Intussusception in cystic fibrosis

Article Abstract:

Intussusception occurs when one portion of the intestine folds inward into another portion of the intestine. This can block the intestines and cause abdominal pain and swelling, vomiting, and constipation. It is not uncommon in infants and it can be corrected with surgery. Intussusception has also been reported to occur in 1 percent of children with cystic fibrosis. Cystic fibrosis is an inherited disease in which large amounts of thick mucus plug up the intestines, pancreas and lungs, and a dense, thick tissue similar to scar tissue develops in the pancreas and lungs. The cases of two children with cystic fibrosis and intussusception are described. Both patients had symptoms of abdominal pain and constipation and were diagnosed as having obstructed or blocked intestines (meconium ileus). Ultrasound and X-rays of the abdomen showed that the intestines were blocked. The patients were treated with gastrografin or acetylcysteine and these drugs were effective in emptying the intestines of fecal material by increasing the daily number of loose stools. However, the symptoms did not disappear and additional X-rays and ultrasound imaging were performed. These tests showed that the intestines were still blocked and surgery (laparotomy) was performed to removed the obstruction. During the surgery it was discovered that the block was caused by intussusception, and the portion of the intestine that was folded inward was removed and the remaining portions of intestine were rejoined. It is concluded that intussusception can occur in patients with cystic fibrosis and that it is difficult to diagnose. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Pediatric diseases, Intussusception in children, Intussusception

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Fluorescein dilaurate test of exocrine pancreatic function in cystic fibrosis

Article Abstract:

Cystic fibrosis (CF) is the most common inherited disease among Caucasians. It is characterized by chronic respiratory infections, insufficient excretion of digestive enzymes by the pancreas, and increased amounts of salts in sweat. Frequently, the ability of the pancreas to excrete digestive enzymes must be evaluated, but the commonly used method, in which a tube is inserted into the digestive tract, is unpleasant and difficult for children. Pancreatic function has been assessed in adults by the fluorescein dilaurate test, in which a fluorescent dye is ingested and its concentration in urine, collected over 10 hours, is measured. Previous efforts to use this test in children have been difficult to implement. However, the test was adapted by reducing the concentration of the dye by half, and was used to measure pancreatic function in 20 children with CF and 12 of their healthy siblings. The fluorescein dilaurate test discriminated well between the two groups of children, suggesting that, adapted in this fashion, it may provide an effective measure of pancreatic function in children with CF. Further testing with larger groups of children and children with only moderate impairment of pancreatic function is needed to establish the full range of normal and abnormal results. (Consumer Summary produced by Reliance Medical Information, Inc.)

Diagnosis, Testing, Medical examination, Physiological aspects, Children, Pancreas, Pancreatic insufficiency

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Pathology mimicking distal intestinal obstruction syndrome in cystic fibrosis

Article Abstract:

Although respiratory problems are the hallmark of cystic fibrosis, the genetic disease causes defects in many organ systems. Distal intestinal obstruction syndrome occurs in about 15 percent of patients with cystic fibrosis, and involves the accumulation of thickened and partially dried material near the end of the small intestine. Gastrointestinal lavage relieves the problem, but only temporarily. The relative frequency of this disorder may, however, lead to its overdiagnosis among patients with cystic fibrosis. This concern is illustrated by eight cases in which distal intestinal obstruction was initially diagnosed. Six patients were found to have other abdominal pathologies which accounted for their symptoms; the diagnoses included Crohn's disease, an appendix abscess, and a small bowel fistula. It is important for the physician not to rush to a diagnosis of distal intestinal obstruction in cystic fibrosis patients who have abdominal pain. Care must be taken to rule out alternative diagnoses, and close cooperation with the radiologist is necessary. The correct diagnosis is important as inappropriate treatment will perpetuate misery for patients already afflicted with an incurable illness. (Consumer Summary produced by Reliance Medical Information, Inc.)

Intestinal obstruction

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