Autor: |
Desmond M Murphy, B J Plant, Joseph A Eustace, Parniya Arooj, David V Morrissy, Yvonne McCarthy, Tamara Vagg, Mairead McCarthy, Claire Fleming, Mary Daly |
Jazyk: |
angličtina |
Rok vydání: |
2023 |
Předmět: |
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Zdroj: |
BMJ Open Respiratory Research, Vol 10, Iss 1 (2023) |
Druh dokumentu: |
article |
ISSN: |
2052-4439 |
DOI: |
10.1136/bmjresp-2022-001590 |
Popis: |
Background Previous studies showed that the combination of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) corrector and potentiator, lumacaftor–ivacaftor (LUMA–IVA) provides meaningful clinical benefits in patients with cystic fibrosis who are homozygous for the Phe508del CFTR mutation. However, little is known about the effect of LUMA–IVA on Proinflammatory Cytokines (PICs).Objectives To investigate the impact of LUMA–IVA CFTR modulation on circulatory and airway cytokines before and after 12 months of LUMA–IVA treatment in a real-world setting.Methods We assessed both plasma and sputum PICs, as well as standard clinical outcomes including Forced Expiratory Volume in one second (FEV1) %predicted, Body Mass Index (BMI), sweat chloride and pulmonary exacerbations at baseline and prospectively for one year post commencement of LUMA–IVA in 44 patients with cystic fibrosis aged 16 years and older homozygous for the Phe508del CFTR mutation.Results Significant reduction in plasma cytokines including interleukin (IL)-8 (p |
Databáze: |
Directory of Open Access Journals |
Externí odkaz: |
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