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连续三个兄弟姐妹出现多处胃肠道闭锁:发病机制观察

Multiple gastrointestinal atresias in three consecutive siblings: observations on pathogenesis.

作者信息

Puri P, Guiney E J, Carroll R

出版信息

J Pediatr Surg. 1985 Feb;20(1):22-4. doi: 10.1016/s0022-3468(85)80385-7.

Abstract

Three consecutive siblings with innumerable membranous gastrointestinal atresias, beginning at the prepyloric region and ending in the rectum, are reported. Microscopic examination of the small and large intestine showed multiple, tiny lumina each surrounded by a separate mucosa and muscularis mucosae but by a single muscular layer at the periphery. The pathological findings and hereditary character of these atresias suggest that a defect in recanalization during a solid stage of development involving the whole of the gastrointestinal tract may be the cause of these atresias.

摘要

本文报告了连续三个兄弟姐妹患有无数的膜性胃肠道闭锁,起自幽门前区,止于直肠。对小肠和大肠的显微镜检查显示,有多个微小管腔,每个管腔都被单独的黏膜和黏膜肌层包围,但外周只有一层肌肉层。这些闭锁的病理发现和遗传特征表明,在涉及整个胃肠道的发育实体阶段,再通缺陷可能是这些闭锁的原因。

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