The diffuse endocrine system in cystic fibrosis

The pathology, aetiology and clinical features of cystic fibrosis (CF) are briefly reviewed. The diffuse endocrine system (DES) may be involved in either a primary or secondary manner. Carbohydrate intolerance is common and the release of islet hormones is deficient, as is the release of the intestinal hormone gastric inhibitory polypeptide (GIP). The post-prandial responses of insulin and GIP can be improved by substituting an elemental meal, suggesting that malabsorption and local intestinal factors could be causative in the deficient responses. Vasoactive intestinal polypeptide-like immunoreactive (VIP-LI) cell numbers are increased in CF and an intimate association with mucous cells is noted suggesting a paracrine relationship. These findings could have implications in the diagnosis, management and aetiology of CF.