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Nutritional impact of CFTR modulators in children with cystic fibrosis

BACKGROUND: Nutritional status is a major prognostic factor for breathing and the survival of patients with cystic fibrosis (CF). Since 2012, the development of CFTR modulators has considerably transformed the outcome of this disease. Indeed, both lung function and body mass index are improved by CF...

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Autores principales: Gaschignard, Margaux, Beaufils, Fabien, Lussac-Sorton, Florian, Gallet, Pauline, Clouzeau, Haude, Menard, Joris, Costanzo, Aurélie, Nouard, Lucie, Delhaes, Laurence, Tetard, Candice, Lamireau, Thierry, Fayon, Michael, Bui, Stéphanie, Enaud, Raphaël
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10091219/
https://www.ncbi.nlm.nih.gov/pubmed/37063653
http://dx.doi.org/10.3389/fped.2023.1130790
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author Gaschignard, Margaux
Beaufils, Fabien
Lussac-Sorton, Florian
Gallet, Pauline
Clouzeau, Haude
Menard, Joris
Costanzo, Aurélie
Nouard, Lucie
Delhaes, Laurence
Tetard, Candice
Lamireau, Thierry
Fayon, Michael
Bui, Stéphanie
Enaud, Raphaël
author_facet Gaschignard, Margaux
Beaufils, Fabien
Lussac-Sorton, Florian
Gallet, Pauline
Clouzeau, Haude
Menard, Joris
Costanzo, Aurélie
Nouard, Lucie
Delhaes, Laurence
Tetard, Candice
Lamireau, Thierry
Fayon, Michael
Bui, Stéphanie
Enaud, Raphaël
author_sort Gaschignard, Margaux
collection PubMed
description BACKGROUND: Nutritional status is a major prognostic factor for breathing and the survival of patients with cystic fibrosis (CF). Since 2012, the development of CFTR modulators has considerably transformed the outcome of this disease. Indeed, both lung function and body mass index are improved by CFTR modulators, such as Lumacaftor/Ivacaftor. However, few data exist regarding the outcome of nutritional intakes under Lumacaftor/Ivacaftor. METHODS: We conducted a prospective single-center study in children with CF treated with Lumacaftor/Ivacaftor to evaluate their nutritional intake before and after treatment RESULTS: Thirty-four children were included in this study, with a median age of 12.4 years [11.9; 14.7]. There was no significant improvement in weight, height or BMI. Patients' total energy intake was not significantly changed with Lumacaftor/Ivacaftor, while carbohydrate intakes decreased significantly. We found that blood levels of vitamin E and Selenium were significantly increased under Lumacaftor/Ivacaftor, without a significant increase in supplementation. In patients with a BMI Z-score < 0 at treatment initiation, there was a significant improvement in weight and BMI Z-score, while TEI and carbohydrate intakes were significantly lower. CONCLUSION: We showed that treatment with Lumacaftor/Ivacaftor improved the nutritional status of patients without necessarily being associated with an increase in nutritional intake. Although these data need to be confirmed in larger cohorts, they support the hypothesis that weight gain under modulators is multifactorial, and may be related to a decrease in energy expenditure or an improvement in absorption.
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spelling pubmed-100912192023-04-13 Nutritional impact of CFTR modulators in children with cystic fibrosis Gaschignard, Margaux Beaufils, Fabien Lussac-Sorton, Florian Gallet, Pauline Clouzeau, Haude Menard, Joris Costanzo, Aurélie Nouard, Lucie Delhaes, Laurence Tetard, Candice Lamireau, Thierry Fayon, Michael Bui, Stéphanie Enaud, Raphaël Front Pediatr Pediatrics BACKGROUND: Nutritional status is a major prognostic factor for breathing and the survival of patients with cystic fibrosis (CF). Since 2012, the development of CFTR modulators has considerably transformed the outcome of this disease. Indeed, both lung function and body mass index are improved by CFTR modulators, such as Lumacaftor/Ivacaftor. However, few data exist regarding the outcome of nutritional intakes under Lumacaftor/Ivacaftor. METHODS: We conducted a prospective single-center study in children with CF treated with Lumacaftor/Ivacaftor to evaluate their nutritional intake before and after treatment RESULTS: Thirty-four children were included in this study, with a median age of 12.4 years [11.9; 14.7]. There was no significant improvement in weight, height or BMI. Patients' total energy intake was not significantly changed with Lumacaftor/Ivacaftor, while carbohydrate intakes decreased significantly. We found that blood levels of vitamin E and Selenium were significantly increased under Lumacaftor/Ivacaftor, without a significant increase in supplementation. In patients with a BMI Z-score < 0 at treatment initiation, there was a significant improvement in weight and BMI Z-score, while TEI and carbohydrate intakes were significantly lower. CONCLUSION: We showed that treatment with Lumacaftor/Ivacaftor improved the nutritional status of patients without necessarily being associated with an increase in nutritional intake. Although these data need to be confirmed in larger cohorts, they support the hypothesis that weight gain under modulators is multifactorial, and may be related to a decrease in energy expenditure or an improvement in absorption. Frontiers Media S.A. 2023-03-29 /pmc/articles/PMC10091219/ /pubmed/37063653 http://dx.doi.org/10.3389/fped.2023.1130790 Text en © 2023 Gaschignard, Beaufils, Lussac-Sorton, Gallet, Clouzeau, Menard, Costanzo, Nouard, Delhaes, Tetard, Lamireau, Fayon, Bui and Enaud. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) (https://creativecommons.org/licenses/by/4.0/) . The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Pediatrics
Gaschignard, Margaux
Beaufils, Fabien
Lussac-Sorton, Florian
Gallet, Pauline
Clouzeau, Haude
Menard, Joris
Costanzo, Aurélie
Nouard, Lucie
Delhaes, Laurence
Tetard, Candice
Lamireau, Thierry
Fayon, Michael
Bui, Stéphanie
Enaud, Raphaël
Nutritional impact of CFTR modulators in children with cystic fibrosis
title Nutritional impact of CFTR modulators in children with cystic fibrosis
title_full Nutritional impact of CFTR modulators in children with cystic fibrosis
title_fullStr Nutritional impact of CFTR modulators in children with cystic fibrosis
title_full_unstemmed Nutritional impact of CFTR modulators in children with cystic fibrosis
title_short Nutritional impact of CFTR modulators in children with cystic fibrosis
title_sort nutritional impact of cftr modulators in children with cystic fibrosis
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10091219/
https://www.ncbi.nlm.nih.gov/pubmed/37063653
http://dx.doi.org/10.3389/fped.2023.1130790
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