ArticleJournal of endocrinological investigation2021
Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion.
Article in Journal of endocrinological investigation, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it, 32 citations in OpenAlex.
- Effect of CFTR modulators on glucose homeostasis in children and young adults with cystic fibrosis-related diabetes: a systematic review.Frontiers in endocrinology · 2025Pooled it
- Beta cell function and insulin sensitivity during elexacaftor/tezacaftor/ivacaftor therapy in people with cystic fibrosis.Frontiers in endocrinology · 2026Article
- Elexacaftor-tezacaftor-ivacaftor enhances first-phase insulin secretion and improves glucose control in cystic fibrosis.Endocrine connections · 2026Article
- Article
- 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.Life (Basel, Switzerland) · 2024Article
- An experimentally validated approach to automated biological evidence generation in drug discovery using knowledge graphs.Nature communications · 2024Article
- An Update in Cystic Fibrosis-Related Diabetes in Children and Adolescents.Children (Basel, Switzerland) · 2023Review
- Oxidative Stress Biomarkers in Cystic Fibrosis and Cystic Fibrosis-Related Diabetes in Children: A Literature Review.Biomedicines · 2023Review
- Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes.Journal of clinical & translational endocrinology · 2023Article
- Distribution of OGTT-Related Variables in Patients with Cystic Fibrosis from Puberty to Adulthood: An Italian Multicenter Study.Journal of personalized medicine · 2023Article
- The effect of cystic fibrosis transmembrane conductance regulator modulators on impaired glucose tolerance and cystic fibrosis related diabetes.Journal of clinical & translational endocrinology · 2022Review
- Impact of CFTR Modulators on Beta-Cell Function in Children and Young Adults with Cystic Fibrosis.Journal of clinical medicine · 2022Article
- Diagnosis of cystic fibrosis-related diabetes: too early or too late?Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2022Article
- The role of modulators in cystic fibrosis related diabetes.Journal of clinical & translational endocrinology · 2022Article
- Short-Term Effects of Elexacaftor/Tezacaftor/Ivacaftor Combination on Glucose Tolerance in Young People With Cystic Fibrosis-An Observational Pilot Study.Frontiers in pediatrics · 2022Article
- The β Cell in Diabetes: Integrating Biomarkers With Functional Measures.Endocrine reviews · 2021Review
- The Potential Causes of Cystic Fibrosis-Related Diabetes.Frontiers in endocrinology · 2021Review
Corrections and comments
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Authors and funding
10 authors at 3 institutions in 1 country.
Funding
Abstract
purposeThe question whether the new cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs aimed at restoring CFTR protein function might improve glucose metabolism is gaining attention, but data on the effect of lumacaftor/ivacaftor treatment (LUMA/IVA) on glucose tolerance are limited. We evaluated the variation in glucose metabolism and insulin secretion in CF patients homozygous for Phe508del CFTR mutation after one-year treatment with LUMA/IVA in comparison to patients with the same genotype who did not receive such treatment.
methodsWe performed a retrospective case-control study on 13 patients with a confirmed diagnosis of CF, homozygous for the Phe508del CFTR mutation, who received LUMA/IVA for one year (cases) and 13 patients with identical genotype who did not receive this treatment (controls). At the beginning and conclusion of the follow-up, all subjects received a modified 3 h OGTT, sampling at baseline, and at 30 min intervals for plasma glucose, serum insulin, and c-peptide concentrations to evaluate glucose tolerance, and quantify by modeling beta-cell insulin secretion responsiveness to glucose, insulin clearance and insulin sensitivity.
resultsLUMA/IVA did not produce differences in glucose tolerance, insulin secretory parameters, clearance and sensitivity with respect to matched controls over one-year follow-up.
conclusionWe found no evidence of improvements in glucose tolerance mechanisms in patients with CF after one-year treatment with LUMA/IVA.
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Registered trials
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