遗传性载脂蛋白A-Ⅰ型淀粉样变性  

Hereditary apolipoprotein A-Ⅰ related amyloidosis

作　　者：梁少姗[1] 范文静[1] 葛永纯[1] 曾彩虹[1] 

机构地区：[1]南京军区南京总医院肾脏科,国家肾脏疾病临床医学研究中心,全军肾脏病研究所,南京210016

出　　处：《肾脏病与透析肾移植杂志》2015年第3期289-293,共5页Chinese Journal of Nephrology,Dialysis & Transplantation

摘　　要：中青年男性患者,慢性病程,临床表现蛋白尿、轻度低白蛋白血症及高血压,肾功能正常,有肾脏疾病家族史。肾活检组织学改变为肾淀粉样变性,淀粉样物质仅沉积于肾小球,肾小管间质及血管不受累,免疫荧光肾小球载脂蛋白A-Ⅰ染色强阳性。最终该患者诊断为遗传性载脂蛋白A-Ⅰ型淀粉样变性。A 40 year-old man presented with proteinuria, hypoalbuminemia, hypertension, and normal renal function for 17 months. The patient＇s father died from nephritic syndrome. A renal biopsy showed amyloidosis characterized by obliterative glomerular involvement without interstitial or vascular deposition,in which the amyloid deposits stained only for apolipoprotein A- I by immunofluorescense.The final diagnosis was hereditary apolipoprotein A- I related amyloidosis.

关 键 词：载脂蛋白A-I 淀粉样变性 肾活检 

分 类 号：R596[医药卫生—内科学]