BACKGROUND: Lung ultrasound is an examination that allows the assessment of pulmonary involvement by analyzing artifacts. Our primary aim was to correlate our lung ultrasound findings with pulmonary function and the modified Bhalla score in patients with cystic fibrosis. METHODS: Subjects with cystic fibrosis were evaluated based on the results of lung ultrasound, pulmonary function exams (ie, spirometry before and after the use of a bronchodilator and S pO 2), and the modified Bhalla score. The partial correlation set by age between lung ultrasound, pulmonary function, and modified Bhalla score was carried out. Lung ultrasound was graded according to a new score, ranging from 0 to 36, with a higher score being associated with a greater degree of involvement. We performed Bland-Altman and linear regression analysis to identify bias between lung ultrasound and modified Bhalla score. Alpha ؍ 0.05. RESULTS: 18 subjects with cystic fibrosis were included. In partial correlation controlled by age, we observed significant ultrasound score values with weight (partial correlation ؍ ؊0.579), body mass index (partial correlation ؍ ؊0.609), S pO 2 (partial correlation ؍ ؊0.728), FVC% (pre-bronchodilator: partial correlation ؍ ؊0.538; post-bronchodilator: partial correlation ؍ ؊0.560), FEV 1 % (pre-bronchodilator: partial correlation ؍ ؊0.536; post-bronchodilator: partial correlation ؍ ؊0.546), and modified Bhalla score (partial correlation ؍ 0.607). We did not identify bias between lung ultrasound and modified Bhalla score measured by z-score. CONCLUSIONS: Lung ultrasound seems to be effective and corroborates with high-resolution computed tomography when evaluated by the modified Bhalla score. At the same time, lung ultrasound had significant correlation with pulmonary function and nutritional status.
Background Among the many consequences of loss of CFTR protein function, a significant reduction of the secretion of bicarbonate (HCO 3 −) in cystic fibrosis (CF) is a major pathogenic feature. Loss of HCO 3 − leads to abnormally low pH and impaired mucus clearance in airways and other exocrine organs, which suggests that NaHCO 3 inhalation may be a low-cost, easily accessible therapy for CF. Objective To evaluate the safety, tolerability, and effects of inhaled aerosols of NaHCO 3 solutions (4.2% and 8.4%). Methods An experimental, prospective, open-label, pilot, clinical study was conducted with 12 CF volunteer participants over 18 years of age with bronchiectasis and pulmonary functions classified as mildly to severely depressed. Sputum rheology, pH, and microbiology were examined as well as spirometry, exercise performance, quality-of-life assessments, dyspnea, blood count, and venous blood gas levels. Results Sputum pH increased immediately after inhalation of NaHCO 3 at each clinical visit and was inversely correlated with rheology when all parameters were evaluated: [G′ (elasticity of the mucus) = − 0.241; G″ (viscosity of the mucus) = − 0.287; G* (viscoelasticity of the mucus) = − 0.275]. G* and G′ were slightly correlated with peak flow, forced expiratory volume in 1 s (FEV 1), and quality of life; G″ was correlated with quality of life; sputum pH was correlated with oxygen consumption (VO 2) and vitality score in quality of life. No changes were observed in blood count, venous blood gas, respiratory rate, heart rate, peripheral oxygen saturation of hemoglobin (SpO 2), body temperature, or incidence of dyspnea. No adverse events associated with the study were observed. Conclusion Nebulized NaHCO 3 inhalation appears to be a safe and well tolerated potential therapeutic agent in the management of CF. Nebulized NaHCO 3 inhalation temporarily elevates airway liquid pH and reduces sputum viscosity and viscoelasticity.
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