P0193 SAFETY OF INTRAFAMILIAL LIVING-RELATED LIVER TRANSPLANTATION FOR PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS

Abstract

Introduction: Progressive familial intrahepatic cholestasis (PFIC) is a severe cholestatic liver disease of early life for which liver transplantation is often required. The use of living related familial donors may be questionable for genetic syndromes, since the donor is heterozygote. The aim of the present study was to investigate the safety of this procedure, for both donors and recipients, in a series of 7 children who received a living related parental donor graft for PFIC between 1994 & 2001. Methods: We compared the evolution in this serie with that of a serie of seven pair matched living related transplantations done for biliary atresia (BA). Median age at transplantation was 24 months (range 4 to 48) for PFIC and 7 months (range 5 to 24) for BA. PFIC donors were the fathers in 5 cases and the mothers in 2, median age 35 years (range 29 to 37). Six donors had left lobectomy and in one lobectomy was extended to part of segment IV. BA donors were the mothers in 4 cases and the fathers in 3, median age 33 years (range 28 to 39), all had left lobectomy. Results: No primary graft dysfunction was observed in both recipient series, and postoperative liver function was not altered in donors from both series. PFIC donors stayed 5 to 8 days in hospital, BA donors 4 to 5days. No significant difference was observed in the evolution of AST, ALT and bilirubin post transplantation in both donor (1st week) and recipient (first 3 weeks) series, with normalization of these tests by day 20. There was a higher postoperative increase of gammaGT in the BA recipient group, (mean 181±50 vs 141 ±73 in PFIC series) but not in donors. One PFIC patient died abroad nine months post OLT from sepsis. All other patients are alive and well 3 to 10 years post OLT. Conclusion: In conclusion, these findings show that living-related liver transplantation is safe in children with PFIC as well as in their parents, without difference in postoperative liver function in PFIC donors heterozygotes and their recipient child, as compared to matched BA donor and recipients. We therefore consider that living related transplantation can be safely proposed to PFIC patients.