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Immunostaining to Visualize Murine Enteric Nervous System Development
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[Intestinal neuronal dysplasia: association with digestive malformations].

R Granero Cendón1, A Millán López, M J Moya Jiménez

  • 1Servicio de Cirugía Pediátrica, H. Infantil Universitario Virgen del Rocío, Sevilla. rociogranero3@yahoo.es

Cirugia Pediatrica : Organo Oficial De La Sociedad Espanola De Cirugia Pediatrica
|November 21, 2007
PubMed
Summary

Isolated Intestinal Neuronal Dysplasia type B affects 1 in 7500 newborns, with 35% experiencing associated congenital anomalies, primarily digestive malformations. Familial constipation history was noted in 40% of cases.

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Area of Science:

  • Gastroenterology
  • Pediatric Surgery
  • Pathology

Context:

  • Isolated Intestinal Neuronal Dysplasia (IND) type B is a condition affecting the enteric nervous system.
  • Diagnosis involves histopathologic findings like giant ganglia and increased acetylcholinesterase activity.
  • This study focuses on IND type B not associated with Hirschsprung's disease.

Purpose:

  • To determine the incidence of congenital malformations in IND type B.
  • To correlate these anomalies with symptom severity.
  • To investigate potential familial involvement in IND type B.

Summary:

  • A retrospective review of 44 IND type B cases (1:7500 newborns) from 1981-2002 was conducted.
  • 35% of patients had associated congenital anomalies, most commonly digestive malformations (20%).
  • Familial constipation history was present in 40%, and 75% of symptoms appeared in the newborn period.

Impact:

  • Provides incidence data for congenital anomalies in IND type B.
  • Highlights the significant association with digestive malformations.
  • Suggests a potential genetic or familial component requiring further investigation.