Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042961 (volvulus)
 4,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Four horses were evaluated for clinical signs consistent with small intestinal disease. During exploratory surgery, primary hematomas in the mesentery were found in 2 horses. The third horse developed a hematoma secondary to a mesenteric rent, whereas in the fourth horse, a hematoma developed secondary to strangulation of the bowel by a mesenteric lipoma. Two horses were euthanatized at surgery, because the extensive nature of the hematoma precluded ligation and there was substantial risk of continued hemorrhage postoperatively. The remaining horses recovered from surgery but developed complications. One horse developed functional ileus and required a second surgery to decompress the bowel, whereas the second horse eventually was euthanatized for severe, recurrent signs of colic. Clinical signs and management of small intestinal lesions related to volvulus, epiploic entrapment, intramural hematoma, mesenteric rent, and intussuception have been described. To our knowledge, reports of small intestinal infarctions secondary to hematomas in the mesentery have not been published. Primary hematomas in the mesentery should be included in the differential diagnoses for horses examined for signs of depression, anorexia, and progressive signs of colic.
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PMID:Acute small intestinal injury associated with hematomas in the mesentery of four horses. 887 Jul 45

A retrospective review of 84 cases of intestinal obstruction admitted to the National Guard Hospital over a period of 10 years was carried out. The main causes of obstruction were: post-operative adhesions, 38 patients (45%); hernia, 17 (20%); pseudo-obstruction, eight (9.5%); intussusception, six (7%); malignant obstruction, four (4.8%); inflammatory obstruction, three (3.6%); volvulus, three (3.6%); and others, five (6%). Large bowel obstruction occurred in only 16 patients (19%). Surgical intervention was necessary in 61 patients (73%) while 23 patients (27%) responded to conservative treatment. Post-operative complications occurred in 14 patients (17%). The main complications were: wound infection, chest infection, prolonged ileus and intestinal fistulae. The mortality rate was 3.5%. The pattern of small bowel obstruction in Saudi Arabia is similar to that in the West, while large bowel obstruction is rather uncommon.
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PMID:Causes and management of intestinal obstruction in a Saudi Arabian hospital. 904 38

Fifteen cases of meconium ileus (MI) were treated between 1986 and 1995; 7 responded to conservative treatment. Eight were operated upon, and comprise the study group. Six of the operated babies had no complications; 1 had meconium peritonitis with a pseudocyst and small-bowel atresia, and 1 had a volvulus of a small-bowel segment with necrosis. In all 8 cases a T-tube (TT) was left via an enterotomy; in the complicated cases the enterotomy was pre-anastomotic. The obstruction was relieved in all the babies, without any stoma or bowel resection in the uncomplicated cases. Two complications occurred: 1 patient died of respiratory failure 1 month following surgery and another required a relaparotomy for lysis of adhesions. We conclude that TT ileostomy is an effective and safe procedure for uncomplicated cases of MI that do not respond to conservative therapy, as well as for complicated cases that need an anastomosis.
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PMID:T-tubes in the management of meconium ileus 906 16

Fifteen cases of meconium ileus (MI) were treated between 1986 and 1995; 7 responded to conservative treatment. Eight were operated upon, and comprise the study group. Six of the operated babies had no complications; 1 had meconium peritonitis with a pseudocyst and small-bowel atresia, and 1 had a volvulus of a small-bowel segment with necrosis. In all 8 cases a T-tube (TT) was left via an enterotomy; in the complicated cases the enterotomy was pre-anastomotic. The obstruction was relieved in all the babies, without any stoma or bowel resection in the uncomplicated cases. Two complications occurred: 1 patient died of respiratory failure 1 month following surgery and another required a relaparotomy for lysis of adhesions. We conclude that TT ileostomy is an effective and safe procedure for uncomplicated cases of MI that do not respond to conservative therapy, as well as for complicated cases that need an anastomosis.
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PMID:T-tubes in the management of meconium ileus. 915 41

A 45-year-old woman was incidentally suspected to have megacolon. Chest X-rays showed elevated left diaphragm due to colonic gas, and the heart was deviated to the midline. Barium enema revealed marked dilation of the sigmoid colon, confirming the diagnosis of megacolon. Maximal diameter of the sigmoid colon was 23 cm, but she had no gastrointestinal symptoms. During the work up for megacolon, the presence of myotonic dystrophy was suspected. She had hatchet face, but was not bald. Muscles of the neck and extremities were slightly atrophic. There was percussion myotonia of the tongue and both hands, and grip myotonia of the hands. Laboratory examinations showed impaired glucose tolerance and low level of serum IgG. EMG showed myotonic discharges and myopathic units in the limbs. Brain CT imaging revealed a thick skull. Cases of myotonic dystrophy associated with marked megacolon are rare in Japan. Megacolon presents a high risk for ileus, volvulus, and rupture, and myotonic dystrophy is associated with a high operative and anesthesic risk. Megacolon, therefore, is an important complication to look for in the management of myotonic dystrophy.
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PMID:[Myotonic dystrophy with marked megacolon: report of a case]. 939 35

Of 36 neonates with meconium ileus secondary to cystic fibrosis treated over a 10-year period, twenty-one (58%) had simple uncomplicated disease while fifteen (42%) had complications which included perforation (5), volvulus (6) and atresia (5). Gastrografin enema was employed in 20 infants with relief of obstruction in 8 (40%). Operative procedures consisted of resection and primary anastomosis in seventeen patients, stomas were fashioned in six, three had an enterotomy with irrigation only and two had Bishop-Koop enterostomy. Post-operative complications developed in 5 (18%) of these 28 patients. The overall survival rate was 97%. The one death occurred in an infant with short bowel syndrome, patent ductus arteriosus, hydrocephalus and pulmonary damage. There were eight additional patients who had meconium obstruction in the absence of cystic fibrosis.
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PMID:Meconium ileus: a ten-year review of thirty-six patients. 940 84

Between June 1, 1990 and December 31, 1996, 58 consecutive patients with unprepared colons were urgently explored for nontraumatic disease with intent to proceed with primary left-sided colonic anastomosis. Unprotected anastomoses were not attempted in 15 patients. The causes of exclusion included preoperative and intraoperative shock in three patients, and three patients were on long-term high-dose steroids, four had gross fecal contamination of the peritoneal cavity, four had large pelvic abscesses, and one had ischemic colitis. All 43 patients undergoing anastomosis without protective colostomy had stapled anastomoses. Indications included complicated diverticular disease in 32 cases. There were nine cases of obstruction from colorectal carcinoma and one obstruction due to sigmoid volvulus. There was one case of perforation from pseudomembranous enterocolitis. The most common complications were: atelectasis in nine cases, wound infection in two cases, and prolonged ileus in two cases. Pelvic abscess occurred in one case. There was one wound dehiscence. There was one anastomotic dehiscence, and there was no mortality. Operative time averaged 85 minutes and hospital length of stay 9.7 days. Primary anastomosis of the unprepared left colon is safe in most urgent and emergent situations, thus avoiding the significant morbidity and cost of colostomy closure.
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PMID:Primary anastomosis in the treatment of acute disease of the unprepared left colon. 973 7

Volvulus of the small intestine is a condition of bowel obstruction due to knotting and twisting of the small intestine. Two types of volvulus are described: 1) primary small intestinal volvulus where no predisposing factors exist, and 2) secondary volvulus where congenital or acquired conditions promote twisting of the small intestine. Over a 5-year period, 18 patients (eleven men and seven women) presenting volvulus of the small intestine are operated in the Emergency Surgery Clinic of the University Hospital "Queen Giovanna", representing 8.7 per cent of the total of 206 cases of small intestinal mechanical ileus (incarcerated herniations involving the small intestine are not included in the series). Primary volvulus is found in one patient. In those presenting secondary volvulus adhesions are the commonest underlying cause of small intestinal rotation--13 cases, next ranking primary tumor of the small intestine--one case, Meckel's diverticulum--one, carcinosis of peritoneum--one, and one patient with small intestine volvulation around colostomy. The most frequently encountered symptoms and laboratory examinations performed are analyzed. Intestinal necrosis is established in four instances (22 per cent). One patient dies of peritonitis and polyorganic insufficiency. Volvulus of the small intestine should be mandatorily considered in patients presenting mechanical ileus of the small intestine. Early operative intervention is a therapeutic approach contributing to preclude intestinal necrosis.
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PMID:[Volvulus of the small intestine]. 973 71


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