Spirometer-triggered high-resolution computed tomography and pulmonary function measurements during an acute exacerbation in patients with cystic fibrosis

• Published in: The Journal of pediatrics 2001-04, Vol.138 (4), p.553-559
• Main Authors: Robinson, Terry E., Leung, Ann N., Northway, William H., Blankenberg, Francis G., Bloch, Daniel A., Oehlert, John W., Al-Dabbagh, Hiba, Hubli, Shital, Moss, Richard B.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Biological and medical sciences; Child; Cystic Fibrosis - diagnosis; Cystic Fibrosis - physiopathology; Cystic Fibrosis - therapy; Female; Forced Expiratory Volume - physiology; Humans; Investigative techniques, diagnostic techniques (general aspects); Lung - physiopathology; Male; Medical sciences; Predictive Value of Tests; Radiodiagnosis. Nmr imagery. Nmr spectrometry; Respiratory system; Severity of Illness Index; Spirometry - methods; Tomography, X-Ray Computed - methods; Treatment Outcome
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1067/mpd.2001.111820

Objective: To evaluate a high-resolution computed tomography (HRCT) scoring system, clinical parameters, and pulmonary function measurements in patients with cystic fibrosis (CF) before and after therapy for a pulmonary exacerbation. Study design: Patients (n = 17) were evaluated by spirometer-triggered HRCT imaging, clinical parameters, and pulmonary function tests (PFTs) before and after treatment. HRCT scans were reviewed by 3 radiologists using a modified Bhalla scoring system. Results: Bronchiectasis, bronchial wall thickening, and air trapping were identified in all subjects on initial evaluation. The initial total HRCT score correlated significantly with the Brasfield score (r = –.91, P < .001) and several PFT measures. After treatment, there were improvements in the acute change clinical score (ACCS) (P < .001), most pulmonary function measurements, and total HRCT score (P < .05). Bronchiectasis, bronchial wall thickening, and air trapping did not significantly change. Mucus plugging subcomponent HRCT score, slow vital capacity (SVC), forced expiratory volume in 1 second (FEV1), and forced vital capacity (FVC) (percent predicted) and reversible and total HRCT scores were most sensitive to change by effect size analysis. Conclusions: Improvements occurred with treatment in total and reversible HRCT scores, PFTs, and ACCS. Total and reversible HRCT scores and percent predicted SVC, FEV1, and FVC were the most sensitive to change. The greatest change was seen in the mucus plugging subcomponent HRCT score. (J Pediatr 2001;138:553-9)