Rayner C F, Rutman A, Dewar A, Cole P J, Wilson R
Department of Thoracic Medicine, Royal Brompton National Heart and Lung Institute, London, United Kingdom.
Am J Respir Crit Care Med. 1995 Mar;151(3 Pt 1):800-4. doi: 10.1164/ajrccm.151.3.7881674.
Random ciliary orientation was recently described as a possible variant of primary ciliary dyskinesia (PCD). The cilia have normal ultrastructure and nearly normal ciliary beat frequency (CBF) but lack efficacy because the beat direction is disoriented. However, delayed mucociliary clearance (MCC), transitory changes in ultrastructure, and slowed CBF can all occur in the presence of inflammation. This study investigated groups of patients with upper respiratory tract inflammation caused by infection to assess whether ciliary disorientation was present and its relation to MCC. The study population consisted of 10 healthy nonatopic nonsmoking volunteers, 15 patients with idiopathic bronchiectasis and chronic mucopurulent sinusitis, 12 patients with cystic fibrosis, and two patients with the clinical features of PCD but normal CBF and ciliary ulstrastructure. Ciliary disorientation was significantly (p < 0.05) increased in the three patient groups compared with the volunteers, being greatest in the two patients with the clinical features of PCD and in bronchiectasis patients with P. aeruginosa, and was positively correlated (r = 0.9) with MCC but not with CBF. Treatment of one patient with antibiotics and topical corticosteroids for a prolonged period resulted in ciliary disorientation returning to normal. Ciliary disorientation may therefore occur secondary to inflammation caused by infection, and the study suggests that ciliary disorientation rather than ultrastructural abnormalities or slow CBF results in delayed MCC.
随机纤毛定向最近被描述为原发性纤毛运动障碍（PCD）的一种可能变体。纤毛具有正常的超微结构和接近正常的纤毛摆动频率（CBF），但由于摆动方向紊乱而缺乏效力。然而，在存在炎症的情况下，黏液纤毛清除（MCC）延迟、超微结构的短暂变化以及CBF减慢都可能发生。本研究调查了由感染引起上呼吸道炎症的患者组，以评估是否存在纤毛定向紊乱及其与MCC的关系。研究人群包括10名健康的非特应性非吸烟志愿者、15名患有特发性支气管扩张和慢性黏液脓性鼻窦炎的患者、12名患有囊性纤维化的患者以及两名具有PCD临床特征但CBF和纤毛超微结构正常的患者。与志愿者相比，三组患者的纤毛定向紊乱显著增加（p < 0.05），在两名具有PCD临床特征的患者和患有铜绿假单胞菌的支气管扩张患者中最为明显，并且与MCC呈正相关（r = 0.9），但与CBF无关。一名患者长期使用抗生素和局部皮质类固醇治疗后，纤毛定向紊乱恢复正常。因此，纤毛定向紊乱可能继发于感染引起的炎症，并且该研究表明纤毛定向紊乱而非超微结构异常或CBF减慢导致MCC延迟。
