P286 Triple CFTR modulator combination improves glucose tolerance in adolescents with cystic fibrosis. Data from the French observational paediatric study MODUL-CF - Université de Reims Champagne-Ardenne Access content directly
Conference Poster Year : 2024

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

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

Abstract

Highly effective CFTR Modulators herald a new era in therapeutic strategy of Cystic fibrosis. Exacaftor/tezacaftor/ivacaftor (ETI) has shown to improve lung function, decrease number of pulmonary exacerbations and improves quality of life of patients carrying at least one p.Phe508del. Apart the pulmonary disease, these drugs may also impact positively on endocrine pancreas. Impact on glucose tolerance is unclear. Methods: We analysed real-world data from the French longitudinal MODUL-CF study that includes children who started a CFTR modulator. We investigate first the effect of ETI on glucose tolerance in adolescents aged 12 to 18. The current analysis includes all the participants enrolled from July 2021 to July 2023 who completed an OGTT before and 12 ± 3 months after the beginning ETI. Participants were excluded if they had a diagnosis of CFRD requiring insulin treatment at baseline. This cohort was stratified in two subgroups based on the baseline OGTT: normal glucose tolerance (NGT) and abnormal glucose tolerance (AGT) defined by impaired fasting glucose or impaired glucose tolerance or diabetes not requiring insulin treatment. Results: We included 106 participants: 79 NGT pwCF, 27 AGT pwCF including 3 CFRD. The baseline characteristics of the two cohorts were similar except for a higher glucose level at 1-h and 2-h in the AGT group. At 12 months, NGT pwCF did not exhibit any change of 1-h and 2-h glucose. In contrast, 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 to NGT group while only 16% of NGT pwCF progressed to AGT or CFRD. 3 participants with CFRD at baseline returned to AGT. Orkambi previous treatment was associated with a lower 2-h glucose at the follow-up OGTT in the AGT group at baseline (p = 0.047). Conclusion: ETI improves glucose tolerance in adolescents with CF.
No file

Dates and versions

hal-04609407 , version 1 (12-06-2024)

Identifiers

Cite

L. Weiss, A. Galderisi, A. Besançon, N. Stremler, P. Reix, et al.. P286 Triple CFTR modulator combination improves glucose tolerance in adolescents with cystic fibrosis. Data from the French observational paediatric study MODUL-CF. ECFC (European Cystic Fibrosis Conference), Jun 2024, Glasgow ( Virtual ), United Kingdom. 23, pp.S157, 2024, ⟨10.1016/S1569-1993(24)00587-3⟩. ⟨hal-04609407⟩

Collections

URCA P3CELL
4 View
0 Download

Altmetric

Share

Gmail Mastodon Facebook X LinkedIn More