Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index

Abstract Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF trans-membrane conductance regulator (CFTR) gene changes a protein (a regulated chloride channel), which is expressed...
Ausführliche Beschreibung

Gespeichert in:

Autor*in:	Terlizzi, Vito [verfasserIn] Castellani, Chiara Taccetti, Giovanni Ferrari, Beatrice

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2022

Schlagwörter:	Mucolytic agents LCI Children Lung function

Anmerkung:	© The Author(s) 2022

Übergeordnetes Werk:	Enthalten in: The Italian journal of pediatrics - London : BioMed Central, 2008, 48(2022), 1 vom: 04. Aug.
Übergeordnetes Werk:	volume:48 ; year:2022 ; number:1 ; day:04 ; month:08

Links:	Volltext

DOI / URN:	10.1186/s13052-022-01331-5

Katalog-ID:	SPR050902539

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allfields	10.1186/s13052-022-01331-5 doi (DE-627)SPR050902539 (SPR)s13052-022-01331-5-e DE-627 ger DE-627 rakwb eng Terlizzi, Vito verfasserin (orcid)0000-0003-1106-4424 aut Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2022 Abstract Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF trans-membrane conductance regulator (CFTR) gene changes a protein (a regulated chloride channel), which is expressed in many tissues. Defective CFTR results in reduced chloride secretion and an overage absorption of sodium across the epithelia, leading to thickened secretions in organs such as pancreas and lung. Gradually, there have been considerable improvements in the survival of people with CF, thanks to substantial changes in specialized CF care and the discovery of new CFTR modulators drugs. Nevertheless, lung disease remains the most common cause of death. For these reasons improvement of sputum clearance is a major therapeutic aim in CF. So far, symptomatic mucolytic therapy is mainly based on inhalation of dornase alfa, hypertonic saline or mannitol, in combination with physiotherapy. The major component of mucus in CF is pus including viscous material such as polymerized DNA derived from degraded neutrophils. Dornase alfa cleaves the DNA released from the neutrophils and reduces mucous viscosity, and further prevent airway infections and damage to the lung parenchyma. In this review we will summarize the current knowledge on dornase alfa in the treatment of CF lung disease, especially highlighting the positive effect on lung clearance index, a sensitive measure of ventilation inhomogeneity. Mucolytic agents (dpeaa)DE-He213 LCI (dpeaa)DE-He213 Children (dpeaa)DE-He213 Lung function (dpeaa)DE-He213 Castellani, Chiara aut Taccetti, Giovanni aut Ferrari, Beatrice aut Enthalten in The Italian journal of pediatrics London : BioMed Central, 2008 48(2022), 1 vom: 04. Aug. (DE-627)352108029 (DE-600)2084688-5 1824-7288 nnns volume:48 year:2022 number:1 day:04 month:08 https://dx.doi.org/10.1186/s13052-022-01331-5 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 48 2022 1 04 08
spelling	10.1186/s13052-022-01331-5 doi (DE-627)SPR050902539 (SPR)s13052-022-01331-5-e DE-627 ger DE-627 rakwb eng Terlizzi, Vito verfasserin (orcid)0000-0003-1106-4424 aut Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2022 Abstract Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF trans-membrane conductance regulator (CFTR) gene changes a protein (a regulated chloride channel), which is expressed in many tissues. Defective CFTR results in reduced chloride secretion and an overage absorption of sodium across the epithelia, leading to thickened secretions in organs such as pancreas and lung. Gradually, there have been considerable improvements in the survival of people with CF, thanks to substantial changes in specialized CF care and the discovery of new CFTR modulators drugs. Nevertheless, lung disease remains the most common cause of death. For these reasons improvement of sputum clearance is a major therapeutic aim in CF. So far, symptomatic mucolytic therapy is mainly based on inhalation of dornase alfa, hypertonic saline or mannitol, in combination with physiotherapy. The major component of mucus in CF is pus including viscous material such as polymerized DNA derived from degraded neutrophils. Dornase alfa cleaves the DNA released from the neutrophils and reduces mucous viscosity, and further prevent airway infections and damage to the lung parenchyma. In this review we will summarize the current knowledge on dornase alfa in the treatment of CF lung disease, especially highlighting the positive effect on lung clearance index, a sensitive measure of ventilation inhomogeneity. Mucolytic agents (dpeaa)DE-He213 LCI (dpeaa)DE-He213 Children (dpeaa)DE-He213 Lung function (dpeaa)DE-He213 Castellani, Chiara aut Taccetti, Giovanni aut Ferrari, Beatrice aut Enthalten in The Italian journal of pediatrics London : BioMed Central, 2008 48(2022), 1 vom: 04. Aug. (DE-627)352108029 (DE-600)2084688-5 1824-7288 nnns volume:48 year:2022 number:1 day:04 month:08 https://dx.doi.org/10.1186/s13052-022-01331-5 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 48 2022 1 04 08
allfields_unstemmed	10.1186/s13052-022-01331-5 doi (DE-627)SPR050902539 (SPR)s13052-022-01331-5-e DE-627 ger DE-627 rakwb eng Terlizzi, Vito verfasserin (orcid)0000-0003-1106-4424 aut Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2022 Abstract Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF trans-membrane conductance regulator (CFTR) gene changes a protein (a regulated chloride channel), which is expressed in many tissues. Defective CFTR results in reduced chloride secretion and an overage absorption of sodium across the epithelia, leading to thickened secretions in organs such as pancreas and lung. Gradually, there have been considerable improvements in the survival of people with CF, thanks to substantial changes in specialized CF care and the discovery of new CFTR modulators drugs. Nevertheless, lung disease remains the most common cause of death. For these reasons improvement of sputum clearance is a major therapeutic aim in CF. So far, symptomatic mucolytic therapy is mainly based on inhalation of dornase alfa, hypertonic saline or mannitol, in combination with physiotherapy. The major component of mucus in CF is pus including viscous material such as polymerized DNA derived from degraded neutrophils. Dornase alfa cleaves the DNA released from the neutrophils and reduces mucous viscosity, and further prevent airway infections and damage to the lung parenchyma. In this review we will summarize the current knowledge on dornase alfa in the treatment of CF lung disease, especially highlighting the positive effect on lung clearance index, a sensitive measure of ventilation inhomogeneity. Mucolytic agents (dpeaa)DE-He213 LCI (dpeaa)DE-He213 Children (dpeaa)DE-He213 Lung function (dpeaa)DE-He213 Castellani, Chiara aut Taccetti, Giovanni aut Ferrari, Beatrice aut Enthalten in The Italian journal of pediatrics London : BioMed Central, 2008 48(2022), 1 vom: 04. Aug. (DE-627)352108029 (DE-600)2084688-5 1824-7288 nnns volume:48 year:2022 number:1 day:04 month:08 https://dx.doi.org/10.1186/s13052-022-01331-5 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 48 2022 1 04 08
allfieldsGer	10.1186/s13052-022-01331-5 doi (DE-627)SPR050902539 (SPR)s13052-022-01331-5-e DE-627 ger DE-627 rakwb eng Terlizzi, Vito verfasserin (orcid)0000-0003-1106-4424 aut Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2022 Abstract Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF trans-membrane conductance regulator (CFTR) gene changes a protein (a regulated chloride channel), which is expressed in many tissues. Defective CFTR results in reduced chloride secretion and an overage absorption of sodium across the epithelia, leading to thickened secretions in organs such as pancreas and lung. Gradually, there have been considerable improvements in the survival of people with CF, thanks to substantial changes in specialized CF care and the discovery of new CFTR modulators drugs. Nevertheless, lung disease remains the most common cause of death. For these reasons improvement of sputum clearance is a major therapeutic aim in CF. So far, symptomatic mucolytic therapy is mainly based on inhalation of dornase alfa, hypertonic saline or mannitol, in combination with physiotherapy. The major component of mucus in CF is pus including viscous material such as polymerized DNA derived from degraded neutrophils. Dornase alfa cleaves the DNA released from the neutrophils and reduces mucous viscosity, and further prevent airway infections and damage to the lung parenchyma. In this review we will summarize the current knowledge on dornase alfa in the treatment of CF lung disease, especially highlighting the positive effect on lung clearance index, a sensitive measure of ventilation inhomogeneity. Mucolytic agents (dpeaa)DE-He213 LCI (dpeaa)DE-He213 Children (dpeaa)DE-He213 Lung function (dpeaa)DE-He213 Castellani, Chiara aut Taccetti, Giovanni aut Ferrari, Beatrice aut Enthalten in The Italian journal of pediatrics London : BioMed Central, 2008 48(2022), 1 vom: 04. Aug. (DE-627)352108029 (DE-600)2084688-5 1824-7288 nnns volume:48 year:2022 number:1 day:04 month:08 https://dx.doi.org/10.1186/s13052-022-01331-5 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 48 2022 1 04 08
allfieldsSound	10.1186/s13052-022-01331-5 doi (DE-627)SPR050902539 (SPR)s13052-022-01331-5-e DE-627 ger DE-627 rakwb eng Terlizzi, Vito verfasserin (orcid)0000-0003-1106-4424 aut Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2022 Abstract Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF trans-membrane conductance regulator (CFTR) gene changes a protein (a regulated chloride channel), which is expressed in many tissues. Defective CFTR results in reduced chloride secretion and an overage absorption of sodium across the epithelia, leading to thickened secretions in organs such as pancreas and lung. Gradually, there have been considerable improvements in the survival of people with CF, thanks to substantial changes in specialized CF care and the discovery of new CFTR modulators drugs. Nevertheless, lung disease remains the most common cause of death. For these reasons improvement of sputum clearance is a major therapeutic aim in CF. So far, symptomatic mucolytic therapy is mainly based on inhalation of dornase alfa, hypertonic saline or mannitol, in combination with physiotherapy. The major component of mucus in CF is pus including viscous material such as polymerized DNA derived from degraded neutrophils. Dornase alfa cleaves the DNA released from the neutrophils and reduces mucous viscosity, and further prevent airway infections and damage to the lung parenchyma. In this review we will summarize the current knowledge on dornase alfa in the treatment of CF lung disease, especially highlighting the positive effect on lung clearance index, a sensitive measure of ventilation inhomogeneity. Mucolytic agents (dpeaa)DE-He213 LCI (dpeaa)DE-He213 Children (dpeaa)DE-He213 Lung function (dpeaa)DE-He213 Castellani, Chiara aut Taccetti, Giovanni aut Ferrari, Beatrice aut Enthalten in The Italian journal of pediatrics London : BioMed Central, 2008 48(2022), 1 vom: 04. Aug. (DE-627)352108029 (DE-600)2084688-5 1824-7288 nnns volume:48 year:2022 number:1 day:04 month:08 https://dx.doi.org/10.1186/s13052-022-01331-5 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 48 2022 1 04 08
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topic_title	Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index Mucolytic agents (dpeaa)DE-He213 LCI (dpeaa)DE-He213 Children (dpeaa)DE-He213 Lung function (dpeaa)DE-He213
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title	Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index
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title_full	Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index
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title_sort	dornase alfa in cystic fibrosis: indications, comparative studies and effects on lung clearance index
title_auth	Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index
abstract	Abstract Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF trans-membrane conductance regulator (CFTR) gene changes a protein (a regulated chloride channel), which is expressed in many tissues. Defective CFTR results in reduced chloride secretion and an overage absorption of sodium across the epithelia, leading to thickened secretions in organs such as pancreas and lung. Gradually, there have been considerable improvements in the survival of people with CF, thanks to substantial changes in specialized CF care and the discovery of new CFTR modulators drugs. Nevertheless, lung disease remains the most common cause of death. For these reasons improvement of sputum clearance is a major therapeutic aim in CF. So far, symptomatic mucolytic therapy is mainly based on inhalation of dornase alfa, hypertonic saline or mannitol, in combination with physiotherapy. The major component of mucus in CF is pus including viscous material such as polymerized DNA derived from degraded neutrophils. Dornase alfa cleaves the DNA released from the neutrophils and reduces mucous viscosity, and further prevent airway infections and damage to the lung parenchyma. In this review we will summarize the current knowledge on dornase alfa in the treatment of CF lung disease, especially highlighting the positive effect on lung clearance index, a sensitive measure of ventilation inhomogeneity. © The Author(s) 2022
abstractGer	Abstract Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF trans-membrane conductance regulator (CFTR) gene changes a protein (a regulated chloride channel), which is expressed in many tissues. Defective CFTR results in reduced chloride secretion and an overage absorption of sodium across the epithelia, leading to thickened secretions in organs such as pancreas and lung. Gradually, there have been considerable improvements in the survival of people with CF, thanks to substantial changes in specialized CF care and the discovery of new CFTR modulators drugs. Nevertheless, lung disease remains the most common cause of death. For these reasons improvement of sputum clearance is a major therapeutic aim in CF. So far, symptomatic mucolytic therapy is mainly based on inhalation of dornase alfa, hypertonic saline or mannitol, in combination with physiotherapy. The major component of mucus in CF is pus including viscous material such as polymerized DNA derived from degraded neutrophils. Dornase alfa cleaves the DNA released from the neutrophils and reduces mucous viscosity, and further prevent airway infections and damage to the lung parenchyma. In this review we will summarize the current knowledge on dornase alfa in the treatment of CF lung disease, especially highlighting the positive effect on lung clearance index, a sensitive measure of ventilation inhomogeneity. © The Author(s) 2022
abstract_unstemmed	Abstract Cystic fibrosis (CF) is the most common inherited disease in Caucasian populations, affecting around 50,000 patients in Europe and 30,000 in United States. A mutation in CF trans-membrane conductance regulator (CFTR) gene changes a protein (a regulated chloride channel), which is expressed in many tissues. Defective CFTR results in reduced chloride secretion and an overage absorption of sodium across the epithelia, leading to thickened secretions in organs such as pancreas and lung. Gradually, there have been considerable improvements in the survival of people with CF, thanks to substantial changes in specialized CF care and the discovery of new CFTR modulators drugs. Nevertheless, lung disease remains the most common cause of death. For these reasons improvement of sputum clearance is a major therapeutic aim in CF. So far, symptomatic mucolytic therapy is mainly based on inhalation of dornase alfa, hypertonic saline or mannitol, in combination with physiotherapy. The major component of mucus in CF is pus including viscous material such as polymerized DNA derived from degraded neutrophils. Dornase alfa cleaves the DNA released from the neutrophils and reduces mucous viscosity, and further prevent airway infections and damage to the lung parenchyma. In this review we will summarize the current knowledge on dornase alfa in the treatment of CF lung disease, especially highlighting the positive effect on lung clearance index, a sensitive measure of ventilation inhomogeneity. © The Author(s) 2022
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title_short	Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index
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Dornase alfa in Cystic Fibrosis: indications, comparative studies and effects on lung clearance index

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