{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T14:05:20Z","timestamp":1790345120404,"version":"4.1.0"},"reference-count":30,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T00:00:00Z","timestamp":1773100800000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To evaluate the prevalence of ineligibility for Elexacaftor\/Tezacaftor\/Ivacaftor (ETI) and Ivacaftor (IVA) and to compare data from four centers in Brazil, considering the racial\/ethnic background of each population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      This cross\u2010sectional study used data from the Brazilian Cystic Fibrosis (CF) Registry. Individuals with a confirmed diagnosis of CF by the identification of two variants in the\n                      <jats:italic>CFTR<\/jats:italic>\n                      gene and\/or elevated sweat chloride levels were included. Variables studied were treatment center, age at diagnosis, current age, sex, race\/ethnicity, genotype, and ineligibility for ETI and IVA. The chi\u2010square test compared the distribution of race\/ethnicity across centers and associations between ineligibility, treatment center, and the presence of the 3120\u2009+\u20091\u2009G\u2009&gt;\u2009A variant. Binary logistic regression was applied to assess the strength of association between the main variables.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Six hundred forty\u2010nine individuals were included, with a median age at diagnosis and current age of 0.25 and 11.6 years, respectively. One hundred thirty\u2010eight participants (21.26%) were ineligible for ETI or IVA. Racial\/ethnic distribution varied across centers, in the total sample, and among ineligible individuals. Non\u2010White individuals were 20% more likely to be ineligible than White individuals. The ineligibility for ETI did not vary significantly among the centers.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The ineligibility for ETI was high across all analyzed regions, but was not different among the centers. In Brazil, characterized by widespread admixture, the public health policies must consider the genotypic heterogeneity and wide variation in ancestry of the Brazilian population.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71557","type":"journal-article","created":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T16:24:18Z","timestamp":1773159858000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["CFTR Modulators in Brazil: Not for Everyone\u2014A Comparison of Ineligibility Across Different Regions"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4916-8483","authenticated-orcid":false,"given":"Edna L\u00facia D'","family":"Souza","sequence":"first","affiliation":[{"name":"Departamento de Pediatria, Faculdade de Medicina da Bahia Universidade Federal da Bahia Salvador Bahia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4948-6876","authenticated-orcid":false,"given":"Victor Hugo Valen\u00e7a","family":"Bomfim","sequence":"additional","affiliation":[{"name":"Faculdade de Medicina da Bahia Universidade Federal da Bahia Salvador Bahia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5595-351X","authenticated-orcid":false,"given":"Luciana de Freitas Velloso","family":"Monte","sequence":"additional","affiliation":[{"name":"Hospital da Crian\u00e7a de Bras\u00edlia Jos\u00e9 Alencar Universidade Cat\u00f3lica de Bras\u00edlia Bras\u00edlia Federal District Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7804-8608","authenticated-orcid":false,"given":"La\u00eds Ribeiro","family":"Mota","sequence":"additional","affiliation":[{"name":"Laborat\u00f3rio de Gen\u00e9tica Humana e Mutag\u00eanese Universidade Federal da Bahia Salvador Bahia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0753-784X","authenticated-orcid":false,"given":"Gabriel Souza Medrado","family":"Nunes","sequence":"additional","affiliation":[{"name":"Faculdade de Medicina da Bahia Universidade Federal da Bahia Salvador Bahia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0456-3233","authenticated-orcid":false,"family":"Regina Terse\u2010Ramos","sequence":"additional","affiliation":[{"name":"Departamento de Pediatria, Faculdade de Medicina da Bahia Universidade Federal da Bahia Salvador Bahia Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4341-7223","authenticated-orcid":false,"given":"Elenara da Fonseca Andrade","family":"Procianoy","sequence":"additional","affiliation":[{"name":"Hospital de Cl\u00ednicas de Porto Alegre, Porto Alegre  Rio Grande do Sul Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8431-1997","authenticated-orcid":false,"given":"Luiz Vicente Ribeiro Ferreira da","family":"Silva\u2010Filho","sequence":"additional","affiliation":[{"name":"Universidade de S\u00e3o Paulo, Faculdade de Medicina, Hospital das Cl\u00ednicas, Instituto da Crian\u00e7a S\u00e3o Paulo Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,3,10]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.10041"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0075145"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra2216474"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2021.01.007"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.3389\/fphar.2023.1178009"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(19)30337-6"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1183\/23120541.00082-2019"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2018.04.004"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1807120"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2022.106878"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.25285"},{"key":"e_1_2_10_13_1","unstructured":"Ivacaftor [Bula] Boston Vertex Dispon\u00edvel em https:\/\/consultas.anvisa.gov.br\/#\/bulario\/q\/?nomeProduto=KALYDECO."},{"key":"e_1_2_10_14_1","unstructured":"Elexacaftor + Tezacaftor + Ivacaftor [Bula] Boston Vertex Dispon\u00edvel em https:\/\/consultas.anvisa.gov.br\/#\/bulario\/q\/?nomeProduto=TRIKAFTA."},{"key":"e_1_2_10_15_1","unstructured":"Brazilian Cystic Fibrosis Study Group (GBEFC) Brazilian Cystic Fibrosis Registry\u2014REBRAFC data from 2021 S\u00e3o Paulo: GBEFC; 2024."},{"key":"e_1_2_10_16_1","unstructured":"April 2023 version http:\/\/www.cftr2.org\/sites\/default\/files\/CFTR2_7April2023.xlsx."},{"key":"e_1_2_10_17_1","volume-title":"IBM SPSS Statistics for Windows","author":"IBM, Corp","year":"2019"},{"issue":"5","key":"e_1_2_10_18_1","first-page":"1122","article-title":"Identification of Common Cystic Fibrosis Mutations in African\u2010Americans With Cystic Fibrosis Increases the Detection Rate to 75%","volume":"60","author":"Macek M.","year":"1997","journal-title":"American Journal of Human Genetics"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201606-481FR"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0017063"},{"key":"e_1_2_10_21_1","volume-title":"Censo Demogr\u00e1fico 2022: Resultados Preliminares","author":"Brasil. Instituto Brasileiro de Geografia e Estat\u00edstica (IBGE)","year":"2023"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/jhg.2009.123"},{"key":"e_1_2_10_23_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201603-192BC"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2020.08.007"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.9173"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1590\/1678-4685-GMB-2020-0275"},{"key":"e_1_2_10_27_1","doi-asserted-by":"publisher","DOI":"10.1086\/644532"},{"key":"e_1_2_10_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24026"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1089\/hum.2020.137"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2023.04.008"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2024.07.012"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71557","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.71557","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.71557","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T13:57:21Z","timestamp":1790344641000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.71557"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3]]},"references-count":30,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,3]]}},"alternative-id":["10.1002\/ppul.71557"],"URL":"https:\/\/doi.org\/10.1002\/ppul.71557","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3]]},"assertion":[{"value":"2025-08-10","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-01","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-10","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e71557"}}