Copenhagen University Hospital, Rigshospitalet, Danish PCD Center, Pediatric Pulmonary Service, Blegdamsvej 9, DK-2100 Copenhagen, Denmark.
Am J Respir Crit Care Med. 2010 Jun 1;181(11):1262-8. doi: 10.1164/rccm.200811-1731OC. Epub 2010 Feb 18.
Early diagnosis and treatment is considered important to prevent lung damage in primary ciliary dyskinesia (PCD).
Few studies have addressed long-term evolution of lung function after PCD diagnosis. We investigated whether long-term lung function was dependent on age or level of lung function at PCD diagnosis.
An observational, single-center, cross-sectional, and three-decade longitudinal study of FEV(1) and FVC related to age at diagnosis until current age was performed. Linear regression was used to describe the relation between first measured lung function values and age at diagnosis across the cohort. Courses of lung function after diagnosis and the according slopes were used to group patients into increasing, stable, or decreasing courses. Additionally, slopes from courses of 10 years of follow-up were related to age at diagnosis and initial level of lung function, respectively, using linear regression.
Seventy-four children and adults with PCD were observed for median 9.5 (range, 1.5-30.2) years during which 2,937 lung function measurements were performed. First measured FEV(1) was less than 80% of predicted in one-third of preschool-diagnosed children. During observation, 34% of patients lost more than 10 percentage points, 57% were stable, and 10% improved more than 10 percentage points in FEV(1). Courses of lung function after diagnosis were related to neither age at diagnosis nor initial level.
Our study strongly suggests that PCD is a disease of serious threat to lung function already at preschool age, and with a high degree of variation in courses of lung function after diagnosis that was not linked to either age or level of lung function at diagnosis. Early diagnosis did not protect against decline in lung function.
原发性纤毛运动障碍（PCD）的早期诊断和治疗被认为对预防肺部损伤很重要。
很少有研究探讨 PCD 诊断后肺功能的长期演变。我们调查了肺功能是否长期依赖于诊断时的年龄或肺功能水平。
对 FEV1 和 FVC 进行了一项观察性、单中心、横断面和 30 年纵向研究，涉及诊断时的年龄直至当前年龄。线性回归用于描述整个队列中首次测量的肺功能值与诊断时年龄之间的关系。使用线性回归分别将诊断后肺功能的变化过程及其相应斜率与诊断时的年龄和初始肺功能水平相关联。
74 名患有 PCD 的儿童和成年人在中位 9.5 年（范围 1.5-30.2 年）的时间内接受了观察，在此期间进行了 2937 次肺功能测量。三分之一的学龄前儿童首次测量的 FEV1 低于预计值的 80%。在观察期间，34%的患者损失了超过 10 个百分点，57%的患者稳定，10%的患者在 FEV1 中改善了超过 10 个百分点。诊断后的肺功能变化过程与诊断时的年龄或初始肺功能水平无关。
我们的研究强烈表明，PCD 是一种严重威胁学龄前儿童肺功能的疾病，而且诊断后肺功能的变化程度很大，与诊断时的年龄或肺功能水平无关。早期诊断并不能防止肺功能下降。
