PANRE/PANCE GU Review Craig Ensign, MPAS, PA-C BPH Incidence: • The most common benign tumor in men • 50% of men over 75 years of age are symptomatic Irritative Symptoms • Urgency • Frequency • Nocturia Obstructive Symptoms • Slow stream • Hesitancy • Intermittency • retention • • • • • Proliferative growth generally in transition zone Size does not correlate directly with symptoms Progression of symptoms is not inevitable 7-10% may develop retention 50% marked improvement over time Differential Diagnoses • Stricture • UTI • Balder Stone • Bladder tumor • Prostate cancer • Neurogenic bladder • Overactive bladder Medical Treatment • PDE5 Inhibitors -Cialis • Alfa Blockers • -Tamsulosin • 5 ARI -Proscar • Phyto Therapy -Saw Palmetto • • • • • • Surgical Treatment TURP TURMT Laser ablation TUIP Open prostatectomy Electrovaporization Erectile Dysfunction ED: Definition & Incidence The inability to achieve an erection sufficient for satisfactory sexual function Men who Meet Definition 100% 50% 40% 50% 60% 70% 0% 40 Years 50 Years 60 Years 70 Years Work Up/Treatment Signs & Symptoms Laboratory Findings Treatment History • Severity of problem maintain vs attain • Comorbidities Lipids, HTN, DM, CKD • Hx of trauma • Medications (25%) BP, BPH, ED, alcohol ED: Work Up/Treatment Signs & Symptoms Laboratory Findings Treatment • • • • • CBC Fasting lipid profile Glucose Testosterone Prolactin FSH & LH if abnormal to distinguish pituitary vs testicular cause ED: Work Up/Treatment Signs & Symptoms Laboratory Findings • • • • • Treatment PDE5 Inhibitors Sildenifil (Viagra) Vardenafil (Levitra) Tadalifil (Cialis) Requires sexual stimulation Potentiate rather than trigger response ED: Work Up/Treatment Signs & Symptoms Laboratory Findings Treatment Other Options • Injections Caverject (alprostadil) PPP (papaverine, prostaglandin, phentolomine) • Vacuum devise • Surgery IPP Vascular Cryptorchidism Cryptorchidism • Failure of one or both testicles to migrate into scrotum • 3% term babies, 30% of preterm babies • 33% spontaneously descent in first four months • • • • Complications Sub fertility Testicular carcinoma Contralateral risk 10% Risk does not decrease with orchiopexy • • • • • Treatment Orchiopexy Surgical removal Surgical treatment best at 6 months of age Fertility best preserved early No treatment for retractile testis Hydrocele • Painless fluid accumulation around testicle • 10% children congenital, 80% resolve spontaneously • 10% of men > 40, does not resolve spontaneously • • • • • Risk Factors Injury Infection/STI Radiation to pelvis Congenital Not cancer or pre cancer • • • • Diagnosis & Treatment History—injury/onset Physical exam: R/O tumor, infection, hernia, spermatocele Transillumination Surgery/observation Scrotal Transillumination Moderate Hydrocele Varicocele Varicocele • Dilation of the pampiniform plexus, “Bag of Worms” • Varicose veins in the scrotum • • • • • • Incidence Found in 15% healthy males Found in 40% subfertal males Rare in prepubescence boys Surgically correctable infertility Will not resolve spontaneously Most occur on left side • • • • Problems Ipsilateral atrophy Poor sperm motility Generally not painful but can be Infertility Grading • Sub-clinical • Grade I: Palpable only with valsalva • Grade II: Palpable without valsalva • Grade III: Visible upon inspection Treatment Options • No treatment • Surgical repair under general anesthesia • Embolization performed by interventional radiologist Incontinence Detrusor Overactivity (OAB) • Symptoms of urgency, frequency and nocturia with or without leakage • Common in men and women • Cause not well understood • May be exacerbated by BPH, inflammation, DM, stress and anxiety Medical/Behavior Treatment • Medications (Anticholinergics): Oxybutynin (Ditropan, Gelnique) Talterodine (Detrol LA) Darifenacin (Enablex) Trospium (Sanctura) Fesoterodine (Toviaz) Solifenacin (Vesicare) • Eliminate exacerbating factors • Bladder retraining Urethral Incompetence (Stress) • Pregnancies increase risk • Result of weakened pelvic floor and poor support of vesicourethral sphincter • Leakage with chronic or sudden increase in abdominal pressure—cough, sneeze, jumping, running • Rare in men, RRP is risk factor Treatment • • • • Surgical: sling, AP repair Injectable bulking agent: collagen Pelvic floor exercises Medications usually not effective Urolithiasis Remember Kramer’s Stone? Stone Types Uric Acid 5% Struvite & 0thers 5% Calcium 90% Work-up • History – Previous, family hx, onset, duration, N/V, fever – 3:1 male to female ratio – 50% recurrence rate in the next 5 years • Physical Exam – Movement, CVAT, other pain location • Imaging – Non contrast CT Treatment • • • • • Pain management, let stone pass Alpha blockers—evidence does not support Copious fluid intake Alkalinization—for uric acid stones Surgery – ESWL, basket retrieval, PCNL, open resection Phimosis & Paraphimosis Phimosis • Foreskin cannot retract over glans • May be congenital or develop as result of chronic infection and poor hygiene • Balanitis may lead to phimosis—eveluate for diabetes mellitus • Phimosis in children almost always physiological Phimosis in a Child Balanitis in Adult Paraphimosis • Retracted foreskin cannot be replaced over the glans • Almost always iatrogenic cause • Can almost always be reduced at bedside • Rarely is surgery indicated—circumcision Forgot to Replace Foreskin Testicular Torsion Definition Testicular torsion is the twisting of a testis on its spermatic cord so that the blood supply is blocked Epidemiology • Most common between puberty and 25 years of age • Occurs due to malformation of the tunica vaginalis • Generally occurs during sleep • Testicle will die within six to eight hours if not resolved Evaluation & Treatment • Signs and Symptoms – Acute onset of severe testicular pain and swelling – May be accompanied by nausea and vomiting – Generally not associated with trauma, usually occurs during sleep • Treatment – Medical emergency—loss of testicle with delay – Requires bilateral surgical repair Infections/Inflammatory Conditions Common Organisms: Applies to All GU Infections • Gram Negative—most common – Escherichia Coli – Klebsiella – Enterobacter – Proteus • Gram Positive—less common – Enterococcus Faecalis – Staphylococcus aureus 75-90% 6-12% 2-12% 4-6% Pathogenesis: Applies to All GU Infections Four possible modes of infection: 1. Periurethral migration of bacteria 2. Hematogenous spread in immunecompromised patients 3. Direct contamination from fistulas 4. Theoretically, urine can be contaminated through the lymphatic system Cystitis Epidemiology Incidence (%) Age <1 Female 0.7 Male 2.7 1-5 6-15 4.5 4.5 0.5 0.5 16-35 36-65 >65 20 35 40 0.5 20 35 Risk Factors Foreskin, anatomic GU abnormality Anatomic GU abnormality Functional GU abnormality Intercourse, diaphragm Surgery, BPH, catheters Surgery, BPH, catheters Symptoms • Symptoms – Dysuria – Urgency/Frequency – Suprapubic pain – Hematuria • Signs – Suprapubic tenderness – UA—nitrites, leukocytes, hemoglobin Treatment Options • Nitrofurantoin (Macrobid) – 100 mg bid X 7 days (no change in resistance) • Fluroquinolones – Cipro 500 mg bid X 1-3 • TMP/SMX (Septra, Bactrim) – DS bid X 1-3 days (increasing resistance) Epididymitis Orchitis Clinical Presentation • Bacterial infection, E. Coli or STI • Scrotal enlargement and induration • Voiding symptoms: dysuria, urgency, frequency • Fever, nausea and vomiting may be present • Prehn’s sign—pain resolves when scrotum is elevated above pubic bone Treatment • • • • • Bed rest Scrotal elevation and ice Antibiotic treatment similar to UTI Treat for gonorrhea or chlamydia as indicated Scrotal US if any concern for torsion Prostatitis Prostatitis: Types • Acute/bacterial prostatitis • Chronic prostatitis/non-bacterial prostatitis • CPPS/CP—should be at top of differential Acute/Bacterial • Irritative voiding symptoms • Perineal or suprapubic discomfort, often dull and poorly localized • Fever • May have positive urine culture • Pathogen similar to UTI • Do not do prostate massage—sepsis risk • DRE elicits chandelier response Chandelier Response Treatment • 4-6 week course of antibiotics is indicated for initial treatment—longer regiments are sometimes necessary • Cipro 500 mg bid x 30-45 days • Septra DS bid x 30-45 days • CPPS/CP—identify exacerbating factors, pain clinic Pyelonephritis Pyelonephritis • Inflammation of the kidney and renal pelvis, diagnosis is usually made clinically • UTI symptoms with fever, chills, flank pain • 20-30% of all sepsis originates with urinary tract infection Lab/Imaging • CBC shows leukocytosis and a left shift • Urine culture grows out offending pathogen— always get culture • Upper tract imaging shows evidence of pyelonephritis and can rule in or out obstructive complicating factors Treatment • Most can be treated outpatient • Complications may require hospitalization: stones, immune compromised patient, sepsis • Urine and/or blood culture is essential • Same antibiotic treatment as cystitis, may treat initially IV, adjust based on culture • Prognosis in healthy individuals is very good with prompt diagnosis and treatment Urethritis Signs & Symptoms • Urethritis usually STI • In both men and women, dysuria, urgency and frequency • Discharge is often yellowish green with gonorrhea • Clear with chlamydia • In women, discharge is less common Treatment • Empiric treatment is initiated based on clinical presentation • Ceftriaxone (Rocephin) 250 mg IM/Gonorrhea • Azithromycin (Zithromax) 1 g single dose/ Chlamydia • If STI is not likely, treatment similar to UTI is appropriate Bladder Cancer Bladder Cancer • • • • • Second most common cancer of the GU tract Lower incidence in African Americans 75% confined to bladder TCC 91%; Squamous 7%; Adenocarcinoma 2% Risk factors smoking, chemical exposure, age > 35, chronic Foley use, history of GU surgery Clinical Findings S&S • Hematuria—90% present with micro or gross hematuria • LUTS—including urgency, frequency, dysuria, bladder pain Treatment • TURBT, BCG, cystectomy, radiation, chemo • Follow up—important due to high rate or recurrence Prostate Carcinoma Prostate Carcinoma • Most common cancer in men • 230,000 new cases per year • Second leading cause of cancer death, but mortality rates declining since 1990’s • 95% adenocarcinoma • 4% Transitional Cell Carcinoma • 1% Neuroendocrine Carcinoma or Sarcoma PSA • Produced by benign and cancerous prostate tissue • Only a screening tool, used in connection with DRE and TRUS/BX • Not specific to cancer—increased with: – BPH, retention – Urethral instrumentation – Infection – Stars lining up just right Signs & Symptoms • • • • Usually asymptomatic DRE—nodule or induration, may be normal Metastatic disease—bone pain Cord compression – Paresthesia, weakness, incontinence • Definitive diagnosis with TRUS/BX • PSA: > 4.0 Caucasian > 2.5 African American Treatment • Watchful waiting • Radical prostatectomy • Brachy therapy (radioactive seed implantation) • External beam radiation Renal Cell Carcinoma Etiology • The cause of RCC is unknown • Risk factors: – Smoking: two times as likely to develop RCC – Industrial exposure to asbestos, solvents and cadmium (batteries) – Hereditary factors: Von Hippel-Lindau disease – Dialysis: especially in presence of cystic kidney – Renal transplant Clinical Findings/Treatment • Triad of gross hematuria, flank pain and palpable mass—only 7-10% at diagnosis • Dyspnea, cough and/or bone pain implies metastases • Incidentally found on CT in >50% of patients • Treatment: partial or total nephrectomy Testicular Tumor Types of Tumors • Two general Divisions • Non-germ Cell—5% of all testicular tumors • Germ Cell—95% of all testicular tumors – Seminoma (40%) – Non-seminoma germ cell tumor (NSGCT) Clinical Presentation Symptoms • Painless enlargement of testicle—pain is present in 10% of patients • Sensation of heaviness in testicle • 10% of patients present with symptoms related to mets: back pain, cough, dyspnea, anorexia, bone pain, edema, gynecomastia Clinical Presentation • • • • • • Signs Testicular mass or enlargement Tumors do not transilluminate Lymphadenopathy: subclavicular or inguinal Gynecomastia Hemoptysis in advanced pulmonary disease Tumor markers: HCG, AFP Treatment • Radical orchiectomy • Pathology: tumor type and staging – CXR, CT for staging • Treatment based on tumor type and staging – Further debulking surgery – RPLND – Chemotherapy – Radiation Renal Disease Acid/Base Disorders Sample ABG Question A wheezing 14-year-old female with cystic fibrosis complains of a productive cough and shortness of breath. The following labs are available: pH: pCO2: HCO3: 7.30 50 mm Hg 24 mEq/L The Question What is the first step in treating this patient? A. B. C. D. Decrease respirations to increase CO2 Alkalinize the blood to counter the acidosis Treat the obstructed airway Start broad spectrum antibiotics Rule 1: pH Determines the Problem— Acidemia vs Alkalemia • • • • Look at the pH Normal arterial pH is between 7.35 and 7.45 pH > 7.45 = alkalemia pH < 7.35 = acidemia Rule 2: HCO3 and PaCO2 Determine the cause of the problem • Normal Values: – HCO3 – PaCO2 22-26 mEq/L 35-45 mm Hg • If HCO3 goes the direction of pH, metabolic • If PaCO2 goes opposite to pH, respiratory Blood Gas Scenarios pH=7.21 PaCO2=40 HCO3=15.6 Rule 3: Mixed Conditions can Occur • When PaCO2 and HCO3 shift in opposite directions, they can both be responsible • Examples: – pH=7.21 – pH=7.51 PaCO2=51 PaCO2=31 HCO3=18 HCO3=29 Rule 4: Chronic Problems can be Compensated • Acute problems follow the rules • Problems can compensate—PaCO2 and HCO3 are on the same side • The offending problem is still identified by the rules – pH=7.35 PaCO2=51 HCO3=27 Rule 5: Respiratory Compensates Faster than Metabolic • PaCO2 is regulated by adjusting the respiratory rate—adjusts within seconds to minutes • Renal adjustment (HCO3) may take hours to days to adjust Rule 6: Treat the respiratory or metabolic problem ABG questions on this test will cause me to drool, Because I know the basics—I can follow these rules When the pH is high there will be alkalosis, But a lower pH indicates acidosis Metabolic problems are easy for me, Since bicarbonate follows pH, you see But if respiration is the one to blame, Carbon dioxide and pH are apposed, not the same Don’t forget, on occasion, mixed problems reside, When carbon dioxide and bicarbonate take opposite sides Be aware acute problems will follow these rules, But a compensated condition can leave me fooled Remember respirations will compensate quickly, But kidneys are slow and can leave one quite sickly Now when that gas question appears on my test, There is one more rule, I know all the rest Labs only show me the pH position, While treatment addresses the primary condition Metabolic Acidosis • Increased anion gap acidosis – Lactic acidosis – Diabetic, alcoholic ketoacidosis • Normal anion gap acidosis – Gastrointestinal HCO3 loss – Renal tubular acidosis (RTA) Metabolic Alkalosis • Saline-responsive – Contraction alkalosis – Posthypercapnia alkalosis • Saline-unresponsive – Hyperaldosteronism – Alkali administration with decreased GFR Respiratory Acidosis • Hypoventilation and subsequent hypercapnia • Respiratory failure • Chronic respiratory acidosis in patients with underlying lung disease • Renal compensation to excrete more acid and generate more HCO3 takes days Respiratory Alkalosis • Hyperventilation reduces PaCO2 and increases pH • High altitude, hypotension • Anxiety, infection, trauma, tumor, aspirin • Interstitial lung disease, pneumonia, PE Sample ABG Question A wheezing 14-year-old girl with cystic fibrosis complains of a productive cough and shortness of breath. The following labs are available: pH: pCO2: HCO3: 7.30 50 mm Hg 24 meq/liter The Question What is the first step in treating this patient? A. B. C. D. Decrease respirations to increase CO2 Alkalinize the blood to counter the acidosis Treat the obstructed airway Start broad spectrum antibiotics Fluid & Electrolyte Disorders Fluid & Electrolyte Disorders Work Up • • • • • History Physical Exam Serum electrolyte concentration Urine electrolyte concentration Serum osmolality Hyponatremia • Defined as < 135 mEq/L • Most common electrolyte abnormality • Mismanagement can result in neurologic catastrophe • Iatrogenic complications may be more harmful than hyponatremia itself • Most cases of hyponatremia reflect water imbalance Hyponatremia Serum Osmolality Normal 280-295 mosm/kg Low <280 mosm/KG High >295 mosm/kg Isotonic Hyponatremia 1. Hyperproteinemia 2. Hyperlipidemia Hypotonic Hyponatremia Hypertonic Hyponatremia 1. Hyperglycemia 2. Mannitol, sorbitoll 3. Radiocontrast agents Hypotonic Hyponatremia Volume Status Hypovolemic Extrarenal Salt loss 1. Dehydration 2. Diarrhea 3. Vomiting Renal Salt Loss 1. Diuretics 2. ACE inhibitors 3. Nephropathies 4. Mineralocorticoid deficiency 5. Cerebral sodium-wasting syndrome Hypotonic Hyponatremia Volume Status Euvolemic 1. SIADH 2. Hypothyroidism 3. Psychogenic polydipsia 4. Beer potomania 5. Idiosyncratic drug reaction 6. Endurance exercise 7. Adrenocorticotropin deficiency Hypervolemic Edematous states 1. CHF 2. Liver disease 3. Nephrotic syndrome 4. Advanced kidney disease Hypernatremia • Sodium concentration > 145 mEq/L • Hypernatremia is almost always secondary to free water loss—dehydration • Rarely due to excess sodium intake • Intact thirst mechanism and access to water are primary defense against hypernatremia Hypokalemia • • • • • • • • • • Causes Insufficient dietary intake Intracellular shifting Extrarenal or renal loss Diarrhea Symptoms & Signs Potassium < 3.5 Muscle weakness/cramps Constipation Hyporeflexia Hypercapnia Hypertension • • • • Treatment Oral supplements Dietary replacement Intravenous Based on cause and tolerability Hyperkalemia • • • • • • • • Causes Advanced kidney disease Acidemia Urinary obstruction ACE-I, ARB, Potassium sparing diuretics Symptoms & Signs Muscle weakness Ileus ECG changes—but only 50% demonstrate changes V-fib, cardiac arrest • • • • Treatment Identify/treat cause Withhold potassium Insulin, bicarbonate, B-agonists, calcium Hemodialysis in AKI Calcium Concentration • Normal total serum Ca 8.5-10.5 mg/dL • Normal Ionized Ca 4.6-5.3 mg/dL Hypocalcemia • Mistaken for neurological disorders • Check for low parathyroid hormone, vitamin D and magnesium • Ionized calcium main indicator of problem Hypercalcemia • Asymptomatic, mild—hyperparathyroidism • Severe, symptomatic—due to malignancy • Hypercalciuria usually precedes hypercalcemia Phosphorus Concentration Normal 2.4-4.1 mg/dL • • • • Hypophosphatemia May cause tissue hypoxia and rhabdomyolysis Renal loss diagnosed by phosphateuria PTH causes renal loss of phosphate Malnutrition and alcoholism common causes Hyperphosphatemia • Advanced CKD is most common cause • In combination with hypercalcemia –high risk of metastatic calcification Magnesium Concentration • Normal 1.8-3.0 mg/dL • Altered magnesium concentration usually associated with alteration of calcium Hypomagnesemia • Serum levels may remain normal even with depletion, check magnesiumuria if wasting is suspected • Causes neurologic symptoms and arrhythmias • Impairs release of PTH Hypermagnesemia • Often associated with advanced CKD and chronic intake of magnesium-containing drugs Acid/Base Disorders Acute Kidney Injury (Acute Renal Failure) Prevalence of Acute Kidney Injury • 5% of hospital admissions have AKI • 30% of ICU admissions have AKI • 25% of all inpatients will develop AKI Acute Kidney Injury • Sudden decrease in kidney function • Inability of kidneys to maintain acid-base, fluid and electrolyte balance • Build up of nitrogenous waste—Azotemia • 20:1 (or greater) ratio of BUN to Creatinine Prerenal Azotemia Postrenal Azotemia Intrinsic Renal Disease Prerenal Azotemia • Most common cause of AKI, 40-80% • Caused by renal hypoperfusion • Immediate resolution can prevent intrinsic kidney injury • If persists can lead to ischemia and intrinsic kidney injury Intravascular Volume Vascular Resistance Low Cardiac Output Post Renal Azotemia • • • • Least common cause for AKI, 5-10% Very reversible once found Caused by urinary obstruction BPH (most common cause), stricture, cancerous mass, ureteral stones Obstruction Luminal pressure Kidney damage Poor Perfusion GFR Intrinsic Renal Disease Acute Tubular Necrosis -Ischemic or toxic Insult -Accounts for 85% Intrinsic AKI Acute Glomerulonephritis Acute Interstitial Nephritis -Immune complex -Interstitial inflamdeposition matory response -Rare, accounts for -Accounts for 5% Intrinsic AKI 10-15% of Intrinsic AKI Chronic Kidney Disease (Chronic Renal Failure) Chronic Renal Failure Chronic Kidney Disease 20 million adults—1 out of 9 15% due to: -Glomerulonephritis -Poly Cystic Kidney -Urological Causes 15% have other or unknown causes 70% Late Stage Kidney Failure Due to HTN and/or DM General Considerations • An inciting event starts the process • Rarely reversible even after inciting event removed • Nephron destruction is the hallmark • Compensatory hypertrophy of remaining nephrons may keep serum creatinine normal • ARBs and ACE inhibitors help reduce hyper filtration injury—slow progression of CKD Staging GFR > 90 • Kidney damage with normal or > GFR • Goals: – Diagnose and treat cause (if found) – Treat comorbidities (HTN & diabetes) – Slow progression Staging GFR 60-89 • Kidney damage with mild < GFR • Goals: estimate progression Staging GFR 30-59 • Moderate < GFR • Goals: evaluate and treat complications Staging GFR 15-29 • Severe < GFR • Goals: begin preparation for dialysis and/or renal transplant Staging GFR < 15 • Kidney failure • Goals: – Dialysis when GFR < 10 mL/min for non diabetic – Dialysis when GFR < 15 mL/min for diabetic – Renal transplantation Complications GFR 60-89 Cardiovascular: CKD patients have > morbidity and mortality than general population – Hypertension: most common complication – Coronary artery disease: address modifiable risk factors – Congestive heart failure – Pericarditis Complications GFR 30-59 • • • • • Disorders of mineral metabolism Bone disease and fracture risk Also leads to increased vascular calcification Anemia: due to < erythropoietin production Coagulopathy: due to platelet dysfunction Complications GFR 15-29 • > phosphate, < calcium, < vitamin D and secondary hyperparathyroidism • > phosphate may be seen in stage 3 disease • Most common bone lesion is osteitis fibrosa cystica—subperiosteal lesions Complications GFR < 15 • Metabolic acidosis because loss of nephrons does not allow for sufficient production of bicarbonate • Uremic syndrome: weakness, fatigue, pruritus, SOB, neuropathies, < concentration, irritability • Endocrine disorders: hypoglycemia in DM Treatment • • • • • Dietary Management/Renal Nutritionist Protein restriction: mixed reviews Salt restriction: 2g/d Na Potassium restriction: < 50-60 mEq/d Phosphorus restriction: < 4.5 mg/dL Pre dialysis, 3.5-5.5dL on dialysis • Magnesium restriction: Magnesium containing laxatives/antacids relatively contraindicated Treatment • Dialysis: – Initiate at GFR 10 (Serum Cr 8) non DM – Initiate at GFR 15 (Serum Cr 6) DM • Types of Dialysis: – Hemodialysis – Peritoneal dialysis • Kidney transplant Glomerulonephritis Glomerulonephritis Glomerulonephritis (Nephritic Syndrome) • Relatively uncommon cause of acute kidney injury, accounts for about 5% of intrinsic renal failure • Disorder of glomeruli • Severity is based on the number of glomeruli involved Signs & Symptoms • • • • Asymptomatic in 50% of acute cases Edema: primarily eyes and scrotum Hypertension with impaired renal function Headache, visual disturbance, nausea and vomiting • Anorexia, abdominal pain, joint pain • Malaise is common in older patients Causes • Peri or post infection—especially throat or skin infection • IgA nephropathy (Berger’s disease) • Lupus nephritis • Cryoglobulinemic glomerulonephritis—usually associated with hepatitis C • Membranoproliferative glomerulonephritis Diagnosis • Hematuria: red blood cell casts are specific to glomerulonephritis • Low to moderate proteinuria • Anemia and elevated WBC • Biopsy can help determine cause, amount of scarring and potential for reversibility • Physical exam as described Treatment/Prognosis • No specific treatment is available in most cases • Protein and sodium restriction • Diuretics, steroids, cyclophosphamide selectively • Leads to chronic renal failure in about 0.1% of children and 25% of adults Nephrotic Syndrome Nephrotic Syndrome Nephrotic Syndrome • Disorder of the glomeruli with excessive proteinuria • 1/3 have systemic renal disease: diabetes, amyloidosis or systemic lupus erythematosus • Remainder have idiopathic nephrotic syndrome • Most common in pediatric patients Signs & Symptoms • Early symptoms: anorexia and malaise • Puffy eyelids and tissue swelling from excess sodium and water retention • Abdominal pain • Muscle atrophy • Frothy urine Diagnosis • • • • Based on history, PE and lab findings Mistaken for heart failure in adults 24 hour urine shows proteinuria Low serum albumin due to increased urinary excretion • Protein and fat casts noted in microscopic UA • Urine: low sodium and high potassium Treatment • ACE Inhibitors or ARBs alone or in combination for prevention and treatment • Diet: normal protein and potassium; low saturated fat, cholesterol and sodium • High blood pressure is treated with diuretics • Anticoagulants • Infections—serious complication • Steroids Prognosis • In infection, allergy or nephrotoxic drug use, prognosis is based on effective treatment of underlying cause • In systemic lupus erythematosus or diabetes mellitus, drug treatment often stabilizes or decreases the amount of protein in the urine • Renal failure develops without treatment Polycystic Kidney Disease Polycystic Kidney Disease: Etiology • PKD is autosomal dominant or recessive • Autosomal recessive PKD is rare; 1/10,000 and causes renal failure during childhood • Clinical manifestations are rare before adulthood • All patients ≥ 80 yr have some manifestations Signs & Symptoms • One half of patients remain asymptomatic and are never diagnosed • Low-grade flank, abdominal and lower back pain due to cystic enlargement and infection • Acute pain is due to hemorrhage into cysts or passage of a calculus Treatment • Strict BP control • Prompt treatment of UTIs • Percutaneous aspiration or surgical removal of cysts • Hemodialysis, peritoneal dialysis, or kidney transplantation (cysts do not recur in grafts) Prognosis • By age 80, 50 to 75% require dialysis or transplantation • Rapid progression risk factors: early diagnosis, male, sickle cell trait, rapidly increasing kidney size, gross hematuria, hypertension • Patients usually die of heart disease (sometimes valvular), disseminated infection, or ruptured cerebral aneurysm Thank You