肝移植治疗家族性淀粉样变多神经病的临床病例分析

Multivariate analysis of Liver transplantation for familial Amyloidotic Polyneuropathy

目的:探讨肝移植手术治疗家族性淀粉样变多神经病的疗效及存活率。方法:回顾性分析自1990～2007年间实施肝移植治疗的86例家族性淀粉样变多神经病的临床资料,总结手术方式及预后情况。结果:在1996～2007年时间段里,第1、3、5年手术后存活率分别为94.6%、92.3%、92.3%;而在1990～1995年时间段里,相应的手术后存活率分别为76.7%、66.7%和66.7%,差异有统计学意义(P=0.0003)。多因素分析显示,手术时年龄(≥40岁)、病程(≥7年)和修正体重指数(<600)是病人存活的独立愈后因素。结论:为使手术病人的预后达到最优化,应在病程的初期及早行肝移植治疗,针对心脏病变的术后病人定期行心电图及心脏彩超检查以了解心功能。

Objective： The aim of this study was to assess mortality and symptomatic response to liver transplantation（Ltx） for familial amyloidotic polyneuropathy（FAP）. Methods： All 86 FAP patients transplanted at our hospital between April 1990 and November 2007 were included in the study. Results： The 1, 3 and 5 year patient survival rates in patients transplanted during 1996~2007 were 94. 6%, 92. 3% and 92.3% respectively, a significant difference from the rates of 76.7%, 66.7% and 66.7% respectively during 1990~1995 （P =0. 0008）. Multivariate analysis revealed that age at the time of LTx （≥40 years） ,duration of the disease （≥7 years）, and modified body mass Index （mBMI） （〈600） were independent prog- nostic factors for patient survival. Conclusions： To optimize the post-transplant prognosis, Ltx should be performed in the early stages of the disease, and close post-Ltx monitoring of heart function by echocardiography and of heart arrhythmia by ECG is mandatory.