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English: The availability of combination therapy with cystic fibrosis transmembrane conductance regulator (CFTR) modulators, elexacaftor–tezacaftor–ivacaftor (ETI), has significantly improved pulmonary outcomes and quality of life in patients with cystic fibrosis. This study aimed to evaluate structural bronchopulmonary changes using computed tomography (CT) after 1 year of ETI treatment, assessed by the Bhalla score, and to correlate these findings with clinical response parameters. This retrospective observational study included 11 patients under 18 years of age who underwent chest CT before and 1 year after ETI initiation. CT images were assessed using the Bhalla score. Scores range from 0 to 25, with lower values indicating more severe structural lung involvement. CT scores were correlated with changes in sweat test values and percent predicted forced expiratory volume in one second (ppFEV1). Statistical analysis included the paired Student’s t-test, Wilcoxon signed-rank test, and Pearson’s correlation coefficient. The mean Bhalla score improved from 14.55 to 18.27 (P < 0.001), with the greatest improvements in mucus plugging (P = 0.002) and peribronchial thickening (P = 0.008). Bronchiectasis improved in some patients but did not reach statistical significance. Structural changes correlated with clinical response: ppFEV1 increased by 13.38% (P = 0.002), while sweat chloride levels decreased by 43.1 mmol/L (P < 0.001). ETI therapy produced significant morphological and functional improvements. CT-detected structural changes correlated with clinical outcomes, and the Bhalla score proved useful for monitoring pulmonary disease.

Cystic fibrosis is a chronic multisystem disease in which pulmonary involvement is the main determinant of morbidity and mortality. Chest computed tomography is the reference standard for assessing structural lung damage, but its repeated use is limited by cumulative radiation exposure. Lung ultrasound has emerged as a radiation-free alternative; however, its role in adult patients remains incompletely defined. This study aimed to evaluate the correlation between CT-derived structural lung damage and ultrasound findings, and to assess the complementary role of these imaging modalities. A prospective cohort study was conducted including adult patients with cystic fibrosis who underwent both chest computed tomography and lung ultrasound during the same clinical episode. Structural lung involvement was assessed using the Bhalla score, while lung aeration was evaluated using the Lung Ultrasound Score. Correlation analyses, severity stratification, regression modeling, and longitudinal comparisons were performed. Thirteen patients contributed 24 imaging evaluations. A strong positive correlation between Bhalla score and ultrasound findings was observed in the cross-sectional analysis and remained consistent when all examinations were included. Ultrasound scores increased significantly across CT-defined severity groups, and regression analysis confirmed a significant association between the two methods. Exploratory analysis showed stronger associations for peripheral and aeration-related abnormalities, while weaker associations were observed for deeper airway changes. No significant correlation was identified in longitudinal analysis. Lung ultrasound correlates well with CT-derived structural lung damage and may serve as a complementary, radiation-free tool for disease assessment in adult cystic fibrosis. However, its limited sensitivity in detecting temporal changes highlights the continued importance of CT in selected clinical scenarios.

Background and Objectives: a “vicious vortex” model was proposed to explain the pathophysiology of bronchiectasis, incorporating abnormal mucus, altered mucociliary clearance and chronic inflammation. Evidently, airway clearance needs to be implemented in the patient’s daily routine for a protracted period in order to ameliorate the clinical outcomes. High Flow therapy (HFT) has several physiologic effects and represents a valid therapy for various respiratory diseases. The aim of this study is to assess clinical and radiologic effects of long-term HFT in adult non-CF bronchiectasis. Materials and Methods: This is a retrospective observational cohort study including adult patients affected by bronchiectasis and frequent exacerbations and hospitalizations. A chest HRCT was performed, and a quantitative evaluation of the scans was conducted applying a modified Bhalla score of five items. A total of 44 patients completed the follow up, 23 in the HF-group and 21 in the controls (No-HF group). Results: The median follow up was 41 months (range 36–48 months). The mean age was 65 years, 45% were females. After treatment the annual rate of exacerbations was significantly lower in the HF group (1.2 ± 0.95 versus 3.5 ± 1.0 per year, p < 0.0001). The annual rate of hospitalizations was significantly lower in the HF group (0.4 ± 0.52 versus 1 ± 0.93 per year, p = 0.01). The total score of the modified Bhalla improved after treatment in the HF group with a mean score of 5.32 versus 8.38, p = 0.034. The difference was substantially due to the lower score of mucoid impactions in the HF group. Conclusions: Bronchiectasis is an evolutive disease. Long-term HFT reduces the annual rate of exacerbation and hospitalization. In addition, HFT prevents mucoid impaction and potentially influences the radiological evolution of the disease.

Objective To investigate the correlation between severity of lung disease determined by chest computed tomography (CT) and 6-min walk test (6MWT) with health-related quality of life (HRQoL) score in cystic fibrosis (CF) patients.MethodsThis cross-sectional study evaluated 76 CF patients referred to CF Clinic, aged 7–14 y. Subjects were asked to complete Pediatric quality of life (PedsQL4.0) forms, during their outpatient visits to determine their HRQoL score. Patients’ lung disease severity was quantified by Bhalla score determined by the child’s chest CT and their 6MWT. These three variables were then analyzed to determine whether there is correlation between HRQoL with severity of lung disease.ResultsThe mean distance of patients 6MWT score was 447.4 ± 81.4 m. There was a positive correlation between distance and HRQoL score in total, social, school and emotional function (p < 0.05). However, in physical function the correlation lacked significance (p = 0.07). Patients with a Bhalla score of less than 15 were older than patients with a Bhalla score of more than 15 (p < 0.001). Physical, emotional, social, school, and total function scores were significantly lower in patients with Bhalla score less than 15, compared to those with Bhalla score greater than 15 (p < 0.05).ConclusionsThe correlation among Bhalla score on CT scan, 6-min walk test, and HRQoL indicates that pulmonary disease has a clear impact on the quality of life of CF patients. HRQoL can be used in the care program of children with CF.

Aim:The aim of our study was to compare clinical severity scores and classic spirometry with impulse oscillometry (IOS) results and thoracic high resolution computed tomography (HRCT) scores in children with cystic fibrosis (CF) in order to determine the utility of the latter approach in patient follow-up.Materials and Methods:CF patients over 6 years of age were included. Shwachman-Kulczycki score, underclassical spirometry and IOS were performed when not in acute exacerbation. Thoracic HRCT images obtained within the previous 6 months were evaluated using the Bhalla scoring system.Results:The mean age of the children studied (n=30) was 12.1±4.2 years and 40% were female. Pseudomonas aeruginosa (P. aeroginosa) was isolated from sputum cultures of 40% of the patients. Patients with forced expiratory volume in one second (FEV1) below 80% exhibited significantly higher (resistance) R5, R10 values and significantly lower (reactance) X5 values on IOS (p=0.03, 0.027, 0.006, respectively). Patients with P. aeruginosa had significantly lower FEV1, forced vital capacity, and forced expiratory flow (25-75) values in classic spirometry when compared with patients without P. aeruginosa (p=0.002, p=0.002, and p=0.005, respectively). P. aeruginosa-positive patients showed significantly higher R5 and lower X5 values (p=0.047, 0.046, respectively). Bhalla scoring, bronchiectasis weight, peribronchial thickening, mucous plaques, saccularization, bronchial division, mosaic pattern parameters in groups with P. aeroginosa growth and/or FEV1 <80%; was found to be significantly more serious than the non-reproductive group (p<0.005, respectively). Again, in the group with P. aeroginosa growth, Shwachman-Kulczycki score was found to be significantly lower (p=0.001). No significant correlation was found between thoracic score data such as bronchiectasis weight and mosaic pattern presence and IOS values. In addition, in the group with high clinical score of Shwachman Kulczycki, resistance values such as R5 R10 R15 which are IOS parameters, and FEV1 were found above 80% (p=0.016, p=0.037, p=0.042, 0.004, respectively).Conclusion:IOS and tomographic scoring can be used safely in early detection of impairment in lung function. Further studies are needed to evaluate the utility of IOS in the clinical monitoring of children with CF who are not compliant with spirometry maneuvers.

OBJECTIVES: The aim of this study was to evaluate radiological, clinical, and demographic data of patients with noncystic fibrosis bronchiectasis and to compare high-resolution computed tomography (HRCT) scores based on the demographic and clinical characteristics. MATERIALS AND METHODS: A total of 34 patients (18 male, 16 female) were assessed in terms of age at symptom onset, age at diagnosis, annual attack frequency, cough severity score, physical examination findings, and pulmonary function test results. Modified Bhalla scoring system (B total) and anatomical prevalence degree score (D total) were used for HRCT examination. RESULTS: There was a strong negative correlation between forced expiratory volume at first second (FEV1) and bronchial dilatation degree (SBRDIL). There was a moderate negative correlation of FEV1, forced vital capacity (FVC), and maximum mid-expiratory flow rate (MEF; 25-75) with bronchiectasis degree (EXBRNC), bronchial wall thickness degree (SBWTHICK), and mucus accumulation in the major airways (PMPLA). The B total, D total, EXBRNC, and SBRDIL scores were significantly higher in patients with hemoptysis and sputum. In comparing B and D total scoring systems, B total provided better results in terms of rale, annual exacerbation frequency (AEF), cough severity score (CSS), and FEV1 values. CONCLUSION: As it is proved using HRCT, pulmonary function impairment, sputum production, hemoptysis, and increase in AEF strongly correlating with objective HRCT scoring can be accepted as markers for pathological changes due to bronchiectasis. Cite this article as: Başaran AE, Başaran A, Maslak İC, et al. Evaluation of Noncystic Fibrosis Bronchiectasis Using Clinical and Radiological Scorings in Children. Turk Thorac J 2018; 19(4): 159-64.

A short story is presented.

Zusammenfassung Bei Bronchiektasen handelt es sich um irreversible Erweiterungen der Bronchien. Sie sind chronisch progredient durch einen Teufelsstrudel aus Sekretretention, Infektion, Inflammation und struktureller Schädigung. Ihre zugrunde liegenden Ursachen sind vielfältig, und der Schweregrad der Erkrankung ist variabel, was sowohl die Diagnostik als auch die Therapie zu einer Herausforderung macht. Die Computertomographie (CT) ist Goldstandard bei der Diagnose von Bronchiektasen und kann bei der Klärung der Ätiologie hilfreich sein. Dafür sind insbesondere der Bronchiektasetyp, die Verteilung der Bronchiektasen innerhalb der Lunge und assoziierte Veränderungen relevant. Die Bildgebung hat auch einen Stellenwert bei der Verlaufskontrolle von Bronchiektasen. Im gewöhnlichen Befundbericht erfolgt diese visuell und deskriptiv, während semiquantitative Scores und computergestützte quantitative Analysen der Atemwege eine genauere Beurteilung ermöglichen und insbesondere für klinische Studien angewandt werden.

A short story is presented.

A short story is presented.