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    Evaluation of respiratory function at 6 years of age in patients with cystic fibrosis with frequent pulmonary exacerbations in the first 2 years of life
    (European Respiratory Soc Journals Ltd, 2023) Tabakci, Sati Ozkan; Cinel, Guzin; Uytun, Salih; Polat, Sanem Eryilmaz; Yalcin, Ebru; Kiper, Nural; Erdal, Meltem Akgul
    [Abstract Not Available]
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    Lung function outcomes of cystic fibrosis patients after early-life pulmonary exacerbations: National registry analysis
    (Wiley, 2026) Ozkan Tabakci, Sati; Uytun, Salih; Eryilmaz Polat, Sanem; Cinel, Guzin; Yalcin, Ebru; Kiper, Nural; Akgul Erdal, Meltem
    Background Pulmonary exacerbations (PEs) are associated with a subsequent decline in lung function. We aim to evaluate lung function in cystic fibrosis (CF) patients with frequent PEs in their first 2 years of age using spirometry at age 6. Methods This retrospective cohort study included CF patients who were 6 years old from the CF registry of Turkey in 2019. According to the number of PEs, patients were classified: those who had two or fewer PEs in the first 2 years of age were defined as Group 1 and those who had more than two PEs were defined as Group 2. The patients' demographics and clinical characteristics were compared between Group 1 and Group 2. Results The study included 88 patients who had data on PE from their first 2 years and completed their sixth year by 2019. Fifty-nine patients were included in Group 1 and 29 in Group 2. The mean percent-predictive FEV1 (ppFEV1), percent-predictive FVC (ppFVC) values, and the mean age at first PE were lower in Group 2 than in Group 1 (p = 0.019, p = 0.017, p < 0.001). The patients with chronic Pseudomonas aeruginosa (PA) colonization had lower mean ppFEV1 and ppFVC values than those without (p = 0.001, p = 0.001). Patients with PA in respiratory-sample culture during their first PE had lower ppFEV1 and ppFVC values than those with SA (p = 0.046; 0.018). Conclusions This study showed that more frequent PEs in the first 2 years of age and chronic PA colonization were associated with poorer FEV1, FVC, and BMI values in CF patients.
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    Patients with cystic fibrosis who could not receive the CFTR modulator treatment: What did they lose in 1 year?
    (Wiley, 2023) Uytun, Salih; Cinel, Guzin; Polat, Sanem Eryilmaz; Tabakci, Sati Ozkan; Kiper, Nural; Yalcin, Ebru; Tural, Dilber Ademhan
    BackgroundCystic 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.