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Aganglionosis, total intestinal

MONDO:0008738

A complete lack of ganglia in the intestine. This is an extremely severe form of aganglionosis distinct from Hirschsprung Disease. This is an n-of-1 use case where only one patient or family has been described with this disorder.

Also known as: aganglionosis, total intestinal, total intestinal aganglionosis

1 clinical trial for this condition and its sub-types.

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Broader categories

Disease (679) Hereditary disease (176) Human disease (14) Disease of genetic or genomic mechanism (2) Disease by etiologic mechanism (0)
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  • Robot-Assisted surgery showdown: which fix works best for rare bowel disease?

    Disease control Not yet recruiting

    This study compares two surgical techniques—Duhamel and Soave—for treating total colonic Hirschsprung disease, a severe birth defect where nerve cells are missing from the colon. Fifty children who had a first surgery as newborns will receive one of the two procedures. Researcher…

    Phase: NA • Sponsor: Zunyi Medical College • Aim: Disease control

    Last updated Jun 27, 2026 08:11 UTC

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