Congenital microvillous atrophy: report of two cases.

General & diagnostic pathology Pub Date : 1997-02-01
H Steininger, R Behrens, G Faller, C Schindler, T Kirchner
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Abstract

2 infants with chronic severe diarrhoea from birth and with lethal outcome have been studied. Small intestinal biopsies were examined by light and electron microscopy. Severe villous atrophy, complete loss of microvilli or rudimentary forms, intracytoplasmic vesicles, and microvillous inclusions are the characteristic features of the disease. The etiology is unknown. A disturbance in the transport of brush-border proteins to the cell surface is assumed to be the reason for congenital microvillous atrophy.

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先天性微绒毛萎缩2例报告。
对出生时患有慢性严重腹泻并具有致命后果的婴儿进行了研究。光镜和电镜检查小肠活检。严重的绒毛萎缩,微绒毛或初级形态的完全丧失,胞浆内小泡和微绒毛包涵体是该病的特征。病因不明。刷状边界蛋白向细胞表面的运输受到干扰,被认为是先天性微绒毛萎缩的原因。
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