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透析相关性淀粉样变:历史与临床表现

Dialysis-related amyloidosis: history and clinical manifestations.

作者信息

Danesh F, Ho L T

机构信息

Division of Nephrology/Hypertension, Northwestern University Medical School, Chicago, Illinois 60611-3008, USA.

出版信息

Semin Dial. 2001 Mar-Apr;14(2):80-5. doi: 10.1046/j.1525-139x.2001.00035.x.

Abstract

Dialysis-related amyloidosis (DRA) or beta(2)-microglobulin amyloidosis (A beta(2)M) is a unique type of amyloidosis that has been described in individuals with both long-standing chronic renal disease and end-stage renal disease (ESRD). It has been associated with serious complications that significantly add to the morbidity of long-term dialysis patients. The deposition of beta(2)M in amyloid fibrils in various joint and osteoarticular surfaces leads to the clinical complaints and findings typical of this disorder. However, a visceral form with systemic organ involvement has also been described. Despite advances in the understanding of this disorder and in the delivery of dialysis, the ability to alter the incidence of DRA and its course remains uncertain.

摘要

透析相关淀粉样变性(DRA)或β2微球蛋白淀粉样变性(Aβ2M)是一种独特的淀粉样变性类型,已在长期患有慢性肾病和终末期肾病(ESRD)的个体中被描述。它与严重并发症相关,这些并发症显著增加了长期透析患者的发病率。β2M在各种关节和骨关节表面的淀粉样纤维中的沉积导致了这种疾病典型的临床症状和体征。然而,也有全身性器官受累的内脏型病例被描述。尽管在对这种疾病的认识以及透析治疗方面取得了进展,但改变DRA发病率及其病程的能力仍不确定。

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