《氢气甲烷呼气文献 (241)》由会员分享，可在线阅读，更多相关《氢气甲烷呼气文献 (241)（8页珍藏版）》请在金锄头文库上搜索。
1、Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adultsS D Mann, H S Debinski, M A KammAbstract BackgroundChronic idiopathic intesti- nal pseudo-obstruction, a syndrome of ineVectualmotilityduetoaprimary disorder of enteric nerve or muscle, is rare. AimsTo determine th
2、e clinical spec- trum, underlying pathologies, response to treatments, and prognosis in a consecu- tive unselected group of patients. MethodsCrosssectionalstudyofall patients with clinical and radiological fea- turesofintestinalobstructioninthe absence of organic obstruction, associated with dilated
3、 small intestine (with or with- out dilated large intestine), being actively managed in one tertiary referral centre at one time. ResultsTwenty patients (11 men and nine women, median age 43 years, range2267) fulfilled the diganostic criteria. Median age at onset of symptoms was 17 years (range two
4、weeks to 59 years). Two patients had an autosomally dominant inherited visceral myopathy. Major pre- senting symptoms were pain (80%), vom- iting(75%),constipation(40%),and diarrhoea (20%). Eighteen patients re- quired abdominal surgery, and a further patient had a full thickness rectal biopsy. The
5、mean time interval from symptomonset to first operation was 5.8 years. His- tology showed visceral myopathy in 13, visceral neuropathy in three, and was indeterminate in three. In the one other patient small bowel motility studies were suggestive of neuropathy. Two patients died within two years of
6、symptom onset, one from generalised thrombosis and theother from an inflammatory myopathy.Of the remaining 18 patients, eight were nutritionally independent of supplements, twohadgastrostomyorjejunostomy feeds, and eight were receiving home parenteral nutrition. Five patients were opiate dependent,
7、only one patient hadbenefited from prokinetic drug therapy,and five patients required formal psycho- logical intervention and support. ConclusionsIn a referral setting visceral myopathy is the most common diagnosis inthisheterogeneoussyndrome,the course of the illness is usually prolonged, and proki
8、netic drug therapies are not usuallyhelpful.Ongoingmanagement problems include pain relief and nutri- tional support. (Gut 1997; 41: 675681)Keywords: adult; intestinal; pseudo-obstruction; my- opathy; neuropathyPatientswithchronicidiopathicintestinal pseudo-obstruction (CIIP) have a variety of under
9、lying pathologies which result in defec- tive gut motility. The primary feature is clinical and radiological evidence of intestinal obstruc- tion in the absence of a mechanical lesion. Thecondition was first recognised by Dudley et al in 1958 who described 13 patients with clinical features of bowel
10、 obstruction in the absence of a mechanical cause in whom recurring symp- toms of pain, vomiting, and distension led to multiplelaparotomies.1Theterm“chronicidio-pathic intestinal pseudo-obstruction” was first coined by Maldonado et al in 1970, whodescribed five patients with recurrent unex- plained
11、 episodes of intestinal obstruction, diar- rhoea, andweightloss, whichsometimes progressed to death from starvation.2 The clinical syndrome of chronic intestinal pseudo-obstruction most commonly occurs secondary to diseases such as progressive systemic sclerosis, amyloidosis, and small cell carcinom
12、a of the lung.3The prognosis in these patients often depends on that of the underly- ing disorder. In contrast there is a group of patients with a similar pseudo-obstructive syn- dromeinwhomthereisnorecognised underlyingnon-gastrointestinaldisease.2 4 These patients with CIIP are all thought to have
13、 an abnormality of either enteric smooth muscle (visceral myopathy)57or the intrinsic entericnervoussystem(visceralneuro- pathy).3 8 9In some patients the gut appears histologicallynormalorthechangesare “indeterminate”.10Some patients with a pri- mary visceral myopathy have involvement of other visc
14、eral smooth muscle, particularly the urinary tract and gall bladder.11 12 The literature about primary CIIP consists mainly of case reports of diVerent clinical syn- dromes and underlying pathologies, involving individuals4 13orfamilieswithvisceral myopathy,7 1428and less commonly, visceral neuropat
15、hy.9 2932Althoughdataaboutthe spectrum of this condition in children have been reported,33 34a perspective about the clinical spectrum of disease in adults is lacking. Two groups have reported on some of these aspects of CIIP in adults. Stanghellini et al studied 42 patients, in whom intestinal path
16、ol- ogy was available in 15 only.35Fifty seven per cent had abnormal radiology. Many of their patients were diagnosed as having CIIP on the basis of symptoms and manometry alone. SchuZer et al studied 27 patients with chronicGut 1997; 41: 675681675St Marks Hospital, Northwick Park, Watford Road, Harrow, Middlesex HA1 3UJ, UK S D Mann H S Debinski M A KammCorrespondence to: Dr M A Kamm.Accepted for publication 10 June on December 25, 2012 - Published by Do
