Kroon A A, Heij J M, Kuijper W A, Veerman A J, van der Baan S
Department of Otolaryngology/Head and Neck Surgery, Free University Hospital, Amsterdam, The Netherlands.
Clin Otolaryngol Allied Sci. 1991 Jun;16(3):294-7. doi: 10.1111/j.1365-2273.1991.tb00934.x.
The activity and ultrastructure of respiratory cilia were studied in 13 individuals with situs inversus but without serious respiratory complaints, in 8 patients with Kartagener's syndrome and in 50 healthy controls without situs inversus. In 3 individuals with situs inversus abnormal ciliary activity was found, comparable in every aspect to the ciliary activity in patients with Kartagener's syndrome. In 4 individuals with situs inversus a dynein arm deficiency was present in the cilia, with abnormal ciliary activity in 3 of them. The number of outer dynein arms per cilium differed significantly between the 3 groups (P less than 0.02). Apparently, situs inversus without serious respiratory complaints can be associated with primary ciliary dyskinesia. Based on the findings in this study, the incidence of primary ciliary dyskinesia in the Caucasian population appears to be 1:12,500.
对13例内脏转位但无严重呼吸系统疾病的个体、8例患有卡塔格内综合征的患者以及50例无内脏转位的健康对照者的呼吸纤毛活动和超微结构进行了研究。在3例内脏转位个体中发现了异常的纤毛活动，在各方面都与卡塔格内综合征患者的纤毛活动相当。在4例内脏转位个体中，纤毛存在动力蛋白臂缺陷，其中3例纤毛活动异常。三组之间每个纤毛的外动力蛋白臂数量有显著差异（P<0.02）。显然，无严重呼吸系统疾病的内脏转位可能与原发性纤毛运动障碍有关。基于本研究的结果，白种人群中原发性纤毛运动障碍的发病率似乎为1:12,500。
