A prospective study to assess the impact of a novel CFTR therapy combination on body composition in patients with cystic fibrosis with F508del mutation
Malnutrition is a prevalent condition in Cystic Fibrosis (CF) and can result in worsening of pulmonary function and other comorbidities. Cystic fibrosis transmembrane regulator (CFTR) modulator therapies are improving the CF-related care and outcomes. Body Mass Index (BMI) is the most commonly used...
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| Veröffentlicht in: | Clinical nutrition (Edinburgh, Scotland) Scotland), 2023-12, Vol.42 (12), p.2468-2474 |
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| creator | Knott-Torcal, Carolina Sebastián-Valles, Fernando Girón Moreno, Rosa María Martín- Adán, José Carlos Jiménez-Díaz, Jessica Marazuela, Mónica Sánchez de la Blanca, Nuria Fernández-Contreras, Raúl Arranz-Martín, Alfonso |
| description | Malnutrition is a prevalent condition in Cystic Fibrosis (CF) and can result in worsening of pulmonary function and other comorbidities. Cystic fibrosis transmembrane regulator (CFTR) modulator therapies are improving the CF-related care and outcomes. Body Mass Index (BMI) is the most commonly used parameter to assess nutritional status, albeit it is a very unspecific indicator. Hence, current guidelines recommend body composition analysis as a part of nutritional assessment. The aim of our study was to evaluate the impact of elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA) treatment on body composition and respiratory function.
We recruited patients with CF from University Hospital La Princesa, with follow-up in the Adult Cystic Fibrosis Unit. All patients were eligible to initiate ELX/TEZ/IVA therapy. Body composition was assessed with a Bioelectrical Impedance Analysis (BIA) and spirometry data were obtained before and after 6 months of treatment.
Our study sample was composed of 36 patients with CF. We observed a significant increase in BMI after 6 months of treatment (p |
| doi_str_mv | 10.1016/j.clnu.2023.10.015 |
| format | Article |
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We recruited patients with CF from University Hospital La Princesa, with follow-up in the Adult Cystic Fibrosis Unit. All patients were eligible to initiate ELX/TEZ/IVA therapy. Body composition was assessed with a Bioelectrical Impedance Analysis (BIA) and spirometry data were obtained before and after 6 months of treatment.
Our study sample was composed of 36 patients with CF. We observed a significant increase in BMI after 6 months of treatment (p<0.001), as well as an increase in fat mass (p=0.008) and visceral fat area (p=0.026). The other body composition parameters did not yield significant changes. Overall, %FEV1 increased from 72.67% (±17.39) to 84.74% (±18.18) after 6 months of treatment. Interestingly, we found an inverse correlation between %FEV1 and fat mass (r= -0,476; p= 0,0058), %FEV1 and age (r= -0,411; p= 0,0196) and between %FEV1 and visceral fat area (r= -0,515; p= 0,0025). On the contrary, we found a direct correlation between %FEV1 and body cell mass (r= 0,367; p= 0,038).
Novel CFTR modulators are emerging for the treatment of CF. Specifically, triple combination with ELX/TEZ/IVA has shown to effectively improve both pulmonary and nutritional status in patients with CF with F508del mutation. Body composition should be a part of the routine assessment for patients with CF.</description><identifier>ISSN: 0261-5614</identifier><identifier>EISSN: 1532-1983</identifier><identifier>DOI: 10.1016/j.clnu.2023.10.015</identifier><identifier>PMID: 38411018</identifier><language>eng</language><publisher>England: Elsevier Ltd</publisher><subject>Adult ; adults ; Benzodioxoles - therapeutic use ; bioelectrical impedance ; Body Composition ; Body Mass Index ; CFTR modulator ; clinical nutrition ; Cystic fibrosis ; Cystic Fibrosis - complications ; Cystic Fibrosis - drug therapy ; Cystic Fibrosis - genetics ; Cystic Fibrosis Transmembrane Conductance Regulator - genetics ; hospitals ; Humans ; lung function ; malnutrition ; Mutation ; nutrition assessment ; nutritional status ; Prospective Studies ; therapeutics ; visceral fat</subject><ispartof>Clinical nutrition (Edinburgh, Scotland), 2023-12, Vol.42 (12), p.2468-2474</ispartof><rights>2023</rights><rights>Copyright © 2023. Published by Elsevier Ltd.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c389t-46519fd6474d841b9e22fdfd3c0d0bf374b5473caf11bcda45cc3bbb2a2afed93</citedby><cites>FETCH-LOGICAL-c389t-46519fd6474d841b9e22fdfd3c0d0bf374b5473caf11bcda45cc3bbb2a2afed93</cites><orcidid>0000-0002-4127-0483 ; 0000-0001-9087-3355 ; 0000-0002-8516-2695 ; 0000-0001-5829-1582 ; 0000-0002-0140-1522 ; 0000-0001-7094-0848</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/38411018$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Knott-Torcal, Carolina</creatorcontrib><creatorcontrib>Sebastián-Valles, Fernando</creatorcontrib><creatorcontrib>Girón Moreno, Rosa María</creatorcontrib><creatorcontrib>Martín- Adán, José Carlos</creatorcontrib><creatorcontrib>Jiménez-Díaz, Jessica</creatorcontrib><creatorcontrib>Marazuela, Mónica</creatorcontrib><creatorcontrib>Sánchez de la Blanca, Nuria</creatorcontrib><creatorcontrib>Fernández-Contreras, Raúl</creatorcontrib><creatorcontrib>Arranz-Martín, Alfonso</creatorcontrib><title>A prospective study to assess the impact of a novel CFTR therapy combination on body composition in patients with cystic fibrosis with F508del mutation</title><title>Clinical nutrition (Edinburgh, Scotland)</title><addtitle>Clin Nutr</addtitle><description>Malnutrition is a prevalent condition in Cystic Fibrosis (CF) and can result in worsening of pulmonary function and other comorbidities. Cystic fibrosis transmembrane regulator (CFTR) modulator therapies are improving the CF-related care and outcomes. Body Mass Index (BMI) is the most commonly used parameter to assess nutritional status, albeit it is a very unspecific indicator. Hence, current guidelines recommend body composition analysis as a part of nutritional assessment. The aim of our study was to evaluate the impact of elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA) treatment on body composition and respiratory function.
We recruited patients with CF from University Hospital La Princesa, with follow-up in the Adult Cystic Fibrosis Unit. All patients were eligible to initiate ELX/TEZ/IVA therapy. Body composition was assessed with a Bioelectrical Impedance Analysis (BIA) and spirometry data were obtained before and after 6 months of treatment.
Our study sample was composed of 36 patients with CF. We observed a significant increase in BMI after 6 months of treatment (p<0.001), as well as an increase in fat mass (p=0.008) and visceral fat area (p=0.026). The other body composition parameters did not yield significant changes. Overall, %FEV1 increased from 72.67% (±17.39) to 84.74% (±18.18) after 6 months of treatment. Interestingly, we found an inverse correlation between %FEV1 and fat mass (r= -0,476; p= 0,0058), %FEV1 and age (r= -0,411; p= 0,0196) and between %FEV1 and visceral fat area (r= -0,515; p= 0,0025). On the contrary, we found a direct correlation between %FEV1 and body cell mass (r= 0,367; p= 0,038).
Novel CFTR modulators are emerging for the treatment of CF. Specifically, triple combination with ELX/TEZ/IVA has shown to effectively improve both pulmonary and nutritional status in patients with CF with F508del mutation. Body composition should be a part of the routine assessment for patients with CF.</description><subject>Adult</subject><subject>adults</subject><subject>Benzodioxoles - therapeutic use</subject><subject>bioelectrical impedance</subject><subject>Body Composition</subject><subject>Body Mass Index</subject><subject>CFTR modulator</subject><subject>clinical nutrition</subject><subject>Cystic fibrosis</subject><subject>Cystic Fibrosis - complications</subject><subject>Cystic Fibrosis - drug therapy</subject><subject>Cystic Fibrosis - genetics</subject><subject>Cystic Fibrosis Transmembrane Conductance Regulator - genetics</subject><subject>hospitals</subject><subject>Humans</subject><subject>lung function</subject><subject>malnutrition</subject><subject>Mutation</subject><subject>nutrition assessment</subject><subject>nutritional status</subject><subject>Prospective Studies</subject><subject>therapeutics</subject><subject>visceral fat</subject><issn>0261-5614</issn><issn>1532-1983</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFUctq3DAUFaEhmTx-IIugZTee6mWPDd2EIdMUAoGQrIWeRINtuZY8Zb6kv9vrmWmXLQgE555zLvcchO4oWVJCqy_bpWn7ackI4wAsCS3P0IKWnBW0qfkntCCsokVZUXGJrlLaEkJKvqov0CWvBQWLeoF-PeBhjGlwJoedwylPdo9zxCollxLOHw6HblAm4-ixwn3cuRavN2-v82hUwx6b2OnQqxxij-HpaA_YEFM4YKHHA0xdnxP-GfIHNvuUg8E-aFgcTuCmJLUF627KB6sbdO5Vm9zt6b9G75vHt_VT8fzy7fv64bkwvG5yIaqSNt5WYiUs3KQbx5i33nJDLNGer4QuxYob5SnVxipRGsO11kwx5Z1t-DX6fPSFFH5MLmXZhWRc26rexSlJTgQRrKoa8V8qazgTvAYFUNmRauDENDovhzF0atxLSuTcndzKuTs5dzdj0B2I7k_-k-6c_Sv5UxYQvh4JDgLZBTfKZCBX42wYoT9pY_iX_2_pD62z</recordid><startdate>202312</startdate><enddate>202312</enddate><creator>Knott-Torcal, Carolina</creator><creator>Sebastián-Valles, Fernando</creator><creator>Girón Moreno, Rosa María</creator><creator>Martín- Adán, José Carlos</creator><creator>Jiménez-Díaz, Jessica</creator><creator>Marazuela, Mónica</creator><creator>Sánchez de la Blanca, Nuria</creator><creator>Fernández-Contreras, Raúl</creator><creator>Arranz-Martín, Alfonso</creator><general>Elsevier Ltd</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>7S9</scope><scope>L.6</scope><orcidid>https://orcid.org/0000-0002-4127-0483</orcidid><orcidid>https://orcid.org/0000-0001-9087-3355</orcidid><orcidid>https://orcid.org/0000-0002-8516-2695</orcidid><orcidid>https://orcid.org/0000-0001-5829-1582</orcidid><orcidid>https://orcid.org/0000-0002-0140-1522</orcidid><orcidid>https://orcid.org/0000-0001-7094-0848</orcidid></search><sort><creationdate>202312</creationdate><title>A prospective study to assess the impact of a novel CFTR therapy combination on body composition in patients with cystic fibrosis with F508del mutation</title><author>Knott-Torcal, Carolina ; Sebastián-Valles, Fernando ; Girón Moreno, Rosa María ; Martín- Adán, José Carlos ; Jiménez-Díaz, Jessica ; Marazuela, Mónica ; Sánchez de la Blanca, Nuria ; Fernández-Contreras, Raúl ; Arranz-Martín, Alfonso</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c389t-46519fd6474d841b9e22fdfd3c0d0bf374b5473caf11bcda45cc3bbb2a2afed93</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>Adult</topic><topic>adults</topic><topic>Benzodioxoles - therapeutic use</topic><topic>bioelectrical impedance</topic><topic>Body Composition</topic><topic>Body Mass Index</topic><topic>CFTR modulator</topic><topic>clinical nutrition</topic><topic>Cystic fibrosis</topic><topic>Cystic Fibrosis - complications</topic><topic>Cystic Fibrosis - drug therapy</topic><topic>Cystic Fibrosis - genetics</topic><topic>Cystic Fibrosis Transmembrane Conductance Regulator - genetics</topic><topic>hospitals</topic><topic>Humans</topic><topic>lung function</topic><topic>malnutrition</topic><topic>Mutation</topic><topic>nutrition assessment</topic><topic>nutritional status</topic><topic>Prospective Studies</topic><topic>therapeutics</topic><topic>visceral fat</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Knott-Torcal, Carolina</creatorcontrib><creatorcontrib>Sebastián-Valles, Fernando</creatorcontrib><creatorcontrib>Girón Moreno, Rosa María</creatorcontrib><creatorcontrib>Martín- Adán, José Carlos</creatorcontrib><creatorcontrib>Jiménez-Díaz, Jessica</creatorcontrib><creatorcontrib>Marazuela, Mónica</creatorcontrib><creatorcontrib>Sánchez de la Blanca, Nuria</creatorcontrib><creatorcontrib>Fernández-Contreras, Raúl</creatorcontrib><creatorcontrib>Arranz-Martín, Alfonso</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>AGRICOLA</collection><collection>AGRICOLA - Academic</collection><jtitle>Clinical nutrition (Edinburgh, Scotland)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Knott-Torcal, Carolina</au><au>Sebastián-Valles, Fernando</au><au>Girón Moreno, Rosa María</au><au>Martín- Adán, José Carlos</au><au>Jiménez-Díaz, Jessica</au><au>Marazuela, Mónica</au><au>Sánchez de la Blanca, Nuria</au><au>Fernández-Contreras, Raúl</au><au>Arranz-Martín, Alfonso</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>A prospective study to assess the impact of a novel CFTR therapy combination on body composition in patients with cystic fibrosis with F508del mutation</atitle><jtitle>Clinical nutrition (Edinburgh, Scotland)</jtitle><addtitle>Clin Nutr</addtitle><date>2023-12</date><risdate>2023</risdate><volume>42</volume><issue>12</issue><spage>2468</spage><epage>2474</epage><pages>2468-2474</pages><issn>0261-5614</issn><eissn>1532-1983</eissn><abstract>Malnutrition is a prevalent condition in Cystic Fibrosis (CF) and can result in worsening of pulmonary function and other comorbidities. Cystic fibrosis transmembrane regulator (CFTR) modulator therapies are improving the CF-related care and outcomes. Body Mass Index (BMI) is the most commonly used parameter to assess nutritional status, albeit it is a very unspecific indicator. Hence, current guidelines recommend body composition analysis as a part of nutritional assessment. The aim of our study was to evaluate the impact of elexacaftor-tezacaftor-ivacaftor (ELX/TEZ/IVA) treatment on body composition and respiratory function.
We recruited patients with CF from University Hospital La Princesa, with follow-up in the Adult Cystic Fibrosis Unit. All patients were eligible to initiate ELX/TEZ/IVA therapy. Body composition was assessed with a Bioelectrical Impedance Analysis (BIA) and spirometry data were obtained before and after 6 months of treatment.
Our study sample was composed of 36 patients with CF. We observed a significant increase in BMI after 6 months of treatment (p<0.001), as well as an increase in fat mass (p=0.008) and visceral fat area (p=0.026). The other body composition parameters did not yield significant changes. Overall, %FEV1 increased from 72.67% (±17.39) to 84.74% (±18.18) after 6 months of treatment. Interestingly, we found an inverse correlation between %FEV1 and fat mass (r= -0,476; p= 0,0058), %FEV1 and age (r= -0,411; p= 0,0196) and between %FEV1 and visceral fat area (r= -0,515; p= 0,0025). On the contrary, we found a direct correlation between %FEV1 and body cell mass (r= 0,367; p= 0,038).
Novel CFTR modulators are emerging for the treatment of CF. Specifically, triple combination with ELX/TEZ/IVA has shown to effectively improve both pulmonary and nutritional status in patients with CF with F508del mutation. Body composition should be a part of the routine assessment for patients with CF.</abstract><cop>England</cop><pub>Elsevier Ltd</pub><pmid>38411018</pmid><doi>10.1016/j.clnu.2023.10.015</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0002-4127-0483</orcidid><orcidid>https://orcid.org/0000-0001-9087-3355</orcidid><orcidid>https://orcid.org/0000-0002-8516-2695</orcidid><orcidid>https://orcid.org/0000-0001-5829-1582</orcidid><orcidid>https://orcid.org/0000-0002-0140-1522</orcidid><orcidid>https://orcid.org/0000-0001-7094-0848</orcidid></addata></record> |
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| ispartof | Clinical nutrition (Edinburgh, Scotland), 2023-12, Vol.42 (12), p.2468-2474 |
| issn | 0261-5614 1532-1983 |
| language | eng |
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| source | MEDLINE; Elsevier ScienceDirect Journals |
| subjects | Adult adults Benzodioxoles - therapeutic use bioelectrical impedance Body Composition Body Mass Index CFTR modulator clinical nutrition Cystic fibrosis Cystic Fibrosis - complications Cystic Fibrosis - drug therapy Cystic Fibrosis - genetics Cystic Fibrosis Transmembrane Conductance Regulator - genetics hospitals Humans lung function malnutrition Mutation nutrition assessment nutritional status Prospective Studies therapeutics visceral fat |
| title | A prospective study to assess the impact of a novel CFTR therapy combination on body composition in patients with cystic fibrosis with F508del mutation |
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