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Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion

PURPOSE: The 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 ar...

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Autores principales: Colombo, C., Foppiani, A., Bisogno, A., Gambazza, S., Daccò, V., Nazzari, E., Leone, A., Giana, A., Mari, A., Battezzati, A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer International Publishing 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8421269/
https://www.ncbi.nlm.nih.gov/pubmed/33586024
http://dx.doi.org/10.1007/s40618-021-01525-4
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author Colombo, C.
Foppiani, A.
Bisogno, A.
Gambazza, S.
Daccò, V.
Nazzari, E.
Leone, A.
Giana, A.
Mari, A.
Battezzati, A.
author_facet Colombo, C.
Foppiani, A.
Bisogno, A.
Gambazza, S.
Daccò, V.
Nazzari, E.
Leone, A.
Giana, A.
Mari, A.
Battezzati, A.
author_sort Colombo, C.
collection PubMed
description PURPOSE: The 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. METHODS: We 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. RESULTS: LUMA/IVA did not produce differences in glucose tolerance, insulin secretory parameters, clearance and sensitivity with respect to matched controls over one-year follow-up. CONCLUSION: We found no evidence of improvements in glucose tolerance mechanisms in patients with CF after one-year treatment with LUMA/IVA.
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spelling pubmed-84212692021-09-09 Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion Colombo, C. Foppiani, A. Bisogno, A. Gambazza, S. Daccò, V. Nazzari, E. Leone, A. Giana, A. Mari, A. Battezzati, A. J Endocrinol Invest Original Article PURPOSE: The 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. METHODS: We 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. RESULTS: LUMA/IVA did not produce differences in glucose tolerance, insulin secretory parameters, clearance and sensitivity with respect to matched controls over one-year follow-up. CONCLUSION: We found no evidence of improvements in glucose tolerance mechanisms in patients with CF after one-year treatment with LUMA/IVA. Springer International Publishing 2021-02-13 2021 /pmc/articles/PMC8421269/ /pubmed/33586024 http://dx.doi.org/10.1007/s40618-021-01525-4 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Original Article
Colombo, C.
Foppiani, A.
Bisogno, A.
Gambazza, S.
Daccò, V.
Nazzari, E.
Leone, A.
Giana, A.
Mari, A.
Battezzati, A.
Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion
title Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion
title_full Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion
title_fullStr Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion
title_full_unstemmed Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion
title_short Lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion
title_sort lumacaftor/ivacaftor in cystic fibrosis: effects on glucose metabolism and insulin secretion
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8421269/
https://www.ncbi.nlm.nih.gov/pubmed/33586024
http://dx.doi.org/10.1007/s40618-021-01525-4
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