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Safety of living‐related liver transplantation for progressive familial intrahepatic cholestasis
Author(s) -
Cutillo Luisa,
Najimi Mustapha,
Smets Françoise,
Janssen Magda,
Reding Raymond,
De Goyet Jean de Ville,
Sokal Etienne M.
Publication year - 2006
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2006.00524.x
Subject(s) - progressive familial intrahepatic cholestasis , medicine , liver transplantation , biliary atresia , cholestasis , transplantation , gastroenterology , liver disease , sepsis , primary sclerosing cholangitis , surgery , disease
Progressive familial intrahepatic cholestasis (PFIC) is a severe cholestatic liver disease of early life often requiring liver transplantation. Organ shortage leads to consider living‐related liver transplantation. Because of possible partial metabolic defect in heterozygotes, the use of familial donors might be questionable. We therefore evaluated the safety of this procedure, for both donors and recipients. We compared a series of seven parental–children pairs, having participated in the living related liver transplant program for PFIC between 1994 and 2001, with that of a series of seven parental–children pairs, performed for biliary atresia (BA) during the same period. No primary graft dysfunction was observed. There was no difference in the course of transaminases, γ ‐glutamyl transpeptidase and bilirubin levels after transplantation in both donor and recipient series. Thirteen recipients and 14 donors are alive and well 3–10 yr post‐surgery. One PFIC recipient died nine months post‐orthotopic liver transplantation from sepsis. We conclude that PFIC heterozygote status of the donor does not increase the risk of liver dysfunction in either recipients or donors, with a similar course compared with BA recipients and donors.