Stangl M J, Beuers U, Schauer R, Lang T, Gerbes A, Briegel J, Da Silva L, Schildberg F W, Rau H G
Chirurgische Klinik und Poliklinik, Ludwig-Maximilians-Universität, Klinikum Grosshadern, München.
Chirurg. 2000 Jul;71(7):808-19. doi: 10.1007/s001040051140.
Liver transplantation is the method of choice for metabolic diseases and end-stage liver failure.
At the Klinikum Grosshadern we have performed liver transplantation for inborn errors of metabolism in 24 patients (5.3% of all transplantations, 16 adults, age 39 +/- 13 years; 8 children, age 9 +/- 3 years); 19 patients received a transplant for end-stage liver disease, and in 5 cases because of fulminant hepatic failure.
Twenty-four patients received 27 transplants. In 3 cases, a split-liver transplantation was performed; one patient received a combined lung-liver graft. The 5-year survival rate for children is 100% and for adults 68%.
Liver transplantation for inborn errors of metabolism not only replaces the diseased organ, but also leads to complete reversal of the metabolic defect.
肝移植是治疗代谢性疾病和终末期肝衰竭的首选方法。
在格罗斯哈登临床医院，我们对24例代谢先天性缺陷患者进行了肝移植（占所有移植手术的5.3%，其中16例为成年人，年龄39±13岁；8例为儿童，年龄9±3岁）；19例患者因终末期肝病接受移植，5例因暴发性肝衰竭接受移植。
24例患者接受了27次移植手术。3例进行了劈离式肝移植；1例患者接受了心肺联合移植。儿童的5年生存率为100%，成人为68%。
代谢先天性缺陷的肝移植不仅替代了患病器官，还能使代谢缺陷完全逆转。
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