Document Detail

Liver transplantation in children with progressive familial intrahepatic cholestasis.
MedLine Citation:
PMID:  18049123     Owner:  NLM     Status:  MEDLINE    
Progressive familial intrahepatic cholestasis (PFIC) is caused by mutations of the bile salt export pump or the multidrug resistance P-glycoprotein, resulting in chronic hepatic failure. Partial external diversion of bile or ileal bypass is effective in some cases and, in others, liver transplantation (OLT) is necessary. Forty-two children were included in this study. Twenty-six children suffered from PFIC type 2 and 16 from PFIC type 3. Symptoms included pruritus, cholestasis, liver cirrhosis, and growth retardation. Seventeen patients received external biliary diversion. Ten had to undergo OLT in the following course. As of this report, three of the remaining patients were on the wait list for OLT. Twenty-three children received a liver graft primarily with excellent outcome. Our data show that OLT is the option of choice in symptomatic PFIC and whenever liver cirrhosis is present. We suggest a very restrictive recommendation of external biliary diversion. However, gene therapy may be a future option for children with PFIC.
Cornelia Englert; Enke Grabhorn; Andrea Richter; Xavier Rogiers; Martin Burdelski; Rainer Ganschow
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Transplantation     Volume:  84     ISSN:  0041-1337     ISO Abbreviation:  Transplantation     Publication Date:  2007 Nov 
Date Detail:
Created Date:  2007-11-30     Completed Date:  2007-12-21     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0132144     Medline TA:  Transplantation     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1361-3     Citation Subset:  IM    
Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Germany.
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MeSH Terms
Cholestasis, Intrahepatic / genetics*,  surgery*
Disease Progression
Graft Survival / physiology*
Liver Transplantation / physiology*
Postoperative Complications
Retrospective Studies
Treatment Outcome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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