Carson J L, Collier A M
Department of Pediatrics, University of North Carolina, Chapel Hill.
Adv Pediatr. 1988;35:139-65.
Considerable progress has been made in achieving a perspective of the pathophysiology of ciliary defects in human disease in the interval between Siewert's and Kartagener's early descriptions of KS. Not only have we achieved a better understanding of some of the mechanisms involved in the pathogenesis of congenital ciliary syndromes, but also we have come to appreciate a new spectrum of ciliary defects, those of acquired etiology. The advent of modern electron microscopy has been a significant element to this progress and its importance is reflected by the numerous studies of ciliary defects that have come in the wake of the pioneering ultrastructural investigations of Afzelius and colleagues. However, each discovery appears to generate additional questions. This is indicative of the importance of the subject to both basic and clinical science and of the vitality of the investigators in this field as a comprehensive understanding of ciliary diseases is sought.
在西韦特（Siewert）和卡塔格纳（Kartagener）早期对卡塔格内综合征（KS）进行描述之后的这段时间里，我们在了解人类疾病中纤毛缺陷的病理生理学方面取得了相当大的进展。我们不仅对先天性纤毛综合征发病机制中涉及的一些机制有了更好的理解，而且还认识到了一系列新的纤毛缺陷，即后天性病因导致的缺陷。现代电子显微镜的出现是这一进展的重要因素，阿费利乌斯（Afzelius）及其同事开创性的超微结构研究之后，大量关于纤毛缺陷的研究反映了其重要性。然而，每一项发现似乎都会引发更多问题。这表明该主题对基础科学和临床科学都很重要，也表明该领域研究人员的活力，因为人们正在寻求对纤毛疾病的全面理解。
