Diabetes Related in Children with Cystic Fibrosis
Norfolk Suffolk Cambridge Cystic Fibrosis Network Guideline.
The prevalence of CFRD rises with increasing age of survival with the median age of onset at 21years [range 3-40 years] 1. It is more prevalent in patients with exocrine pancreatic deficiency and as such can be correlated to genotype 20% of subjects with class I – III mutations vs 1.5% of subjects with class IV-V mutations 2. There is however no association with positive family history or HLA class II blood group gene3. CFRD is a distinct type of diabetes WHO/NCD/NCS/99.2 with features of both Type 1 and Type 2 diabetes. However there are important differences due to glucose metabolism in CF being affected by infective respiratory status increased basal metabolic rate malnutrition and gastrointestinal abnormalities