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.

Pseudohypoaldosteronism type 1: a rare cause of severe dyselectrolytemia and cardiovascular collapse in neonates.

Severe hyperkalemia is a medical emergency and occurs due to a variety of underlying illnesses. We present a 7 day old neonate who presented with life threatening hyperkalemia due to pseudohypoaldosteronism type 1 (PHA1). The clinical picture resembled congenital adrenal hyperplasia (CAH).