Glucose Derangements in Very Young Children With Cystic Fibrosis and Pancreatic Insufficiency

ISPAD Clinical Practice Consensus Guidelines 2018: Management of cystic fibrosis-related diabetes in children and adolescents

“…While diabetes per se is rare before age 10, 42% to 78% of children aged 9 years and under are reported to have abnormal glucose tolerance. [78][79][80] A prospective longitudinal study at one North American CF center found that in children aged 6 to 9 years, IGT or indeterminate glycemia each predicted a high risk of progression to diabetes in the early adolescent years. 80 For this reason, some centers chose to begin screening at age 6 years.…”

Update on cystic fibrosis-related diabetes

“…Data from the University of Minnesota where annual OGTT screening is recommended for all patients ≥ 6 years, find CFRD affects 2% of children, 19% of adolescents, and 40-50% of adults [50]. A study that included 24 young Italian children (age 2-5.9 years) with CF found 33% had varying degrees of glucose intolerance ranging from Indeterminate-GT to CFRD [77]. A 15 year study of 775 patients ≥ 6 years identified CFRD diagnosed by OGTT in over a third [59].…”

“…With newer data identifying a nutritional benefit, treatment of CFRD without hyperglycemia is now recommended. The role of treating pre-diabetes is now receiving increasing attention [53,54,77]. The CFRD treatment of choice is insulin.…”

Abnormal Glucose Tolerance in Infants and Young Children with Cystic Fibrosis

“…There is a paucity of data regarding the prevalence of AGT among children with CF younger than 6 years of age (8). However, glucose tolerance has been studied in animal models of CF at very young ages, including CFTR null and deltaF508 pigs and CFTR null ferrets.…”