S. Kolouskova (Presenting)(1) , D. Zemkova (1), J. Bartosova (1) , M. Macek Jr. (2), Z. Sumnik (1) , J. Vavrinec (1), V. Vavrova (1)

(1) Pediatric Clinic, Charles University Prague-University Hospital Motol, Prague, Czech Republic, (2) Institute of Biology and Medical Genetics, Charles University Prague-University Hospital Motol, Prague, Czech Republic

Introduction: Screening of CF-related diabetes (CFRD), including the first phase insulin response in patients with impaired glucose tolerance (GT), identifies cases with severe insulinopenia (IP) prior to the onset of typical symptoms. Our aim was to elucidate the influence of severe IP in asymptomatic CF patients on their body composition, pulmonary function, including monitoring of their changes during treatment by small dosage of intermediate insulin.

Patients and methods: IP was assessed by i.v.GTT as a sum of insulin response in 1 and 3 min. <1 percentile in healthy controls (48 mIU/l). Body composition was assessed by anthropological parameters (height, weight, mid-arm circumference-S.D.s). Controls were sex/age matched to cases.

Results: IP was found in 31/144 CF patients (11.1-28.5; med-16.5 yr). IP patients were diagnosed significantly earlier (1.8±3.2 vs. 3.6±3.6; P=0.04) likely due to the higher prevalence of ‘severe' (Class I-III) mutations (P=0.04). In controls pancreatic insufficiency appeared later. We did not find any differences in sweat chloride concentrations, occurrence of MI and chronic bacterial infection. Body height and weight did not differ in both groups. Mid-arm circumference in IP-CF cases was significantly lower (-1.2±0.9 S.D. vs. controls -0.8±0.9 S.D.; P=0.02) and worsening of the mid-arm circumference 1 yr prior tothe diagnosis of IP (P=0.05) was observed. During the first year of insulin treatment nutritional status (NS) has improved (P<0.01) and pulmonary function tests (PFTs) became stable (FEV 72.4"18.4 vs. 71.9"19). However, in controls the 1NS did not change, but PFTs deteriorated (74.3±22.4 vs. 67.6±18.9; P= 0.006).

Conclusion: Early detection of IP by i.v.GTT and its early treatment improves NS, stabilizes PFTs and improves life expectancy.

Supported by IGA MZCR: 6464-3, 6461-3, 00000064203 and MSMT: LN00A079, 111300003.