布什% 0期刊文章%,%科尔,P %哈里里,M %麦凯,我%菲利普斯,G %奥卡拉汉,C %威尔逊,R %华纳,乔% T原发性纤毛运动障碍:护理诊断和标准% D J欧洲呼吸杂志1998% % P 982 - 988 X % V 12% N 4%原发性纤毛运动障碍(PCD)的特点是上、下呼吸道的疾病,与内脏镜像布置在50%的情况下,由于纤毛的结构和/或功能异常。本文就PCD的临床特点、诊断及治疗作一综述。临床表现包括新生儿呼吸窘迫、复发性下呼吸道感染、慢性鼻窦炎和男性不育。PCD进入支气管扩张、非典型哮喘和异常严重的上呼吸道疾病的鉴别诊断。诊断是通过一系列的调查,从10岁以上患者的糖精试验开始;光镜下纤毛搏动频率与模式的研究电镜观察纤毛的形态和方向。重要的是不要混淆原发性和继发性纤毛异常。鼻一氧化氮在PCD中含量很低,这项测量显示出作为PCD筛查试验的前景。诊断是重要的,以防止发展支气管扩张和避免任何不必要的耳鼻喉手术。 Regular follow-up is essential, and management should be multidisciplinary, with input from centres with a special interest in PCD, having access to paediatric and adult chest physicians, otolaryngologists and audiological physicians, physiotherapists, counselling services and fertility clinics. The prognosis is good, but morbidity can be considerable if PCD is incorrectly managed. %U //www.qdcxjkg.com/content/erj/12/4/982.full.pdf