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Query: UMLS:C0021843 (bowel obstruction)
 9,927 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The diagnosis of cystic fibrosis in utero is based on amniotic fluid analysis. False negative results of this method underline the importance of antenatal ultrasonography to detect intestinal obstruction in affected fetuses.
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PMID:Intestinal obstruction and cystic fibrosis: antenatal ultrasound appearance. 687 18

Meconium Ileus Equivalent is a condition which occurs in patients with cystic fibrosis where the ileum becomes obstructed by bowel content of abnormal consistency. Intercurrent chest infection or absence of pancreatic extract supplements are often the exacerbating cause. Plain films of the abdomen show small bowel obstruction and in some a nodular pattern in the small bowel, the nodules being larger than those seen in quiescent cystic fibrosis. Gastrografin enemas and/or enemas with acetyl cysteine can be used in the treatment of the condition, the Gastrografin having the advantage of confirming the diagnosis radiologically. The condition is important as it can be the presenting feature of cystic fibrosis in childhood and even in early adult life, and the operative mortality and morbidity are high. Conservative treatment should be pursued vigorously and operation only undertaken if unavoidable.
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PMID:The radiology of Meconium Ileus Equivalent. 726 May 9

Two neonates with clinical features of meconium plug syndrome (MPS) were found to have ileocolic intussusception associated with meconium plugs inspissated in the distal ileum. Hydrostatic reduction of the intussusception relieved the intestinal obstruction. Cystic fibrosis was excluded by sweat test and follow-up.
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PMID:Meconium plug syndrome associated with neonatal intussusception. 726 1

The number of neonates with functional intestinal obstruction being admitted to this surgical unit is increasing. The obstruction may be transient and curable within a few days, or weeks or, more rarely, may be prolonged and intransigent, usually resulting in death despite parenteral nutrition. In the former group, the infants are often the result of an abnormal pregnancy or delivery. Recently, a small number of infants has been identified in whom prolonged jaundice is a notable feature, but the aetiology of this syndrome is not apparent. All of these infants must be screened to exclude mucoviscidosis, septicaemia, meningitis, and Hirschsprung's disease. Ideally, neonates suspected of having Hirschsprung's disease should not be submitted to surgery until after histological proof of the diagnosis. Infants with the rarer forms of intransigent ileus should be referred to special centres for intensive investigations, much of which would be somewhat experimental, in order to discover more about the aetiology of the various syndromes. For the adequate investigation and treatment of any these infants, an experienced team of clinicians, radiologists and pathologists is essential, and these infants should not be treated where the necessary facilities are unavailable.
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PMID:Functional intestinal obstruction in the neonate. 734 79

Meconium ileus equivalent is an unusual cause of intestinal obstruction in adults. In this paper we report of our experience with a 29-year-old male with a long-standing history of cystic fibrosis and recurrent abdominal pain. Following barium examination of the stomach and small bowel, the patient developed increasing abdominal pain and evidence of meconium ileus equivalent as the etiology of his small bowel obstruction. The obstruction was relieved by administration of a 20% sodium diatrizoate enema and oral saline cathartics. The clinical and radiographic findings of meconium ileus equivalent are reviewed, as is the use of water-soluble contrast agents in the management of this condition. The role of prior barium study in precipitating this condition is discussed.
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PMID:Meconium ileus equivalent: treatment with Hypaque enema. 738 74

We used a whole-gut perfusion technique to study subclinical gut inflammation in children with cystic fibrosis (18 elective tests, three lavages to treat distal intestinal obstruction syndrome); and in 12 control children with constipation or pre-colonoscopy. We assayed for haemoglobin, IgG, albumin, alpha-1-antitrypsin, granulocyte elastase, interleukin-1 beta (IL-1 beta) and IL-8 concentrations in whole-gut lavage fluid. Results for two children with distal intestinal obstruction syndrome, the only children in the series taking Nutrizym 22, were strikingly abnormal. This new test has revealed subclinical gut mucosal inflammation in a minority of CF children, for which distal intestinal obstruction syndrome, Nutrizym 22 treatment, or both, may be risk factors.
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PMID:Gut inflammation in children with cystic fibrosis on high-dose enzyme supplements. 856 82

To develop an animal model for cystic fibrosis (CF), targeted gene disruption in embryonic stem (ES) cells was used to generate a duplication of exon 3 (cftrm1Bay allele) of the mouse CF gene. ES cells containing this mutation were used to generate chimeric animals that transmitted the mutant allele through the germline. Homozygous mutant animals display a severe phenotype, with approximately 40% dying within 1 week from intestinal obstruction. RNAase protection analysis of the cftrm1Bay allele did not detect any normal mRNA (< 1-2% of wild-type) in mutant animals. Pathologic changes in the intestines from mutant mice included mucus accumulation in the crypts and intestinal lumen, dilatation of the bases of the crypts, enlargement of goblet cells, and the presence of concretions in the crypts or between the villi. Changes were also present in the mucosal glands of the pharynx and the minor sublingual glands, where dilatation of acini and accumulation of eosinophilic material were evident. Atrophy of acinar cells that may be secondary to nutritional deficiency and mild inflammation in the main pancreatic duct were present in the pancreas of mutant animals. No changes were noted in the lung, trachea, liver, or male reproductive tract of mutant animals, and mutant males were fertile. Homozygous mutant mice showed defects in cAMP-mediated ion transport both in ileum and in cultured fetal tracheal explants. Thus, an additional mouse model for CF has been generated that should prove useful for the understanding of the pathogenesis and the development of treatments for CF.
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PMID:A severe phenotype in mice with a duplication of exon 3 in the cystic fibrosis locus. 750 91

Cystic fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). A potential animal model of CF, the CFTR-/- mouse, has had limited utility because most mice die from intestinal obstruction during the first month of life. Human CFTR (hCFTR) was expressed in CFTR-/- mice under the control of the rat intestinal fatty acid-binding protein gene promoter. The mice survived and showed functional correction of ileal goblet cell and crypt cell hyperplasia and cyclic adenosine monophosphate-stimulated chloride secretion. These results support the concept that transfer of the hCFTR gene may be a useful strategy for correcting physiologic defects in patients with CF.
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PMID:Correction of lethal intestinal defect in a mouse model of cystic fibrosis by human CFTR. 752 88

Fibrotic strictures of the colon in association with colon-wall thickening on abdominal ultrasound have been described in children with cystic fibrosis. We determined the prevalence of colon-wall thickening in 99 children with cystic fibrosis (aged 6 months to 17 years, 45 male) and 38 healthy controls (aged 1 month to 39 years, 21 male). In controls, peristalsis was uniformly rapid and maximum wall thickness of the fully relaxed colon was 0.8 mm (median 0.6 mm). 24% of the patients had a colon-wall thickness of 1.5 mm or more (maximum 3.4 mm); 48% of children on high-strength pancreatin receiving more than 265 units/kg per day of protease had thickening of more than 1.5 mm compared with less than 12% of children on any other dose. Children on high-strength pancreatin were 5.2 (95% CI 1.2-21) times more likely to have colon-wall thickness of 1.5 mm or more than children taking less than 265 units/kg per day of protease in a standard-strength preparation. This risk rose to 10 (2.5-39) times more likely if laxatives were taken with a high-strength preparation. Lipase or amylase intake, age, sex, genotype, and other medical or drug history were not significantly associated with colon-wall thickening. 1 child required a hemicolectomy for bowel obstruction and another developed haemorrhagic colitis. In 17 children, 2 months after stopping high-strength, high protease, preparations and efforts to reduce enzyme intake, colon-wall thickness had regressed from a median 2.0 mm (range 1.0-3.4) to 1.8 mm (0.9-2.7) (p = 0.005). The use of high-strength pancreatin preparations in conjunction with a high protease intake probably causes thickening of the wall of the colon.
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PMID:Relation of thickening of colon wall to pancreatic-enzyme treatment in cystic fibrosis. 753 69

Many problems may arise within the gastrointestinal tract of patients with cystic fibrosis. We report a new cause of subacute intestinal obstruction due to a fibrotic stricture of the ascending colon in a child with cystic fibrosis. Treatment was with a right hemicolectomy. There has been no recurrence after nine months follow-up. Recent similar cases suggest that this new pathology is linked to the use of enteric-coated high-strength pancreatin microspheres.
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PMID:Colonic stricture in a boy with cystic fibrosis. 759 43


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