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Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice
Cystic fibrosis (CF) is a multisystem disorder, caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. These cause a reduced secretion of chloride, a marked absorption of sodium and, therefore, of water, through the epithelium, resulting in the formation of thickened secretio...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8343926/ https://www.ncbi.nlm.nih.gov/pubmed/34362426 http://dx.doi.org/10.1186/s13052-021-01117-1 |
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author | Terlizzi, Vito Masi, Eleonora Francalanci, Michela Taccetti, Giovanni Innocenti, Diletta |
author_facet | Terlizzi, Vito Masi, Eleonora Francalanci, Michela Taccetti, Giovanni Innocenti, Diletta |
author_sort | Terlizzi, Vito |
collection | PubMed |
description | Cystic fibrosis (CF) is a multisystem disorder, caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. These cause a reduced secretion of chloride, a marked absorption of sodium and, therefore, of water, through the epithelium, resulting in the formation of thickened secretions in organs such as lung or pancreas. These viscous secretions lead to airway obstruction, chronic infection and inflammation resulting in progressive lung damage, bronchiectasis and eventual respiratory failure. Although the average life expectancy has increased over the last 30 years, lung disease is the most common cause of death in people with CF. For these reasons, the improvement of sputum clearance is a major therapeutic aim in CF and early initiation of airway clearance is widely recommended and implemented. Symptomatic mucolytic therapy today is mainly based on inhalation of DNase, hypertonic saline or mannitol, in combination with physiotherapy. Mucolytic agents break down the gel structure of mucus and therefore decrease its elasticity and viscosity, reducing the pulmonary exacerbation frequency and to improve and stabilize lung function. Nevertheless, high quality studies comparing these mucolytic drugs are still few, and the individual experiences of patients and caregivers explain the high variability of their use globally. This review will summarize the current knowledge on hypertonic saline in the treatment of CF lung disease. Furthermore, we report the real-world prescription of inhaled mucolytic agents in CF. |
format | Online Article Text |
id | pubmed-8343926 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-83439262021-08-09 Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice Terlizzi, Vito Masi, Eleonora Francalanci, Michela Taccetti, Giovanni Innocenti, Diletta Ital J Pediatr Review Cystic fibrosis (CF) is a multisystem disorder, caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. These cause a reduced secretion of chloride, a marked absorption of sodium and, therefore, of water, through the epithelium, resulting in the formation of thickened secretions in organs such as lung or pancreas. These viscous secretions lead to airway obstruction, chronic infection and inflammation resulting in progressive lung damage, bronchiectasis and eventual respiratory failure. Although the average life expectancy has increased over the last 30 years, lung disease is the most common cause of death in people with CF. For these reasons, the improvement of sputum clearance is a major therapeutic aim in CF and early initiation of airway clearance is widely recommended and implemented. Symptomatic mucolytic therapy today is mainly based on inhalation of DNase, hypertonic saline or mannitol, in combination with physiotherapy. Mucolytic agents break down the gel structure of mucus and therefore decrease its elasticity and viscosity, reducing the pulmonary exacerbation frequency and to improve and stabilize lung function. Nevertheless, high quality studies comparing these mucolytic drugs are still few, and the individual experiences of patients and caregivers explain the high variability of their use globally. This review will summarize the current knowledge on hypertonic saline in the treatment of CF lung disease. Furthermore, we report the real-world prescription of inhaled mucolytic agents in CF. BioMed Central 2021-08-06 /pmc/articles/PMC8343926/ /pubmed/34362426 http://dx.doi.org/10.1186/s13052-021-01117-1 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/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Review Terlizzi, Vito Masi, Eleonora Francalanci, Michela Taccetti, Giovanni Innocenti, Diletta Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice |
title | Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice |
title_full | Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice |
title_fullStr | Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice |
title_full_unstemmed | Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice |
title_short | Hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice |
title_sort | hypertonic saline in people with cystic fibrosis: review of comparative studies and clinical practice |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8343926/ https://www.ncbi.nlm.nih.gov/pubmed/34362426 http://dx.doi.org/10.1186/s13052-021-01117-1 |
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