Long-term results of liver transplantation in familial amyloidotic polyneuropathy type I
Long-term results of liver transplantation in familial amyloidotic polyneuropathy type I
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DOI：
10.1097/00007890-199708270-00017
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发表时间：
1997-08-27
期刊：
影响因子：
6.2
通讯作者：
MunarQues, M
中科院分区：
文献类型：
--
作者：
Parrilla, P;Ramirez, P;MunarQues, M
背景本文的目的是报告我们对18例家族性淀粉样变性多发性神经病I型肝移植患者的生存和四肢多发性神经病和自主神经功能障碍的演变的经验，其中13例患者平均随访超过2.5年。18例患者12个月和58个月的生存率分别为72.2%和60.1%。在所有患者中，我们注意到在移植后的前6个月内四肢多发性神经病和自主神经功能障碍的临床改善。由于自主神经系统受累的临床数据显示比神经运动受累的患者更早改善。总之，我们的研究结果表明，肝移植可能是有用的，在某些情况下，家族性淀粉样变性多发性神经病的治疗，以阻止患者的神经功能恶化，以避免致命的疾病。
Background. The aim of this article is to report our experience regarding the survival and the evolution of polyneuropathy of the extremities and autonomic dysfunction in 18 liver transplant patients with familial amyloidotic polyneuropathy type I after a mean follow-up of more than 2.5 years for 13 patients.Methods. The actuarial survival rate of the 18 patients is 72.2% and 60.1%, respectively, at 12 and 58 months.Results. In all the patients we noted clinical improvement of the polyneuropathy of the extremities and autonomic dysfunction during the first 6 months after transplant. The clinical data due to autonomic nervous system involvement showed an earlier improvement than those due to nervous motor involvement.Conclusions. In conclusion, our results suggest that liver transplant may be useful in the treatment of certain cases of familial amyloidotic polyneuropathy to stop the neurological deterioration of the patients and to avoid the fatal end of the disease.