Biliary Atresia in Israel 2008 to 2018 : A Multicenter Long Term Observational Study
2Pediatric gastroenterology, Hadassah University Medical Center, ישראל
3Pediatric gastroenterology, Dana-Dwek Children's Hospital, ישראל
4Pediatric gastroenterology, Shaare Zedek Medical Center, ישראל
Introduction: Biliary atresia (BA) is a rare neonatal cholangiopathy without effective therapy. BA remains the leading indication for pediatric liver transplant (LT) worldwide. Data on disease course in Israel is scarce thus we aimed to explore the incidence, prognosis and long-term outcome of BA in Israel in the past decade.
Methods: This retrospective multi-center study reviewed the medical records of children with BA from 4 main centers in Israel including demographic profiles, sentinel events and outcomes such as survival with native liver (SNL), LT and death between 2008-2018.
Results: There were 72 patients diagnosed with BA from January 2008 to December 2018. The estimated incidence of BA was 1:15,000 live births. The mean age of performing KPE was 59.83 days (28-95 days, SD=16 days). KPE was successful (bilirubin Primary antibiotic prophylaxis was given in 48.6% of patients. 68.1% of patients had cholangitis, with median time to 1st cholangitis of 93 days.
Primary prophylactic use of antibiotics resulted in earlier occurrence of 1st cholangitis event compared with no prophylaxis (median 77 vs 239 days, p=0.016). 38.9% of patients underwent LT, three patients died prior to LT.
SNL was 54.4% at 5 years.
Conclusions: This is the largest cohort of BA patients described in Israel, which shows similar outcomes to other developed countries. In our cohort there was lack of benefit for primary antibiotic prophylaxis for cholangitis.