ArticleAnnals of hepato-biliary-pancreatic surgery2022
Liver transplantation in pediatric patients with progressive familial intrahepatic cholestasis: Single center experience of seven cases.
Article in Annals of hepato-biliary-pancreatic surgery, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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2 citing papers in PubMed, 5 citations in OpenAlex.
- Odevixibat after liver transplant in patients with progressive familial intrahepatic cholestasis type 1: A case series.Journal of pediatric gastroenterology and nutrition · 2025Article
- Long-Term Results of Pediatric Liver Transplantation for Progressive Familial Intrahepatic Cholestasis.Journal of clinical medicine · 2022Article
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7 authors at 1 institution in 1 country.
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Abstract
BACKGROUNDS/
aimsProgressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). The objective of this study was to investigate the clinicopathological features and posttransplant courses of seven LT recipients with PFIC.
methodsThis was a retrospective single-center study of patients with PFIC who underwent LT from January 2013 to June 2020.
resultsTwo and five patients were diagnosed with PFIC type 1 and type 2, respectively. For all seven patients, age of PFIC onset was at birth. Jaundice was present in all cases. Mean pretransplant total and direct bilirubin levels were 16.1 ± 8.1 mg/dL and 12.4 ± 6.2 mg/dL, respectively. Median patient age and body weight at LT were 10 months and 7 kg, respectively. Types of donors were mothers of patients in four and deceased donors in three. All five patients with PFIC type 2 recovered uneventfully. One patient each with PFIC type 1 underwent retransplantation due to graft failure or died due to multi-organ failure. Overall graft and patient survival rates at five years were 66.7% and 83.3%, respectively. Bile salt export pump immunohistochemical staining showed normal canalicular expression in two patients with PFIC type 1, focal loss in two patients with PFIC type 2, and total loss in three patients with PFIC type 2.
conclusionsLT is currently the only effective treatment for PFIC-associated end-stage liver diseases. It is mandatory to perform regular follow-up due to the risk of complications including steatohepatitis, especially for patients with PFIC type 1.
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