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Long-term outcomes after cholecystocolostomy for progressive familial intrahepatic cholestasis

Long Chen1,2, Hui Xiao1,2, Xiang-Hai Ren1,2

  • 1Department of Pediatric Surgery, Capital Institute of Pediatrics, Beijing, China.

Hepatology Research : the Official Journal of the Japan Society of Hepatology
|June 24, 2018
PubMed

Insights

Cholecystocolostomy surgery significantly improves cholestasis and pruritus in children with progressive familial intrahepatic cholestasis (PFIC). This well-tolerated procedure offers long-term benefits for PFIC patients.

Area of Science:

  • Hepatology
  • Pediatric Surgery
  • Gastroenterology

Background:

  • Progressive familial intrahepatic cholestasis (PFIC) encompasses several genetic disorders affecting bile flow.
  • Current treatment options for PFIC are limited, often necessitating liver transplantation.
  • Cholecystocolostomy offers a potential surgical alternative for managing PFIC.

Purpose of the Study:

  • To assess the long-term effectiveness of cholecystocolostomy in pediatric patients diagnosed with PFIC.
  • To evaluate the impact of cholecystocolostomy on key clinical markers and patient outcomes.

Main Methods:

  • Retrospective analysis of 34 children with PFIC treated between 2003 and 2014.
  • Included patients with familial intrahepatic cholestasis-1 (FIC1), bile salt export pump (BSEP) disease, low γ-glutamyl transpeptidase (GGT) disease, and multidrug resistance class III (MDR3) disease.
  • Data collected on surgical operations and outcomes, including serum bilirubin, bile acid levels, pruritus, growth, and defecation.

Main Results:

  • Cholecystocolostomy led to significant reductions in serum total bilirubin and bile acid levels in PFIC patients (P < 0.001).
  • Marked improvement in pruritus (88.2% vs. 16.1%, P < 0.001) and reduced growth retardation were observed.
  • Increased defecation frequency (P = 0.002) and generally well-tolerated outcomes were noted, though some patients required reoperation or liver transplantation.

Conclusions:

  • Cholecystocolostomy is a well-tolerated, single surgical approach for PFIC.
  • The procedure demonstrates long-term efficacy in improving cholestasis and pruritus.
  • This study provides valuable insights into cholecystocolostomy as a treatment for various types of PFIC.
Abstract

Keywords:
cholecystocolostomydefecation frequencyliver transplantationprogressive familial intrahepatic cholestasis

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