Irish Journal of Medical Science Abstracts of the Irish Paediatric Association

Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
B. Paturi, K. Reynolds, Dr.H.Greaney.
Paediatric Department, Sligo General Hospital.
Asthma in Ireland continues to increase at an alarming rate. Asthma now affects at least one in six
Irish children. In a worldwide study completed by the International Study of Asthma and Allergies in
Childhood (ISAAC, 1998). Ireland was ranked as having the 4th highest incidence in the world after the
United Kingdom, Australia and New Zealand1.
A clinical audit of the assessment and management of acute asthma in children presenting to the
paediatric department was undertaken in 2000. Following the audit, the findings were disseminated to
the specialty team and the importance of improving the recording of information and history taking was
There was also a role for introducing consistency in frequency of re-evaluating individual cases and
discharge planning identified.
To measure the impact of this action it was decided to undertake a re-audit and in doing so to close
the audit loop.
• To assess current practice in the assessment and management of children with acute asthma
presenting to the Paediatric Department
• To determine if change had occurred since the previous audit
• To identify areas for improvement.
• Asthma Guidelines for the Republic of Ireland, 2000
Following a literature review, a proforma was designed to collect details on history taking,
examination, assessment and management in the first 24 hours, documentation, discharge planning
and follow up.
A total of 28 patients were included in the study. Cases were identified concurrently on admission
between October – December 2003. The Paediatric Registrar undertook a retrospective chart review
in February 2004. (Source of data included medical and nursing notes). Data analysis was undertaken
by the Clinical Audit Support Team.
64% of children admitted were <3 years of age. 50% were referred from a general practitioner.
Routine admission information documented by the admitting doctor had improved since the original
audit. However important measures of the severity of the attack were not. Reassessment of the
patient occurred at irregular intervals, but all patients were reassessed within 24 hours of admission.
Recording of indicators of control of asthma prior to admission was poor.
A more structured approach to patient discharge was evident as almost all (90%) of cases had a
written discharge plan documented in patient notes.
There is room for improvement in staff training regarding the recording of information, history taking
and management of the child with asthma. The need for full clinical examination requires emphasis.
There is also a role for the introduction of consistency in the frequency of re-evaluating cases and
discharge preparation, such as assessment of inhaler technique and asthma self-management
The development of a structured template or care pathway for the assessment and management of
acute asthma might be helpful in ensuring that all children receive appropriate assessment and care.
On going education updates with each new group of medical staff to introduce and encourage the use
of evidence based guidelines in the management of asthma.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Paediatric Department, Erne Hospital, Enniskillen
AIM – To identify the percentage of Down’s syndrome children effectively screened for thyroid
dysfunction over the last 2 years in County Fermanagh The standard we used was that advocated by
the Down’s Syndrome Medical Interest Group which says all children with Down’s syndrome should
have biochemical testing of Thyroid Function every 2 years starting from age 1 & throughout life.
METHOD Children aged 0-16 years with Down ’s syndrome were identified through the Child Health
System(CHS). Both Hospital & Community Notes were studied and full details of all Thyroid Function
tests that had been done were obtained.
Analysis via Microsoft Excel.
35 children with Down's syndrome were identified of which notes were available on 33.
21/33(64%) were screened as per guidelines.Of the 12 who were not screened appropriately no
record of Thyroid function was available in 10 children.
9/33 (27%) had abnormal TFT’s.
Of these 1 (3.3%) was hyperthyroid with high T4 & low TSH
1 (3.3%) hypothyroid with low T4 & high TSH
7 (23.1%) with high TSH only (probable hypothyroid)
Age at presentation with abnormal TFT’s
Mean 2.5 years
Median 2 years
Range: 1 month – 13 years
This study confirms the high incidence of Thyroid dysfunction in children with Down's syndrome. There
are well researched guidelines as to how this condition can be identified. This study shows that
despite these, we are still not effectively screening this population. It is not all gloom and despair as a
similar audit in 1999 across the whole of Western Health and Social Services Board area showed that
only 33% were effectively screened at that time. We have therefore made some improvement. In
response to this study the following recommendations were made:
• All children with Down’s syndrome to be reviewed by Community Paediatricians yearly.
• Every Down’s child to have TFT at age 1 & every 2 years thereafter.
• More co-ordination between Community Health Visitors & Community Paediatricians
• Parents to be made aware of getting their Down’s children screened every 2 years via the
insertion of a Down's syndrome insert to the parent-child health record(Red Book)
• Re-audit in 2 years time.
Reference -
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
K Hassan, A Shankar, S Elkashif
Letterkenny General Hospital.
Children with Down’s syndrome have a variety of problems that need to be addressed individually.
Letterkenny is one of the few hospitals in Ireland that holds a monthly clinic for children with Down’s
1. To review the range of problems of children with Down’s syndrome in our catchment area.
2. To assess the feasibility of following children with Down’s syndrome at a dedicated monthly
All patients attending the clinic for children with Down’s syndrome were selected for the study. Data
was retrieved by a structured chart review.
Charts were studied with respect to age, gender, and organic problems including cardiac, endocrine,
ophthalmic, and auditory.
57 children attend the clinic for children with Down’s syndrome.
30 (57%) are male. On average six children visit the clinic each month. Each child visits the clinic
every three to twelve months, depending upon his/her clinical condition.
20 (35.1%) are aged less than 5 years, 17 (30%) between 5 and 10 years, 12 (21%) 11 – 15 years,
and 8 (14%) are 15 – 17 years of age.
32 (56%) had cardiac problems. The most common auditory defect noted was transient conductive
deafness secondary to chronic suppurative ottitis media ( 28 out of 57) (49%).
21 (37%) had ophthalmic complications, myopia being the commonest.
9 (16%) had thyroid defects (all hypothyroidism), two (22%) of which presented before two years of
Obesity was six times more common than failure to thrive. Three (5%) had asthma.
Other complications noted included acute myeloid leukemia, coeliac disease, insulin dependent
diabetes mellitus, autism, rheumatoid arthritis, and generalised epilepsy (one patient each).
Problems requiring surgical intervention were noted in 24 (42%) children, 21 of whom required cardiac
surgery, two surgery for Hirshprung’s disease, and one for pyloric stenosis.
Cardiac, auditory, and ophthalmic problems ( in that order) are most frequent in children with Down’s
syndrome in our catchment area, followed by hypothyroidism. They also have a variety of other
problems that are more common than in the general population.
For this reason, a dedicated clinic for children with Down’s syndrome is desirable.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Kelly. V, Wall. A, Elkashif. S, Kyne. L
Paediatric Unit, Letterkenny General Hospital, Letterkenny, Donegal
Introduction: In recent years controversy had surrounded the routine vaccination of children in this
country. For some primary care physicians and parents alike it is now a source of anxiety and has lead
to requests for single strain vaccines and administration of vaccines in a hospital setting. The referrers
were uncomfortable in administering the vaccine in their own practices as they felt that previous
reactions were significant and there was high parental anxiety. Our aim was to improve vaccine
uptake in this area. The overall vaccine uptake rates for Donegal at the beginning of the audit was
85% (MMR 79%) and at the end of 2001 it was 91%
(MMR 85%). We report on the results of an
audit of vaccines administered in our unit over a two year period.
Methods: Over a 2 year period 37 children were admitted to Letterkenny General Hospital for
vaccination. All the admissions were logged into a database. Each chart was reviewed and a
telephone questionnaire was carried out with a parent which was the mother in all cases. The
questionnaire included the source and reason for referral. It also included background details such as
previous reactions and allergy testing.
Results: In total 27 (72%) cases were fully reviewed. We were unable to make contact with 10
parents. 77% were boys and 23% girls. Children were referred by a variety of sources with the
majority (55%) from GPs. 60% had no formal allergy testing. The 40% who had been tested had
abnormal results. Among the reasons for referral were egg allergy (50%) and previous allergic
reaction to vaccination (26%). The previous reactions documented were skin rash (66%), irritability
(37%), and pyrexia (33%). None had had a severe anaphylactic reaction. The reactions had occurred
within 24 hours of the vaccine and the majority of the children (74%) were subsequently seen by the
GP. The children were admitted as a day case. Each child was seen at admission and consent was
obtained by the consultant. The vaccines administered were MMR (48%), Men C (25%), Egg free
MMR (12%), MMR booster (25%) and 5 in 1 (3%). They were observed for up to four hours post
vaccination and examined pre discharge.
Conclusion: Only one child had an adverse reaction (groin lymphadenopathy). This occurred in a child
who had received Egg free MMR. We reached the conclusion that there was no proven benefit to
administering vaccines in hospital. We also conclude that egg allergy is not a contraindication to MMR.
This audit outlines the workload involved in administering vaccines in hospital both for the medical and
nursing staff. However we can continue to expect these referrals from primary care physicians. Finally,
if this situation is to improve we must provide better education and support for our primary care
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Finn S., Kinsella W., Chan L., Byrne O., O’Mahoney O., Bourke B.
The Children’s Research Center, Dept Paediatrics, University College Dublin, Our Lady’s Hospital for
Sick Children.
Background: Spider naevi are vascular lesions on the skin and contain a central arteriole from which
radiates numerous fine vessels superficially resembling spider’s legs. They are observed in the region
of the superior vena cava and range in size from a pinhead to 0.5cm in diameter. Pressure on the
centre of the lesion with a pin causes blanching of the entire lesion. In adults, spider naevi are a well
documented sign of liver disease. There appears to be little agreement on the significance of spider
naevi in children.
Aim: We undertook to determine the number of naevi in children without liver disease, of different ages
to elicit the normal number of naevi in the paediatric population. We also examined those children
under our care with liver disease to see whether these had a significantly different number of naevi.
Materials and Methods: Children were examined in the gastroenterology and general medical out
patient, as well as children on admission to hospital, some in accident and emergency for the
presence of spider naevi. The age of the child, number, size and distribution of naevi were recorded
as well as presence or absence of liver disease. In those children with liver disease the presence or
absence of portal hypertension, cirrhosis, liver transplant and other stigmata of liver disease were
The hospital ethics committee approved the study and parental consent was obtained Results: 528
children were examined, 260 males and 268 females. Those children who were less than 1 year old
and over 15 years old were excluded as were those children who had liver transplant. Therefore, 34
children with liver disease and 426 children without liver involvement were eligible for the study. Of the
426 children without liver disease 61.9% had no naevi. A small minority of patients less than 5 years
old had any spiders. However, nearly half of all children over 5 years had at least 1 spider and 2.5% of
children in the over 5 year age group had more than 5 spiders, despite the absence of any signs of
liver disease. Forty seven percent of patients with liver disease had no spiders and 11.7% of them had
5 or more naevi. Of the 34 children with liver disease, 8 of 9 with cirrhosis had naevi, yet only 3 had
more than 5 spiders.
Conclusion: Children with liver disease rarely have large numbers of spider naevi. The finding of more
than 5 spider naevi is more frequent among children with liver involvement but also maybe seen
occasionally in unaffected children.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
E. Fitzpatrick , M.Rowland , B.Drumm .
Department of Gastroenterology, Our Lady’s Hospital for Sick Children, Crumlin. 2Department of
Paediatrics, University College Dublin,
Cyclical Vomiting Syndrome (CVS) is a disorder of children characterized by recurrent episodes of
intense vomiting lasting hours to days with a symptom free interval of weeks to months. We have
observed that the provision of a positive diagnosis and appropriate reassurance and explanation has
helped alleviate symptoms in some children.
Our aim was to follow up children who were diagnosed with CVS in our centre during the period 19932003 to determine (i) if making a positive diagnosis of CVS altered symptoms, (ii) the outcome
including the development of migraine.
We identified children diagnosed with CVS through our database. We reviewed the clinical records of
these children to define demographic features and the spectrum of disease in our population. We
contacted the caregivers of the CVS patients and administered a telephone questionnaire to establish
the resolution or persistence of symptoms. We also examined the perception of the family regarding
the diagnosis of CVS as well as the impact of a positive diagnosis and the provision of written
information on the child’s symptoms. The study was approved by the Ethics Committee of Our Lady’s
Hospital for Sick Children Crumlin.
Between 1993 and 2003, 51 children were diagnosed with CVS. The mean age at onset of CVS
symptoms was 5.9 S.D 3.3 years, and the mean interval between onset of symptoms and diagnosis
was 2.4 SD 2.1 years. M :F 1.5:1. 26/40 had previous investigations, and 21/40 had been admitted to
hospital with vomiting. Forty one caregivers were contacted (80% follow up) and forty agreed to
participate. The mean interval from onset of symptoms to telephone contact was 6.8 S.D 3.7 years No
new medication was prescribed at first visit. At follow up, vomiting had resolved completely in 24
(60%) of patients. With 33% reporting that a resolution of symptoms occurred either immediately or
within weeks of diagnosis. In the 16 with persistent vomiting, 10 reported a major improvement in
symptoms, and 4 reported a slight improvement. 16/40 (40%) now suffer either regular or occasional
migraine headaches, and 21/40 (53%) have travel sickness. At time of diagnosis only 50% of parents
said they agreed with the diagnosis, but at follow up 88% agree. 33 (83%) of parents remember
receiving the information leaflet at time of diagnosis. 22(56%) of parents felt that the provision of a
positive diagnosis and information made a significant impact on the severity of the vomiting.
The diagnosis of CVS is often delayed. The provision of a positive diagnosis and explanation was
followed by a complete resolution of symptoms in 33%. Long-term follow-up confirmed a major
improvement in symptoms in 25% of children. Thirty percent of children continue to vomit and forty
percent now suffer from migraine.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Hughes J.A.F.1 , Carson D.1 , McGlone O.1 , Hart P.2 , Wong K.M.3 and Ang M.3
1.Royal Belfast Hospital for Sick Children, 2 Ophthalmology Department, Royal Victoria Hospital, 3
Queens University Belfast
Diabetic Retinopathy is the leading cause of blindness and visual impairment in the adult working
population. The National Screening Committee has recommended annual retinal screening for all
people with diabetes over the age of 12 years. Poor glycaemic control is associated with an increased
risk of diabetic retinopathy.
To determine the prevalence of retinopathy in children and adolescents over the age of 10 years
attending the diabetic clinic at RBHSC. If retinopathy is present, does it correlate with the presence of
Microalbuminuria, Limited Joint Mobility (LJM) and glycaemic control?
152 children and adolescents enrolled in the study. The presence of retinopathy was assessed by
coloured digital retinal photography. The photographs were assessed and graded by a consultant
Ophthalmologist. All patients were clinically assessed for the presence of LJM. The presence of
Microalbuminuria was calculated as an albumin/creatinine ratio of greater than 3 and was taken from
the Twinkle database as was the mean HBA1c since diagnosis.
152 patients were enrolled. Age ranged from 10.0 to 19.9 years with a mean of 14.5 years. The
prevalence of retinopathy was 7.89% (12 cases). All had stage 1 retinopathy, 3 bilateral and 9
unilateral. The presence of microalbuminuria and LJM were 22.4% (34 cases) and 5.9% (9 cases).
The mean HBAIc ranged from 5.9% to 11.8% with a mean of 8.0%. In the patients with retinopathy
there was no correlation with the presence of microalbuminuria or LJM.
Early signs of retinopathy are present in adolescents with type 1 diabetes. All patients over the age of
12 should be screened annually.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
C. Martin, M McKay , T Clarke
There is increased recognition of alcohol abuse in society. Recently highlighted issues concern public
order disturbances and the prevalence of underage drinking, though these largely take the form of
anecdotal media reports. It has been our experience that there are increasing numbers of underage
drinkers presenting to the emergency department and we presented our figures from Tallaght
Children’s Hospital last year. Further to this study we undertook a national study through the IPSU
To collect reliable countrywide statistics on alcohol related problems in children.
Through the IPSU we contacted 135 consultant paediatricians monthly. We asked them to report all
patients who came under their care because of alcohol related illness or injury.
Once notified of a case we forwarded a questionnaire to the relevant consultant and have collected
and compiled the results for the period of January 2003 to January 2004.
The questions related to the Patient (demographic and social), to the incident (type of alcohol, location
etc) and to the clinical presentation.
The IPSU sent out a total of 1620 cards and received 1198 replies (74%)
59 alcohol related admissions were notified to us, 59 questionnaires sent out and so far we have data
returned on 44 cases.
Age: median 14 (range 12 – 17) 26 female and 18 male.
Location: Our study showed that 16(36%) of patients were brought to the hospital from a public
outdoor place.16 (36%) of incidents occurred at home or in a friends house. 13 patients were alone
when found
32 patients reported that this was their first incident.
There was a definite preference for Spirits 32 (72%) followed in preference by beer 10(22%)
Levels were measured in 30 cases with a median of 216, range (112 –380)
Clinical symptoms were reported in 33 cases, 18 (54%) had altered neurological status, 6 had
gastrointestinal symptoms, there were 4 injuries reported.
39 were admitted (88%)
Alcohol misuse amongst adolescents is nationwide and increasing.
The drinking of spirits in particular is a modern phenomenon and is reflected in the significantly high
blood alcohol levels at presentation.
The IPSU has been a valuable method of collecting data. Its strengths are its nationwide distribution, it
reminds people of ongoing studies and has a return rate of almost 75%
We have been disappointed with the numbers of alcohol related incidents reported to us. The Tallaght
and Castlebar studies would suggest a higher rate of incidents.
We would welcome discussion on this issue and would like to explore other data collection methods
e.g. HIPE data or emergency department statistics
Finally we need to move from collecting the data to influencing behaviour. Paediatricians need to
come forward as a group and call for prevention strategies to be implemented and use our position as
point of contact in these cases to make a significant change.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Finan E(1), McCoy B(1), Nicholson AJ(1), Vincenten J(2)
Paediatric Dept., Our Lady of Lourdes Hospital, Drogheda
European Child Safety Alliance, 1070 AD Amsterdam
Background: Drowning is the second leading cause of unintentional injury death amongst children,
with toddlers being the group at greatest risk.
Aims: (1) To study the pattern of drowning/near drowning in Irish children over ten years (2) To
compare Irish figures with EU statistics and (3) To make recommendations for drowning prevention
Methods: Childhood drowning deaths over a ten year period were examined using statistics from the
Central Statistics Office in Ireland and World Health Organisation data from EU and candidate
countries in Europe. We placed particular emphasis on identifying at- risk groups by looking at deaths
according to age, sex and place of occurrence. Near drowning statistics for Ireland were also
examined looking at age, sex, season of occurrence, length of hospital stay and subsequent outcome.
Results: 90 drowning deaths occurred in children 0-14 years in Ireland from 1992 to 2002 giving an
annual rate of 0.9 per 100,000 population. In 1-4 year olds, drowning mortality was 1.14 for boys and
0.4 for girls. For 5-14 year olds, drowning mortality was 1.88 for boys and 0.21 for girls. Best EU
figures were seen in the United Kingdom and Sweden (0.59 and 0.68 respectively for 1-4 year olds)
Age- standardised mortality rates in Europe from 8.7 per 100,000 in Latvia to 0.85 per 100,000 in
France and 1.26 per 100,000 in Germany. A striking male predominance (3:1) was evident in Ireland
and throughout Eirope. 64/90 (71%) of fatal drownings in Ireland occurred in open water areas, 8/90
(9%) occurred at home and a further 4/90 (5%) on farmyards.
Near drowning incidents requiring hospitalisation were on average 21 per year with, again, males
outnumbering females by2:1. Toddlers were found to be at greatest risk (accounting for 48% of total)
with the greatest incidence in summer months
Conclusions: Drowning is a major cause of accidental death in children, with males and toddlers at
greatest risk. For every death, an estimated 4 hospitalisations occur, accounting for a significant
childhood morbidity and events in the first 10 minutes following a submersion dictate the subsequent
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
E Finan, C Breathnach, F Sharif, M Kelly, AJ Nicholson
Paediatric Department,
Our Lady of Lourdes Hospital, Drogheda, Co. Louth
Background: Despite legislation, many young children in Ireland still travel in cars without appropriate
child restraints
Aims: (1) To study the impact of an antenatal educational intervention on car seat use for the journey
home and (2) to study all car passenger injuries and fatalities under 5 years of age presenting to
hospital over a 6 year period (1997-2003)
Methods: A prospective cross-sectional observational study of the means of transport of newborn
infants home was carried out for four 6 week periods from 1997 to 2002. In 1997, no information was
given and we observed whether mothers used a car seat for the journey home. From January 1998, a
30 minute educational intervention highlighting the importance of car seats and demonstration of their
use was delivered by the midwife at the final antenatal class. Prospective electronic injury surveillance
took place using the minimal dataset and all car passenger injuries under 5 years old were studied.
Data collected included the date, day and time of the injury, a text description, injury diagnosis,
whether admitted or not and the use of appropriate child restraints. Data analysis was via the Extractor
software package.
Results: Car seat use on discharge from the maternity unit rose from 16% (32/198) in 1997 to 93%
(264/283) in 2002 (p<0.001). In 1997, car seat use was associated with higher social class (p<0.01)
and breast feeding (p<0.05). Subsequent cohorts demonstrated car seat usage among all social
classes but a persistent non-compliant group (asylum seekers and those leaving hospital in a taxi)
was evident. Of 157 under 5 year old car passenger injuries, 100 (64%) occurred between 12.00 and
20.00 hours and the highest numbers (54%) presenting at weekends. There were 2 deaths, both
unrestrained rear seat passengers. Child restraint use rose from 32/70 (46%) in 1997-2000 to 57/85
(66%) in 2000-2003 (p<0.01). Front seat position dropped from 10/70 (14%) in 1997-2000 to 1/87
(1%) in 2000-3. Admissions halved over the same time periods (18.5% vs. 9%). Head injuries were
reduced in 2000-3 cohort (p<0.05)
Conclusions: Observed car seat use improves with an antenatal educational intervention with
subsequent reductions in car passenger head injuries and admissions.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
M. P. Stack, CA Ryan
University College Cork/ CUH
In 1979 Johns Hopkins University began a programme for gifted youth, and its influence spread
throughout America and has bases in Cork and Dublin in recent years. The programme works with
young people between 7 and 16 years of age of exceptional ability. The aim is to inspire them by
offering distinct opportunities in education that nurture intellectual ability. Academic achievement and
personal development are enhanced. In their own way ‘special needs’ are catered for. The aim of my
study was to compare certain known associations with intelligence in a group of gifted children
compared with age and sex matched controls attending national school.
This study took the form of a case control study. I used two postal questionnaires- one for parents of
the gifted children (GC) and a modified questionnaire for parents of a varied group of primary school
children (PC). Children between 8 and 12 years were included. The case group GC were based in the
cork region and would have IQ > 135, with abilities in abstract, verbal and numerical reasoning
formally measured via Ravens Standard progressive Matrices and the Drumchondra Reasoning test.
Controls PC were taken from 4 Cork Primary Schools, making an assumption on previous studies that
90% of these will fall in normal IQ limits. GC had a 58% (87) response rate and PC had 65 %( 128)
response rate. Case: Control 1:1.5.
1. Breast Feeding: The rate of breast feeding in the GC group was 73% (63/87) as compared to
29% (37/128) in the PC group. The mean duration of breast feeding was 7.16 months (SD
8.42) in the GC as compared to 4.79 months (SD 3.7) in the PC.
2. Maternal and paternal smoking was significantly lower in the gifted group. Nicotine induces
fetal brain hypoxia; also it causes increased cadmium accumulation in placenta which
interferes with metabolism and utilisation of zinc resulting in low birth weight infants.
3. Using the central statistics office social class coding system it was found that there were fewer
parents in social class 4, 5 and 6.
4. A higher percentage of mothers and fathers would have been more likely to achieve third level
education, mothers GC 66%: PC 32% and fathers GC 63%: PC 41%. Overall parents were
older in the GC group.
5. There was a significant difference in incidence of myopia in the GC group 32/87 (37%) as
compared to 7/128 (0.05%) in the controls.
6. Five developmental milestones were examined and compared with median values from the
Denver 2 developmental test and no difference was found between the groups.
This case control study identifies that gifted children can tend to come from parents of higher social
class, with parents who have attained higher levels of education, who are also less likely to smoke.
They were more likely to have been breast fed and seem to have a higher incidence of myopia. Some
genetic factors are obviously not modifiable however environmental risk factors seem here to offer
benefit to the development of intelligence. Visual and brain development are advantaged by
docosahexaenoic acid arachadonic acid provided in greater concentrations in breast milk. Maternal
smoking causes poorer performance on central auditory processing tasks. Better retinal development
in breast fed infants could lead to increased amounts of nearwork which can lead to myopia. The
origins and associations of high intelligence are difficult to pinpoint. Social class differences do exist,
however many confounding factors exist, including quality of parenting, quality of schooling and
stimulation provided. In conclusion multiple social, genetic and environmental influences have roles to
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
play in aetiology of intelligence, which has relevance to our practice from the point of view of children
with normal or low intelligence also.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
C Earley, C Costigan
Diabetes and Endocrinology Unit, Our Lady’s Hospital for Sick Children, Crumlin , Dublin 12
McCune Albright Syndrome is a rare, sporadically occurring genetic disorder, the exact incidence of
which is unknown. Clinical diagnosis requires the presence of at least two features of the triad of
polyostotic fibrous dysplasia, café au lait skin pigmentation and autonomous endocrine hyperfunction.
Precocious puberty is the most common endocrine feature of McCune Albright syndrome resulting
from gonadotrophin independent autonomous ovarian or testicular function. This occurs more
commonly in girls than boys. Other endocrine abnormalities include hyperthyroidism, hypercortisolism,
pituitary gigantism or acromegaly.
McCune Albright syndrome results from a postzygotic somatic mutation in the gene coding for the
alpha subunit of the stimulatory G protein (Gsa), an essential component of the adenyl cyclase
receptor. The upregulated Gsa results in hyperstimulation of the receptor with increased production of
cAMP.and increased hormone production.
Case Report
We describe two cases of McCune Albright syndrome in young girls. The first case presented at the
age of 3 years and nine months as a referral from the orthopaedic services. A lytic lesion had been
noted on the left femoral neck and fibrous dysplasia was suspected. Four months later episodes of pv
bleeding, increased breast tissue and increased weight were reported. Clinical examination revealed
the presence of two café au lait patches and signs of pubertal development. Her bone age was
considerably advanced at 6 years and 10 months and pelvic ultrasound demonstrated the presence of
bilateral ovarian cysts. LHRH stimulation tests showed a depressed LH, FSH response (peak LH<
0.4IU) and her serum oestradiol was 34pmol/l. Other endocrine investigations were normal. Due to
her young age ,major behavioural difficulties and considerably advanced bone age , treatment was
commenced with anastrazole, an aromatase inhibitor. Her symptoms and signs responded. Six
months later relapse occurred with vaginal bleeding, increase in breast size and a marked elevation of
the serum oestradiol 3160pmol/l. Tamoxifen, an oestrogen receptor inhibitor was added and clinical
remission was resumed.
The second case is a two years and 7 month old girl who presented with a 3 day history of pv
bleeding; breast development and increase in shoe size .Two areas of pigmentation were present. A
pelvic ultrasound showed a large cyst on the left ovary and serum oestradiol was markedly elevated
2221 pmol/l with a flat LHRH stimulation test (peak LH<0.1). Her symptoms settled spontaneously
without treatment .However she has now represented 3 months later with a further episode of pv
bleeding, increasing breast size and rapidly advancing bone age. Fibrous dysplasia was noted on x
ray. She is commencing on anastrazole.
Peripheral precocious puberty in McCune Albright syndrome is unresponsive to LHRH agonist
therapy. However recent experience suggests that oestrogen production can be successfully reduced
by aromatase inhibition (anastrazole) and oestrogen effect inhibited by oestrogen receptor blockade
with Tamoxifen.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Boilson B1, Moran A2, Oslizlok P2, Walsh K2,3
1Specialist Registrar in Cardiology, Mater Misericordiae Hospital, Dublin 7
2Consultant Paediatric Cardiologist, Our Lady’s Hospital for Sick Children, Crumlin, Dublin 12
3Consultant in Adult CHD, Mater Misericordiae Hospital, Dublin 7
Until the advent of the Amplatzer perimembranous VSD closure device in 2002 the only method
available to close perimembranous VSDs was surgery. The aim of this study was to evaluate the
success of transcatheter closure of perimembranous VSDs in Dublin.
Patients and Methods:
The charts of eighteen patients were reviewed. 13 patients were children where the youngest patient
at closure was 10 months old, the eldest 12 years. Five adults were also included in the study and
their ages ranged between 47 and 78 yrs. All patients had perimembranous VSDs. Four patients, two
adults and two children, had residual VSDs post previous surgical repair for Tetralogy of Fallot. One
child had Trisomy 21 and also had a PDA which was coiled and an ASD which did not require closure.
Another child had an associated bicuspid stenotic pulmonary valve for which successful percutaneous
balloon valvotomy was undertaken. One other child had two small associated ASDs which were
treated conservatively.
Six patients had significant pulmonary hypertension, in only one this related purely to the VSD. The
degree of left-right shunting observed was significant in only three patients.
Doppler velocities detected across the VSDs ranged from 2 to 5 m/s, the smallest defect diameter in
short axis view being 4 mm in diameter and the largest 14mm.
A device was successfully implanted in 17 out of 18 patients. In most an 8mm device was sufficient
for complete closure. The largest device used was 18mm. Only two patients had residual VSDs post
closure and in one case a second device may be required. One patient had mild to moderate TR post
procedure but this had been noted previously. No significant AI was detected as a consequence of the
procedure and no other serious complications such as high grade AV block or vascular problems were
reported. Two patients developed RBBB and left axis deviation post procedure but these patients have
not required pacing. Those patients who were significantly restricted prior to closure are now
symptomatically and functionally improved. Mean inpatient stay was approximately 48 hrs.
Transcatheter closure of perimembranous VSDs is a safe alternative to surgery in selected patients
with a high success rate and short duration of hospital stay. No serious complications occurred in this
small series.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Awareness, opinions and attitudes towards the August 1st 2004 58 hour working week.
Blackburn C1, Fitzpatrick E1, Kelly V2, Sherlock M3, Corcoran D1, Clarke T1.
Dept. of Paediatrics, Rotunda Hospital, Dublin, 2Dept. of Paediatrics, Coombe Women’s Hospital,
Dept. of Paediatrics National Maternity Hospital.
Context: The provisions of the EWTD relating to NCHDs are due for implementation on August 1st
2004.This states that by law from that date average weekly working hours must be reduced to 58
hours per week and that the maximum working day is to be 13 hours.
Aims: To ascertain neonatal NCHD opinions regarding the EWTD and it is effect on training, salary
and quality of life.
Method: We conducted a confidential postal survey of neonatal NCHDs in the Dublin and Cork
maternity hospitals.
Results: 37/47 completed surveys were returned (response rate 79%). 15 respondents were senior
house officers, 15 registrars, and 7 specialist registrars.
Male: Female 17:20, 18/37 were married and 13/37 had children.
96% (36/37) were aware of the EWTD, however only 19% believed it will be enforced. Mean hours
worked in the present system is 68 +/- 8.2 hours per week. 54% feel this is too many and 73% feel
these hours are not conducive to a good quality of life. 40% believe their quality of life will improve with
the proposed shift in working patterns, however 57% worry quality of patient care will disimprove.
70% feel their salary is satisfactory at present but 76% are worried about future remuneration. 68%
are concerned that there will be inadequate provision or time for teaching and research after the
EWTD is implemented. Other major concerns regarding the new system were unsociable hours
(94%), continuity of patient care (97%) and 97% of respondents felt that the emphasis will be on
service rather than training. 11/37 (30%) of respondents have experience of shift work, 9/11 prefer oncall.
When presented with sample rosters the majority would choose to work a block of seven nights
followed by a week off, rather than split nights.
Conclusion: Our study highlights significant NCHD concerns regarding the implementation of the
EWTD particularly in the areas of patient care, remuneration, quality of life and training.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Sri Paran T., Koenigs I., Fitzgerald RJ
Our Lady’s Hospital for Sick Children, Crumlin, Dublin
While VP shunts are effective way of draining CSF in hydrocephalus, complications are still a
significant problem. We retrospectively analysed charts of 94 children between the years 1993 and
2003 to estimate the common complications.
The commonest complication was shunt blockage (30%). This was followed by shunt fractures (4.2%).
Infection was 2.2%. When a shunt malfunctions due to blockage, the commonest area of blockage
was within the intraventricular component (90%). Most children who presented with shunt blockage
needed more than one revision. Overall 44 revisions were carried out in 23 children. Protein levels of
CSF did not correlate with frequency of blockage. In an endeavour to establish whether shunt
blockage is related to intraventricular catheter position, an analysis is made of the available images.
Furthermore, the recent literature in relation to shunt blockage is also reviewed.
There were no shunt fractures below the age of three years, and the routine policy of doing an X-ray
shunt series should be discouraged in children less than 2 years old with unitised shunts.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
M McCauley (Paediatric Registrar, Temple Street Hospital), S Dockeray ( Consultant Paediatrician,
Central Remedial Clinic), Dublin
AIM: To review the prevalence of neural tube defects in Ireland for the 2 year period 2001-2002 as
identified by the IPSU reporting system , subsequent to the high profile campaign by the dept of health
in the republic of Ireland and northern Ireland on folic acid supplementation peri-conceptually.
METHODS: During the 2 year period, the IPSU agreed to include neural tube defects as one of their
reporting conditions. Paediatricians with a case to report did so via the green card system to the IPSU
office. They were then asked to complete a questionnaire which contained information on the
following: infant’s DOB, sex, gestational age, maternal nationality, family history, maternal folic acid
Other cases of NTD’s not identified via the IPSU system, were collected from other sources, namely,
maternity hospital reports, HIPE, Hospital information unit, hospital databases, Eurocat.
RESULTS: During the 2 year period, 62 cases were reported via the IPSU system, however, 3 of
these were duplication,( eg. Reported by both neonatologist and paediatrician) giving a reported
incidence of 59. Of these 59 cases, we received completed questionnaires on 42. In the case of the
returned questionnaires, information was complete in only 16 of them. The information most frequently
omitted was that regarding family history and maternal folic acid intake peri-conceptually. 29 of the 42
completed questionnaires had definite information on maternal folic acid intake.
An additional 35 babies were identified from the other sources listed above, giving a total of 94 cases
for the 2 year period.
DISCUSSION: Why are we failing to identify more cases of NTD’s via the IPSU system? Why are we
receiving incomplete questionnaires? These questions are important ones, as given the falling
prevalence of NTD’s in Ireland, we need to establish exactly why that is the case. Also, the number of
paediatric NTD cases is diminishing and thus we need to set up appropriate services and
multidisciplinary follow-up for these patients. This, & all results, will be discussed at the forthcoming
international meeting in Spina Bifida & Hydrocephalus.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Fleming P1, Carroll A1, Lanigan B1,2, O Keefe M1,2, Gorman W A1, Murphy JA1
1. National Maternity Hospital, Holles Street, Dublin 2.
2. Children’s University Hospital, Temple Street, Dublin 1.
Introduction: Retinopathy of Prematurity (ROP) is a developmental vascular proliferative disorder that
occurs in the incompletely vascularised retina of premature infants.
Aim: We included all extremely low birth weight infants (ELBW) at NMH from 1996-2001. We
assessed a) the rate of ROP, b) the severity of eye disease, c) need for laser therapy and d) follow up
Method: Eye examination data was obtained by retrospective chart review and eye examination
records. Data included gestational age (Gest age), birth weight (BW), Apgar 5 mins-, ventilation,
antenatal steroids, ROP stage and laser treatment. Follow up progress was reviewed. Infants
admitted just for eye assessment only from other institutions were excluded from the study.
Results: A total of 117 ELBW were included. Mean Gest 27.3 (23-32)wks, mean BW 836.68 (5001000)g. M: F 1:1.38. 75 (64%) had ROP.
In the ROP subgroup mean BW 811 (500-1000)g and mean Gest 26.4 (23-31) wks. M: F-1:1.41.
In the No ROP subgroup mean BW was 882.26 (500-1000)g and mean Gest was 28.8 (26-32) wks.
M: F 1:1.33.
Of the 75 infants with ROP 29 (38.6%) required Laser. Laser was required in 100% of infants born at
23 wks, 75% (24 wks), 25% (25 wks), 50% (26 wks), 41% (27 wks), 20% (28 wks) and no laser was
required for infants born at 29 weeks and above.
On f/u 4 infants (3%) were bilaterally blind, 1 had unilateral blindness. 13/61 (21%) required glasses.
10 (16%) strabismus, and 38/ (62%) normal vision.
Follow up was available for 40/42 of our unaffected infants. Of these 38/40 (95%) had a normal
examination and 2 had squints.
In ROP subgroup 63% received antenatal steroids. In the No ROP subgroup 75% received antenatal
Conclusion ROP remains a major problem in NICUs. Screening and appropriate laser intervention is
essential in the achievement of good outcomes. Our findings show that laser therapy is very likely for
those under 27 weeks. We also demonstrate that follow-up is imperative.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Department of Paediatrics. Cork University Hospital. Cork. Ireland.
Department of Rheumatology and Sports Medicine, Cork University Hospital. Cork. Ireland.
Department of Accident and Emergency. Cork University Hospital. Cork. Ireland.
Department of Epidemiology and Public Health. University College Cork. Cork. Ireland.
Background: Childhood injuries are a major source of morbidity in industrialized countries, and many
of these injuries occur on playgrounds. The demographics of playground injuries occurring in Irish
children however has not previously been adequately described, as reflected by the paucity of
publications in the medical literature.
Objectives: The purpose of this cross-sectional study was to provide up-to-date demographic
information on the nature of these injuries in an Irish population.
Materials and Method: Data was collected on all children (< 17 years of age), injured in playground
activities, presenting to an emergency medical department of a major teaching hospital in Ireland, over
a 6 month period. The data, which included activity, age, sex, mechanism, type, site, and
management, was entered into a specially designed database.
Results: Retrospective analysis was performed on 23,000 charts, and 81 children were found to have
presented with a playground related injury, none of which were wearing any protective gear. Swing
injuries presented most frequently (26%), followed by slide (24%), climbing frame (22%) and bouncy
castle (16%) injuries. Saturday (9%) and Sunday (11%) were the least frequent days of injury
presentation. Swing injuries presented proportionately more frequently in girls than boys (p=0.04), and
there was a strong trend towards significance of a higher frequency of presentation of climbing frame
injuries in boys compared to girls (p=0.07). The most frequently injured age category was the 5-6 year
old category, accounting for 35.8% of the injuries. The most frequent mechanism of injury was fall
(88%), followed by collision with persons (6.2%). The most frequent general site of injury was upper
limb (64%), followed by lower limb (17%) and head (12%). The most frequent specific site of injury
was the elbow (28%), followed by wrist (17%), both of which occurred more frequently in climbing
frame injuries. Fractures were the commonest type of injury (56%), followed by skin / soft tissue injury
(29%). Plain X-Rays were required in 85% and blood samples in 2.5%. Pain relief was required in
47% of injuries, but children aged 1-6 years were administered analgesia less than children aged 7-16
years (28% v 70% respectively, p=0.007). Physiotherapy was not requested for the management of
any injury. Only 22% of injured children were given advice regarding RICE. None were given injury
preventive advice. 12% of children required admission.
Conclusions: This is the first reported study to describe the demographics of playground injury in
Ireland. The data provided in this study may raise awareness of the nature of injury affecting children
in each of these activities, and would be helpful in formulating much needed injury prevention
strategies. Initially this could be focused on providing better advice on RICE and injury preventive
advice, possibly with an emphasis on protective gear.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
S O’Connell1, A O’Marcaigh2, C McMahon2, O Smith2, M Cotter1
Children’s University Hospital, Temple Street, Dublin.
Our Lady’s Hospital for Sick Children, Dublin
Introduction: Immune thrombocytopenic purpura (ITP) in children is usually a benign condition, which
recovers spontaneously within six to eight weeks. Current management guidelines advise that children
with acute ITP and mild clinical disease may be managed expectantly, irrespective of platelet count.
Although most common between the ages of 2 and 5 years, it also occurs in infants. Neonatal
autoimmune thrombocytopenia, caused by transplacental transfer of maternal platelet autoantibodies,
occurs in around 10% of infants born to mothers with ITP. Recent evidence indicates that serious
haemorrhage, including intracranial haemorrhage, occurs in 1% or less. It is reported that platelet
counts in affected infants are lowest in the first week of life.
Objectives: To review the presentation and management practice of young infants with severe
thrombocytopenia (platelet count less than 20 x 109/l).
Methods: Seven patients under the age of 4 months (range 4 to 14 weeks) presented with severe
thrombocytopenia. We examine the presenting features and treatment.
Results: Severe thrombocytopenia was present in all seven patients at presentation (range 4 -11 x
109/l). Neutropenia (less than 1.0 x 109/l) was also present in three of the patients.
Diagnosis: In two patients, the cause of thrombocytopenia was neonatal autoimmune
thrombocytopenia secondary to maternal ITP. In both of these patients, the infants’ platelet count had
been normal in the first week of life. In both patients, the mother had been treated with
immunoglobulin (IVIG) in pregnancy. In the other five patients, the diagnosis was ITP.
Treatment: All children except one were treated with IVIG. Two infants also received platelet
transfusions. Two patients also received steroid therapy.
Associated features: There was a history of recent vaccination in two patients. One child had a
clinically proven urinary tract infection. Infection was clinically suspected but not proven in two
patients. One patient was found to have coarctation of the aorta.
Conclusions: Although uncommon, ITP does present in the very young infant. The differential
diagnosis should include a delayed onset of neonatal autoimmune thrombocytopenia. In this latter
condition, it is commonly reported that the lowest platelet counts occur at 3 to 5 days of age.
However, it has been our observation that patients may present at several weeks of age, despite
having had normal platelets during the first week of life. The administration of maternal IVIG or
acquired infection may affect the timing of presentation. Hence, we would advise that platelet counts
should be checked again after the first week of life even if normal initially; a fall in platelet counts
should prompt further monitoring including screening for infection. Precipitating factors for the
development of ITP include recent vaccination and intercurrent infection. IVIG was used in all but one
patient, although current guidelines advocate a watch and wait approach. IVIG is a pooled multi-donor
blood product, which is not without side-effects. There appears to be a bias towards active treatment
with IVIG in infants with ITP, although there is no evidence that this age group should be treated
differently from older children.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
J Acheson, MG Jenkins, C Macleod, C. Patterson.
Emergency and Paediatric Department, Antrim Area Hospital.
Department of Medical Statistics, Queen’s University Belfast.
Introduction - Meningococcal disease mortality rates remain high and outcome is heavily dependant
on early recognition, prompt treatment and appropriate placement. There are a number of known
meningococcal prognostic scoring systems currently used to predict fatal outcome.
Objectives - Could a new scoring system be developed, applied to children with meningococcal
disease in A+E, and used to promptly determine those who will require intensive care admission?
Design - Retrospective case note study of 88 cases in children who presented to Antrim Area Hospital.
The cases were identified through Public Health notification of meningococcal cases over a three year
Method – Using the raw data, category variables underwent chi-squared testing and continuous
variables underwent t-tests. Multi logistic regression was performed to examine the independent effect
of each significant variable. Using the co-efficient from logistic regression a new scoring system was
produced which we have called the Antrim score. Scores were then calculated for this new score and
in eight known meningococcal scoring systems including Garlund, MOC, Leclerc, Tesoro, Stokland,
Lewis, Glasgow Meningococcal Septicaemia Prognostic score (GMPSS) and Algren. ROC curves
were calculated for each scoring system and compared. The sensitivity and specificity for ICU
admission was then calculated for each separate score in the Antrim system. The score with the
highest percentage in both sensitivity and specificity was taken as an indication for possible ICU
Results – The Antrim score consists of 4 variables; age, disturbed consciousness, capillary refill time
and peripheral vasoconstriction. These scores were then compared to the prognostic scoring systems
using a ROC curve. The Antrim score had an area of 0.97. This compares to an area of 0.94 for the
GMPSS, which was the best of the current eight prognostic scoring systems. A score of 8 or greater in
the Antrim score had the highest percentage in both sensitivity and specificity.
Conclusion – This simple clinical score shows potential but will need validated. A score of 8 or greater
in the Antrim score could be used as an indication for possible ICU admission.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Department of Paediatrics. Cork University Hospital.Cork. Ireland.
Department of Rheumatology and Sports Medicine, Cork University Hospital. Cork. Ireland.
Department of Accident and Emergency. Cork University Hospital. Cork. Ireland.
Department of Epidemiology and Public Health. University College Cork. Cork. Ireland
Department of Accident and Emergency. Waterford Regional Hospital.
Background: In the Irish sporting arena, participation by children in soccer, rugby and Gaelic football is
increasing in popularity. As a result, sport related injuries (SRIs) are common. A comparison of the
demographics of injuries in these 3 physical contact ball sports in Irish children has not previously
been adequately performed, as reflected by the paucity of publications in the medical literature.
Objectives: The purpose of this cross-sectional study was to provide up-to-date demographic
information on the nature of these SRIs.
Materials and Method: Data was collected on all children (< 17 years of age), injured in these 3 sports,
presenting to an emergency department of a major teaching hospital, over a 6 month period. The data,
which included sport, age, sex, cause, type, site, and management, was entered into a specially
designed database.
Results: Retrospective analysis was performed on 23,000 charts, and 409 SRIs were identified over a
6 month period. Soccer injuries presented most frequently (56%), followed by Gaelic football (24%)
and rugby (20%) injuries. Overall, injured males presented more frequently (82-93%), and were 0.5
years older than their female counterparts in each of the three sports. In the 5-8 and 9-12 year age
groups, soccer and Gaelic football injuries predominated, but in the 13-16 year age groups, rugby
injuries predominated. The predominant mechanism of injury was different in each sport, in soccerfalls (38%), in Gaelic football- collisions with football (37%), and in rugby- collisions with person (55%).
Upper limb injuries predominated in all 3 sports, but were the commonest injury in Gaelic football
(66%). Lower limb injuries were more common in soccer (35%) and head (14%) and trunk (9%)
injuries were more common in rugby. Fractures were the commonest type of injury in soccer (50%)
and Gaelic football (42%), and soft tissue injuries were the commonest type of injury in rugby (44%).
X-rays were performed in 91% of cases, with 48% revealing fractures. Analgesia was required by 50%
of cases. 8% required admission and 82% required follow-up. No documented use of protective gear
or preventative advice was recorded for any of the SRIs seen.
Conclusions: This is the first reported study to compare injury in these 3 physical contact ball sports.
The data provided from this study may raise awareness of the nature of SRIs affecting children in
each of these sports, and would be helpful in formulating much needed injury prevention strategies.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Bhatia R*., Hughes C*., Hananoe B† ., Cormican M†., Loftus B.G*
*Dept. of Paediatrics, University College Hospital, Galway
Dept. of Microbiology, University College Hospital, Galway
We present the case of a two and a half year old girl who presented with a brief febrile seizure, and
drowsiness. She had a past history of pneumococcal meningitis treated at another institution when
she was nine months of age with resulting developmental delay and deafness. On examination she
was drowsy, pyrexial and tachycardic with poor peripheral perfusion and clinical evidence of
meningism. Blood cultures were drawn and fluid resuscitation commenced. She was immediately
commenced on intravenous cefotaxime 50mg/kg qds empirically. Culture of cerebrospinal fluid taken
twelve hours after admission grew Streptococcus Pneumoniae serotype 9V with intermediate
susceptibility to penicillin but susceptible to cefotaxime and vancomycin. Over the subsequent seven
days, there was some clinical improvement but she had persistent fever, irritability, neck stiffness and
headache. A repeat lumbar puncture on day eight was still positive for S. pneumoniae on culture.
Vancomycin therapy was added and there was prompt resolution of residual clinical signs. A repeat
lumbar puncture three days after initiation of vancomycin was sterile and vancomycin levels in the
spinal fluid were confirmed as adequate to inhibit the isolate. She completed fourteen days of
combined vancomycin and cefotaxime therapy. Immunodeficiency investigations were negative,
ultrasonography showed a normal spleen and computed tomography of her brain showed no evidence
of a dural defect.
Failure of cefotaxime monotherapy for S. Pneumoniae meningitis is well described for isolates that are
resistant to cefotaxime on in-vitro testing. In this case laboratory parameters indicated susceptibility to
cefotaxime. However, there was unequivocal clinical and laboratory evidence of failure of therapy.
This case emphasises the importance of recommendations for inclusion of vancomycin in initial
empirical therapy of meningitis when S. Pneumoniae is confirmed or suspected and reflects the
limitations of in-vitro susceptibility testing in predicting therapeutic response
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
A Chillingworth, S Thavagnanam, J Davis, J S Craig
Royal Jubilee Maternity Services , Belfast
Immediately after birth sick preterm infants are at risk of significant protein catabolism and energy
deficit. Parenteral nutrition (PN) is the mainstay of promoting protein accretion, and traditionally is
prescribed during office hours on an individualised basis. This leads to an inevitable time delay in
many infants in the provision of protein and energy. It has been suggested that “off the shelf” bags of
standardised parenteral nutrition (SPN) may allow earlier introduction of protein and energy and
therefore reduce the duration of negative nitrogen balance after birth.
To assess whether availability of SPN would lead to the earlier provision of protein to infants admitted
to the Regional Neonatal Unit, Royal Jubilee Maternity Service.
Forty infants were studied. Twenty infants received individualised parenteral nutrition (IPN) and twenty
infants received SPN. The time from admission to the commencement of PN, protein content and
reasons for interruption of PN were collected on each infant. The two groups were compared using
non parametric statistical tests.
The mean (SD) time to introduction of PN was 18.8 (6.7) hours in the SPN group versus 35.2 (15.9)
hours in the IPN group, P< 0.005.
Availability of a standardised parenteral nutrition bag allowed significantly earlier introduction of amino
acids to neonates. Further research is required in order to determine the most appropriate SPN
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
M Doddakula, SM Gormally
Dept. Neonataology, Our Lady of Lourdes Hospital, Drogheda.
Background: Neonatal jaundice is a common neonatal problem, occurring in 80% of full term babies.
Various non-invasive methods of bilirubin levels have been developed. The Minolta Hill-Rom AirShields Jaundicemeter ( JM-103) is a non invasive optical method which assesses jaundice by
measuring the absorbance of reflected light in blue and green wavebands to measure yellowness of
the skin.
Aim: To evaluate the accuracy and usefulness of a transcutaneous bilirubinometry method (TcB)
developed by Minolta (Jaundicemeter JM-103) as a method of assessing jaundice in full-term babies.
Methods: The study was conducted between July 2003 and February 2004 in the postnatal ward of
Our Lady of Lourdes Hospital, Drogheda. The study group consisted of neonates over 35 weeks
gestation and less than 6 days of age, who were clinically deemed to be jaundiced enough to require a
bilirubin level. A serum bilirubin (SBR) was performed. At the same time, a TcB measurement was
recorded on the forehead by AM, using a Minolta HillRom Airshields Jaundicemeter JM-103. Prior to
use on a daily basis, the instrument was calibrated. Serum bilirubin was measured by an Olympus
AU-640 analyser, utilizing oxidation of bilirubin to biliverdin by potassium ferricyanide in the presence
of caffeine. None of the infants had received phototherapy at the time of measurements.
Results: A total of 132 serum bilirubin measurements were performed on 120 neonates. One hundred
and one infants had 3 measurements performed while the remaining 31 infants a single measurement
The male to female ratio is 1.3:1. The infants (mean gestation 38 weeks, range 35-41 weeks) ranged
in age from 6 to 144 hours (mean 56 hours). The study group consisted of 102 white caucasian
infants, 12 infants from African parents, and 1 from Lituanian,, Philipino, Ukranian, Chinese, Polish
and Italian parents respectively. The average birth weight was 3582g. Seventeen babies were less
than 3000gms, 82 babies between 3000 and 4000gms and 21 were greater than 4000gms in birth
weight. The overall correlation coefficient was 0.88.
Conclusion: The Minolta Hill-Rom Air-Shields Jaundicemeter ( JM-103) is an accurate and noninvasive method of assessing jaundive in term, well infants under 144 hours of age.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Ni Shuibhne N, Ryan S, Costigan C, Matthews TG, Corcoran D, Clarke T.
Department of Neonatology, the Rotunda Hospital, Dublin.
Hypopituitarism can cause life-threatening hypoglycaemia.1 We describe an unusual case of
hypoglycaemia and the challenges in making the diagnosis.
Case Report:
A three-day-old term female infant presented with a twelve-hour history of poor feeding, and brief
apneic spells shortly before admission.
Birth history was uneventful and baby was discharged home day 2 well. Her parents were
nonconsanguineous. However, four maternal uncles had died during the neonatal period, two of
unknown causes, one stillbirth and one of cardiac causes.
On examination, the baby was hypotensive, hypoglycaemic (0.6mmol/l), hypothermic (346), hypotonic
and apneic, and subsequently had a generalized seizure. Cardiac disease and sepsis were excluded.
Initial investigations were as follows: blood glucose 1.6mmol/l, urinary ketones, abnormal
transaminases (AST 112, ALT 120), mixed hyperbilirubinaemia (direct serum bilirubin 20(0-25),
indirect 213), hyponatraemia 126(133-142), markedly elevated creatine kinase 1618(0- 200), low CSF
glucose 1.7(2.5-3.9) and elevated CSF lactate 3.75 (1-2.2). Serum thyroxine was 9 (12-26) and serum
cortisol 100nmol/l. Ophthalmologic examination revealed bilateral optic nerve hypoplasia. Cranial
Ultrasound was normal however subsequent MRI Brain confirmed optic nerve hypoplasia with tiny
chiasm, and posterior pituitary ectopia with no detectable anterior pituitary or pituitary stalk.
A presumptive diagnosis of Hypopituitarism was made and baby was treated with exogenous
thyroxine and hydrocortisone, which normalized her glucose levels. However she remains hypotonic
and slow to feed.
Hypopituitarism can have devastating consequences. It is unlikely our case will have a normal
neurodevelopmental outcome. The prognosis for normal visual acuity is also guarded. Heightened
clinical suspicion of possible endocrine aetiology is paramount in early diagnosis.
The spectrum of Septo optic dysplasia (Optic Nerve hypoplasia, absent septum pellucidum and
hypopituitarism) is well described. Our case is a further illustration of the spectrum. Posterior pituitary
ectopia in conjunction with optic nerve hypoplasia is highly predictive of pituitary hormone deficiency.2
In our case, MRI brain and ophthalmologic examination provided the diagnosis.
1.Stanley CA, Baker L. The causes of Neonatal Hypoglycaemia. N Engl J Med 1999;340:1200-1.
2.Brodsky MC, Glasier CM. Optic Nerve Hypoplasia.Clinical significance of associated central nervous
system abnormalities on magnetic resonance imaging. Arch Opthalmol 1993;111:66-74.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
M. Sherlock, W. Gorman, N. Teo, P.McParland, J. Murphy
The National Maternity Hospital, Holles Street, Dublin 2.
Introduction: The incidence of non – immune hydrops (N.I.H) is 1/2500 – 1/4000. However, it is seen
more frequently in tertiary referral centres. While there are multiple causes, a significant proportion
remain idiopathic. It is now more readily diagnosed with the advent of sophisticated ultrasound
Aim: The aim of this study was to review the cases of N.I.H. cared for at the NMH over a 12 month
period to assess their diagnosis and subsequent treatment.
Methods: A retrospective chart review was performed of all cases of non-immune hydrops over a 12
month period.
Results: 4 infants were identified. 2 infants were diagnosed antenatally with supraventricular
tachycardia. In both cases the cardiac rhythm was restored to normal with maternal use of flecainide
and/or digoxin. In one of these babies, the hydrops had resolved by the time of delivery. 2 infants were
diagnosed with Down Syndrome. Neither of these infants had a significant cardiac abnormality. Both
of these infants had a complicated postnatal course and one infant died.
Conclusion: This condition clearly poses a perinatal diagnostic dilemma. The causes are multiple.
Increasingly, the underlying cause is identified antenatally. Immune causes must be excluded. The
use of middle cerebral artery doppler studies is beneficial in identifying underlying haematological
causes. It is essential to consider arrhythmias as a cause as they are often amenable to treatment.
Chromosomal analysis is important as Downs syndrome is being increasingly recognised in cases of
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
M. Sherlock, W. Gorman, V. Donoghue, J. Murphy
Introduction. The occurrence of neonatal cerebral infarction is being increasingly recognised.
Infarctions can be ischaemic or haemorrhagic. It most commonly presents as seizures but can also
present with respiratory compromise. This series represents five babies who recently presented to our
neonatal unit and were subsequently diagnosed as having a stroke.
Methods: A prospective review of all infants diagnosed with a stroke, over a 12 month period, in the
neonatal unit of the National Maternity Hospital, was performed. Factors assessed included gestation,
mode of delivery, Apgar scores, cranial ultrasound and MRI brain findings. Placental histology was
also assessed.
Results: Five infants ( 3 male, 2 female) were identified. All infants were term (gestation ranged from
37 – 40 weeks). 3 infants were delivered by SVD. One infant had a ventouse–assisted vaginal
One infant was delivered by emergency LSCS. All infants had an Apgar Score of ≥ 7 at 1 minute. 4
infants presented with seizures. One infant presented with apnoea. The stroke lesion was detected on
cranial ultrasound in all infants and confirmed on MRI brain scanning. Placental histology was varied
and included normal findings, fetal vessel vasculopathy and mild chorioamnionitis. The clinical follow
up time ranges from 3 weeks to 9 months. To date, 4 of the infants are neurodevelopmentally normal.
One infant had a normal follow up examination at 3 weeks. The remainder of his follow up is in the
Conclusion: Neonatal stroke can present in a variety of ways including apnoea and seizure. With the
advent of better imaging, it is being increasingly recognised. The condition has a good outcome.
Recovery is common, most likely due to the newborns brain plasticity. All infants will require long-term
follow up.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
S, Deiratany, RM, Watson, A, Irvine
Department of Dermatology, Our Lady’s Hospital for Sick Children, Dublin 12 Ireland.
Kerion is an inflammatory form of tinea capitis, which may progress rapidly into a fulminant infection
(severe large abscess) if untreated.
This study underscores the need for prompt recognition and appropriate treatment of this condition
and to avoid the surgical treatment.
Clinicians and surgeons need to be aware of varying of presentation of inflammatory fungal disease to
avoid misdiagnosis as bacterial infection.
In our review we found 18 cases of Kerion presented to the dermatology clinic from1994 to February
5 treated surgically with incision and drainage and anti fungal treatment.
13 were treated conservatively with anti fungal treatment only.
All the surgically treated Kerion had significant scaring and the entire conservative treated cases; the
scars were less than 3 cm.
4 out of 18 cases had positive fungal culture.
Antifungal treatment of Kerion for 6 weeks or more is appropriate treatment instead of surgical
treatment. And leaves less or no scaring.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Sri Paran T., Koenigs I., Fitzgerald R J
Our Lady’s Hospital for Sick Children, Crumlin, Dublin
Shunt complications are serious problems when they occur and can be reduced significantly by taking
great care during initial insertion. In 1984, we reported an incidence of 2.4% infection, following the
introduction of an antiseptic technique based on Listerian principles. We wanted to review our latest
infection rate.
This is a 10 year retrospective study, reviewing all medical records of children who had their first VP
shunt insertion between 1993 and 2003. A total of 94 children had 150 shunt insertions. Sixty seven
out of the 94 children had spina bifida, 13 had progressive hydrocephalus due to intraventricular
haemorrhage, 7 had aqueduct Stenosis, 3 had holoproencephaly, 2 following encephalocoele
resection, 1 post meningitis, and 1 with Dandy-Walker syndrome.
There were 3 deaths, two of which were in children with severe spina bifida who had been managed
conservatively. These shunts were inserted for palliative reasons in the presence of significant
myelomeningocoele infections. The third death was due to late arrival of a child with a blocked shunt.
There were 4 children with shunt infections (2.2%) and all were treated successfully. Though normal
CRP levels at 6 weeks follow up was reassuring, raised levels did not always correlate with infections.
In the 4 children who had shunt infections, it was picked up well before the 6 weeks CRP check.
However, no patient with a normal CRP level at 6 weeks was subsequently shown to have shunt
In conclusion, we feel that strict antisepsis is still one of the best ways to prevent shunt infections.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
B. Paturi, E. Capra
Eighteen years ago, the terms HIV/AIDS were not known to the medical fraternity. Today it has a
profound impact on the health of people worldwide. Around 16,000 new HIV infections are being
reported daily, of which 90% are from developing countries. Almost all countries have reported AIDS
cases. As per latest data, India has over 80,000 HIV+ve persons and 6600 cases of AIDS reported.
But these may not be the actual figures due to gross under testing and under reporting.
Eluru has a population of 300,000 from urban to rural areas. To facilitate medical care, villages are
divided into units. This report gives an overview of a 6 month project implementing care and support
for adults and children with AIDS at the Damian Hospital, a voluntary organisation operating in Eluru.
The WHO clinical management HIV/AIDS guidelines have been adapted to the region for standard
clinical management practice. The application of the guidelines is at the level of the district within
existing constraints of the health services. To achieve this a low cost approach to treatment and
clinical tests is emphasised, as the more expensive ones are not often available at the district level.
The main aims of this project are:
• To assist health care personnel in the diagnosis and clinical management of HIV
• To reduce the economic burden of HIV infection by preventing excessive use of diagnostic tests
and inappropriate treatment
• To assess resource requirements
• To provide education and counselling.
Data was collected using a structured proforma based on WHO criteria. Details recorded included:
• Demographic profile of aids cases
• Symptoms reported
• Routes of transmission of newly registered cases
• Care, counselling and education support undertaken.
Each month a medical team would visit all units with a mobile clinic.
Data was collected on a monthly basis over a 6-month period (January – June 2003) using a
computerised database.
The number of new cases registered monthly ranged from 44 to 60. Age range of newly diagnosed
cases ranged between 0 to >50 years, average age 30 – 39 years. In the 6-month period, 18 new
cases of mother to child transmission were registered. Patients were categorised into 2 groups to
facilitate medical care, adults and children (0-19 years inclusive).
The most frequent reported presenting symptoms were weight loss and chest problems. The most
frequent route of transmission for adults was sexually transmitted disease. The transmission of
HIV/AIDS from mother to child remains the main cause of HIV/AIDS in children.
Tests carried out at the hospital include: HIV 1 ELISA test and HIV 2 Tri dot test.
Immune functions: CD4, CD8 cell counts, CD4/CD8 ratio.
Plasma viral leads: Full Blood Count, liver and renal function tests, Mantoux test and chest x-rat.
Co-infections: tests for syphilis, gonorrhoea, hepatitis A, B, C, Toxoplasma, E-B virus and Pap smear
for women. The current AIDS cases reveal the transmission dynamics of past years and burden of
illness in the near future.
As the epidemic of AIDS increases in the Western World, there are lessons that can be learned from
the experiences of our project. The clinical management of HIV infection and AIDS is a part of
comprehensive care for individuals affected by the disease. In order to meet the total needs of
persons affected by the infection the concept of education and comprehensive care across a
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
continuum is advocated. This is the pooling together and creation of linkages of medical, nursing and
social services within the community. With a high case fatality rate, significant impact on health and
society, lack of definite curative treatment, HIV/ AIDS pandemic is one of the most serious health
problems of this century.
Clinical Management of HIV and AIDS at District Level, World Health Organisation, Regional Office
for South-East Asia, New Delhi, 1998.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
P.J. Kearney, M O’Reilly, A Gaffney
Paediatrics and Child Health, Epidemiology and Public Health Medicine, University College Cork;
Leukaemia Unit, Mercy University Hospital.
Aims: Our initial study (Arch Dis Child 2002;86:A4 (suppl 1)), compared the issues raised by parents
involved in either a centralised or a decentralised shared care service in Paediatric Oncology. We
found that the parents associated with a more centralised service identified several burdens of travel,
which were hardly an issue for the decentralised group. This study sought to determine if the travel
issues in one shared care centre could be generalised to other distant Irish shared care centres.
Methods: The staff of five Irish shared care centres agreed to participate, and we were able to
approach parents in four of the five shared care centres. Parents were purposefully selected and
invited to participate. A sociologist or a psychologist moderated five focus group interviews. The
interviews were recorded, transcribed and then analysed into themes and categories. University
College Cork Clinical Research Ethics Committee approved the study.
Results: The quest for survival was the core category in all centres. Parents were aware that this
core value dominated their perceptions so that other values became less important. In particular
sibling neglect and reduction in schooling were tolerated which in other circumstances would have
been unacceptable. The parents identified several burdens of travel. These parents were exhausted
because of the uncertainty of travel arrangements and the huge distances involved. The experience of
travelling with a sick child was frightening and worsened by the worry of accidents and the risk of
infection. Some of the children’s behaviour was very difficult for the parents, as travelling with a sick
child was aggravated by treatment related nausea and vomiting. The children often had to travel
fasting, and steroids aggravated their hunger. Financial pressures posed problems due to
employment issues, the need for a second car, and accommodation in Dublin. The parents’
relationship with the shared care centre was often problematic and they found themselves in role
reversal as paramedic parents. The shared care was with the parents rather than the shared care
centre. The parents’ experiences of shared care endorsed their desire to be in the Paediatric
Oncology Centre, even though the parents were bewildered by both the diagnosis and dislocation
from home,
Conclusions: Our initial findings can be generalised to shared care centres that are beyond
commuting distance from the Paediatric Oncology Centre. Shared care centres in Ireland only
contribute to shared cared in a crisis; the consequent lack of decentralisation is a major factor in family
disruption. There are no National Standards for the structures and staffing of shared care centres.
Different levels of shared care are recognised in other countries, and would be appropriate to the
regional policy of the National Cancer Forum.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Lee Chong A, Clinton F, Breatnach F, O’Marcaigh A, Butler K*, O’Meara A.
National Paediatric Haemopoietic Stem Cell Transplant Unit & Infectious Diseases Dept*,
Our Ladys’ Hospital for Sick Children, Crumlin, Dublin12, Ireland.
Background & Aims: Infection with cytomegalovirus (CMV) is a major cause of morbidity & mortality
following BMT. A retrospective study was undertaken to determine the prevalence and outcome of
CMV infection in a paediatric population with particular reference to surveillance, treatment and cost
Methods: All patients (n=170) undergoing HSCT between January 1994 and December 2003 were
included in the study. Indications for transplant included ALL, ANLL, CML, NBL, AA, WAS & MPS. Of
the 124 allogeneic transplants performed, 57 were unrelated; 46 patients had autologous grafts. Shell
vial cultures of throat swabs and urine, and blood samples for pp65 antigenemia +/- PCR were tested
once to twice weekly post transplant for a mean of 147 days. BAL was performed on four patients with
respiratory symptoms. CMV negative blood products were used in all patients.
Results: 37 patients were seropositive; 15 transplants were donor –ve/recipient +ve, 12 +ve/-ve and
10 were +ve/ +ve. All of these patients received prophylactic high-dose acyclovir and IVIG. 2147
screening tests were performed. There were 119 (5.5%) positive tests in 16 patients during the study
period: 58 in urine, 18 throat swabs, 42 blood (antigenemia/PCR) and one BAL. The most common
first positive test was urine in 6 cases, four of whom developed antigenemia. Throat swabs were of no
predictive value. Ganciclovir was commenced pre-emptively in 7 patients all of whom survived. A
further two patients developed CMV pneumonitis and died. Five patients undergoing autologous
transplant were CMV positive, none of whom developed antigenaemia.
Conclusion: The current policy of pre-emptive therapy appears to be satisfactory in preventing CMV
pneumonitis in patients in the peritransplant period. Elimination of throat swabs from the screening
process would reduce costs significantly. The risk of CMV reactivation in patients undergoing
autologous transplant is negligible.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Epilepsy is a chronic condition of various aetiologies characterized by a predisposition to recurrent,
usually spontaneous epileptic seizures. Intractable epilepsy is a loosely defined concept that
encompasses seizure frequency and severity as well as the psychosocial consequences and effects
of the pharmacotherapy.
The ketogenic diet has been used for more than seventy years in intractable epilepsy with varying
outcomes. The mechanism of action is unknown but it is postulated that the seizure threshold is raised
in the presence of ketosis/acidosis. It is used in a variety of seizure types.
We describe a cohort of twelve patients who were labeled as having intractable epilepsy. Age at
presentation of seizures varied from two days to six years. Nine of this cohort were diagnosed with
idiopathic epilepsy, two with cryptogenic epilepsy; having cortical malformations on MRI brain scans
and one with seizures secondary to herpes encephalitis.
Significant family history included epilepsy and learning difficulties. Developmental milestones were
normal up to the time of onset of seizures in eighty three percent of this group. All had normal
metabolic screens performed. None of these patients fell into the category for epilepsy surgery.
Seizure types were multiple. A combined total of twenty six pharmacological agents were used in the
treatment of this group and all were treated with polytherapy. EEG abnormalities were present in
hundred percent of cases.
From time of onset of seizures all patients demonstrated developmental delay, ranging from speech
and language difficulties to severe global deficits. Developmental regression was noted in one case.
Behavioural deterioration was also a prominent feature amongst this cohort.
All of these patients were commenced on the ketogenic diet and followed up closely by neurological
and dietetic departments. Two patients have only recently commenced this diet regime and as yet are
still achieving optimal ketosis. Five patients demonstrated significant improvement in terms of seizure
control, cognitive function and behaviour. One of these patients relapsed however while on the diet
despite initial success.
Seven patients in total discontinued this diet, the reasons being poor response, severe failure to thrive,
difficulty enforcing the diet, increased seizure frequency and finally re-occurrence of seizures after an
initial period of control.
One patient died while on this diet of sudden unexpected death in epilepsy (SUDEP) but had been
seizure free for over a year prior to his death. Length of duration of treatment with this diet ranged
from two months to almost three years. All patients were treated simultaneously with anti-epileptic
The most common problems demonstrated on this diet were difficulty achieving optimal ketosis and
adequate caloric intake. All patients required vitamin supplementation. Sugar free medications were
used to minimize the carbohydrate intake. High triglycerides were demonstrated in one patient.
Overall five out of ten of these resistant cases derived benefit, at times dramatic, when the diet was
used in conjunction with their anti-epileptic medications. Dietary planning is difficult and tedious with
extensive parent training and child monitoring necessary. However we have demonstrated that with
extreme commitment on the part of the patient, their physicians and their families, the ketogenic diet
still has an important role to play in the treatment of resistant paediatric epilepsy.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
S ,Deiratany, Y Najam 1,D Grant 2, A tynan3, J McMenamin 1.D Webb1.
Dept of Neurology, Our Lady’s Hospital for Sick Children, Dublin 12. Ireland
Fourth Hurdle Consulting ,London,UK
Medical dept, GlaxoSmithKline (Ireland), Dublin Ireland
To investigate in a sample of Irish children with epilepsy, the relationship between seizure type and a
range of morbidity and heath related quality of life ( HRQoL) variables.
Th e sample was drawn from a population attending two paediatic neurology clinics in Dublin,
Ireland.Data were collected on seizure type and frequency, physical and cognitive ability, behaviour,
co-morbidities, demographic and socio-economic variables. The attending parent completed the Child
Health Questionnaire 50 times version (CHQ-50).
132 patients were enrolled. The median age was 8.4 years and 55 % were Male. 51% had partial
seizures only. Children with partial and generalised seizures were equally well enrolled on study entry
and had no differences in CHQ-50 physical (PhS) or psychosocial (PsS) aggregate scores. Children
with generalised seizures had higher levels of abnormal behaviour (p<0.02), cognitive disability
(p<0.06) and co-morbidity (p>0.02). children with generalised seizures were more likely to be
attending a special school (p<0.05) but many received no extra resources from the educational
Parents of children with generalised seizures scored significantly higher for seizure impact on
emotional behaviour (p<0.04), time expended and impact on family activities (p<0.03) on the CHQ-50.
In our study children with generalised seizures had more abnormal behaviour, cognitive disability, comorbidity and a greater Impact on quality of life. We speculate that unlike the adult population children
with generalised seizures suffer a greater impact from their epilepsy because of the childhood
subgroup wit polymorphic seizures also referred to as epileptic encephalopathy.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Power M, Geogheghan R, Cotter M, Smith OP, O Marcaigh A, Mc Mahon C.
AMNCH, Tallaght, Children’s University Hospital Temple Street, Our Lady’s Hospital for Sick Children
Crumlin, Dublin 12
Sickle cell disease (SCD) is a multisystem disorder. Its cardinal features, anaemia and recurrent
vasoocclusive crises relate to the presence of a mutant haemoglobin (HbS) within red cells. This
abnormal haemoglobin forms insoluble sickled cells which occlude blood vessels under conditions of
stress e.g. cold, infection, dehydration and menstruation. In Africa, mortality associated with SCD in
children less than five years of age is at least 20-50% and even in the UK and the USA the current life
expectancy is 42 years for men and 48years for women. Until recently, SCD was relatively unknown in
Ireland, but within the past 3 years, increasing numbers of adults and children have been diagnosed
with this condition. We have reviewed the numbers of children with SCD presenting to the Dublin
Paediatric Haematology centres between 1998-2003 to evaluate the nature of the disease and the
impact on haematology services.
One Hundred and fifty children (88 boys and 62 girls), aged 3 months-14 years, median 4.5years,
have been diagnosed with SCD. Ten children (6 boys and 4 girls) have HbSC and the remainder
HbSS. The country of parental origin was Nigeria in 75% of cases, but 80% of the children were born
in Ireland. Only 15% of children were referred for diagnosis in the neonatal period and less than half of
the Irish born children were diagnosed before the age of six months. Ten percent of children were
diagnosed following an admission with sickle cell crisis.
The commonest cause for admission in children less than 1 year of age was splenic sequestration.
Three children have had recurrent events and one has had a preventative splenectomy. Fifty percent
of children older than1 year have had one or more sickle event, most commonly a bony crisis (75%),
chest crisis (20%), mesenteric vasoocclusion or stroke. Four children are on a chronic transfusion
regimen and four others are on hydroxyurea therapy. Sixty children, aged 3 months -6 years have
never had a sickle crisis. Eighty percent of these have HbF >5% but there is currently no explanation
for the less aggressive course of the remainder.
Sickle cell disease is a relatively new disease in the Irish population but it has serious implications for
health care provision. Early diagnosis and institution of prophylactic penicillin and pneumococcal
vaccination programmes have proven efficacy. Aggressive management of crisis and the definition of
a high risk group for specific therapy are imperative to improve quality of life and reduce mortality.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Hickey L., Scanlan P., O’Shea D., Geoghegan R., Smith O.P., O’Marcaigh A, McMahon C.
Department of Paediatric Haematology, Our Lady’s Hospital for Sick Children, Crumlin D12.
Background: Hereditary haemolytic anaemia represents a heterogeneous group of disorders. The
three most frequently encountered types of hereditary haemolytic anaemia in Ireland are hereditary
spherocytosis (HS), glucose-6-phosphate dehydrogenase deficiency(G6PD) and pyruvate kinase
deficiency(PKD). These disorders vary in severity from asymptomatic to life-threatening anaemia.
Epidemiology, diagnostic criteria, management and outcome vary greatly and have never been
reviewed in Ireland.
Aims: To describe and compare the demographic and clinical features of HS, G6PD and PKD in a
tertiary referral centre in Ireland.
Methods: Patients were indentified using the hospital laboratory computer system. All relevant
investigations since January 1998 were studied. Those diagnosed with HS, G6PD or PKD were
included in the retrospective chart review.
Results: 126 patients were identified in total, 46 with HS (60% male), 77 with G6PD (60% male) and
3 with PKD (all female). 100% of those with HS and PKD but only 8% of those with G6PDD were
Irish. 75% of those with G6PD were from West Africa.
No child with HS or PKD had a second haematological diagnosis, whereas 65% of those with G6PD
had another diagnosis (sickle cell disease or trait, thalassaemia minor or iron deficiency anaemia).
For HS the median age of diagnosis was 2.8yrs (range 1month-9years) and 95% of these are on folic
acid.74% had a positive family history. G6PD was diagnosed at a mean age of 13.1 months (range
1month-6years) and 65% are on no medications. 35% had a positive family history. The median age
of diagnosis for PKD was 15 months (range 1month-3years). All are on folic acid and there is no
relevant family history.
Screening led to the diagnosis in 10% of those with HS and 47% of those with G6PD. The diagnosis of
G6PD was made with a positive screen and assay in all cases. HS was diagnosed with various
techniques including osmotic fragility, blood smear and more recently immunophenotyping of the red
cell membrane (19.5%). PKD was diagnosed using screen and assay techniques in all cases.
There were no symptoms in 60% of G6PD, 25% of HS patients and 0% of the PKD patients. Of those
who presented symptomatically, jaundice and haemolysis were the common presenting features.
17% of G6PD patients required admission (range1-3) for this disorder and 5/77 children required
transfusion. No splenectomies were performed. The average haemoglobin in this group is 10.6g/dl.
All symptomatic HS patients required admission (range 1-5). Approximately 50% of these required a
blood transfusion (range 1-4) and 3 children had splenectomies. The average haemoglobin is 9.8g/dl.
All PKD patients were admitted (range 1-30, two patients on chronic transfusion programme). All
children were transfused (range 2-30 units). 2 splenectomies were performed. The average
haemoglobin is 8.0g/dl.
Conclusion: HS, G6PD and PKD are the most common hereditary haemolytic anaemias in Ireland.
We have shown, the degree of haemolysis may be severe and potentially life threatening and it is
therefore important we are familiar with these disorders.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
C. Corry, SHO, RBHSC, P. Jackson, Consultant, RBHSC and Dr. P. Coyle, Consultant, Regional
Virology Laboratory, Royal Group of Hospitals
An estimated 170 million people, 3% of the world population, are HCV positive. In NI the estimated
prevalence of HCV infection is 0.3%. In the population as a whole intravenous drug use is the most
common cause of infection but children are primarily infected as the result of vertical transmission.
Infection is usually asymptomatic and the clinical illness mild. However, 85% of cases become
chronically infected and are at risk of development of chronic progressive liver damage. The outlook
for these patients can be improved with early treatment such as pegylated interferon.
The 7th draft of the NI Strategy for Hepatitis C, 2002, states that all children born to hepatitis C positive
mothers should be tested at least once and that hepatitis C positive children should be managed by a
paediatric hepatologist or an identified paediatrician who will undertake to manage them in
collaboration with a regional unit in Great Britain. It also calls for national guidelines stating the
frequency and timing of hepatitis C testing in children. In the absence of such guidelines, some
regional centres have devised their own testing protocols, the most comprehensive of which is that
from Birmingham Children’s’ Hospital. This study aims to identify those children in NI who have had a
positive HCV test and to determine whether their management has met the current standards set out
in the Birmingham Children’s Hospital hepatitis C protocol.
The database of the Regional Virology Laboratory at the Royal Group of Hospitals, where all HCV
testing in NI is carried out, was searched. All children who had had a positive HCV serology test from
January 1995 to January 2003 were identified and a full history of all subsequent tests was obtained.
The clinical notes of all patients were requested and information in relation to reasons for initial testing,
family contacts and follow-up was recorded.
17 patients were identified. Of these, it was possible to view the notes of 12. 23.5% of our sample
were confirmed to be HCV positive. 41.2% proved to be negative on further serology or PCR testing.
35.3% have had no further testing. 12 children were tested because of maternal hepatitis C infection,
of these 7 were initially tested in the first week of life and a further 5 between 2 and 6 months of age.
4 have siblings who have not been tested. 2 patients were tested because they had received multiple
blood products prior to the introduction of screening. 15 of the 17 initial tests were requested by
hospital consultants. Of the 4 children with confirmed hepatitis C infection, 3 are under the care of Dr.
McKiernan, paediatric hepatologist at Birmingham Children’s Hospital and 1 has been lost to followup.
Lack of clear guidelines or referral pathway in NI has resulted in children receiving inadequate care. A
large proportion of our children with positive hepatitis serology have had no follow-up testing. Some
children born to hepatitis C infected mothers are not ever being tested and there is lack of consistency
in testing even among members of the same family. There is poor understanding among doctors of
appropriate tests and their timing, particularly in the neonatal period.
We propose employment of a testing protocol similar to that used in Birmingham Children’s Hospital
which would provide guidelines as to diagnostic tests and their timing and ultimately a clear referral
pathway for HCV positive patients to a regional unit. Further, we propose closer links with the adult
hepatology service, ensuring testing of all babies born to HCV-positive mothers. We propose that the
situation be re-audited in 2 years time.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
G Williamson, DG Sweet
Royal-Jubilee Maternity Services, Belfast
Chronic lung disease [CLD] is increasingly important as more extremely low birth weight babies
survive. With increasing use of antenatal steroids and surfactant administration, duration of
mechanical ventilation may be reduced with a corresponding fall in CLD.
To study trends in CLD incidence, we have audited outcomes of babies less than 30wks gestation
admitted to Royal Maternity Hospital, Belfast over 2 separate time periods [1995/6 and 2000/1].
We wanted to compare our CLD incidence with published data from comparable units.
Data was obtained from the NICORE database. Missing data was obtained by pulling hospital charts.
We studied all babies <30 wks admitted in 1995/6 and 2000/1to the regional NICU. We compared
gestational age, birth weight, gender, antenatal corticosteroid treatment and surfactant administration
between the two groups. We defined CLD as the requirement for supplementary O2 beyond 36 weeks
post-conceptual age. We were interested in changes in mortality and CLD incidence.
Time period
No of babies
Median birth weight
Antenatal steroids
Surfactant administration
CLD incidence
Death rates
We have shown a reduction in administration of antenatal steroids and surfactant. CLD rates and
mortality have increased. Our CLD rates are comparable with lower rates described in the literature.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
M. Williamson, B Hayes, D. Corcoran, T.Clarke, T.Matthews
Rotunda Hospital, Dublin
Necrotising enterocolitis affects approximately 11% of premature neonates weighing <1500g.The
average mortality is 20 –40% and survivors are often left with significant morbidity. We postulated that
maternal antenatal antibiotic usage in the antenatal period may have an effect on subsequent
neonatal bowel bacterial colonisation. Likewise many drugs administered antenatally such as opioids
and beta-blockers could affect neonatal bowel perfusion. Maternal antenatal drug administration may
therefore be an independent risk factor for the development of necrotising enterocolitis in the preterm
In a retrospective chart study all neonates less than 1500g admitted to Rotunda NICU who
subsequently developed NEC during the period 2001,2002 and 2003 were chosen as cases. For each
case we chose two controls, the neonates less than 1500g admitted to Rotunda NICU immediately
before and after each of our cases. Analysis of maternal factors included dermographics, past medical
history, antenatal complications, drug administration and placental histology. Neonatal factors included
gestation, birth weight, Apgar scores, medication administration in the first 30 days, feeding regimes
until bolus feeds were established, comorbidity and outcomes.
24 neonates developed NEC during the study period. Significant differences were found in gestational
age ( P = 0.045 ), antepartum haemorrhage ( P= 0.03 ) and neonatal transfusions ( P = 0.023 ). 36.3%
of mothers in the case group received antenatal antibiotics as compared to 37.2% of controls. Results
for all other factors studied were found to be not statistically significant.
In our study APH and low gestational age are significant risk factors for the development of NEC in
infants weighing < 1500g. Antenatal antibiotic administration was ruled out as an independent risk
factor as it showed no statistical or clinical significance.
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
Aim: It is well recognised that early recognition of sensorineural hearing loss and thereby early
rehabilitation results in improved outcomes for speech and language development.
This study looked at the efficacy of a targeted neonatal hearing screening programme that was
introduced in Sligo.
Method: Sligo General Hospital covers the Sligo, Leitrim and West Cavan region and has in the
region of 2200 deliveries per year. All babies born between --/--/---- and --/--/---- who were identified as
high risk for sensorineural hearing loss were screened with oto acoustic emission testing.
This is a simple minimally-invasive test. A probe is introduced into the external auditory meatus and a
click emitted and the response from the eardrum recorded. The device gives a ‘pass’ or ‘refer’ result to
the operator. It is a well tolerated test. The only real requirement of the test is to have a quiet
Criteria for high risk for sensorineural hearing loss were as follows:
Family history of congenital deafness
Elevated aminoglycoside levels.
The test is carried out by the audiology department either before discharge from hospital or
attendance at the ‘well baby clinic’ at four weeks of age. Those who have a refer result go on to be
tested with ABR testing.
Results: Of the --- children tested by OAE --- were referred for further testing and two were identified
to have sensorineural hearing loss and are receiving appropriate intervention.
However a further two children were identified to have severe sensorineural hearing loss but neither
met the criteria for high risk screening. Both of these children were identified at greater than 12
months old.
This data cannot support the use of OAE as a targeted screening tool alone. If it is to be used it has to
be in the context of universal neonatal hearing screening. Following the inclusion in the departmental
service plan funding as has been secured for equipment and personnel to roll out the UNHS
programme for the local region.
Figures to be confirmed
Irish Journal of Medical Science
Abstracts of the Irish Paediatric Association
May 2004
Volume 173 Issue 2 e5
PERIOD (2001- 2003) FROM
Letterkenny General Hospital to Altnagelvin Area Hospital , Derry
B Thursby- Pelham, A Shankar, S McGuire
L McFetridge, D Brown
Letterkenny General Hospital, Co Donegal
Altnagelvin Area Hospital, Derry
Although managed by different health administrative systems, a distance of only 22 miles separates
Letterkenny General Hospital (LGH) from Altnagelvin Area Hospital, Derry (AAH).
The CAWT (Cooperation and Working Together) initiative, established in 1992, welcomed proposals
which would effect improvement in the health and well-being of the population in border regions. One
of the accepted submissions under this initiative involved the purchase of neonatal intensive care
facilities at AAH by LGH.
In 2001, the NWHB agreed to purchase 0.5 of one cot at AAH for approximately 180 days per year at
a cost of £11,500 per month. This allowed AAH to increase its complement of intensive care cots from
2.0 to 2.5. It was envisaged that in-utero transfer of women with high risk pregnancies would take
place in addition to transfer of newborns requiring tertiary level care.
To review the existing arrangement between the two hospitals since its commencement in 2001,
examining the number of in-utero and newborn transfers during a three-year period, the accessibility
of the service, and the outcome in each case.
Data was collected retrospectively from transfer books at each hospital and from a card catalogue
system at AAH. Data retrieved was studied with respect to gestational age, sex, duration of stay,
ventilator days, nasal CPAP days, low-flow oxygen days, evidence of IVH, and other significant
neonatal complications.
During the 3 year period studied, 13 in-utero and 2 neonatal transfers from LGH to AAH took place.
The total number of babies was 18 (one set of triplets, one twin pregnancy)
Because of geographical factors, in-utero transfer and admission to Altnagelvin was more acceptable
to the majority of Donegal mothers than transfer to Dublin.
Neonatal transfer was not possible in the case of 6 infants, due to lack of available intensive care cots
at Altnagelvin at the time of requested transfer.
The average gestational age in the study group was 28.6 weeks (range 22-32 weeks), while the mean
birth weight was 1184 grams (range 645 –2268 grams).The 18 newborns studied accounted for 650
bed days with an average of 36 bed days per baby. One baby was transferred from AAH to Belfast on
day 1 for cardiac surgery ; a 22 week gestation infant died in the resuscitation room; a third family
enjoyed their stay in Derry so much that they moved to Northern Ireland !
Respiratory support for the infants in the study group included 58 conventional ventilator days, 17
HFO days, 61 nCPAP days. 4 babies required low-flow oxygen until discharge. 3 babies had IVH, 5
had significant PDA, 4 had sepsis, and one each had pneumothorax, NEC and coagulopathy.