title: Safety of living-related liver transplantation for progressive familial intrahepatic cholestasis.
abstract: 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, gamma-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.
authors.affiliation: Pediatric Liver Transplant Program, Universite Catholique de Louvain, Faculty of Medicine and Cliniques Saint Luc, Brussels, Belgium.
authors.forename: Luisa
authors.initials: L
authors.lastname: Cutillo
authors.forename: Mustapha
authors.initials: M
authors.lastname: Najimi
authors.forename: Françoise
authors.initials: F
authors.lastname: Smets
authors.forename: Magda
authors.initials: M
authors.lastname: Janssen
authors.forename: Raymond
authors.initials: R
authors.lastname: Reding
authors.forename: Jean
authors.initials: J
authors.lastname: de Ville de Goyet
authors.forename: Etienne M
authors.initials: EM
authors.lastname: Sokal
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000235:genetics / Q000601:surgery*; D005260:Female; D006801:Humans; D008111:Liver Function Tests; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome