Amano S, Yoshida Y, Shimizu H, Takeda T, Urabe N, Mizoo A, Kimura H, Kuriyama T
Department of Medicine, Numazu-city Hospital, Shizuoka, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1997 Nov;35(11):1265-70.
Williams-Campbell syndrome is a unique form of bronchiectasis caused by a congenital defect in bronchial cartilage, and is rare in Japan. A 34-year-old man was admitted to our hospital with a fever, and a productive cough. Arterial blood gas analysis revealed severe type II-respiratory failure. Many thin-walled cystic shadows (5-60 mm in diameter) were present in the entire lung field. Pulmonary function tests revealed obstructive impairment. Bronchograms demonstrated cystic bronchiectasis, with ballooning on inspiration and collapse on expiration, characteristic of Williams-Campbell syndrome. Despite severe hypoxia, he did not suffer from dyspnea. We examined ventilatory response to hypercapnea (HCVR) and hypoxia (HVR), and both HCVR and HVR were abnormal. In addition, the mean pulmonary artery pressure was 26 mmHg, indicating pulmonary hypertension.
威廉姆斯 - 坎贝尔综合征是一种由支气管软骨先天性缺陷引起的独特形式的支气管扩张症，在日本较为罕见。一名34岁男性因发热和咳痰入院。动脉血气分析显示严重的II型呼吸衰竭。全肺野可见许多薄壁囊性阴影（直径5 - 60毫米）。肺功能测试显示阻塞性损害。支气管造影显示囊性支气管扩张，吸气时扩张，呼气时塌陷，这是威廉姆斯 - 坎贝尔综合征的特征。尽管存在严重缺氧，但他并未出现呼吸困难。我们检查了对高碳酸血症（HCVR）和低氧血症（HVR）的通气反应，HCVR和HVR均异常。此外，平均肺动脉压为26 mmHg，提示肺动脉高压。
