1PAEDIATRICS DEPARTMENT, NATIONAL CHILDRENS HOSPITAL, DUBLIN, IRELAND
2DIETETICS DEPARTMENT, NATIONAL CHILDRENS HOSPITAL, DUBLIN, IRELAND
3PAEDIATRICS DEPARTMENT, NATIONAL CHILDRENS HOSPITAL, DUBLIN, IRELAND AIM: we report a case of protein energy malnutrition (PEM). Baby S was admitted via the accident and emergency of the National Childrens Hospital, Tallaght at 4 months of age. She presented with a 4 week history of non-bloody diarrhoea, decreased oral intake and poor weight gain.
METHOD: a case report study of Baby S, a 4 month old baby girl who recently arrived in Ireland having being adopted by Irish parents from Ethiopia. Prior to arrival in Ireland she had undergone health screens to exclude retrovirus and hepatitis B and C.
RESULTS: Baby S presented with facies and physical examination consistent with marasmus, a form of PEM. Investigations demonstrated metabolic acidosis, electrolyte imbalance and hypoalbuminemia;
stool culture positive for salmonella. Management included correction of metabolic imbalance and significant dietetic involvement with re-feeding, use of semi elemental formula and micronutrient supplementation.
CONCLUSION: This patient presented with marasmus, a rare primary presentation in the Irish population. We believe this case is important in highlighting the changing disease profile in our
population. This presents a challenge to us as professionals in terms of specific disease recognition and management, and is an important consideration in planning medical and allied professional resources.
CAFFEY'S DISEASE IN INFANTS; MIMIC OF INFECTION, TRAUMA AND NON ACCIDENTAL INJURY C Purcell 1, M Dominguez 2, K Butler 1, 2, E Twomey 3, P Gavin 1, 2
1Infectious Diseases, Childrens University Hospital, Temple Street, Dublin, Ireland
2Infectious Diseases, Our Ladies Hospital for sick children, Crumlin, Dublin, Ireland
3Department of Radiology, Childrens University Hospital, Temple St, Dublin, Ireland Introduction:
Caffey's Disease ('infantile cortical hyperostosis') is an uncommon, benign proliferating disease of infancy, characterised by irritability, bone pain and radiographic bony changes. Diagnosis may be delayed as differential diagnosis includes conenital syphilis, hypervitaminosis A, scurvy, bone tumours, trauma and non accidental injury.
Methods:
Rotrospective chart review of 2 cases of Caffey's disease Results:
Case1: an 11-week old twin boy presented with a 3 week history of worsening lower limb pain and swelling over his shins. Examination revealed bilateral, tender bony prominences of the anterior tibiae.
Radiographs showed symmetrical dense subperiosteal new bone foramtion. Maternal history was notable for syphilis, diagnosed and treated in a previous pregnancy. Maternal syphilis serology (RPR negative, TPPA positive 1/80) was consistent with successful treatment. Infant syphilis serology suggested passive transfer of maternal antibody. A diagnosis of Caffeys disease was made and the infant was managed symptomatically.
Case 2: a 3-week old girl with in utero HIV and Hepatitis C exposure, presented with lower limb pain after a suspected injury while being played with by her siblings. Radiographs showed periosteal reaction and a possible fracture. Despite a history of similar transient phenomenon in older family members, non accidental injury was considered. Medical social work review was reassuring.
Ophthalmology review was normal. Radiographs showed multi focal involvement of the mandible, right humerus, tibia, ulna and scapulae. A diagnosis of Caffeys disease was made
Conclusion: Increased awareness of Caffey's disease and recognition of characteristic radiologic changes, may avoid diagnostic confusion and unecessary investigation of this otherwise self-limiting condition. Confounders in the history, as in these cases, can mislead and delay diagnosis.
THE OUTCOME TO DISCHARGE OF A COHORT OF INFANTS BORN WITH AN ISOLATED GASTROSCHISIS J R Purna1, C Hensey2, R Mahony3, P MacParland3, A Twomey1
1Neonatology, National Maternity Hospitl, Dublin, 2Paediatrics, Sligo General Hospital, Sligo,
3Obstetrics and Gynaecology, National Maternity Hospital, Dublin, 4Obstetrics and Gynaecology, National Maternity Hospitl, Dublin, 5Neonatology, National Maternity Hospital, Dublin
Objective:
To review the outcome to discharge of infants with an antenatal diagnosis of isolated gastroschisis.
Study Design:
Retrospective review of all cases born with an isolated gastroschisis in the National Maternity Hospital over an eight year period.
Results:
Of 31cases identified antenatally, all cases were liveborn. The mean gestational age was 37+/-1.3 weeks and mean birthweight was 2688g+/-453g.16(52%) were male, 22(71%) infants achieved vaginal delivery and only 11(35%) babies delivered during normal working hours. Detailed follow up was available on 27(87%)infants. The mean time from birth to transfer to a paediatric surgical centre was 3+/-1.0hours and the mean time to initial surgery (either primary repair or placement of a silo) was 4+/-1.6hours. 23(85%) infants were diagnosed with simple gastroschisis (ie bowel intact, continuous and uncompromised) and the remainder were complex (ie bowel perforation, necrosis or atresia).
5(18%) were large defects with the presence of other organs apart from an intact bowel outside the abdomen. Of the 23 infants with simple gastroschisis, 13(57%) had primary closure. While infants with simple gastroschisis who underwent primary closure commenced oral feeds earlier, established full enteral feeds earlier and had shorter hospital stays compared to those who had a silo placed, there differences were not significant. Infants with complex gastroschisis took twice as long to commence feeds (24days vs. 12 days, p=0.001), twice as long to establish full enteral feeds (43days vs. 22days, p=0.01) and had longer stays in hospital (61days vs. 29days, p=0.001) compared to those with simple gastroschisis. 6(22%) developed intestinal failure (need for >28 days of parenteral nutrition) and 3(11%) required an unplanned re-operation.25(93%) survived to discharge. Two died, one of NEC and one from perfortaion.
Conclusion:
Survival rate to discharge is high. Infants with simple gastroschisis do better than those with complex gastroschisis. In our cohort, undergoing a primary repair did not appear to confer an advantage.
OSTEOGENISIS IMPERFECTA IN A DEFINED BIRTH COHORT: REGIONAL EXPERIENCE AND THE NEED