P286 Triple CFTR modulator combination improves glucose tolerance in adolescents with cystic fibrosis. Data from the French observational paediatric study MODUL-CF

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Weiss, L. | Galderisi, A. | Besançon, A. | Stremler, N. | Reix, P. | Wizla, N. | Rames, C. | Marguet, C. | Tatapoulos, A. | Perrisson, C. | Dalphin, M.-L. | Troussier, F. | Houdouin, V. | Abely, M. | Cosson, L. | Gabsi, A. | Corvol, H. | Deneuville, E. | Storni, V. | Ramel, S. | Bui, S. | Heraud, M.-C. | Epaud, R. | Huet, F. | Scalbert, M. | Mely, L. | Gachelin, E. | Giannantonio, M. | Sahki, D. | Lustre, A. | Bonnel, A.-S. | Sermet, I.

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International audience. Highly effective CFTR Modulators herald a new era in therapeutic strategyof Cystic fibrosis. Exacaftor/tezacaftor/ivacaftor (ETI) has shown to improve lung function, decrease number of pulmonary exacerbations and improvesquality of life of patients carrying at least one p.Phe508del. Apart thepulmonary disease, these drugs may also impact positively on endocrinepancreas. Impact on glucose tolerance is unclear.Methods: We analysed real-world data from the French longitudinalMODUL-CF study that includes children who started a CFTR modulator. Weinvestigate first the effect of ETI on glucose tolerance in adolescents aged 12to 18. The current analysis includes all the participants enrolled from July2021 to July 2023 who completed an OGTT before and 12 ± 3 months afterthe beginning ETI. Participants were excluded if they had a diagnosis ofCFRD requiring insulin treatment at baseline. This cohort was stratified intwo subgroups based on the baseline OGTT: normal glucose tolerance(NGT) and abnormal glucose tolerance (AGT) defined by impaired fastingglucose or impaired glucose tolerance or diabetes not requiring insulintreatment.Results: We included 106 participants: 79 NGT pwCF, 27 AGT pwCFincluding 3 CFRD. The baseline characteristics of the two cohorts weresimilar except for a higher glucose level at 1-h and 2-h in the AGT group. At12 months, NGT pwCF did not exhibit any change of 1-h and 2-h glucose. Incontrast, AGT pwCF displayed a reduction of glucose level at 1-h and 2-h(p = 0.013 and p < 0.001). 15 patients out of the 24 AGT group switched toNGT group while only 16% of NGT pwCF progressed to AGT or CFRD. 3participants with CFRD at baseline returned to AGT. Orkambi previoustreatment was associated with a lower 2-h glucose at the follow-up OGTTin the AGT group at baseline (p = 0.047).Conclusion: ETI improves glucose tolerance in adolescents with CF.

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