Royal Brompton & Harefield NHS Foundation Trust, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK.
National Heart and Lung Institute, Imperial College, London, UK.
Lung. 2017 Aug;195(4):441-443. doi: 10.1007/s00408-017-0022-5. Epub 2017 Jun 20.
Primary ciliary dyskinesia is a condition in which abnormal cilia structure or function leads to reduced mucociliary clearance and obstructive lung disease. Twenty-nine patients had lung clearance index (LCI) measured in 2009 and we attempted to perform a 5-year follow-up. Only 12 patients could be re-recruited, but in this small group LCI was stable over the 5 years, which confirms previous data showing that spirometry is also stable in these patients over the medium term. The two patients with the highest LCI in 2009 had since died, despite one having relatively preserved spirometry at the time. These data may be used to inform sample size calculations of future studies.
原发性纤毛运动障碍是一种由于纤毛结构或功能异常导致黏液纤毛清除功能降低和阻塞性肺疾病的病症。29 名患者于 2009 年进行了肺清除指数（LCI）的测量，我们试图进行 5 年随访。仅重新招募到 12 名患者，但在这个小群体中，LCI 在 5 年内保持稳定，这证实了之前的数据表明，这些患者的中期肺功能检查也是稳定的。2009 年 LCI 最高的两名患者此后死亡，尽管其中一名患者当时的肺功能检查相对保留。这些数据可用于为未来研究的样本量计算提供信息。
