作者: Kavitha Kotha , Rhonda D. Szczesniak , Anjaparavanda P. Naren , Matthew C. Fenchel , Leo L. Duan
DOI: 10.1016/J.JCF.2015.07.002
关键词: Cohort 、 Medicine 、 Interquartile range 、 Excretion 、 Cystic fibrosis transmembrane conductance regulator 、 Ivacaftor 、 Gastroenterology 、 Internal medicine 、 Spirometry 、 Cystic fibrosis 、 COPD 、 Surgery
摘要: Abstract Background Lower airway biomarkers of restored cystic fibrosis transmembrane conductance regulator (CFTR) function are limited. We hypothesized that fractional excretion nitric oxide (FENO), typically low in CF patients, would demonstrate reproducibility during CFTR-independent therapies, and increase CFTR-specific intervention (ivacaftor) patients with CFTR gating mutations. Methods Repeated FENO spirometry measurements children (Cohort 1; n=29) were performed hospital admission for acute pulmonary exacerbations routine outpatient care. before after one month ivacaftor treatment (150mg every 12h) completed mutations 2; n=5). Results Cohort 1: Mean forced expiratory volume 1s (FEV 1 % predicted) at enrollment was 72.3% (range 25%–102%). varied minimally over the two inpatient (9.8–10.9ppb). There no clear changes related to exacerbations, gender, genotype or microbiology, weak correlation inhaled corticosteroid use ( P predicted increased by 7.3% 10.2%. Conclusions stable whereas all treated ivacaftor. Acute may serve as a biomarker lower modulator treatment.