背驮式肝移植治疗家族性淀粉样多神经变(附1例成功的病例报道)

目的 :为探讨外科治疗家族性淀粉样多神经变 (Familialamyloidpolyneuropathy ,FAP)的疗效。方法 :对一FAP患者实施了背驮式肝移植。结果 :手术顺利 ,术后 1周感觉运动神经及植物神经紊乱综合症开始恢复 ,失明的双眼开始感光。术后 35d ,多神经变综合症不同程度改善后出院。初步随诊 3月 ,病情正处康复阶段。结论 :FAP这一内科治疗无效的常染色体显性遗传性疾病采用背驮式肝移植是唯一有效的外科措施

THERAPY OF FAMILIAL AMYLOID POLYNEUROPATHY WITH PIGGYBACK LIVER TRANSPLANTATION

Objective:To discuss the surgical therapy of familial amyloid polyneuropathy (FAP).Methods: One case of FAP was treated with piggyback liver transplantation.Results:The operation was smooth without events, and the patient's sensational, motive and vegetative nervous syndromes began to improve one week after operation, and both his blind eyes began to have light sense. The patient was discharged 35 days after operation when his polyneuropathy syndromes improved with different degrees. He has been followed up for 3 months and he is still in convalescence.Conclusions:FAP is a hereditary disorder with common chromosomal dominance which has no medicine therapy, and it can only be treated with piggyback liver transplantation. The mechanism, diagnosis of FAP, and timing and the surgical curative effect of the operation were discussed due to their inadequate common knowledge.