Sunther Meera, Bush Andrew, Hogg Claire, McCann Lauren, Carr Siobhán B
Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, United Kingdom.
Pediatr Pulmonol. 2016 Dec;51(12):1362-1366. doi: 10.1002/ppul.23479. Epub 2016 Jun 8.
Spirometry in children with cystic fibrosis (CF) frequently fails to return to baseline after treatment for a pulmonary exacerbation. It is unclear whether the same is true for children with primary ciliary dyskinesia (PCD).
To determine in children with PCD treated with intravenous antibiotics for a pulmonary exacerbation: (1) the proportion who recover to baseline forced expiratory volume at 1 sec (FEV ) within 3 months after treatment and (2) to try to identify factors which are associated with failure to regain pre-exacerbation FEV .
Cohort study using the PCD database for children at the Royal Brompton Hospital, 2003-2013. We selected the first pulmonary exacerbation treated with intravenous antibiotics. The best FEV within 3 months after treatment was compared to the best FEV in the 12 months before treatment (baseline). Recovery to baseline was defined as any FEV after treatment that was greater than or equal to 90% of the baseline FEV .
32/150 children (21%) had at least one pulmonary exacerbation. 23/30 (77%) regained baseline spirometry within 3 months of treatment. There was no difference between responders and non-responders in any baseline characteristics.
Around 25% of children with PCD fail to recover to baseline lung function within 3 months following treatment for a pulmonary exacerbation, similar to CF. Better treatment strategies are needed, and the results also suggest that prevention of exacerbations would be a useful end-point in clinical trials. Pediatr Pulmonol. 2016;51:1362-1366. © 2016 Wiley Periodicals, Inc.
囊性纤维化（CF）患儿在肺部加重期接受治疗后，肺功能检查结果常常无法恢复到基线水平。目前尚不清楚原发性纤毛运动障碍（PCD）患儿是否也是如此。
对于因肺部加重期接受静脉抗生素治疗的PCD患儿，确定：（1）治疗后3个月内恢复到基线第1秒用力呼气量（FEV₁）的患儿比例；（2）试图找出与未能恢复到加重前FEV₁相关的因素。
采用皇家布朗普顿医院2003年至2013年PCD患儿数据库进行队列研究。我们选取首次因静脉抗生素治疗的肺部加重期病例。将治疗后3个月内的最佳FEV₁与治疗前12个月内的最佳FEV₁（基线）进行比较。恢复到基线定义为治疗后的任何FEV₁大于或等于基线FEV₁的90%。
150名患儿中有32名（21%）至少有一次肺部加重期。23/30名（77%）患儿在治疗后3个月内恢复了肺功能检查的基线水平。反应者和无反应者在任何基线特征上均无差异。
约25%的PCD患儿在肺部加重期治疗后3个月内未能恢复到基线肺功能，与CF患儿相似。需要更好的治疗策略，研究结果还表明，预防病情加重将是临床试验中一个有用的终点。《儿科肺科杂志》。2016年；51:1362 - 1366。©2016威利期刊公司
