Miranda Ileana C, Granick Jennifer L, Armién Anibal G
1 Department of Epidemiology and Public Health, Veterinary Institute, Federal Rural University of Rio de Janeiro, Seropedica, Rio de Janeiro, Brazil.
2 Ultrastructural Pathology Unit, Veterinary Diagnostic Laboratory, University of Minnesota, Saint Paul, MN, USA.
Vet Pathol. 2017 Sep;54(5):802-812. doi: 10.1177/0300985817705170. Epub 2017 May 11.
Mucociliary clearance is a main defense mechanism of the respiratory tract, which can be inherently impaired in primary ciliary dyskinesia (PCD) or reversibly altered in secondary ciliary dyskinesia (SCD). Limited diagnostic test availability likely leads to misdiagnosis or underdiagnosis of PCD in animals. This study evaluated the light and transmission electron microscopy (TEM) changes in the respiratory mucosa of 15 dogs with chronic respiratory disease suspected of PCD. Necropsy was performed in 1 case and 2 dogs were used as negative controls. PCD was confirmed in 1 dog, which presented with chronic otitis, bronchopneumonia, hydrocephalus and ultrastructural abnormalities in 84% of the assessed cilia, including absence of dynein arms and microtubular changes. The 14 other cases showed only nonspecific alterations, such as ciliary disorientation, compound cilia, ciliary edema, and axoneme bubbles in a minority of the evaluated cilia and were classified as SCD. Ciliary ultrastructural analysis can confirm a diagnosis of PCD if specific abnormalities exist. TEM remains an important investigational tool in veterinary medicine, as no other specific test for PCD in dogs has been standardized yet.
黏液纤毛清除是呼吸道的主要防御机制,在原发性纤毛运动障碍(PCD)中可能存在先天性损害,而在继发性纤毛运动障碍(SCD)中则可能发生可逆性改变。有限的诊断测试可用性可能导致动物PCD的误诊或漏诊。本研究评估了15只疑似患有PCD的慢性呼吸道疾病犬的呼吸道黏膜的光学显微镜和透射电子显微镜(TEM)变化。对1只犬进行了尸检,并将2只犬用作阴性对照。1只犬被确诊为PCD,其患有慢性中耳炎、支气管肺炎、脑积水,并且在84%的评估纤毛中存在超微结构异常,包括动力蛋白臂缺失和微管变化。其他14例仅表现出非特异性改变,如少数评估纤毛中的纤毛排列紊乱、复合纤毛、纤毛水肿和轴丝泡,被归类为SCD。如果存在特定异常,纤毛超微结构分析可以确诊PCD。TEM仍然是兽医学中的一项重要研究工具,因为目前尚无针对犬PCD的其他标准化特异性检测方法。