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Influence of pre-diabetic and pancreatic exocrine states on pulmonary and nutritional status in adults with Cystic Fibrosis
Institution:1. Montreal Clinical Research Institute (IRCM), Montréal, Québec, Canada;2. Department of Nutrition, Faculty of Medicine, Université de Montréal, Montréal, Québec, Canada;3. Cystic Fibrosis Clinic, Centre Hospitalier de l''Université de Montréal, Université de Montréal, Montréal, Québec, Canada;4. Division of Experimental Medicine, Faculty of Medicine, McGill University, Montréal, Québec, Canada;5. Department of Medicine, Faculty of Medicine, Université de Montréal, Montréal, Québec, Canada;6. Division of Endocrinology, University of Alberta, Edmonton, Alberta, Canada;1. Halo Research Group, Queen''s University Belfast, Belfast, UK;2. Wellcome-Wolfson Institute for Experimental Medicine. School of Medicine, Dentistry and Biomedical Sciences Queen''s University Belfast, Belfast, UK;3. School of Pharmacy, Queen''s University Belfast, Belfast, UK;4. Cork Centre for Cystic Fibrosis, Cork University Hospital, University College Cork, Ireland;5. HRB Clinical Research Facility, University College Cork, Cork, Ireland;6. Department of Medicine, Cork University Hospital, Wilton, Cork, Ireland;1. The Ohio State University, School of Health and Rehabilitation Sciences, 453 W. 10th Avenue, Columbus, OH, 43210, USA;2. Nationwide Children''s Hospital, Division of Gastroenterology, Hepatology, and Nutrition, 700 Children''s Drive, Columbus, OH 43205, USA;3. The Ohio State University Wexner Medical Center, Division of Gastroenterology, Hepatology, and Nutrition, 2nd Floor Doan Office Tower, 395 W. 12th Ave., Columbus, OH 43210-1228, USA;4. Nationwide Children''s Hospital, Division of Pulmonary Medicine, 700 Children''s Drive, Columbus, OH 43205, USA;1. Children''s Hospital of Philadelphia, Philadelphia, PA USA;2. Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA USA;1. SUNY Upstate Medical University, Syracuse NY;2. Wadsworth Center, New York State Department of Health, Albany NY;1. University of Washington, Seattle, USA;2. St. Vincent''s University Hospital, Dublin, Ireland;1. Department of Paediatrics, Christian Medical College, Vellore, India;2. Kuppuswamy Naidu Memorial Hospital, Coimbatore, India;3. Department of Clinical Genetics, Christian Medical College, Vellore, India;4. National Institute for Health Research, Southampton Respiratory Biomedical Research Centre, University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom
Abstract:BackgroundIn 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.MethodsThe 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.ResultsSubjects (n = 86) had a mean age of 31.7 ± 8.1 years, BMI of 23.0 ± 4.0 kg/m2, 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/m2 vs 22.6 ± 4.2 kg/m2 vs 22.1 ± 3.5 kg/m2, p = 0.0016). FEV1 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.ConclusionIn 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.
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