Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?

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dc.contributor.authorUytun, Salih
dc.contributor.authorCinel, Guzin
dc.contributor.authorPolat, Sanem Eryilmaz
dc.contributor.authorTabakci, Sati Ozkan
dc.contributor.authorKiper, Nural
dc.contributor.authorYalcin, Ebru
dc.contributor.authorTural, Dilber Ademhan
dc.date.accessioned2024-02-23T13:03:31Z
dc.date.available2024-02-23T13:03:31Z
dc.date.issued2023
dc.departmentNEÜen_US
dc.description.abstractBackgroundCystic fibrosis (CF) is an autosomal recessive disorder caused by CF transmembrane conductance regulator (CFTR) genetic variants. CFTR modulators improve pulmonary function and reduce respiratory infections in CF. This study investigated the clinical and laboratory follow-up parameters over 1 year in patients with CF who could not receive this treatment. MethodsThis retrospective cohort study included 2018 and 2019 CF patient data from the CF registry of Turkey. Demographic and clinical characteristics of 294 patients were assessed, who had modulator treatment indications in 2018 but could not reach the treatment. ResultsIn 2019, patients younger than 18 years had significantly lower BMI z-scores than in 2018. During the 1-year follow-up, forced expiratory volumes (FEV1) and FEV1 z-scores a trend toward a decrease. In 2019, chronic Staphylococcus aureus colonization, inhaled antipseudomonal antibiotic use for more than 3 months, oral nutritional supplement requirements, and oxygen support need increased. ConclusionsPatients who had indications for modulator treatments but were unable to obtain them worsened even after a year of follow-up. This study emphasized the importance of using modulator treatments for patients with CF in our country, as well as in many countries worldwide.en_US
dc.identifier.doi10.1002/ppul.26535
dc.identifier.endpage2512en_US
dc.identifier.issn8755-6863
dc.identifier.issn1099-0496
dc.identifier.issue9en_US
dc.identifier.pmid37278544en_US
dc.identifier.scopus2-s2.0-85161522932en_US
dc.identifier.scopusqualityQ1en_US
dc.identifier.startpage2505en_US
dc.identifier.urihttps://doi.org/10.1002/ppul.26535
dc.identifier.urihttps://hdl.handle.net/20.500.12452/10654
dc.identifier.volume58en_US
dc.identifier.wosWOS:001001347000001en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakScopusen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherWileyen_US
dc.relation.ispartofPediatric Pulmonologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.subjectBody Mass Indexen_US
dc.subjectCftr Modulatoren_US
dc.subjectCystic Fibrosisen_US
dc.subjectForced Expiratory Volumeen_US
dc.subjectZ-Scoresen_US
dc.titlePatients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?en_US
dc.typeArticleen_US

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