重複上大静脈に下大静脈欠損を伴うKartagener症候群の1例

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  • A case of Kartagener's syndrome with bilateral superior vena cava and absent inferior vena cava.

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A 39-year-old man with a history of repeated attacks of acute bronchitis and pneumonia since childhood was admitted complaining of cough, sputum, and a high fever. A diagnosis of Kartagener's syndrome was made because of the presence of mirror-image dextrocardia with complete situs inversus, bronchiectasis in right B6 and B10, and chronic sinusitis. Examination of the ciliary ultrastructure of the bronchial epithelium showed defective inner dynein arms and impaired nasal clearance was suggested by the saccharin particle method. Contrast venography of great veins revealed a McCotter type II bilateral superior vena cava and an absent inferior vena cava with (hemi-) azygos continuation. A search to find a similar case in the literature failed. Abnormal ciliary movement from the embryonic period was implicated as a causative factor in Kartagener's triad and the morphological abnormalities of the great veins.

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