Hattori Y, Watanabe Y, Okuda M
Department of Otolaryngology, Nippon Medical School, Tokyo.
Nihon Jibiinkoka Gakkai Kaiho. 1991 Jul;94(7):949-62. doi: 10.3950/jibiinkoka.94.949.
Nasal mucosal cilia were observed with electron microscope in 14 patients with immotile cilia syndrome (ICS), 9 with nasal papilloma (NP), 23 with sinobronchial syndrome (SB), 2 with sinusitis combined with dextrocardia (SC), 1 with Kartagener's syndrome (KS), and 5 normal controls (C). Abnormalities such as complex cilia, cilia with abnormal axonemes and cilia with randomly oriented central microtubules were frequently found in the groups of ICS (8.1%) and NP (10.4%) while less in other groups: SB (4.9%), SC (5.3%), KS (4.7%) and C (3.9%). The percentage of cilia with defective dynein arms (DA) was the highest in the ICS group (94.0%), followed by the groups of SC (53.7%), SB (47.5%), NP (41.2%), C (35.8%) and KS (33.3%). The ICS group was found to be the largest in the number of defective DA per a cilium (4.1), followed by the groups of NP (1.0), SB (0.6), SC (0.7), KS (0.4) and C (0.4). Increased rates of defective DA were also recognized in cilia of tracheal mucosa and flagella of sperm in 7 patients with ICS examined. In conclusion, neither abnormal cilia nor defective DA of cilia are specific findings for ICS. However, when we observe these findings in high percentage in nasal mucosa as well as in other organs, we may define this condition as ICS.
对14例纤毛不动综合征(ICS)患者、9例鼻乳头状瘤(NP)患者、23例鼻窦支气管综合征(SB)患者、2例鼻窦炎合并右位心(SC)患者、1例卡塔格内综合征(KS)患者及5名正常对照者(C)的鼻黏膜纤毛进行了电子显微镜观察。在ICS组(8.1%)和NP组(10.4%)中,经常发现复杂纤毛、轴丝异常的纤毛以及中央微管随机排列的纤毛等异常情况,而在其他组中较少见:SB组(4.9%)、SC组(5.3%)、KS组(4.7%)和C组(3.9%)。动力蛋白臂(DA)缺陷的纤毛百分比在ICS组最高(94.0%),其次是SC组(53.7%)、SB组(47.5%)、NP组(41.2%)、C组(35.8%)和KS组(33.3%)。发现ICS组每根纤毛中DA缺陷的数量最多(4.1),其次是NP组(1.0)、SB组(0.6)、SC组(0.7)、KS组(0.4)和C组(0.4)。在检查的7例ICS患者中,气管黏膜纤毛和精子鞭毛中DA缺陷率也有所增加。总之,异常纤毛和纤毛DA缺陷均不是ICS的特异性表现。然而,当我们在鼻黏膜以及其他器官中高比例观察到这些发现时,我们可能将这种情况定义为ICS。