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Familial and genetic aspects of neuronal intestinal dysplasia and Hirschsprung's disease
Authors:S. W. Moore  R. O. C. Kaschula  S. Cywes
Affiliation:(1) Department of Pediatric Surgery, University of Stellenbosch, Tygerberg Hospital, 7505 Tygerberg, South Africa;(2) Departments of Pathology and Pediatric Surgery, University of Cape Town and Red Cross War Memorial Children's Hospital, 7700 Rondebosch, South Africa
Abstract:
A familial occurrence of Hirschsprung's disease and neuronal intestinal dysplasia (NID) is reported. Familial occurrence of NID in a parent and long-segment Hirschsprung's disease in two children in a family as well as a further report of NID in monozygotic twins is described. The linkage of these neurodevelopmental conditions is explored and a common etiologic mechanism for the two conditions is postulated.Correspondence to: S. W. Moore
Keywords:Neuronal intestinal dysplasia  Hirschsprung's disease  Familial  Genetic
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