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Article

Impact of CFTR Modulators on Beta-Cell Function in Children and Young Adults with Cystic Fibrosis

by
Claudia Piona
1,†,
Enza Mozzillo
2,†,
Antonella Tosco
3,*,
Sonia Volpi
4,
Francesco Maria Rosanio
2,
Chiara Cimbalo
3,
Adriana Franzese
2,
Valeria Raia
3,
Chiara Zusi
1,
Federica Emiliani
1,
Maria Linda Boselli
5,
Maddalena Trombetta
5,
Riccardo Crocina Bonadonna
6,7,
Marco Cipolli
4 and
Claudio Maffeis
1
1
Department of Surgery, Dentistry, Pediatrics and Gynecology, Section of Pediatric Diabetes and Metabolism, University and Azienda Ospedaliera Universitaria Integrata of Verona, 37126 Verona, Italy
2
Regional Pediatric Diabetes Center, Department of Translational Medical Sciences, Section of Pediatrics, Federico II University of Naples, 80131 Naples, Italy
3
Regional Pediatric Cystic Fibrosis Center, Department of Translational Medical Sciences, Section of Pediatrics, Federico II University of Naples, 80131 Naples, Italy
4
Cystic Fibrosis Unit, Regional Center for Cystic Fibrosis, University and Azienda Ospedaliera Universitaria Integrata of Verona, 37126 Verona, Italy
5
Department of Medicine, Section of Endocrinology, University and Azienda Ospedaliera Universitaria Integrata of Verona, 37126 Verona, Italy
6
Department of Medicine and Surgery, University of Parma, 43121 Parma, Italy
7
Division of Endocrinology and Metabolic Diseases, Azienda Ospedaliero-Universitaria di Parma, 43125 Parma, Italy
*
Author to whom correspondence should be addressed.
†
These authors equally contributed to this study.
J. Clin. Med. 2022, 11(14), 4149; https://doi.org/10.3390/jcm11144149
Submission received: 16 May 2022 / Revised: 30 June 2022 / Accepted: 9 July 2022 / Published: 17 July 2022
(This article belongs to the Special Issue Cystic Fibrosis: Genotype, Clinical Features, and Current Comorbidities)
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Abstract

Background: To date, no consistent data are available on the possible impact of CFTR modulators on glucose metabolism. The aim of this study was to test the hypothesis that treatment with CFTR modulators is associated with an improvement in the key direct determinants of glucose regulation in children and young adults affected by Cystic Fibrosis (CF). Methods: In this study, 21 CF patients aged 10–25 underwent oral glucose tolerance test (OGTT) before and after 12–18 months of treatment with Lumacaftor/Ivacaftor or Elexacaftor-Ivacaftor-Tezacaftor. β-cell function (i.e., first and second phase of insulin secretion measured as derivative and proportional control, respectively) and insulin clearance were estimated by OGTT mathematical modelling. Insulin sensitivity was estimated by the Oral Glucose Sensitivity Index (OGIS). The dynamic interplay between β-cell function, insulin clearance and insulin sensitivity was analysed by vector plots of glucose-stimulated insulin bioavailability vs. insulin sensitivity. Results: No changes in glucose tolerance occurred after either treatment, whereas a significant improvement in pulmonary function and chronic bacterial infection was observed. Beta cell function and insulin clearance did not change in both treatment groups. Insulin sensitivity worsened in the Lumacaftor/Ivacaftor group. The analysis of vector plots confirmed that glucose regulation was stable in both groups. Conclusions: Treatment of CF patients with CFTR modulators does not significantly ameliorate glucose homeostasis and/or any of its direct determinants.
Keywords: cystic fibrosis; cftr modulators; lumacaftor/ivacaftor; elexacaftor-ivacaftor-tezacaftor; oral glucose tolerance test; glucose metabolism; β-cell function; insulin clearance; insulin sensitivity cystic fibrosis; cftr modulators; lumacaftor/ivacaftor; elexacaftor-ivacaftor-tezacaftor; oral glucose tolerance test; glucose metabolism; β-cell function; insulin clearance; insulin sensitivity

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MDPI and ACS Style

Piona, C.; Mozzillo, E.; Tosco, A.; Volpi, S.; Rosanio, F.M.; Cimbalo, C.; Franzese, A.; Raia, V.; Zusi, C.; Emiliani, F.; et al. Impact of CFTR Modulators on Beta-Cell Function in Children and Young Adults with Cystic Fibrosis. J. Clin. Med. 2022, 11, 4149. https://doi.org/10.3390/jcm11144149

AMA Style

Piona C, Mozzillo E, Tosco A, Volpi S, Rosanio FM, Cimbalo C, Franzese A, Raia V, Zusi C, Emiliani F, et al. Impact of CFTR Modulators on Beta-Cell Function in Children and Young Adults with Cystic Fibrosis. Journal of Clinical Medicine. 2022; 11(14):4149. https://doi.org/10.3390/jcm11144149

Chicago/Turabian Style

Piona, Claudia, Enza Mozzillo, Antonella Tosco, Sonia Volpi, Francesco Maria Rosanio, Chiara Cimbalo, Adriana Franzese, Valeria Raia, Chiara Zusi, Federica Emiliani, and et al. 2022. "Impact of CFTR Modulators on Beta-Cell Function in Children and Young Adults with Cystic Fibrosis" Journal of Clinical Medicine 11, no. 14: 4149. https://doi.org/10.3390/jcm11144149

APA Style

Piona, C., Mozzillo, E., Tosco, A., Volpi, S., Rosanio, F. M., Cimbalo, C., Franzese, A., Raia, V., Zusi, C., Emiliani, F., Boselli, M. L., Trombetta, M., Bonadonna, R. C., Cipolli, M., & Maffeis, C. (2022). Impact of CFTR Modulators on Beta-Cell Function in Children and Young Adults with Cystic Fibrosis. Journal of Clinical Medicine, 11(14), 4149. https://doi.org/10.3390/jcm11144149

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