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Published ahead of print on March 17, 2004, doi:10.1164/rccm.200303-347OC

Am. J. Respir. Crit. Care Med., Volume 169, Number 11, June 2004, 1209-1216

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Submitted on March 13, 2003
Accepted on March 14, 2004

Serial Lung Function and Responsiveness in Cystic Fibrosis during Early Childhood

KIM G NIELSEN1*, TACJANA PRESSLER1, BENT KLUG2, CHRISTIAN KOCH1, and HANS BISGAARD3

1 Department of Pediatrics, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark, 2 Department of Pediatrics, Copenhagen University Hospital, Hvidovre, Copenhagen, Denmark, 3 Department of Pediatrics, Copenhagen University Hospital, Gentofte, Copenhagen, Denmark

* To whom correspondence should be addressed. E-mail: KGN{at}DADLNET.

In a 4-year prospective study we evaluated specific airway resistance (sRaw) by whole-body plethysmography; respiratory resistance by interrupter technique (Rint) and respiratory resistance and reactance at 5 Hz by impulse oscillation technique (IOS) 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: mean z-score (SD) 2.52 (2.02) (p< 0.001) at start and unchanged 36 mo later at 2.74 (2.02). Mean z-score (SD) for FEV1 at first satisfactory measurement, mean age (range) 6.1 (4.9 to 7.5) years was -1.2 (1.2) and further reduced to -1.85 (1.2) 4 yr from inclusion, at mean age (range) 9.9 (6.8 to 12) years. Neither Rint nor IOS demonstrated consistent abnormal levels. CF patients as group did not differ from healthy subjects in responsiveness to bronchodilators and cold air. In conclusion, 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 study. BDR and response to CACh was normal.
Key words: LUNG FUNCTION, CHILDREN, CYSTIC FIBROSIS




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