Longitudinal study of lung function in a cohort of primary ciliary dyskinesia
Longitudinal study of lung function in a cohort of primary ciliary dyskinesia
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DOI：
10.1183/09031936.97.10102376
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发表时间：
1997-10-01
影响因子：
24.3
通讯作者：
Bisgaard, H
中科院分区：
文献类型：
--
作者：
Ellerman, A;Bisgaard, H
原发性纤毛运动障碍（PCD）患者有明显的呼吸道分泌物停滞，因此经常发生下呼吸道感染，这引起了对肺功能发展的关注。24例PCD患者在我们的诊所进行了2-16年的标准化治疗方案的前瞻性研究，包括每年2-4次肺活量测定，每日物理治疗和每月痰培养，随后进行特异性抗生素治疗。与儿童时期进入队列的PCD患者相比，12名成年PCD患者的肺功能显著降低(用力肺活量（FVC） 70 vs 85%预测；1秒用力呼气量（FEV1）为59%，对照组为72%。肺损伤与纤毛运动障碍的类型无关。在随后对两组的监测中位数分别为14年和7年，大多数患者的肺功能保持稳定。结论：原发性纤毛运动障碍伴肺功能进行性恶化，如果治疗不充分，但肺功能可以通过适当的抗生素治疗和定期物理治疗维持。这强调了早期诊断原发性纤毛运动障碍的必要性。
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.