Bulent Karadag

Background: Upper airway disease is common in primary ciliary dyskinesia (PCD), but management evidence is limited. We aimed to describe management practices and identify factors influencing management decisions. Methods: Using data from the Ear-Nose-Throat (ENT) Prospective International Cohort of patients with PCD (EPIC-PCD) and an ENT-specialist survey across participating centres, we described management practices recorded at routine follow-up. We assessed clinical factors associated with practices via mixed-effects logistic regression models. In a subgroup of patients, we assessed factors associated with initiation or discontinuation of practices. Results: We included 579 patients: median age 15 years, 46% female. Nasal rinsing (54%) and nasal corticosteroids (22%) were most frequently prescribed. Among 466 patients with available data, 47 had grommets (10%) and 42 hearing aids (9%). Nasal corticosteroids and rinsing were more frequently prescribed in patients with polyps (odds ratio [OR] 3.74, 95% confidence interval [CI] 1.80–7.76; OR 3.39, 95% CI 1.37–8.37) or turbinate hypertrophy (OR 1.89, 95% CI 1.03–3.47; OR 2.89, 95% CI 1.55–5.38), and upper airway nebulisation in patients with frequent nasal symptoms (OR 2.86, 95% CI 1.11–7.39). Management practices differed between centres, as seen also by the specialists’ survey responses. In 177 patients with multiple visits, initiation of nasal rinsing was associated with frequent nasal symptoms (OR 3.18, 95% CI 1.24–8.18) and turbinate hypertrophy (OR 3.21, 95% CI 1.20–8.59). Conclusion: Upper airway disease management in PCD varies and is partly guided by symptom burden and clinical findings. This variation across centres highlights the need for care standardisation and PCD-specific management guidelines.

Background: Upper airway disease is common in primary ciliary dyskinesia (PCD), but management evidence is limited. We aimed to describe management practices and identify factors influencing management decisions. Methods: Using data from the Ear-Nose-Throat (ENT) Prospective International Cohort of patients with PCD (EPIC-PCD) and an ENT-specialist survey across participating centres, we described management practices recorded at routine follow-up. We assessed clinical factors associated with practices via mixed-effects logistic regression models. In a subgroup of patients, we assessed factors associated with initiation or discontinuation of practices. Results: We included 579 patients: median age 15 years, 46% female. Nasal rinsing (54%) and nasal corticosteroids (22%) were most frequently prescribed. Among 466 patients with available data, 47 had grommets (10%) and 42 hearing aids (9%). Nasal corticosteroids and rinsing were more frequently prescribed in patients with polyps (odds ratio [OR] 3.74, 95% confidence interval [CI] 1.80-7.76; OR 3.39, 95% CI 1.37-8.37) or turbinate hypertrophy (OR 1.89, 95% CI 1.03-3.47; OR 2.89, 95% CI 1.55-5.38), and upper airway nebulisation in patients with frequent nasal symptoms (OR 2.86, 95% CI 1.11-7.39). Management practices differed between centres, as seen also by the specialists’ survey responses. In 177 patients with multiple visits, initiation of nasal rinsing was associated with frequent nasal symptoms (OR 3.18, 95% CI 1.24-8.18) and turbinate hypertrophy (OR 3.21, 95% CI 1.20-8.59). Conclusion: Upper airway disease management in PCD varies and is partly guided by symptom burden and clinical findings. This variation across centres highlights the need for care standardisation and PCD-specific management guidelines.

Environmental exposure to irritants may contribute to asthma pathogenesis through increased epithelial permeability. This study aimed to investigate the association between chlorinated swimming pool exposure and allergic diseases, as well as its relationship with objective markers of airway inflammation, epithelial permeability, bronchial hyperreactivity, and atopy in elite swimmers. This cross-sectional study was conducted among pediatric swimmers attending a professional swimming club in Istanbul. All eligible participants were invited to complete the International Study of Asthma and Allergies in Childhood (ISAAC) questionnaire. A subset underwent objective assessments, including exercise-induced bronchoconstriction testing, measurement of fractional exhaled nitric oxide (FeNO), serum surfactant protein-D (SP-D), serum club cell protein (CC16), and total and aeroallergen-specific immunoglobulin E (IgE) measurements. Of the 172 invited children, 169 (98%) completed the questionnaire, and 84 (49%) underwent objective assessments (44% male; mean age 10.0 ± 2.1 years). The median duration of swimming and cumulative pool attendance were 36 months and 1430 h, respectively. The prevalence of atopy was 41.6%. Among the participating swimmers, the prevalence of asthma (ever, current, and physician-diagnosed) was 25%, 9.5%, and 15.5%, respectively. Corresponding prevalences for eczema were 16.7%, 4.8%, and 7.1%, and for allergic rhinitis were 19.0%, 19.0%, and 16.7%. Cumulative exposure to chlorinated swimming pools was not associated with allergic diseases, airway inflammation, epithelial permeability, or bronchial hyperreactivity. However, children who had been swimming for more than 72 months demonstrated higher rates of atopy and current allergic symptoms. In this cohort of elite swimmers, cumulative exposure to chlorinated swimming pools was not associated with allergic diseases or objective markers of airway inflammation. However, given the cross-sectional design, these findings should be interpreted with caution. The observed association between longer swimming duration and atopy appears to be exploratory. Overall, chlorinated pool exposure alone may be unlikely to be a major determinant of allergic disease in this population. • Chlorinated pool exposure has been proposed to contribute to asthma and allergic diseases through airway epithelial injury; however, findings in pediatric populations are inconsistent, and many studies rely primarily on questionnaire-based data without objective airway assessments. • In this cohort of elite swimmers, cumulative pool exposure was not associated with allergic diseases or objective markers of airway inflammation and epithelial integrity. However, longer swimming duration was associated with increased atopy, and subgroup analyses suggested that early-life exposure in combination with tobacco smoke may influence airway inflammation.

Background/Objectives: Childhood non-cystic fibrosis (non-CF) bronchiectasis is clinically heterogeneous. We aimed to describe fractional exhaled nitric oxide (FeNO) levels in affected children and examine associations with etiology, spirometry, and CT-defined disease extent. Methods: This single-center prospective observational study included 100 clinically stable children aged 6–18 years with CT-confirmed non-CF bronchiectasis evaluated between September 2014 and December 2015. FeNO was measured before spirometry using an online single-breath electrochemical technique. Chest CT was reviewed at the lobar level, with the lingula counted separately, and disease extent was summarized by the number of involved lobar regions. Associations were assessed using Spearman correlation and non-parametric tests. Results: Mean age was 14.9 ± 2.0 years, 55% were male, and mean FeNO was 20.9 ± 14.0 ppb. FeNO correlated positively with FEV1 (% predicted), FVC (% predicted), and FEF25–75 (% predicted) (all p < 0.01). FeNO was higher in males and adolescents than in females and younger children, respectively. FeNO did not differ by CT-defined lobar extent. It was lower in primary ciliary dyskinesia than in asthma overlap. Overall, 82% of the cohort received an ICS-containing maintenance regimen and household tobacco smoke exposure was present in 58%. Conclusions: FeNO was associated with selected functional indices and etiologic subgroups, but not with CT-defined structural extent, suggesting a greater role in clinical phenotyping than in reflecting radiologic burden. Rather than reflecting overall disease severity, FeNO may be more relevant as a marker of T2-leaning airway inflammatory phenotype in selected children with non-CF bronchiectasis. These findings should be interpreted as exploratory and hypothesis-generating, particularly for etiologic subgroup comparisons and for FeNO interpretation in the setting of treatment and environmental confounding.

Cystic fibrosis (CF) care has advanced rapidly, yet diagnosis and inclusion in patient registries remain severely limited in low‐ and middle‐income countries (LMICs). Barriers include restricted newborn screening, limited availability of sweat chloride testing, and underrepresentation of non‐European CFTR variants in standard panels. Sparse registries further impede epidemiological insight and resource planning. Also, access to comprehensive CF care remains a major challenge in LMICs, where limitations in basic therapies, including pancreatic enzyme replacement, airway clearance strategies, and infection control, continue to result in markedly reduced life expectancy. Within this context, access to CFTR modulators (CFTRm) represents an additional and critical barrier, constrained by prohibitive costs, restricted variant eligibility, and few tiered pricing mechanisms. Patient‐derived intestinal organoids (PDIOs) offer a versatile platform to help address some of these gaps. PDIOs enable functional diagnosis, genotype–phenotype characterization, and renewable material for comprehensive CFTR sequencing and drug testing, including for rare variants. While implementation in LMICs is challenging, targeted mentorship, collaborative screening pipelines, and sustained funding could expand access. Integrating PDIOs into CF care could help bridge some global disparities, but success will require coordinated scientific, clinical, and policy efforts.

Background/Objectives: Childhood non-cystic fibrosis (non-CF) bronchiectasis is clinically heterogeneous. We aimed to describe fractional exhaled nitric oxide (FeNO) levels in affected children and examine associations with etiology, spirometry, and CT-defined disease extent. Methods: This single-center prospective observational study included 100 clinically stable children aged 6–18 years with CT-confirmed non-CF bronchiectasis evaluated between September 2014 and December 2015. FeNO was measured before spirometry using an online single-breath electrochemical technique. Chest CT was reviewed at the lobar level, with the lingula counted separately, and disease extent was summarized by the number of involved lobar regions. Associations were assessed using Spearman correlation and non-parametric tests. Results: Mean age was 14.9 ± 2.0 years, 55% were male, and mean FeNO was 20.9 ± 14.0 ppb. FeNO correlated positively with FEV1 (% predicted), FVC (% predicted), and FEF25–75 (% predicted) (all p < 0.01). FeNO was higher in males and adolescents than in females and younger children, respectively. FeNO did not differ by CT-defined lobar extent. It was lower in primary ciliary dyskinesia than in asthma overlap. Conclusions: FeNO was associated with selected functional indices and etiologic subgroups, but not with CT-defined structural extent, suggesting a greater role in clinical phenotyping than in reflecting radiologic burden.

Cystic fibrosis has been transformed by the development of CFTR modulator therapies, with substantial improvements in survival and quality of life. However, access to these therapies remains profoundly unequal worldwide. The greatest benefits have been realized in high‐income countries, while people with cystic fibrosis in low‐ and middle‐income countries and underserved populations within high‐income settings continue to face limited access and poorer outcomes. Underdiagnosis is a major contributor to these disparities, as limited newborn screening, restricted access to sweat testing, and incomplete genetic characterization directly limit treatment eligibility and registry inclusion. Beyond diagnosis, disparities are driven by differences in genetic variant distribution, pricing and reimbursement policies, regulatory processes, and health system capacity. This review examines how these interrelated factors shape global access to therapies, with particular emphasis on CFTR modulators. Emerging strategies—including differential pricing, licensing mechanisms, regulatory adaptation, international collaboration, and health system strengthening—are discussed. Achieving equitable access will require coordinated action across diagnostic, economic, and policy domains to ensure that advances in cystic fibrosis care benefit patients regardless of geographic or socioeconomic context.

Background: Cystic fibrosis (CF) is a life‐threatening disease that requires extensive knowledge for effective management. This study aimed to assess the knowledge levels of parents of children diagnosed with CF through newborn screening (NBS), evaluate their experiences during the diagnostic process, and determine the impact of education on their CF knowledge. Methods: This quasi‐experimental study involved 47 parents of children aged 0–30 months diagnosed with CF through NBS at four CF centers in Istanbul, Turkey. Parents completed a questionnaire assessing their CF knowledge before and after receiving face‐to‐face education, informational brochures, and an online webinar. The questionnaire covered general CF features, lung health, sexual function and infertility, and gastrointestinal issues. Results: The study revealed significant deficiencies in parental knowledge about CF and the NBS process. Only 25.5% of parents were informed about NBS prenatally, and 51.1% received information about CF when NBS results were positive. After educational intervention, correct response rates significantly increased for general characteristics ( p = 0.003), sexual health ( p < 0.001), lung health ( p = 0.007), and overall knowledge ( p < 0.001). Parents of children older than 12 months showed more pronounced improvement in knowledge across various sections compared to parents of younger children. Conclusion: The study highlights the need for a more robust educational framework to equip parents with comprehensive knowledge about CF. Improved communication strategies about NBS processes and repeated educational interventions are necessary to address knowledge gaps and enhance the quality of life for CF patients and their families.