A pediatric cystic fibrosis arthropathy case who responded to Elexacaftor/ Tezacaftor/Ivacaftor therapy

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dc.contributor.authorArslan, Melikşah
dc.contributor.authorBahadır, Zeynep
dc.contributor.authorBasiaga, Matthew L.
dc.contributor.authorChalmers, Sarah J.
dc.contributor.authorDemirel, Nadir
dc.date.accessioned2024-01-15T09:47:09Z
dc.date.available2024-01-15T09:47:09Z
dc.date.issued2023
dc.departmentİstanbul Medipol Üniversitesi, Tıp Fakültesi, Dahili Tıp Bilimleri Bölümü, Çocuk Sağlığı ve Hastalıkları Ana Bilim Dalı
dc.description.abstractCystic fibrosis arthropathy (CFA) is a transient, intermittent form of arthritis that cannot be associated with any other disease other than CF thus making CFA a diagnosis of exclusion. NSAIDs, short-term intermittent splinting, glucocorticoids, and disease-modifying anti-rheumatic drugs are treatment options for CFA. Currently, there is no consensus on how to best treat CFA. Diagnosis and treatment of CFA remain a challenge for physicians and people with CF. The newest CFTR modulator therapy, elexacaftor/tezacaftor/ivacaftor (ETI), was approved by the FDA recently for children over the age of 6 with at least one Phe508del allele in the CFTR gene. Multiple clinical benefits of ETI in pulmonary functions and overall disease burden have been reported since its approval, however, the data on the musculoskeletal therapeutic benefits of ETI has been limited. In this report, we present a 7-year-old female with CF whose CFA symptoms resolved after starting ETI therapy.
dc.identifier.citationArslan, M., Bahadır, Z., Basiaga, M. L., Chalmers, S. J. ve Demirel, N. (2023). A pediatric cystic fibrosis arthropathy case who responded to Elexacaftor/ Tezacaftor/Ivacaftor therapy. Journal of Cystic Fibrosis, 22(6), 1120-1122. https://dx.doi.org/10.1016/j.jcf.2023.08.012
dc.identifier.doi10.1016/j.jcf.2023.08.012
dc.identifier.endpage1122
dc.identifier.issn1569-1993
dc.identifier.issn1873-5010
dc.identifier.issue6
dc.identifier.pmid37709627
dc.identifier.scopus2-s2.0-85171381039
dc.identifier.scopusqualityQ1
dc.identifier.startpage1120
dc.identifier.urihttps://dx.doi.org/10.1016/j.jcf.2023.08.012
dc.identifier.urihttps://hdl.handle.net/20.500.12511/12139
dc.identifier.volume22
dc.identifier.wos001128633200001en_US
dc.identifier.wosqualityQ2
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.institutionauthorBahadır, Zeynep
dc.language.isoen
dc.publisherElsevier
dc.relation.ispartofJournal of Cystic Fibrosisen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/closedAccess
dc.subjectArthropathy
dc.subjectCFTR Modulators
dc.subjectCystic Fibrosis
dc.subjectElexacaftor/Tezacaftor/Ivacaftor
dc.subjectInflammatory Arthritis
dc.subjectMusculoskeletal Symptoms
dc.subjectNSAIDs
dc.titleA pediatric cystic fibrosis arthropathy case who responded to Elexacaftor/ Tezacaftor/Ivacaftor therapy
dc.typeArticle

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