https://www.ncbi.nlm.nih.gov/pubmed/29183423
Biliary atresia

S Afr Med J. 2017 Nov 6;107(10):12131.

De Maayer T, Lala SG, Loveland J, Okudo G, Mohanlal R, Hajinicolaou C.

Abstract

BACKGROUND:
Without timely surgical intervention, most children with biliary atresia (BA) are not expected to live beyond 2 years of age. The initial intervention, the Kasai hepatoportoenterostomy (KPE), aims to achieve biliary drainage. Liver transplantation (LT) is performed if jaundice fails to clear or when biliary cirrhosis occurs. In under-resourced South African (SA) academic state hospitals, KPE procedures are the standard of care for the majority of children with BA, but LT is becoming more routinely available.

OBJECTIVES:
To describe the outcomes of children with BA undergoing KPE, and to identify presenting clinical, laboratory and histological features that were associated with a more favourable outcome.

METHODS:
All children with BA who underwent KPE between January 2009 and June 2012 at the Johannesburg academic-hospital complex were included. Clinical and laboratory parameters, including paediatric end-stage liver disease (PELD) score at the time of KPE, liverhistology fibrosis score, clearance of jaundice at 6 months and 24-month survival were determined.

RESULTS:
Of 70 children with BA diagnosed during the study period, 43 (61.4%) underwent KPE, but only 12 (27.9%) achieved early resolution of jaundice. By 24 months, 14 (32.6%) of 43 children undergoing KPE were alive with their native liver, and 2 (4.7%) other children underwent LT. PELD score <15 and early resolution of jaundice, but not age at surgery or histological fibrosis score, predicted a favourable outcome.

CONCLUSION:
Children with BA undergoing KPE in SA state hospitals have a poor prognosis. The PELD score at the time of KPE best predicts 24-month survival.