淀粉样变肾病研究进展

Research progress of amyloidosis nephropathy

淀粉样变是由一组累及器官，在治疗和预后等方面大不相同的特定蛋白质的致病性错误折叠引起的疾病。淀粉样物质肾小球沉积表现主要为蛋白尿、明显的肾病综合征和不同程度的肾功能不全。然而，肾小管间质的累及占大部分，特别是一些遗传性类型载脂蛋白A-I淀粉样变性表现为进行性肾功能降低，但没有明显蛋白尿出现。研究表明，激光显微切割联合质谱分析（LMD／MS）的应用可以明确前体蛋白进而分型，对淀粉样变性肾病的组织病理学特征及不同分型的临床表现和治疗方法进行总结具有重要意义。

The amyloidosis encompasses a group of diseases caused by the pathogenic misfolding of specific proteins that differ substantially with respect to organ involvement, management, and prognosis. Immuno- globulin light-chain associated （AL） and secondary （AA） amyloidoses are by far the most prevalent subtypes with renal involvement. Renal presentation mainly reflects glomerular deposits with proteinuria, often overt ne- phrotie syndrome, and varying renal insufficiency. However, where tubulointerstitial involvement predominates, particularly in some types of hereditary apolipoprotein A-1 amyloidosis, a gradual decline in renal excretory func- tion,is obsewed without significant proteinuria. The use of mass spectrometry-based proteomies can the clearly identify precursor proteins and then typing. It＇s important to summarize the, histopathological characteristics of nephropathy amyloidosis, the clinical manifestations, and treatment methods.