Familial Aspects of Hirschsprung's Disease

Abstract excerpt

Twenty-eight of 370 patients (14 families) treated for Hirschsprung's disease (HD) over a 34 year period had a family member with histologically proven HD. These 14 represented 4 % of the 351 families: more than one affected child per family in 10 (2.8 %) and both parent and child in 2 families. Neuronal intestinal dysplasia (NID) in a parent was associated with total colonic aganglionosis in two siblings of one...

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