Cutillo L, Najimi M, Smets F, Janssen M, Reding R, de Ville de Goyet J, Sokal EM.
小儿肝移植计划，鲁汶大学，医学系和Saint Luc学院，布鲁塞尔，比利时

进行性家族性肝内胆汁淤积（PFIC）是生命早期一种严重的肝胆疾病，通常需要进行肝脏移植。器官来源的短缺致使患者和家属考虑进行亲属活体肝移植。但是由于潜在的杂合子局部代谢缺陷，是否能利用亲属捐赠的肝源进行移植还有待商榷，因此我们对捐赠者和受体的安全性都进行了评估。

我们比较了在1994年至2001年间因PFIC进行亲属活体肝移植的七对父母--子女；同时还对在同一时期内因胆道闭锁（BA）而进行亲属活体肝移植的另外七对父母---子女进行了比较。研究结果表明，没有一例出现原发性移植肝无功能。移植术后，捐赠者和受体的转氨酶、γ-谷氨酰转肽酶和胆红素水平没有明显的差别。13名移植受体和14名捐赠者存活，且移植术后3至10年的生活质量很好，仅1名PFIC受体在原位肝移植术后9个月因败血症死亡。

因此我们得出以下结论，即PFIC杂合子并不会增加受体和捐赠者术后肝脏失功能的风险；BA受体和捐赠者也出现相似的结果。

Safety of living-related liver transplantation for progressive familial intrahepatic cholestasis
Cutillo L, Najimi M, Smets F, Janssen M, Reding R, de Ville de Goyet J, Sokal EM.
Pediatric Liver Transplant Program, Universite Catholique de Louvain, Faculty of Medicine and Cliniques Saint Luc, Brussels, Belgium.

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.

PMID: 16856993 [PubMed - indexed for MEDLINE]

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