Regional differences in the evolution of lung disease in children with cystic fibrosis.
ABSTRACT Progression of lung disease is a major event in children with cystic fibrosis (CF), but regional differences in its evolution are unclear. We hypothesized that regional differences occur beginning in early childhood. We examined this issue by evaluating 132 patients followed in the Wisconsin Neonatal Screening Project between 1985 and 2010. We scored chest X-rays obtained every 1-2 years with the Wisconsin chest X-ray system, in which we divided the lungs into quadrants, and gave special attention to ratings for bronchiectasis (BX) and nodular/branching opacities. We compared the upper and lower quadrant scores, and upper right and left quadrant scores, as patients aged using a multivariable generalized estimation equation (GEE) model. We did a confirmatory analysis for a subset of 81 patients with chest computerized tomography (CT) images obtained in 2000 and scored using the Brody scoring system. The chest X-ray analysis shows that the upper quadrants have higher BX (P<0.001) and nodular/branching opacities (P<0.001) scores than the lower quadrants. CT analysis likewise reveals that the upper quadrants have more BX (P=0.02). Patients positive for mucoid PA showed significantly higher BX scores than patients with non-mucoid PA (P=0.001). Chest X-ray scoring also revealed that the upper right quadrant has more BX (P<0.001) than the upper left quadrant, and CT analysis was again confirmatory (P<0.001). We conclude that pediatric patients with CF develop more severe lung disease in the upper lobes than the lower lobes in association with mucoid PA infections and also have more severe lung disease on the right side than on the left side in the upper quadrants. A variety of potential explanations such as aspiration episodes may be clinically relevant and provide insights regarding therapies.
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ABSTRACT: Detecting and tracking early cystic fibrosis (CF) lung disease are difficult due to lack of sensitive markers of airway dysfunction. The goals were to detect regional distribution of airway disease through high-resolution computed tomography, correlate abnormalities to lower airway inflammation/infection, and compare computed tomography findings before and after intravenous antibiotic therapy in children with CF younger than 4 years experiencing a pulmonary exacerbation. High-resolution computed tomography was performed in 17 children scheduled for bronchoscopy. The radiologist identified the lobes with the "greatest" and "least" disease based on computed tomography, and bronchoalveolar lavage was performed in these areas. In 13 subjects, imaging was repeated after antibiotic completion. Modified Brody scores were assigned by two radiologists. The lobe with greatest disease was predominantly localized to the right and had higher modified Brody scores, indicating more severe abnormalities (p < 0.01), compared with the lobe with least disease. The total modified Brody score (p < 0.01), hyperinflation subscore (p < 0.01), and bronchial dilatation/bronchiectasis subscore (p < 0.01) improved after antibiotics and intensified airway clearance. Interleukin-8 levels (p < 0.01) and % neutrophils (p = 0.04) were increased in the lobe with greatest disease compared with the lobe with least disease. These results indicate that, in young children with CF experiencing a pulmonary exacerbation, computed tomography detects regional differences in airway inflammation, may be a sensitive outcome to evaluate therapeutic interventions, and identifies early lung disease as being more prominent on the right.American Journal of Respiratory and Critical Care Medicine 05/2007; 175(9):943-50. · 11.04 Impact Factor
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ABSTRACT: To assess the type, severity, and regional lung distribution of cystic fibrosis (CF) lesions as shown using high-resolution CT (HRCT), comparing these findings with chest radiographs and pulmonary function tests (PFTs), we obtained HRCTs in 36 patients with CF (mean age 13), who were clinically stable. We assessed four lung regions (upper and lower, right and left) and assigned each a semiquantitative score for (a) bronchial abnormalities, (b) parenchymal abnormalities, and (c) overinflation, based on the severity and profusion of the corresponding lesions. A similar regional assessment of chest radiographs was also done using the Chrispin-Norman method. PFT results were correlated with the radiological data. On HRCT, bronchial lesions were present in 89% of the patients and in 78% of the regions; bronchiectasis was the predominant abnormality in our population, visible in 100% of the abnormal regions. Less frequent were bronchial wall thickening (48%) and mucous plugs (29%). Parenchymal abnormalities were recognizable in 58% of the patients and 31% of the regions; alveolar consolidation was more frequent (80%) than were destructive changes (36%). Overinflation was found in 81% of the patients and 85% of the regions. We found the severity and profusion of bronchial lesions and parenchymal destructive changes to be unevenly distributed among the different regions, the upper lungs being more heavily involved than the lower, particularly on the right. Alveolar consolidation and overinflation were more uniform in distribution. HRCT patient scores correlated significantly with radiographic scores (r = 0.861) and with PFTs, especially with forced expiratory volume for 1 s (FEV1; r = 0.658). HRCT can be useful in the clinical management of patients with CF, depicting the type and distribution of bronchial and parenchymal lesions, particularly when chest radiographic results are unclear. In the planning and postural drainage, special attention should be given to the apical and posterior parts of the lungs, especially on the right; these are the areas most frequently and most severely involved by the disease.Journal of Thoracic Imaging 02/1996; 11(1):27-38. · 1.26 Impact Factor
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ABSTRACT: Chest radiography and high resolution computed tomography (HRCT) were used to evaluate the degree of lung involvement in 38 adult cystic fibrosis patients with unusually mild pulmonary disease. Abnormal features were present on the chest radiograph and/or high resolution CT scans of 35 patients, while in three patients both investigations were normal. Thickening of the wall of proximal right upper lobe bronchi was the earliest abnormal feature on HRCT. The commonest abnormal feature in these patients, including those whose chest radiograph was normal, was mild, uniform dilatation of proximal bronchi. The lumen dilatation was always less marked than the degree of thickening. Ten of the patients were first diagnosed in adult life. Although HRCT scans were normal in two of these patients, there were features suggestive of early pulmonary disease in the remaining eight.Clinical Radiology 08/1991; 44(1):20-2. · 1.82 Impact Factor