Long-Term Outcomes of Living-Donor Liver Transplantation for Progressive Familial Intrahepatic Cholestasis Type 1

Study of 12 PFIC-1 patients undergoing living donor liver transplantation over 30 years reveals 58% long-term survival, with outcomes correlating to ATP8B1 mutation type and post-transplant intestinal function. Patients with unsuccessful transplants showed growth retardation and severe diarrhea, suggesting extrahepatic manifestations predict transplant success.