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神经元性肠发育异常

Neuronal intestinal dysplasia.

作者信息

Ryan D P

机构信息

Department of Surgery, Massachusetts General Hospital, Boston 02114, USA.

出版信息

Semin Pediatr Surg. 1995 Feb;4(1):22-5.

PMID:7728504
Abstract

Neuronal intestinal dysplasia (NID) is one of the disorders of gastrointestinal motility with defined pathological changes. Although first described more than 20 years ago, the disease remains controversial. The histological findings do not correlate with the degree of symptoms. The etiology of NID is obscure, but an immunologic process has been suggested. The diagnosis can be made on biopsy specimens performed to identify Hirschsprung's disease, but treatment must be individualized. The clinical course is variable, with spontaneous improvement in some, relentless constipation or severe dysmotility, unresponsiveness to all therapy, in others. Duhamel or Soave pull-through procedures can be effective in selected cases.

摘要

神经元性肠发育异常(NID)是一种具有明确病理改变的胃肠动力障碍性疾病。尽管该病于20多年前首次被描述,但至今仍存在争议。组织学检查结果与症状程度不相关。NID的病因尚不清楚,但有人提出与免疫过程有关。诊断可通过为诊断先天性巨结肠而进行的活检标本作出,但治疗必须个体化。临床病程多变,一些患者可自发改善,另一些患者则有顽固的便秘或严重的动力障碍,对所有治疗均无反应。在某些选定的病例中,杜哈梅尔或索阿韦拖出式手术可能有效。

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