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. 2024 Oct 16;14(10):1309.
doi: 10.3390/life14101309.

One-Year Effect of Elexacaftor/Tezacaftor/Ivacaftor Therapy on HbA1c Levels and Insulin Requirement in Patients with Insulin-Dependent Cystic Fibrosis-Related Diabetes: A Retrospective Observational Study

Marta Bassi  1   2 , Marina Francesca Strati  1 , Gaia Spiandorello  1 , Marta Scalas  1 , Federico Cresta  3 , Maria Grazia Calevo  2 , Giuseppe d'Annunzio  4 , Carlo Castellani  3 , Nicola Minuto  4 , Mohamad Maghnie  1   4 , Rosaria Casciaro  3
Affiliations

One-Year Effect of Elexacaftor/Tezacaftor/Ivacaftor Therapy on HbA1c Levels and Insulin Requirement in Patients with Insulin-Dependent Cystic Fibrosis-Related Diabetes: A Retrospective Observational Study

Marta Bassi et al. Life (Basel). .

Abstract

Introduction: The impact of ETI therapy on pulmonary function and nutritional status has been widely studied; the literature on the possible outcomes on glycemic control and insulin requirement in patients affected by CFRD is controversial.

Aim: The main objective of our study was to evaluate HbA1c levels in patients with cystic fibrosis-related diabetes (CFRD) after one year of therapy with elexacaftor/tezacaftor/ivacaftor (ETI). The secondary objective was to study the changes in the total daily insulin dose (TDD), pulmonary function and metabolism in this population.

Materials and methods: A retrospective single-center observational study was conducted at the Regional Cystic Fibrosis Centre and Diabetology Centre of IRCCS Istituto Giannina Gaslini. The observation period was divided into four different time points: initiation (T0), 3 months (T3mo), 6 months (T6mo) and 12 months (T12mo) of ETI therapy. Demographic and clinical data were collected. The results were then stratified by genotype (homozygous or heterozygous F508del).

Results: Twenty-eight patients with CFRD undergoing insulin therapy were included. TDD (IU) significantly decreased at T3mo and T6mo, but not at T12mo, whereas HbA1c decreased significantly at all three times. The number of hospitalizations and pulmonary exacerbations decreased significantly.

Conclusion: We demonstrated both improvement in glycemic control (by means of HbA1c) and insulin requirement in insulin-dependent CFRD patients after one year of ETI treatment.

Keywords: CFRD; CFTR modulators; ETI treatment; cystic fibrosis; diabetes.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as potential conflicts of interest.

References

    1. Moran A., Pekow P., Grover P., Zorn M., Slovis B., Pilewski J., Tullis E., Liou T.G., Allen H. Cystic Fibrosis Related Diabetes Therapy Study Group. Insulin therapy to improve BMI in cystic fibrosis-related diabetes without fasting hyperglycemia: Results of the cystic fibrosis related diabetes therapy trial. Diabetes Care. 2009;32:1783–1788. doi: 10.2337/dc09-0585. - DOI - PMC - PubMed
    1. Granados A., Chan C.L., Ode K.L., Moheet A., Moran A., Holl R. Cystic fibrosis related diabetes: Pathophysiology, screening and diagnosis. J. Cyst. Fibros. 2019;18((Suppl. 2)):S3–S9. doi: 10.1016/j.jcf.2019.08.016. - DOI - PubMed
    1. Gottlieb P.A., Yu L., Babu S., Wenzlau J., Bellin M., Frohnert B.I., Moran A. No relation between cystic fibrosis–related diabetes and type 1 diabetes autoimmunity. Diabetes Care. 2012;35:e57. doi: 10.2337/dc11-2327. - DOI - PMC - PubMed
    1. Ntimbane T., Mailhot G., Spahis S., Rabasa-Lhoret R., Kleme M.-L., Melloul D., Brochiero E., Berthiaume Y., Levy E. CFTR silencing in pancreatic β-cells reveals a functional impact on glucose-stimulated insulin secretion and oxidative stress response. Am. J. Physiol. Endocrinol. Metab. 2016;310:E200–E212. doi: 10.1152/ajpendo.00333.2015. - DOI - PubMed
    1. Lanng S., Thorsteinsson B., Nerup J., Koch C. Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis. Eur. J. Pediatr. 1992;151:684–687. doi: 10.1007/BF01957574. - DOI - PubMed

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