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β2-微球蛋白淀粉样变性:过去、现在和未来。

Beta-2 Microglobulin Amyloidosis: Past, Present, and Future.

机构信息

Division of Nephrology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts.

Harvard Medical School, Boston, Massachusetts.

出版信息

Kidney360. 2020 Oct 21;1(12):1447-1455. doi: 10.34067/KID.0004922020. eCollection 2020 Dec 31.

Abstract

Almost half a century has elapsed since the first description of dialysis-related amyloidosis (DRA), a disorder caused by excessive accumulation of -2 microglobulin (B2M). Within that period, substantial advances in RRT occurred. These improvements have led to a decrease in the incidence of DRA. In many countries, DRA is considered a "disappearing act" or complication. Although the prevalence of patients living with RRT increases, not all will have access to kidney transplantation. Consequently, the number of patients requiring interventions for treatment of DRA is postulated to increase. This postulate has been borne out in Japan, where the number of patients with ESKD requiring surgery for carpal tunnel continues to increase. Clinicians treating patients with ESKD have treatment options to improve B2M clearance; however, there is a need to identify ways to translate improved B2M clearance into improved quality of life for patients undergoing long-term dialysis.

摘要

自首次描述透析相关性淀粉样变(DRA)以来,已经过去了近半个世纪,这种疾病是由β2 微球蛋白(B2M)过度积累引起的。在此期间,RRT 取得了实质性进展。这些改进导致 DRA 的发病率下降。在许多国家,DRA 被认为是一种“消失的病症”或并发症。尽管接受 RRT 的患者人数不断增加,但并非所有人都能进行肾移植。因此,需要进行 DRA 治疗干预的患者数量预计会增加。这一假设在日本得到了证实,日本需要手术治疗腕管综合征的终末期肾病患者数量持续增加。治疗终末期肾病患者的临床医生有改善 B2M 清除率的治疗选择;然而,需要找到将改善的 B2M 清除率转化为长期透析患者生活质量改善的方法。

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