BACKGROUND: Chronic @entity8442 , a syndrome of @entity1319 due to a primary @entity121 , is rare. AIMS: To determine the clinical spectrum, underlying pathologies, response to treatments, and prognosis in a consecutive unselected group of @entity1 . METHODS: Cross sectional study of all @entity1 with clinical and radiological features of @entity1321 in the absence of organic obstruction, associated with dilated small intestine (with or without dilated large intestine), being actively managed in one tertiary referral centre at one time. RESULTS: Twenty @entity1 (11 @entity1 and nine @entity1 , median age 43 years, range 22-67) fulfilled the diagnostic criteria. Median age at onset of symptoms was 17 years (range two weeks to 59 years). Two @entity1 had an autosomally dominant @entity10478 . Major presenting symptoms were @entity158 (80%), @entity391 (75%), @entity1142 (40%), and @entity294 (20%). Eighteen @entity1 required abdominal surgery, and a further @entity1 had a full thickness rectal biopsy. The mean time interval from symptom onset to first operation was 5.8 years. Histology showed @entity10478 in 13, @entity16 in three, and was indeterminate in three. In the one other @entity1 small bowel motility studies were suggestive of @entity16 . Two @entity1 died within two years of symptom onset, one from generalised @entity715 and the other from an @entity5442 . Of the remaining 18 @entity1 , eight were nutritionally independent of supplements, two had gastrostomy or jejunostomy feeds, and eight were receiving home parenteral nutrition. Five @entity1 were opiate dependent, only one @entity1 had benefited from prokinetic drug therapy, and five @entity1 required formal psychological intervention and support. CONCLUSIONS: In a referral setting @entity10478 is the most common diagnosis in this heterogeneous syndrome, the course of the illness is usually prolonged, and prokinetic drug therapies are not usually helpful. Ongoing management problems include @entity158 relief and nutritional support.

Clinical characteristics of chronic XXXX in adults.

@entity1 :: ('9606', 'Species') :: ['women', 'patients', 'patient', 'men'] @entity10478 :: ('OMIM:155310', 'Disease') :: ['visceral myopathy', 'inherited visceral myopathy'] @entity5442 :: ('MESH:D009220', 'Disease') :: ['inflammatory myopathy'] @entity294 :: ('MESH:D003967', 'Disease') :: ['diarrhoea'] @entity8442 :: ('MESH:D007418', 'Disease') :: ['idiopathic intestinal pseudo-obstruction'] @entity391 :: ('MESH:D014839', 'Disease') :: ['vomiting'] @entity16 :: ('MESH:D009422', 'Disease') :: ['visceral neuropathy', 'neuropathy'] @entity1321 :: ('MESH:D007415', 'Disease') :: ['intestinal obstruction'] @entity158 :: ('MESH:D010146', 'Disease') :: ['pain'] @entity715 :: ('MESH:D013927', 'Disease') :: ['thrombosis'] @entity1142 :: ('MESH:D003248', 'Disease') :: ['constipation'] @entity121 :: ('MESH:D009135', 'Disease') :: ['disorder of enteric nerve or muscle'] @entity1319 :: ('MESH:D015835', 'Disease') :: ['ineffectual motility']
@entity8442 :: (MESH:D007418,Disease) :: ['idiopathic intestinal pseudo-obstruction']
