Hellinckx J, Demedts M, De Boeck K
Department of Paediatrics, Herestraat, Leuven, Belgium.
Eur J Pediatr. 1998 May;157(5):422-6. doi: 10.1007/s004310050843.
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 microg 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.
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.
对11例原发性纤毛运动障碍患者进行了肺功能测试。他们的平均年龄为15岁（范围6 - 32岁）。他们的肺功能呈阻塞性，肺活量（平均值±标准差）为预测值的75%±20%，第1秒用力呼气量（FEV1）为预测值的63%±20%，残气量升高至预测值的169%±50%。吸入200微克沙丁胺醇后，FEV1的平均变化为基线值的 + 13.2%±9.6%。在10例年龄较大的患者中，在3至20年期间定期测量了肺功能。有趣的是，在儿童期和青春期，病情进展并非不利：肺活量增加了8%±20%，FEV1保持稳定（平均变化0.3%±12%）。只有2例患者病情进展不利。
在诊断时，原发性纤毛运动障碍患者患有部分可逆性阻塞性气道疾病。在定期随访和治疗期间，没有证据表明肺功能会进一步下降。伴有免疫缺陷或在治疗开始时存在严重损害的患者预后可能更差。
