{"id":"https://openalex.org/W4409105340","doi":"https://doi.org/10.36416/1806-3756/e20240406","title":"Impact of elexacaftor/tezacaftor/ivacaftor on the small airways in cystic fibrosis","display_name":"Impact of elexacaftor/tezacaftor/ivacaftor on the small airways in cystic fibrosis","publication_year":2025,"publication_date":"2025-03-31","ids":{"openalex":"https://openalex.org/W4409105340","doi":"https://doi.org/10.36416/1806-3756/e20240406","pmid":"https://pubmed.ncbi.nlm.nih.gov/40531731","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/12401089"},"language":"en","primary_location":{"id":"doi:10.36416/1806-3756/e20240406","doi":"https://doi.org/10.36416/1806-3756/e20240406","is_oa":true,"landing_page_url":"https://doi.org/10.36416/1806-3756/e20240406","pdf_url":"https://jornaldepneumologia.com.br/export-pdf/4086/2030_1_1_4086_english.pdf","source":{"id":"https://openalex.org/S143346047","display_name":"Jornal Brasileiro de Pneumologia","issn_l":"1806-3713","issn":["1806-3713","1806-3756"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","dongbi-c","jufo-1","norway-1","oarl","poland-40","russia-white-list-3","vabb-shw"],"host_organization":"https://openalex.org/P4310312248","host_organization_name":"Sociedade Brasileira de Pneumologia e Tisiologia","host_organization_lineage":["https://openalex.org/P4310312248"],"host_organization_lineage_names":["Sociedade Brasileira de Pneumologia e Tisiologia"],"type":"journal"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Jornal Brasileiro de Pneumologia","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref","doaj","pubmed"],"open_access":{"is_oa":true,"oa_status":"diamond","oa_url":"https://jornaldepneumologia.com.br/export-pdf/4086/2030_1_1_4086_english.pdf","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":null,"display_name":"Sofia Campos Silva1","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210129048","display_name":"Hospital Beatriz \u00c2ngelo","ror":"https://ror.org/03kyy9y42","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210129048"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Sofia Campos Silva1","raw_affiliation_strings":["1. Hospital Beatriz \u00c2ngelo, ULS Loures-Odivelas, Loures, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"1. Hospital Beatriz \u00c2ngelo, ULS Loures-Odivelas, Loures, Portugal","institution_ids":["https://openalex.org/I4210129048"]}]},{"author_position":"middle","author":{"id":null,"display_name":"Andreia Barroso2","orcid":null,"observed_orcids":[]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Andreia Barroso2","raw_affiliation_strings":["2. Hospital de Faro, ULS do Algarve, Faro, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"2. Hospital de Faro, ULS do Algarve, Faro, Portugal","institution_ids":[]}]},{"author_position":"middle","author":{"id":null,"display_name":"Maria Cunha3","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210140419","display_name":"Hospital Vila Franca de Xira","ror":"https://ror.org/03nk3j490","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210140419"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Maria Cunha3","raw_affiliation_strings":["3. Hospital de Vila Franca de Xira, ULS Estu\u00e1rio do Tejo, Vila Franca de Xira, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"3. Hospital de Vila Franca de Xira, ULS Estu\u00e1rio do Tejo, Vila Franca de Xira, Portugal","institution_ids":["https://openalex.org/I4210140419"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5111512593","display_name":"Elsa Fragoso","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I141596103","display_name":"University of Lisbon","ror":"https://ror.org/01c27hj86","country_code":"PT","type":"education","lineage":["https://openalex.org/I141596103"]},{"id":"https://openalex.org/I4210149283","display_name":"Hospital de Santa Maria","ror":"https://ror.org/05bz1tw26","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210149283"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Elsa Fragoso4,5","raw_affiliation_strings":["4. Hospital de Santa Maria, ULS de Santa Maria, Lisboa, Portugal. 5. Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"4. Hospital de Santa Maria, ULS de Santa Maria, Lisboa, Portugal. 5. Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal","institution_ids":["https://openalex.org/I141596103","https://openalex.org/I4210149283"]}]},{"author_position":"last","author":{"id":null,"display_name":"Pilar Azevedo4,5","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I141596103","display_name":"University of Lisbon","ror":"https://ror.org/01c27hj86","country_code":"PT","type":"education","lineage":["https://openalex.org/I141596103"]},{"id":"https://openalex.org/I4210149283","display_name":"Hospital de Santa Maria","ror":"https://ror.org/05bz1tw26","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210149283"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Pilar Azevedo4,5","raw_affiliation_strings":["4. Hospital de Santa Maria, ULS de Santa Maria, Lisboa, Portugal. 5. Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"4. Hospital de Santa Maria, ULS de Santa Maria, Lisboa, Portugal. 5. Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal","institution_ids":["https://openalex.org/I141596103","https://openalex.org/I4210149283"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":4,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":0.5303,"has_fulltext":true,"cited_by_count":1,"citation_normalized_percentile":{"value":0.57867413,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":91,"max":95},"biblio":{"volume":"51","issue":"2","first_page":"e20240406","last_page":"e20240406"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10665","display_name":"Cystic Fibrosis Research Advances","score":0.9990000128746033,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},"topics":[{"id":"https://openalex.org/T10665","display_name":"Cystic Fibrosis Research Advances","score":0.9990000128746033,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T11651","display_name":"Inhalation and Respiratory Drug Delivery","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T10143","display_name":"Chronic Obstructive Pulmonary Disease (COPD) Research","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2740","display_name":"Pulmonary and Respiratory Medicine"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/cystic-fibrosis","display_name":"cystic fibrosis","score":0.9179999828338623},{"id":"https://openalex.org/keywords/elexacaftor-tezacaftor-ivacaftor","display_name":"elexacaftor/tezacaftor/ivacaftor","score":0.9980000257492065},{"id":"https://openalex.org/keywords/cftr-modulator-therapy","display_name":"CFTR modulator therapy","score":0.9459999799728394},{"id":"https://openalex.org/keywords/small-airways","display_name":"small airways","score":0.9160000085830688},{"id":"https://openalex.org/keywords/f508del-mutation","display_name":"F508del mutation","score":0.9269999861717224},{"id":"https://openalex.org/keywords/lung-function","display_name":"lung function","score":0.8709999918937683},{"id":"https://openalex.org/keywords/ground-glass-opacities","display_name":"ground-glass opacities","score":0.7799999713897705},{"id":"https://openalex.org/keywords/advanced-lung-disease","display_name":"advanced lung disease","score":0.8349999785423279},{"id":"https://openalex.org/keywords/fev1","display_name":"FEV1","score":0.7099999785423279}],"concepts":[{"id":"https://openalex.org/C2776502428","wikidata":"https://www.wikidata.org/wiki/Q6095693","display_name":"Ivacaftor","level":4,"score":0.9734488725662231},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.9452908039093018},{"id":"https://openalex.org/C2776938444","wikidata":"https://www.wikidata.org/wiki/Q178194","display_name":"Cystic fibrosis","level":2,"score":0.8531620502471924},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.622434675693512},{"id":"https://openalex.org/C2778428886","wikidata":"https://www.wikidata.org/wiki/Q420470","display_name":"Cystic fibrosis transmembrane conductance regulator","level":3,"score":0.5726791620254517},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.5367717742919922},{"id":"https://openalex.org/C2776301714","wikidata":"https://www.wikidata.org/wiki/Q259346","display_name":"Sputum","level":3,"score":0.522351861000061},{"id":"https://openalex.org/C72563966","wikidata":"https://www.wikidata.org/wiki/Q1303415","display_name":"Cohort","level":2,"score":0.4441729485988617},{"id":"https://openalex.org/C3018587741","wikidata":"https://www.wikidata.org/wiki/Q7886","display_name":"Lung function","level":3,"score":0.44414564967155457},{"id":"https://openalex.org/C2777714996","wikidata":"https://www.wikidata.org/wiki/Q7886","display_name":"Lung","level":2,"score":0.39863574504852295},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.23724046349525452},{"id":"https://openalex.org/C2781069245","wikidata":"https://www.wikidata.org/wiki/Q12204","display_name":"Tuberculosis","level":2,"score":0.06561052799224854}],"mesh":[{"descriptor_ui":"D000293","descriptor_name":"Adolescent","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000328","descriptor_name":"Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000627","descriptor_name":"Aminophenols","qualifier_ui":"Q000008","qualifier_name":"administration & dosage","is_major_topic":true},{"descriptor_ui":"D000627","descriptor_name":"Aminophenols","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D003550","descriptor_name":"Cystic Fibrosis","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":true},{"descriptor_ui":"D003550","descriptor_name":"Cystic Fibrosis","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":true},{"descriptor_ui":"D003550","descriptor_name":"Cystic Fibrosis","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":true},{"descriptor_ui":"D004338","descriptor_name":"Drug Combinations","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D005541","descriptor_name":"Forced Expiratory Volume","qualifier_ui":"Q000187","qualifier_name":"drug effects","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007211","descriptor_name":"Indoles","qualifier_ui":"Q000008","qualifier_name":"administration & dosage","is_major_topic":true},{"descriptor_ui":"D007211","descriptor_name":"Indoles","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008875","descriptor_name":"Middle Aged","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D009154","descriptor_name":"Mutation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D011720","descriptor_name":"Pyrazoles","qualifier_ui":"Q000008","qualifier_name":"administration & dosage","is_major_topic":true},{"descriptor_ui":"D011720","descriptor_name":"Pyrazoles","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D011725","descriptor_name":"Pyridines","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D011759","descriptor_name":"Pyrrolidines","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D011804","descriptor_name":"Quinolines","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D012129","descriptor_name":"Respiratory Function Tests","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D012189","descriptor_name":"Retrospective Studies","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D015363","descriptor_name":"Quinolones","qualifier_ui":"Q000008","qualifier_name":"administration & dosage","is_major_topic":true},{"descriptor_ui":"D015363","descriptor_name":"Quinolones","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D016896","descriptor_name":"Treatment Outcome","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D019005","descriptor_name":"Cystic Fibrosis Transmembrane Conductance Regulator","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D052117","descriptor_name":"Benzodioxoles","qualifier_ui":"Q000008","qualifier_name":"administration & dosage","is_major_topic":true},{"descriptor_ui":"D052117","descriptor_name":"Benzodioxoles","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D055815","descriptor_name":"Young Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D065101","descriptor_name":"Chloride Channel Agonists","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false}],"locations_count":6,"locations":[{"id":"doi:10.36416/1806-3756/e20240406","doi":"https://doi.org/10.36416/1806-3756/e20240406","is_oa":true,"landing_page_url":"https://doi.org/10.36416/1806-3756/e20240406","pdf_url":"https://jornaldepneumologia.com.br/export-pdf/4086/2030_1_1_4086_english.pdf","source":{"id":"https://openalex.org/S143346047","display_name":"Jornal Brasileiro de Pneumologia","issn_l":"1806-3713","issn":["1806-3713","1806-3756"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","dongbi-c","jufo-1","norway-1","oarl","poland-40","russia-white-list-3","vabb-shw"],"host_organization":"https://openalex.org/P4310312248","host_organization_name":"Sociedade Brasileira de Pneumologia e Tisiologia","host_organization_lineage":["https://openalex.org/P4310312248"],"host_organization_lineage_names":["Sociedade Brasileira de Pneumologia e Tisiologia"],"type":"journal"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Jornal Brasileiro de Pneumologia","raw_type":"journal-article"},{"id":"pmh:oai:europepmc.org:11211658","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/12401089","pdf_url":null,"source":{"id":"https://openalex.org/S4306400806","display_name":"Europe PMC (PubMed Central)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1303153112","host_organization_name":"European Bioinformatics Institute","host_organization_lineage":["https://openalex.org/I1303153112"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":null,"raw_type":"Text"},{"id":"pmid:40531731","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/40531731","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia","raw_type":"Journal Article"},{"id":"pmh:oai:pubmedcentral.nih.gov:12401089","doi":null,"is_oa":true,"landing_page_url":"https://pmc.ncbi.nlm.nih.gov/articles/PMC12401089/","pdf_url":null,"source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"J Bras Pneumol","raw_type":"Text"},{"id":"pmh:oai:doaj.org/article:d7c59ed3f90944818fb82de506130d18","doi":null,"is_oa":true,"landing_page_url":"https://doaj.org/article/d7c59ed3f90944818fb82de506130d18","pdf_url":null,"source":{"id":"https://openalex.org/S4306401280","display_name":"DOAJ (DOAJ: Directory of Open Access Journals)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by-sa","license_id":"https://openalex.org/licenses/cc-by-sa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Jornal Brasileiro de Pneumologia, Vol 51, Iss 2 (2025)","raw_type":"article"},{"id":"pmh:oai:sapientia.ualg.pt:10400.1/27316","doi":null,"is_oa":true,"landing_page_url":"http://hdl.handle.net/10400.1/27316","pdf_url":null,"source":{"id":"https://openalex.org/S7407051323","display_name":"Universidade do Algarve","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":"cc-by","license_id":"https://openalex.org/licenses/cc-by","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":null,"raw_type":"journal article"}],"best_oa_location":{"id":"doi:10.36416/1806-3756/e20240406","doi":"https://doi.org/10.36416/1806-3756/e20240406","is_oa":true,"landing_page_url":"https://doi.org/10.36416/1806-3756/e20240406","pdf_url":"https://jornaldepneumologia.com.br/export-pdf/4086/2030_1_1_4086_english.pdf","source":{"id":"https://openalex.org/S143346047","display_name":"Jornal Brasileiro de Pneumologia","issn_l":"1806-3713","issn":["1806-3713","1806-3756"],"is_oa":true,"is_in_doaj":true,"is_core":true,"listed_in":["cwts-core","doaj","dongbi-c","jufo-1","norway-1","oarl","poland-40","russia-white-list-3","vabb-shw"],"host_organization":"https://openalex.org/P4310312248","host_organization_name":"Sociedade Brasileira de Pneumologia e Tisiologia","host_organization_lineage":["https://openalex.org/P4310312248"],"host_organization_lineage_names":["Sociedade Brasileira de Pneumologia e Tisiologia"],"type":"journal"},"license":"cc-by-nc","license_id":"https://openalex.org/licenses/cc-by-nc","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Jornal Brasileiro de Pneumologia","raw_type":"journal-article"},"sustainable_development_goals":[{"display_name":"Good health and well-being","score":1.0,"id":"https://metadata.un.org/sdg/3"}],"study_designs":[{"display_name":"Observational Study","id":"https://openalex.org/study-designs/observational-study"}],"awards":[],"funders":[],"has_content":{"grobid_xml":true,"pdf":true},"content_urls":{"pdf":"https://content.openalex.org/works/W4409105340.pdf","grobid_xml":"https://content.openalex.org/works/W4409105340.grobid-xml"},"referenced_works_count":10,"referenced_works":["https://openalex.org/W2982296199","https://openalex.org/W2985405196","https://openalex.org/W3089095538","https://openalex.org/W3214022819","https://openalex.org/W4210526629","https://openalex.org/W4211056322","https://openalex.org/W4385372187","https://openalex.org/W4387746862","https://openalex.org/W4389726630","https://openalex.org/W4404634786"],"related_works":["https://openalex.org/W4410997472","https://openalex.org/W7083022898","https://openalex.org/W4415010355","https://openalex.org/W4387970741","https://openalex.org/W4404103506","https://openalex.org/W4416983528","https://openalex.org/W7116642133","https://openalex.org/W4319072614","https://openalex.org/W4313254968","https://openalex.org/W2941835465"],"abstract_inverted_index":{"OBJECTIVE:":[0],"To":[1],"evaluate":[2,263],"the":[3,6,11,26,36,74,80,84,108,112,119,122,124,165,175,190,206,209,223,230,264],"impact":[4,232],"of":[5,62,83,101,114,161,205,222,267],"elexacaftor/tezacaftor/ivacaftor":[7],"(ELX/TEZ/IVA)":[8],"combination":[9],"on":[10,238],"small":[12,37,239],"airways":[13,38],"in":[14,99,130,138,154,164,174,197,203,220,242,260],"adults":[15,63],"with":[16,64,67,91,157,211,244,246,252,273],"cystic":[17],"fibrosis":[18],"(CF),":[19],"a":[20,40,57,150,158,171,184,187],"genetic":[21],"disorder":[22],"that":[23,97,233],"primarily":[24],"affects":[25],"respiratory":[27],"system,":[28],"leading":[29],"to":[30,44,262],"progressive":[31],"lung":[32,102,135,155,213,254],"disease.":[33,255],"In":[34],"CF,":[35,245],"play":[39],"critical":[41],"role,":[42],"contributing":[43],"chronic":[45],"symptoms":[46],"such":[47],"as":[48],"cough,":[49],"sputum":[50],"production,":[51],"and":[52,107,111,137,170,270],"dyspnea.":[53],"METHODS:":[54],"This":[55],"was":[56,149,183,201],"single-center,":[58],"retrospective":[59],"observational":[60],"study":[61],"CF":[65,85],"treated":[66],"ELX/TEZ/IVA":[68,234],"for":[69,79,96,250],"12":[70],"months.":[71],"We":[72],"compared":[73],"patients":[75,120,210,243],"who":[76,93,132,140],"were":[77,94,127,216],"homozygous":[78,192],"F508del":[81,191],"mutation":[82],"transmembrane":[86],"conductance":[87],"regulator":[88],"(CFTR)":[89],"gene":[90],"those":[92,131,139,251],"heterozygous":[95],"mutation,":[98],"terms":[100],"function":[103,241],"outcomes":[104],"(FEV1,":[105],"FEF25-75%,":[106],"RV/TLC":[109,177],"ratio)":[110],"extent":[113],"non-homogeneous":[115,198],"ground-glass":[116,199],"opacity.":[117],"Among":[118,208],"within":[121],"cohort,":[123],"same":[125],"parameters":[126,224],"evaluated":[128],"separately":[129],"had":[133,141],"advanced":[134,212,253],"disease":[136],"previously":[142],"undergone":[143],"CFTR":[144],"modulator":[145],"therapy.":[146],"RESULTS:":[147],"There":[148,182],"significant":[151,195],"post-treatment":[152,218],"improvement":[153,189],"function,":[156],"median":[159],"increase":[160],"0.42":[162],"L/s":[163],"FEF25-75%":[166],"(p":[167,179],"<":[168,180],"0.001)":[169],"5%":[172],"reduction":[173,196],"mean":[176],"ratio":[178],"0.001).":[181],"trend":[185],"toward":[186],"higher":[188],"patients.":[193,207],"A":[194],"opacity":[200],"observed":[202],"79.5%":[204],"disease,":[214],"there":[215],"notable":[217],"improvements":[219],"all":[221],"assessed.":[225],"CONCLUSIONS:":[226],"Our":[227],"results":[228],"highlight":[229],"positive":[231],"treatment":[235,269],"can":[236],"have":[237],"airway":[240],"potential":[247],"benefits":[248],"even":[249],"Further":[256],"research":[257],"is":[258],"needed":[259],"order":[261],"long-term":[265],"effects":[266],"this":[268],"its":[271],"relationship":[272],"patient-reported":[274],"outcomes.":[275]},"counts_by_year":[{"year":2026,"cited_by_count":1}],"updated_date":"2026-11-12T12:28:57.059296","created_date":"2025-04-03T00:00:00"}