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Background Fibrosing colonopathy has been reported in young children with cystic fibrosis, the majority of whom take high-strength pancreatic-enzyme supplements to control intestinal malabsorption. We conducted a case–control study in the United States to investigate the relation between dose and type of pancreatic-enzyme supplement and fibrosing colonopathy.
Methods Children with histopathologically confirmed cases of fibrosing colonopathy who required colectomy for colonic strictures from January 1, 1990, through December 31, 1994, were identified. Each of these patients was matched according to age at the time of surgery and medical center with up to four controls with cystic fibrosis who did not have fibrosing colonopathy.
Results We studied 29 patients (mean age, 5.0 years) with fibrosing colonopathy (case patients) and 105 controls (mean age, 5.2 years). The mean dose of pancreatic-enzyme supplement was 50,046 units of lipase per kilogram of body weight per day for the case patients and 18,985 units per kilogram per day for the controls. A history of gastrointestinal complications attributed to cystic fibrosis and the use of histamine H2-receptor blockers, corticosteroids, or recombinant human DNase (dornase alfa) were associated with a higher incidence of fibrosing colonopathy. After adjustment for a history of such complications and the use of these medicines, the relative risk of fibrosing colonopathy that was associated with a dose of 24,001 to 50,000 units of lipase per kilogram per day, as compared with a dose of 0 to 24,000 units per kilogram per day, was 10.9 (95 percent confidence interval, 1.6 to 71.8), and that associated with a dose of more than 50,000 units per kilogram per day was 199.5 (95 percent confidence interval, 9.9 to 4026.0). The strength, coating, and manufacturer of the products used were not associated with the risk of fibrosing colonopathy.
Conclusions In young children with cystic fibrosis, we found a strong relation between high daily doses of pancreatic-enzyme supplements and the development of fibrosing colonopathy. Our findings support recommendations that the daily dose of pancreatic enzymes for most patients should remain below 10,000 units of lipase per kilogram.
Source Information
From the Medical Department, Cystic Fibrosis Foundation, Bethesda, Md. (S.C.F.); the Center for Drug Evaluation and Research, Food and Drug Administration, Rockville, Md. (G.A.B., T.H.); the Department of Pediatric Pulmonology, Children's Hospital of Buffalo, Buffalo, N.Y. (D.B.); the Division of Pediatric Gastroenterology and Nutrition, Tufts University School of Medicine, Boston (R.J.G.); the Division of Pediatric Gastroenterology and Nutrition, Hospital for Sick Children, Toronto (P.R.D.); the Department of Pediatrics, Rush Medical College, Chicago (J.D.L.-S.); and the Department of Surgery and Community and Preventive Medicine, New York Medical College, Valhalla, and the European Institute of Oncology, Milan, Italy (A.B.L.).
Address reprint requests to Dr. FitzSimmons at the Cystic Fibrosis Foundation, 6931 Arlington Rd., Bethesda, MD 20814.
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