Vichyanond P, Thakerngpol K, Susiva C, Chavalittamrong B
Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Asian Pac J Allergy Immunol. 1993 Dec;11(2):103-9.
Kartagener's syndrome is a well known classical triad of presentations consisting of bronchiectasis, sinusitis and situs inversus. It is now recognized that the syndrome is an extreme presentation of primary ciliary dyskinesia, a large group of conditions with ultrastructural ciliary defects, leading to poor ciliary motility in various organ systems. A case of Kartagener's syndrome is presented in an eight year old Thai boy in whom the ultrastructural ciliary defects have been examined and described in detail for the first time in Thailand. Incomplete lack of dynein arms was recognized. In addition, disorientation of ciliary axis was noticed. Due to severe bronchiectatic changes of the right lower lobe and right lingular lobe which did not improve despite adequate antibiotics, these lobes were surgically removed. The child has done well since, but still suffers occasional and recurrent bouts of sinusitis.
卡塔格内综合征是一种广为人知的典型三联征表现，包括支气管扩张、鼻窦炎和内脏转位。现在人们认识到，该综合征是原发性纤毛运动障碍的一种极端表现，原发性纤毛运动障碍是一大类具有超微结构纤毛缺陷的病症，会导致各个器官系统的纤毛运动不良。本文报告了一例8岁泰国男孩的卡塔格内综合征病例，首次在泰国对其超微结构纤毛缺陷进行了详细检查和描述。发现存在动力蛋白臂不完全缺失的情况。此外，还注意到纤毛轴方向紊乱。由于右下叶和右舌叶严重的支气管扩张性改变，尽管使用了足够的抗生素仍未改善，因此对这些肺叶进行了手术切除。此后该患儿情况良好，但仍偶尔复发鼻窦炎。
