Pancreatic insufficiency leading to malabsorption and failure to thrive is a common mode of presentation of cystic fibrosis.
The recent isolation of the cystic fibrosis gene has shown that pancreatic insufficiency is associated with certain mutations and not with others - the phenylalanine 508 deletion mutant is frequently associated with pancreatic insufficiency.
Treatment is with pancreatic enzyme supplements, and efforts should be made to ensure high calorie and protein levels in the diet, together with added vitamins.
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