Influence of Pre-Diabetic and Pancreatic Exocrine States on Pulmonary and Nutritional Status in Adults with Cystic Fibrosis.

Background: In 1992, a landmark study demonstrated clinical deterioration in respiratory function and nutritional status prior to the onset of cystic fibrosis-related diabetes (CFRD). We re-evaluated this outcome. Methods: The Montreal Cystic Fibrosis Cohort is a prospective CFRD screening study. We performed a 6-year retrospective analysis of nutritional parameters and FEV1 (%) in subjects who developed incident CFRD and in controls who maintained normoglycemia (NG). In the former group, data was collected over 6 years prior to diabetes onset. Results: Subjects (n = 86) had a mean age of 31.7 +/- 8.1 years, BMI of 23.0 +/- 4.0 kg/m(2), and FEV1% of 70.1 +/- 24.2%. Eighty-one percent had pancreatic insufficiency (PI). Patients were grouped as follows: NG+PS (pancreatic sufficient) (n = 16), NG+PI (pancreatic insufficient) (n = 21), CFRD+PS (n = 3) and CFRD+ PI (n = 46). At their most recent screen NG+PS subjects had significantly greater BMI, as compared to NG+PI and CFRD+ PI groups (26.2 +/- 3.6 kg/m(2) vs 22.6 +/- 4.2 kg/m(2) vs 22.1 +/- 3.5 kg/m(2), p = 0.0016). FEV 1 was significantly greater in the NG+PS group (91.5 +/- 16.8% vs 67.8 +/- 25.3% vs 63.5 +/- 22.2%, p = 0.0002). The rates of change in weight, BMI, fat mass (%), and FEV1 prior to the most recent visit (NG+PS, NG+PI groups) or to the diagnosis of de novo CFRD were similar between groups. Conclusion: In a contemporary context, CFRD onset is not preceded by deterioration in BMI, fat mass, or pulmonary function. Low BMI and FEV1 are more closely associated with PI than a pre-diabetic state. (C) 2020 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.