Cystic fibrosis colonopathy

Abstract

An epidemic of fibrosing colonopathy, a new disease caused by the prolonged administration of excessive doses of pancreatic enzymes, was first reported in 1994. More than 60 cases were known to occur worldwide before dosage guidelines were enforced. Predisposing factors were young age, previous intestinal surgery, meconium ileus equivalent, and use of H2 blockers, corticosteroids, and DNase. Abnormal features included foreshortened colon, strictures, marked submucosal fibrosis, ascites, and nodular hyperplasia of the liver. Histologic examination showed eosinophilia, mild cryptitis, epithelial regeneration, and widespread interruption of the muscularis mucosa. These findings are distinct from, but share many of the features of, those of Crohn's's disease and ischemic bowel disease. The pathogenic mechanisms remain unknown.