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: Pulmonary function tests were obtained in 11 patients with primary ciliary dyskinesia. Their mean age was 15 years (range 6-32). Their pulmonary function was obstructive, with a vital capacity (mean /- SD) of 75% /- 20% predicted, a forced expiratory volume in 1s (FEV1) of 63% /- 20% predicted and a raised residual volume of 169% /- 50% predicted. After inhalation of 200 [mu]g of salbutamol the mean change in FEV1 was 13.2% /- 9.6% of the baseline value. In the 10 oldest patients, lung function had been measured at regular intervals during 3-20 years. Interestingly, during childhood and adolescence the evolution was not unfavourable: vital capacity increased by 8% /- 20% and FEV1 remained stable (mean change 0.3% /- 12%). Only 2 patients had an unfavourable evolution.

Conclusion: At time of diagnosis, patients with primary ciliary dyskinesia have partially reversible obstructive airway disease. During regular follow up and therapy, there is no evidence of a further decline in lung function. Patients with associated immunodeficiency or important damage at the start of therapy may have a worse prognosis.

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