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CF Lung Disease

Laube BL, Sharpless G, Benson J et al.

The Johns Hopkins Medical Institutions, Baltimore, MD, USA.

 J Pediatr 2013 Dec 24; Epub ahead of print.

Aleksandra Norek’s review: Individuals with cystic fibrosis (CF) become infected with numerous bacteria and fungi during the course of their disease. Chronic infection of the airways is the major cause of morbidity and mortality. The most common bacterium affecting individuals with CF is Pseudomonas aeruginosa, with the majority of individuals being affected by the age of 18 years. There has been little, if any, research on whether infection with P aeruginosa affects removal of mucus from the lung in individuals with CF, or if it contributes to airway damage and morphological changes such as bronchiectasis.

In this study, the authors compared mucus removal in children with mild CF lung disease who had been recently infected with P aeruginosa with mucus removal in children with mild CF lung disease and no infection with P aeruginosa. CF children aged 7–14 years with a percent of predicted forced expiratory volume in 1s (FEV1) ≥80% were recruited to this study. All of the individuals underwent the following procedures: a focused medical history and physical examination; pulmonary function measurements of FEV1 and forced vital capacity; an induced sputum test; a high-resolution computed tomography (HRCT) scan; a xenon-ventilation scan; inhalation of aerosol generated from a solution of 99mtechnetium sulfur-colloid; and gamma camera imaging of the lungs for 90 min. There were no statistically significant differences at baseline in age, sex, pulmonary function, total and right lung HRCT scores, height, weight, body–mass index, or genotype between the P aeruginosa-positive and P aeruginosa-negative groups.

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