Clinical and Nutritional Outcomes in Biliary Atresia Patients in an Irish Paediatric Centre.
Fiona Barron, DIT/TCD, Tara Raftery, OLCHC, Sheila Sugrue, DIT, Michelle Hurley, OLCHC, Seamus Hussey, OLCHC.
Dublin Institute of Technology, Trinity College Dublin and Our Lady's Children's Hospital, Crumlin.
Biliary Atresia (BA) is a life-threatening, progressive cholestatic disease that leads to the obliteration of the bile ducts. This disease affects infants within the first weeks of life. Surgical management is the only option available to manage this disease. The Kasai portoenterostomy (KPE) is an important primary therapy that aims to restore bile flow. However, 80-90% of patients will eventually require liver transplantation (LT) throughout their lifetime.
The care of BA patients in Ireland is shared between The National Centre for Paediatric Gastroenterology, Hepatology and Nutrition (NCPGHN) at Our Lady’s Children Hospital, Crumlin (OLCHC) and King’s College Hospital, London (KCH). The aim of this study was to assess the clinical and nutritional outcomes of BA patients in Ireland.
This study is a retrospective case review of BA patients treated in The NCPGHN between the years 1992-2014. Clinical, biochemical and anthropometric data was collected from medical, dietetic and electronic records as appropriate. Anthropometric data was described in weight-for-age and length-for-age z scores. Fiftyfour patients were included in the final analysis of this study.
Overall survival of BA patients is 91% in this study (4 patients died post KPE and 1 post LT). Five and 10-year survival with native rates were calculated as 42% and 33%, respectively using Kaplan Meier survival statistics. 18 out of 50 (36%) patients cleared jaundice by 6 months in the present study. Failure to clear jaundice was significantly associated with reduced survival with native liver post KPE (P=0.00) and increased progression to LT prior to the age of 1 year (P=0.00). Patients who failed to clear jaundice have poorer median (IQR) weight [-2.04 (0.19) vs -0.19 (0.41), P=0.00] and height [-1.04 (0.37) vs 0.00 (0.09), P=0.12] z scores from as early as 6 months post KPE.
This is the first study to analyse the clinical and nutritional outcomes in a cohort of Irish BA patients. The clinical outcomes in this Irish centre are comparable to international centres. It is evident that poorer outcomes are reported in BA patients who fail to clear jaundice post KPE. These patients a have poorer SNL rates, increased progression to LT at a younger age and poorer nutritional status. These patients should be closely monitored for symptoms of progressive liver disease and nutritional status should be optimised in all BA patients.