Transthyretin amyloid-related cerebral angiitis after liver transplantation
Transthyretin amyloid-related cerebral angiitis after liver transplantation
复制标题
肝移植后运甲状腺素蛋白淀粉样蛋白相关脑血管炎
DOI：
10.1080/13506129.2019.1583179
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发表时间：
2019
期刊：
影响因子：
5.5
通讯作者：
Ando Yukio
中科院分区：
文献类型：
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作者：
Yamashita Taro;Ohnishi Koji;Ueda Mitsuharu;Masuda Teruaki;Inoue Yasuteru;Misumi Yohei;Ueda Akihiko;Obayashi Konen;Takeya Motohiro;Ando Yukio
背景甲状腺素运载蛋白（TTR）Y114 C患者发生致命性小叶出血和快速进展性痴呆，表现为脑淀粉样血管病（CAA）[引文1，引文2]。由于TTR是由肝脏产生的，肝移植可预防遗传性ATTR V30M淀粉样变性中的系统性淀粉样变性和系统性淀粉样血管病。我们最近的研究表明，肝移植通过降低血液中突变TTR的水平，对ATTR Y114 C患者的致死性小叶出血和快速进展性痴呆部分有效[引文1]。然而，轻度认知障碍即使在肝移植后也会缓慢持续。本研究旨在描述一例CAA ATTR Y114C肝移植术后尸检病例的临床过程和病理变化。
BackgroundPatients with transthyretin (TTR) Y114C develop fatal lobular hemorrhage and rapidly progressive dementia that presents with cerebral amyloid angiopathy (CAA)[Citation 1, Citation 2]. Because TTR is produced by the liver, liver transplantation prevents systemic amyloidosis and systemic amyloid angiopathy in hereditary ATTR V30M amyloidosis. Our recent studies revealed that liver transplantation was partially effective for fatal lobular hemorrhage and rapidly progressive dementia in patients with ATTR Y114C by reducing the level of mutant TTR in the blood [Citation 1]. However, mild cognitive impairment continues slowly even after liver transplantation. This study aimed to describe the clinical course and pathological changes in an autopsy case of CAA ATTR Y114C after liver transplantation.