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[微小病变肾病综合征合并薄基底膜病初期肾小球足细胞的超微结构]

[Ultrastructure of glomerular podocyts in the incipient phase of minimal change nephrotic syndrome with thin basement membrane disease].

作者信息

Ogawa Ryo, Miyoshi Ken-ichi, Nagao Tomoaki, Jotoku Masanori, Irita Jun, Okura Takafumi, Higaki Jitsuo

机构信息

Postgraduate Clinical Training Center, Ehime University Hospital, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 2012;54(8):1192-6.

Abstract

An 80-year-old woman was referred to the Division of Nephrology at Ehime University Hospital because of leg edema in December 2010. She had been treated with 300 mg of tocopherol for scleroderma since 2007 and treated with 9 mg of prednisolone (PSL) for autoimmune hearing loss since 2010. Due to the occurrence of mild hematuria (5-9/HPF), proteinuria (0.9 g/day) and an increased serum creatinine level (1.31 mg/dL), a renal biopsy was performed. Light microscopy (LM) showed minor abnormality in the glomeruli, and immunohistology showed the absence of deposits of immunoglobulins and complements. Electron microscopy (EM) showed a thin glomerular basement membrane with a limited level of podocyte abnormalities. Due to the findings of intimal thickening of interlobular arteries and subcapsular accumulation of global sclerosis on LM, she was diagnosed with nephrosclerosis and thin basement membrane disease. Four weeks later, her leg edema had increased considerably and urinary protein had increased to 12.4 g/day. The second biopsy showed similar findings in LM and IF as the first biopsy, but EM revealed diffuse foot process effacement. She was diagnosed with minimal change nephrotic syndrome (MCNS) and treated with methylprednisolone pulse therapy followed by 40 mg of oral PSL. Her urinary protein had completely disappeared 6 weeks later. Complete remission with PSL treatment indicates that urinary protein at first renal biopsy was due to MCNS. Our case exhibited podocyte features in the incipient phase of human MCNS.

摘要

2010年12月,一名80岁女性因腿部水肿被转诊至爱媛大学医院肾病科。自2007年起,她因硬皮病接受300毫克生育酚治疗,自2010年起因自身免疫性听力损失接受9毫克泼尼松龙(PSL)治疗。由于出现轻度血尿(5 - 9/HPF)、蛋白尿(0.9克/天)以及血清肌酐水平升高(1.31毫克/分升),遂进行了肾活检。光镜检查(LM)显示肾小球有轻微异常,免疫组织学检查显示无免疫球蛋白和补体沉积。电镜检查(EM)显示肾小球基底膜变薄,足细胞异常程度有限。鉴于光镜检查发现小叶间动脉内膜增厚以及肾小球球囊下全球硬化积聚,她被诊断为肾硬化和薄基底膜病。四周后,她的腿部水肿显著加重,尿蛋白增加至12.4克/天。第二次活检在光镜和免疫荧光检查中显示与第一次活检相似的结果,但电镜检查显示弥漫性足突消失。她被诊断为微小病变肾病综合征(MCNS),并接受了甲泼尼龙冲击治疗,随后口服40毫克PSL。六周后她的尿蛋白完全消失。PSL治疗完全缓解表明首次肾活检时的尿蛋白是由MCNS引起的。我们的病例在人类MCNS的初期表现出足细胞特征。

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