Kartagener's Syndrome: A Case Report and Review of the Literature

Kartagener氏症候群－病例報告及文獻回顧

吳國安(Kuo-An Wu) ； 廖忠義(Chung-Yi Liao) ； 彭萬誠(Wann-Cherng Perng) ； 吳清平(Chin-Pyng Wu)

《胸腔醫學》 21卷3期 (2006/06) Pp. 255-260

https://doi.org/10.29806/TM.200606.0005

摘要

Kartagener氏症候群為一罕見之先天性疾病。此症後群包括三種病症：l)內臟反位，2)支氣管擴張症，3)鼻竇炎。這種遺傳性疾病包含在纖毛運動不良症候群或原發性纖毛運動困難的疾病群之中。此症是因為纖毛不活動或不正常的拍動而導致纖毛無法有效地清除呼吸道的分泌物。Kartagener氏症候群會引起一些臨床的問題，最主要的併發症是肺部感染。本篇報告一位58歲男性因反覆性的咳嗽有痰，合併有膿樣的鼻腔分泌物而到本院求診。經過系列檢查確定診斷為Kartagener氏症候群。病人接受抗生素及支氣管擴張劑治療後臨床症狀明顯改善。在此並回顧歷年來對此種病歷相關的文獻報告。

關鍵字

Kartagener氏症候群 ； 內臟反位 ； 支氣管擴張症 ； 鼻竇炎 ； 纖毛運動不良症候群 ； 原發性纖毛運動困難

並列摘要

Kartagener's syndrome (KS) is a rare congenital malformation, which consists of a classic triad of situs inversus, bronchiectasis, and sinusitis [1]. It is a genetic disorder that is included in either the group of diseases defined as immotile cilia syndrome, or diseases due to primary cilia dyskinesia [2]. These disorders are characterized by the immotility or abnormal beating of the cilia which leads to insufficient mucociliary clearance. KS can cause substantial clinical problems, mainly because of the complications of pulmonary infections.
The patient we encountered was a 58-year-old man with complaints of recurrent productive cough and purulent nasal discharge for about half a year. After a work-up, KS was eventually diagnosed. The patient's symptoms were relieved after treatment with antibiotics and a bronchodilator. We also review the associated literature.

並列關鍵字

Kartagener's syndrome ； situs inversus ； bronchiectasis ； sinusitis ； immotile cilia syndrome ； dyskinetic cilia syndrome ； primary ciliary dyskinesia