P350 Impact of one year of treatment with elexacaftor/tezacaftor/ivacaftor on clinical outcomes in adult patients with cystic fibrosis - first experience from Croatia

نویسندگان

چکیده

The CFTR modulator elexacaftor/tezacaftor/ivacaftor has been shown to improve lung function, body mass index (BMI) and quality of life in patients with cystic fibrosis (CF). On October 15th, 2021, triple therapy was approved Croatia for the CF (homozygous F508del heterozygous F808del/minimal function mutation). Our aim evaluate whether initiation this treatment is associated improved BMI our adult population. This retrospective study included adults mutation) Cystic Fibrosis Centre Paediatrics Adults - University Hospital Zagreb, Croatia. were evaluated before after at least 6 months therapy. Clinical outcome data comprised BMI. 44 (25.782 ± 5.24 years old, 54% female, 77% homozygous) included. We compared spirometry results (FEV1, FVC, FEF 25–75 PEF) elexacaftor/tezacaftor/ivacaftor. Significant changes (% change mean; Wilcoxon p-values reported) documented (13%, p = 0.000), FEV1% (33%, FVC% (25%, 25–75% (57%, 0.000) PEF (34%, novel a clinically significant improvement

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ژورنال

عنوان ژورنال: Journal of Cystic Fibrosis

سال: 2023

ISSN: ['1569-1993', '1873-5010']

DOI: https://doi.org/10.1016/s1569-1993(23)00720-8