Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis.

Background: Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. We present our data of LT for each type of PFIC and compare their early, and long-term outcomes, highlighting their individual differences and management strategies.

Methods: Prospectively collected data over a decade (2011-2022) of children with PFIC who underwent LT was analyzed.

Non-standard hepatic venous reconstruction in the setting of absent inferior vena cava in partial graft pediatric liver transplantation-A matched retrospective cohort study.

Recipient cava may be unavailable for outflow reconstruction in some children undergoing liver transplantation (PLT) due to caval agenesis, tumor, or fibrotic caval occlusion. Non-standard hepatic venous reconstruction (NHVR) with a direct veno-caval anastomosis or neo-cava reconstruction is necessary in such cases. Retrospective review of all PLT needing NHVR performed in our unit from January 2010 to September 2019 was performed.