Liver Transplantation for Familial Amyloidotic Polyneuropathy: Impact on Swedish Patients' Survival
Liver Transplantation for Familial Amyloidotic Polyneuropathy: Impact on Swedish Patients' Survival
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DOI：
10.1002/lt.21817
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发表时间：
2009-10-01
影响因子：
4.6
通讯作者：
Suhr, Ole B.
中科院分区：
文献类型：
--
作者：
Okamoto, Sadahisa;Wixner, Jonas;Suhr, Ole B.
肝移植（LTx）治疗家族性淀粉样变性多发性神经病（FAP）是一种公认的治疗这种致命疾病的方法。然而，相对于非移植患者的长期结果尚未完全阐明。本研究的目的是比较瑞典LTx FAP患者与历史对照组的长期生存率，特别是在发病年龄和性别方面。为了评估LTx作为FAP治疗的结果，从疾病开始计算生存率。141例FAP患者（108例移植，33例未移植）纳入研究。与对照组相比，LTx患者的生存率显著提高。与早发性对照组相比，早发性疾病患者（发病年龄< 50岁）的结果尤其有利（P < 0.001）。相比之下，晚发病例（>= 50岁）无显著差异。与移植的晚发性男性相比，移植的晚发性女性的存活率显著提高（P = 0.02）。我们无法发现长期（>= 7年）和短期（>= 7年）患者的生存率有显著差异。
Liver transplantation (LTx) for familial amyloidotic polyneuropathy (FAP) is an accepted treatment for this fatal disease. However, the long-term outcome with respect to that of nontransplanted patients has not been fully elucidated. The aim of this study was to compare the long-term survival of Swedish LTx FAP patients with that of historical controls, especially with respect to the age at onset of the disease and gender. In order to evaluate the outcome of LTx as a treatment for FAP, survival was calculated from the onset of disease. One hundred forty-one FAP patients, 108 transplanted and 33 not transplanted, were included in the study. Significantly increased survival was noted for LTx patients in comparison with controls. The outcome was especially favorable for those with an early onset of the disease (age at onset < 50 years) in comparison with early-onset controls (P < 0.001). In contrast, no significant difference for late-onset cases (>= 50 years) was found. Transplanted late-onset females had significantly improved survival in comparison with transplanted late-onset males (P = 0.02). We were unable to find significant differences in survival between patients with long (>= 7 years) or short (