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Copyright ©ERS Journals Ltd 2005
Long-term clearance from small airways in patients with cystic fibrosis
1 Division of Occupational Medicine, Dept of Public Health Science, Karolinska Institutet and Occupational and Environmental Health, Stockholm, County Council, 2 Stockholm CF center, Dept of Paediatrics, Karolinska Institutet, Karolinska University Hospital, Huddinge site, 3 Division of Inhalation Toxicology, Institute of Environmental Medicine, Karolinska Institutet, and 4 Swedish Radiation Protection Authority (SSI), Stockholm, Sweden
CORRESPONDENCE: M. Lindström, Dept of Paediatrics B57, Karolinska University Hospital, Huddinge Site, S-141 86 Stockholm, Sweden. Fax: 46 858581410. E-mail: maria.lindstrom@phs.ki.se
Keywords: Cystic fibrosis, lung deposition, mucociliary clearance, small airways
Received: October 27, 2003
Accepted October 10, 2004
Impaired mucociliary clearance is a hallmark of cystic fibrosis (CF). Early morphological changes first appear in the small airways. Lung clearance was investigated in 11 young CF adults with mild-to-moderate lung disease using a method depositing particles mainly in the small airways.
Radiolabelled Teflon particles (6 µm) were inhaled with an extremely slow inhalation flow, 0.05 L·s–1. Lung retention was measured immediately following inhalations and, on four occasions up to 21 days. The results were compared with data from healthy subjects.
The lung retention at 24 h in % of deposition was 67% (95% confidence interval 58–76) in the CF patients, compared to 48% (42–53) in the healthy subjects. Clearance on days 1–7 was larger in the CF patients, 22% (15–29) compared to the healthy subjects, 14% (12–16). No difference was observed between the CF patients and the healthy subjects in the slow clearance phase at day 7 to day 21, representing small airway clearance. Impaired mucociliary clearance in CF patients results in increased 24-h retention and a prolonged rapid clearance phase.
The results of the study do not support the current authors' hypothesis that clearance from small airways is slower in cystic fibrosis patients compared to healthy subjects. Furthermore, the data suggest that mucociliary transport is not the dominant clearance mechanism in small airways.
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