Kawamura T, Mochizuki Y, Nakahara Y, Nakahara Y, Kawanami R, Hirata K, Amitani R
Department of Internal Medicine, Himeji National Hospital.
Nihon Kyobu Shikkan Gakkai Zasshi. 1992 Sep;30(9):1719-23.
Williams-Campbell syndrome is a unique type of bronchiectasis which shows ballooning during expiration and collapse during inspiration. Its etiology is thought to be a congenital decrease in bronchial cartilage. We experienced two adult cases of Williams-Campbell syndrome. Since the mucociliary transport system was normal in both cases and neither case had chronic sinusitis, these cases differed from sino-bronchial syndrome. Cases of Williams-Campbell syndrome reported in Japan show characteristic bronchography, but a decrease in bronchial cartilage may or may not be present and its congenital nature is very difficult to demonstrate. We therefore advocate referring to such cases of bronchiectasis with these bronchographic characteristics Williams-Campbell-type bronchiectasis, even if a decrease in bronchial cartilage is not observed.
威廉姆斯-坎贝尔综合征是一种独特类型的支气管扩张症，其在呼气时呈气囊样扩张，吸气时则塌陷。其病因被认为是支气管软骨先天性减少。我们诊治了两例成年威廉姆斯-坎贝尔综合征患者。由于这两例患者的黏液纤毛转运系统均正常，且均无慢性鼻窦炎，因此这些病例与鼻窦支气管综合征不同。日本报道的威廉姆斯-坎贝尔综合征病例显示出特征性的支气管造影表现，但支气管软骨减少可能存在也可能不存在，且很难证实其先天性本质。因此，我们主张将具有这些支气管造影特征的支气管扩张症病例称为威廉姆斯-坎贝尔型支气管扩张症，即使未观察到支气管软骨减少。
