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透析相关淀粉样变的研究进展。

Recent progress in understanding dialysis-related amyloidosis.

机构信息

Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Science, Niigata, 951-8510, Japan.

出版信息

Bone. 2009 Jul;45 Suppl 1:S39-42. doi: 10.1016/j.bone.2009.03.655. Epub 2009 Mar 19.

Abstract

Dialysis-Related Amyloidosis (DRA) is a general amyloidosis, which is specifically found in CKD stage 5 patients. DRA causes various osteoarticular lesions in dialysis patients, and therefore it is not practical to regard this condition separately from chronic kidney disease-mineral and bone disorder (CKD-MBD), at least from the viewpoint of daily clinical practice. However, it is still controversial whether this disease condition should be included in CKD-MBD. Recently, a better understanding of the pathogenesis of DRA has been obtained by examination of beta(2)-microglobulin-related amyloid fibril formation, extension, and depolymerization in vitro. Apoliprotein E, proteoglycans, and glycosaminoglycans stabilize the amyloid fibrils. In addition, some lysophospholipids and non-esterified fatty acids accelerate amyloid fibril formation and extension under physiological conditions in vitro. Those molecules may enhance the amyloid deposition in vivo. The frequency and severity of osteoarticular disorders that may be associated with DRA accelerate with the duration of dialysis therapy. We have shown that patients undergoing dialysis therapy for 30 years or more survive with serious complications from osteoarticular disorders. DRA is one of the most harmful osteoarticular complications with regard to the maintenance of daily activities and quality of life in patients undergoing long-term dialysis therapy, in addition to the classical complications of CKD-MBD.

摘要

透析相关性淀粉样变(DRA)是一种全身性淀粉样变,主要发生在 CKD 5 期患者中。DRA 可导致透析患者出现各种骨与关节病变,因此,从日常临床实践的角度来看,至少不能将其与慢性肾脏病-矿物质和骨异常(CKD-MBD)分开来看。然而,关于这种疾病是否应包含在 CKD-MBD 中,目前仍存在争议。最近,通过对β(2)-微球蛋白相关淀粉样纤维形成、延伸和体外解聚的研究,更好地了解了 DRA 的发病机制。载脂蛋白 E、蛋白聚糖和糖胺聚糖可稳定淀粉样纤维。此外,一些溶血磷脂和非酯化脂肪酸可在体外生理条件下加速淀粉样纤维的形成和延伸。这些分子可能会增强体内的淀粉样沉积。可能与 DRA 相关的骨与关节疾病的频率和严重程度随透析治疗时间的延长而增加。我们已经表明,接受透析治疗 30 年或更长时间的患者会因骨与关节疾病的严重并发症而存活。除了 CKD-MBD 的经典并发症外,DRA 是导致长期透析治疗患者日常活动和生活质量受损的最严重的骨与关节并发症之一。

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