一名接受携带Ser23Asn突变供体肝脏的多米诺肝移植受者发生的快速进展性转甲状腺素蛋白介导的淀粉样变性
Rapidly Progressive Transthyretin-Mediated Amyloidosis in a Domino Liver Transplant Recipient of a Ser23Asn Donor.
作者信息
Dixit Neel, Castano Adam, Farr Mary Jane, Traub Rebecca, Lentzsch Suzanne, Brown Robert S, Maurer Mathew S, Brannagan Thomas H
机构信息
*Peripheral Neuropathy Center, Neurological Institute; Departments of †Cardiology; ‡Hematology and Oncology; and §Gastroenterology, Columbia University, College of Physicians and Surgeons, New York, NY.
出版信息
J Clin Neuromuscul Dis. 2016 Mar;17(3):142-5. doi: 10.1097/CND.0000000000000110.
Domino liver transplantation, in which the liver of a patient with transthyretin-mediated amyloidosis is transplanted into another patient, has been an established procedure performed at several centers across the world. The risk of developing systemic amyloidosis in transthyretin-mediated amyloidosis liver transplant recipients is a topic of ongoing investigation. We report a case of rapidly progressive transthyretin amyloidosis in a patient who received a liver from a donor with a rare Ser23Asn mutation. We advise exercising caution when considering domino liver transplantation in patients with this particular mutation.
多米诺肝移植是将转甲状腺素蛋白介导的淀粉样变性患者的肝脏移植给另一名患者，这一手术在世界多个中心都已开展。转甲状腺素蛋白介导的淀粉样变性肝移植受者发生系统性淀粉样变性的风险是一个正在研究的课题。我们报告了一例接受了具有罕见Ser23Asn突变供体肝脏的患者发生快速进展性转甲状腺素蛋白淀粉样变性的病例。我们建议，对于有这种特定突变的患者，在考虑多米诺肝移植时应谨慎行事。
Zotero 插件