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薄基底膜肾病:一项临床病理研究。

Thin membrane nephropathy: a clinico-pathological study.

作者信息

Aarons I, Smith P S, Davies R A, Woodroffe A J, Clarkson A R

机构信息

Institute of Medical and Veterinary Science, Adelaide, South Australia.

出版信息

Clin Nephrol. 1989 Oct;32(4):151-8.

PMID:2805457
Abstract

Thin membrane nephropathy is common, representing approximately 11% of non-transplant renal biopsies. A family history of renal disease is present in at least 40% of patients. Electron microscopy is essential for its diagnosis. There are no immunofluorescence markers but light microscopic changes, usually mild, are invariably present and predict the ultrastructural findings although there is no correlation with their degree. The extent of the morphological changes bears no obvious relationship either to clinical or familial features. Immunogold studies indicate that there is reduction or loss of the subepithelial portion of the basement membrane, which apparently contains normal amounts of type IV collagen. Unnecessary urological investigations may be avoided by awareness of the condition and microscopic examination of urine for dysmorphic red blood cells. Prospective long-term studies are necessary to determine the nature and consequences of the condition.

摘要

薄基底膜肾病很常见,约占非移植肾活检病例的11%。至少40%的患者有肾脏疾病家族史。电子显微镜检查对其诊断至关重要。虽然没有免疫荧光标志物,但光镜下的改变通常很轻微,总是存在,并且可以预测超微结构的结果,尽管与改变程度无关。形态学改变的程度与临床或家族特征均无明显关系。免疫金研究表明,基底膜上皮下部分减少或缺失,而基底膜中IV型胶原含量看似正常。认识到这种疾病并对尿液进行显微镜检查以发现畸形红细胞,可避免不必要的泌尿外科检查。有必要进行前瞻性长期研究以确定该疾病的性质和后果。

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