Intestinal neuronal dysplasia type B: How do we understand it today?

被引：10

作者：

Bruder E. ^{[2
]}

Meier-Ruge W.A. ^{[1
,2
]}

机构：

[1] Institut für Pathologie, Universitätsspital Basel, 4031 Basel

[2] Institut für Pathologie, Universitätsspital Basel

来源：

Der Pathologe | 2007年 / 28卷 / 2期

关键词：

Constipation; Intestinal neuronal dysplasia; Pseudo-obstruction; Submucosal plexus;

D O I：

10.1007/s00292-007-0894-x

中图分类号：

学科分类号：

摘要：

Intestinal neuronal dysplasia type B (IND B) is currently considered to be a subtle malformation of the submucosal plexus, leading to an increased proportion of over-sized ganglia and potentially accompanied by a mild, chronic gastrointestinal motility disturbance. The diagnosis of IND B is morphologically based and involves the demonstration of an increased proportion of giant ganglia in the submucous plexus related to the patient's age. Giant ganglia are physiologically frequent in the neonatal period. Therefore, IND B should not be diagnosed prior to 1 year of age. Morphological features of IND B may occur as an isolated finding or may be observed proximal to an aganglionic segment. IND B and constipation may resolve spontaneously up to the age of 4 years. Treatment of IND B is usually conservative, surgical resection is currently deemed necessary only in a minority of patients. The pathogenesis of IND B is still incompletely understood and the etiology unknown. Future research on the basis of standardized diagnostic conditions is expected to result in a better understanding of this disease, and to reveal the cause of aberrant ganglion development. © 2007 Springer Medizin Verlag.

引用

页码：137 / 142

页数：5

共 33 条

[1]

Coerdt W., Michel J.S., Et al., Quantitative morphometric analysis of the submucous plexus in age-related control groups, Virchows Arch, 444, pp. 239-246, (2004)

[2]

Costa M., Evaluation of the Hox11L1 gene as a candidate for congenital disorders of the intestinal innervation, J Med Genet, 37, (2000)

[3]

Fava M., Et al., HOX11L1: A promoter study to evaluate possible expression defects in intestinal motility disorders, Int J Mol Med, 10, pp. 101-106, (2002)

[4]

Gabriel S.B., Salomon R., Pelet A., Et al., Segregation at three loci explains familial and poluplation risk in Hirschsprung disease, Nat Genet, 31, pp. 89-93, (2002)

[5]

Gath R., Et al., Analysis of the RET, GDNF, EDN3 and EDNRB genes in patients with intestinal neuronal dysplasia and Hirschsprung disease, Gut, 48, pp. 671-675, (2001)

[6]

Gillick J., Tazawa H., Puri P., Intestinal neuronal dysplasia: Results of treatment in 33 patients, J Pediatr Surg, 36, pp. 777-779, (2001)

[7]

Hatano M., Et al., A novel pathogenesis of megacolon in Ncx/Hox11L1 deficient mice, J Clin Invest, 100, pp. 795-801, (1997)

[8]

Hirschsprung's disease and allied disorders, (2000)

[9]

Kapur R.P., Multiple endocrine neoplasia type 2B and Hirschsprung's disease, Clin Gastroenterol Hepatol, 3, pp. 423-431, (2005)

[10]

Koletzko S., Jesch I., Faus-Kebetaler T., Et al., Rectal biopsy for diagnosis of intestinal neuronal dysplasia in children: A prospective multicentre study on interobserver variation and clinical outcome, Gut, 44, pp. 853-861, (1999)

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