51, Heart rate 114, Saturation
89 % on room air. There was dried coffee ground blood
at the corner of her mouth. Cardiovascular and left lung
examinations were normal. There was absence of breath
sounds over mid and lower parts of the right hemithorax,
with tympanic percussion and prominent gurgling sounds
over the same area. The abdominal exam was normal.
Admitting Laboratories were significant for hemoglobin
10.6, hematocrit 32, BUN 61 and creatinine 1.7. A chest
x-ray revealed a right base infiltrate and hernia. Patient
was started on Ceftriaxone, levaquine and clindamycin
for possible aspiration pneumonia. She was given Protonix 40 mg IV bid for gastrointestinal bleeding and was
admitted to the Intensive Care Unit. An upper endoscopy
revealed diffuse esophageal ulcerations, which were the
source of her bleeding, giant hiatal hernia with free reflux
of gastrointestinal content, J-shaped stomach and edematous mucosa of duodenal bulb. Subsequently, we ordered
an abdominal CT scan with oral contrast to better visualize the anatomy, which revealed a Giant Hernia extending in the right thoracic cavity. Her gastrointestinal bleeding stopped and she was transferred out of the ICU. During the rest of the hospital course, she remained asymptomatic. After considering surgical vs. non-surgical
treatment, it was decided that the best option for her at
this point was observation rather than surgical intervention. She was discharged to a boarding care home on
Omeprazole and Ranitidine.
Discussion: Our patient had a congenital defect in the
diaphragm, i.e Bochdalek’s hernia with the entire stomach herniating into the right thoracic cavity. Diaphragmatic hernia can often be missed. A correct diagnosis
remains difficult and is usually made late. Our patient had
long standing history of GERD. Her Bochdalek’s hernia
was diagnosed only after she developed severe ulcerative
esophagitis and gastrointestinal bleeding. Diagnosis of
diaphragmatic hernia may not have been entertained earlier because it is rare and may have misleading clinical
features. Possibility of Bochdalek’s hernia with delayed
presentation should always be kept in mind in the differential diagnosis of adult with GERD. Earlier recognition
of the Bochdalek’s hernia may lead to earlier surgical
intervention and/or more aggressive medical management
of GERD; thus potentially slowing the progression of
ulcerative esophagitis and other complications.
Pandora’s Box: HIV, Disseminated KS, MDS and
Gangrene-Complication on Complication
Visal Nga, DO; Howard Van Gelder, MD; Benjamin
Montoya, MD; James Kefee, MD; Joyti Datta, MD;
Chester Choi, MD; Jasminka Criley, MD
St. Mary Medical Center and David Geffen SOM at
UCLA
Case Presentation: Pandora’s Box, a gift from the Greek
God Zeus, once opened will unleash evil on mankind.
HIV, once controlled with HAART, but when stopped,
can progress beyond control. We present a case in which
stopping HAART led to Disseminated Kaposi Sarcoma
(KS), Myelodysplastic Syndrome (MDS), and Severe
Gangrene leading to the demise of our patient.
A 45 year old Caucasian man with HIV/AIDS for 20
years, presented with generalized weakness and pain in
his feet. Two months prior, he was diagnosed with AIDS
related KS. He had discontinued HAART about 2-years
ago. His skin lesions were violaceus, but started turning
black after he received a one time dose of radiation for
KS in his feet. He had dark tarry stools with intermittent
diarrhea for several months. On admission he was pale,
febrile, with diffuse purple-nodular lesions on the skin of
upper and lower extremities, with swollen feet and edema
to the ankles. The toes had multiple black ulcerations;
yellow, purple discoloration; nails covered with white
plaque, and serosangineous fluid from the ulcerations
consistent with acute gangrene. LABS: WBC 26,000, Hb
4, platelet 25, BUN 112, Cr 2.25 and INR 1.81. EGD
revealed bleeding gastric mucosal ulceration and KS lesions in duodenum that required multiple transfusions.
Persistent thrombo-cytopenia led to a bone marrow biopsy which revealed MDS. Blood sepsis from MSSA,
VRE, & E. coli was treated with antibiotics. MRI showed
soft tissue swelling without osteomyelitis. The patient
became afebrile and was restarted on HAART therapy. In
addition to superficial debridement, amputation was recommended for definitive treatment of his gangrene,
which the patient declined. Chemotherapy could not be
instituted due to active infection and low platelets. Due to
his poor prognosis, the patient decided on comfort care
48
only and subsequently died from respiratory failure due
to pneumonia and pulmonary KS.
Discussion: KS is a spindle-cell tumor likely derived
from endothelial cell lineage seen most often in patients
with HIV/AIDS. Current frequency in the United States
is listed at 2500 cases/year. Our patient was initially controlled with HARRT, but developed aggressive disseminated KS after stopping HAART. His foot lesions,
likely gangrene, were amongst the most serious complications, leading to sepsis. Complicating matters is the
patient’s statement that his foot lesions did not worsen
until initial treatment with local radiation, even after restarting HAART. Is it possible that the patient had a
paradoxical reaction to radiation therapy? KS can cause
gangrene, but there is no substantial literature that gangrene caused from low radiation doses typically used for
treating KS lesions. His rapid decline and worsening
gangrene were likely also attributable to his severe preexisting conditions (MDS, high HIV viral load, extensive
tumor burden). Though rare, epidemic KS is still present
in the HAART era. The case shows that patients with
advanced KS can be a true “Pandora’s Box,” –once
opened, the possible complications and organ systems
involved may be limitless.
An Atypical Form of Lung Cancer: Intrapulmonary
Schwannoma
Jennifer Le Nguyen, MD; Jennifer I. Chang, MD;
Grant Lee, MD
Kaiser Permanente Los Angeles
Case: A 70 year old gentleman was admitted to the hospital with a chief complaint of orthopnea, several week
history of shortness of breath and mild, productive cough.
His past medical history is significant for chronic pulmonary obstructive disease (COPD). He had been in his
usual state of health until he returned home from a vacation to Cabo San Lucas, Mexico. Upon return from his
trip, he was with subjective fever, cough, and mild production of white sputum alleviated with home nebulizer
therapy with bronchodilators. Due to persistence of
symptoms, he presented to his primary care physician and
was prescribed an outpatient therapy of bronchodilators, a
short course of prednisone, and oral antibiotics. His
symptoms initially improved but recurred and persisted
intermittently for an additional two weeks. This patient
ultimately presented to the emergency department after
experiencing new onset orthopnea in association with his
previous two week history of cough and shortness of
breath. He was initially diagnosed with an acute COPD
exacerbation and treated with IV steroids, antibiotics, and
nebulized bronchodilators. His workup for cardiac etiology and infectious precipitants was unremarkable. The
patient was further evaluated with computed tomography
(CT) scan of the thorax. CT was remarkable for a distal
endotracheal tumor approximately 2.2 by 1.4 centimeters
in size with near obstruction of the lumen. He was taken
to the bronchoscopy suite for biopsy. During the procedure he developed respiratory distress with near obstruc-
tion of the airway. He was brought emergently to the operating room for rigid bronchoscopy. The majority of the
tumor was removed with the rigid bronchoscope. Remainder of hospital course was unremarkable and patient
was discharged to home.
Discussion: Primary pulmonary schwannoma is an extremely rare intrapulmonary, neurogenic tumor. This rare
condition was first reported in 1940 and subsequently,
less than several hundred cases have been identified. The
symptoms and radiographic manifestations may be variable and nonspecific. Patients with history of underlying
pulmonary disorders such as asthma or chronic pulmonary obstructive disease (COPD) may obscure the clinical
manifestations of this rare condition, leading to delayed
diagnosis. In this clinical vignette, we present a 60 year
old gentleman with a history of COPD presenting with a
several week history of shortness of breath and stridor.
He subsequently underwent bronchoscopy and diagnosed
with primary pulmonary schwannoma confirmed on pathology obtained from transbronchial biopsy. In this
clinical vignette, we provide a clinical analysis and review of the current literature on this rare condition.
Nodular Regenerative Hyperplasia: A Rare and Silent
Cause of Noncirrhotic Portal Hypertension
Jennifer Le Nguyen, MD; Brandon Beaber, MD;
Theodore Sy, MD
Kaiser Permanente Los Angeles
CaseS: In this case series report we present two patients
with a history of HIV presenting with progressive abdominal distension. The first patient is a 63 year old gentleman with a two month history of progressively worsening abdominal distension. His past medical history is significant for a history of HIV in which he had previously
been compliant with anti-retroviral treatment: Kaletra,
Didanosine, and Atazanavir. The second patient is a 42
year old female presenting with a two to three month
history of progressive, painless abdominal distension. Her
medications included Didanosine, Truvada, and Kaletra.
Workup for both patients was extensive in evaluating for
etiology of portal hypertension and included evaluation
of viral and autoimmune hepatitis etiologies, liver
transaminases, and evaluating for malignancy with imaging studies such as ultrasound and computed tomography.
Management included diagnostic and therapeutic paracentesis, liver biopsy and esophagogastroduodenoscopy.
Liver biopsy with reticulin staining was significant for
findings consistent with nodular regenerative hyperplasia
in both patients. Following diagnosis via liver biopsy and
alterations in anti-retroviral treatment, including cessation
of didanosine, there has been an interval improvement of
ascites and abdominal distension in both patients.
Discussion: Nodular regenerative hyperplasia (NRH) of
the liver is a rare cause of noncirrhotic portal hypertension in adults and children. This uncommon condition is
characterized by diffuse transformation of normal hepatic
parenchyma into small, regenerative nodules. Although it
49
is a rare cause of portal hypertension, it is one of the main
causes of noncirrhotic portal hypertension in the Western
world. NRH
has been reported to occur in association with certain
medications, rheumatologic disorders, vascular disorders,
and myeloproliferative disorders. In this clinical vignette,
we present two patients and their initial presentations
leading to the diagnosis of NRH in both patients. The
first is a 63 year old gentleman presenting with a two
month history of progressively worsening abdominal
distension and bloating and diagnosed with NRH. The
second patient is a 42 year old female presenting also
with a two to three month history of progressive, painless
abdominal distension. We also review the hypothesized
pathogenesis, histopathology, current literature, and varying management options in a rare condition that can lead
to noncirrhotic portal hypertension and associated serious
and longterm sequelae such as variceal bleeding, ascites,
and splenomegaly.
Anemia, Epigastric Pain, and Itching in an HIV+ Patient
Sean Novak, BS
University of California, Los Angeles
Introduction: Itching is an unpleasant sensory experience which elicits a desire to scratch. Unfortunately, because itching is a non-specific and seemingly benign
symptom, it is often dismissed as secondary to dry skin or
a reaction to laundry detergent and treated with diphenhydramine or a dermatology followup. However, itching
can be an essential clue to a number of dangerous and
occult conditions.
Case: A 42-year-old man with history of HIV infection
presented with a 5-6 month history of fatigue, epigastric
and pleuritic pain, night sweats, and generalized itching.
Additional history revealed an episode of dark, sticky
stool approximately two months previously, and an episode of Pneumocystis jiroveci pneumonia one year earlier. On physical exam, he was febrile to 100.6 and
tachycardic, with orthostatic hypotension, pale conjunctivae, and lymphadenopathy of posterior auricular and axillary nodes. He had bibasilar pulmonary crackles, tenderness over the epigastric region without rebound or guarding, and a rectal exam negative for gross blood. Labs
revealed hemoglobin 7.4 g/dL (normal 13.5-17.5) with
inadequate reticulocyte compensation, CD4 count of 18
(normal 500-1600), and occult blood tests negative times
three. Creatinine and blood urea nitrogen were elevated.
Liver function tests were within normal limits. A chest xray was clear.
Two units of blood were transfused with an appropriate
hemoglobin increase. Diphenhydramine was administered in hopes of alleviating the patient's itching, but had
only a minor effect. Lymph node biopsies were considered but deferred until the patient was hemodynamically
stable.
Gastroenterology was consulted and performed an upper
endoscopy which revealed multiple gastric ulcers. Biopsies were taken and sent for analysis, with high suspicion
for cytomegalovirus gastritis given the immunocompromised state. A proton-pump inhibitor was started. Ulcer
biopsies were found to be positive for lymphoma, diffuse
large B cell type. PET-CT imaging for staging revealed
uptake in the stomach and focal areas in the lung and
bowel, as well as diffuse lymph nodes in the axillary,
cervical, and inguinal regions. A bone marrow biopsy
was performed and confirmed marrow involvement. Oncology soon began treatment with R-CHOP chemotherapy.
Discussion: This case demonstrates how seemingly minor complaints can be clues to major illness. In regards to
this patient with a GI bleed and anemia, it would have
been easy to treat the acute issues, and dismiss the itching
to a primary care physician followup visit. Doing so neglects the patient's comfort level as well as the underlying
condition. While this patient's itching, epigastric pain,
and anemia were all related, in another patient each problem could easily be due to a separate pathologic process.
Itching can be due to malignancies, renal disease (uremia), cholestasis, infections (dermatophytes, scabies), as
well as the more well known causes such as dry skin,
contact/atopic dermatitis, urticaria, and psoriasis.
A Case of Amiodarone-Induced Thyrotoxicosis with
Severe Myopathy and Dysphagia
Ashley Ono, MD
Scripps Mercy Hospital
Case report: A 69 year old Caucasian male with a history of non-ischemic cardiomyopathy (EF:20%) s/p
biventricular AICD placement in 2005 presented with
severe weakness, lightheadedness, significant weight loss
and dysphagia. Due to repeated AICD firing from recurrent VT, he was placed on amiodarone 3 years prior. On
initial evaluation, TSH was undetectable, FT4 and FT3
were markedly elevated and CK was normal.
Discussion: Amiodarone is a widely used, potent antiarrhythmic drug with significant side effects on non-cardiac
organs of which thyroid dysfunction is a frequent manifestation. Although most commonly associated with hypothyroidism, amiodarone-induced thyrotoxicosis (AIT)
occurs in 3% of patients and is associated with adverse
cardiac events. Amiodarone induced thyroid dysfunction
occurs as a result of combined intrinsic and iodineinduced effects. Type 1 AIT is an iodine-induced hyperthyroidism that occurs in patients with underlying thyroid
disease (i.e. Graves’ or multinodular goiter) as a result of
increased substrate delivery and unregulated hormone
synthesis. Type 2 AIT, is a drug-induced destructive thyroiditis that occurs due to release of pre-formed thyroid
hormone. Differentiation of AIT, although often difficult,
is essential to initiating appropriate therapy as Type 1
AIT requires anti-thyroid medication while Type 2 AIT is
responsive to steroids. Frequently, thyroid ultrasound
50
with Doppler flow is used to distinguish type of AIT,
however, TSI, IL6, and radio-iodine uptake scans can
also be used. Upon diagnosis of AIT, continuation of
amiodarone therapy must be carefully considered. While
therapy may be continued in Type 2 AIT, current recommendations support the cessation of therapy, if possible,
for those with Type 1 AIT. Ultimately, the risk of unstable, fatal arrhythmias must be weighed against those of
thyrotoxicosis.
described. The use of furosemide could not account for
each episode of hypokalemia in this patient. While elevated aldosterone levels can cause hypokalemia in cirrhotics, her serum sodium remained in the low normal
rang. She did not respond to spironolactone. She eventually developed a distal Type 1 RTA during the final hospitalization for end stage liver disease.
Additionally, thyrotoxicosis is also associated with neurologic manifestations, including myopathy. Approximately 60-80% of patients with untreated hyperthyroidism experience myopathy manifested as proximal muscle
weakness. In rare cases, those suffering from acute thyrotoxic myopathy may present with severe proximal and
distal weakness and bulbar dysfunction. While features
including increased cellular metabolism, increased protein degradation and decreased muscle carnitine contribute to myopathy, the pathogenesis remains unknown. In
this case, cardiopulmonary exercise testing, EMG and
nerve conduction studies were used to elucidate the cause
of our patient’s myopathy. The cause of our patient’s
myopathy was confounded by side effects of his concurrent medical therapy with amiodarone and simvastatin.
Winnie Pang, MD
Kaiser Permanente Southern California
Recurrent Symptomatic Hypokalemia in an Outpatient with Cirrhosis
Katie O’Sullivan, MD; Susan E. Trompeter, MD,
FACP
University of California, San Diego
Furosemide has been used frequently in the treatment of
ascites. Hypokalemia may subsequently occur as an adverse reaction. In addition, hypokalemia presumably due
to an increase in aldosterone can be seen in cirrhotics
with ascites. In 2004, a 47-year-old woman presented
with encephalopathy, jaundice, and ascites and was diagnosed with end stage liver disease secondary to alcohol
abuse. Liver biopsy showed chronic hepatitis with severe
fibrosis. The patient recovered clinically and was placed
on a transplant list. She, however, was lost to follow up
and continued to drink. In 2008, she presented to the GI
clinic at our institution with jaundice, elevated INR, and
thrombocytopenia. Abdominal CT scan revealed a markedly cirrhotic liver. She returned with ascites and weight
gain and was placed on spironolactone with the addition
of furosemide. Furosemide was subsequently discontinued due to presyncopal events. Over the course of the
next 12 months, the patient had recurrent hypokalemia
ranging from 2.8 to 3.0 presenting clinically as muscle
cramping despite 20 - 30 MEQ of KCL daily. Spironolactone between 50 – 100 mg daily was also prescribed during this time. The use of furosemide 10 mg was infrequent and did not account for episodes of hypokalemia.
She presented with jaundice and acute renal failure and
was diagnosed with potassium-wasting renal tubular acidosis as an inpatient prior to her death. This case describes frequent episodes of symptomatic hypokalemia in
a woman with cirrhosis, a treatment challenge not well
Central Pontine Myelinolysis: Not Just about Sodium
Introduction: Central pontine myelinolysis (CPM) is a
rapidly progressive neurological syndrome characterized
by symmetrical, non-inflammatory demyelinization in the
central base of the pons. CPM is often associated with
rapid correction of hyponatremia but is also seen in many
other conditions.
Case: A 64 year old African American male with a past
medical history of COPD and a 25 pack year history of
smoking presented to the emergency department with gait
instability, dysarthria, and bilateral upper and lower extremity tremors for 1 week. Review of systems was positive for fatigue, anorexia, unintentional weight loss, dry
cough, and hallucinations. On admission, the patient was
afebrile and vital signs were stable. PE was remarkable
for clubbing. Neurological exam revealed dysarthria,
truncal and appendicular ataxia, left upper extremity
weakness, and absent reflexes throughout. Laboratory
data was significant for a sodium level of 129 mEq/L,
compared to 133 mEq/L 3 months prior, and an albumin
of 2.8 g/dL. Urine osmolality was increased at 562
mOsm/L and serum osmolality low at 258 mOsm/kg.
After excluding other causes of hyponatremia, the patient
was felt to have euvolemic hyponatremia due to SIADH,
which was treated with fluid restriction. An MRI of the
brain was performed, revealing increased FLAIR and T2
signal intensity in the bilateral pons suggestive of central
pontine myelinolysis. CT chest showed a 5.1 x 2.7 cm
subcarinal soft tissue density. Transbronchial biopsy of
the subcarinal mass was consistent with small cell lung
carcinoma. During hospitalization, the patient showed no
clinical improvement. He was scheduled to start chemotherapy for small cell carcinoma as an outpatient.
Discussion: In this case, the patient presented with a
myriad of neurological and constitutional symptoms,
along with hyponatremia likely due to SIADH. These
findings were highly suggestive of a paraneoplastic syndrome. Interestingly, CPM was discovered through
neuroimaging and, later, small cell lung carcinoma was
confirmed on biopsy. This case is unusual in that CPM
developed without the rapid correction of hyponatremia
and was likely multifactorial, occurring in the setting of
occult malignancy, hyponatremia from SIADH, and malnutrition. It highlights the importance of considering
lesser known etiologies for CPM.
51
CPM was originally described in 1959 by Adams et al. in
patients with chronic alcoholism and malnutrition. However, it is now typically linked with the correction of severe, chronic hyponatremia. Other conditions associated
with CPM include liver failure, organ transplant, burns,
and chronic kidney disease. CPM is hypothesized to be
related to changes in osmotic forces causing cellular
edema and degeneration of myelin sheaths. Manifestations may vary, with symptoms including pseudobulbar
palsy, ataxia, spastic para- or quadraparesis, seizures,
diminished reflexes, lethargy, delirium, and a “locked-in
syndrome.” Outcome is variable, from complete resolution of symptoms to no improvement and death. Treatment is supportive.
A Rare Case of an Inflammatory Myofibroblastic
Tumor of the Bladder Masquerading as Everything
But
Christopher Parrish, MD; Elaine Ku, MD; Vanita
Chiang, MD; Brian Jason Brotherton, MD; Michael
Welker, MD
University of Southern California
acid-fast bacilli. Concern regarding the potential for malignant conversion of urachal remnant tumors prompted
elevective surgical excision of the mass with bladder
preservation. On gross examination and intraoperative
frozen section no malignancy was identified and a preliminary diagnosis of urachal cyst was made. However,
the final pathologic diagnosis was inflammatory myofibroblastic tumor.
Discussion: Inflammatory myofibroblastic tumors
(IMTs) are rare tumors known to occur in the lungs, intestines, breast, kidneys, and rarely, the bladder. IMTs are
commonly mistaken for other entities due to their underrecognition amongst surgeons, radiologists, pathologists,
and internists. A history of bladder instrumentation and
recurrent urinary tract infections have been linked to the
development of IMTs. Urinary tract infections in males
over one year of age are rare and should always prompt
further workup in a man with no other predisposing factors. Heightened awareness of the existence and varied
presentations of IMTs acan lead to prompt diagnosis and
proper treatment.
5150 HOLD - Not Always Psychiatric
Introduction: Inflammatory myofibroblastic tumors
(IMTs), also known as plasma cell granulomas, are rare
tumors of unknown etiology. IMTs represent a diagnostic
challenge because of their infrequent occurrence and potential to mimic numerous malignancies.
Case: An 18-year-old man with no significant past medical history presented with a 1-year history of a lower abdominal mass. He initially sought care for new-onset dysuria and lower abdominal pain associated with a palpable mass below the umbilicus. Computerized tomography
(CT) of the abdomen and pelvis showed a mass above the
urinary bladder, adjacent to and involving the rectus
muscles, felt to be a hematoma. A urinary tract infection
was diagnosed clinically, and the patient reported resolution of all symptoms after antibiotics. Two months prior
to admission, the expanding abdominal mass recurred,
accompanied by dull lower abdominal pain. No history of
weight loss, fatigue, fevers, hematuria, change in bowel
patterns or family history of cancer was elicited. The patient consulted his physician again and was referred for
imaging. An umbilical hernia and an irregular mass superior to the bladder that appeared to be a hematoma involving the rectus muscles was seen on MRI. The patient
was referred for surgical and hematological evaluation
and presented to a tertiary care center for an alternate
opinion. Vital signs were normal. Physical examination
was unremarkable except for a 6-cm nontender mass palpable in the midline of the abdomen inferior to the umbilicus. No lymphadenopathy was noted. All laboratories,
including serum creatinine and hemoglobin, were normal.
Urinalysis was also unremarkable. Repeat MRI showed a
complex mass anterior to the bladder. Differential diagnosis was considered to favor urachal remnant tumor
versus primitive neuroectodermal tumor. CT-guided biopsy of the mass showed only granulation tissue formation and chronic inflammation, with negative stains for
Kalpesh Patel, MD; Marian Kaldas, MD
Olive View-UCLA Medical Center
Learning Objective: Important differential to consider in
acute psychosis-- Infectious vs. Metabolic vs. Autoimmune vs. Psychiatric vs. Neurological diseases vs. Malignancy vs. substance abuse
Case Information: 23 y/o Hispanic male, no PMH, presented to psych ER with 3 days history of sudden change
in behavior, delusional thoughts, generalized weakness
and headache. He was alert and oriented x 2 and placed
on 5150 hold for danger to self and others with combative
behavior. Medicine consult was requested and patient
admitted to medicine service to rule out other causes of
acute psychosis. He was born in Mexico, vaccinated up to
date, intermittently visiting Mexico every 6 months and
admits having stressful relations with wife and at job, no
family history of medical, psychiatric problems. Patient
continues to be altered and delusional on floor so 5150
hold was continued and placed on 2+ intermittent soft
restraints. Patient had a normal vital signs except tachycardia, normal PE findings.Chemistry, CBC, LFTs, TSH
were normal. Serum and urine toxicology, CXR, urine
analysis, troponin, CT chest angiogram, blood/urine cultures (-). EKG showed sinus tachycardia. LP was done
prior to antibiotics that revealed no WBC, normal glucose
and slightly elevated protein and negative infectious work
up including gram stain & culture, viral culture, CSF
VDRL, Indian ink, HSV DNA PCR, and WNV. MRI
brain and MRA brain and neck were negative. RPR, HIV,
Hepatitis panel was negative. On recommendations of
Neurology and ID services, further work up including
Antithyroglobulin Abs, Urine porphyrins, ceruloplasmin,
Testicular exam, ASO titer, mononucleosis Ab, Lyme
antibody, EBV serum titer, CSF EBV PCR, CSF cysti-
52
cercosis Ab, Urine heavy metal screen, CD4 count, ACE
level–all were negative. ESR 47(slightly elevated), EEG
was normal. Repeat Lumbar puncture and Brain imaging
negative. Autoimmune work up including ANA, Anti
Smith Abs and Anti SSA were positive with low C3, C4
and Total Complements. Patient was diagnosed with lupus cerebritis with neuropsychiatric manifestations and
placed on pulse dose of steroids; all other antibiotics and
antiviral discontinued. Patient did not respond to pulse
dose steroids, started on IV soulmedrol high dose – followed by IV cyclophoshamide (CYTOXAN) one dose
with no improvement in mental status. Later on he was
treated with IV Immunoglobulin over a period of 4
days. Patient responded to IVIG very well and mental
status improved. His Psychiatric hold was discontinued
and discharged home with subsequent follow up in primary care and rheumatology. On subsequent follow up
visit in outpatient clinic he was back to his normal behavior and normal life.
Discussion: SLE is a chronic inflammatory disease of
unknown cause which commonly affects skin, joints,
kidneys, lungs, serous membranes. It is predominantly
common in female in their 20s and 30s than men. It is
usually presents with systemic signs of inflammation and
clinical findings. First presentation of acute severe psychosis secondary to lupus cerebritis is very rare. This case
highlights the unusual findings of Lupus and one important diagnosis to consider in the differential diagnosis of
acute psychosis since we, as an internist/primary care
physician, are getting so many psychiatric patients admitted to medicine service to rule out medical causes of psychiatric problems.
A Case of the Iron Heart
Jonathan A. Peng, MD; David Chang, MD; Arnold
Baas, MD
University of California, Los Angeles
Case: A 48 year old female with end stage liver failure
from underlying hepatitis C complicated by refractory
ascites and severe encephalopathy received a liver transplant and within two days developed extensive hypotension and renal failure. She was started on vasopressors
however eventually required an intraaortic balloon pump
(IABP) to maintain blood pressure. The initial differential
included sepsis, cardiac damage from vasopressors, or a
direct cardiac effect by the hepatitis C virus. Echocardiography done prior to the transplant was within normal
limits, however at that time revealed a left ventricular
ejection fraction (LVEF) of 13%, right ventricular ejection fraction (RVEF) of 30%, and biventricular dysfunction consistent with dilated cardiomyopathy. In the next
few days, the patient continued to be hypotensive and
was found to be in pulseless electrical activity (PEA)
requiring compressions multiple times. The patient expired after asystole within the week after transplant. An
autopsy was done which revealed extensive hemosiderin
deposition throughout the cardiac myocytes, pancreas,
kidneys, adrenal glands, and small bowel consistent with
hemochromatosis. Interestingly enough, the pretransplant and post-transplant liver did not contain the
amount of hemosiderin found in hemachromatosis.
Discussion: Hemachromatosis is a multifactorial genetic
syndrome characterized by excess circulating iron which
leads to parenchymal iron deposition in numerous organs
throughout the body. The underlying mechanism is due to
defective synthesis or decreased activity of hepcidin, a
peptide hormone produced by hepatocytes in response to
elevated serum iron. Genetic mutations, direct destruction
of hepatocytes, and hepcidin resistance can lead to iron
overload. The most common mutation is a homozygous
defect of HFE (which was not found on PCR in the patient post-mortem). There are other rare causes of hemochromatosis which were not investigated and may be the
cause of this patient’s presentation of primarily cardiac
hemochromatosis, which has been documented. Interestingly enough, of patients receiving liver transplants, those
with hemochromatosis are disproportionately more likely
to develop cardiac complications particularly dilated cardiomyopathy with conduction abnormalities.
Lymphandenopathy and Fevers that Progressed to a
Skull Lesion as a Presentation of Langerhans Cell
Histiocytosis
Duc Phan, MD; Jeffrey Chen, MD; Katherine Lin,
MD; Kathyn Lee Serwoka, BE; Eric Finlayson, MD;
Jeffrey Canceko, MD
University of Southern California
Case: Mr. KM is a 30 year-old Caucasian male with history of diffuse lymphadenopathy for 5 months, presented
to the emergency room for severe left-sided headache,
photophobia, and hyperacusis for two months. Five
months prior to admission, he presented to an outside
hospital with pain and swelling of left axilla mass. He
was initially treated with bactrim for two weeks without
much improvement. The patient has had multiple axillary
biopsies at an outside hospital, however all the biopsies
were non-diagnostic. He was then referred to USC for
further evaluation. Infectious etiologies were ruled out.
Excisional biopsy performed 2 months prior showed
mantle cell hyperplasia; however immunohistochemistry
was inconclusive. He started having severe left-sided
frontal headache with nausea, photophobia and left ear
hyperacusis and came to the ED. He had no other neurological deficits. CT and MRI scans of the head showed a
destructive lesion involving the left frontal calvarium
with indistinct borders extending into the scalp with scalp
swelling progressing into the extra-axial space. The lesion measured 16 mm x 27 mm x 17 mm. A left fontal
craniotomy with an excisional biopsy was preformed.
Pathology of the left frontal bone mass showed Langerhans cell hyperplasia. Patient’s symptoms resolved after
the surgery and he was discharged home to be followed
by Hematology, Neurosurgery, and his primary care physician for further treatment. The plan is to assess for other
lesions and if amenable surgical resection and to followed
with chemotherapy.
53
Discussion: Langerhans Cell Histiocytosis (LCH), formerly known as Histiocytosis X, is a rare disease. It is
characterized by abnormal proliferation of cells found in
epidermal histiocytes (Langerhan Cells). LCH can present as lesions in the bone (most common), skin, liver
spleen, bone marrow, lymph nodes, hypothalamicpituitary region and lung. LCH is more commonly seen in
children ages 0-15. However, reports in young adults
have been documented. Diagnosis is made by cellular
morphology and the presence of CD1a and CD207. Birbeck granules are classically found in LCH lesions by
electron microscopy. Because LCH is more commonly
seen in children, diagnosis is commonly delayed in young
adults. LCH in our patient’s age group is rare. Note: bony
erosive skull lesions are a common presentation. He has
multisystem LCH given his lymph node involvement.
The rarity and indolent manner of LCH in adults makes
its incidence difficult to assess and diagnosis. This case
stresses the importance for a proper tissue biopsy and the
need for multiple biopsies, in addition to close monitoring for progression of disease. Since LCH can be a systemic disease affecting so many areas of the body a multidiscipline approach should be instituted. The internist
should pursue consults from various specialists such as
hematologists, oncologists, radiologists, surgeons, pulmonologists, and/or dermatologists for evaluation depending on the patient’s unique presentation and for assistance on diagnosis and treatment. The treatment for
this rare condition is not well established. Treatment for
multisystem LCH would include excision of the bony
lesions, oral steroids and chemotherapy, namely, single
agent vincristine. Cladribine appears to have some activity, as do vinblastine with steroids and anti-inflammatory
agents. There are open protocols at some institutions.
This being the case surveillance is important, for if there
is no active disease simple observation is an option. Otherwise treatment plans consist of some combination of
surgical intervention and chemotherapy.
TB or not TB: A Patient with Abdominal Imaging
Suggestive of Peritoneal Carcinomatosis, Proven on
Biopsy To Be Mycobacterium tuberculosis
Duc Phan, MD; Lusine Soghbatyan, MD; Nazanin
Azadi, MD; Jeffrey Canceko, MD
University of Southern California
In 2006 the CDC had 139 documented cases of abdominal tuberculosis (ATB). ATB signs and symptoms are
wide and varied. For tuberculosis peritonitis there are
three forms: the “wet” (ascites), the “dry-plastic” (peritoneal involvement without ascites) and the “fibroid-fixed”
type (omental thickening and extensive adhesion formation). The outcomes can be severe even though it is readily treatable, mainly due to a delay in diagnosis.
Mr. JZ is a 67-year-old male with a history of benign
prostrate hypertrophy and depression complaining of
abdominal pain. He has had mild symptoms for the past
two years, but worsening in the last three months. The
pain was diffuse, constant, and cramping; and worse after
eating. He had symptoms of feeling feverish with chills,
but only one episode of nausea and vomiting. He denied
any hematemesis. He also denied any respiratory problems in the past or on this admission. He noted a weight
loss of about 30 pounds in the last two months.
CT of the abdomen and pelvis showed diffuse peritoneal
carcinomatosis, retroperitoneal and mesenteric nodules,
and cardiophrenic adenopathy. He was admitted to Medicine for workup and treatment. He continued to spike
fevers despite being on broad-spectrum antibiotics. His
pan-cultures were negative. Infectious Diseases, Gastroenterology, and Surgery services were consulted. The
patient had an esophagogastroduodenoscopy (EGD),
colonoscopy, and an EGD with push enteroscopy which
all showed no evidence of disease. The patient's CA19-9,
CEA, ACE, and LDH levels were normal. A PPD was
placed and proved positive.
A laparoscopic biopsy of his omentum and mesentery
was performed. Preliminary evaluation of the biopsies
showed evidence of TB. The patient was started on rifampin, INH, pyrazinamide, and ethambutol (RIPE) therapy. Final results showed the omental and mesentery biopsies had necrotizing granulomatous inflammation and
were positive for AFB on special stains. Cultures from
the biopsies grew M. tuberculosis complex, with a high
probability for M. tuberculosis. On RIPE his symptoms
improved; he was discharged to be followed by his primary physician and Public Health.
Peritoneal TB should be considered in the differential
diagnosis when presented with symptoms of abdominal
pain, fever, and weight loss. Especially in a patient with
peritoneal carcinomatosis as Mr. JZ. An awareness of this
diagnosis can expedite the necessary procedures for diagnosis and early treatment. Procedures such as endoscopy
or paracentesis for fluid assessment are valuable tools. In
addition PCR testing may be beneficial with a suggestive
history, but negative special stains for acid-fast bacilli on
tissue sections. Laparoscopy, when indicated, should not
be delayed for it can provide visual inspection to aid early
assessment and intraoperative frozen sections to provide
definitive diagnosis.
Early Myocardial Infarction Reveals a Triplet of
Prothrombotic States
Michelle Phillips, MD; Robert Russo, Jeffrey Andrey
Scripps Green Hospital
A 45 year old woman presented with persistent left-sided
chest and arm pain, increased cardiac markers, and inferior T wave inversions. Cardiac catheterization showed
80-90% occlusion of LAD and 40-50% occlusion of the
first diagonal coronary artery. Her early onset coronary
heart disease (CHD) prompted consideration of a
prothrombotic or hypercoagulable disorder. Her PMH
included hypothyroidism, HTN, and 4 normal pregnancies. There was no history of miscarriage, venous or arte-
54
rial clots prior to her presentation with acute myocardial
infarction (AMI). Family history was likewise (-) for
early CHD or evidence of hypercoagulable states. PE
showed a BMI of 28 and was otherwise (-). Basic lab
studies and fasting lipid panel were within normal limits.
Protein S function, Protein C activity, and Factor V activity were normal. Prothrombin gene mutation was (-). The
patient had the following unusual combination of defects:
R506Q Factor V Leiden mutation (linked with activated
protein C resistance), (+) for antiphosphatidylserine IgM
and beta-2 glycoprotein I IgG antibodies (indicating antiphospholipid syndrome), and the methylenetetrahydrofolate reductase (MTHFR) 677; T homozygous “TT”
genotype. Activated protein C resistance confers a 3-to-8fold increased risk for venous thrombosis; conflicting
evidence exists regarding an association with arterial
thrombosis and acute MI, but a modest association has
been reported in young females. Antiphospholipid syndrome is associated with recurrent venous and arterial
thrombosis & recurrent fetal loss. MTHFR is involved in
the conversion of homocysteine to methionine in a folatedependent process. The TT genotype predisposes to increased serum homocysteine, which correlate with risk of
CHD, especially in the presence of low folate levels. Hyperhomocysteinemia has been the subject of much interest and research in the past decade. The in-vivo effects of
hyperhomocysteinemia on the coagulation cascade, endothelial function, atherosclerotic lesion formation, and
platelet aggregation are well-established; however, studies of the administration of folate, B6 and B12 to reduce
homocysteine levels demonstrates no efficacy in secondary prevention of CHD. Careful review of the most recent evidence demonstrates a likely significance in early
CHD in young female patients with the MTHFR 677 TT
genotype especially in the setting of folate deficiency, but
the value of screening or treatment for this state in the
general population or for secondary prevention remains
in question. Lifelong full-dose aspirin and anticoagulation with warfarin were recommended for this patient
with multiple hypercoagulable disorders.
When the Solution is the Problem: Polypharmacy of
Supplements and a Case of Myopathy
Michelle Phillips, MD; Nitasha Bakhru, MD; Enoch
Wang, MD; Laura Nicholson, MD
Scripps Green Hospital
Patients commonly present with a list of nutritional supplements, over the counter medications, and traditional
remedies used in addition to their prescribed medications.
Clinicians must persist in obtaining this history, and selfeducate regarding the effects of unfamiliar agents on the
health of our patients. This is a case of an elderly woman
presenting with a constellation of complaints and a
myopathy attributable to one home remedy among a
lengthy list of supplements.
A 74 year old woman with history of dyslipidemia, migraine, and hypothyroidism presented with progressive
weakness over three months. The patient had previously
been able to perform aerobics for an hour at a time, and
now could not walk further than the length of a room
before feeling weak and stopping to rest. The patient also
reported sores on her tongue, constipation, pustules on
her face, right lower extremity tingling sensations, excessive salivation, weight gain, and hair loss. She admitted
to using multiple “medications” she ordered over the
Internet, and had stopped using prescribed medications
for dyslipidemia and hypothyroidism.
The patient’s daughter produced a list of supplements
including L-carnitine, selenium, zinc, lutein, iron, folic
acid, vinpocetine, milk thistle, choline, inositol, calcium,
potassium, taurine, biotin, magnesium, carnosine, 5hydroxytryptophan, L-lysine, methylsulfonylmethane,
gamma-aminobutyric acid, vitamin B6, alpha-lipoic acid,
malic acid, beta carotene, methylcobalamin, fish oil, flax
seed oil, castor oil, molybdenum, Lugol’s solution, riboflavin, idebenone, valerian root, chlorella, and CardioPeptase.
Physical exam revealed a well-nourished woman with
normal vital signs. There were scaling, excoriated pustules on the scalp and face as well as shallow ulcerations
on the tongue. Neurologic exam revealed no focal deficits, but motor retardation and easy fatigability with ambulation. Laboratory studies demonstrated creatine kinase
(CK) of 5,110 u/L [21-215] and CK-MB of 95 ng/mL
[0.6-3.6] with normal EKG, troponin, urinalysis, and
erythrocyte sedimentation rate (ESR). Thyroid stimulating hormone was 77.7 mcIU/mL [0.358-3.8] and free T4
was 0.25 ng/dL [0.76-1.46]. The clinical picture was consistent with hypothyroid myopathy secondary to iodine
toxicity from chronic ingestion of Lugol’s solution.
Lugol’s solution is a 130 mg/ml blend of elemental iodine and potassium iodide used to treat thyroid storm,
with historical use in severe hypothyroidism. Ingesting
supratherapeutic doses of iodine in the setting of autoimmune thyroiditis caused prolonged, severe hypothyroidism via the Wolff-Chaikoff effect. Hypersalivation and
acneiform skin eruptions are signs of iodine toxicity. Patients are treated with thyroid hormone supplementation
and withdrawal of iodine-containing agents. This case
highlights the importance of careful history-taking and
awareness of the toxicities and possible adverse events
associated with supplements and traditional remedies.
Löfgren Syndrome: A 41 Year-Old Hispanic Man
with Acute Sarcoidosis
Donald Phillips, MD; Frank Degregorio, MD; Thomas Zung, MD; Leland Powell, MD, PhD
Olive View-UCLA Medical Center
Most physicians in the United States know sarcoidosis as
a chronic, often disseminated disease with long-term disability. However, an acute form of sarcoidosis, most
prevalent in Northern Europe, often permanently remits
with no lasting morbidity. Its relative scarcity in the US
makes Löfgren Syndrome a diagnostic challenge.
55
A previously healthy 41 year-old Hispanic man presented
to a community clinic with fevers, chills, a painful, symmetric polyarthritis first appearing in the ankles, and erythema nodosum. He was found to have an elevated ESR
and was prescribed a seven-day steroid taper, which temporarily improved his symptoms. A few weeks later he
presented to the emergency department with similar
symptoms, plus increasing fatigue, malaise, night sweats,
and a thirty-pound unintentional weight loss. His ESR
remained elevated, but autoimmune serologies were
negative. He had a normal white blood count with a normal differential. A chest x-ray revealed symmetric hilar
lymphadenopathy. He had no respiratory complaints, a
normal ACE level, a non-reactive PPD, and negative coccidioidomycosis antibody titers. A CT scan revealed
enlarged and confluent mediastinal, hilar, celiac, and
porta hepatic lymph nodes, concerning for lymphoma. He
received an extensive work-up, including bronchoalveolar lavage negative for signs of infection or neoplasic
cells, as well as two non-diagnostic trans-bronchial biopsies. Finally, pathology obtained from mediastinoscopy
revealed non-caseating granulomatous
inflammation consistent with sarcoidosis. When rechecked, his ACE level was high. The patient has since
recovered, his chest radiograph normalized, and is currently asymptomatic without further treatment.
This case illustrates the classic presentation of Löfgren
Syndrome, typified by an acute onset, a symmetric polyarthritis involving the ankles, erythema nodosum, and
hilar adenopathy. The diagnosis was made difficult, however, by geography. Löfgren Syndrome patients in Northern Europe are often diagnosed clinically, with no biopsy
needed to provide histologic evidence. The lesson of this
case is that Löfgren Syndrome occurs in the United States
probably more often than currently recognized, and
prompt recognition of this variant may spare patients
from an invasive work-up and the psychological stress of
possibly having cancer. Lastly, understanding the usual
course of Löfgren Syndrome may prevent unnecessary
treatment given its favorable prognosis.
A Case of Solitary Fibrous Tumor of the Pleura
Shahriar Pirouz, MD; Alison Kole, MD; Nader Kamangar, MD, FCCP
Olive View-UCLA Medical Center
Case: A 61 year-old white male presented to the ER with
complaints of lower extremity swelling and shortness of
breath. His symptoms began 4 months prior to admission,
when he noticed a gradual decrease in appetite, 24 lb
weight loss, chronic cough, progressive weakness, and
fatigue. He also noted a palpable mass in his right lower
quadrant, with subsequent right lower extremity swelling,
which gradually progressed bilaterally. Review of systems was notable for worsening constipation, though otherwise was negative. Patient had history of lumbar spine
osteosarcoma with resection at the age of 19. On exam,
the patient was afebrile; heart rate 92; blood pressure
143/82; respiratory rate of 18, with 98% oxygen saturation on room air. He was cachectic, speaking in full sentences. Notable physical exam findings include decreased
breath sounds over the right lung with increased tactile
fremitus and increased dullness to percussion; a firm right
upper quadrant without hepatosplenomegaly; and bilateral pitting edema. Chest x-ray revealed subtotal opacification of the right hemithorax with contralateral mediastinal shift. CT Chest with contrast revealed a 20.6 cm x
20cm large heterogeneous noncalcified and noncavitary
mass abutting the chest wall without invasion. The patient underwent a percutaneous CT guided needle biopsy
of the mass. Pathology revealed a CD34 positive, S-100
negative spindle cell lesion, consistent with a solitary
fibrous tumor. The patient was referred for possible resection.
Discussion: Solitary fibrous tumors (SFTs) of the pleura
are rare neoplasms of mesenchymal origin. The initial
presentation typically is a large incidental asymptomatic
mass, with concomitant ipsilateral pleural effusion. Respiratory symptoms occur in approximately a third of patients and include cough, dyspnea, pleuritic chest pain,
and hemoptysis. Extrapulmonary symptoms include hypoglycemia and hypertrophic osteoarthropathy, thought
to be related to increased production of hyaluronic acid
by tumor cells. Additional manifestations include weight
loss, galactorrhea, and arthritic pain. Histologically, SFTs
are characterized by fibroblast-like cells and connective
tissue in varying proportions, often arranged in a "patternless pattern". Histochemically, SFTs are CD34 positive, and are keratin and S-100 negative. Benign SFTs are
curable with resection. Malignant SFTs may be curable
provided complete resection is possible. Local recurrence
is common (up to 63%), thus close follow-up of highrisks patients is recommended. There is little data on the
efficacy of chemotherapy or radiation in high-risk patients.
The Pulsating Sternal Mass
Shahriar Pirouz, MD; Jonathan Neyer, MD; Henry
Honda, MD
Olive View-UCLA Medical Center, UCLA Medical
Center
Case: Patient is a 59 year old male with h/o multiple cardiac surgeries who presented with a large protruding
mass at the superior aspect of his sternum. His cardiac
history includes VSD repair and aortic valvuloplasty as a
teenager. At age 53, he developed atrial fibrillation, severe mitral stenosis and underwent mitral valve replacement. He eventually developed congestive heart failure
with aortic insufficiency and underwent a porcine aortic
valve replacement and Bentall procedure in April 2009.
The patient did well for a month after the Bentall procedure but soon developed decreasing exercise tolerance
and night sweats, which were reminiscent of his symptoms at the time of his endocarditis episode. He was admitted and found to have coag-negative staph and bioprosthetic aortic valve endocarditis. While receiving anti-
56
biotics, he developed the enlarging pulsatile chest mass.
The mass proved to be a hematoma which was contiguous with a large anterior mediastinal and pericardial soft
tissue collection; it was the product of leakage from the
proximal and distal Bentall anastomosis sites. He was
treated with tight blood pressure control and serial aspiration of the mass. The patient has been referred for surgical repair of the leak.
Discussion: Periprosthetic leakage (PPL) is a frequent
cause of reoperation after aortic valve replacement
(AVR), occurring in 1% to 3.5% of patients after AVR.
This represents the second most frequent indication for
repeated surgery in this patient population, second only to
degeneration of biologic prosthetic valves. PPL occurs
due to incomplete apposition of the sewing ring to the
native tissue. Prior or active infection increases the risk of
PPL. Factors predisposing to PPL have not been fully
identified, although prosthetic endocarditis, Marfan syndrome, bicuspid aorta, and severely calcified aortic annulus may be predisposing factors. Detection has increased
as a result of improved techniques, particularly transesophageal echocardiography. Most PPLs are asymptomatic and benign. Clinically significant PPL’s may be
due to regurgitation, with reduced effective cardiac output, or congestive failure, resulting in dyspnea on excursion. Large PPL’s are generally associated with a murmur, though occasionally an eccentric jet may be relatively inaudible. PPL’s may lead to hemodynamic, hematologic, or other clinical problems. More than mild periprosthetic regurgitation, presence of hemolysis, and
decreased ejection fraction are standard indications for
surgical reintervention. Operative risk is higher than firsttime surgery with recent published reports indicating a
hospital mortality ranging from 5.4% to 16%.
Disseminated Cryptococcosis in a Renal Transplant
Patient
Donald J Portocarrero, DO; Juan C Barrio, MD; Byron K Williams, MD
White Memorial Medical Center
Case: A 75 year-old Hispanic male with a PMH of HTN,
DM II, ESRD s/p cadaver transplant presented with generalized weakness for 2-3 weeks. Patient had been on
Rapamune for 4 years, but due to recent papilloma virus
infection, it was switched to Leflunomide 6 months prior
to admission. Patient’s baseline creatinine was 1.5. The
patient admitted to decreased urine output. Review of
systems was essentially negative, including headache,
fever, chills, neck stiffness, chest pain, cough, shortness
of breath, purulent sputum. The patient was afebrile with
stable vital signs. He was awake and alert but disoriented.
There was no neck stiffness or photophobia. Cardiovascular, respiratory, and gastroenterological evaluations
were negative. Kernig’s and Brudzinski's signs were
negative. The patient had a left arm AV shunt with an
eschar distal to it. The neurological exam was nonfocal.
Initial labs revealed a mild leukocytosis of 11.9 with neutrophils of 89%, lymphocytes 6%, no bands. Chemistry
panel: CO2 level of 18, BUN of 129, and Cre of 2.6. CT
of the head was negative. The patient was admitted with
initial diagnoses of acute renal failure and rule out sepsis.
IV fluids and empiric antibiotics, featuring Vancomycin
and Zosyn, were started. The immunosuppressants were
discontinued. Blood Cultures were positive for Cryptococcus Neoformans on two sets and Amphotericin B was
started. Given the patient’s renal insufficiency, Amphotericin B was switched to its lipid formulation Abelcet on
Day 2. Flucytosine was held due to renal failure. The
initial cryptococcal serum antigen titer was 1:4096. Biopsies of the eschar distal to the AV shunt revealed Cryptococcus neoformans infection with extensive necrosis and
severe acute inflammatory reaction. Due to deteriorating
mental status, a brain MRI was performed. This was essentially negative. Lumbar puncture revealed yeast in the
spinal fluid consistent with Cryptococcus Neoformans
meningitis. The HIV screen was negative. The patient
had a hospital course complicated by NSTEMI, shock
liver, septic shock, and DIC. Supportive and aggressive
medical management saved the patient’s life. However,
his renal function never recovered, necessitating resumption of hemodyalisis. The patient was subsequently transferred to a skilled nursing facility. After 40 days of systemic Abelcet, the serum cryptococcal Ag titer was repeated and remained elevated at 1:4096.
Discussion: Mortality rates of Disseminated Cryptococcus are 70-80% in untreated patients compared to those
treated with antifungal medications. The Crypto A/D
Study concluded that initial dissemination, high serum
Ag titer (>1:512), and lack of flucytosine during induction therapy were associated with treatment failure at two
weeks independent of HIV status. This case not only
validates those results, but also further extends the time
limit of mycologic failure to greater than 5 weeks. Given
the patient’s poor renal condition, two important management decisions were made (1) Flucytosine was not
used for induction treatment; and (2) Abelcet was used
instead of regular Amphotericin B for treatment. Nevertheless, our patient’s clinical status remained poor and the
serum cryptococcal Ag titer was still elevated despite
medical treatment. One should consider flucytosine induction with or without lipid formulations of Amphotericin B regardless of renal function.
Transient Apical/Mid Left Ventricular Ballooning in
a Woman with Severe Asthma Exacerbation
Vinoy Prasad, MD; Kenneth Narahara, MD
Harbor-UCLA Medical Center
A 73-year-old Hispanic woman with a history of hyperthyroidism treated with propylthiouracil and moderate
persistent asthma, but no cardiac history, presented with
shortness of breath and diffuse wheezing concerning for
asthmatic exacerbation. She had frequent hospitalizations
and ED visits in the past for asthmatic exacerbations. She
had a non-productive cough and shortness of breath for 2
days requiring increasing usage of her rescue inhaler, but
denied any fever or chest pain and her initial cardiac
57
biomarkers were negative. Symptoms improved with
nebulizers, and she was admitted for steroid treatment
and observation. Overnight, she acutely decompensated
and was noted to be in severe respiratory distress, appearing cyanotic with increased accessory muscle use and
pursed lip breathing, with an arterial blood gas supporting
the diagnosis of hypercarbic respiratory failure. The patient subsequently had substernal, squeezing chest pain.
Cardiac biomarkers drawn showed an elevated troponin-I
of 4.54 with a creatine kinase MB fraction of 21.2; thyroid hormone levels were normal. ECG showed Q-waves
and ST-elevations in leads V2-V6 concerning for acute
anterolateral infarction. She was emergently taken for
cardiac catheterization, where she was noted to have angiographically normal epicardial coronary arteries. A 2-D
echocardiogram performed thereafter demonstrated a
depressed LV EF of 30-35% with an akinetic apical wall
and hypokinetic mid-septal, anterior-inferior, and lateral
walls. All basal segments contracted normally. The patient was treated supportively, and her cardiac biomarkers
trended down after a troponin-I peak of 9.45. A follow-up
2-D echocardiogram 11 weeks later showed normal LV
size and function (EF 65-70%) and no regional wall motion abnormalities.
Transient apical/mid-ventricular ballooning, also known
as “Takotsubo cardiomyopathy,” is a syndrome which
mimics acute myocardial infarction, but without angiographic evidence of coronary artery stenosis. For unclear reasons, post-menopausal women, such as our patient, are predominantly affected. Wall motion abnormalities typically involve the apical one-half to two-thirds of
the left ventricle, with sparing or hypercontractility of
basal segments. Echocardiographic and ECG findings are
characteristically out of proportion to the degree of elevation of cardiac biomarkers. This transient cardiomyopathy is thought to be triggered by intense emotional or
physical stress, possibly through the release of supraphysiologic levels of plasma catecholamines and stressrelated neuropeptides resulting in direct myocyte injury
or coronary arterial or microvascular spasm. Supportive
therapy and removal of the stress resolves the cardiac
dysfunction, as it did with our patient. This case study
illustrates an increasingly reported but peculiar transient
cardiomyopathy, induced by the stress of a severe asthmatic exacerbation requiring intubation.
Acute Changes in Renal Function in Severe Pulmonary Hypertension
Reza Rafie, MD; Ali Farvid, MD; Ashkan Attaran,
MD; Nima Taha, MD;
Sharmineh Shams, MD; Mehdi Manzari, MD;
Mohammad Pashmforoush, MD
University of Southern California
Introduction: Pulmonary hypertension affects right ventricular function that may cause venous congestion. Previous studies showed the effect of venous congestion on
glomerular filtration in patients with heart failure. In this
case report we present a patient with severe pulmonary
hypertension that had elevated creatinin on admission.
Her creatinin decreased to baseline with sildenafil and
lasix that was resumed to treat her elevated pulmonary
pressure
Case: 32 year-old woman with history of idiopathic pulmonary hypertension since 2 years ago. She was admitted
with shortness of breath after running out of her medications. She was diagnosed initially with elevated pulmonary pressure by transthoracic echocardiography that was
confirmed later by catheterization. She had ventilationperfusion scan that showed normal results. She was
started on sildenafil, lasix and home oxygen. She ran out
of her medication 2 months prior to admission. She had
progressive shortness of breath associated with swelling
in her lower extremities. Her vital sign on admission
showed blood pressure 98/60 mmHg, Heart rate 93 bpm,
respiratory rate 26 per minute with oxygen saturation
98% on 6 liter oxygen. Her cardiac exam showed 3/6
systolic murmur in left sternal border, 3/6 diastolic murmur in pulmonic site and loud P2. She was started on
lasix and sildenafil. Her creatinin on admission was 2.3
mg/dl that increased from her baseline values 0.7 mg/dl.
She denied previous history of kidney disease. She had
work up for her acute kidney dysfunction that all results
came back normal. Meanwhile she had transthoracic
echocardiography that showed severely reduced right
ventricular systolic pressure with estimated pressure at
110-120 mmHg. Estimated pulmonary artery pressure
was at least 40 mmHg and right atrial pressure was 15-20
mmHg. There was severe pulmonary and tricuspid regurgitation. She was continued on sildenafil and low dose of
lasix. Her symptoms improved after 2 days. Her creatinin
gradually declined and after 3 days returned to her baseline level. She was a candidate for combination therapy
with bosentan and sildenafil due to her severe pulmonary
hypertension.
Discussion: Previous studies showed patients with advanced heart failure have increased incidence of concurrent renal function impairment but low cardiac output
alone could not explain the deterioration in renal function. Recent study that published on May 2009 showed
that venous congestion is the most important factor in
worsening renal function in heart failure patients. In this
case report, for the first time we present a patient with
severe pulmonary hypertension with normal left ventricular ejection fraction and severely impaired right ventricular function that her acute renal dysfunction improved
after treatment with sildenafil and lasix. Future studies
can show the beneficial effect of decreasing venous congestion on renal function in severe pulmonary hypertension.
Electric Dissociation within Left Atrial Appendage
Reza Rafie, MD, David Cesario MD,PhD, Tasneem Z
Naqvi, MD
University of Southern California
58
Background: Electrical cardioversion is one of the modalities that has been used to treat atrial fibrillation. Before proceeding to cardioversion , transesophageal echocardiography (TEE) is performed to rule out thrombus
within the left atrial appendage. We describe a patient
who was admitted for cardioversion with underlying refractory atrial fibrillation, who was found during TEE to
have an accessory lobe within the left atrial appendage
that was electrically dissociated from the remaining of the
body of left atrial appendage and left and right atria.
Case: An 79-year-old man with history of severe mitral
regurgitation, atrial fibrillation was admitted with refractory atrial fibrillation for cardioversion. He had history of
mitral valve and tricuspid valve repair and Maze procedure on 2008. 3 months later he developed transient complete heart block that was recognized incidentally during
his admission for a right groin abscess. He underwent
dual chamber pacemaker placement. He was admitted on
May 2009 to have ablation for persistent atrial arrhythmias. He underwent TEE that showed an elongated accessory lobe within left atrial appendage. During evaluation by Doppler imaging the accessory lobe was found to
be in sinus rhythm that was electrically dissociated from
the body of left atrial appendage, left atria and right atria
with atrial fibrillation rhythm. He subsequently underwent electrophysiology study to have ablation. Activation
map and bioscience intracardiac 3 dimensional mapping
confirmed electrical dissociation data obtained by echocardiography.
Discussion: The accessory lobule of left atrial appendage
has been recognized if it’s length exceeds than that of the
entrance lumen. Previous studies have reported coexistence of sinus rhythm and atrial fibrillation after Maze
procedure however the lines of incision used during Maze
procedure in our patient, do not anatomically explain the
electric dissociation within the left atrial appendage. Several case reports previously described the electrical dissociation between the atrial appendage and rest of the body
of ipsilateral arium but to our best knowledge there is
only one description on electric dissociation within the
left atrial appendage previously reported by our group. In
that case report, Doppler echocardiography showed sinus
rhythm within the main body of left atrial appendage
whereas left atrial appendage accessory lobe had rapid
atrial waveforms of atrial fibrillation. Our current patient
had normal sinus rhythm in left atrial appendage accessory lobe on Doppler echocardiography while the main
body of left atrial appendage as well as the left and right
atria were in atrial fibrillation. Our TEE findings were
confirmed during electrophysiologic study. These results
emphasize the need to carefully evaluate the left atrium
and its appendage by Doppler echocardiography. Atrial
dissociation phenomenon may also explain occurrence of
appendage thrombi with or without stroke in some patients in whom surface ECG may show sinus rhythm.
Fentanyl Induced Atrioventricular Nodal Block
Reza Rafie, MD; Ashkan Attaran, MD; Sharmineh
Shams, MD; Ali Farvid, MD;
Nima Taha, MD; Mehdi Manzari, MD; Mohammad
Pashmforoush, MD
University of Southern California
Background: Several studies in dogs showed the effect
of fentanyl on prolongation of sinoatrial and atrioventricular conduction time. There are several case reports
about the atrioventricular nodal block by fentanylpropofol combination that are used for anesthesia. In this
case report, we describe a patient with unknown cause of
AV nodal block that fully recovered after stopping fentanyl.
Case: 51 year-old man with history of intravenous drug
abuse, bilateral upper extremity cellulites, infective endocarditis and was admitted with falling episode on the day
of admission. In emergency room, he had CT scan of the
neck that showed no fracture but there was possible
phlegma and discitis between C2-C7 so his C-collar was
kept in place. He subsequently developed respiratory
distress and was intubated for airway protection with
possible diagnosis of acute respiratory distress. He was
transferred to medical intensive care unit. He had bilateral upper extremity cellulites and 1/6 systolic murmur at
cardiac apex. His initial EKG showed sinus tachycardia.
Result of his blood culture was positive for oxacillin resistant staphylococcus aureus. He was started on empiric
antiobiotic treatment with vancomycin. He underwent
transthoracic echocardiography that showed thickened
mitral leaflet with possible healed endocarditis. A vegetation on mitral valve could not be excluded. It was recommended to have transesophageal echocardiography(TEE) to rule out vegetation on mitral valve but TEE
could not be performed at that point due to cervical spine
phlegma and the high risk for complication with removing C-collar. On day three of admission, he suddenly developed 2 episodes of atriventricular nodal block that
each last 5 seconds. External pacemaker was placed. He
was intubated and was treated with fentanyl at that time
for sedation. Fentanyl was considered as the main culprit,
although there was concern about suspected endocarditis
and its complications. He was considered high risk for
possible cardiac operation. His fentanyl was discontinued
and his heart rhythm returned to normal. He never had
repeated episode of atrioventricular nodal block. His condition improved and he was extubated successfully. His
TEE after temporary removing his C-collar showed no
vegetation on mitral, aortic and tricuspid valves.
Conclusion: Fentanyl can cause prolongation of cycle
length and antegrade effective refractory periods of the
A-V node and ventricles in dogs. These effects can be due
to vagus nerve stimulation or the direct effect on sinoatrial and atrioventricular (AV) nodes. Although several previous case reports showed the AV nodal blocking
effect by combination of fentanyl with other medications
such as propofol, in the patient that we described here it
appears that the sole effect of fentanyl caused reversible
AV block. With extensive use of fentanyl in intensive
care setting, its cardiac effect should be evaluated more
carefully.
59
Massive Right Sided Cardiac Thrombus in Patient
with
Dilated
Cardiomyopathy
and
Hyperhomocysteinemia
Reza Rafie, MD; Ashkan Attaran, MD; Sharmineh
Shams, MD; Ali Farvid, MD;
Nima Taha, MD; Mehdi Manzari, MD; Mohammad
Pashmforoush, MD
University of Southern California
Introduction: Homocysteine has been recognized as a
risk factor for arterial and venous thrombotic events. Previous studies showed the association of hyperhomocysteinemia with intracardiac thrombus especially left atrial
thrombus in stroke patients. In this case report we describe a patient with elevated homocystein level and
ischemic cardiomyopathy that was admitted with massive
thrombus in right side of the heart expanding from inferior vena cava entrance to right ventricle.
Case: 68 year-old woman with history of myocardial
infarction, was admitted with acute onset shortness of
breath and exacerbation of peripheral edema. Before admission to emergency room, she developed pressure like
chest pain, lasting 30 minutes that was relieved by nitroglycerin given in emergency room. Her vital signs were
stable on admission. On physical exam she had crackles
in lower part of both lungs. Her cardiac exam showed
regular rhythm with 2/6 systolic murmur in xephoid area.
Her ECG showed sinus rhythm with Q waves in leads II,
III, AVF and V4-V6 but without ST-T changes. She had
normal troponin levels. She underwent transthorasic
echocardiography that showed dilated cardiomyopathy
with severely reduced left ventricular function and elevated right ventricular pressure. There was a mass in right
atrium that appeared to be attached at the level of Eustachian valve, between the right atrium and inferior vena
cava. This mass prolapsed into the right atrium, right ventricle, and inferior vena cava. The largest diameter of
mass was 5.8 cm. She also had CTPA that showed
thrombus in right and left pulmonary arteries, she was
started on heparin. She also had cardiac catheterization
that showed severe 3 vessel disease. Results of her anticoagulation work up only revealed elevated homocysteine level ( 20.6 µmol/L with normal level < 11.5
µmol/L). She was considered a poor surgical candidate
due to her physical condition at that point. She was
treated with lovenox with close follow up in cardiothoracic surgery to have CABG after improve her general
condition.
Discussion: Homocysteine level has an established effect
on coronary thrombotic events but there is limited data
about the effect of hyperhomocysteinemia on intracardiac
thrombi formation. Several studies also described large
intracardiac thrombi in patients with dilated cardiomyopathy. The patient that we described in this case report had huge intracardiac thrombi in right atrium and
right ventricle. Her underlying dilated cardiomyopathy
predisposed her to have thrombus formation in her heart,
but the additive role of elevated homocysteine level in
thrombi formation in right side of heart needs more
evaluation. In patients with dilated cardiomyopathy, the
intracardiac thrombus formation in the context of hyperhomocysteinemia can be a target for treatment in the future.
Possible Faces of Patent Foramen Ovale (PFO)
Reza Rafie, MD; Samuel Daneshvar, MD; Tasneem
Naqvi, MD
University of Southern California
Introduction: In approximately 27% of the population,
the communication that provides oxygenated placental
blood flow to the fetus remains patent as a patent foramen
ovale (PFO). In this report we describe unusual manifestations of PFO.
Case 1: A 59 year-old man with history of diabetes presented with progressive weakness in his upper and lower
extremities that started 1 week prior to admission. He was
bed bound since admission. MRI of the spine showed a
large expansile intramedullary lesion from C3 to C7. He
underwent midline myelotomy at the level of C3-C4 and
C6-C7 levels and also found to have an epidural abscess
with pus. Culture was positive for streptococcus viridians. He had a transesophageal echocardiography(TEE)
that showed a 4 mm PFO. He completed the antibiotic
treatment after the surgery and was transferred to rehabilitation center to consider PFO closure after improvement in his medical condition.
Case 2: A 35-year-old man with h/o severe scoliosis
since birth and frequent migraine HA complained of severe HA and slurred speech 1 month prior to admission.
MRI of brain at the time was normal and MRI of the
spine showed rotoscoliosis. He underwent transcranial
Doppler that was (+) for bubbles. He was referred for
further evaluation. Before admission he developed another episode of severe HA with transient R-sided weakness. TEE showed moderate right to left shunting, which
was exacerbated by body habitus with extrinsic compression of right atrium by the liver. Resting hypoxemia of
87% on room air was noted during TEE. Pulmonary artery pressure was normal. The patient underwent percutaneous closure of the PFO. At 2 months follow-up after
PFO closure, he denied further TIA and also mentioned
improved HA.
Discussion: Our first patient describes a patient with
cryptogenic spinal abscess in association with PFO. Multiple case reports describe the association between cryptogenic cerebral abscess with PFO but to the best of our
knowledge there is only one case report within a case
series that describes spinal abscess in a patient with underlying PFO. Howevert in that case there was no evidence of right-to-left shunting despite Valsalva maneuver
and cough. In patients with possible cardiac source of
septic emboli, PFO should be evaluated as a potential
source of cardioembolic disease.
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Second patient presented with a history of multiple abnormalities of the spine, migraine headaches, and TIA. In
this patient, abnormalities of the spine caused right atrial
compression by the right hemidiaphragm. This in turn
resulted in opening up of a normally closed PFO channel
resulting right-to-left shunting across PFO. Cardiac and
spinal anomalies are both part of the VACTERL syndrome. Our report describes occurrence of hypoxemia at
rest and of right to left shunting across PFO resulting
from extrinsic right atrial compression from an elevated
diaphgram. More studies are required to evaluate the PFO
incidence in patients with other congenital anomalies
including congenital anomalies of the spine.
HaNDL with Care
Santhi Raja, MD, FJFICM; Jasminka Criley, MD,
FACP, FHM;
Ali Khoiny, MD; Chester Choi, MD, MACP
St. Mary Medical Center
Introduction: Pseudomigraine or HaNDL (Headache
with transient Neurologic Deficits and Lymphocytic
pleocytosis) is a self limited syndrome of unclear etiology. The clinical picture includes episodes of severe bilateral headaches, often without meningismus, accompanied by transient neurological deficits. CSF shows a lymphocytic pleocytosis with negative etiological results and
neuroradiological studies are normal.
Case History: This 26-year-old Hispanic male, previously in good health, presented with recurrent episodes of
headaches associated with transient severely altered mentation. His neurological exam was otherwise normal. His
hemogram, serum chemistry, toxicology screen and chest
x-ray were normal. He had a total of 6 episodes in a span
of 8 weeks and 2 of the episodes were associated with
severe agitation. All episodes were associated with CSF
lymphocytosis (WBC’s of 166 to318), elevated protein
but normal glucose and negative etiological results. Repeated neuroimaging was also normal. He was asymptomatic between the episodes. Each episode lasted for 2-6
days.
Encephalitic
antibody
panel,
CSF
VDRL,cryptococcal antigen,cocci antibody and India ink
preparation were all-negative. CSF culture was negative
for fungi, bacteria including TB. CSF PCRs for HSV,
CMV, HIV, and AFB were also negative. Antibodies for
toxoplasmosis, leptospirosis, and borreliosis were all
negative in CSF. Autoimmune work up was negative.
The patient was treated empirically with antibiotics and
acyclovir in the initial 2 admissions and improved rapidly
each time with discharge to home within 4-6 days of admission.
Discussion: Our patient appears to have had Pseudomigraine or HaNDL, which is defined by the International
Headache Society as a self-limited syndrome of unknown
origin characterized by recurrent severe bilateral headache accompanied by transient neurologic symptoms and
CSF lymphocytosis. These patients are usually between
15 and 40 years of age. It is more frequent in men. Lym-
phocytic pleocytosis (10 to 760 cells mm3) and increased
CSF protein are found with negative microbiological and
immunologic studies. CT scan and MRI are normal, but
an electroencephalogram frequently shows focal slowing.
It is possible that pseudomigraine could result from an
activation of the immune system secondary to a recent
viral infection, which would produce antibodies against
neuronal or vascular antigens.
This syndrome is not well known as a cause of recurrent
aseptic meningitis and can be confused with Mollaret’s
syndrome. The later usually recurs over a period of years
while Pseudomigraine is self-limited over a period of few
weeks to months and no residual sequlae, although the
neurological deficits such as sensory symptoms (78%),
aphasia (66%) and motor deficits (56%) may be quite
marked. Acute confusional state and agitation are less
common, but have been previously described. Recognizing this syndrome may help to avoid repetitive investigations, offer a better prognosis, and eventually lead to an
improved understanding of the etiology and pathophysiology involved.
From 'Black & White' to Red All Over - How a
Straightforward Case of Streptococcal Pharyngitis
Evolved Into Guttate Psoriasis
Sangeetha Reddy, BA; Jason Napolitano, MD
University of California, Los Angeles
Case: A 32 year old female presented to the emergency
department with a painful generalized erythematous rash,
arthralgias, and low grade fever of 100 F. Eight weeks
prior, patient was treated for streptococcal pharyngitis
with 10 day course of amoxicillin, of which she completed 3 days. Two days after stopping antibiotics, she
developed an erythematous rash in her intertriginous areas. Patient began treatment with topical antifungals with
no improvement. Over the next several weeks, the rash
worsened with development of bilateral arthralgias of her
knees and ankles. Four days prior to presentation, the
rash spread diffusely to her chest, face, and extremities.
Physical exam was notable for multiple well defined erythematous papules, with central scaling, on her entire
body, with palmoplantar sparing. The papules coalesced
into large plaques on the face and chest. Laboratory investigations were significant for white count of 10.3, ESR
of 53, and ASO titer of 541. Of note, HIV antibodies and
pharyngeal swab were negative. A punch biopsy of the
skin was consistent with psoriasis. Treatment with topical
steroids and systemic therapy with infliximab was initiated, and the rash improved.
Discussion: Streptococcal pharyngitis has been known to
cause several post-infectious skin syndromes. This case
illustrates that in addition to the more commonly considered scarlet fever, antibiotic drug reactions, rheumatic
fever associated erythema marginatum, and TSS, it is
important to keep psoriasis on our differential. Approximately two thirds of first presentations of guttate psoriasis are preceded by streptococcal pharyngitis. One pro-
61
posed mechanism suggests that streptococcus superantigens induce the expression of skin homing receptors in T
cells, which then migrate to the skin and cause the pathological characteristics of psoriasis. Guttate psoriasis can
resolve on its own, beginning several weeks to months
after the rash begins. Otherwise, treatment is similar to
other types of psoriasis, which can include topical steroids, UV therapy, and systemic anti-inflammatory medications. Antibiotic treatment for the rash is not recommended based on randomized controlled trials. Several
case reports have shown improvement of skin lesions
after tonsillectomy, but this has not yet been studied in
trials. In addition to streptococcus, other infectious triggers of psoriasis include HIV, candida, malassezia, and
staph aureus.
The Disease of Kings…With a Twist
Eugene Richardson, MD
Stanford University Medical Center
Case: A 62 year-old obese Tongan male with a history of
type II diabetes, hypertension, and gout presented to the
emergency department with two days of severe left knee
swelling and pain. He was admitted to the short stay unit
with the diagnosis of gouty arthritis and was given fluids,
prednisone, and hydromorphone. The patient continued to
require high doses of hydromorphone, and medicine was
consulted. A joint aspiration was performed which demonstrated a neutrophil count of 52,000, negatively birefringent crystals, and gram-positive cocci growing in
pairs and chains. Blood cultures drawn previously in the
ED demonstrated similar gram-positive organisms which
were later speciated as group B streptococcus (Streptococcus agalactiae). The patient was started on 5 million
units penicillin IV q4hours (he weighed 325 pounds) and
was taken for surgical irrigation and debridement. His
pain improved considerably after the operation requiring
only infrequent Percocet. The patient was discharged to a
skilled nursing facility for four weeks of IV antibiotics
via PICC as well as physical therapy. Follow-up blood
cultures performed weekly were negative for growth.
Discussion: Gout was known throughout history as the
“disease of kings” due to its association with overindulgence in food and wine. Our patient was a pastor and
shunned alcohol, but did report a hearty dinner at the local Chinese restaurant the night before onset of symptoms. What complicated this case was the concomitant
joint infection and bacteremia. A retrospective case series
of 265 patients found the incidence of simultaneous gout
and septic arthritis to be 1.5%. One theory, known as
“crystal shedding and strip mining,” posits that inflammation induced by infection releases crystals from the synovial membrane. Alternatively, gouty erosion may provide
a substrate for bacterial seeding. GBS is an uncommon
cause of septic arthritis, causing 2.9% of cases in one
series. The incidence of invasive disease in non-pregnant
adults is on the rise, however, with reports of a two to
four-fold increase over the past two decades. Clinicians
should thus be aware of this trend as well as the associa-
tion invasive GBS with serious underlying conditions—
namely diabetes, malignancy, neurologic disease, and
cirrhosis.
Postpartum Diabetes Insipidus: An Interesting Path
to a Rare Diagnosis
Lisa Riley, MS4, DMA; Nardine Saad, MD; Hilary K.
Seligman, MD, MAS
University of California, San Francisco
Case: A 31-year-old woman presented with 4 days of
worsening thirst and increased urination 7 months after
normal delivery of a healthy baby girl. Her symptoms
began acutely during a cross-country airplane flight 5
weeks prior to her presentation and had progressively
worsened, particularly over the previous 4 days. She reported daily fluid intake of about 4 gallons, associated
with hourly urination during the day and frequent nocturia. Her review of systems was significant for increased
fatigue and a mild, diffuse headache that was partially
relieved by acetaminophen. She continued to exclusively
breastfeed her infant without a noticeable change in her
milk supply. The infant’s growth and development were
reportedly normal.
The patient’s VS and PE were normal. Serum sodium
(141mmol/L), Ca (9.4 mg/dL), Cr (0.72mg/dL), and osmolality (288mOsm/ kg) were normal. Her urinalysis
showed a specific gravity of 1.002, sodium of 28mmol/L,
potassium of 17mmol/L, chloride of 32mmol/L, and osmolality of 124mOsm/kg. A urine pregnancy test was
negative.
With 24 hours of water deprivation, her urine specific
gravity increased from 1.002 to 1.021 and urine osmolality increased from 124 to 664mOsm/kg. Her serum
osmolality and serum sodium remained steady. Two
hours after intranasal desmopressin (dDAVP) was administered, her urine osmolality increased to 842mOsm/kg
without any change in her serum osmolality, indicating a
partial central diabetes insipidus. Over the subsequent
weeks, dDAVP was titrated to achieve relief of her thirst
symptoms.
Discussion: Diabetes insipidus (DI) presents with
polydipsia and polyuria. The source of DI (central or
nephrogenic) can be assessed by responses to a water
deprivation test and a dDAVP challenge. Central DI is
caused by inadequate anti-diuretic hormone (ADH) production by the pituitary gland. Generally in central DI,
urine osmolality increases somewhat with water deprivation, but increases dramatically with dDAVP, a synthetic
analogue of ADH. In nephrogenic DI, ADH production is
intact but kidney response to ADH is impaired. Therefore, urine osmolality increases only mildly (if at all) with
both water deprivation and dDAVP administration.
With water deprivation, our patient's urine osmolality
rose from 124 to 664mOsm/kg, considered a submaximal
response. After dDAVP, the patient's urine osmolality
62
rose significantly higher, from 664 to 842. This 27% increase confirms that the patient was producing inadequate
ADH to maintain homeostasis. This submaximal increase
indicates a partial central DI, as opposed to a complete
central DI which would show an increase of 100% or
more.
Springing a Leak: Pancreatic Duct Disruption from
Acute Pancreatitis
Michelle Rios, MD
University of California, Los Angeles
Case: A 34-year-old man with a history of alcohol dependence presented to the emergency room with complaints of epigastric abdominal pain and increased abdominal girth over the previous two months. Physical
examination was notable for significant cachexia, abdominal distention with shifting dullness, and dullness to
percussion over the right lung field. He had no stigmata
of chronic liver disease. Laboratory studies revealed an
elevated serum lipase. His serum albumin was low, but
coagulation studies and transaminase levels were within
normal limits. A CT of the abdomen showed massive
ascites, peripancreatic fluid collections, and fat stranding
consistent with acute pancreatitis. Chest imaging showed
a large right pleural effusion. Thoracentesis and paracentesis were performed, and pleural fluid analysis was consistent with an exudate with an elevated amylase level.
Ascitic fluid analysis revealed a low serum-albumin ascites gradient, elevated total protein, and elevated amylase level. After therapeutic paracentesis and thoracentesis, the fluid rapidly re-accumulated despite treatment
with fluid restriction, sodium restriction, furosemide, and
octreotide. ERCP showed extravasation of contrast into a
three-centimeter cystic structure in the pancreatic head. A
plastic stent was placed in the area of extravasation. Dietary restrictions were continued along with furosemide
and octreotide. After repeat paracentesis the patient did
not re-accumulate fluid and was discharged from the hospital.
Discussion: This case illustrates the need for a thorough
evaluation of ascitic fluid, particularly on the initial presentation of ascites. Given this patient’s drinking history a
preliminary diagnosis of cirrhotic ascites could have been
entertained. The lack of evidence of severe liver disease
and the presence of a low serum-albumin ascites gradient
warranted further investigation, ultimately revealing a
pancreatic source. As pancreatic ascites does not often
respond to medical therapy alone, it is important to diagnose to allow appropriate endoscopic or surgical intervention.
A Fine Balance
Kristen Rogers, MD; Payam Afshar, MD
Scripps Green Hospital
Case Presentation
A 53-year-old male with cirrhosis secondary to HCV-1
and known esophageal varices presented with a one week
history of intermittent cramping pain in the lower abdomen. The pain increased from mild to severe one day
prior to admission. Associated factors included loose
stools for three days and an isolated fever of 102.2 one
week prior to admission. Physical exam revealed diffuse
abdominal tenderness to palpation and was otherwise
normal. CT scan of the abdomen and pelvis with IV contrast demonstrated splenomegaly, varices, superior mesenteric vein (SMV) filling defect and mesenteric stranding. MRI with MRA/V revealed a patent left portal and
splenic vein, SMV thrombus with extension into the main
and right portal vein, and subtotal thrombus of the SMV
branches. Of note, the patient had an ultrasound of the
abdomen one month prior to admission positive for portal
hypertension but with no evidence of portal vein thrombosis. The patient’s Activated Protein C Resistance/ Factor V Leiden ratio was abnormal at 1.2 suggesting Factor
V Leiden mutation. The patient was discharged home on
Lovenox and Coumadin.
Discussion:
Mesenteric ischemia is a relatively rare disorder characterized by decreased intestinal blood flow resulting in
ischemia and subsequent damage. Early recognition is
imperative secondary to its high morbidity and mortality
rate. The most common presenting symptom is severe
abdominal pain out of proportion to physical exam findings. Associated symptoms include nausea and vomiting
(50%), diarrhea (50%), and melena or hematochezia
(15%). Mesenteric ischemia can be caused by an arterial
or venous etiology and can be either hemodynamically
occlusive or non-occlusive. Approximately 5-10% of
mesenteric ischemia cases are due to mesenteric vein
thrombosis of which 80% has an identifiable cause.
Known risk factors include inherited and acquired hypercoagulable states, infection, inflammation, portal hypertension and trauma. 75% of patients with SMV thrombosis have an underlying hypercoagulable state. Hence, all
patients with SMV thrombosis should be tested for a hypercoagulable state even if they have another known risk
factor for SMV thrombosis. Our patient was found to
have Factor V Leiden mutation which is only found in
about 5% of the population. Factor V Leiden is a codominantly inherited disease due to substitution of
glutamine for arginine at position 506 in factor Va. This
position is necessary for Activated Protein C (APC) binding and subsequent Factor V degredation. However, the
binding site is altered by the mutation resulting in ongoing thrombin production and a hypercoagulable state.
Even though our patient has known portal hypertension,
his abdominal ultrasound immediately prior to presentation revealed a patent portal vein with no evidence of
thrombus. This suggests that the thrombus originated in
the SMV with extension to the portal vein. In comparison, a mesenteric thrombus secondary to portal hypertension typically originates in the portal vein and may extend to the SMV. It is important to distinguish that this
patient’s SMV thrombosis occurred as a probable independent cause from his portal hypertension because the
63
former requires anticoagulation but the latter does not
always. All patients with SMV thrombosis should receive
lifelong anticoagulation therapy in the presence of a hypercoagulable state unless contraindicated. Our patient
will need close follow-up and continual assessment of the
risk-to-benefit ratio regarding anticoagulation therapy in
the face of progressive liver disease.
son who has one or more of the following cardinal symptoms: (1) dermal macule or plaque with sensory loss, (2)
nerve enlargement, or (3) skin smear positive for acidfast bacilli. Although leprosy can have a protracted onset
and be difficult to recognize, cure is achievable with appropriate multidrug therapy.
A Clinic Presentation of Borderline Lepromatous
Leprosy
Radiation-Related Left Main Coronary Artery Disease and Subclavian Artery Stenosis in a Patient Previously Treated for Non-Hodgkin Lymphoma
Yasmin Sacro, MD; Stanley Amundson, MD
Scripps Mercy Hospital
Arash M. Saemi, MD; Dylan Wessman, MD
Naval Medical Center San Diego
Case: An 82-year-old Cuban male presented to a community clinic with complaints of a left wrist dermal
plaque and multiple maculopapular pruritic lesions on his
extremities present for approximately 3 months. Patient
also complained of longstanding right hand paresthesias
and weakness. Anti-histamine blockers provided mild
relief of pruritic symptoms, yet persistence of lesions and
paresthesias remained. Past medical history included
moderate-severe aortic insufficiency, coronary artery
disease, and chronic renal insufficiency. He had no new
medications. Patient had immigrated from Cuba 20 years
prior and had no return travel to his country of origin. He
had no recent foreign travel nor sexual activity. On
physical exam pertinent findings of the skin revealed a
left dorsal wrist 3 x 5 cm plaque with normal coloration
and central anesthesia, right extensor olecranon 1.2 x 1.3
cm plaque also with central anesthesia and ulceration, as
well as multiple maculopapular erythematous lesions
measuring 0.5-1cm scattered on bilateral posterior calves.
Neurological exam revealed an enlarged, palpable right
ulnar nerve and positive right Tinel’s sign. Atrophic muscle loss of the right thenar eminence as well as bilateral
intraosseous muscle atrophy were also noted. Deep tendon reflexes and sensation were notably decreased in the
distal extremities. Labs revealed lymphopenia with WBC
of 4.0 and 22% lymphocytes, and microcytic anemia with
hemoglobin 10.4 and MCV 78. Complete metabolic
panel, TSH, B12, glucose, folate and treponema antibody
were within normal limits. HIV was found to be negative
with a CD4 count of 270. Skin biopsy was performed
which revealed AFB positive smear (3+) with perineural
granuloma formation seen. Given the patient’s multiple,
asymmetric lesions with diminished sensation and many
AFB seen on skin biopsy, a diagnosis of borderline
lepromatous leprosy was made.
Case: A 38 year-old male was diagnosed with Stage IIB
NHL and treated with CHOP-R (Cyclophosphamide,
Doxorubicin, Vincristine, Prednisone, Rituxan) and mantle field irradiation. Four years later, he developed dyspnea on exertion and exercise intolerance. An exercise
treadmill test was positive for ischemia, and he underwent coronary angiography revealing severe stenosis of
the left main coronary artery. Initially, this was treated
percutaneously, but he twice developed in-stent restenosis and subsequently underwent 2-vessel CABG surgery.
Six months later, the patient developed recurrent anginal
symptoms, and an exercise stress echocardiogram was
concerning for ischemia of the anteroseptal wall. Angiography revealed patent bypass grafts and severe stenosis of the left subclavian artery, which was treated successfully by balloon angioplasty and an 8 x 18 mm stent.
The patient’s anginal symptoms resolved, and a repeat
exercise stress echocardiogram was negative for inducible ischemia.
Discussion: Hansen Disease, commonly known as leprosy, is uncommon in the U.S.and poses difficult diagnostic and treatment challenges. Leprosy has a range of
clinical manifestations which are determined by the
host’s cell-mediated immune response. The route of
transmission for leprosy remains unclear, but evidence
suggests respiratory transmission via droplets. It is also
one of the most common causes of peripheral neuropathy
in developing countries. Perineural granuloma formation
and nonspecific inflammatory infiltrate/fibrosis can result
in neuropathic symptoms. Leprosy is diagnosed in a per-
Discussion: Patients with lymphoma potentially can be
cured by combination chemotherapy and radiation therapy. However, long-term survivors are at increased risk
for developing cardiac and vascular disease from their
prior treatments. Radiation therapy to the chest can cause
accelerated atherosclerosis involving the ostial and
proximal segments of large arteries. We present a unique
case of a patient successfully treated for NHL with combination chemotherapy and mantle field radiation, who
subsequently developed both left main coronary artery
disease and left subclavian artery stenosis requiring percutaneous and surgical interventions. This case highlights
the increased risk among long-term lymphoma survivors
for accelerated atherosclerosis related to prior radiation
therapy, as well as the need for physicians to maintain a
high level of clinical suspicion whenever these patients
present with anginal symptoms.
Fulminant Liver Failure as Initial Presentation of Disseminated Strongyloides Hyperinfection in a HIVNegative Alcoholic Patient
Neha Sampath, MD; Steve Yung; Sally Stipho
Scripps Mercy Hospital
Parasitic infection with Strongyloides sp. is a rare and
unusual cause of liver failure in developed countries. If
64
left untreated or misdiagnosed, mortality with disseminated Strongyloides hyperinfection is almost 100%.
Timely diagnosis, is thus, of critical importance in suspected cases.
Case: 69 year old Laotian male with a history of noninsulin dependent diabetes, alcoholism and variceal
bleeding presented with acute onset of hematemesis and
melena. Patient was also found to be in fulminant hepatitic liver failure associated with a severe coagulopathy,
thought initially to be secondary to alcoholic liver disease. He also had an unusual rash on his torso and bilateral lower extremities. After the patient’s recurrent
variceal bleed had been stabilized with emergent endoscopic treatment and blood product transfusions, he continued to have non-bloody diarrhea and abdominal distention. He underwent imaging studies which revealed diffuse colonic wall thickening, severe circumferential gall
bladder wall thickening in absence of cholecystitis, and
severe liver heterogeniety indicative of innumerable
masses or microabscesses. He also had bilateral patchy
infiltrates on chest xray. Stool studies came back positive
for Strongyloides stercoralis larvae as well as Clostridium
difficile toxin. Lumbar puncture was performed and CSF
studies were negative for CNS involvement. Patient was
treated with Metronidazole and five day course of Ivermectin. His abdominal distention and diarrhea improved.
However, he had a complicated hospital course with respiratory failure requiring tracheostomy, and renal failure
requiring dialysis. He also had persistent direct hyperbilirubinemia, and fever concerning for cholangitis, for
which he underwent percutaneous cholecystostomy. The
procedure was complicated by hemoperitoneum. He was
eventually made comfort care by his family and passed
shortly thereafter.
Discussion: Disseminated strongyliadisis in a patient
without evidence of immunosuppresion is an unsual
cause of multi-organ failure, but should be considered in
a patient with risk factor such as previous residence in or
travel to endemic areas. No consensus exists on exact
duration of treatment with anti-parasitic medicines, and
further clinical trials are needed to determine this.
Canned Tuna: A Biological Weapon?
Pallabi Sanyal, MD; Kandan Baban, DO; Kenneth
Grudko, MD
Scripps Green Hospital
Introduction: An 85 year old retired fisherman with emphysema was admitted with 2 days of non-bloody emesis,
diplopia, bilateral ptosis, and orpharyngeal secretion
pooling. Extraocular movements were slow and he had
limited tongue protrusion. Strength and sensation were
otherwise grossly intact. On day 3, the patient's
ability to clear secretion worsened and he was transferred
to ICU for ventilatory support. On Day 4, the patient's 91
year old wife (pt#2) came to visit him, and was not feeling well. She awoke in the AM with profound weakness,
ataxia, dysarthria, diplopia, and dysphagia. Her physical
exam was essentially identical to her husband’s except
for 3/5 right upper extremity proximal strength.
Case: Pt#1's initial CBC and chemistry were normal.
Head CT and MRI/MRA did not reveal any evidence of
an acute stroke. Lumbar puncture showed 1 white cell, red cells, 31 mg/dl protein, and 99 mg/dl glucose. The
gram stain and the acid-fast bacilli stain were negative.
Nerve conduction study and electromyogram showed
non-specific mild slowing of motor and sensory velocities. Repetitive stimulation testing was not obtainable.
Acetylcholine receptor binding and blocking antibodies
were negative. Pt#2 also had a negative head Ct scan. A
niece accompanying pt#2 informed staff that the wife has
been canning food for years, particularly tuna.
Blood,stool cultures, serum and food samples from home
were collected from both patients, and were sent to the
Public Health Department for indentification of
botulinum spores and biotoxin assay. Given the high suspicion of botulism, with state approval, equine antitoxin
flown in from Los angeles was administered. Serum
samples from both patients returned positive for
botulinum toxin A; stool was negative. Pt#1 showed mild
improvement on repeat EMG days later. Pt#2 exhibited
mild improvement, but neither pt fully recovered.
Discussion: The clinical presentation of food borne botulism (eight strains) occurs within 12 to 36 hours of ingestion. Both patients in this case unfortunately presented
much later. Our differential initially included brainstem
stroke, myasthenia gravis, and guillain barre syndrome.
Arrival of pt#2 in addition to the added canning information solidified our diagnosis. The botulism toxin itself
binds to the presynaptic site, and causes irreversible damage to acetylcholine release. Plasticity of these sites requires at least six months. Treatment, in addition to supportive care, consists of an equine serum antitoxin. Duration of mechanical ventilation and decreased fatality have
been loosely associated with treatment. C.Botulism is the
deadliest bacterial toxin known and has been developed
and employed as a bio-weapon. It is important for physicians to be able to diagnosis and treat this rare form of
poisoning.
Lithium-Induced Thyroiditis: A Rare Case of Thyrotoxicosis
Maria Scremin, MD; Daniel Peng, MD; Anna
Milanesi, MD
Cedars-Sinai Medical Center
Lithium has been recognized for many years to have inhibitory effects on the thyroid, most commonly hypothyroidism and goiter. In the setting of lithium’s anti-thyroid
effects, lithium-associated thyrotoxicosis is unexpected
and critical to recognize given the potential to worsen
mania in bipolar patients. Rarely, lithium has been associated with thyroiditis, an inflammation of the thyroid
gland. In such cases, rather than a hypothyroid state, the
patient experiences high circulating levels of thyroid
hormone. A 23 year old man with bipolar disorder on
65
chronic lithium therapy was admitted to the hospital with
confusion, tachycardia, and acute mania. On physical
exam, patient was acutely manic, diaphoretic and tachycardic with heart rate of 130 bpm. There was no apparent
goiter or neck pain. Initial serum lithium levels were elevated well into the toxic range. Laboratory studies were
significant for a suppressed TSH and elevated T4. The
patient had no personal or family history of thyroid dysfunction. Radioactive uptake scan was virtually undetectable at less than 1%, consistent with a diagnosis of thyroiditis. Lithium was discontinued and the patient was
started on atenolol for his tachycardia. Three weeks later,
thyroid studies showed adjusted T4 levels trending
downward, while TSH remained suppressed at < 0.02.
Lithium levels decreased from 2.3 on presentation to below toxic range. The patient’s tachycardia and diaphoresis resolved with b-blocker therapy, although his mania
persisted. The patient was transferred to the psychiatric
ward where he continued to be treated for his manic state.
This case illustrates the potential for acute thyroiditis
causing thyrotoxicosis in a patient treated with lithium
carbonate. Almost exclusively, lithium causes hypothyroidism due to interference with synthesis and release of
thyroid hormone. Although hyperthyroidism is rarely
seen while on lithium, it is an important side effect to
consider given it’s potential to worsen or incite mania.
Correct diagnosis of the etiology for the thryrotoxicosis is
also vital, as the course and treatment of thyroiditis differs significantly from that of hyperthyroidism due to
more common causes such as Grave’s disease or toxic
nodular goiter.
Huge Benign Mass Leading to Shock
Roshni Shah, DO; Babak Eshaghian, MD, MS; Michael Hochman, MD; Babak Saedi, MD; Aarti
Chawla, DO; Justin Lee
University of Southern California
Case: A 52 year old G2P2 Hispanic female with significant history of menororrhagia presented with the complaint of abdominal pain, 1 year of increasing abdominal
mass, nausea, vomiting & dyspnea. PE was significant
for hypotension (97/61), tachycardia (131), tachypnea
(40), and a large, firm, non-tender abdominal mass with
warm periphery. She had an anion gap metabolic acidosis
27, bicarbonate 11, Cr 2.83 (baseline 0.7 in 2003), leukocytosis with maximum white count 24.9 (87.4% neutrophils), anemia with Hb 5.7 and lactate 12.0. CT of abdomen/pelvis revealed a 33 cm bilobed heterogeneous enhancing vascular mass arising from the uterine fundus.
Initially the patient was presumed to be in septic shock,
and aggressively fluid resuscitated, transfused several
units of packed red blood and empirically treated with
cefepime, vancomycin, and flagyl. All the cultures drawn
were negative. She did not respond to the aforementioned
management and continued to remain hemodynamically
unstable; therefore she was started on norepinephrine and
vasopressin. On the following day she experienced what
was presumed to be Acute Respiratory Distress Syndrome (ARDS) and shock from her severe inflammatory
process and was intubated. She subsequently became
oliguric and required dialysis. She eventually became
anuric with elevated urea nitrogen levels and FeNa of
0.7%. She continued to remain anemic despite the several
attempted transfusions, the hypothesis being she was
bleeding from the mass and it needed to be removed. On
day five, pressors were weaned, and her renal function
improved so that she no longer required dialysis. On day
nine, she was taken to the operating room by OBGYN
and underwent colposcopy, exploratory laparotomy, total
abdominal hysterectomy, and bilateral salpingooophorectomy. The operative findings revealed entire
abdominal and pelvic cavity to be filled with a large,
fleshy, soft tissue tumor with a necrotic and hemorrhagic
center which appeared to be rising from the fundus of the
uterus weighing five kilograms. There were multiple
large ruptured fibroids. There was some nectrotic tissue
on the omentum. Pathology report confirmed necrotic
leiomyoma. Post-op she remained stable, and on post-op
day 17 she was ambulating, voiding, tolerating regular
diet, and was discharged.
Discussion: Leiomyoma is the most common tumor in
women; its behaviors and complications are wellrecognized. Clinical manifestations include uterine bleeding, pelvic pressure and pain, and reproductive dysfunction. Increased size can cause the mass to become necrotic, hemorrhage, or cause obstruction of surrounding
structures, leading to severe complications. The presence
of large areas of damaged or necrotic tissue can mimic an
infectious focus. Systemic inflammatory response syndrome (SIRS) is characterized by 2 or more of: temp
>38.5C or <35C, HR >90, RR>20, or PaCO2 <32mmHg
& can initiate shock and multi organ failure. The tissue
injury from necrotic mass can initiate an acute and severe
inflammatory response. Multiple organ failure may be
due to an over vigorous manifestation of this normally
protective defense mechanism. This case is unique due to
the severe potentially life-threatening inflammatory syndrome leading to shock from a relatively benign mass.
The ruptured necrotic 5kg leiomyoma led to intraabdominal hemorrhage, severe inflammatory response,
and multi-organ failure. After excising the 33cm mass,
she stabilized and recovered. Prompt surgical management was essential to make the diagnosis and save the
patient’s life.
Strongyloides Hyperinfection in a Patient with Dermatomyositis
Linda Sharp, MD; Darrell Harrington, MD
Harbor-UCLA Medical Center
A 39-year old Guatemalan man had 1 day of shortness of
breath, nausea, vomiting, and abdominal pain. He had a
tracheostomy 2 months prior, and he noted 1 day of increased tracheal secretions. Normally he used a ventilator
at night, but for several days he had required constant
respiratory support. On the morning of admission the
patient noted diffuse, constant abdominal pain as well as
constipation for several days. He had dermatomyositis for
66
5 years and a recent hospitalization complicated by A.
baumanii pneumonia. Medications included prednisone,
methotrexate, and leucovorin, plus rituximab 4 months
prior. T 99.8, HR 123, BP 90/60. He was cachectic and
had coarse breath sounds bilaterally. Abdomen was distended, diffusely tender; bowel sounds were absent, and
there were positive peritoneal signs. He had diffuse muscle wasting. WBC 30.5, P83, bands 12, E none. ABG: pH
7.47, PCO2 56 mmHg, PO2 56 mmHg. CT scan showed
bibasilar infiltrates, dilated fluid filled loops of small
bowel with 4-5 mm wall thickness.
He was given antibiotics to cover for pulmonary and enteric sources, 2 units of PRBCs after his Hgb dropped
from 10.7 g/dL to 6.1 g/dL, pressors, and stress-dose corticosteroids. After 24 hours, his abdominal exam improved and after 48 hours he no longer required pressors.
On hospital day 3 he had a macular rash that spread to his
legs, abdomen, and back. Initial blood cultures grew K.
pneumoniae and E. Coli. Sputum grew P. mirabilis, A.
calcoaceticus-baumanii complex, K. pneumoneae, and E.
Coli. Fever persisted, and blood and respiratory cultures
remained positive throughout the hospital course despite
adequate antibiotic therapy. After one week, the patient’s
level of consciousness declined, and he required 70%
FiO2. Bronchioalveolar lavage returned bloody, frothy
fluid in several segments consistent with alveolar hemorrhage, and showed Strongyloides stercoralis. Biopsy of
the rash also revealed strongyloides. He was started on
albendazole on day 10 and switched to ivermectin on day
11. However, on day 11, the patient required escalating
amounts of oxygen, with poor arterial oxygenation on
100% oxygen. The patient expired on day 12.
Strongyloides stercoralis is endemic to tropical and subtropical regions, with global prevalence of 30 to 100 million cases. Prevalence in endemic areas ranges from 1%
to 30%, including some areas in the U.S. Individuals may
be asymptomatic carriers with or without peripheral
blood eosinophilia. This patient likely acquired Strongyloides in the remote past, then developed hyperinfection
as a result of immunosuppressive therapy. Understanding
the geographic distribution of Strongyloides endemicity
may help clinicians identify at-risk patients prior to immunosuppressive therapy, thus preventing the often-fatal
hyperinfection. outcome.
Sometimes Only Time Will Tell
Bryant Sheh, MD; Siamak Basiratmand, MD
Olive View-UCLA Medical Center
Case: A patient presented to UCLA-Olive View Medical
Center several times over a 2 week period, each time with
new symptoms and signs, before a satisfactory diagnosis
was reached.
A 25 year old female presented to the ED complaining of
several years of intermittent abdominal pain, diarrhea,
and pelvic pain, acutely worsened the past 3 days. She
denied fevers and weight loss. Review of systems was
otherwise negative. Past medical history was significant
for treatment of Salmonella gastroenteritis, Helicobacter
pylori gastritis, and Gardnerella vaginosis in Mexico over
the last year. Her medications included famotidine, butylscopalamine, and recently begun oral contraceptives.
Family history was significant for an aunt with lupus.
Social history was noncontributory.
Vital signs, physical examination and labs were within
normal limits. She was diagnosed with irritable bowel
syndrome, urinary tract infection and discharged with
ciprofloxacin, psyllium, donnatal, and omeprazole.
The patient returned ten days later reporting dysuria, a
new oral lesion, and lower extremity pain. A 1cm
aphthous ulcer was noted on the lower lip. Diffuse tenderness was present in both lower extremities. Urinalysis
was abnormal and the patient was discharged with nitrofurantoin and a diagnosis of resistant urinary tract infection.
Two days later, she returned with severe bilateral ankle
pain, vaginal pain, and a new rash. Her temperature was
38.1°C. Two new 5mm ulcers were seen on the right labia majora. Raised, erythematous, tender nodules on her
shins were consistent with erythema nodosum. Laboratory testing revealed WBC 10.1, ESR 33, CRP 51, and a
negative ANA.
The patient was admitted for observation. An infectious
and serologic workup for secondary causes of erythema
nodosum was ultimately negative. A PPD was considered
positive when a 2cm red, tender bump formed at the site.
Biopsy of the oral ulcer revealed nonspecific inflammatory cells and a negative viral culture. When pain and
ambulation improved with ibuprofen, the patient was
discharged home.
The patient was readmitted three days later with severe
odynophagia. New oral ulcers were seen on the soft palate. Her right eye was red and the right knee swollen.
Knee arthrocentesis revealed lymphocytosis and negative
cultures. Ophthalmologic exam confirmed uveitis. Given
the clinical picture of uveitis, oral and genital ulcers, arthritis, erythema nodosum, and what now appeared to be
a positive pathergy test, the patient was diagnosed with
Behcet’s disease. Colchicine and prednisone were begun.
Within days, her symptoms resolved and she was discharged home.
Discussion: Behcet’s disease is a clinically-diagnosed
systemic inflammatory disorder. Patients such as this who
initially present with partial symptoms and signs can
make recognition of the disease challenging for any physician. A delay in diagnosis is typical. Ultimately, clinicians should maintain a broad differential when confronted with unusual symptoms or those that are refractory to usual treatment.
Refractory Hyponatremia and Abnormal Thyroid
Function Test in an End Stage HIV Patient
67
Rong (Cindy) Shi, MD; Terence Gan, MD, MPH;
Bhupinder Khehar MD
Scripps Mercy Hospital
Background: Symptomatic HIV infection is a common
cause of hyponatremia & many patients have SIADH.
Common cause of HIV related SIADH include pneumonia, CNS infection, and malignancy. In euvolemic hyponatremia, adrenal insufficiency or hypothyroidism needs
to be ruled out before diagnosis of SIADH is made.
Case Presentation: 44 year old homosexual male, end
stage HIV presented with generalized weakness. Recent
admission for weakness, found hypovolemic hyponatremia, improved after hydration; discharge Na 123 with
diagnosis SIADH secondary to HIV. Re-admitted 1 week
later with progressive weakness, admission Na 124. PE:
T 100.7, RR 22, HR 75, BP 97/50 without orthostatic
changes. Cachectic, lethargic but A&O x3 male, otherwise no pertinent positive findings. LABs: WBC 1.8,
CD4=3, Na 124, K 3.5, renal and liver panel normal,
TSH 1.4520, fT4 0.53, fT3 1.83, thyroglobulin 10.4, antithyroglobulin 1.0, urine Na 130, urine Osm 951, FeNa
1.17, basal cortisol 4.9, after ACTH 20.3, extensive ID
work up negative, rest of pituitary hormone workup normal. Imaging: CT head on prior admission negative. MRI
brain (HD #3): 2.3 cm mildly enhancing lesion in the
region of left caudate head. Repeat CT (HD #9):
Enlargement of mass-like density in left caudate nucleus.
Pathology: Brain biopsy at left basal ganglion lesion (HD
#14): Diffuse large B-cell lymphoma, (+) CD20, (-) CD5,
(-) CD10.
Treatment: Refractory euvolemic hyponatremia: Minimally response to fluid restriction, salt tablets, demeclocycline, normal saline, or levothyroxine, requires continuous hypertonic saline to maintain serum Na. Only
after lymphoma treatment initiated, serum Na quickly
improved to normal range, no additional treatment necessary. Hypothyroidism: levothyroxine started on HD #2,
titrated up to 75 mcg on discharge with no improvement
in fT4, eventually fT4 corrected at dose 125 mcg. CNS
lymphoma: dexamethasone started after pathology report,
then MTX with leucovorin rescue. Patients overall condition much improved including energy level and normalized Na level, despite low fT4. Patient was discharged on
HD #27 with oncology follow-up.
Discussion: Refractory euvolemic hyponatremia: hyponatremia not responsive to conventional SIADH treatment, neither to thyroid hormone replacement, but dramatically improved after dexamethasone started for
treatment of CNS lymphoma. Thus, etiology might be
multifactorial, but mostly CNS lymphoma causing
SIADH. CNS lymphoma: Quick-growth mass lesion with
doubling time ≤ 3 weeks, if ruled out infection, highly
suspicious for lymphoma, which has quick response to
steroids, dexamethasone would reduces edema, also is
treatment for lymphoma, thus quickly improving hyponatremia. Hypothyroidism: May represent an extreme of
euthyroid sick syndrome, central hypothyroidism debatable, as rest of pituitary hormones was all normal. Possible hypotheses: HIV related vs. mass effect vs. lymphocytic infiltration.
Conclusion: SIADH in HIV patient is not uncommon,
however, with symptomatic refractory hyponatremia,
further work up should be warranted, including MRI to
rule out primary CNS lymphoma.
Anti-NMDA Receptor Encephalitis: A Rare Diagnosis
with a Treatment
Neeharika Srivastva, MD
University of California, Los Angeles
Learning Objectives:
1. Develop a differential diagnosis of reversible etiologies
of encephalitis
2. Management of anti-NMDA receptor encephalitis
A 27-year-old female presented after her first witnessed
generalized tonic-clonic seizure. One week prior to presentation, she had had flu-like symptoms, difficulty with
speech & typing, & personality changes. Her symptoms
were attributed to her identical twin sister’s recent death
from meningo-encephalitis. In the ER, she had fluctuating levels of consciousness with episodes of nonsensical
laughing and hysterical crying. She was unable to follow
commands, and had decreased muscle tone, rhythmic
movements alternating with semi-purposeful movements
(automatisms) of the upper extremities.
A lumbar puncture demonstrated 65 WBC with 100%
lymphocytes. She was given phenytoin, acyclovir, vancomycin and ceftriaxone.
An MRI study of the brain demonstrated a T2 FLAIR
hyperintensity within the cerebral sulci. MRV, MRA and
CT scans of the brain were unremarkable. Thorough investigations to evaluate for encephalitis were pursued that
demonstrated N-methyl-D-aspartic acid (NMDA) receptor antibodies in the CSF. Incidentally, the patient’s twin
sister’s final autopsy results were completed at this time
and also showed these antibodies in the CSF.
Anevaluation was undertaken for an underlying malignancy, including a bilateral salpingo-oophorectomy, all of
which was unrevealing. The patient received immunotherapy with plasmapheresis, cyclophosphamide, and
methylprednisolone with no improvement. She remained
non-communicative, unable to follow commands, combative, and displayed oral dyskinesias.
Following surgery and further cycles of immunotherapy
including rituximab, she began to open her eyes and
within two weeks of surgery she could follow commands
appropriately.
After three months of neuro-rehabilitation, the patient is
now independent in her self-care activities. She has fre-
68
quent facial twitches, but is able to read and socialize
appropriately with friends. She plans to attend community college.
Anti-NMDA receptor encephalitis presents with a neuropsychiatric syndrome due to antibodies against NR1-NR2
heteromers in the hippocampus. It occurs mostly in
young women who have tumors, commonly ovarian teratomas. Early tumor removal followed by immunotherapy
leads to a better prognosis with minimal risk of recurrences.
This case illustrates the importance of developing a comprehensive differential diagnosis for encephalitis presenting with neuro-psychiatric symptoms and then pursuing
definitive diagnostic evaluation for those illnesses that
can be treated. The differential diagnosis of reversible
causes of encephalitis includes drug ingestion, porphyria,
Systemic Lupus Erythematosus cerebritis, Sjogren's syndrome, Hashimoto's encephalopathy, Cytomegalovirus,
Herpes Simplex Virus and paraneoplastic encephalitis.
Early diagnosis of anti- NMDA receptor encephalitis
allowed for aggressive management of the patient based
on experiences from various case reports. This led to a
meaningful recovery for the patient.
Bizarre Behavior And Bezoar - A Thought To Chew
On
Joanna Tan, MD; Jasminka Criley, MD, FACP, FHM
St. Mary Medical Center
Background: Chewing gum is a common behavior.
Swallowing gum is often thought to be harmless. Gastric
bezoars are a rare finding involving <1% of upper gastrointestinal endoscopy and are often related to decreased
gastrointestinal motility. Chewing gum has been described as a foreign object bezoar, typically in textbooks.
Reported cases of chewing gum bezoar in the literature
are few and often involve the
pediatric population. In contrast to nonmedication bezoars, pharmacobezoars may produce additional symptoms related to the release of active ingredients or produce subtherapeutic medication levels.
Case: 54 year old with history of seizure, pseudoseizure,
depression and diabetic gastroparesis presented with yet
another episode of an acute periumbilical abdominal pain,
nausea, vomiting, “seizure” and bizarre behavior. Patient
was recently discharged from a psychiatric ward and had
a subtherapeutic phenytoin level. History was difficult to
obtain but it appeared that gastrointestinal symptoms
were most prominent. Patient had poor dentition and was
mostly edentulous. Abdominal physical exam findings
were inconsistent ranging from benign to periumbilical
tenderness. Laboratory data was consistent with dehydration. Abdominal x-ray, ultrasound and computer tomography scans with oral contrast revealed no abnormalities. An esophagogastroduodenoscopy (EGD) performed
2 months prior revealed esophagitis and gastritis. Due to
her failed medical management, a repeat EGD was per-
formed revealing a large yellow crystalline mass, removed per os using basket with remnants broken by forceps and irrigated. Subsequently, her bizarre behavior
and abdominal pain resolved. The patient then admitted
to frequently swallowing gum – up to a pack a day. It is
suspected the bezoar consisted of chewing gum and
pharmacobezoar.
Conclusion: In a patient with multiple psychiatric disorders, a history of non-compliance, poor presentation history, inconsistent physical exam findings and benign
laboratory and imaging results, it is difficult to not suspect malingering or a psychological component as a
likely etiology of abdominal pain. However, a bezoar
should be in the differential diagnosis if patients have a
history of decreased GI motility, particularly with the
presence of psychiatric disorders and subtherapeutic
medication levels. It is still unknown if the bezoar caused
her bizarre behavior by its mere physical presence or by
the pharmacobezoar component.
Stop Being a “Pain in the Buttocks”
Laren Tan, MD; Raymond Yau; Gary Thompson
Loma Linda University
Pyomyositis is rare in the United States and is often overlooked when muscular pain is the initial presenting symptom. The concurrence of systemic illness including fever
and leukocytosis obscures the clinical picture and without
appropriate imaging the diagnosis will remain elusive.
An 18-year-old female with no significant PMH was admitted because of sharp left gluteal pain. One week prior
to admission, she reported of nocturnal sharp pain in the
same region, which progressively worsened until she was
no longer able to ambulate. The patient was febrile on
admission and had leukocytosis. Other laboratory tests
were including, urine analysis, C-reactive protein, ESR
were normal. Initial CT scan of the abdomen/pelvis did
not reveal any abnormalities. She was started on empiric
antibiotics while blood cultures were pending. Despite
aggressive treatment, she continued to have intermittent
fevers and complained of increasing pain over her affected area. The patient had magnetic resonance imaging
(MRI) of the abdomen and pelvis demonstrating significant diffuse muscle inflammation of the left iliacus muscle. Bacteremia was reported which later cultured coagulase-negative S. aureus. Significant inflammation of the
iliacus muscle, along with fever and bacteremia were
consistent with the diagnosis of pyomyositis. After initial
MRI, repeat CT scan showed a small abscess that was
percutaneously drained, resulting in considerable clinical
improvement. A survey with transesophageal echocardiogram was done which documented no evidence of
vegetations. Her symptoms continued to improve and she
was discharged home.
Historically pyomyositis has been differentiated into
tropical pyomyositis or temperate pyomyositis. The latter
is considered atypical in the United States and it gener-
69
ally occurs in immuno-compromised host and in intravenous drug users. The case presented here is amongst a
handful of reports where spontaneous occurrence had
taken place in a normal host with no risk factors. Even
though temperate pyomyositis was once thought to be
uncommon, early clinical suspicion along with proper
imaging can help in the early diagnosis and aggressive
treatment that usually results in a successful outcome.
Treatment Failure of Ganciclovir for Ebstein-Barr
Virus Induced Transverse Myelitis in a Systemic Lupus Erythematosus Patient
T. Tan DO; R. Stewart BS; A. Zadeh DO; K. Raoufi
MD
Western University of Health Sciences
We report a case of a 28 year old female, on prednisone
and cellcept for systemic lupus erythematosus (SLE),
who was diagnosed with EBV induced transverse myelitis. Ebstein-Barr virus (EBV) is a herpes virus that is
spread by salivary secretions. Clinical manifestations can
range from infectious mononucleosis to Burkitt’s lymphoma. Cellular immunity is important in controlling the
proliferation of EBV infected cells.
On March 5, 2009, the patient presented to the hospital
with lower back pain and bilateral lower extremity paresis with no loss of sensation. During her hospital stay, she
progressed to complete paraplegia, complete anesthesia
below T10, urinary retention and fecal incontinence. The
diagnosis was made by magnetic resonance imaging
(MRI) of the thoracic and lumbar spine, viral serologies
and cerebrospinal fluid (CSF) analysis. The total protein
in the CSF was 76 mg/dl, and the glucose was 48 mg/dl.
PCR studies of the spinal fluid were negative for enterovirus, HHV6, HSV I and II, CMV and Varicella Zoster
virus DNA, but were positive for EBV DNA at 44,984
copies per mL. The patient was treated with ganciclovir
10mg/kg/day intravenously for 4 weeks and then oral
ganciclovir 60mg/kg/day for 4 weeks. The patient was
seen in the hospital two and a half months later with no
change in her neurological examination.
The degree of damage to the nervous system prior to initiation of therapy may be an important factor in the
treatment of EBV induced transverse myelitis. Patients
with severe, irreversible axonal damage are less likely to
benefit from ganciclovir alone. It is also possible that
CMV hyperimmune globulin plays a role in the recovery
of patients presented by Gruhn et al and Oettle et al.
Loss of Health Insurance Leads to Rapid Progression
of Kaposi Sarcoma
J. Taylor, MA, MSIII1; R. Gharibjanyan, MD2; H.
Van Gelder, MD2; J. Criley, MD1, 2
1
David Geffen School of Medicine at UCLA
2
St. Mary Medical Center
Introduction: Kaposi´s Sarcoma (KS) is associated with
HHV 8 in AIDS patients. Highly active anti-retroviral
therapy (HAART) decreases KS risk. In this health insurance market, it is difficult for some patients to afford
HAART.
Case Report: A 49-year-old Caucasian male with 20year history of HIV/AIDS was admitted with GI bleed
and necrotic KS lesions of the feet. He lost health insurance 2 years prior. He could not afford HAART. He followed a healthy diet and lifestyle to control his illness.
He was doing well until about 6 months prior when he
noticed violaceous lesions on his feet. Later , the lesions
appeared on his face and subsequently the palate. Foot
biopsy confirmed KS. Hewas treated with radiation therapy for local control. Three weeks later, he presented to
the ED with weakness and foot pain. He also had diarrhea
and dark creamy stools. On exam he had purpuric, nummular lesions over his extremities. His feet were necrotic
and malodorous. His WBC was 26.7, Hb 4, Hct 12, platelets 25,000, T bili 1.3, D bili 0.8, AST 68, ALT 15, CD4+
count 14. He was transfused multiple times but continued
to have GI bleed. Endoscopy showed hyperpigmented
lesions in the duodenum, confirmed to be KS. He was
found to have KS of the trachea, bronchial tree and likely
the lungs. His condition rapidly deteriorated. He expired
1 month after presentation.
Discussion: KS has become a rare AIDS-defining lesion.
In 2004, the incidence of KS was 1/10 of what it was 10
years prior. HAART reduces KS incidence, prolongs time
to treatment failure, and prolongs survival. The United
States government passed the Medicare Modernization
Act of 2003 requiring that Medicare Part D insurers provide medication therapy management (MTM) services.
This legislation was to educate patients, improve outcomes and adherence. Previous studies have demonstrated the power of MTM programs in the setting of
HIV/AIDS therapy. Patients were more likely to adhere
to medical regimens, achieve significant reduction in
viral load and improvement in CD4+ T-lymphocyte
counts.
Informing HIV/AIDS patients of such programs would
have made a difference in the life of this patient. Had the
patient been able to receive continuous management on
HAART, he would have been less likely to develop end
stage Kaposi´s Sarcoma. HIV/AIDS patients in California
should be informed of possible Medical Part D eligibility.
Uncomplicated Amebic Hepatic Abscess
J. Taylor, MA, MSIII 1; A. C. Ortiz, MD2; T. Yang,
MD2; C. Craig, MD1,2
1
University of California, Los Angeles
2
UCLA-Olive View Medical Center
Introduction: The presenting symptoms of a hepatic
abscess can be vague, with fever being the most commonly reported symptom by physicians. A high level of
clinical suspicion is key to an appropriate diagnosis.
70
Case Report: A 42-year-old Latino male presented to his
primary care physician with a one-week history of fevers,
chills, myalgias and abdominal pain. His fever had been
present for one week, and he symptoms of nausea, vomiting or diarrhea. His past medical history did not include
any recent viral infections or gallbladder disease. He denied any recent surgeries. He was sent home from his
initial outpatient visit with reassurance alone. As the pain
persisted, the patient returned to his primary care physician and began a three-day course of antibiotics. A CT
with contrast of the abdomen and pelvis revealed a 10x6
cm right-lobe hepatic abscess, and he was referred to our
service for inpatient evaluation. Further history taking
revealed a 2 pack-year smoking history, and occasional
use of intranasal cocaine. The patient had traveled to
Mexico three years prior to presentation. He was sexually
active with several female partners, with intermittent use
of condoms. He had intentionally lost weight in the previous three weeks by taking an herbal medication
(HerbaLife). On Physical examination, the patient was
anicteric, had a soft, non-tender abdomen. There was no
guarding or rebound tenderness. The liver edge was not
palpable. At the time of admission, the patient had a leukocyte count of 10.8/mm3 and a platelet count of
712,000/mm3. The patient had an AST of 20 u/l, ALT of
17 u/l. His alkaline phosphatase was slightly elevated at
171 u/l, bilirubin was normal at 0.5 g/dl and serum albumin was slightly decreased at 3.0 g/dl. He was started on
intravenous metronidazole and ceftriaxone. The patient’s
symptoms improved and repeat imaging three days after
admission revealed a slight decrease in size of the abscess. Percutaneous culture of the abscess fluid was positive for Entamoeba histolytica. The patient was continued
on intravenous metronidazole alone. A percutaneous
drain was placed, and 75cc of bloody purulent fluid was
removed. The drain was subsequently removed and the
patient was discharged on oral metronidazole two days
later.
Discussion: This case illustrates that hepatic abscess’ can
present with few symptoms, and recognition necessitates
a high level of clinical suspicion along with mindfulness
of risk factors. Risk factors associated with increased
mortality include a bilirubin greater than 3.5 g/dl, serum
albumin less than 2 g/dl, multiple abscesses and/or a large
abscess cavity. Mortality associated with an uncomplicated hepatic abscess, such as the case we present, is less
than 1% if treated with antibiotics and appropriate drainage.
The Great Escape: An Unexpected Diagnosis from
Lung Entrapment
Ana Cecilia Uribe-Wiechers, MD; Visal Nga, MD;
Hemal Kadakia, MD; Joyti Datta; Alexander Stein,
Jasminka Criley, MD
St. Mary Medical Center and David Geffen School of
Medicine at UCLA
Introduction: Finding the etiology of lung entrapment
can be challenging. Lung entrapment can result from malignant or nonmalignant diseases, including sequela of
remote inflammatory condition. We present a case of
lung entrapment that led to a diagnosis of pleural tuberculosis only after surgical pathology.
Case Description: A 48 year old previously healthy
Caucasian man was admitted with one month history of
worsening shortness of breath, dry cough, easy fatigability, night sweats and fevers. He denied hemoptysis,
weight loss, weakness, or recent travel. Physical examination was significant for fever to 102 F, decreased
breath sounds and dullness to percussion on the right
hemithorax. Patient’s cell counts and chemistries were
normal and HIV was negative. Chest x-ray showed a
large right pleural effusion, subsequently determined to
be exudative with lymphocytic predominance. Pathological review of the pleural fluid was suspicious for lymphocytic lymphoma, however flow cytometry and cytology were negative. Broncho-alveolar lavage, and sputum
cultures were unrevealing. Bone marrow biopsy was
negative. Other laboratories including blood cultures, RF,
ANA, and ADA were also non-diagnostic. Thus, cardiothoracic surgeon was consulted for mediastinoscopy and
lymph node biopsies. On mediastinoscopy, paratracheal
lymph nodes were firm and rubbery; a frozen section
showed small caseating granulomata. A full thoracotomy
was performed, revealing trapped right lung. A formal
decortication was performed to liberate the lung. Analysis
of the lymph nodes and pleural biopsy revealed caseating
granulomata with AFB stain positive for tuberculosis. Pt
was immediately started on Rifampin, INH, Pyrazinamide and Ethambutol (RIPE). Six weeks later the pleural
fluid culture grew mycobacterium tuberculosis.
Discussion: Pleural effusions and trapped lung are challenging diagnosis requiring multi pronged approach to
obtain a definitive diagnosis.
Lung entrapment is characterized by inability of the lung
to expand and fill thoracic cavity due to a restricting fibrous visceral pleural strip. It is usually asymptomatic but
in some patients, it may be the cause of dyspnea. In
symptomatic patients, the only available treatment is surgical decortication.
Tuberculous pleural effusion occurs in approximately 5%
of patients with Mycobacterium tuberculosis infection.
The HIV pandemic has been associated with increasing
incidence of extrapulmonary tuberculosis, which has resulted in increased recognition of TB pleural effusions
even in developing nations. Interestingly our patient did
not have any risk factors for developing extrapulmonary
tuberculosis.
Here we presented a case with an extensive unrevealing
workup including negative ADA assay, but the fact that
the patient had symptoms of lung entrapment led to more
invasive procedures. Such procedures uncovered the etiology and treatment of his dypsnea.
71
Cardiac Metastasis of Malignant Melanoma:
A Rare Cause of Right Bundle Branch Block
Nicky Wadiwala, MD
University of Southern California
Background: Ocular melanoma is diagnosed in approximately 6 million people annually. Ocular melanoma
metastasizes less frequently than cutaneous melanoma
75% vs. 96%, respectively. The liver is the most common
location for metastases from ocular melanoma and the
heart is thought to be another common site that is clinically underdiagnosed. Cardiac metastases are uncommon,
with the exception of malignant melanoma. A large autopsy study showed cardiac metastases in 64% of patients
dying from metastatic melanoma. However, cardiac metastases are rarely detected antemortem (2% of cases),
despite sometimes massive involvement due to the lack
of specific clinical manifestation.
Case: A 45-year-old patient, with a history of right ocular
choroidal melanoma treated with radiation 10 years ago,
was admitted due to fatigue, mild dyspnea on exertion
and dry cough. The electrocardiography showed a new
right bundle branch block.
Discussion: The clinician should be alert to the development of new cardiac conduction defects in a patient with
prior melanoma history and should perform a detailed
cardiac examination including echocardiography to exclude cardiac metastasis of the tumor. Early detection of
cardiac melanoma is important for therapeutic and prognostic reasons.
Churg-Strauss Syndrome: An Uncommon Cause of
Adult Respiratory Distress Syndrome (ARDS)
Tzu-Fei Wang, MD; Naveen El-Farra, MD
University of California, Los Angeles
Case: An 80-year-old man with history of asthma, coronary artery disease, remote history of polyarteritis nodosa
(PAN) presented with progressive dyspnea, lethargy,
cough, and fever for three weeks. He was intubated for
respiratory failure, and presumptively treated for pneumonia and congestive heart failure. His condition did not
improve. During routine laboratory studies he was found
to have an eosinophilia with 23% eosinophils. Additional
studies were sent and notable for a markedly elevated
ESR, CRP, and IgE. His serum anti-neutrophil cytoplasmic antibody (ANCA) panel was negative. Given his
history of asthma and pauci-immune glomerulonephritis,
eosinophilia, and highly elevated IgE, he was diagnosed
with Churg-Strauss syndrome. He was started on high
dose steroids and daily oral cyclophosphamide and within
two weeks had marked clinical improvement was extubated.
Discussion: Churg-Strauss Syndrome is an uncommon
disease with annual incidence of one to three per million.
Common manifestations include asthma, peripheral eosinophilia, and vasculitis in multiple organ systems. Lungs
are most commonly affected; however, sinuses, skin,
heart, kidney, gastrointestinal tract, and peripheral nervous system can all be involved. The exact pathogenesis is
unknown, but it is thought to be autoimmune in nature.
The pathology of involved organs often reveals eosinophilic vasculitis and perivascular necrotizing granulomas.
IgE is frequently elevated. About 30-50% of patients are
ANCA positive, of which 70-80% is p-ANCA (antimyeloperoxidase antibody). Treatment of choice is high
dose glucocorticoids. In patients with multi-system failure due to vasculitis, daily cyclophosphamide or azathioprine can be added. This case demonstrates the importance of broadening differential diagnoses when initial
treatments yield suboptimal responses.
A Case of Thrombotic Microangiopathy in Systemic
Lupus Erythematosus
Brandon B. Woodbury, MD; James Wilson, MD
Olive View-ULA Medical Center
A 25 year-old woman with systemic lupus erythematosus
and a history of cerebritis and World Health Organization
(WHO) Stage V nephritis presented with pleuritic chest
pain, shortness of breath, dry cough, and fever. She had
experienced intermittent swelling of her legs and face for
several months prior to presentation. She was febrile and
tachycardic, with an elevated blood pressure of 189/111.
Pulmonary auscultation was clear except for bibasilar
decreased sounds. A preexisting 3/6 systolic murmur
throughout her precordium was noted. She had trace bilateral leg edema, but no jugular venous distension.
Creatinine was elevated from her baseline of 1.2 to 1.9
mg/dL, and she had a leukocytosis of 14.6 cells/uL. Urinalysis showed specific gravity of 1.021, pH of 7, with 10
red blood cells/field and proteinuria>300 mg/dL. Serum
tests also revealed C3/C4 of 52/10 mg/dL, anti-dsDNA
>1:1280, positive lupus anticoagulant, ANA 1:320 (homogenous pattern), and an elevated ESR of 63 mm/h.
Direct coombs, anti-cardiolipin, anti-Smith, anti-DNAse
B, beta-2 glycoprotein, and RPR were negative. An HIV
test was negative 3 months previously. Heparin drip was
begun after a ventilation-perfusion scan showed intermediate probability of pulmonary embolus. Intravenous
steroids were also started for suspected lupus exacerbation. Renal ultrasound showed bilateral increased cortical
echogenicity and pelviectasis. Her renal function continued to decline, eventually requiring hemodialysis. A renal
biopsy showed WHO stage IV (diffuse proliferative) and
WHO stage V (membranous) lupus nephritis, as well as
evidence of WHO thrombotic microangiopathy (TMA).
Cyclophosphamide and plasmapheresis were started to
treat her stage IV nephritis and thrombotic microangiopathy, respectively. Her platelet count decreased, and argatroban was substituted for heparin due to concern for
heparin-induced thrombocytopenia (HIT assay was eventually negative). After several days of plasmapheresis, the
patient’s renal function improved and her creatinine returned to baseline. She returned home and was doing well
72
at a follow-up visit several weeks later. TMA manifests
itself in thrombotic thrombocytopenic purpura, hemolytic
uremic syndrome, malignant hypertension, systemic sclerosis, preeclampsia, and drug reactions. As the laboratory
findings are similar in the disorders resulting in TMA,
specific diagnosis is best determined by history. TMA is
characterized by endothelial dysfunction and platelet aggregation with resulting thrombocytopenia and injury to
erythrocytes. Examination of the platelet thrombi shows
von Willebrand factor (VWF) without fibrin or fibrinogen. VWF normally exists as a spectrum of multimeres—
the smaller ones are secreted while the larger (& more
pro-thrombotic) multimeres are stored in the synthesizing
cells. Normally these larger multimeres are broken down
by the metalloproteinase ADAMTS-13. Dysfunction of
ADAMTS-13, VWF, or the platelets themselves predispose to pathologic platelet aggregation and thus TMA.
Therapy mainly involves replacing the larger-multimered,
prothrombotic VWF with normal VWF through plasmapheresis. This case represents a rare occurrence of
TMA in a patient with systemic lupus erythematosus.
Acute Myocardial Infarct in Patient with Idiopathic
Anasarca and Chronic Eosinophilic Leukemia Following Treatment with Imatinib
Kevin Wu, MD; Casey O’Connell, MD
University of Southern California
Chronic eosinophilic leukemia is a rare myeloproliferative disorder that by WHO 2009 criteria has a clonal cytogenetic abnormality, most commonly FIP-PDGFR-α
mutation. It is treated with Imatinib, a tyrosine kinase
inhibitor targeting this fusion protein. In this report I describe a previously unreported phenomenon. 62 y/o Hispanic male with a h/o of idiopathic anasarca presents with
progressive dyspnea on exertion, orthopnea, chronic and
left chest pressure worsens with inspiration, escalating
over a 3 month period. Exam is significant for decreased
breath sounds and anasarca. CXR shows complete
opacification of his right hemithorax and left pleural effusion. Patient was ruled out for ACS. Labs: WBC 88k,
(90% eosinophilia). Peripheral smear shows significant
eosinophilia but otherwise normal. Secondary causes
such as infectious, rheumatologic, and other secondary
hematologic processes were ruled out. Marrow biopsy
shows normocellular marrow, severe hypereosinophilia.
FIP-PDGFR mutation analysis is (-) in the context of a
(+) control that’s (-). We diagnose the patient with
chronic eosinophilic leukemia (CEL) and treat with
Imatinib and hydroxyurea. Patient was followed in clinic
4 days post-discharge and his WBC decreased from 108K
to 93k (99% eos). He complained of substernal chest
pressure unchanged from initial admission. Four days
later, he was admitted for worsened substernal chest pressure. He had new findings of ST depression in V3 – V6,
increased troponins (0.64,0.69,0.72) and mildly decreased
EF with regurgitation which were all new findings. His
WBC had further decreased to 42k on admission and ultimately 16k. Cardiology did not perform catheterization,
attributing his disease process to CEL. He was managed
with heparin & beta-blockers. He had asystole. CPR revived him. Troponin continued to increase, and he developed multiorgan failure. Care was withdrawn. Autopsy
revealed pale yellow discoloration and hemorrhage in left
ventricular posterior/ lateral wall, with moderate atherosclerosis, but no intraluminal thrombus or plaque. Microscopic evaluation showed neutrophilic infiltration but no
eosinophils. The pathological diagnosis was severe acute
MI as cause of death. It is unclear what roles idiopathic
anasarca, the eosinophilia, or Imatinib treatment played.
In the literature there are few case reports of acute development of CHF in CEL patients treated with Imatinib, as
well as AMI in patients with untreated hypereosinophilia.
There are no reports of Imatinib treatment being associated with AMI especially in the setting of a normalized
WBC. This case may improve future decision-making.
Complete A [V8] block: A Case of V8-Associated Hyperkalemia and Subsequent Complete Heart Block
Kevin Wu, MD; Brandon Itagaki, MD; John Sim, MD
Kaiser Permanente Southern California
Case: A 71-year-old female with a history of aortic
stenosis, hypertension, diabetes mellitus and hyperlipidemia presented to the emergency room with a three day
history of generalized weakness, fatigue and dyspnea. On
examination, she was noted to have a heart rate of 30
beats per minute with a blood pressure of 101/71. Her
examination was significant for a bradycardic heart rate,
with a 2/6 early systolic murmur. Lungs were clear, abdomen soft and extremities warm. Initial laboratories
revealed potassium of 6.7 mmol/L, BUN of 18, and
creatinine of 0.9. Outpatient medications include Metformin, Glipizide, Aldactone, Lisinopril, Atenolol, and
Zocor. EKG revealed complete AV dissociation with
peaked T waves. The patient was treated for hyperkalemia and started on isoproterenol for bradycardia.
Additional history revealed that over the past 2 weeks,
the patient was drinking more than four, 12 ounce cans of
V8 per day because she saw advertisements promoting
this supplement for seniors.
The patient’s potassium level returned to normal and isoproterenol was weaned off. Her 3rd degree heart block
resolved and she was discharged two days later.
Discussion: This case illustrates the potential harm that
can result from overuse of a nutritional supplement. We
describe a case of hyperkalemia and subsequent third
degree heart block that resulted from consumption of a
high potassium concentration drink while on 2 medications that inhibited kaliuresis. The patient had also been
on an additional medication that predisposes her to hyperkalemia from intracellular shifting. V8, which is a
common readily available juice, has 670mg of potassium
per 12 ounce serving. This amount of potassium is considered safe in moderation, but there is a clinical need to
be aware of hyperkalemia in certain populations who are
73
at risk of developing it and its clinical consequences such
as heart block in our case illustration.
Two Distinct Clinical Entities Causing Severe Hypereosinophilia
Stroke at 21: Catastrophic Antiphospholipid Syndrome
Kevin Wu, MD; Casey O’Connell, MD
University of Southern California
Wei-I Vickie Wu, MD, MHS; Kuo-Chiang Lian, MD
University of California, Los Angeles
Hypereosinophilia is a nondescript clinical term that can
be associated with many different diseases including infectious, allergic, rheumatologic and hematologic processes. Here I illustrate two patients with very different
causes of eosinophilia. In the first case, a 58 y/o Hispanic
female with no significant history presents with 6 month
history of right upper quadrant abdominal pain, nausea
and bloating. She denies other significant complaints on
ROS. She does report frequent trips to Mexico and has a
pet dog. Exam is significant for liver edge palpable below
the R costal margin. Chest radiograph shows elevation of
right hemidiaphragm. Initial labs are significant for WBC
of 75k with 70% eosinophilia and mild elevations in
AST, ALT and alkaline phosphatase. CT scan shows
large 6 cm lesion with central necrosis in the right lobe of
the liver. Stool O&P, strongyloides, visceral larval migrans, echinococcus and entamoeba histolytica serologies
are all negative. Biopsy of the lesion shows necrotic material. Patient is empirically treated with ivermectin for
two weeks, with improvement of eosinophilia. WBC increases after treatment completion. Patient is treated with
a 6 week course of ivermectin, with complete normalization of leukocytosis and eosinophilia. There is complete
liver regeneration at one year follow-up. In the second
case, a 62 y/o Hispanic male with a history of idiopathic
anasarca presents with progressive dyspnea on exertion,
orthopnea, chronic and left chest pressure worsens with
inspiration, escalating over a three month period. Exam is
significant for decreased breath sounds on right and left
base and anasarca of extremities. Chest radiograph shows
complete opacification of his right hemithorax and left
pleural effusion. Serial troponins are negative and echo
shows normal systolic function. Basic labs are significant
for WBC of 88k, with 90% eosinophilia. Effusion is exudative on analysis. Peripheral smear is significant for
significant eosinophilia but otherwise normal. A workup
to exclude secondary causes of hypereosinophilia is initiated. Bacterial, mycobacterial and fungal cultures of sputum and pleural fluid are negative. Cocciodiodes CF and
histoplasmosis antigens are negative. Stool O&P,
strongyloides and visceral larval migrans antibody are
negative. Churg-Strauss and other rheumatologic causes
are unlikely given the clinical history. Pleural fluid cytology shows no malignant cells. Flow cytometry of pleural
fluid shows no evidence of atypical lymphocytes, CT
shows no lymphadenopathy, and IgE/serum tryptase levels are normal indicating that lymphoma is unlikely to be
the cause. Marrow biopsy shows normocellular marrow,
severe hypereosinophilia with normal maturation. FIPPDGFR mutation analysis is negative in the context of a
positive control that’s negative. We diagnose the patient
with chronic eosinophilic leukemia and treat with
Imatinib and hydroxyurea leading to normalization of
leukocytosis and eosinophilia within two weeks. With the
Catastrophic antiphospholipid syndrome is a rare event
associated with a high mortality rate. Suspicion should be
high in young patients who present with unexplained
sudden thrombotic events. With prompt recognition and
intensive treatment, outcomes can be dramatically improved.
Case: A previously healthy 21-year-old woman with a
history of asthma and eczema was found to be acutely
unresponsive with seizure-like activity. On further questioning, she had been experiencing headaches for the past
six months but denied asociated fevers, chills, weight
loss, sick contacts, or unusual travel. Upon arrival at the
hospital she was hemiplegic on exam and initial head CT
found a total right internal carotid artery occlusion and a
right anterior cerebral artery and middle cerebral artery
stroke with significant associated edema. She underwent
a decompressive craniotomy due to the concern for potential herniation. Echocardiography revealed a large
mitral valve vegetation consistent with Libman-Sacks
endocarditis and she subsequently underwent a mitral
valvuloplasty. Because her laboratories showed an elevated INR and thrombocytopenia, she underwent an aggressive hematology workup revealing a positive ANA,
DRVVT, beta-2 glycoprotein, and anticardiolipin IgG to
790. Initial urinalysis was also significant for proteinuria
and hematuria. The patient was diagnosed with antiphospholipid syndrome and initiated on anticoagulation given
her procoagulable state. She subsequently developed severe thrombocytopenia and anemia without evidence of
heparin-induced thrombocytopenia. She was found to
have Coombs' positive autoimmune hemolytic anemia
and was treated with steroids and intravenous immunoglobulin with resultant improvement in her thrombocytopenia and anemia. She was diagnosed with systemic
lupus erythematous given a positive ANA, positive antiphospholipid antibodies, proteinuria, and thrombocytopenia.
Discussion: Catastrophic antiphospholipid syndrome is a
rare presentation of a small subset of patients with antiphospholipid syndrome. Currently proposed criteria
include involvement of at least three organs, rapid development of sequalae (less than one week), histopathologic
confirmation of small vessel occlusion, and positive antiphospholipid antibodies. Comprehensive therapy including anticoagulation, steroids, plasmapheresis, and
intravenous immunoglobulin has been associated with
improved outcomes. In patients who survive the initial
catastrophic event, long-term anticoagulation is necessary
to prevent further thromboembolic events.
74
two cases, I’m illustrating the complexities in the workup
of eosinophilia.
A Case of Elephantiasic Nodular Pretibial Myxedema
E.G. Yan, MD; Ronald S. Swerdloff, MD
Harbor-UCLA Medical Center
Case: A 46-year-old woman with hypothyroidism after
radioactive iodine ablation for Graves' disease twenty
years ago presented with bilateral lower extremity edema.
Since her therapy, the patient had been non-adherent to
levothyroxine and subsequently developed dryness to her
hands and lower extremities. On review of systems, she
endorsed chronic fatigue, cold intolerance, constipation,
slow speech but was remarkably thin. Of note, her mother
also was hypothyroid after a similar radioablation therapy
for Graves' disease in the 1950s. The examination was
notable for hypothermia with a temperature of 95.8, pulse
78, and blood pressure 111/56. The patient was disheveled and malodorus. She had mild proptosis, and the thyroid was non-palpable. The lower extremities were
grossly edematous with several large, tumorous growths
around the circumferential distal limbs with overlying
erythema, brawny induration, and areas of necrotic tissue.
The skin on her hands were erythematous and dry with
deep fissures and thinning of the hair. The laboratory
results were significant for a white blood count 20.7, thyroid simulating hormone168, free T4 <0.4, and free T3
1.9. The patient was started on levothyroxine 100 mcg
daily, and was diagnosed with elephantiasic nodular
pretibial myxedema. Given the extensive swelling to her
lower extremities, she underwent surgical debridement of
the necrotic tissue and was instructed to wear compressive bandages to promote lymphatic flow. Pathological
examination with colloidal iron did not demonstrate glycosaminoglycan, which would confirm the diagnosis of
pretibial myxedema. Over the course of the next several
months, the skin to her lower extremities eventually softened and the swelling decreased markedly.
Discussion: Pretibial myxedema is an uncommon presentation of Graves’ disease, occurring approximately 5% of
the time with the elephantiasic nodular type being rare.
The cause of this dermopathy is unclear but is known to
preferentially involve the pretibial aspects of the legs.
Fibroblast-simulating factors, antibodies against fibroblasts, and production of glycosaminoglycans have been
suggested. Additionally, proptosis is invariably seen with
pretibial myxedema since the deposition of glycosaminoglycans behind the eyes leads to this condition. The
diagnosis can be made based on clinical presentation with
the associated findings of Graves' disease and confimed
by the presence of glycosaminoglycans, but it is unclear
why the patient’s tissue was
stain-negative. Patients may have clinical hyperthyroidism while others are rendered hypothyroid before the
onset of pretibial myxedema. In addition to the therapies
mentioned previously, other modalities include routine
topical corticosteroids under occlusion with plastic wrap
or systemic immunotherapy. Upon review of the litera-
ture, to the best of our knowledge, there have been no
prospective trials evaluating the efficacies of various
therapies for this advanced stage of thyroid dermopathy.
Management options have largely been based on anecdotal evidence and case series.
A Case of Microscopic Polyangitis Refractory to Steroids and Immunosuppressants
Wan-ting Yang, MD; Nader Kamangar, MD, FACP,
FCCP
Olive View-UCLA Medical Center
Case: A 57 year old Filipino female with no past medical
history presented with complaints of fever, night sweats,
20 pound weight loss, abdominal pain, and left lower leg
numbness for 2 months. Her initial labs were notable for
renal failure of unclear chronicity, normocytic anemia,
and leukocytosis with bandemia. Her initial urine analysis
showed trace protein, 1 RBC, and 1 RBC cast. A chest
CT was significant for bilateral upper lobe centrilobular
nodules and tree-in-bud opacities suspicious for an atypical infection. Her hospital course was complicated by
acute respiratory distress requiring intubation. Fiberoptic
bronchoscopy with bronchoalveolar lavage revealed sequentially bloody aliquots consistent with diffuse alveolar
hemorrhage (DAH). Her renal failure had also progressed
requiring hemodialysis. Her laboratory work up was significant for an elevated CRP, low total complement,
negative ANA, negative GBM Ab, negative Proteinase3Ab, and a positive Myeloperoxidase (MPO-ANCA)
value of >100. The infectious work up was negative with
the exception of strongyloides stercoralis larvae found in
her stools but not in the BAL, and the BAL findings were
only significant for inflammatory cells on cytology. In
light of these abnormal findings, she was started on the
treatment for microscopic polyangitis (MPA) that included three days of pulse steroids with cyclophosphamide followed by maintenance steroids. She responded immediately to the steroids and was clinically
stable until day 9 when she suddenly developed respiratory distress with recurrent hemoptysis leading to emergent intubation. She was again given the same therapy of
pulse steroids and cyclophosphamide. Once again her
clinical response was immediate, but two days later she
redeveloped hemoptysis and respiratory distress requiring
reintubation. A repeat bronchoscopy was done and reconfirmed the DAH. A decision was then made to initiate
plasmapharesis in addition to the corticosteroids and
cyclophosphamide.
Discussion: ANCA-associated vasculitis is an important
differential for patients with hemoptysis. Wegener’s
granulomatosis (WG) and microscopic polyangitis
(MPA) are medium- to small-size vasculitides, and patients classically present with diffuse alveolar hemorrhage and glomerulonephritis. The diagnosis is suggested
by the presence of antineutrophil cytoplasmic antibodies
(ANCA), which can be detected by immunofluorescence
and ELISA. The indirect immunofluorescence assay reveals 2 types of staining patterns: cytoplasmic (c-ANCA)
75
and perinuclear (p-ANCA). The ELISA detects the antibodies against Proteinase 3 (PR3) and Myeloperoxidase
(MPO), which are responsible for the cytoplasmic and
perinuclear staining patterns respectively. This is an interesting case of MPA with a classic presentation and
diagnostic findings but is unresponsive to conventional
treatment. Plasmapharesis is not traditionally the first line
therapy for MPA, but there has been reported cases showing its benefit when patients are not in remission on steroids and immunosuppressive therapy.
Urgent dialysis was critical in the improved mental status
by removing lactate and repleting bicarbonate. This case
also illustrates several potentially life-saving techniques
that can be applied when encountering severe metabolic
acidosis.
Oral Contrast Induced Acute Respiratory Distress
Syndrome
Joseph Yeh, MD; Seth Rivera, MD
University of California, Los Angeles
“Basic” Treatment of Metabolic Acidosis
Neda Yazdi, MD; Stanley Amundson, MD
Scripps Mercy Hospital
Case: A 67 year old female presented to the Emergency
Department complaining of 1-day history of lethargy and
visual changes specifically that surrounding appeared
dark. Her medical history is pertinent for diabetes
mellitus type 2, hypertension, and coronary artery disease
status post CABG. Two days prior to admission she had a
few episodes of nausea, vomitting, and diarrhea but
continued taking her medications. Her medications
included Metformin, Glipizide, Lisinopril, Metoprolol,
Aspirin, HCTZ, and Lipitor, none of which were recently
prescribed or adjusted. On examination, the patient had
SBP 100/60 and tachypnea on nonrebreather mask. She
was alert and oriented, speaking full sentences but
speaking slowly. Exam was essentially unremarkable.
Arterial blood gas was significant for pH 6.64, CO2 15,
HCO3 <5, O2 saturation 97% on 32% O2. Basic
metabolic panel was significant for Cr 7.3 with baseline
Cr 1.6. The patient had a severe anion gap acidosis likely
MALA. Beta hydroxybutyrate was 6.5, lactic acid 20 and
positive serum osmolar gap greater than 30. Urinalysis
showed specific gravity greater than 1.030. Nephrology
was consulted for urgent dialysis and bicarbonate drip
was started. Within 24 hours of admission, the patient
was feeling nearly at her baseline state and her creatinine
quickly improved to baseline function within 48 hours.
The cause of the acute renal failure was likely secondary
to prerenal etiology from viral gastroenteritis and
continuation of blood pressure medications.
Discussion: Metformin associated lactic acidosis
(MALA) is a rare condition with an estimated prevalence
of one to five cases per 100,000. Accumulation of
metformin alone is rarely reported as cause as a cause of
lactic acidosis, and tissue hypoxia is the usual cause.
Acute lactic acidosis is a known complication of
metformin. This case is unique both in severity of
acidosis and that the patient was concious at presentation.
The risks and benefits of metformin need to be carefully
assessed prior to initiating the drug particularly when
used in combination of Lisinopril. Secondly, the
differential diagnosis of anion gap acidosis should be
carefully reviewed and evaluate for less common
etiologies when applicable. A large osmolar gap can be
secondary to severe metabolic acidosis but ethylene
glycol, methanol, and electrolytes need to be considered.
Case: A 28 year-old woman with end-stage renal disease
secondary to FSGS, status post three kidney transplants,
and PUD with 2-weeks of nausea, vomiting and abdominal pain. As part of her work-up, she drank oral contrast
for a CT abdomen and pelvis. She aspirated in her haste
and, shortly thereafter, developed pruritic hives on her
back which resolved with Benadryl 40mg IV and hydrocortisone 100mg IV. Her initial chest x-ray showed no
interval change. Eleven hours later, she became obtunded
and required intubation for hypoxemic and hypercapneic
respiratory failure with PaO2/FiO2 53. Her lungs were
coarse bilaterally and repeat chest x-ray showed diffuse
bilateral opacities. She was transferred to the ICU, placed
on broad spectrum antibiotics, and started on ARDSnet.
All cultures returned negative. She was given Solumedrol
125mg IV bid for suspected delayed anaphylactoid reaction. Four days after admission the ICU, she was extubated. She was discharged home eight days later with
steroid taper.
Discussion: Anaphylactoid reactions are rapid and severe
allergic reactions induced by the release of mast cell mediators such as histamine, leukotrienes and prostaglandins. Unlike traditional anaphylaxis, in anaphylactoid reactions, the offending substance directly causes the release
of contents in basophils and mast cells. This process is
not mediated by immunoglobulin E (IgE) and, consequently, does not require prior sensitization to an allergen. The most common manifestations are cutaneous
(90%) and respiratory (70%). Our patient clearly described pruritis, utricaria, cough and dyspnea during the
course of her reaction. Gastrointestinal (40%) and cardiovascular manifestations (35%) are less commonly recognized. Although the initial anaphylactoid reaction usually
occur within minutes to hours of exposure, a recurrence
of symptoms, defined as a biphasic reaction, can occur up
to 72 hours after resolution of initial symptoms. Even
though anaphylactoid reactions are a clinical diagnosis,
there are blood tests including plasma histamine and total
tryptase. Unfortunately, these tests have poor overall sensitivity (36%). Treatment requires immediate removal of
the offending agent. In an acute decompensation, ABC’s
should be addressed. Epinephrine 0.5mg IM has been
shown to be effective in anaphylactic shock. Furthermore, rapid normal saline boluses, H1 and H2 antihistamines, oxygen and steroids can be used as appropriate.
Home is Where the Heart is: Chest Pain and Syncope
in a Patient with Agoraphobia
76
Andrew Young, MD; Arta Bakshandeh, MD; Radha
Sarma, MD
University of Southern California
Introduction: Panic attacks are a discrete period of intense fear or discomfort, with a variety of symptoms including chest pain, dyspnea, palpitations, dizziness,
sweating and chills. Recurrent attacks are associated with
drastic behavioral changes & agoraphobia. We present a
patient with a diagnosis of panic disorder with agoraphobia found to have an underlying cardiac condition.
Case Presentation: A 46 year old Caucasian female with
a history of intermittent syncope since childhood presented with three day history of dyspnea, chest pain and
syncope. The patient had a history of chest pain and syncope beginning at the age of 13. These episodes, which
occurred when she went outdoors or in public, were diagnosed as panic attacks. The panic attacks stopped in her
twenties, when she developed agoraphobia and began
spending most of her time at home. Since that time she
has led a generally sedentary lifestyle. A year before admission the patient began having similar episodes again.
On physical exam, she had a systolic murmur, loudest
along the left sternal border and increasing with valsalva
maneuvers. Work-up for syncope began with an electrocardiogram showing sinus bradycardia, negative cardiac
enzymes and a nuclear stress test which was negative.
Carotid ultrasound showed atherosclerotic disease without significant stenosis. An echocardiogram showed hypertrophy, an ejection fraction of 70% and a left ventricular outflow gradient of 19 mmHg that increased to 81
mmHg with valsalva. A diagnosis of obstructive Hypertrophic Cardiomyopathy (HCM) was made. An exercise
treadmill test showed moderate functional capacity. The
patient was treated with diltiazem, advised to restrict her
physical activity, to stay well hydrated and to have her
family screened for HCM.
Discussion: Hypertrophic Cardiomyopathy (HCM) is a
disease that is a well-described autosomal dominant genetic disorder in the cardiac sarcomere. It is associated
with a spectrum of manifestations ranging from no symptoms to chest pain, syncope and sudden cardiac death
(SCD). Although the overall annual incidence of SCD in
patient with HCM is approximately 1 percent, there are
features that increase a patient’s risk of SCD. These include prior cardiac arrest or arrhythmias, a family history
of SCD, syncope (especially if exercise-related), outflow
obstruction and an abnormal blood pressure response to
exercise. Physical activity restriction, specifically
avoiding high-intensity sports and activities, is recommended for patients with HCM. Patients at higher risk for
SCD may be considered for an implantable defibrillator.
Our patient’s panic attacks were likely manifestations of
undiagnosed HCM, and her agoraphobia effectively restricted her activities, perhaps saving her from SCD. This
case demonstrates not only how the diagnosing of HCM
can be missed, but also how if left undiagnosed HCM can
be at least debilitating and potentially life-threatening.
Research Abstracts
Patent Foramen Ovale Size as a Predictor of Stroke
Size in Patients with Paradoxical Embolization
Andre Akhondi, MD; Rubine Gevorgyan, MD; Leo
Slavin, MD; Catherine Dao, MD; David S. Liebeskind, MD; Jonathan M. Tobis, MD
David Geffen School of Medicine at UCLA
Background: Patent foramen ovale (PFO) has been implicated in the pathogenesis of cryptogenic stroke via
paradoxical embolization to the cerebral circulation. The
criteria for PFO closure in stroke prevention have not
been established. The purpose of this study was to evaluate the relationship between the morphological and functional size of the PFO and cerebral infarct volume identified on magnetic resonance imaging (MRI).
Methods: Patients who were referred to the UCLA Interventional Cardiology Program with the diagnosis of cryptogenic stroke and who had both a Transesophageal
echocardiogram (TEE), intracardiac echo (ICE) and a
brain MRI at the time of stroke were selected. TEE or
ICE were used to obtain PFO measurements, specifically
PFO opening height, length, and the thickness of the septum secundum. The degree of right-to-left shunt was
quantified by agitated saline injection. MRI of the brain
with three sequences (T2, DWI, and FLAIR) was used to
diagnose acute stroke and measure the infarct volume.
Results: In the 62 patients studied, the average measured
stroke volume was 8.2 cm3 on DWI, 7.9 cm3 on T2, and
8.2 cm3 on FLAIR. There was no significant correlation
between the PFO height, length, septum secundum thickness, septal excursion distance, the presence of atrial septal aneurysm, or echo bubble grade compared with the
infarct volume measured from the three MRI sequences
(p = NS).
Conclusions: This analysis revealed no significant association between PFO size or function by echo and brain
infarct volume. The size of PFO by echo or right-to-left
shunt on bubble study cannot be used to predict the potential size of subsequent strokes. Therefore PFO size
should not be used to decide if a PFO should be closed.
We conclude that the size of the thrombus is the major
determinant of stroke size due to paradoxical embolism.
The PFO size, presence of atrial septal aneurysm or function by echo does not appear to be the limiting factor for
a thrombus to pass from the right atrium (RA) to left
atrium (LA).
Prevalence and Screening Methods for Depression in
Chronic Heart Failure Patients
Andre B. Akhondi, MD; Haneen Khamag, MD; Aurelia Macabasco-O'Connell, PhD, RN; Shahriar
Pirouz, MD; Tracy Finegan, NP, RN; Mark McDon-
77
nell, MD, MBA; Robert Bryg, MD; Sheba Meymandi,
MD, FACC
Olive View-UCLA Medical Center
Introduction: The association between chronic heart
failure (CHF) and depression are well documented and
associated with poor prognosis and poor quality of life.
However, little is known about this relationship within
the Hispanic population and non-Hispanic population.
Therefore, the purpose of this study is to determine the
prevalence of depression in patients with CHF.
Methods: A total sample of 274 patients (46% Hispanic
54% non-Hispanic, 75% male, mean age± SD was
52.6±13, 60% had less than a high school education, 61%
unemployed, 75% with non-ischemic cardiomyopathy,
and 34% in New York Heart Association (NYHA) Class
III-IV) were enrolled in a CHF Disease Management
Program (DMP) at a Los Angeles County Facility. Each
patient underwent a thorough initial screening evaluation,
including sociodemographic information and clinical
history, baseline ejection fraction, and NYHA functional
class. All patients were asked two depression screening
questions from the Patient Health Questionnaire-2
(PHQ2). If the patient responded yes to either, they were
asked to complete the Patient Health Questionnaire-9
(PHQ9), which is a validated depression screening tool.
Results: A total of 150 subjects (75%) reported having
depressive symptoms: 25% with minimal, 26% mild,
16.5% moderate and 8.5% severe. Mean PHQ9 scores for
the total sample was 9.85±6.6. Hispanics revealed more
severe depressive symptoms than non-Hispanics with
mean PHQ9 scores of 11.08±6 vs 8.74±6, (p=0.007) with
moderate and severe symptoms (61% vs. 39%, moderate;
69% vs. 31%, severe, p=0.04). More Hispanics had less
than a high school education (<12 years) than nonHispanics (63% vs. 37%, respectively, p<0.001) while
more non-Hispanics were unemployed (55% vs. 45%,
respectively, p=0.008). There is a significant relationship
between depression severity and initial NYHA Classification with those in NYHA class III and IV revealing
higher levels of depressive symptoms [p<.001].
Conclusion: A high prevalence of depressive symptoms
was found in both Hispanic and non-Hispanic patients
with CHF. It appears that Hispanics tend to have more
severe depressive symptoms when compared to nonHispanics. Better outcomes in health care, particularly in
Hispanics, may result from incorporating depression
screening and management protocols into the standard
treatment of CHF.
How Does Structured Data Entry Affect Measured
Quality of Care: A Case Study in Patients with Falls
& Mobility Disorders
Shone Almeida, BS; Carol P. Roth, NP, MPH; David
B. Reuben, MD; Neil S. Wenger, MD, MPH; David A.
Ganz, MD, PhD
University of California, Los Angeles
Background: Structured data entry and electronic medical records are increasingly prevalent in the healthcare
industry. However, an understanding of how structured
data affects quality measurement and patient care is unknown. This study examines how structured visit notes
(SVNs) are utilized and how they affect measured quality
of care across 3 clinics in the Assessing Care of Vulnerable Elders: Practice Redesign for Improved Medical
Care for Elders (ACOVEprime) study. Focusing specifically on patients age; 75 who screened positive for falls
or fear of falling, we had 3 primary objectives: 1) Compare measured quality of care between patients with and
without an SVN as determined from previously calculated ACOVEprime quality scores 2) Compare how thorough physicians were in completing different sections of
the SVN, and 3) Evaluate the SVN’s potential for acting
as a surrogate for full-chart reviews in analyzing quality
of care.
Methods: Across the 3 clinic sites (Salem, Canandaigua,
and Scranton), we reviewed 226 primary care records of
patients who had screened positive for falls or fear of
falling. We checked for the presence of the SVN and, if
present, we abstracted patient data from it. We calculated
descriptive statistics regarding how often various sections
of the SVN were utilized and developed algorithms for
measuring quality of care for falls solely from the SVN.
We compared our algorithms with previously calculated
quality scores based on abstraction of the whole chart.
Results: Of 226 patient charts, 41% contained the structured visit note. Among those charts containing the SVN,
Salem, Canandaigua, and Scranton had quality scores of
87%, 89%, and 74%, respectively. Without an SVN, the
quality scores were 30% and 45% for Salem and Canandaigua, respectively; similar results for Scranton cannot
be computed since it was a fully-electronic site. The
“History” and “Exam” sections of the SVN had much
higher rates of utilization (80.9% and 70.9% respectively)
than the “Assessment” and “Plan” components (16.8%
and 20.8% respectively). An average of 55% of the charts
had identical quality scores using SVN-based and fullchart based quality measurement algorithms, while the
SVN was shown to underestimate chart-based quality in
34% of patients and overestimate in 10%.
Conclusion: Structured data entry may be a means of
both prompting appropriate care as well as capturing
quality data without relying on a full-chart review. Although this study is limited by lack of direct observation
of how structured data entry affected physicians’ workflow, we hope to conduct additional research along these
lines in the future.
Is There an Association Between Premature Ventricular Contractions and LV Dysfunction?
Kandan Baban, MD; T. Haywood, MD
Scripps Green Hospital
78
Introduction: Isolated premature ventricular contractions
(PVCs) are commonly encountered in clinical practice.
They are typically considered benign entities brought on
by a multitude of factors. More recent studies have demonstrated that there may be a causal relationship between
frequent PVCs and left ventricular dysfunction, even dilated cardiomyopathy. The hypothesis of PVC-induced
cardiomyopathy is based on the observation that there is
improvement in LV function after suppression of the ectopic focus with medication or radiofrequency ablations.
We decided to evaluate our own population of patients at
Cardiology division at Scripps Green Hospital for frequent PVCs and determine if there is any significant decrease in ejection fraction (EF).
Crohn’s disease (CD) is not as well characterized. Colon
cancer was first reported as a complication of “cicatrizing
enteritis” (regional enteritis) in 1948 and has been described in a number of reports. In fact, there is data to
support that the risk of colorectal cancer is similar in
Crohn’s colitis (CC) and UC of similar duration and severity, and significantly higher in CC than in the general
population. While outcomes of screening and surveillance colonoscopy with biopsy have been widely reported
in patients with UC, only one study exists, to the best of
our knowledge, which evaluates these outcomes in CD.
In this study, we report a high detection rate of dysplasia
and cancer in chronic extensive CC. Herein, we report
our colonoscopy and biopsy findings in CC.
Method: We conducted a retrospective, Case-Controlled
chart review and statistical analysis of the effect of frequent PVC (as defined by PVC >10,000) on left ventricular ejection fraction. 1024 holter monitors were evaluated
for the presence or absence of more than 10,000 premature ventricular contractions in a 24 hour period from
December 2007 through August 2008. Study population
consisted of 50 patients (avg. age 70 +/- 0.6) with PVC >
10,000. 42% of patients had coronary artery disease.
Controlled population consisted of 100 patients (average
age of 68 +/- 0.6) with PVC < 5,000. Two controls were
matched to each case, the matching factors being sex,
presence or absence of CAD, and age (+/- two years).
Transthoracic echocardiograms within 2 years of 24
hours holter monitoring were compared for EF in both
study populations and controls. Once data was assembled
we compared cases with 2 matched controls to evaluate
for a significant difference in EF.
Methods: This is a retrospective study that involves prospectively collected data. A CD patient database at a
large inflammatory bowel disease (IBD) center was reviewed for colonoscopies and biopsies performed between January 1995 to July 2009 from patients with a
long duration of disease. Those who had colonoscopies
presumably had screening and surveillance for colonic
CD, not colorectal cancer. During colonoscopy, random
biopsies were typically taken at 10 cm intervals, as well
as from strictures, masses, or polyploid lesions. Disease
distal to the splenic flexure was defined as left-sided.
Biopsy pathology was specified as normal, polyp (i.e.
neoplasia without dysplasia), indefinite for dysplasia
(ID), low-grade dysplasia (LGD), high-grade dysplasia
(HGD), or carcinoma (CA). ID is included in our definition of dysplasia.
Results: Patients with PVC’s > 10,000 had lower mean
ejection fraction (57 +/- 14%) than patients with PVC <
5,000 (EF 64 +/-14%) with P value of 0.002. Conclusion:
There is clear evidence from recent literature and our data
that frequent PVCs place an individual at increased risk
for left ventricular dysfunction and the development of
significant cardiomyopathy. Patients with frequent PVC
>10,000 were more likely to have abnormal EF. Our data
support that frequent PVCs are associated with a reduced
ejection fraction. Although the study is suggestive of causality there is no way to prove a clear relationship. Future
goal of our study is a prospective follow up over time of
the LV function in patient with >10000 PVCs, who have
no current evidence of LV dysfunction.
Colorectal Cancer in Patients with Crohn’s Colitis
Robert J. Basseri, MD; Benjamin Basseri, MD; Konstantinos Papadakis, MD
Department of Gastroenterology, Cedars-Sinai Medical Center
Introduction: Chronic ulcerative colitis (UC) is an established risk factor for colonic neoplasms and carcinoma.
This observation has resulted in the common practice of
surveillance colonoscopies with biopsies for patients with
UC. Contrastingly, the risk of dysplasia and carcinoma in
Results: 411 patients were included in the study, with a
total of 869 colonoscopies. The mean number of examinations per patient was 2.2 (range, 1-8), 19.5 months
apart and 74 patients (18%) were found to have colorectal
neoplasia. This shines light on a group previously not
associated with higher incidence CRC. The study population was composed of 50% females (Table 1). Mean age
at diagnosis was 26.7 years, with a mean disease duration
of 19.4 years. In those with neoplasia, eleven percent had
a first degree relative with inflammatory bowel disease
and only twenty five percent presented with increased
symptoms on colonoscopy. Age greater than 45 years
correlated with a two fold incidence of colorectal cancer,
while disease duration and earlier onset of CC were also
risk factors for developing CRC.
Conclusion: There is a significant incidence of colorectal
cancer in patients with Crohn’s colitis, demonstrating that
screening and surveillance in Crohn’s patient population
could benefit risk reduction of colorectal cancer.
MRSA Colonization Rates among Housestaff in Different Departments at a University County Hospital
Lex Chen, MD; Arthur Jeng, MD
Olive View- UCLA Medical Center
79
Background: Once primarily contracted in the hospital
setting,
methicillin-resistent Staphylococcus
aureus (MRSA) has now become prevalent in the community setting, causing infections in otherwise healthy
individuals. Because of this prevalence in the community
and the greater exposure to patients with MRSA, concern
exists that healthcare workers may themselves be colonized. In 41 studies that provided data on the nasal colonization with MRSA, 4.6% (1545 of 33318) of healthcare
workers were found to be colonized with MRSA; however, no studies have targeted resident physicians. Current infection control guidelines mandate the screening of
all inpatients and the cohorting and isolation of MRSAcolonized patients. Many physicians believe this to be a
costly and unnecessary measure, because they feel health
care providers are also likely to be colonized, but are not
isolated per se. Also unknown is whether a difference in
MRSA colonization exists among the various departments, as exposure and interaction with patients in the
different medical specialties vary tremendously.
Methods: This study was conducted at Olive ViewUCLA Medical Center, a county hospital of Los Angeles.
Swabs from the anterior nares were collected from all
housestaff rotating through the hospital during the study
period, which included those from the departments of
medicine, surgery, OB-gyn, emergency medicine, and
pediatrics. All participants were asked to fill out a standardized survey regarding their perceptions about MRSA.
Results: Eighty-two resident physicians (post graduate
year 1-6, 34 males, 48 females), including 57 medicine,
10 surgery/OG-gyn, 10 emergency medicine, and 5 pediatric residents; three housestaff refused participation. Of
82 residents, 1 from the department of medicine was
found to be positive for MRSA, giving a 1.2% housestaff
colonization rate during the study period. No emergency
room residents were found to be colonized, despite the
lack of patient isolation/formal infection control procedures (gowning) in that department. Of the 82 housestaff
physicians, 64 responded to the pretest survey. The majority of participants (46/64, 73%) believed that they
were colonized with MRSA, and 34% believed that that
41-60% of their colleagues were also colonized with
MRSA. Nine participants (9/64, 14%) answered correctly
that 0-20% of their fellow residents were colonized with
MRSA.
Conclusion: This study revealed that despite a community MRSA prevalence of 50% in Staphylococcus aureus
isolates, housestaff caring for such patients have very low
rates of MRSA carriage in the nares, even among those in
hospital areas where infection control is difficult (emergency department). No difference in MRSA colonization
rates was seen among the various departments. These
findings lead credence to the basis for the current patient
isolation recommendations, despite the prevailing beliefs
among housestaff.
Is There a Role for Coronary Calcium Screening in
Diabetes Patients Younger than 40 Years Old to Identify a Statin In-Need Group?
Nikhil Daga, MD; Hussain Ismaeel, MD; Yasmin
Hamirani, MD; Jigar Kadakia, MD; Philip Bach,
MD; Matthew Budoff, MD
Harbor-UCLA Medical Center
Background: The ADA recommends statin use in diabetes patients > 40 years old with multiple cardiovascular
risk factors. Coronary artery calcium (CAC) is a very
sensitive surrogate of CAD and is a powerful and independent predictor of mortality and cardiac events. In this
retrospective study we aimed to report the prevalence of
CAC among those < 40 y.o. and to describe the determinants of CAC in diabetics.
Methods: The charts of 2142 diabetic patients who had
undergone CAC scoring at our center were reviewed.
Their clinical and demographic data were collected. CAC
score (CACS) was categorized into CAC 0= normal, 1-99
low, 100-399 intermediate and >=400 severe; and statistical analysis was performed.
Results: The overall prevalence of CACS is 83%; in
those < 40 y.o, prevalence of CACS 45%. Approximately
35% of diabetics had a severe CACS measurement
(>400) - none < 40 years old. CACS median severity
increased generally with age, with median CACS >100
seen in patients > 61-65 y.o, our mean and median age.
Gender, male>female and hypertensive patients had statistically significant (SS) greater (p<0.001) CACS medians. Initially, ethnicity median CACS were SD (p<0.024)
between Asians vs. Caucasians, Hispanics, and Others,
but age was a confounding risk factor. SS differences in
median CACS were not associated with family hx
(p<0.177), and smoking (p<0.185). Odds ratios (OR)
calculated for low, intermediate, and high CACS category values compared to age were 1.046 (p<0.001), 1.099
(p<0.001), and 1.119 (p<0.001), respectively; and compared to female gender were 0.525 (p<0.003), 0.328
(p<0.001), and 0.180 (p<0.001), respectively. Finally, OR
for intermediate and high CACS compared to not having
hypertension were 0.496 (p<0.006) and 0.477 (p<0.002),
respectively.
Conclusion: CACS were markedly higher among male
diabetics and hypertensive diabetic patients independently. Age in diabetic patients only had a minor, but significant roll with regards to CAC.
Oxidized High-Density Lipoprotein (HDL) from Patients with Coronary Artery Disease Can Regain its
Anti-Inflammatory Properties Using HDL Mimetic
Peptides
Omeed Elboudwarej; Susan Hama, B.Sc; Greg
Hough, MS; Mckenna Garife; Mohamad Navab, PhD
University of California, Los Angeles
80
Introduction: Plasma levels of high-density lipoprotein
(HDL) are known to inversely correlate with the incidence of coronary artery disease (CAD). The protective
function of HDL is attributed to its role in reverse cholesterol transport from peripheral tissues for elimination,
preventing lipid oxidation and inhibiting the formation of
inflammatory molecules. Under inflammatory conditions,
however, HDL can lose its beneficial properties and become pro-inflammatory.
Methods: In the current study, we used plasma from 13
CAD patients and 10 healthy controls to determine if
treatment with HDL mimetic peptides L-4F, apoJ[113122] or the tetrapeptide FREL would lower the HDL inflammatory index (HII). We used a bioassay in which
normal low-density lipoprotein (LDL) from healthy controls was added to human aortic endothelial cultures to
determine the extent of lipid oxidation in LDL and the
resulting induction of monocyte chemotactic protein 1
(MCP-1).
Results: In the absence of HDL, the cells oxidized the
lipids in LDL, which in turn stimulated the cells to produce MCP-1. In the presence of normal HDL, the extent
of LDL oxidation and thus MCP-1 production was reduced. HDL from CAD patients, however, had a high HII
and actually amplified LDL oxidation. Treatment of both
patient plasma and control plasma with mimetic peptides
resulted in patient HDL preventing LDL oxidation and
MCP-1 induction (i.e. resulted in HDL with low HII).
Conclusion: HDL mimetic peptides can restore the antiinflammatory properties of HDL from patients with
CAD, suggesting a potential therapeutic benefit using
these mimetic peptides.
Barrett's Esophagus Eradication by Radiofrequency
and Cryoablation
Avegail Gascon-Flores, MD; Sofiya Reicher; David S.
Chung; Binh V. Pham; Viktor E. Eysselein
Harbor-UCLA Medical Center
Background and Aims: Radiofrequency ablation (RFA)
with Halo devices (Barrx Inc) and cryoablation (CSA
Medical) with liquid nitrogen/low pressure system are
emerging as effective & safe techniques for Barrett's
esophagus (BE) eradication. However, in our experience,
RFA can be technically challenging in the distal esophagus, especially in the presence of moderate to large hiatal
hernia. Here, we report interim analysis of a study aimed
to evaluate the efficacy and safety of RFA with addition
of cryoablation for nondysplastic and dysplastic BE.
Methods: From 2005-2008, 41 patients with histologically confirmed BE (dysplastic and nondysplastic; short
and long segment) were included in the study. All initially received RFA with follow-up endoscopy at 3
months. Patients with endoscopically visible BE on follow up underwent repeated RFA. Plate-based Halo-90
ablation device was used for small (<1 cm) BE islands;
otherwise, balloon-based Halo-360 was used. If there was
no endoscopically visible BE, 4-quadrant biopsies every
1 cm were performed. Cryoablation was performed if BE
persisted after at least 2 RFA sessions. Cryoablation was
done at 10-sec freeze/thaw cycles (4 cycles per each BE
lesion).
Results: 41 patients received at least one RFA session.
32 patients had no dysplasia, 3 patients were indeterminate, 5 low grade and 1 high grade. 6 patients were lost to
follow up. Mean BE length was 4.4cm (1-14 cm). To
date, 12 patients (1 high grade, 3 low grade, and 8
nondysplastic) achieved complete BE eradication endoscopically and histologically with all negative biopsies.
The average number of RFA sessions in this group was
1.8. One patient with low grade dysplasia had small residual islands of BE in distal esophagus after 4 RFA sessions, which were successfully eradicated after one
cryoablation. 7 more patients (1 low grade, 1 indeterminate and 5 nondysplastic) had small residual islands of
BE after the average of 2.3 RFA sessions and thus received cryoablation. Follow-up endoscopy is pending at
this time. 4 patients with residual BE or no change in BE
after the average of 2.6 RFA sessions are planned for
cryoablation. 12 patients await the second RFA. To date
we have found no major complications when using dual
ablative therapies.
Conclusion: RFA and cryoablation are both promising
new techniques for BE eradication. Our rates of BE
eradication after RFA are similar to previous reports. Our
study highlights the use of cryoablation as an adjunct tool
to RFA, in particular in distal esophagus where contact
with RFA devices can be hampered.
Studying the Ferroportin Expression in the Eyes and
the Brain
Matthew Hakimi, BS; Tomas Ganz, MD, PhD
University of California, Los Angeles
Hepcidin is a small peptide hormone that is the principal
regulator of iron absorption and distribution among tissues. Hepcidin binds to ferroportin (FPN), its receptor, on
membranes of duodenal enterocytes, macrophages and
hepatocytes. These cells normally export iron into plasma
through ferroportin. Upon binding hepcidin, ferroportin is
endocytosed and degraded, and iron export to plasma is
diminished. Continued consumption of iron then decreases plasma iron levels. Our understanding of iron
regulation is limited to the periphery and little is known
about iron regulation inside the blood-brain-barrier. Several pathologies such as Parkinson’s disease, Friedrich’s
ataxia, age-related macular degeneration and possibly
Restless Legs Syndrome are linked to iron homeostasis
imbalance in this region. To determine how the hepcidinFPN system functions in the CNS, I am analyzing the
expression of ferroportin on the different cells within the
retina and brain in knock-in mice whose endogenous FPN
has been tagged with green fluorescent protein (GFP).
FPN-GFP may be directly observable in highly express-
81
ing tissues under fluorescence microscopy, or in lowerexpressing cells by immunostaining for GFP. Detecting
protein expression in tissues requires fixing them in a
solution of formaldehyde in order to immobilize proteins.
Because this step can interfere with GFP detection by
changing the protein’s conformation, I assessed a variety
of fixation techniques to detect their effects on GFP fluorescence. I grew HEK 293(Human Embryonic Kidney)
cells that were expressing FPN-GFP on Poly-D-Lysine
chamber slides and multi-well plates and induced them
with ponasterone A. Fixatives at different concentrations
(4% and 10% formalin, 4% paraformaldehyde) were used
and fixations were stopped at different times (10 minutes
up to 24 hours) and analyzed by epifluorescent microscopy and flow cytometry for GFP signals. These experiments suggest an optimal GFP signal at 10 minutes of
fixation in 4% formalin, with a decline in GFP fluorescence intensity as fixation time is increased. Based on my
experiments on wild-type mice, ferroportin-rich organs
including the spleen and the duodenum can be harvested
fresh and observed by immunofluorescence after 10 min
fixation with 4% formalin and disaggregation. More delicate organs such as the eye will be cryosectioned. After
the mice are killed, I remove the eyes, take out the cornea
and lens under a dissecting microscope, immerse them in
a fixative solution for 10 minutes, then cryoprotect them
in sucrose before freezing them in the OCT freezing media at -80°c overnight. Then, using a cryostat machine, I
section the eyes at 7 micron thickness and stain with specific antibodies for markers of different cell types. I am
using immunohistochemistry with anti-ferroportin antibodies as well as markers for the cell types which are
suspected to express FPN at moderate levels, namely
photoreceptor cells, Müller and retinal pigment epithelium (RPE) cells.
Yield of Colonoscopy in Patients with Metastatic Cancer of Unknown Primary
Andrew Ho, MD; Brennan Spiegel, MD, MSHS
Greater Los Angeles VA Healthcare System
Introduction: Metastatic cancer of unknown primary(MCUP) is a diagnosis given to patients with evidence of cancer that has already spread in the body, but
where the original or primary site of cancer has not been
determined based on history , physical exam, laboratory
testing and imaging studies. MCUP accounts for 2-10%
of cancer diagnoses and since colon cancer is one of the
most common cancers in the U.S. , gastroenterologists
are often asked to perform a colonoscopy to evaluate if
colon cancer is the primary site of metastasis. Despite this
common practice, in searching through databases(Pubmed, Ovid, Cochrane), there have been no studies on the utility of colonoscopy for MCUP.
Methods: Patients having a colonoscopy with indication
of MCUP were eligible. Exclusion criteria included prior
colon cancer, familial colon cancer syndromes, positive
fecal occult blood test, iron deficiency anemia, or imaging study suggesting colonic abnormality. Colonoscopy
reports from 2000-2009 for inpatients and outpatients at
the West Los Angeles VA were reviewed retrospectively
to identify eligible patients. This study was in conjunction with a concurrent study at LAC-USC Medical Center.
Results: 15 patients were identified. Mean age was 59
years (range 40-74). 1 was female. 10 of the 15 were
Caucasian. 8 of the patients died with the mean survival
of 236 days(~8 months) after colonoscopy performed.
None of the 15 patients were identified as having primary
colon cancer. 5 of the 15 had the final diagnosis of
MCUP despite a thorough workup including biopsies. 3of
the 15 were found to have a lung primary. 2of the 15
were found to have a liver primary. 2 of the 15 were
found to have a prostate primary. The remaining patients
had as their primary cancer, peritoneal, bladder and pancreatic cancer.
Conclusions: Patients with MCUP but no standard indications for diagnostic colonoscopy rarely have primary
colon cancer. Patients with colonic abnormality on imaging were more likely to have colon cancer as their primary site and further studies are needed. We would not
recommend routine colonoscopy for MCUP at this time.
Comparison of Coronary Artery Bypass Graft Surgery with Drug-Eluting Stenting in Diabetic Patients
with Multivessel Disease: Meta-Analysis of Clinical
Studies
Zahid Iqbal, BS; Michael Lee, MD
David Geffen School of Medicine at UCLA
Objectives: This meta-analysis was undertaken to assess
the efficacy and safety of coronary artery bypass graft
(CABG) surgery compared with drug-eluting stenting
(DES) in diabetic patients with multivessel disease.
Background: Previous studies that compared the merits
of CABG with PCI suggested that CABG may be the
preferred revascularization strategy in diabetic patients.
Drug-eluting stents reduce the rate of target vessel revascularization compared with bare metal stents. The ideal
revascularization strategy for diabetic patients with multivessel disease in the DES era is unknown.
Methods: A literature search was performed to identify
clinical studies comparing CABG with DES in diabetic
patients with multivessel disease with a minimum followup of one year.
Results: Five studies which included 1543 diabetic patients (CABG: n=757 and DES: n=786) met the selection
criteria. The mean follow-up was 18 months (range from
12 to 36 months). Compared with DES, CABG is associated with a lower risk of major adverse cardiac events
(odds ratio [OR] 0.48, 95% confidence interval [CI] 0.36
to 0.65), driven mainly by a lower risk of repeat revascularization (OR 0.18, 95% CI 0.11 to 0.30), despite having
82
a higher percentage of triple-vessel disease. There was no
difference in death (OR 0.85, 95% CI 0.52 to 1.39) and
myocardial infarction (OR 0.82, 95% CI 0.41 to 1.61).
Patients in the CABG group had a higher risk of cerebrovascular events (OR 2.15, 95% CI 0.99 to 4.68). There
was no significant heterogeneity of the treatment effect in
clinical subgroups.
Conclusions: In diabetic patients with multivessel disease, CABG is associated with a lower risk of major adverse cardiac events mainly driven by a lower risk of
repeat revascularization despite having more extensive
coronary disease. Mortality and myocardial infarction are
similar after CABG and DES, but CABG is associated
with a higher rate of cerebrovascular events.
Cholecystectomy for Acute Cholecystitis at Night Does
Not Result in Adverse Outcomes
Brandon Ishaque, BA; Arezou Yaghoubian, MD;
Christian DeVirgilio
David Geffen School of Medicine at UCLA
Background: In December 2008, the Institute of Medicine report on resident duty hours recommended further
cuts in duty hours to avoid fatigue related errors. The
purpose of our study was to determine whether the outcomes of laparoscopic cholecystectomy, the most frequently performed procedure by surgical residents, were
affected by the time of operation.
Methods: A retrospective review of all laparoscopic
cholecystectomies at Harbor-UCLA Medical Center for
acute cholecystitis by the Acute Care Surgery residents
was performed between 1/1/2002-3/31/2009. Data collected include patient age, ethnicity, gender, length of
hospital stay as well as need for intensive care, time of
operation, need for intraoperative cholangiogram (IOC)
and endoscopic retrograde cholangiopancreatography
(ERCP). A comparison was performed of cholecystectomies performed in the first 16 hours of the typical resident on call day (6AM-10 PM) and the last 8 hours of a
resident on call day (10 PM-6AM). Outcome measures
were complications, bile duct injury, need to convert to
an open operation, and mortality.
Results: A total of 1337 patients with acute cholecystitis
received laparoscopic cholecystectomy. 1019 patients
received the operation between 6AM-10PM (Period 1),
and 265 patients received the operation between 10PM6AM (Period 2). When comparing the two time periods,
the median age was similar between the two groups (37
vs. 36) and there were a similar proportion of females
(74.1% vs. 79.2%). The majority of patients were Hispanic (86.2% vs. 85.7%). The median length of hospital
stay was also similar (5 vs. 4 days). With respect to the
outcomes, there was a similar rate of complications for
the two periods (2.6% vs. 3.4%). One percent of patients
in the Period 1 had a bile duct injury vs. 0.4% in Period 2
(p=0.5). There was only one mortality in the total patient
population.
Conclusion: The findings of the study suggest that in the
current duty hour era, laparoscopic cholecystectomy performed at night, by residents working beyond 16 hours
does not affect outcomes. The new recommendations by
the IOM to further decrease duty hours is unlikely to result in improvement in outcomes of this frequently performed operation.
Progression Rates of Coronary Artery Calcification in
Diabetic Subjects Compared to Matched Controls
Sarkis Kiramijyan, MD; Naser Ahmadi, MD; Ferdinand Flores, MS; Khurram Nasir, MD; Matthew
Budoff, MD
Harbor-UCLA Medical Center
Background: Coronary artery calcium (CAC) is a
marker that is correlated with the presence of coronary
artery disease (CAD). This study evaluates the rates of
CAC progression between diabetics (DM) and matched
controls.
Methods: 296 asymptomatic DM and 300 matched controls (age 59±6 years, 29% female) underwent baseline
and follow-up electron beam computed tomography
within a 2 year interval. The controls were matched to the
DM with age, gender, CAD risk factors and baseline
CAC. Measurements of baseline CAC, annual changes in
CAC and ΔCAC(%) ([annual changes in CAC/ baseline
CAC] x 100) were assessed. Follow-up was completed
56±11 months after the 2nd scan. Multivariable Cox proportional hazards models were developed to predict allcause mortality.
Results: Annual change in CAC was 34.3±4.8 in
matched controls vs. 80.6±10 in DM (p=0.0001). The
ΔCAC(%) was 10.2±6.7 in matched controls vs. 29.4±8.7
in DM (p=0.0001). Hazard ratios of death in matched
controls vs. DM was 1.88 (95% CI 1.51-2.36, p=0.0001)
for 10≤ΔCAC≤20%, 2.29 (95% CI 1.56-3.38, p=0.0001)
for 20<ΔCAC≤30%, and 6.95 (95% CI 2.23-11.53,
p=0.0001) for ΔCAC>30% compared to ΔCAC<10%
independent of age, gender and CAD risk factors. The
event free survival decreased more in DM than in
matched controls in proportion to the rate of CAC progression (Figure).
Conclusion: CAC progression rate is higher in DM compared to matched controls and event free survival decreased more in DM than in matched controls in proportion to the rate of CAC progression.
Severe Cases of Pandemic H1N1 2009 (Also Known as
“Novel Influenza A H1N1” or “Swine Flu”) Requiring
Intensive Care: a Case Series from Olive View-UCLA
Medical Center
Julie Lam, MD; Tarik Ngab, MD; Richard Tennant,
MD; Nidhi Nikhanj, MD; Anita Sircar, MD; Kamyar
83
Shahedi, MD; Glenn Mathisen, MD; Suzanne Donovan, MD; Nader Kamangar, MD
Olive View-UCLA Medical Center
Background: The World Health Organization raised the
level of the influenza pandemic alert to phase 6 on June
11, 2009. The range of illness for this new strain of virus
is still uncertain, varying in severity between different
patients, countries, and other factors. The recommendations are constantly evolving as new information is obtained.
Objective: To analyze the Intensive Care Unit cases of
the pandemic H1N1 2009 virus at Olive View-UCLA
Medical Center and report the similarities observed between these cases.
Design: Case series.
Setting: Olive View-UCLA Medical Center, a Los Angeles County Hospital in Sylmar, California.
Patients: 8 patients with confirmed or high suspicion for
H1N1 who required ICU admission and intubation.
Results: All patients with severe illness at our hospital
were men between ages 23 and 56. Obesity was the most
common and often the only co-morbid condition. All
patients presented with fevers, cough, and infiltrates on
chest x-ray, usually bilateral. Most patients were also
found to be hypoxic, tachycardic, and tachypneic in the
emergency room during triage. Laboratory data was significant for leukopenia, elevated creatine kinase levels,
and transaminitis in most of the patients, even if the patient did not complain of myalgias. Patients received
oseltamivir at different times and experienced different
outcome.
Conclusions: Our patients were not in the extremes of
age. They were young, obese men with no significant
PMH, who presented with respiratory distress and rapid
deterioration in the summer. They had bilateral pneumonias and increasing oxygen requirements. Some patients
could not oxygenate well even with maximal mechanical
ventilation. Doubling the dose of oseltamivir and administering it on admission may have improved the outcome
of our later patients. We were made aware that a (-) rapid
influenza diagnostic test does not exclude pandemic
H1N1.These patients should still be treated with high
dose oseltamivir. Although the recommendations for
management of these critically ill patients are being
modified, early recognition is crucial in preventing mortality and morbidity. We hope our report would help raise
clinical suspicion, especially in patients who do not appear to have known risk factors for severe influenza.
Combination Strategies Using Kinase Inhibitors in
Colorectal Cancer In Vitro
Jeremy Lorber, BA; Adrian Anghel, MD; Zev A.
Wainberg, MD
University of California, Los Angeles
The RAS and PI3 kinase pathways play a critical role in
cancer cell survival, motility, and angiogenesis. It was
recently shown in in vitro and in vivo breast cancer cell
lines that the growth inhibition caused by MEK (part of
the RAS pathway) inhibitors is limited due to upregulation of the PI3k pathway. The aim of this research is to
identify subsets of colorectal cell lines sensitive to a PI3k
inhibitor alone and in combination with a MEK inhibitor.
It is hypothesized that the combination of the inhibitors
would provide synergistic inhibition. We exposed colorectal cancer cell lines to 12 different concentrations of
MEK inhibitors, PI3k inhibitors, and a combination of the
two inhibitors. The ability of inhibitors to inhibit proliferation and induce cell death was measured and IC50
values were calculated. Sensitivities of the 27 cell lines
exposed to the PI3k inhibitor varied from IC50 of .010 to
.669 µM. Additionally, there was no clustering of sensitivities based on mutational statuses of the PI3k or KRAS
genes. When applied in combination, PI3k and MEK
inhibitors caused a synergistic growth inhibition effect in
all cell lines exposed to the combination treatment. These
findings confirm the hypothesis of interaction between
the PI3k and MEK pathways. Furthermore, it suggests
study of additional colorectal cancer cell lines using
combination treatment and exploring the mechanism of
such synergy.
Increased Duration of Red Blood Cell Storage Is Associated with Elevated Potassium Concentrations during Liver Transplantation in Adults
Masood Memarzadeh, BA; Victor W. Xia, MD
University of California, Los Angeles
Background: Increased potassium (K+) concentration
poses serious perils to patients undergoing orthotopic
liver transplantation (OLT). Although there are several
interventions that can be used to lower serum K+, prevention remains most effective in the management of intraoperative hyperkalemia. Previous studies have shown that
increased duration of red blood cell (RBC) storage is associated with higher mortality and risk of complications
in patients receiving RBC transfusions. Moreover, it is
well known that stored RBCs undergo progressive structural and functional changes after 2 to 3 weeks that reduces their viability and functionality. We tested the hypothesis that increased duration of red blood cell (RBC)
storage in transfused blood is associated with significantly higher K+ concentrations during OLT.
Methods: After receiving IRB approval, we retrospectively studied 602 adult patients who underwent OLT at
the UCLA medical center between January 2004 and
April 2007. RBCs were categorized into two groups
based on the mean storage duration (newer RBC ≤14 and
older RBC >14 days). The relationship between serum K+
and duration of RBC storage was analyzed at multiple
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time points of OLT using Student t test or chi-square
analysis. The incidence of hyperkalemia in these groups
was also recorded. Hyperkalemia was defined as a serum
K+ ≥5.5 mmol/L. Recipient, donor and intraoperative
variables of patients receiving newer and older blood
were compared as well.
Results: 258 patients received fresh RBCs (mean RBC
age of 9.3 days) and 397 patients received older RBCs
(mean RBC age of 21.7 days). Baseline demographics
including age, gender, MELD scores, K+, and creatinine
were similar in both groups. Patients in both groups received similar amounts of RBCs, insulin and furosemide.
However, patients in the older blood group had significantly higher serum K+ at 1 hour pre-reperfusion and at
15 minutes, 1 hour, 2 hours and 3 hours post-reperfusion
(Table 1, all p<0.02). Patients in this group also had a
significantly higher incidence of hyperkalemia at 1 hour
pre-reperfusion and at15 minutes, 2 hours and 3 hours
post-reperfusion (all p<0.05).
Discussion: Our results indicate that increased duration
of RBC storage is associated with higher intraoperative
serum K+ during OLT. This information can be used to
better prepare anticipatory interventions in patients at
increased risk for developing hyperkalemia during OLT.
These results may also be applicable to other operations
requiring massive blood transfusions, such as trauma
surgery or ruptured aortic aneurysm repair. They may
also warrant efforts to assign relatively newer blood to
patients at particularly high risk of intraoperative hyperkalemia during OLT, such as those with renal failure or
high preoperative serum K+.
The Effects of Race on Do-Not-Resuscitate (DNR)
Status in a Tertiary Military Medical Center
LT Russell J. Miller, MD; LCDR Christopher Stafford, MD
Depts. of Internal Medicine and Pulmonary/ Critical
Care
Naval Medical Center San Diego
Introduction: Do Not Resuscitate (DNR) orders were
developed to document patients' wishes regarding medical care in the event of cardiopulmonary arrest. Multiple
factors influence end-of-life care discussions including
understanding of disease process, medical staff and family influence, and cultural and religious beliefs. Studies
have found large variations in rates of DNR orders across
hospitals. When evaluating the effects of race in previous
studies, African Americans were substantially less likely
to choose DNR status than Caucasians. Speculation concerning this disparity is often based on socio-economic
status and access to care. We performed a retrospective
review of code status, demographic information, and outcomes in patients admitted to a military medical center.
Since all patents are TRICARE beneficiaries’, we postulated our data would be more revealing than previous
studies in assessing whether race plays a role in end-of-
life wishes independent of factors affecting access to
care.
Methods: We performed a retrospective observational
study of patients > 18 years of age admitted to Naval
Medical Center San Diego between January 2005 and
January 2009 through an electronic search. Information
obtained included code status, ethnicity, length and cost
of hospital stay.
Results: During the study period 56,988 patients were
admitted, of which 438 selected DNR status (0.76%).
1.05% (348/32968) of Caucasians chose to be DNR compared to only 0.37% (25/6757) African Americans and
0.5% (27/5408) of Asian/Pacific Islanders (p value <
0.01). Although Caucasians choose DNR status more
often, African Americans and Asian/Pacific Islanders
who were DNR had hospital stays significantly longer
than their full-code counterparts. In Caucasians hospital
stay was only extended by 1.4 days in DNR patients
compared to 3.5 days in African Americans and 5.1 days
in Asian/Pacific Islanders. Cost of hospitalization was
also similar between races in the full code group, but in
DNR patients the cost of hospitalization rose significantly
more in African Americans and Asian/ Pacific Islanders
compared to Caucasians.
Conclusion: The higher cost and longer stays associated
with DNR status in ethnic minority patients compared to
Caucasians may indicate that non-white patients choose
DNR in more advanced stages of disease. The reason for
this disparity is unclear since issues of access to care are
largely nullified in a military hospital setting. Potential
explanations for the discrepancy include provider bias,
socio-economic status, and cultural beliefs. We believe
further research is warranted to understand the complex
issues involved in patient choices regarding end-of-life
care.
A Highly Purified Omega-3 Fatty Acid Derivative
Does Not Alter QTc Interval but Does Reduce QT
Dispersion in Patients with Stable Coronary Artery
Disease
Tarik Ngab, MD; Sheba Meymandi, MD; Mahmoud
Traina, MD; Mark McDonnell, MD, MD; Atef ElGassier, MBBch; Mohamed Labedi, MD; Robin
Wachsner, MD
Olive View-UCLA Medical Center
Background: Fish oil has been shown to reduce sudden
death in patients with coronary artery disease (CAD) potentially by decreasing fatal arrhythmias. Increased QTc
and QT dispersion (QTd) have been found to be associated with increased risk of ventricular arrhythmias. Several epidemiologic trials have suggested increased fish
intake may reduce the QTc interval, potentially reducing
the risk of sudden death. However, no prior randomized
trial has evaluated the effects of fish oil on QTc interval
and QTd in this population.
85
Methods: In a randomized, double-blinded, placebocontrolled trial, 71 patients with established CAD and
normal or near-normal ejection fraction received either 1
gram twice a day of a highly purified omega-3 fatty acid
derivative, Lovaza, (n=37) or placebo (n=34) for 2
months. One patient in each group developed atrial fibrillation and were excluded from the analysis. At day 0 and
day 60, electrocardiograms were obtained. Measurements
of QTd were performed manually.
Results: Fish oil supplementation did not result in any
significant change in QTc (2.1ms in fish oil, -2.0ms in
placebo, P=0.34). However, a significant reduction in QT
dispersion was noted (6.4±13.2ms in fish oil, 0.07±9.8ms in placebo, P=0.047).
Conclusions: Fish oil supplementation in patients with
CAD did not result in any significant effect on QTc interval. However, there was a significant reduction in QTd.
This effect may partly explain the mechanism of reduction of sudden death with fish oil supplementation.
The Prevalence of Chagas Disease in a US Population
of Latin American Immigrants with Conduction Abnormalities on EKG
Tarik Ngab, MD; Sheba Meymandi, MD; Mahmoud
Traina, MD; Salvador Hernandez, MBBch; Aiman
Smer, MBBch; Haneen Khamag, MBBch; Eva Garcia, MBBch; Yacin Zwam, MBBch; Mahmoud Shebel,
MBBch; Robin Wachsner, MD
Olive View-UCLA Medical Center
Chagas disease is the leading cause of cardiomyopathy in
Latin America and is believed to infect 16-18 million
people. Given recent immigration trends in the United
States, there is a large population at risk. Early stage cardiac involvement usually presents with conduction abnormalities on electrocardiogram (EKG), including right
bundle branch block (RBBB), left anterior and posterior
fascicular blocks (LAFB/LPFB), and left bundle branch
block (LBBB).
Identification of disease at this stage may lead to early
treatment and potentially delay the progression to endstage cardiomyopathy. This study aims to determine the
prevalence of Chagas disease in a US population of Latin
American immigrants with conduction abnormalities on
EKG. All EKGs performed in a Los Angeles County
hospital and clinic system were screened for presence of
RBBB, LAFB, LPFB, or LBBB. Patients were contacted
and enrolled in the trial if they resided in Latin America
for at least 12 months and had no history of cardiac disease. 238 consecutive patients were screened for Chagas
disease was performed with both an immunoflourescence
assay (IFA) and enzyme-linked immunosorbent assay
(ELISA). Testing was performed by the Center for Disease Control and Prevention (CDC). The mean age of
patients was 47.6 years and had lived in the US for an
average of 20.15 years. 32.7% of patients had isolated
RBBB, 43.7% LAFB, 7.2% LBBB, 7.2% RBBB/LAFB,
and 8.8% RBBB/LPFB. Countries of origin were as follows: Mexico 146, El Salvador 56, Guatemala 19, Nicaragua 4, Honduras 5, Argentina 4, Peru 2, Costa Rica 2,
and Bolivia 1. Eleven patients were positive by both IFA
and ELISA (4.6%). 6 patients were from El Salvador
(10.7%), 3 from Mexico (2.1%), 1 from Honduras (20%),
and 1 from Argentina (25%). 4 patients had RBBB
(5.1%), 4 LAFB (3.8%), 2 RBBB/LAFB (11.8%), and 1
RBBB/LPFB (4.8%). No positive patients had LBBB.
One patient was positive by IFA and negative by ELISA
with LAFB. Our study demonstrates a significant prevalence of Chagas disease in Latin American immigrants
with conduction abnormalities on EKG. Evidence of conduction abnormalities in Latin American immigrants
without explanation should prompt consideration of Chagas disease as the etiology.
Respiratory Muscle Weakness in Neuromuscular Disease: Association with Lung Function, Respiratory
Impairment and Initiation of Non-Invasive Ventilation
Nathan Nguyen, MD; Ahmet Baydur, MD, FACP
University of Southern California
Introduction: Patients with neuromuscular disorders
exhibit a decrease in lung function and respiratory muscle
strength. Symptoms such as orthopnea, daytime drowsiness, and the forced vital capacity (FVC) (50 % predicted) are used as guides to determine the need for assisted ventilation. The change in FVC from seated to supine position has been suggested as an indication for initiation of non-invasive mechanical ventilation (NIV). In
individuals with normal respiratory function this difference has been shown to be 4%-6%, versus 16%-18% in
patients with neuromuscular disease. Furthermore, a
forced expiratory flow (FEF) less than 2.7 L/s predicts
successful extubation or conversion from tracheostomy to
NIV (Bach JR, Saporito LR. Chest 1996; 110:1566–
1571).
Objectives: In this retrospective study of patients with
neuromuscular disease who developed respiratory impairment, we assessed (1) respiratory function in seated
and supine postures, and (2) the relation between lung
volumes and respiratory muscle strength as close to when
NIV initiated.
Methods: Spirometry in seated and supine posture and
seated peak inspiratory and expiratory mouth pressures
(Pimax and Pemax, respectively) measured in closest
proximity to the initiation of NIV were assessed in 12
patients (6 males). The relation between FVC and FEF,
between FVC and Pmax, and between postural change in
FVC (FVCseated – FVCsupine)/FVCseated [=
%&#8710;FVC], Pmax and respiratory muscle strength
[RMS = (Pimax + Pemax)/2] were assessed.
Results: Diagnoses included motor neuron disease (n=
5), autoimmune disease (n=3), post-polio syndrome (n=
3), and paralyzed left hemi-diaphragm (n=1). All patients
86
experienced orthopnea or dyspnea. Respiratory function
was assessed within [median (range)] 3 (1-132) months
of initiation of NIV. Anthropometric and physiologic
values (mean ± SD) were as follows: Age 62.8 ± 9.91,
BMI 30.3 ± 10.7, FVCseated 2.0 ± 1.0 L (61 ± 27 % predicted), FVCsupine 1.74 ± 0.99 L (56.3 ± 29.7 % pred),
%&#8710;FVC 17 ± 17%, FEFseated 5.1 ± 2.1 L/s (81 ±
35 % pred), FEFsup 4.5 ± 2.4 L/s (69 ± 39 %pred); Pimax 49 ± 24 cm H2O (55 ± 29 %pred), Pemax 67 ± 24
cm H2O (47 ± 29 %pred); RMS 58.6 ± 21.7 cmH2O
(50.6 ± 23 %pred). Correlations (Spearman) between
lung function and respiratory muscle strength were as
follows: FVCseated (l) vs FEF (l/sec): r=0.59, p=0.04;
FEF (l/sec) vs RMS (cmH20): r=0.69, p=0.02;
%&#8710;FVC vs Pi max (% pred) r=-0.52, p=0.08;
%&#8710;FVC vs FVCseated (% pred): r=0.52, p=0.08.
Conclusion: These findings support FVC of 17 % is a
useful marker of respiratory muscle weakness in patients
with neuromuscular disease, and more useful than FEF
and FVC as a guide to determine need for NIV, sometimes even long before respiratory failure ensues.
Population Based Analysis of Prognostic Factors for
Survival in Rectal Cancer
Chuan-Ju Pan, MD; Kavitha Raj; Argyrios Ziogas,
PhD; Steven D. Mills, MD; Andrew Barleben, MD;
Michael J. Stamos, MD; Jason A. Zell, DO
University of California, Irvine
Background: Colorectal cancer is the second most
common cause of cancer-related death among men and
women in the United States. Several key prognostic factors have been extensively studied for colorectal cancer
including TNM staging, numbers of lymph nodes removed, tumor grade, socioeconomic status (SES), race
and treatment; however, prognostic factors specifically
related to rectal cancer are not well-defined. This study
focused on analysis of prognostic factors for survival in
rectal cancer patients. We set out to determine these factors by using data from a large population-based California Cancer Registry (CCR).
Methods: A retrospective analysis of mid/distal rectal
cancers from 1994-2006 with a follow-up until March
2007 in CCR was conducted. Rectosigmoid cancers were
excluded for the analysis. Multivariate colorectal cancerspecific survival analyses were performed using Cox
proportional-hazard ratios (HR). Multivariate survival
regression models were stratified by TNM stage and adjusted for the following relevant clinical factors: age, sex,
race, SES, histology, tumor grade, number of lymph
nodes removed, surgical procedure, radiation therapy and
chemotherapy.
Results: A total of 23,240 incident cases of stage I, II and
III rectal cancer were identified. CRC-specific mortality
benefit was observed for Stage II rectal cancer patients
with 7 to 11 lymph nodes removed (HR, 0.68; 95% CI,
0.56-0.82) and with more than 12 lymph nodes removed
(HR, 0.59; 95% CI, 0.48-0.72) compared with no lymph
nodes removed; no mortality differences were noted for 1
to 3 or 4 to 6 lymph nodes removed vs no lymph node
removed. In addition, African-Americans, and cases from
lower SES had increased risk of CRC-specific mortality.
Compared with well differentiated tumor after adjustment
for relevant clinical factors and treatments, cases with
poor (Grade III) and undifferentiated (Grade IV) tumor
grade also had increased risk of CRC-specific mortality
among stage I (Grade III HR= 2.01; 95% CI, 1.59-2.52;
Grade IV HR= 4.15; 95% CI, 2.04-8.44), stage II (Grade
III HR=1.83; 95% CI, 1.49-2.23; Grade IV HR= 2.66;
95% CI, 1.50-4.72) and Stage III (Grade III HR= 1.75;
95% CI, 1.43-2.14; Grade IV HR= 3.76; 95% CI, 2.385.93).
Conclusions: Number of lymph nodes removed, AfricanAmerican race/ethnicity, SES, and tumor grade were observed to be independent prognostic factors for survival
among rectal cancer patients. Examination of at least 7
lymph nodes was associated with decreased CRC-specific
mortality. Interestingly, within each cancer stage tumor
grade was noted to be the strongest prognostic factor of
CRC-specific mortality. These data may be useful for
evaluation of prognosis and for guiding rectal cancer
treatment.
Conditional Knockout of Nkx2.5 in Mesp1 Expressing
Cells Results in Cardiac Developmental Abnormalities and Early Embryonic Lethality
Reza Rafie, MD; Sina Tavakoli, MD; Ali
Darahzereshki, MD; Sharmineh Shams, MD; Amir
Hajrasouliha, MD; Roodabeh Mohamad zadeh, MD;
Mohamad Pashmforoush, MD, PhD
University of Southern California
Background: Recent advances in cardiovascular genetics
have improved our understanding of congenital heart
disease. Developmental biology and molecular genetics
studies have clarified the role of various gene products in
cardiac development. Nkx2.5 and Mesp1 are two transcription factors that have been extensively studied in
cardiovascular development. Mesp1, a member of basic
helix-loop-helix transcription factor family located on
chromosome 7, is being considered as the earliest transcription factor which can be detected in cardiac precursor cells. Nkx2.5 mutations have been associated in humans with disorders such as atrial septal defect, ventricular septal defect, Tetralogy of Fallot, and mitral valve
abnormalities. In order to learn more about the effect of
Nkx2.5 and Mesp1 in cardiac development, we conditionally knocked out Nkx2.5 in cardiac Mesp1 expressing
cells by using a Cre-Lox p system.
Methods: Using a Nkx2.5 floxed allele and Mesp1-Cre
transgenic mice lines, we conditionally knocked out
Nkx2.5 in the majority of right and left atrial and ventricular cardiomyocytes, with the exception of a small
group of cells that are derived from non-Mesp1 expressing progenitors. We also crossed Mesp1-Cre and Nkx2.5-
87
Cre with ROSA26R mice lines to better identify the contribution of Mesp1 and Nkx2.5 expressing cells to different components of embryonic heart at different developmental stages.
Results: Our results showed no live neonate with Msep1Cre+, Nkx2.5 genotype. As we evaluated embryos at different developmental stages, we noticed early lethality
around embryonic day 10.5. Conditional Nkx2.5 knockout embryos had smaller body size and heart/body size
ratio compared to wild type embryos. Conditional Nkx2.5
knockout embryos had pericardial effusion and did not
start looping. According to previous studies, lineage
analysis using ROSA26R line showed extensive expression of Mesp1 in both atria and ventricles and conditional
knockout of Nkx2.5 in these cells was associated with
severe cardiac malformations.
Conclusion: Previous studies showed early embryonic
lethality with global knockout of Nkx2.5 in cardiac cells.
In this study, conditional knockout of Nkx2.5 in cardiac
cells deriving from Mesp1 expressing progenitors caused
severe cardiac malformation and early embryonic lethalithy. Cardiac malformation is one of the main causes
of early pregnancy loss in human.
Ectopic Expression of Pacemaker Channel Marker in
Atrial Cardiomyocytes Adjacent to Vessels by Conditional Knockout of Nkx2.5
Reza Rafie, MD; Sina Tavakoli, MD; Sharmineh
Shams, MD; Amir Hajrasouliha, MD; Roodabeh
Mohamad zadeh, MD; Ali Darahzereshki, MD;
Mohamad Pashmforoush, MD, PhD
University of Southern California
Introduction: Previous studies depicted the role of
Nkx2.5 in cardiac conduction system development. In this
study conditional knockout of Nkx2.5 in cardiomyocytes
originating from Islet1 expressing progenitors resulted in
ectopic expression of pacemaker channel (defined by
HCN4) in limited areas of atria and ventricles. Islet1 and
Nkx2.5 have extensive expression in atria and right ventricle but we found few ectopic sites in knockout mice.
We attempted to figure out the underlying cause for this
discrepancy.
Methods: We evaluated 100 mice at neonatal and late
embryonic stages with conditionally ablated Nkx2.5, using Islet1-IRES-Cre transgenic mouse line and the floxed
allele of Nkx2.5. We studied the cardiac tissues by immunostaining using antibodies against the pacemaker channel marker (HCN4), actinin and connexin 40. We also
performed hematoxyllin and eosin staining on these tissues. We crossed Islet1-IRES-Cre with ROSA 26R line
to define the expression pattern of Islet1.
Results: Conditional knockout of Nkx2.5 caused ectopic
sites of HCN4 expression in atrial and small part of right
ventricular myocytes. Lineage analysis using ROSA26R
line showed most of the cells in atrial and right ventricu-
lar myocytes express Islet1 but ectopic expression of
HCN4 was limited to few areas in atrium and right ventricle. These areas with pacemaker channel marker expression outside of sinoatrial node (SA node) has histologic characteristics similar to SA node with compact,
round cells that is different from the rest of atrium. We
performed immunostaining with HCN4 and connexin 40
on atrium that showed ectopic HCN4 sites in atrium do
not express connexin 40 unlike the rest of atrium but
similar to SA node. More careful evaluation of large ectopic HCN4 sites depicted the vessel section in middle or
adjacent to that area. Ectopic HCN4 sites in ventricle
were also located near to vascular sections.
Discussion: Previous studies showed upregulation of
HCN4 after Nkx2.5 knocking out at embryonic stage 8.59 post coital day. In this study we showed ectopic expression of HCN4 after conditionally knockout of Nkx2.5 in
Islet1 expressing cells. Islet1 has diffuse expression in
atrium and right ventricle. With conditional knockout of
Nkx2.5, we expected to detect ectopic sites of HCN4 expression in most parts of atrium and right ventricle but
there was only few ectopic sites in each knock mouse.
With more evaluation, we noticed the vessel section in
large HCN4 ectopic sites similar to artery that can be
visualized in SA node. This needs more evaluation if
different cytokine that are secreted from endothelium
may have role in pacemaker cells formation. This finding
can help to produce cells with intrinsic pacemaker activity in the future.
Late-Onset Dilated Cardiomyopathy in Cap Knockout Mice
Reza Rafie, MD; Sina Tavakoli, MD; Sharmineh
Shams, MD; Amir Hajrasouliha, MD; Roodabeh
Mohamad zadeh, MD; Ali Darahzereshki, MD;
Mohamad Pashmforoush, MD, PhD
University of Southern California
Introduction: Insulin affects cardiac tissues by translocation of the glucose transporter 4 (GLUT4). Cbl-associated
protein (Cap) is involved in the recruitment of GLUT4 to
cell membrane through a phosphatidylinositol 3-kinaseindependent pathway. Deletion of the Cap gene protects
against high-fat diet-induced insulin resistance. However,
the functions of Cap gene have never been studied in
cardiac tissue.
Methods: Cardiac metabolism was evaluated by dynamic
micro-positron emission tomography (microPET) imaging studies after intravenous injection of 200 µCi 18Ffluorodeoxyglucose in Cap knockout and wild type mice.
Transthoracic echocardiography was also performed in
young (2-3 months) and aged (1 year) mice. Pressure
overload induced cardiac failure was induced by thoracic
aortic constriction.
Results: MicroPET scan studies demonstrated an early
increased and a late decrease in 18F-FDG uptake in
knockout mice. Basal echocardiographic findings as well
88
as response to thoracic aortic constriction at young age
were comparable between wild type and knockout mice
and no statistically significant changes were found. However, senescent knockout mice demonstrated echocardiographic evidence of dilated cardiomyopathy characterized
by a decreased ejection fraction (wild type: 67.6% versus
knockout: 56.8%; P<0.05) and an increased end systolic
volume (wild type:`20.15 µ versus knockout: 37.39 µ;
P=0.055).
Conclusion: Previous studies showed the changes in cardiac metabolism during human and mouse development.
There is tendency toward using glucose instead of fatty
acids by cardiomyocytes at older ages. This study demonstrates the critical function of Cap gene in regulation of
cardiac glucose metabolism. The indispensable role of
CAP in maintenance of cardiac function may be one an
underlying mechanism for development of idiopathic
dilated cardiomyopathy in senescence.
QRS Duration Variability as Predictor of Cardiac
Resynchronization Therapy (CRT) Response
Reza Rafie, MD; Pedram Razavi, MD; Michael Cao,
MD
University of Southern California
Introduction: Cardiac resynchronization therapy (CRT)
attempts to correct for ventricular dyssynchrony. Current
indications for CRT therapy includes: NYHA class IIIIV, left ventricular ejection fraction < 35%, prolonged
QRS duration( QRS duration>120 ms). Recent studies
have shown that baseline QRS duration is not a predictor
for CRT response. Studies have included a prolonged
QRS duration >120-130ms. This is determined by a single 12 lead ECG prior to the CRT implant. Patient with
underlying heart failure (HF) can have variable QRS duration. In this study we attempted to measure QRS duration variability before a CRT implant and to evaluate if
this helps predict CRT response.
Methods: Our study examined 45 patients that were
treated with CRT in USC between 2005-2008 . Patients
were selected for CRT based on the current indications:
1) NYHA classes II to IV that was unresponsive to
maximum pharmacologic therapy. 2) Ejection fraction
<35% 3)QRS duration > 120 ms on the last visit before
CRT implantation. A total of 31 patients had at least 2
EKG available for interpretation prior to CRT implantation
Results: CRT response was defined as either a functional
improvement or increase in LVEF. A total of 82% of
patients improved clinically and 55% had an increase in
LVEF. The mean LVEF increased by 20% and the mean
functional class improved by 1.1. Our results showed
patients with clinical and/or echocardiographic response
to CRT had longer QRS duration not only 1 month before
starting CRT but also in period of 1 to 6 months before
CRT implantation. (p=0.23). Our study was limited by
the small study population. The results of Box plot de-
picts that a chronically prolonged QRS duration >1
month prior to implantation was associated with CRT
response.
Conclusion: Approximately 30%-40% of patients treated
with CRT are considered non-responders for following
reasons: scar tissue, echocardiographic parameter variability in to detect dssynchrony and positions of the LV
lead. ECG is an inexpensive and simple method to evaluate cardiac dssynchrony. Previous studies have demonstrated weakness of QRS duration to predict response to
CRT in spite of its current use as one of the criteria to
select patients for CRT. Our study suggests that QRS
duration variability can help to identifying patients who
respond to CRT therapy.
An Atherogenic Diet Reduces the Anti Inflammatory
Capacity of High Density Lipoprotein and Increases
the Susceptibility of Low Density Lipoprotein to Oxidation
Shila Safarpoor, MD; Susan Hama, B.Sc.; Greg
Hough, MS
David Geffen School of Medicine at UCLA, Division
of Cardiology
Background: Our antioxidant and anti inflammatory
defense mechanisms contribute to a balance between the
stimulators and the inhibitors of inflammation. Beyond a
point, however, these systems might be overwhelmed and
eventually fail. High density lipoprotein (HDL) is one of
the efficient anti oxidant and anti inflammatory systems
in our organism. Under high oxidative and /or inflammatory pressure HDL can lose its ant inflammatory capacity
and even become proinflammatory.
Method: In the present study LDL receptor deficient
mice (n=8 per group) were place on a Western type diet
or on a rodent chow diet for 6 weeks. Plasma was fractionated by fast performance liquid chromatography and
HDL (at 14 ug per ml) was added to cultures of human
aortic endothelial cells that also received standard normal
low density lipoprotein (LDL) (at 100 ug per ml).
Result: In the absence of HDL the cells oxidize LDL and
oxidized LDL induces the potent proinflammatory molecule monocyte chemotactic protein (MCP-1). In the presence of HDL from mice on the chow diet HDL prevented
LDL oxidation and MCP-1 formation as compared with
LDL alone without HDL (p=0.021). HDL obtained from
the mice on the Western diet however was not anti inflammatory and did not prevent LDL oxidation and
MCP-1 induction (p=0.53). Additionally, LDL from the
mice that were on the Western diet was incubated with
the cells (alone, and without HDL), LDL was oxidized to
a higher degree as compared with LDL from the mice on
the chow diet (p=0.017) and induced a significantly
higher levels of MCP-1 (p=0.026).
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Conclusion: We conclude that an atherogenic diet renders HDL proinflammatory and makes LDL more susceptible to oxidation by artery wall cells.
Heritability of Bone Mineral Density: Regional and
Gender Differences in Monozygotic Twins
Kevin Y. Tse, MD; Brandon R. Macias, MS; R. Scott
Meyer, MD; Alan R. Hargens, MD
Scripps Mercy Hospital
Introduction: Bone mineral density (BMD) is a measure
of a person’s skeletal mineral content, and assessing
BMD by dual x-ray absorptiometry (DEXA) can diagnose diseases of low bone density. In this study we determine the heritability of BMD in male and female
monozygotic twin subjects using DEXA in thirteen specific anatomical regions, as well as various serum and
urine markers of bone metabolism.
Methods: In an attempt to quantify the genetic contribution of gender and skeletal region to BMD heritability,
we scanned fourteen pairs of identical twins using DEXA
and calculated the broad-sense heritability coefficient
(H2) in each of the thirteen different body regions. We
also collected serum and urine markers related to bone
turnover to calculate H2.
Results: The region of the body that was most heritable
for both genders was the head (H2 > 95%). When males
were compared to females, H2 values for male hip (H2 =
87%) and lower extremities (H2 = 90%) were higher than
those in females (H2 = 49% and 56%, respectively). Conversely, H2 value for the female pelvis (H2 = 68%) was
higher than that for males (H2 = 26%).
Discussion: These data show that different regions of the
skeleton exhibit different degrees of heritability, and that
the variation depends on gender. Specifically, we show
that among twins, females exhibit more variability in
load-bearing regions, while males show more variability
in non-load bearing regions.
Does Medical Television Influence Physicians InTraining?
Nicky Wadiwala, DO; Brian Haas, DO
University of Southern California
Objective: To determine if residents are influenced by
medical television programs and to what extent that influence plays in their clinical decision-making.
Design: Single page surveys were distributed to medical
residents ranging from PGY1 through PGY7 at different
specialties core conferences at University of Southern
California / Los Angeles County Medical Center from
January 2009 to March 2009.
of those polled had seen at least 10 episodes of medical
television (TV) with Scrubs®, ER®, House MD®,
Grey’s Anatomy®, and Doogie Howser MD® being the
top 5 shows respectively. Overall, 16% of residents had
used a case from medical TV for discussion on rounds,
31% were able to take something they learned on medical
TV and apply it into their medical training, 56% saw
something on medical TV which led them to further research on the subject, and 13 % were influenced to the
point that they ordered more tests and/or increased their
differential diagnosis based upon a case that they had
seen on television.
Conclusions: Medical TV influences a large percentage
of residents. Residents in their first year of training were
more likely to be influenced by medical TV than those
residents in later years of training. Either in the form of
using a case for discussion during rounds, applying it to a
case they are currently working on, or helping in the diagnosis of a case. There is an overwhelming stigma in the
medical community about medical TV shows and their
perceived inaccuracies, but our data demonstrate that
although medical television programming may not be
100% accurate it encourages many physicians in-training
to learn more about the disease they saw on medical TV,
thus further contributing to their medical knowledge.
What Test Characteristics Are Needed for New Tests
to Evaluate Cardiovascular Prevention Strategies?
Brandon B. Woodbury, MD; David J. Bryg, PhD;
Robert J. Bryg, MD
Olive View-UCLA Medical Center
Purpose: As new therapies for primary and secondary
prevention of cardiac disease become available (and as
previously available therapies become better validated),
estimation of future cardiac risk has become increasingly
important to determine for whom the benefit of using
these therapies outweighs their inherent risks and costs.
To this end, new tests are continuing to be developed in
an effort to improve risk prediction and thus optimize
treatment strategies. Although these tests are often evaluated as to whether they independently predict risk, their
true value lies in how well they improve stratification
compared to or in combination with existing strategies.
Method: We developed a Bayesian model utilizing the
concept of the expected value of imperfect information to
evaluate optimal testing strategies based on the a
cost/benefit ratio. We then evaluated the use of a theoretical test in conjunction with cholesterol, highsensitivity C-reactive protein, and coronary calcium scoring over different age groups to determine the minimum
diagnostic odds ratio an additional test would need to
justify its use. Diagnostic odds ratios were varied from
1.5 to 5.0 and age was varied from 40 to 80. A ten year
timeframe for events, similar to the Framingham risk
score, was utilized for this analysis.
Results: A total of 232 surveys were collected with an
overall response rate of 84% (232/275). 91% (209/232)
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Result: The expected value provided is a function of cost
of the test, cost of treatment, and expected loss from non
treatment. The diagnostic odds ratio required for the additional test to be included in the optimal stratification
scheme varied by age. For a strategy involving lower-cost
treatment, the odds ratios required for age groups 40, 50,
and 60 were, respectively, 2.5, 2.0, and 2.5. At age 70
and higher, even the maximum evaluated odds ratio of 5
was insufficient for the additional test to be of utility. For
a higher-cost treatment strategy, no testing was of use at
age 40, and an odds ratio of 1.5 was sufficient for the 50,
60, and above 70 age groups. At high treatment cost, the
primary purpose of the fourth test was to find patients
unlikely to receive benefit of treatment.
Conclusion: There was no significant difference in risks
of death or of the composite end point of death, myocardial infarction, or stroke in patients who underwent
CABG compared with PCI with DES for ULMCA disease. However, PCI with DES was associated with higher
risk of target vessel revascularization.
Conclusion: The above analysis demonstrates that not
only must tests meet minimum criteria in order to be useful, but that these criteria can vary markedly with age and
cost of the treatment. As new tests emerge, they should
be evaluated in light of how much truly new information
they add to current testing strategies.
Can Drug-Eluting Stents Replace CABG in Unprotected Left Main Coronary Disease? A Meta-Analysis
Tae Yang, MD; Michael S. Lee MD
Olive View-UCLA Medical Center; David Geffen
School of Medicine at UCLA
Objective: We sought to compare the safety and efficacy
of coronary artery bypass grafting (CABG) with percutaneous coronary intervention (PCI) with drug-eluting
stents (DES) in patients with unprotected left main coronary artery (ULMCA) disease.
Background: The current American College of Cardiology/American Heart Association guidelines recommend
CABG for the treatment of patients with ULCMA disease
based upon clinical trials that demonstrate a survival
benefit with CABG as compared with medical therapy.
Drug-eluting stents reduce the rate of target vessel revascularization compared with bare metal stents in ULMCA
PCI and may be a safe alternative to CABG.
Methods: A meta-analysis of clinical studies comparing
CABG and PCI with DES for ULMCA disease with respect to death, the composite of death, myocardial infarction, or stroke, and target vessel revascularization at oneyear follow-up.
Results: The analysis included 2905 patients from 8
clinical studies (2 randomized randomized trials and 6
non-randomized studies). At one-year follow-up, there
was no significant difference between the CABG and
DES groups in the risk of death (odds ratio [OR] 1.12,
95% confidence interval [CI], 0.80 to 1.56) or the composite endpoint of death, myocardial endpoint of death,
myocardial infarction, or stroke (OR 1.25, 95% CI 0.86
to 1.82). The risk of target vessel revascularization was
significantly lower in the CABG group compared with
the PCI group (OR 0.44, 95% CI 0.32 to 0.59).
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