Hirschsprungs

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Hirschsprung's Disease

Summary: This disease (LS-HSCR) is a very rare (one in 5 million by one estimate) disease where the nerve (ganglion) cells that drive peristalsis of the intestine fail to migrate very far down the intestine. It is distinct in several ways (and much rarer) from the more common form of Hirschsprung's that only affects the large intestine (colon). It has many of the same initial symptoms as meconium ileus (MI) -- a blockage of the small intestine due to meconium buildup that is usually caused by cystic fibrosis (CF). It leads almost inevitably to short gut syndrome.

Note that it is possible to have meconium ileus without cystic fibrosis, for example, due to immature ganglion cells. This seems to be especially true in Asian populations.

Sub Topics

• HscrDiagPath -- diagnosis and pathology
• HscrGenetics -- genetics
• HscrTreatment -- treatment details that are specific to this etiology

Links

• http://www.hirschsprungs.info/index.html
• John Hopkins University has a family study that is looking at the genetics of Hirshsprung's Disease. To participate in the study and get more information, click on the link [1]