透析相关性淀粉样变

Dialysis related amyloidosis

透析相关性淀粉样变(DRA)是维持性血液透析患者的严重并发症之一,β2微球蛋白(β2-MG)淀粉样物质在骨关节和内脏沉积是DRA的主要特征,临床表现为腕管综合征、囊性骨损害与病理性骨折、骨关节病、破坏性关节病及全身淀粉样变性。随着透析技术的发展,DRA患病率和严重程度下降。高浓度β2-MG是发生DRA的先决条件,β2-MG淀粉样纤维具有细胞毒性,成核聚合模型可部分解释β2-MG淀粉样纤维的形成机制。高通量血液透析、血液透析滤过和β2-MG特异性吸附均能增加β2-MG清除,新型延展性透析技术清除效率更高;应用高生物相容性膜和超纯透析液可以减少β2-MG产生。肾移植是预防DRA的最佳方式。

Dialysis-related amyloidosis(DRA)is a serious complication of long-term dialysis therapy and is characterized by deposition of amyloid fibrils,principally composed of β2-microglobulin(β2-MG),in the osteoarticular structures and viscera.The clinical manifestations of DRA are carpal tunnel syndrome,cystic bone damage and pathological fracture,osteoarthropathy,destructive joint disease,and systemic amyloidosis.The prevalence and severity of DRA would be lower with hemodialysistechnology.Amyloid fibrils have been shown to possess cytotoxic potentials.A nucleation-dependent polymerization model could explain the mechanisms of amyloid fibril formation.A high plasma concentration of β2-MG is considered as a prerequisite for developing DRA.High-flux hemodialysis,hemodiafiltration and immuno-adsorption are able to removeβ2-MG significantly,especially expanded hemodialysis.The use of biocompatible membranes and ultrapure dialysate can minimize or delay DRA onset.Renal transplantation is the best way to prevent DRA.