Function and structure of cilia in the Fallopian tube of an infertile woman with Kartagener's syndrome
Function and structure of cilia in the Fallopian tube of an infertile woman with Kartagener's syndrome
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DOI：
10.1093/humrep/12.1.55
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发表时间：
1997-01-01
影响因子：
6.1
通讯作者：
Soules, MR
中科院分区：
文献类型：
--
作者：
Halbert, SA;Patton, DL;Soules, MR
在Kartagener综合征（KS）中，纤毛轴丝的原发性缺陷引起纤毛运动障碍，因为纤毛运动是正常卵子运输的重要因素，纤毛运动障碍可能导致不孕，另一方面，某些纤毛活动的存在，尽管异常，可能解释某些KS妇女的生育能力，在本病例研究中，1例KS不孕症患者在所有常规不孕症检查中均正常。在体外受精周期中，在腹腔镜下进行输卵管粘膜活检以回收卵子，通过激光光散射光谱法检测所有输卵管标本中的纤毛活动;但大部分区域无活动，活动区搏动频率为5 ~ 10 Hz，约为正常值的30%。电镜下输卵管和鼻粘膜均出现类似的形态学缺损。每个细胞的纤毛数与正常的20%相似。纤毛的主要超微结构异常是中央微管的缺失，该患者不孕的唯一可证实的解释是与KS相关的原发性纤毛运动障碍。
In Kartagener's syndrome (KS), primary defects of the ciliary axoneme cause dyskinetic ciliary motion, Because ciliary motion is an important factor in normal ovum transport, ciliary dyskinesia may cause infertility, On the other hand, the existence of some ciliary activity, albeit abnormal, may account for fertility in some women with KS, In this case study, an infertile woman diagnosed with KS had normal results in all usual infertility tests, Biopsies of tubal mucosa were obtained at laparoscopy for ovum recovery during an in-vitro fertilization cycle, Ciliary activity, measured by laser light-scattering spectroscopy, was detected in all tubal specimens; however the majority of regions sampled showed no activity, In active regions, beat frequency ranged from 5 to 10 Hz, similar to 30% of normal, Electron microscopy showed similar morphological defects in both tubal and nasal mucosa. The number of cilia per cell was similar to 20% of normal. The major ultrastructural abnormality of cilia was an absence of the central microtubules, The only demonstrable explanation for this patient's infertility was primary ciliary dyskinesia associated with KS.