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芬兰型先天性肾病综合征中肾小球基底膜的厚度

The thickness of the glomerular basement membrane in congenital nephrotic syndrome of the Finnish type.

作者信息

Autio-Harmainen H, Rapola J

出版信息

Nephron. 1983;34(1):48-50. doi: 10.1159/000182978.

Abstract

Electron microscopic examination was carried out on 10 glomeruli from 4 infants with congenital nephrotic syndrome of the Finnish type (CNF) and 5 glomeruli from 1 control. The thickness of the peripheral glomerular basement membrane (GBM) and lamina densa were measured. The width of the GBM was thinner in CNF than in the control but still within normal limits for the age and the width of the lamina densa was thinner in CNF compared to the control. This study supports earlier findings that the basic defect in CNF could be a faulty synthesis of the lamina densa.

摘要

对4例芬兰型先天性肾病综合征(CNF)婴儿的10个肾小球和1例对照的5个肾小球进行了电子显微镜检查。测量了外周肾小球基底膜(GBM)和致密层的厚度。CNF中GBM的宽度比对照中窄,但仍在该年龄的正常范围内,且与对照相比,CNF中致密层的宽度更窄。本研究支持了早期的发现,即CNF的基本缺陷可能是致密层合成错误。

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