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先天性巨结肠相关神经源性肠发育异常(HANID)的临床相关性。

Clinical relevance of Hirschsprung-associated neuronal intestinal dysplasia (HANID).

作者信息

Hanimann B, Inderbitzin D, Briner J, Sacher P

机构信息

Chirurgische Klinik, Universitätskinderspital Zürich, Schweiz.

出版信息

Eur J Pediatr Surg. 1992 Jun;2(3):147-9. doi: 10.1055/s-2008-1063425.

Abstract

The rate of Hirschsprung-associated neuronal intestinal dysplasia (HANID) is reported to be as high as 20-75% but no report deals with its sequelae. The aim of our study was to evaluate the influence of the retained neuronal dysplastic segment in patients with Hirschsprung's disease (HD). We report on 47 patients with HD including 11 cases with HANID (23%). All 47 children had a Duhamel procedure subsequent to colostomy and in none of the 11 cases with HANID was the neuronal dysplastic segment resected. There was no significant difference of early and late complications in both groups and the results of the mean follow-up of 5 years after the Duhamel procedure show that the patients with HANID did as well as the patients with isolated HD. It is suggested therefore that HANID may be a distinct disease compared to isolated NID and that the NID attained segment may be retained without increased risks or morbidity.

摘要

据报道,先天性巨结肠相关的神经元性肠发育异常(HANID)的发生率高达20%-75%,但尚无关于其后遗症的报道。我们研究的目的是评估先天性巨结肠(HD)患者中保留的神经元发育异常节段的影响。我们报告了47例HD患者,其中11例患有HANID(23%)。所有47名儿童在结肠造口术后均接受了杜哈梅尔手术,11例HANID患者中无一例切除神经元发育异常节段。两组的早期和晚期并发症无显著差异,杜哈梅尔手术后平均5年的随访结果表明,HANID患者与孤立性HD患者的情况一样好。因此,有人认为HANID可能是一种与孤立性NID不同的疾病,并且可以保留获得性NID节段,而不会增加风险或发病率。

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