LUNG-FUNCTION IN SOUTH-AFRICAN CHILDREN WITH CYSTIC-FIBROSIS

Objective. To determine the pattern of lung function in stable cystic fibrosis (CF) patients and to investigate the relationship of abnormal lung function to demographic variables, CF genotype and pulmonary col onisation with Pseudomonas aeruginosa (PA). Design. A descriptive stud y done at the CF clinic at Red Cross War Memorial Children's Hospital in Cape Town. Methods. Data were recorded and pulmonary function testi ng (PFT) was performed in 42 CF patients. Results. 29 patients (69%) h ad mild disease, while 11 (26%) and 2 (5%) had moderate and severe dis ease respectively, Twenty-four patients (57%) demonstrated lower airwa y obstruction (LAO). Patients with moderate or severe disease were sig nificantly older than those with mild disease (13.3 (3.7) years (mean (SD)) compared with 11.1 (3.0) years (t = 2.1; P = 0.04)). PA colonisa tion status differed significantly with the pattern of lung function ( chi(2) = 5.6; P = (0.04) and severity of lung disease (chi(2) = 12.6; P = 0.002). Nine (35%) of the 26 patients tested before and after bron chodilator therapy showed a positive response. Conclusion. The majorit y of patients had mildly impaired or normal lung function, with LAO pr edominating. A minority of patients were bronchodilator-responsive. PA colonisation may be associated with the development of abnormal lung function and more severe pulmonary disease.