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Tracheal Ciliary Ultrastructure of Kartagener's Syndrome
卡達吉那症候群之氣管纖毛超微構造
摘要
本研究以兩位正常人與兩位卡達吉那症候群症群患者為研究對象。利用纖維支氣管鏡檢取氣管下端接近嵴部（carina）的組織，以掃描電子顯微鏡與穿透電子顯微鏡檢視纖毛的超微構造；更進一步，我們比較正常人與卡遠吉那症候群患者的氣管纖毛，並比較異常的超微構造的嚴重度與臨床症狀及肺功能異常的程度。結果顯示，卡遠吉那症候群患者的纖毛型態以掃描電子顯微鏡檢視，與正常型態相似，只是纖毛的方向較不一致。另外在病例2中則發現纖毛上覆有一層黏液。卡達吉那症候群患者的纖毛型態以穿透電子顯微鏡檢視，發現超微構造的異常如下：病例1，動力蛋白臂缺失：病例2，動力蛋白臂缺失、微細雙小管移位（transposition）和放射輪輻異常（defective radial spokes）。正常人為一對單獨小管，在病例2中則為三個小管或兩對單獨小管。同時，正常人橫切面織毛的外鞘呈平滑的圓形或六角形，但在病例1與2中則呈波狀的類圓形。病例2的纖毛顯微構造異常較病例1為多，其臨床症狀也較病例1為嚴重，同時肺功能研究亦顯示病例2較病例1患有較嚴重的阻塞性通氣不良。文獻尚無報告纖毛顯微構造異常的多寡與臨床病症的嚴重程度關係如何？根據本研究可推斷，若發現較多顯微構造的異常，可能引起較嚴重的纖毛運動不正常，因而導致更嚴重的臨床病症與肺功能異常。病例2的精液檢視發現精子運動不良，此結果可導致不孕症；但病例，女性已結婚生子。此結果與過去文獻報告相同，即男性易導致不孕症，但女性則否。
並列摘要
To examine the ciliary ultrastructure of Kartagener's syndrome, two normal control and two cases with Kartagener's syndrome were studied. The tracheal mucosa was biopsied near the carina. Ciliary ultrastructure and gross morphology were examined by transmission and scanning electron microscopy respectively. Comparing the normal with Kartagener's syndrome, we found that there were absence of dynein arm (in cases 1, 2), defective radial spokes (in case 2), transposition of microtubules (in case 2), two pairs or three central microtubules (in case 2) and wave form in shape of out layer sheath (in case 1, 2). We postulated that cilia containing more defects caused more impairment of motility. According to our observation, case 2 with more defects had more severe clinical symptoms and impaired pulmonary function than case 1 who had only defective dynein arm in cilia. There was no significant abnormality in appearance of cilia on scanning electron microscopy but multidirectional oriention of cilia or a layer of mucus on mucosal surface was noted in patients with Kartagener's syndrome.