Munro N C, Currie D C, Lindsay K S, Ryder T A, Rutman A, Dewar A, Greenstone M A, Hendry W F, Cole P J
Department of Thoracic Medicine, Royal Brompton National Heart and Lung Institute, London.
Thorax. 1994 Jul;49(7):684-7. doi: 10.1136/thx.49.7.684.
Primary ciliary dyskinesia is characterised by chronic rhinosinusitis, chronic bronchial sepsis (usually with bronchiectasis), dextrocardia in approximately 50% of cases, and male infertility. The latter, described in patients attending infertility clinics, results from immotile but viable spermatozoa. Experience in a respiratory clinic suggests that infertility in men is not invariable.
The seminal fluid of 12 men with primary ciliary dyskinesia, six with dextrocardia, who presented consecutively with upper and lower respiratory tract sepsis was examined. Nasal ciliary beating was dyskinetic or absent in all cases, and nasal ciliary ultrastructure was abnormal in those 11 patients examined.
Viable but immotile spermatozoa with abnormal tail ultrastructure were found in the ejaculate of only two patients. Two other patients had apparently fathered children; seminology in both these cases showed a normal spermatozoa count, one with normal spermatozoal motility and normal ultrastructure, the other with moderately reduced spermatozoal motility and abnormal ultrastructure (dynein arm deficiency on the peripheral microtubule doublets). A further two patients had normal spermatozoa counts, normal spermatozoa tail ultrastructure, and normal or only moderately reduced motility of spermatozoa. The spermatozoa of one patient were normally motile but there was severe oligozoospermia, and five patients were azoospermic.
Not all men with primary ciliary dyskinesia have immotile spermatozoa. Seminal analysis is recommended in men with primary ciliary dyskinesia so that accurate counselling about reproductive capability may be given.
原发性纤毛运动障碍的特征为慢性鼻窦炎、慢性支气管感染(通常伴有支气管扩张),约50%的病例有右位心,以及男性不育。后者在不育门诊患者中有所描述,是由精子不动但有活力导致的。呼吸科门诊的经验表明男性不育并非一成不变。
对12例原发性纤毛运动障碍患者的精液进行了检查,其中6例有右位心,这些患者均因上、下呼吸道感染而相继前来就诊。所有病例的鼻纤毛运动均异常或缺失,11例接受检查的患者鼻纤毛超微结构异常。
仅在2例患者的精液中发现有活力但不动的精子,其尾部超微结构异常。另外2例患者显然育有子女;这两例患者的精液分析显示精子计数正常,一例精子活力和超微结构正常,另一例精子活力中度降低且超微结构异常(外周微管双联体上的动力蛋白臂缺陷)。另有2例患者精子计数正常,精子尾部超微结构正常,精子活力正常或仅中度降低。1例患者的精子运动正常,但严重少精子症,5例患者无精子症。
并非所有原发性纤毛运动障碍男性患者都有精子不动的情况。建议对原发性纤毛运动障碍男性患者进行精液分析,以便能就生殖能力提供准确的咨询。