Paradis K, Weber A, Seidman E G, Larochelle J, Garel L, Lenaerts C, Roy C C
Department of Pediatrics and Radiology, Hôpital Sainte-Justine, Montreal, Quebec, Canada.
Am J Hum Genet. 1990 Aug;47(2):338-42.
Sixteen tyrosinemic patients were evaluated in our institution for a possible liver transplantation. All patients showed biochemical and/or radiological evidence of liver dysfunction. Renal involvement was found to be more abnormal than expected. Seven patients have been transplanted, with two patients receiving a combined liver-kidney transplant. Hepatocarcinoma was detected in two of eight patients in whom the whole liver was examined. Six (37.5%) of the initial 16 patients have died since evaluation, one of the six dying after combined liver-kidney transplantation. Posttransplantation survival was 86%, with normal liver function, normal growth, and no recurrence of neurological crises on a normal diet.
我们机构对16名酪氨酸血症患者进行了肝移植可能性评估。所有患者均有肝功能不全的生化和/或影像学证据。发现肾脏受累比预期更异常。7名患者已接受移植，其中2名患者接受了肝肾联合移植。在接受全肝检查的8名患者中，有2名检测出肝癌。自评估以来，最初的16名患者中有6名（37.5%）死亡，其中1名在肝肾联合移植后死亡。移植后生存率为86%，肝功能正常，生长正常，正常饮食情况下无神经危机复发。
