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Query: UMLS:C0021933 (intussusception)
 3,822 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Abdominal pain, vomiting, and obstipation often occur in children and young adults with cystic fibrosis (CF). The common causes include meconium ileus equivalent, intussusception, and adhesions from previous surgery. One of our patients with CF who had meconium peritonitis as a neonate presented with duodenal obstruction during childhood. This was caused by colonic polyps arising in the hepatic flexure and eroding through the colonic and duodenal walls into the duodenum. She was treated with total parenteral nutrition, right colectomy, gastric diversion, and a controlled duodenal fistula that healed uneventfully. She has remained well 1 year after discharge from the hospital. To our knowledge, a similar case has not been reported previously.
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PMID:Colonic polyps and coloduodenal fistula: unusual complications in patient with cystic fibrosis. 724 43

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

Since the initial description of Munchausen Syndrome by Proxy (MSBP) (Meadow, 1977), numerous cases have been reported varying from as simple as the complaint of a nonexistent symptom to those as complicated as altered laboratory tests leading to the false diagnosis of cystic fibrosis (Orenstein & Wasserman, 1986; Rosenberg, 1987). We report three findings previously unreported: esophageal perforation, retrograde intussusception, and tooth loss. Bradycardia has been previously reported associated with suffocation (Meadow, 1984), but in our case may have been caused by carotid artery massage. We also suggest possible induction by the mother of premature rupture of fetal membrane leading to delivery of infected infants.
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PMID:Munchausen syndrome by proxy: unusual manifestations and disturbing sequelae. 883 12

Cystic fibrosis (CF), the most common lethal autosomal recessive disease in white populations, is characterized by dysfunctional chloride ion transport across epithelial surfaces. Although recurrent pulmonary infections and pulmonary insufficiency are the principal causes of morbidity and death, gastrointestinal symptoms commonly precede the pulmonary findings and may suggest the diagnosis in infants and young children. The protean gastrointestinal manifestations of CF result primarily from abnormally viscous luminal secretions within hollow viscera and the ducts of solid organs. Bowel obstruction may be present at birth due to meconium ileus or meconium plug syndrome. Complications of meconium ileus include volvulus, small bowel atresia, perforation, and meconium peritonitis with abdominal calcifications. Older children with CF may present with bowel obstruction due to distal intestinal obstruction syndrome or colonic stricture, and tenacious intestinal residue may serve as a lead point for intussusception or cause recurrent rectal prolapse. Radiologic studies often demonstrate thickened intestinal mucosal folds in older children and uncommonly show colonic pneumatosis, peptic esophageal stricture due to gastroesophageal reflux, and duodenal ulcer. Appendicitis due to inspissated secretions is uncommon. Obstruction of ducts and ductules produces exocrine pancreatic insufficiency, pancreatitis, cholestasis, cholelithiasis, and cirrhosis with portal hypertension. On imaging studies, the pancreas is commonly small and largely replaced by fat, sometimes displays calcifications, and is rarely replaced by macrocysts. Radiologic features of hepatobiliary disease include an enlarged radiolucent liver from steatosis, gallstones, a shrunken nodular liver, splenomegaly, and portosystemic collateral vessels. With the improved survival of CF patients, an increased risk for developing gastrointestinal carcinomas has been established, many occurring as early as the 3rd decade.
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PMID:Gastrointestinal manifestations of cystic fibrosis: radiologic-pathologic correlation. 883 77

CFTR, or cystic fibrosis transmembrane conductance regulator, the gene product that is defective in cystic fibrosis, is present in the apical membrane of the epithelial cells from the stomach to the colon. In the foregut, the clinical manifestations are not directly related to the primary defect of the CFTR chloride channel. The most troublesome complaints and symptoms originate from the oesophagus as peptic oesophagitis or oesophageal varices. In the small intestinal wall, the clinical expression of CF depends largely on the decreased secretion of fluid and chloride ions, the increased permeability of the paracellular space between adjacent enterocytes and the sticky mucous cover over the enterocytes. As a rule, the brush border enzyme activities are normal and there is some enhanced active transport as shown for glucose and alanine. The results of continuous enteral feeding of CF patients clearly show that the small intestinal mucosa, in the daily situation, is not functioning at maximal capacity. Although CFTR expression in the colon is lower, the large intestine may be the site of several serious complications such as rectal prolapse, meconium ileus equivalent, intussusception, volvulus and silent appendicitis. In recent years colonic strictures, after the use of high-dose pancreatic enzymes, are being increasingly reported; the condition has recently been called CF fibrosing colonopathy. The CF gastrointestinal content itself differs mainly from the normal condition by the lower acidity in the foregut and the accretion of mucins and proteins, eventually resulting in intestinal obstruction, in the ileum and colon. Better understanding of the CF gastrointestinal phenotype may contribute to improvement of the overall wellbeing of these patients.
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PMID:Gastrointestinal manifestations in cystic fibrosis. 886 67

A right iliac fossa mass may be a difficult diagnostic problem in a patient with cystic fibrosis. We present a patient with such a mass who was thought to have a non-obstructing intussusception on clinical and radiological grounds. However, at laparotomy she was found to have a pathology not previously described in cystic fibrosis. She had a partial diaphragm almost blocking the lumen to her appendix. The differential diagnosis of a right iliac fossa mass is considered and the cause of her pathology discussed.
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PMID:A caecal diaphragm in cystic fibrosis. 922 42

Children and adolescents with cystic fibrosis (CF) may manifest bowel pathology with resulting bowel obstruction. Recognized causes of bowel obstruction in CF patients include meconium ileus, intussusception, distal intestinal obstruction syndrome and postoperative adhesions. Additionally, the development of colonic strictures in children with CF has recently been described. We report an unusual cause of partial obstruction of the ascending colon in a child with CF due to pathologically proven diverticulitis.
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PMID:Colonic diverticulitis causing partial bowel obstruction in a child with cystic fibrosis. 938 82

Cystic fibrosis (CF) is the most common autosomal recessive disease in the Caucasian population. The primary cellular defect, the reduced expression of the cystic fibrosis transmembrane conductance regulator (CFTR), leading to a chloride secretory defect, is present in all epithelial cells of endodermal and mesodermal origin and has been described in sweat glands, the airway epithelium and the small intestine, the colon and rectum, including the pancreas. In the upper GI-tract the most troublesome complaints and symptoms originating from the esophagus are peptic esophagitis or esophageal varices. In the small intestinal wall, the clinical expression of CF largely depends on the decreased secretion of fluid and chloride ions and the sticky mucous covering the enterocytes. Although CFTR expression in the colon is lower, the large intestine may be the site of several serious complications such as rectal prolapse, distal intestinal obstruction (DIOS), and intussusception. In recent years an increase in colonic strictures after the use of high-dose pancreatic enzymes, has been increasingly reported. CF has also been reported to be increasingly associated with a number of hepatic and/or biliary abnormalities, of which chronic cholestatic liver disease is by far the most relevant. Plugging of intrahepatic bile ducts with inspissated secretions is thought to play a major role in the pathogenesis. It has been estimated that about 15% of cystic fibrosis patients reveal serum liver enzyme abnormalities, but prevalence of liver involvement is likely to be higher. Oral bile acid therapy is promising, but its long-term benefits in terms of survival and prevention of major complications by liver cirrhosis remain to be established. Pancreatic dysfunction in cystic fibrosis (CF) is characterized by an insufficient pancreatic exocrine function. However, 10-15% of CF patients have pancreatic sufficiency and this status is genetically determined by one or two "mild" mutations in the CFTR.
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PMID:[Gastrointestinal complications of adult patients with cystic fibrosis]. 1049 10

Search of the autopsy files of the Department of Histopathology at the Women's and Children's Hospital (WCH), Adelaide, Australia for cases of intussusception from January 1961 to December 1995 revealed two deaths due to intussusception out of a total of 4,384 autopsies (0.05%). Both cases occurred in infants (aged five months and six months respectively) who had only non-specific and apparently minor manifestations of illness, until precipitate deterioration occurred. The intussusceptions were ileo-ileal and ileocaecal in location, respectively. A total of 204 cases of intussusception were found in a search of 28,123 surgical pathology cases (0.73%) at the WCH over the 20-year period from 1976 to 1995. Mesenteric lymphadenopathy was found in 16 cases (including one with possible Yersinia infection), Meckels diverticulum in 14, isolated gastric/pancreatic heterotopia in two, cystic fibrosis in two and Henoch-Schonlein purpura in one. Although it has been shown that unexpected death is more likely to occur in older children with purely small intestinal intussusception, the current cases demonstrate that unexpected death may occur at any age, with intussusception at any level. The autopsy assessment of deaths due to intussusception requires careful evaluation of cases for evidence of local or systemic disease, particularly given the hereditary nature of certain predisposing conditions. Careful review of the presenting history is also required to assess the quality of care received by the child in the time preceding death.
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PMID:Sudden death and intussusception in infancy and childhood--autopsy considerations. 1121 22

By 1996, the median survival of patients with cystic fibrosis (CF) in North America had increased to 31 years. With the markedly improved life expectancy, many CF patients are now adults. There is an associated increased risk of certain colonic disorders, and the emergence of other previously unrecognized disorders, in adult CF patients. The distal intestinal obstruction syndrome (DIOS), which is more common in older patients, is a frequent cause of abdominal pain. Intussusception may complicate DIOS; other differential diagnoses include appendiceal disease, volvolus, Crohn's disease, fibrosing colonopathy and colonic carcinoma. The diagnosis of acute appendicitis, although uncommon in patients with CF, is often delayed, and appendiceal abscess is a frequent complication. The prevalence of Crohn's disease in CF has been shown to be 17 times that of the general population. Right-sided microscopic colitis is a recently recognized entity in CF of uncertain clinical significance. Fibrosing colonopathy has been confined mostly to children with CF, attributed to the use of high strength pancreatic enzyme supplements, but it has been reported in three adults. Nine cases of carcinoma of the large intestine have been reported worldwide, associated with an apparent excess risk of digestive tract cancers in CF. Despite high carrier rates of Clostridium difficile in patients with CF, pseudomembranous colitis is distinctly rare, but severe cases complicated by toxic megacolon have been reported. In these patients, watery diarrhea is often absent. Adult CF patients with refractory or unexplained intestinal symptoms merit thorough investigations.
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PMID:Colonic disorders in adult cystic fibrosis. 1157 1


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