Familial and genetic aspects of neuronal intestinal dysplasia and Hirschsprung's disease |
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Authors: | S. W. Moore R. O. C. Kaschula S. Cywes |
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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 |
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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 |
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Keywords: | Neuronal intestinal dysplasia Hirschsprung's disease Familial Genetic |
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