Pediatric liver transplantation remains the gold standard for life-threatening acute and
chronic liver diseases and multiple liver-based inherited metabolic defects. Advances in …

Background: Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous rare
congenital cholestatic liver disease. Disease progression might necessitate liver …

Background: Progressive Familial Intrahepatic cholestasis type I (PFIC1) is a rare congenital
hepatopathy causing cholestasis with progressive liver disease. Surgical interruption of the …

Genetic cholestatic liver diseases are caused by (often rare) mutations in a multitude of
different genes. While these diseases differ in pathobiology, clinical presentation and …

Patients post liver transplant (LT) with progressive familial intrahepatic cholestasis type 1
(PFIC-1) often develop progressive graft steatohepatitis, intractable diarrhea, and growth …

Background Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of
inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT) …

Background Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an autosomal
recessive cholestatic liver disorder caused by ATP8B1 gene mutations. Although liver …

Age at Initial Data Collection 6 y 9 y 13 y 17 y 10 y, 7 mo 16 y aAvailable data based on
genetics initiated at another institution; bPatient received a second LT approximately 24 …

Progressive familial intrahepatic cholestasis is a heterogeneous group of autosomal
recessive disorders, and liver transplant is the only curative treatment. A biliary diversion …