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 colonisation with Pseudomonas aeruginosa (PA)
Design: A descriptive study done at the CF clinic at Red Cross War Memorial Children's Hospital in Cape Town.
Methods: Data were recorded and pulmonary function testing (PFT) was performed in 42 CF patients.
Results: 29 patients (69%) had mild disease,. while 11 (26%) and 2 (5%) had moderate and severe disease respectively. Twenty-four patients (57'%) demonstrated lower airway obstruction (LAO). Patients with moderate or severe disease were significantly older than those with mild disease (13.3 (3.7) years (mean (SD)) compared with 11.1 (3.0) years (t =2.1; P =OM)). PA colonisation status differed significantly with the pattern of lung function (X2 =6.6; P = 0.04) and severity of lung disease (X2=12.6; P =0.002.). Nine (35%) of the 26 patients tested before and after broncbodilator therapy showed a positive response.
Conclusion: The majority of patients had mildly impaired or normal lung function, with LAO predominating. A minority of patients were bronchodilator-responsive. PA colonisation may be associated with the development of abnormal lung function and more severe pulmonary disease..