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.

The fatty acid compositions of serum phospholipids, cholesteryl esters, triglycerides, and free fatty acids were determined on a group of cystic fibrosis patients. These were compared with similar data from random hospitalized patients of the same age groups of both sexes. Fatty acid patterns in all lipid classes were skewed in the direction of essential fatty acid deficiency, but the differences were most dramatic in phospholipids. Many calculated parameters useful as indices of essential fatty acid status indicated that essential fatty acid deficiency exists in cystic fibrosis. Treatment of 11 cystic fibrosis patients with safflower oil (1 g/kg/day) failed to correct the aberrations in fatty acid pattern. The biochemical data suggest that there may be an impairment in conversion of linoleate to arachidonate as well as an impairment of absorption.

Advances in investigative techniques have led to increasing reports of Crohn's disease in CF patients. A retrospective review of the literature on IBD in CF showed findings characterized by ileocolitis with fistula formation; 83% required surgery. A prospective survey of 11,321 CF patients attending 49 CF centers revealed 28 with IBD (25 Crohn's, three ulcerative colitis), ages 4-20 years, mean 15.6 years. The prevalence rate of IBD (247/10(5)) was 7x controls and was accounted for by Crohn's disease (221/10(5)) which was 17x controls. The mechanisms that predispose CF patients to Crohn's disease are discussed.