Kawakami M, Hattori Y, Nakamura S
Department of Pneumology, Tokyo Metropolitan Hiroo General Hospital.
Intern Med. 1996 Aug;35(8):617-23. doi: 10.2169/internalmedicine.35.617.
In immotile cilia syndrome (ICS), respiratory symptoms show a wide range of severity. Various types of axonemal abnormalities related to ICS are known, but it is unclear whether the symptom severity is related to the type of abnormality. We sent a questionnaire concerning respiratory symptoms to ICS patients. Another type of the questionnaire regarding the clinical features and axonemal abnormality of ICS was sent to the physicians in charge. Analysis of the results showed that in the 48 patients responding, those lacking outer dynein arms (LO) in cilia were more likely to be associated with situs inversus (SI) than those lacking inner dynein arms (LI). Sputum symptoms were more serious in those associated with SI than those unassociated with SI, as was the case in those associated with LO compared to those with LI. Based on these results, it may be concluded that the type of dynein arm defect in cilia is related to the clinical features in ICS.
在不动纤毛综合征（ICS）中，呼吸道症状的严重程度差异很大。已知与ICS相关的各种轴丝异常类型，但尚不清楚症状严重程度是否与异常类型有关。我们向ICS患者发送了一份关于呼吸道症状的问卷。另一份关于ICS临床特征和轴丝异常的问卷发送给了主治医生。结果分析表明，在48名有回应的患者中，与缺乏内动力蛋白臂（LI）的患者相比，纤毛中缺乏外动力蛋白臂（LO）的患者更易伴有内脏反位（SI）。与未伴有SI的患者相比，伴有SI的患者痰液症状更严重，与伴有LI的患者相比，伴有LO的患者也是如此。基于这些结果，可以得出结论，纤毛中动力蛋白臂缺陷的类型与ICS的临床特征有关。
