Ellerman A, Bisgaard H
Eur Respir J. 1997 Oct;10(10):2376-9. doi: 10.1183/09031936.97.10102376.
Patients with primary ciliary dyskinesia (PCD) have pronounced stasis of their respiratory secretions and therefore recurrent lower airway infections, which raises concerns for the development of lung function. Twenty four patients with PCD have been studied prospectively with a standardized regime in our clinic for 2-16 yrs with clinic visits, including spirometry 2-4 times per year, daily physiotherapy and monthly sputum cultures with subsequent specific antibiotic treatment. Lung function was significantly lower in the 12 PCD patients entering the cohort as adults when compared to the PCD patients entering as children (forced vital capacity (FVC) 70 versus 85% predicted; forced expiratory volume in one second (FEV1) 59 versus 72% pred). The lung damage did not relate to the type of ciliary dyskinesia. During the subsequent surveillance of the groups for a median of 14 and 7 yrs, respectively, the lung function remained stable in most patients. It is concluded that primary ciliary dyskinesia is accompanied by a progressive deterioration in lung function if undertreated, but lung function can be maintained with appropriate antibiotic treatment and regular physiotherapy. This emphasizes the need for early diagnosis of primary ciliary dyskinesia.
原发性纤毛运动障碍（PCD）患者存在明显的呼吸道分泌物潴留，因此会反复发生下呼吸道感染，这引发了对肺功能发展的担忧。我们诊所对24例PCD患者进行了前瞻性研究，采用标准化方案，为期2至16年，包括门诊就诊，每年进行2至4次肺活量测定、每日物理治疗以及每月进行痰培养并随后进行针对性抗生素治疗。与儿童期确诊的PCD患者相比，12例成年期确诊的PCD患者的肺功能显著更低（用力肺活量（FVC）预计值分别为70%和85%；一秒用力呼气容积（FEV1）预计值分别为59%和72%）。肺损伤与纤毛运动障碍的类型无关。在随后分别对两组进行中位时间为14年和7年的监测期间，大多数患者的肺功能保持稳定。得出的结论是，如果治疗不足，原发性纤毛运动障碍会伴有肺功能的进行性恶化，但通过适当的抗生素治疗和定期物理治疗可以维持肺功能。这强调了早期诊断原发性纤毛运动障碍的必要性。
