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megaduodenum

Monday 13 February 2006

Hereditary megaduodenum is a rare disease with autosomal dominant transmission characterized by visceral myopathy that involves the digestive and urinary tracts, and usually presents as intestinal pseudo-obstruction (IPO).

Recently, a high rate of spontaneous chromosomal damage was found in blood lymphocytes of patients with hereditary megaduodenum, predictive of an increased cancer risk.

Synopsis

- visceral myopathy
- severe digestive pseudo-obstruction
- recurrent urinary tract infections
- vacuolar degeneration and fibrosis of the longitudinal layer of gastrointestinal muscle

Etiology

- infantile visceral myopathy

References

- Basilisco G. Hereditary megaduodenum. Am J Gastroenterol. 1997 Jan;92(1):150-3. PMID: 8995957