Chemes H E, Morero J L, Lavieri J C
Laboratory of Testicular Physiology and Pathology, Buenos Aires Childrens' Hospital, Argentina.
Int J Androl. 1990 Jun;13(3):216-22. doi: 10.1111/j.1365-2605.1990.tb00979.x.
Two patients suspected of suffering from ciliary dyskinesis were investigated. They consulted for primary infertility and chronic respiratory disease. Functional lung studies showed obstructive changes in one patient. Both had immotile sperm with short, thick and rigid tails. Ultrastructural studies of nasal biopsies showed abnormal cilia with almost complete lack of inner dynein arms (mean number of inner arms per axoneme 0.67 +/- 1.21 in patient 1 and 1.49 +/- 1.17 in patient 2, compared with normal values of 5.3 +/- 0.13). Other abnormalities included lack of parallel orientation of cilia and central translocation of microtubular doublets. Electron microscopy of sperm revealed hyperplasia of the fibrous sheath and axonemal disruption. This is the first report of an association of different anomalies in cilia and flagella leading to clinical manifestation of the immotile cilia syndrome. These findings emphasize the need for ultrastructural examination of respiratory cilia in men suffering from fibrous sheath alterations of sperm which so far have not been described in patients with the classical form of immotile cilia syndrome.
对两名疑似患有纤毛运动障碍的患者进行了调查。他们因原发性不孕和慢性呼吸道疾病前来就诊。肺功能研究显示其中一名患者存在阻塞性改变。两人的精子均无活动能力,尾部短、粗且僵硬。鼻腔活检的超微结构研究显示纤毛异常,几乎完全缺乏内动力臂(病例1每个轴丝的内动力臂平均数为0.67±1.21,病例2为1.49±1.17,而正常值为5.3±0.13)。其他异常包括纤毛缺乏平行排列以及微管双联体向中心移位。精子的电子显微镜检查显示纤维鞘增生和轴丝破坏。这是首次报道纤毛和鞭毛的不同异常与不动纤毛综合征临床表现相关的病例。这些发现强调了对精子纤维鞘改变的男性患者进行呼吸道纤毛超微结构检查的必要性,而在经典型不动纤毛综合征患者中迄今尚未描述过这种情况。