Primary ciliary dyskinesia in children
10.3760/cma.j.issn.2095-428X.2018.04.017
- VernacularTitle:儿童原发性纤毛运动障碍
- Author:
Hao WANG
1
;
Baoping XU
Author Information
1. 100045 国家儿童医学中心,首都医科大学附属北京儿童医院呼吸科,国家呼吸系统疾病临床医学研究中心
- Keywords:
Primary ciliary dyskinesia;
Cilia;
Child
- From:
Chinese Journal of Applied Clinical Pediatrics
2018;33(4):306-308
- CountryChina
- Language:Chinese
-
Abstract:
Primary ciliarry dyskinesia (PCD) is a group of inherited genetic disorder caused by ciliary dyskinesia,the onset age of which is often in childhood,manifestations as recurrent respiratory infections,chronic bronchitis,chronic sinusitis,chronic otitis media,bronchiectasis and so on.Ciliary ultrastructural defects seen by transmission electron microscopy are considered as the golden criteria of PCD diagnosis.Other investigations mav help the diagnosis,which include ciliary beat frequency and pattern analysis,nasal nitric oxide,and gene test.There is no special effective therapy of PCD,and mainly symptomatic treatment is used.Early diagnosis and the prevention of recurrent respiratory infections can delay the bronchiectasis formation and improve the prognosis.
