Edwards D F, Patton C S, Kennedy J R
Department of Pathobiology, College of Veterinary Medicine, University of Tennessee, Knoxville.
Probl Vet Med. 1992 Jun;4(2):291-319.
A disorder caused by congenital ciliary dysfunction occurs in dogs. Most of the clinical signs are directly or indirectly attributable to immotile or dyskinetic cilia and spermflagella. Due to severely impaired mucociliary clearance, a continuous mucoid nasal discharge and intermittent sneezing and coughing are typically observed during the neonatal period. Recurrent bacterial rhinosinusitis and bronchopneumonia usually start within a few weeks of birth. Hypoplastic nasal sinuses and atresia of the frontal sinuses are variable features of the disease that may be caused by neonatal colonization of these structures by specific bacteria. Bronchiectasis is an acquired lesion resulting from chronic inflammation and obstruction of airways. A secretory otitis media is caused by dysfunction of the cilia in the middle ear, and is manifested in some dogs by sclerotic tympanic bullae. Male infertility is caused by live, but immotile to hypomotile spermatozoa; however, unexplained oligospermia and azoospermia have been reported. Hydrocephalus and situs inversus are common but variable features of the disease; the genesis of these lesions has not yet been determined. The probable mode of inheritance is autosomal recessive, but dominant mutations cannot be excluded. The diagnosis can be confirmed by demonstrating the absence or near absence of nasal or tracheal mucociliary clearance and the presence of a specific ultrastructural lesion in a large percentage of cilia from multiple sites (airways, middle ear, or oviduct). The ultrastructure of sperm flagella should mirror that of the cilia. Not all dogs have ultrastructural ciliary lesions, and in these cases, results of in vitro analysis of ciliary activity may be highly suggestive, if not diagnostic. In dogs without mucociliary clearance in which structural and functional analysis of cilia are not diagnostic, confirmation of congenital ciliary dysfunction can be established only by ruling out other diseases with similar signs (e.g., congenital immunodeficiency syndromes). The clinical course in an longevity of affected dogs are highly variable. Appropriate antibiotic treatment and pulmonary physical therapy may result in prolonged survival, although cor pulmonale and reactive systemic amyloidosis are potential sequelae of chronic hypoxia and chronic bacterial infection of the airways, respectively.
犬类会发生由先天性纤毛功能障碍引起的一种病症。大多数临床症状直接或间接归因于纤毛和精子鞭毛运动不能或运动障碍。由于黏液纤毛清除功能严重受损，在新生期通常会观察到持续的黏液性鼻分泌物以及间歇性打喷嚏和咳嗽。复发性细菌性鼻窦炎和支气管肺炎通常在出生后几周内开始出现。鼻窦发育不全和额窦闭锁是该疾病的可变特征，可能由特定细菌在这些结构中的新生期定植引起。支气管扩张是由慢性炎症和气道阻塞导致的后天性病变。分泌性中耳炎由中耳纤毛功能障碍引起，在一些犬中表现为鼓室硬化。雄性不育是由存活但运动不能至运动减弱的精子导致的；然而，也有不明原因的少精子症和无精子症的报道。脑积水和内脏反位是该疾病常见但可变的特征；这些病变的发生机制尚未确定。可能的遗传方式为常染色体隐性遗传，但不能排除显性突变。通过证明鼻腔或气管黏液纤毛清除功能缺失或几乎缺失，以及在多个部位（气道、中耳或输卵管）的大部分纤毛中存在特定的超微结构病变，可确诊该病。精子鞭毛的超微结构应与纤毛的超微结构一致。并非所有犬都有超微结构的纤毛病变，在这些情况下，即使纤毛活动的体外分析结果不能确诊，也可能具有高度提示性。对于没有黏液纤毛清除功能且纤毛结构和功能分析不能确诊的犬，只有通过排除具有类似症状的其他疾病（如先天性免疫缺陷综合征），才能确诊先天性纤毛功能障碍。患病犬的临床病程差异很大。适当的抗生素治疗和肺部物理治疗可能会延长生存期，尽管肺心病和反应性系统性淀粉样变分别是慢性缺氧和气道慢性细菌感染的潜在后遗症。
