Association between MucoidPseudomonasInfection and Bronchiectasis in Children with Cystic Fibrosis

Abstract

Purpose:To correlate the severity of bronchiectasis in children with cystic fibrosis with clinical and microbiologic variables in order to clarify risk factors for the development of irreversible lung disease. Materials and Methods:After institutional review board approval and parental informed consents were obtained, a HIPAA-compliant longitudinal epidemiologic evaluation was performed in patients with cystic fibrosis who were enrolled in the Wisconsin trial of newborn screening from 1985 to 2009. Thinsection chest computed tomography (CT) was used in a prospective cross-sectional design to study patients ranging in age from 6.6 to 17.6 years (mean, 11.5 years). Thin-section CT scores were determined objectively on coded images by multiple raters in a standardized fashion. Microbiologic data were obtained by means of culture of respiratory secretions by using methods for differentiation of Pseudomonas aeruginosa (PA) as either nonmucoid or mucoid. Results:Eighty-three percent of patients (68 of 82) showed bronchiectasis of varying severity. Of 12 potential risk factors, only respiratory infection with mucoid PA correlated significantly with bronchiectasis (P ϭ .041). Conclusion:The severity of bronchiectasis in children with cystic fibrosis is significantly related to respiratory infection with mucoid PA; attempts to prevent bronchiectasis should include reducing exposure to and early eradication of PA. Note: This copy is for your personal, non-commercial use only. To order presentation-ready copies for distribution to your colleagues or clients, use the Radiology Reprints form at the end of this article. Patients with cystic fibrosis (CF) are susceptible in early childhood to chronic lung disease associated with recurrent respiratory infections due to unusual respiratory pathogens such as Staphylococcus aureus and Pseudomonas aeruginosa (PA) (1). At a variable age, patients with CF will develop obstructive pulmonary dysfunction (2). In addition, the majority of children with CF show structural bronchopulmonary abnormalities (3-5), including bronchiectasis, mucous plugging, peribronchial thickening, air trapping, and parenchymal opacities. Bronchiectasis is an early and prominent feature of CF lung disease. Bronchiectasis is considered an irreversible abnormality (4,6) and thus indicates the presence of permanent damage to the lungs. Chronologically, bronchiectasis is the first irreversible change seen in children with CF (7). Because the identification of bronchiectasis indicates that treatment can no longer be expected to return the status of the lungs to normal, the prevention of bronchiectasis is of great importance in the care of the child with CF.Although there has been limited longitudinal research focused on bronchiectasis determinants in patients with CF (4), cross-sectional and short-term studies suggest that bronchiectasis correlates with significantly increased airways obstruction (8,9). In addition, Evans et al (6) reported that patients with bronchiectasis "who become colonized by P aeruginosa hav...

Escore tomográfico em pacientes com fibrose cística colonizados por Pseudomonas aeruginosa ou Staphylococcus aureus

“…(12) A study involving 82 patients with CF and evaluating 12 risk factors for developing irreversible lesions (such as bronchiectasis) found that only colonization with mucoid P. aeruginosa was significantly correlated with the presence of bronchiectasis. (19) The results of the present study are in agreement with those of the studies cited above. All of the patients who were chronically colonized with P. aeruginosa presented with the mucoid phenotype, and HRCT revealed a significantly higher prevalence of bronchiectasis in those patients.…”

High resolution computed tomography and classification of children with interstitial lung diseases

“…One case was also combined with patchy ground-glass opacities. Results of previous studies (Brody, 2008;Farrell et al, 2009) and the present study suggest that bronchiectasis is the first indicator of the final pathological stage of children's ILD and is an unrecoverable image manifestation.…”

Clinical Significance of Microbial Infection and Adaptation in Cystic Fibrosis

“…Interpretation of these investigations is complicated by the fact that some experts feel that emergence of the mucoid phenotype coincides with the transition from the intermittent to the chronic phase of infection (293,452,464) whereas others feel that chronic infection usually precedes the emergence of mucoid P. aeruginosa (30). Regardless of these distinctions, it is clear that chronic infection with mucoid P. aeruginosa is associated with poorer outcomes in CF patients (184,250,269). Pedersen and colleagues performed spirometry on 73 patients with CF over a period of 13 years (453).…”