Armengot M, Juan G, Carda C, Montalt J, Basterra J
ENT Department, Valencia University General Hospital, Spain.
Rhinology. 1996 Mar;34(1):35-7.
Three males--aged 32, 35, and 27 years--presented Young's syndrome: a combination of obstructive azoospermia and chronic sinopulmonary infection. The evaluation of nasal mucociliary transport using an isotopic technique revealed mucociliary stasis in one case and decreased clearance in the others (< 2 mm/min). Ciliary ultrastructure was normal in two patients, while the other showed mucous hyperplasia and low ciliary density which made correct ciliary evaluation not possible. The clinical development of this syndrome is chronic, although less severe than in the other two syndromes that exhibit primary failure of mucociliary transport: cystic fibrosis and primary ciliary dyskinesia. Young's syndrome should be considered in the differential diagnosis of patients suffering from chronic rhinosinusitis, particularly with cystic fibrosis and primary ciliary dyskinesia syndrome.
三名男性——年龄分别为32岁、35岁和27岁——患有杨氏综合征:一种梗阻性无精子症与慢性鼻窦肺部感染的组合。采用同位素技术对鼻黏膜纤毛运输进行评估,结果显示1例存在黏膜纤毛淤滞,其他病例清除率降低(<2毫米/分钟)。两名患者的纤毛超微结构正常,而另一名患者表现为黏液增生和纤毛密度降低,无法进行准确的纤毛评估。该综合征的临床发展呈慢性,尽管不如另外两种表现为原发性黏膜纤毛运输功能障碍的综合征——囊性纤维化和原发性纤毛运动障碍——严重。在慢性鼻窦炎患者的鉴别诊断中,尤其是与囊性纤维化和原发性纤毛运动障碍综合征鉴别时,应考虑杨氏综合征。