Bastien Misgault1, Eva Chatron2, Quitterie Reynaud3, Sandrine Touzet4, Michel Abely5, Laurent Melly6, Stéphane Dominique7, Françoise Troussier8, Olivia Ronsin-Pradel9, Michèle Gerardin10, Julie Mankikian11, Laure Cosson12, Raphael Chiron13, Leila Bounyar1, Michel Porzio2, Isabelle Durieu3, Laurence Weiss14, Romain Kessler15, Laurence Kessler16. 1. Service d'endocrinologie, diabète et nutrition, Hôpitaux Universitaires de Strasbourg, place de l'hôpital, Strasbourg 67091, France. 2. Service de pneumologie et CRCM adulte, Hôpitaux Universitaires de Strasbourg, 1 place de l'hôpital, Strasbourg 67091, France. 3. Department of Internal Medicine Adult Cystic Fibrosis Care Center, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, France, EA HESPER, Lyon 7425, France. 4. Pôle de Santé Publique, Hospices Civils de Lyon, France, Université Claude Bernard Lyon 1, EA HESPER, Lyon 7425, France. 5. CRCM, Hôpitaux Universitaires de Reims, 47, rue Cognacq-Jay, Reims 51092, France. 6. Centre de Giens, Hospices Civils de Lyon, Lyon, France. 7. CRCM, CHU Rouen, Rouen, France. 8. CRCM Angers-Le Mans, CHU Angers, Angers, France. 9. CRCM, CHU Marseille, Marseille, France. 10. CRCM enfant, Hôpital Robert Debré, Paris, France. 11. CRCM, Hôpital Bretonneau, CHRU Tours, Tours, France. 12. CRCM, Hôpital de Clocheville, CHRU Tours, Tours, France. 13. CRCM, Raphael Chiron, Hôpital Arnaud de Villeneuve, Montpellier, France. 14. CRCM pédiatrique, Hôpitaux Universitaires de Strasbourg, 1 Avenue Molière, Strasbourg 67098, France. 15. Service de pneumologie et CRCM adulte, Hôpitaux Universitaires de Strasbourg, 1 place de l'hôpital, Strasbourg 67091, France; Inserm UMR 1260 Regenerative Nanomedicine Fédération de Médecine Translationnelle, Université de Strasbourg, Strasbourg, France. 16. Service d'endocrinologie, diabète et nutrition, Hôpitaux Universitaires de Strasbourg, place de l'hôpital, Strasbourg 67091, France; Inserm UMR 1260 Regenerative Nanomedicine Fédération de Médecine Translationnelle, Université de Strasbourg, Strasbourg, France. Electronic address: laurence.kessler@chru-strasbourg.fr.
Abstract
OBJECTIVES: To investigate the effects of 1-year lumacaftor-ivacaftor treatment on abnormalities in glucose tolerance (AGT) in Phe508del homozygous cystic fibrosis (CF) patients. METHODS: Untreated CF patients with glucose intolerance or newly diagnosed diabetes were included in a prospective, observational study. After 1-year lumacaftor-ivacaftor treatment, AGT were evaluated by using oral glucose tolerance test. RESULTS: Forty patients participated. 78% of patients had glucose intolerance and 22% diabetes at baseline. After one-year treatment, 50% of patients had normal glucose tolerance, 40% glucose intolerance, and 10% diabetes (p <0.001). The two-hour OGTT glycemia decreased from 171 (153-197) to 139 (117-162) mg/dL (p <0.001). 57.5% (n = 23) of patients improved their glucose tolerance with a significant decrease in both 1-hour (p<0.01) and 2-hour (p<0.001) OGTT glycemia. CONCLUSION: Improvements in AGT were observed following 1-year lumacaftor-ivacaftor treatment. Larger studies are needed to comprehensively assess CF transmembrane conductance regulator (CFTR) modulators.
OBJECTIVES: To investigate the effects of 1-year lumacaftor-ivacaftor treatment on abnormalities in glucose tolerance (AGT) in Phe508del homozygous cystic fibrosis (CF) patients. METHODS: Untreated CFpatients with glucose intolerance or newly diagnosed diabetes were included in a prospective, observational study. After 1-year lumacaftor-ivacaftor treatment, AGT were evaluated by using oral glucose tolerance test. RESULTS: Forty patients participated. 78% of patients had glucose intolerance and 22% diabetes at baseline. After one-year treatment, 50% of patients had normal glucose tolerance, 40% glucose intolerance, and 10% diabetes (p <0.001). The two-hour OGTT glycemia decreased from 171 (153-197) to 139 (117-162) mg/dL (p <0.001). 57.5% (n = 23) of patients improved their glucose tolerance with a significant decrease in both 1-hour (p<0.01) and 2-hour (p<0.001) OGTT glycemia. CONCLUSION: Improvements in AGT were observed following 1-year lumacaftor-ivacaftor treatment. Larger studies are needed to comprehensively assess CF transmembrane conductance regulator (CFTR) modulators.
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