Diabetes Linked With Complications and Death in Cystic Fibrosis

In patients with cystic fibrosis, diabetes is common early in life and is linked with impaired nutrition and growth, increased rates of terminal respiratory failure, and death, French researchers report.

As senior investigator Dr. Jean-Jacques Robert of Hopital Necker – Enfants Malades, stated,  "The longitudinal follow-up of a large population of cystic fibrosis patients strongly confirms the high rate of diabetes mellitus early in life — 40% by 30 years of age — and its major impact on the deterioration of pulmonary function and survival."

In an analysis of 237 patients with cystic fibrosis followed between 1988 and 2005, Dr. Robert and his team found that 20% had impaired glucose tolerance at 10 years of age, and 82% had impaired glucose tolerance by age 32. Diabetes was present in more than 20% at age 15, 45% at age 20, and 70% at age 30. Thirty percent were on insulin by 20 years of age; this was true of 40% by age 30.

Regardless of gender, survival was significantly lower, and the rate of lung transplantation was significantly higher, in patients who developed impaired glucose tolerance before age 15.

Given the effect on pulmonary function and survival, Dr. Robert advises, "these observations fully justify the regular screening of abnormalities of glucose tolerance — Oral Glucose Tolerance Test — in these patients and the careful evaluation… of their early treatment." This approach, he added, is currently taken in Germany, Italy and France.

J Pediatr 2008;152:540-545.

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