TY - JOUR
T1 - Safety of living-related liver transplantation for progressive familial intrahepatic cholestasis
AU - Cutillo, Luisa
AU - Najimi, Mustapha
AU - Smets, Françoise
AU - Janssen, Magda
AU - Reding, Raymond
AU - De Ville De Goyet, Jean
AU - Sokal, Etienne M.
PY - 2006/8
Y1 - 2006/8
N2 - 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.
AB - 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.
KW - Autosomal recessive
KW - Biliary atresia
KW - Donor shortage
KW - Living-related liver transplantation
KW - Progressive familial intrahepatic cholestasis
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U2 - 10.1111/j.1399-3046.2006.00524.x
DO - 10.1111/j.1399-3046.2006.00524.x
M3 - Article
C2 - 16856993
AN - SCOPUS:33745064532
VL - 10
SP - 570
EP - 574
JO - Pediatric Transplantation
JF - Pediatric Transplantation
SN - 1397-3142
IS - 5
ER -