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Serial Lung Function and Responsiveness in Cystic Fibrosis during Early Childhood

Kim G. Nielsen, Tacjana Pressler, Bent Klug, Christian Koch and Hans Bisgaard

Department of Pediatrics, Copenhagen University Hospital, Rigshospitalet; Department of Pediatrics, Copenhagen University Hospital, Hvidovre; Department of Pediatrics, Copenhagen University Hospital, Gentofte, Copenhagen, Denmark

Correspondence and requests for reprints should be addressed to Kim G. Nielsen, M.D., Department of Pediatrics, Pulmonary Service, 5003, Copenhagen University Hospital, Rigshospitalet, DK-2100 Copenhagen, Denmark. E-mail: kgn{at}dadlnet.dk

In a 4-year prospective study, we evaluated specific airway resistance (sRaw) by whole-body plethysmography, respiratory resistance by the interrupter technique, and respiratory resistance and reactance at 5 Hz by the impulse oscillation technique combined with measurement of responsiveness to bronchodilators and cold air in 30 children (mean [range] age 5.7 [2 to 8] years) with cystic fibrosis (CF). Spirometry was done at school age. Mean sRaw was consistently abnormal: the mean z score (SD) was 2.52 (2.02) (p < 0.001) at the start and was unchanged 36 months later at 2.74 (2.02). Mean z score (SD) for FEV₁ at first satisfactory measurement, at a mean age (range) of 6.1 (4.9–7.5) years was –1.2 (1.2) and was further reduced to –1.85 (1.2) 4 years from inclusion at a mean age (range) of 9.9 (6.8–12) years. Neither respiratory resistance by the interrupter technique nor the impulse oscillation technique demonstrated consistent abnormal levels. Patients with CF as a group did not differ from healthy subjects in responsiveness to bronchodilators and cold air. sRaw may be a useful tool in CF during early childhood. Reduced lung function was documented from consistently abnormal levels of sRaw and FEV₁ during the study. Bronchodilator responsiveness and response to cold air challenge were normal.

Key Words: cystic fibrosis • children • lung function

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