Experience of flexible bronchoscopy in the pediatric pulmonary diseases clinic.

Introduction: Flexible bronchoscopy is a valuable method in the diagnosis and treatment of respiratory tract diseases in children. This study aimed to examine the indications for and results of flexible bronchoscopy in children. Materials and Methods: The study included patients aged 0-18 years who underwent flexible bronchoscopy between 1 January 2017 and 31 December 2022. The patients were evaluated for demographic characteristics, indications for bronchoscopy, comorbidities, bronchoscopy findings, and the results of bronchoalveolar lavage. Result: During the defined study period, a total of 410 flexible bronchoscopy procedures were performed. 51.9% of the patient population were male, and 48.1% were female, with a mean age of 96.93 ± 63.45 months. The most common indication for flexible bronchoscopy was recurrent lower respiratory tract infection (26.8%), followed by chronic cough (19.1%). The bronchoalveolar lavage culture results showed that the most commonly isolated microorganisms were H. influenzae non-type b (7.8%) followed by M. catarrhalis (7.3%). Mucus obstruction and secretion (33.0%) constituted the most common bronchoscopic findings, while the flexible bronchoscopy examination was normal in 27% of patients. No serious complications occurred in any patient during or after the procedure. Conclusions: The results of this study demonstrated that the most common indication for flexible bronchoscopy was recurrent lower respiratory tract infection and the most common bronchoscopy finding was purulent secretion with mucus obstruction. Flexible bronchoscopy is an important diagnostic and treatment tool for patients with recurrent respiratory symptoms. It is a highly valuable method as it enables direct visualization of the airways and facilitates the collection of bronchoalveolar lavage samples.

Comparison of clinical features of cystic fibrosis patients eligible but not on CFTR modulators to ineligible for CFTR modulators.

INTRODUCTION: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator drugs target the underlying defect and improve CFTR function. They are a part of standard care in many countries, but not all patients are eligible for these drugs due to age and genotype. Here, we aimed to determine the characteristics of non-eligible patients for CFTR modulators in the CF registry of Turkey (CFRT) to highlight their clinical needs. METHODS: This retrospective cohort study included CF patient data from the CFRT in 2021. The decision of eligibility for the CFTR modulator was determined according to the 'Vertex treatment-Finder' on the Vertex® website. Demographic and clinical characteristics of patients were compared between eligible (group 1) and ineligible (group 2) groups for CFTR modulators. RESULTS: Among the study population (N = 1527), 873 (57.2%) were in group 1 and 654 (42.8%) were in group 2. There was no statistical difference between groups regarding sex, meconium ileus history, diagnoses via newborn screening, FEV1 z-score, CF-associated complications, organ transplant history, and death. Patients in group 2 had a higher incidence of pancreatic insufficiency (87.7% vs. 83.2%, p = .010), lower median height z-scores (-0.87 vs. -0.55, p < .001), lower median body mass index z-scores (-0.65 vs. -0.50, p < .001), longer days receiving antibiotics due to pulmonary exacerbation (0 [interquartile range, IQR: 0-2] vs. 0 [IQR: 0-7], p = 0.001), and more non-invasive ventilation support (2.6% vs. 0.9%, p = 0.008) than patients in group 1. CONCLUSION: The ineligible group had worse clinical outcomes than the eligible group. This highlights their need for life-changing drugs to improve clinical outcomes.

Investigation of dynamic hyperinflation and its relationship with exercise capacity in children with bronchiectasis.

BACKGROUND AND AIM: Dynamic hyperinflation (DH) is a major contributor to exercise intolerance in patients with obstructive lung diseases. However, it has not been investigated in children with bronchiectasis (BE). We aimed to investigate dynamic ventilatory responses and their influence on functional exercise capacity in children with BE. METHODS: Forty children with BE (mean forced expiratory volume in 1 s [FEV1 ] = 78 ± 19%pred) were included. Six-minute walk test (6MWT) was conducted using Spiropalm 6MWT® for evaluating dynamic ventilatory responses including inspiratory capacity (IC), minute ventilation (VE), breathing reserve (BR) and respiratory rate (RR). A decrease of ≥100 ml in IC during exertion was defined as DH. Also, spirometry was performed, and peripheral muscle strength were measured. RESULTS: Twenty patients (50%) developed DH, and four patients (10%) were ventilatory limited (BR < %30) during 6MWT. There was a 176 [100-590] ml decrease in IC after exertion in patients with DH. DH did not correlate to clinical or functional indicators of the disease, except for an increase in RR (∆RR) during exertion. High ∆RR was associated with presence of DH (rpb = 0.390; p < 0.05). Clinical features, peripheral muscle strength, and Spiropalm 6MWT metrics including 6MWT distance did not differ between patients with and without DH. Univariate analysis revealed FVC% (R = 0.340), VEpeak (R = 0.565), quadriceps strength (R = 0.698) and handgrip strength (R = 0.711) were the only predictors of 6MWT distance (p < 0.05). CONCLUSION: Although DH is common in children with BE, the severity of DH is rather low and may not seem to affect functional exercise capacity. However, peripheral muscle strength was a major contributor to functional exercise capacity.