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家族性小叶性肾小球病:亚洲首例病例报告。

Familial lobular glomerulopathy: first case report in Asia.

作者信息

Sato H, Matsubara M, Marumo R, Soma J, Kurosawa K, Taguma Y, Saito T

机构信息

The Second Department of Internal Medicine, Tohoku University School of Medicine,1-1, Seiryo-cho, Aoba-ku, Sendai 980-77, Japan.

出版信息

Am J Kidney Dis. 1998 Jun;31(6):E3. doi: 10.1053/ajkd.1998.v31.pm10074583.

Abstract

A 23-year-old male Japanese student presented a unique lobular glomerulopathy characterized by mesangial and subendothelial expansion with numerous periodic acid-Schiff-positive deposits. Electron microscopy showed massive fine granular deposits with a homogeneous distribution. Fibrillar or microtubular structures were not demonstrated. Fibronectin was positive on immunostaining, as was immunoglobulin G and fibrinogen. Familial study revealed that the patient's grandfather, two aunts, and one cousin on his father's side had developed end-stage renal failure. Clinicopathologic features of this patient are identical with those of familial lobular glomerulopathy, which has been previously described by several investigators. Seven of the previously reported families were white and resided in the United States or in European countries. This is the first report of an Asian case, and indicates that this disease universally occurs independently of racial specificity.

摘要

一名23岁的日本男性学生表现出一种独特的小叶性肾小球病,其特征为系膜和内皮下扩张,伴有大量高碘酸-希夫染色阳性沉积物。电子显微镜显示有大量均匀分布的细颗粒沉积物。未发现纤维状或微管状结构。免疫染色显示纤连蛋白呈阳性,免疫球蛋白G和纤维蛋白原也呈阳性。家族研究表明,患者的祖父、两位姑姑以及父亲一方的一位堂兄已发展为终末期肾衰竭。该患者的临床病理特征与先前几位研究者所描述的家族性小叶性肾小球病相同。先前报道的家族中有7个是白人,居住在美国或欧洲国家。这是亚洲病例的首例报告,表明这种疾病普遍存在,与种族特异性无关。

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