Idiopathic intestinal pseudo-obstruction in a family.
A patient with severe manifestations of idiopathic intestinal pseudo-obstruction is presented. Three members of his family suffer from similar symptoms but in a much milder form. The disease has a wide range of clinical and radiologic presentations and its diagnosis is dependent upon the exclusion of all known causes of intestinal pseudo-obstruction. A high incidence of associated megacystis in this family as well as in some reported cases in the literature lead us to conclude that idiopathic intestinal pseudo-obstruction is not a primary disease of the gastrointestinal tract. Its etiology remains obscure.
D000328:Adult; D004108:Dilatation, Pathologic; D005260:Female; D005769:Gastrointestinal Motility; D006801:Humans; D007415:Intestinal Obstruction / Q000000981:diagnostic imaging* / Q000235:genetics / Q000503:physiopathology; D007421:Intestine, Small / Q000000981:diagnostic imaging*; D008297:Male; D008875:Middle Aged; D011859:Radiography