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[一例伴有双侧上腔静脉及下腔静脉缺如的卡塔格内综合征]

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

作者信息

Tabeta H, Hiroshima K

机构信息

Division of Internal Medicine, Funabashi Municipal Medical Center, Japan.

出版信息

Nihon Kyobu Shikkan Gakkai Zasshi. 1991 Apr;29(4):507-11.

PMID:1865604
Abstract

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.

摘要

一名39岁男性,自幼有反复急性支气管炎和肺炎发作史,因咳嗽、咳痰和高热入院。由于存在镜像右位心伴完全性内脏转位、右肺B6和B10支气管扩张以及慢性鼻窦炎,诊断为卡塔格内综合征。支气管上皮纤毛超微结构检查显示内动力蛋白臂缺陷,糖精颗粒法提示鼻腔清除功能受损。大静脉造影显示双侧上腔静脉为麦科特II型,下腔静脉缺如,由(半)奇静脉延续。检索文献未发现类似病例。胚胎期纤毛运动异常被认为是卡塔格内三联征和大静脉形态异常的致病因素。

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