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Idiopathic intestinal pseudo‐obstruction: A familial visceral neuropathy
Author(s) -
Roy A. D.,
Bharucha H.,
Nevin N. C.,
OdlingSmee G. W.
Publication year - 1980
Publication title -
clinical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 102
eISSN - 1399-0004
pISSN - 0009-9163
DOI - 10.1111/j.1399-0004.1980.tb00888.x
Subject(s) - medicine , abdominal distension , laparotomy , gastroenterology , atrophy , intestinal pseudo obstruction , bowel obstruction , surgery , distension , anastomosis
Four individuals with idiopathic intestinal pseudo‐obstruction (IIP), a familial visceral neuropathy with autosomal dominant inheritance, are presented. The disorder is characterised by abdominal colic, abdominal distension and diarrhoea, and is of a progressive nature with relapses and remissions. The main feature is a disturbance of motility in the affected intestine, with histological evidence of a neuronal disorder, characterised by hyperplasia and eventual atrophy. Management of the condition is difficult; initially, treatment should be conservative by nasogastric suction with intravenous nutrition. When laparotomy is undertaken to exclude mechanical obstruction, resection of the intestine is inadvisable, and ileocolic anastomosis may be beneficial.