| Intestinal neuronal dysplasia | |
|---|---|
| Classification and external resources | |
| OMIM | 243180 601223 |
Intestinal neuronal dysplasia (or neuronal intestinal dysplasia or NID) is an inherited disease of the intestine that effects one in 3000 children and adults. The intestine uses peristalsis to push its contents toward the anus; IND sufferers have a problem with the motor neurons that lead to the intestine, inhibiting this process and thus preventing digestion.
It can often be confused for Hirschsprung's disease, as both have similar symptoms.[1]
It can be grouped into NID A and NID B, with the "A" form affecting the sympathetic innervation, and the "B" version affecting the parasympathetic innervation.[2][3]
Conservative treatment involves the long term use of laxatives and enemas, and has limited success. Dietary changes in order to control the disease are ineffective and high fiber diets often worsen the symptoms in children. As a last resort, surgical treatment (internal sphincter myectomy or colon resection) is used.[4] In extreme cases, the only effective cure is a complete transplant of the affected parts. The threat of IND is such that, even though it is relatively rare, all new born babies are kept in hospital until they defecate.
A famous case of IND is that of Adele Chapman, who had a triple transplant of the small intestine, pancreas and liver, the first of its kind in the UK; therefore the official charity of IND is the Adele Chapman Foundation.