Natural history and the outcome of complications of children with progressive familial intrahepatic cholestasis in a resource-limited setting: A cohort study.

Authors

Nasrin Motazedian, Seyed Mohsen Dehghani, Alireza Shamsaeefar, Kourosh Kazemi, Ali Ghorbanpour, Mehrab Sayadi, Maryam Ataollahi, Seyed Ali Moosavi, Saman Nikeghbalian

Published in

Arab journal of gastroenterology : the official publication of the Pan-Arab Association of Gastroenterology. Jul 08, 2026. Epub Jul 08, 2026.

Abstract

PFIC comprises a heterogeneous group of rare genetic cholestatic liver disorders that lead to progressive liver damage, cirrhosis, and the need for liver transplantation. Understanding its clinical course and survival outcomes is essential for improving management, particularly in resource-limited settings like Iran. This study aimed to evaluate the clinical characteristics, survival outcomes, and predictors of mortality in children with PFIC.
This cohort study was conducted as part of the Shiraz Pediatric Liver Cirrhosis Cohort Study (SPLCCS), initiated in 2018. Data on demographic characteristics, clinical features, and laboratory findings were collected. Cox regression analysis was used to identify risk factors for mortality.
A total of 100 children with PFIC were included, with a mortality rate of 35%. Younger age at diagnosis and higher Pediatric End-Stage Liver Disease (PELD) scores were significantly associated with increased mortality risk. Liver transplantation (LTx) was performed in 50% of cases, with post-transplant survival rates of 74%, 70%, and 66% at 12, 24, and 60 months, respectively. Elevated white blood cell (WBC) counts, aspartate aminotransferase (AST), and direct bilirubin levels were also significant predictors of mortality.
Early diagnosis and timely liver transplantation are critical for improving survival in children with PFIC. Higher PELD scores, WBC counts, AST, and direct bilirubin levels are strongly associated with increased mortality risk, highlighting the need for early intervention and consistent monitoring.

PMID:
42420132
Bibliographic data and abstract were imported from PubMed on 09 Jul 2026.

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