Buchdahl R M, Reiser J, Ingram D, Rutman A, Cole P J, Warner J O
Paediatric Respiratory Department, Brompton Hospital, London.
Arch Dis Child. 1988 Mar;63(3):238-43. doi: 10.1136/adc.63.3.238.
One hundred and sixty seven children, ranging in age from 5 weeks to 16 years, with chronic upper or lower respiratory tract problems, or both, were investigated for ciliary dyskinesia. Abnormal ciliary function was found in 18 cases all of whom had chronic lower respiratory disease and most of whom also had upper respiratory problems. Fifteen of the 18 cases had reduced ciliary beat frequencies (less than 10 Hz) associated with dyskinesia and the other three showed apparent absence of ciliated cells. Of the 15 cases with reduced ciliary beat frequencies, ciliary ultrastructure was normal in seven cases but abnormal with missing dynein arms and occasional abnormalities of microtubular arrangement in eight. Respiratory symptoms in the perinatal period were more common in children with abnormal ciliary function and present in all those with ultrastructural abnormalities or absence of ciliated cells compared with 34 (26%) of 132 children, in whom symptoms were recorded, with normal ciliary function. This study would suggest that all children with unexplained chronic respiratory disease, in particular those with symptoms starting in the perinatal period, should be investigated for ciliary dyskinesia.
对167名年龄在5周至16岁之间、患有慢性上呼吸道或下呼吸道问题或两者皆有的儿童进行了纤毛运动障碍调查。在18例中发现纤毛功能异常，所有这些病例均患有慢性下呼吸道疾病，且大多数也有上呼吸道问题。18例中的15例纤毛摆动频率降低（低于10Hz）并伴有运动障碍，另外3例显示明显无纤毛细胞。在15例纤毛摆动频率降低的病例中，7例纤毛超微结构正常，8例异常，有动力蛋白臂缺失且偶尔有微管排列异常。与132例纤毛功能正常且有呼吸道症状记录的儿童中的34例（26%）相比，纤毛功能异常的儿童围生期呼吸道症状更常见，且所有有超微结构异常或无纤毛细胞的儿童均有此症状。这项研究表明，所有患有无法解释的慢性呼吸道疾病的儿童，尤其是那些围生期开始出现症状的儿童，都应进行纤毛运动障碍调查。
