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先天性巨结肠症:儿童慢性便秘的一个病因。

Hirschsprung's disease: a cause of chronic constipation in children.

作者信息

Worman S, Ganiats T G

机构信息

University of California, San Diego, School of Medicine, La Jolla.

出版信息

Am Fam Physician. 1995 Feb 1;51(2):487-94.

PMID:7840044
Abstract

Constipation is common in neonates and children. Most constipation is benign and easily treated. Hirschsprung's disease is an unusual, but well-recognized cause of chronic constipation. An example of the group of disorders known as dysganglionoses, Hirschsprung's disease is characterized by an absence of the intramural ganglionic cells at the submucosal and myenteric levels of the intestine. The course of the disease is variable, with five distinct patterns of varying severity. Without treatment, mortality approaches 50 percent by one year of age, but early diagnosis and intervention are associated with a favorable outcome. The treatment is surgical, and several approaches are currently used.

摘要

便秘在新生儿和儿童中很常见。大多数便秘是良性的,易于治疗。先天性巨结肠是一种罕见但已被充分认识的慢性便秘病因。作为神经节发育异常疾病组的一个例子,先天性巨结肠的特征是在肠道黏膜下层和肌间神经丛水平缺乏壁内神经节细胞。该病的病程多变,有五种不同严重程度的明显模式。未经治疗,一岁时死亡率接近50%,但早期诊断和干预可带来良好预后。治疗方法是手术,目前有几种手术方式。

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