Mnatsakanova Diana, Živković Saša A
Diana Mnatsakanova, Saša A Živković, Department of Neurology, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, United States.
World J Hepatol. 2017 Jan 28;9(3):126-130. doi: 10.4254/wjh.v9.i3.126.
Liver transplantation has been used in treatment of transthyretin amyloidosis, and some patients undergo domino liver transplantation (DLT) with explanted liver being transplanted to another patient with liver failure as the liver is otherwise usually functionally normal. Until end of 2015, there were 1154 DLT performed worldwide. DLT for transthyretin amyloidosis is associated with the risk of developing systemic amyloidosis and amyloid neuropathy, and the risk may be greater with some non-Val30Met mutations. amyloid neuropathy has been described in up to 23% of transplant recipients. Neuropathy may be preceded by asymptomatic amyloid deposition in various tissues and symptoms of neuropathy started after a median of 7 years following DLT (5.7 ± 3.2 years; range 2 mo to 10 years). Typical initial symptoms include neuropathic pain and sensory loss, while dysautonomia usually starts later. Progression of neuropathy may necessitate liver re-transplantation, and subsequent improvement of neuropathy has been reported in some patients. Explant allograft recipients need close monitoring for signs of systemic amyloidosis, neuropathy and dysautonomia as progressive symptoms may require re-transplantation.
肝移植已被用于治疗转甲状腺素蛋白淀粉样变性，一些患者接受多米诺肝移植（DLT），切除的肝脏被移植给另一位肝功能衰竭患者，因为该肝脏在其他方面通常功能正常。截至2015年底，全球共进行了1154例DLT。用于转甲状腺素蛋白淀粉样变性的DLT与发生系统性淀粉样变性和淀粉样神经病变的风险相关，某些非Val30Met突变患者的风险可能更高。高达23%的移植受者出现过淀粉样神经病变。神经病变之前可能在各种组织中出现无症状淀粉样沉积，神经病变症状在DLT后中位时间7年（5.7±3.2年；范围2个月至10年）开始出现。典型的初始症状包括神经性疼痛和感觉丧失，而自主神经功能障碍通常较晚出现。神经病变进展可能需要再次肝移植，一些患者随后的神经病变有所改善。切除的同种异体肝移植受者需要密切监测系统性淀粉样变性、神经病变和自主神经功能障碍的体征，因为症状进展可能需要再次移植。
