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成人中与血尿、蛋白尿或肾衰竭相关的异常薄的肾小球基底膜。

Abnormally thin glomerular basement membranes associated with hematuria, proteinuria or renal failure in adults.

作者信息

Dische F E, Weston M J, Parsons V

出版信息

Am J Nephrol. 1985;5(2):103-9. doi: 10.1159/000166914.

Abstract

Clinical and pathologic findings are described in 14 patients whose main abnormality was an excessively thin glomerular basement membrane (GBM). The subjects were aged 11-51 years, the majority males: most were referred because of hematuria, but proteinuria was the main problem in 2, while hypertension or renal functional impairment was found in several, and 1 was in end-stage renal failure. A history of apparently similar renal symptoms was obtained in another 3 family members. In addition to GBM abnormalities, renal biopsy features included a slight mesangial matrix increase, occasional mesangial cell excess and often appreciable pedicel effacement. There were scanty electron-dense deposits. The mean thickness of the GBM varied from 206 to 301 nm, whereas in IgA nephropathy patients used as controls it was 356-464 nm. It is concluded that some of the lesions in adults are 'benign', and some progressive. 'Thin membrane nephropathy' is comparatively common among patients seen by the authors, and it is suggested that awareness of this condition will result more often in its recognition.

摘要

对14例主要异常为肾小球基底膜(GBM)过度变薄的患者的临床和病理表现进行了描述。这些受试者年龄在11至51岁之间,大多数为男性:大多数因血尿就诊,但2例以蛋白尿为主要问题,数例有高血压或肾功能损害,1例处于终末期肾衰竭。另外3名家庭成员有明显相似的肾脏症状病史。除GBM异常外,肾活检特征还包括轻度系膜基质增加、偶尔系膜细胞增多以及常可见的足突消失。电子致密沉积物稀少。GBM的平均厚度在206至301nm之间,而作为对照的IgA肾病患者的GBM平均厚度为356 - 464nm。结论是,成人中的一些病变是“良性的”,一些是进行性的。“薄基底膜肾病”在作者诊治的患者中相对常见,建议提高对这种疾病的认识将更常导致其被识别。

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