Ty -jour t1-卡尔塔格纳综合症JF中的支气管炎 - 欧洲呼吸杂志JO -EUR RESSIR J SP -1332 LP -1332 LP -1339 VL -14 IS -6 AU -HOMA -HOMMA，SAU -KAWABATA，MAU -KAWABATA，MAU -KISHI -KISHI -KISHI，KAU -TSUBOI -TSUBOI，KAU -TSUBOI，TSUBOI，KAU -TSUBOI，e au -narui，k au -nakatani，t au -saiki，s au -nakata，k y1-1999/12/01 ur -http：//www.qdcxjkg.com/content.com/content/14/6/6/13322.Abstract N2222.Abstract N2 N2- 先前尚未报道Kartagener's综合征（KS）患者的弥漫性支气管炎的关联。这项研究的目的是介绍KS患者细支气管炎的形态特征。评估了八名患者（男性，四名女性；平均年龄37.9岁+/- 18.7岁），在临床上被诊断为具有经典的慢性胰腺炎，支气管张和SITUS的经典三合会的KS。常规的胸部射线照相术显示所有患者的支气管扩张和右心脏。胸部计算机断层扫描（CT）显示，在六名患者中有六个肺部，整个肺部的弥漫性中心小结节直径高达2 mm。肺功能测试显示所有患者的阻塞性损伤明显（强迫呼气量为一秒57.0 +/- 11.3％，残留体积/总肺部容量45。+/ - 12.7％，最大培训时间为0.92 +/- 0.92 +/- 0.72 l x S（ -1），强制生命能力74.1 +/- 12.2％（全平均+/- SD）。对支气管的纤毛运动的检查显示，在检查的所有五名患者中，都没有免疫力。超微结构在​​所有患者中均显示出睫状动力蛋白的缺陷。 Histopathological examination of lung specimens obtained at autopsy or by video-assisted thoracoscopic surgery showed obliterative thickening of the walls of the membranous bronchioli with infiltration of lymphocytes, plasma cells and neutrophils, but most of the distal respiratory bronchioli were spared and alveolar spaces were overinflated. Pathologically, the diffuse centrilobular small nodules on the chest CT mainly corresponded to membranous bronchiolitis. This is the first report demonstrating that the association of diffuse bronchiolitis might be one of the characteristic features of the lung in Kartagener's syndrome. ER -