Endocrine Abstracts

Life expectancy has improved for all children and adolescents with cystic fibrosis (CACF); children now survive into adult life. Cystic fibrosis related diabetes (CFRD) is increasing^1–3. The combination of CF and diabetes has a negative impact on survival. From 2002 to date, CFRD has been related to decreased survival³ and survival gender differences are also described⁴. Patients with CFRD have a sixfold increase in morbidity and mortality³.

CFRD is usually asymptomatic and can remain undetected for up to 4 years prior to diagnosis. Recent International Society of Pediatric Diabetes Consensus Guidelines on CFRD highlight the use of continuous glucose monitoring (CGM) in the normal glucose tolerance (NGT) and impaired glucose tolerance (IGT) stages may be important in early diagnosis of CFRD⁵. CGM has also been validated for use in children and adolescents with CF⁶. Early insulin therapy in CF improves growth, lung function and reduces the number of chest infections⁷. Diabetes is now the most common morbidity in CF patients, some reports estimate 50–75% will be diagnosed with CFRD by the age of 30 years. Early detection and identification of abnormalities in glyceamia in CF is essential by all screening methods.

References:
1. Hardin DS & Moran A 1999 Diabetes mellitus in cystic fibrosis. Endocrinol Metab Clin North Am 28 787–800, ix.

2. Milla CE, Warwick WJ & Moran A 2000 Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline. Am J Respir Crit Care Med 162 891–895.

3. Moran A 2002 Diagnosis, screening, and management of cystic fibrosis-related diabetes. Curr Diab Rep 2 111–115.

4. Milla CE, Billings J & Moran A 2005 Diabetes is associated with dramatically decreased survival in female but not male subjects with cystic fibrosis. Diabetes Care 28 2141–2144.

5. O’Riordan SM, Robinson PD, Donaghue KC & Moran A 2008 Management of cystic fibrosis-related diabetes ISPAD Clinical Consesus Guidelines. Pediatr Diabetes 9 338–344.

6. O’Riordan SM, Hindmarsh P, Hill NR, Matthews DR, George S, Greally P, Canny G, Slattery D, Murphy N, Roche E, Costigan C & Hoey H 2009 Validation of continuous glucose monitoring in children and adolescents with cystic fibrosis: a prospective cohort study. Diabetes Care 32 1020–1022.

7. Lanng S, Thorsteinsson B, Nerup J & Koch C 1994 Diabetes mellitus in cystic fibrosis: effect of insulin therapy on lung function and infections. Acta Paediatr 83 849–853.