Lung function from infancy to the preschool years after clinical diagnosis of cystic fibrosis.
BACKGROUND
After recent standardization of forced expiratory maneuvers for both infants and preschool children, longitudinal measurements are now possible from birth.


OBJECTIVE
The aim of this study was to investigate the evolution of lung function during the first 6 years of life after a clinical diagnosis of cystic fibrosis (CF) in infancy in children with CF and in healthy control subjects.


METHODS
The raised volume technique was used during infancy and incentive spirometry during the preschool years.


RESULTS
Forty-eight children with CF and 33 healthy control subjects had up to seven (median, 3) measurements. Over these early years, the diagnosis of CF itself accounted for a significant mean reduction of 7.5% (95% confidence interval, 0.9 - 13.6%) in FEV(0.75) and 15.1% (95% confidence interval, 3.6 - 25.3%) in FEF(25-75). Wheeze on auscultation, recent cough, and Pseudomonas aeruginosa (PsA) infection (even if apparently effectively treated) were all independently associated with further reductions in lung function. Premorbid lung function did not predict infection with PsA.


CONCLUSIONS
This is the first study to describe physiologic measurements from infancy through the preschool years in subjects with CF and healthy control subjects, the understanding of which is critical for future intervention trials. Airflow obstruction in uncomplicated CF persists through the preschool years despite treatment, with PsA acquisition being associated with further deterioration in lung function, even when apparently eradicated. This suggests that new therapies are needed to treat the airflow obstruction of uncomplicated CF, and rigorous strategies to prevent PsA acquisition.
Kozlowska
Wanda J
WJ
Portex Anaesthesia, Intensive Therapy and Respiratory Medicine Unit, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK. w.kozlowska@ich.ucl.ac.uk
Bush
Andrew
A
Wade
Angela
A
Aurora
Paul
P
Carr
Siobhán B
SB
Castle
Rosie A
RA
Hoo
Ah-Fong
AF
Lum
Sooky
S
Price
John
J
Ranganathan
Sarath
S
Saunders
Clare
C
Stanojevic
Sanja
S
Stroobant
John
J
Wallis
Colin
C
Stocks
Janet
J
D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000382:microbiology / Q000503:physiopathology*; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008297:Male; D008450:Maximal Midexpiratory Flow Rate; D011552:Pseudomonas Infections / Q000150:complications; D012129:Respiratory Function Tests; D013147:Spirometry; D014797:Vital Capacity