{"id":"https://openalex.org/W4387980337","doi":"https://doi.org/10.1183/13993003.congress-2023.pa2157","title":"Sweat Chloride Concentration as a Surrogate Marker of CFTR Modulator Therapy Response","display_name":"Sweat Chloride Concentration as a Surrogate Marker of CFTR Modulator Therapy Response","publication_year":2023,"publication_date":"2023-09-09","ids":{"openalex":"https://openalex.org/W4387980337","doi":"https://doi.org/10.1183/13993003.congress-2023.pa2157"},"language":"en","primary_location":{"id":"doi:10.1183/13993003.congress-2023.pa2157","doi":"https://doi.org/10.1183/13993003.congress-2023.pa2157","is_oa":false,"landing_page_url":"https://doi.org/10.1183/13993003.congress-2023.pa2157","pdf_url":null,"source":{"id":"https://openalex.org/S4210207896","display_name":"European Respiratory Journal","issn_l":"0903-1936","issn":["0903-1936","1399-3003"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310317833","host_organization_name":"European Respiratory Society","host_organization_lineage":["https://openalex.org/P4310317833"],"host_organization_lineage_names":["European Respiratory Society"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Adult CF","raw_type":"proceedings-article"},"volume":null,"type":"conference-abstract","is_traditional":false,"indexed_in":["crossref"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5073170501","display_name":"Am\u00e9lia Simas Ribeiro","orcid":"https://orcid.org/0000-0002-5150-8395","observed_orcids":["https://orcid.org/0000-0002-5150-8395"]},"institutions":[],"countries":[],"is_corresponding":false,"raw_author_name":"Am\u00e9lia Simas Ribeiro","raw_affiliation_strings":["Centro Hospitalar Barreiro Montijo, Barreiro, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Centro Hospitalar Barreiro Montijo, Barreiro, Portugal","institution_ids":[]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5019952468","display_name":"Pilar Azevedo","orcid":"https://orcid.org/0000-0001-7609-200X","observed_orcids":["https://orcid.org/0000-0001-7609-200X"]},"institutions":[{"id":"https://openalex.org/I4210114390","display_name":"Centro Hospitalar Lisboa Norte","ror":"https://ror.org/020sr6z07","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210114390","https://openalex.org/I4405255475"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Pilar Azevedo","raw_affiliation_strings":["Centro Hospitalar Universit\u00e1rio Lisboa Norte, Lisboa, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Centro Hospitalar Universit\u00e1rio Lisboa Norte, Lisboa, Portugal","institution_ids":["https://openalex.org/I4210114390"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5036229647","display_name":"Carlos Lopes","orcid":"https://orcid.org/0000-0001-7901-5720","observed_orcids":["https://orcid.org/0000-0001-7901-5720"]},"institutions":[{"id":"https://openalex.org/I4210114390","display_name":"Centro Hospitalar Lisboa Norte","ror":"https://ror.org/020sr6z07","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210114390","https://openalex.org/I4405255475"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Carlos Lopes","raw_affiliation_strings":["Centro Hospitalar Universit\u00e1rio Lisboa Norte, Lisboa, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Centro Hospitalar Universit\u00e1rio Lisboa Norte, Lisboa, Portugal","institution_ids":["https://openalex.org/I4210114390"]}]},{"author_position":"last","author":{"id":null,"display_name":"Patr\u00edcia Veloso","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I4210114390","display_name":"Centro Hospitalar Lisboa Norte","ror":"https://ror.org/020sr6z07","country_code":"PT","type":"healthcare","lineage":["https://openalex.org/I4210114390","https://openalex.org/I4405255475"]}],"countries":["PT"],"is_corresponding":false,"raw_author_name":"Patr\u00edcia Veloso","raw_affiliation_strings":["Centro Hospitalar Universit\u00e1rio Lisboa Norte, Lisboa, Portugal"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Centro Hospitalar Universit\u00e1rio Lisboa Norte, Lisboa, Portugal","institution_ids":["https://openalex.org/I4210114390"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":1,"corresponding_author_ids":[],"corresponding_institution_ids":[],"apc_list":null,"apc_paid":null,"fwci":null,"has_fulltext":false,"cited_by_count":2,"citation_normalized_percentile":null,"cited_by_percentile_year":null,"biblio":{"volume":null,"issue":null,"first_page":"PA2157","last_page":"PA2157"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T10665","display_name":"Cystic Fibrosis Research Advances","score":1.0,"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":1.0,"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":0.0,"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":0.0,"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":0.8669999837875366},{"id":"https://openalex.org/keywords/cftr-modulator-therapy","display_name":"CFTR modulator therapy","score":0.949999988079071},{"id":"https://openalex.org/keywords/elexacaftor-tezacaftor-ivacaftor","display_name":"elexacaftor/tezacaftor/ivacaftor","score":0.9919999837875366},{"id":"https://openalex.org/keywords/f508del-mutation","display_name":"F508del mutation","score":0.9829999804496765},{"id":"https://openalex.org/keywords/surrogate-markers","display_name":"surrogate markers","score":0.6110000014305115},{"id":"https://openalex.org/keywords/treatment-response","display_name":"treatment response","score":0.9340000152587891},{"id":"https://openalex.org/keywords/fev1","display_name":"FEV1","score":0.7129999995231628},{"id":"https://openalex.org/keywords/pulmonary-exacerbations","display_name":"pulmonary exacerbations","score":0.9760000109672546},{"id":"https://openalex.org/keywords/body-mass-index","display_name":"body mass index","score":0.8899999856948853}],"concepts":[{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.7419244647026062},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.6429595947265625},{"id":"https://openalex.org/C2776502428","wikidata":"https://www.wikidata.org/wiki/Q6095693","display_name":"Ivacaftor","level":4,"score":0.616546094417572},{"id":"https://openalex.org/C2780221984","wikidata":"https://www.wikidata.org/wiki/Q131191","display_name":"Body mass index","level":2,"score":0.5533443093299866},{"id":"https://openalex.org/C2776938444","wikidata":"https://www.wikidata.org/wiki/Q178194","display_name":"Cystic fibrosis","level":2,"score":0.5359903573989868},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.47730720043182373},{"id":"https://openalex.org/C141341695","wikidata":"https://www.wikidata.org/wiki/Q2368661","display_name":"Surrogate endpoint","level":2,"score":0.4444698691368103},{"id":"https://openalex.org/C2777785397","wikidata":"https://www.wikidata.org/wiki/Q492194","display_name":"Group B","level":2,"score":0.43521153926849365},{"id":"https://openalex.org/C2778428886","wikidata":"https://www.wikidata.org/wiki/Q420470","display_name":"Cystic fibrosis transmembrane conductance regulator","level":3,"score":0.2107061743736267}],"mesh":[],"locations_count":1,"locations":[{"id":"doi:10.1183/13993003.congress-2023.pa2157","doi":"https://doi.org/10.1183/13993003.congress-2023.pa2157","is_oa":false,"landing_page_url":"https://doi.org/10.1183/13993003.congress-2023.pa2157","pdf_url":null,"source":{"id":"https://openalex.org/S4210207896","display_name":"European Respiratory Journal","issn_l":"0903-1936","issn":["0903-1936","1399-3003"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-class-a","anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-3","medline","norway-2","poland-200","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310317833","host_organization_name":"European Respiratory Society","host_organization_lineage":["https://openalex.org/P4310317833"],"host_organization_lineage_names":["European Respiratory Society"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Adult CF","raw_type":"proceedings-article"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":1.0,"display_name":"Good health and well-being"}],"study_designs":[],"awards":[],"funders":[],"has_content":{"grobid_xml":false,"pdf":false},"content_urls":null,"referenced_works_count":0,"referenced_works":[],"related_works":["https://openalex.org/W4389857071","https://openalex.org/W4410997538","https://openalex.org/W7213233949","https://openalex.org/W2137497736","https://openalex.org/W4230814260","https://openalex.org/W4401272752","https://openalex.org/W4404103506","https://openalex.org/W4300716594","https://openalex.org/W2533625518","https://openalex.org/W7163180551"],"abstract_inverted_index":{"Introduction:":[0],"Elexacaftor/Tezacaftor/Ivacaftor":[1],"(ELX/TEZ/IVA)":[2],"has":[3],"proved":[4],"to":[5,53,90,162,233],"improve":[6],"lung":[7],"function":[8],"and":[9,49,111,117,187,202],"quality":[10],"of":[11,32,67,76,121,152,184,189,214,225,237,242],"life":[12],"in":[13,58,98],"cystic":[14],"fibrosis":[15],"(CF)":[16],"patients":[17,59,77,138,226],"with":[18,44],"the":[19,99,119,150,182,212,220,235],"F508del":[20],"mutation.":[21],"Sweat":[22],"chloride":[23],"concentration":[24],"(SCC)":[25],"is":[26,37,62],"used":[27],"as":[28,239],"a":[29,63,73,82,240],"surrogate":[30,65],"marker":[31,66,241],"drug":[33,243],"effectivity.":[34,244],"However,":[35],"it":[36,42],"not":[38,218],"clear":[39],"how":[40],"well":[41],"correlates":[43],"clinical":[45,221],"treatment":[46,68],"outcomes.":[47],"Aims":[48],"Objectives:":[50],"We":[51,71,195],"aimed":[52],"assess":[54,234],"if":[55],"SCC":[56,142,185,200,215,238],"change":[57],"on":[60,78,94,139,227],"ELX/TEZ/IVA":[61,79],"good":[64],"response.":[69],"Methods:":[70],"performed":[72],"retrospective":[74],"study":[75],"followed":[80],"at":[81,156,174],"Portuguese":[83],"CF":[84],"Reference":[85],"Centre":[86],"from":[87],"March":[88],"2021":[89],"January":[91],"2023.":[92],"Data":[93],"forced":[95],"expiratory":[96],"volume":[97],"first":[100],"second":[101],"(ppFEV1),":[102],"SCC,":[103],"Body":[104],"Mass":[105],"Index":[106],"(BMI),":[107],"pulmonary":[108],"exacerbations":[109],"(Pex)":[110],"computerized":[112],"tomography":[113],"(CT)":[114],"scans":[115],"before":[116],"after":[118,149],"beginning":[120],"therapy":[122],"was":[123,127,178],"collected.":[124],"Statistical":[125],"analysis":[126],"undertaken":[128],"using":[129],"SPSS":[130],"IBM\u00ae,":[131],"Version":[132],"27.":[133],"Results:":[134],"There":[135,177],"were":[136],"45":[137,146],"ELX/TEZ/IVA.":[140,228],"Mean":[141,154],"decreased":[143],"significantly":[144],"by":[145,168],"mmol/L":[147],"(p<,001)":[148,173],"start":[151],"therapy.":[153],"ppFEV1":[155,164,190],"12-24":[157],"weeks":[158],"improved":[159],"11,6%":[160],"compared":[161],"baseline":[163],"(p<,001).":[165],"Pex":[166,206],"reduced":[167],"79,6%.":[169],"BMI":[170,193],"increased":[171],"8%":[172],"24":[175],"weeks.":[176],"no":[179,197],"correlation":[180],"between":[181,199],"percentage":[183],"reduction":[186],"that":[188],"improvement":[191,204],"or":[192,205,222],"increase.":[194],"found":[196],"association":[198],"normalization":[201],"radiologic":[203],"reduction.":[207],"Conclusions:":[208],"In":[209],"our":[210],"study,":[211],"degree":[213],"decrease":[216],"did":[217],"reflect":[219],"radiological":[223],"changes":[224],"Further":[229],"studies":[230],"are":[231],"necessary":[232],"suitability":[236]},"counts_by_year":[{"year":2025,"cited_by_count":1},{"year":2024,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2023-10-28T00:00:00"}