Background & Aims: Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare liver disease caused by biallelic variations in . Data reporting on the impact of genotype and of response to ursodeoxycholic acid (UDCA) therapy on long-term outcomes are scarce.
Methods: We retrospectively describe a cohort of 38 patients with PFIC3 with a median age at last follow-up of 19.
J Pediatr Gastroenterol Nutr
October 2019
Objectives: This study analyses the prognosis of biliary atresia (BA) in France since 1986, when both Kasai operation (KOp) and liver transplantation (LT) became widely available.
Methods: The charts of all patients diagnosed with BA born between 1986 and 2015 and living in France were reviewed.
Results: A total of 1428 patients were included; 1340 (94%) underwent KOp.