Pulmonary function tests in preschool children with cystic fibrosis.
Pulmonary function tests have rarely been assessed in preschool children with cystic fibrosis (CF). The objective of this multicenter study was to compare pulmonary function in 39 preschool children with CF (height, 90-130 cm; 16 homozygous Delta F508) and in 79 healthy control children. Functional residual capacity (helium dilution technique) and expiratory interrupter resistance (Rint(exp)) (interrupter technique) were measured. As compared with control children, children with CF had significantly higher Rint(exp), expressed as absolute values and as Z-scores (1.05 +/- 0.36 versus 0.80 +/- 0.20 kPa.L(-1). second, p < 0.0001; and 1.31 +/- 1.72 versus 0.19 +/- 0.97, p < 0.0001), and significantly lower specific expiratory interrupter conductance (1.29 +/- 0.34 versus 1.63 +/- 0.43 kPa(-1). second, p < 0.0001). The effect of the bronchodilator salbutamol on Rint(exp) was not significantly different between children with CF and control children. Rint(exp) Z-scores were significantly higher in children with CF who were exposed to passive smoke (n = 8) (p < 0.03). Children with CF and with a history of respiratory symptoms (n = 31) had significantly higher functional residual capacity Z-scores (p < 0.02) and lower specific expiratory interrupter conductance Z-scores (p < 0.04). Genotype did not influence the data. We conclude that Rint(exp) and functional residual capacity measurements may help to follow young children with CF who are unable to perform reproducible forced expiratory maneuvers.
Beydon
Nicole
N
Department of Physiology of the Robert Debré Teaching Hospital, Paris, France.
Amsallem
Francis
F
Bellet
Mireille
M
Boulé
Michèle
M
Chaussain
Michèle
M
Denjean
André
A
Matran
Régis
R
Pin
Isabelle
I
Alberti
Corinne
C
Gaultier
Claude
C
D000403:Airway Resistance; D001993:Bronchodilator Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005260:Female; D005652:Functional Residual Capacity; D006720:Homozygote; D006801:Humans; D008297:Male; D015656:Respiratory Mechanics; D014028:Tobacco Smoke Pollution / Q000009:adverse effects
D001993:Bronchodilator Agents; C505032:CFTR protein, human; D014028:Tobacco Smoke Pollution; D019005:Cystic Fibrosis Transmembrane Conductance Regulator