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[结直肠神经支配畸形的分类]

[Classification of malformations of colorectal innervation].

作者信息

Meier-Ruge W

机构信息

Institut für Pathologie, Universität Basel.

出版信息

Verh Dtsch Ges Pathol. 1991;75:384-5.

PMID:1724847
Abstract

Inborn errors of colorectal innervation can be classified in four different forms: 1. Aganglionosis (including total aganglionosis of the colon, Hirschsprung's disease, ultrashort Hirschsprung's segment and neurogenic sphincter achalasia) 2. Hypoganglionosis 3. Congenital malformation of sympathetic innervation of the colon (Neuronal Intestinal Dysplasia Typ A or NID A) 4. Congenital malformation of the submucous plexus (Neuronal Intestional Dysplasia Typ B or NID B). We find in 52.2% of our biopsies with a characteristic malformation of the colorectal innervation an aganglionosis. 40.6% are malformations of the submucous plexus (NID B). Half of the cases with Hirschsprung's disease are combined with NID B. 5.0% of the classified colorectal malformations of the innervation are a hypoganglionosis and 2.2% a NID A. An additional half of our biopsies cannot be introduced into the above given classification due to moderate malformations of the colorectal innervation (mild dysganglionosis, hypogenetic nerve cells of the submucous plexus, heterotopic nerve cells of the submucous and myenteric plexus).

摘要

结直肠神经支配的先天性异常可分为四种不同形式

  1. 无神经节症(包括结肠完全无神经节症、先天性巨结肠、超短先天性巨结肠段和神经源性括约肌失弛缓症)2. 神经节减少症3. 结肠交感神经支配的先天性畸形(A型神经元性肠发育异常或NID A)4. 黏膜下丛的先天性畸形(B型神经元性肠发育异常或NID B)。在我们52.2%具有结直肠神经支配特征性畸形的活检病例中发现无神经节症。40.6%为黏膜下丛畸形(NID B)。先天性巨结肠病例中有一半合并NID B。在分类的结直肠神经支配畸形中,5.0%为神经节减少症,2.2%为NID A。由于结直肠神经支配存在中度畸形(轻度神经节发育异常、黏膜下丛神经细胞发育不全、黏膜下丛和肌间神经丛异位神经细胞),我们另外一半的活检病例无法归入上述分类。

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