CYSTIC FIBROSIS-ASSOCIATED COLITIS AND FIBROSING COLONOPATHY

Objective: To describe our experience with cystic fibrosis (CF)-associ ated colitis and fibrosing colonopathy, and to assess treatment strate gies. Study design: We reviewed hospital charts and autopsy reports of all University of Minnesota patients with CF between 1975 and August 1994. We identified six patients with colonopathy and compared them wi th a cohort of 79 patients with CF in the same age range and seen duri ng the same period, Results: All patients with colonopathy had bloody diarrhea; five of the six had abdominal pain. Stool frequency and rela ted symptoms distinguished the patients with colonopathy from the coho rt population, All took a higher median dose of pancreatic enzymes tha n the cohort population during the 3 months preceding the onset of sym ptoms (p < 0.002). For all six patients, barium studies revealed loss of haustration, and shortening and diffuse narrowing of the colonic lu men with relative rectal sparing, The distal ileal mucosa was irregula r in four patients, A histopathologic study reveal fibrosis of the sub mucosa or lamina propria, and focal acute cryptitis in all six patient s. Other features included ascites (2/6) and nodular regenerative hype rplasia of the liver (1/6), One patient continues to have symptoms, th ree had subtotal colectomy, and the condition of two improved after a regimen including a low-felt diet, withholding of pancreatic enzymes, and supplemental parenteral nutrition was initiated, Conclusions: Fibr osing colonopathy represents a newly recognized gastrointestinal compl ication of cystic fibrosis, Affected persons have taken larger doses o f pancreatic enzymes than similar patients with cystic fibrosis, and h ave bloody diarrhea, We developed a medical protocol that may avoid su rgical resection of the colon in some of these patients.