肾脏轻/重链沉积病诊治进展  

Research progress in diagnosis and treatment of renal light/heavy chain deposition disease

作　　者：李丽[1] 刘芳[1] 

机构地区：[1]四川大学华西医院肾内科,成都610041

出　　处：《中华肾病研究电子杂志》2014年第2期43-46,共4页Chinese Journal of Kidney Disease Investigation（Electronic Edition）

摘　　要：轻/重链沉积病是临床少见病,肾脏是其最常见受累脏器,临床常表现为大量蛋白尿和肾功能不全。血/尿游离轻链检测具有重要诊断价值。肾脏病理是诊断肾脏轻/重链沉积病的重要手段,且需要光镜、电镜及免疫病理共同诊断,缺一不可。结节样肾小球硬化是光镜下典型的肾脏病理改变。自体干细胞移植被认为有利于改善肾脏受累患者的预后。本文对该病的发病机制、诊治进展及预后进行了综述。Light/heavychaindepositiondiseaseisaclinicallyrarediseaseinwhichkidneyisthe most commonly affected organ,and usually manifested heavy proteinuria and renal insufficiency. Blood/urine free light chain ratio is of diagnostic value. Renal pathology is an important method for diagnosis of kidney-involved light/heavy chain deposition disease in which light microscopy, electron microscopy, and immunopathology are all indispensable for the diagnosis. Nodular glomerulosclerosis is a typical pathological change under light microscope. Autologous stem cell transplantation is considered conducive to improve the prognosis of patients with renal involvement. Here,the research progress in the pathogenesis,diagnosis and treatment,as well as prognosis of light/heavy chain deposition disease has been reviewed.

关 键 词：轻/重链沉积病 结节样肾小球硬化 自体干细胞移植 

分 类 号：R692[医药卫生—泌尿科学]