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Familial and genetic aspects of neuronal intestinal dysplasia and Hirschsprung's disease

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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.

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Authors and Affiliations

  1. Department of Pediatric Surgery, University of Stellenbosch, Tygerberg Hospital, 7505, Tygerberg, South Africa

    S. W. Moore

  2. Departments of Pathology and Pediatric Surgery, University of Cape Town and Red Cross War Memorial Children's Hospital, 7700, Rondebosch, South Africa

    R. O. C. Kaschula & S. Cywes

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  1. S. W. Moore
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  2. R. O. C. Kaschula
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  3. S. Cywes
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Correspondence to: S. W. Moore

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Moore, S.W., Kaschula, R.O.C. & Cywes, S. Familial and genetic aspects of neuronal intestinal dysplasia and Hirschsprung's disease. Pediatr Surg Int 8, 406–409 (1993). https://doi.org/10.1007/BF00176729

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