chapter  24
16 Pages

- Insulin deficiency and cystic fibrosis–related diabetes

ByChristopher Sheldon, Rachel Rowe

Diabetes mellitus was rst described as a complication of cystic brosis (CF) in 1955.1 Improving survival of CF patients has resulted in the emergence of cystic brosisrelated diabetes (CFRD) as a signicant complication. Substantial evidence now conrms the negative clinical impact of declining insulin secretion and the eventual development of CFRD. Loss of pancreatic function is usually rst related to exocrine function, followed by deteriorating endocrine function some years later. Acute or chronic infection contributes to variable resistance to the eects of insulin. e combination of gradual onset insulin deciency combined with variable insulin resistance is characteristic of CFRD. e deteriorating clinical status seen before the diagnosis of CFRD has drawn attention away from the direct eects of blood glucose toward the potentially more important and widespread eects of insulin deciency, the fundamental pathophysiological process underlying CFRD.