PT -期刊文章盟Rubbo布鲁纳AU -最佳Sunayna AU -赫斯特,罗伯特•AU - Gahleitner Florian AU -卡尔,西沃恩·B非盟- Chetcuti,菲利普AU -豪格,克莱尔盟——Kenia Priti AU -卢卡斯,简非盟-火山泥,爱德华多盟——Narayanan Manjith AU -奥卡拉汉,克里斯托弗AU -威廉姆森,迈克尔AU -沃克,伍尔夫TI -临床特征,英国国家队列援助(English National Cohort AID)中334名原发性纤毛运动障碍儿童的治疗。PA5024 DP - 2018年9月15日TA -欧洲呼吸杂志PG - PA5024 VI - 52 IP - 62 4099 - //www.qdcxjkg.com/content/52/suppl_62/PA5024.short 4100 - //www.qdcxjkg.com/content/52/suppl_62/PA5024.full -欧元和J2018 9月15日;52 AB -简介:原发性纤毛运动障碍(PCD)是一种罕见的情况，粘液纤毛清除受损导致复发性窦肺感染。支气管扩张。2012年，英国4个中心委托成立了全国儿童PCD管理服务中心。这项研究的目的是描述在这项新服务中所有英国儿童的特点。方法:详细诊断、表型分析及临床应用。管理数据是在2014年所有儿童的年度回顾评估中收集的。结果:对334名儿童进行了年度回顾。研究中位年龄为9.8岁。诊断时的中位年龄为2.6岁，然而右位心患者的中位年龄显著降低(1.0岁vs 6.2岁，p<0.0001)。 Mean (SD) FEV1 z score was -1.85 (1.39) equivalent to a mean (SD) % predicted FEV1 76.9% (17.6) (n=242). Compared with National data in cystic fibrosis (CF), FEV1 was lower in PCD children of all age groups. This was more evident in younger children, in 6-7yr olds median predicted FEV1 was 96% in CF vs 81.4% in PCD. 56% of children had varying degrees of hearing impairment but this improved significantly with age, p=0.03. Children with a lower BMI had a lower FEV1, p<0.0001. Regular intravenous antibiotics, prophylactic antibiotics & nebulised hypertonic saline were used in children with lower mean FEV1 z scores compared to those not on these treatments (-2.37 vs -1.62, p=0.0001; -2.02 vs -1.61, p=0.02 & -2.13 vs -1.43, p=0.02 respectively).Conclusion We provide evidence that, far from being a mild respiratory condition, children with PCD have worse lung function than those with CF. This highlights the need for intensive management of PCD by a multi-professional specialist team.FootnotesCite this article as: European Respiratory Journal 2018 52: Suppl. 62, PA5024.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).