Wada Hiroshi, Seto Ruriko, Yamada Hideto, Nagao Taishi, Hajiro Takashi, Nakano Yasutaka
Division of Respiratory Medicine, Department of Internal Medicine, Shiga University of Medical Science, Japan.
Intern Med. 2011;50(16):1729-32. doi: 10.2169/internalmedicine.50.4971. Epub 2011 Aug 15.
Williams-Campbell syndrome is a rare disease, characterized by a congenital deficiency of cartilage in the fourth to sixth order bronchi, leading to chronic respiratory failure with recurrent pulmonary infections. An effective and practical treatment has not yet been established. A 31-year-old man who was diagnosed as Williams-Campbell syndrome by inspiratory and expiratory computed tomography findings developed recurrent pulmonary infections and showed progressive deterioration of dyspnea. Domiciliary NPPV was administered, followed by a dramatic improvement of respiratory failure and a decrease in the episodes of pulmonary infections. NPPV may have an advantage in adults with Williams-Campbell syndrome who have severe respiratory failure and recurrent pulmonary infections.
威廉姆斯-坎贝尔综合征是一种罕见疾病，其特征为第四至六级支气管先天性软骨缺乏，导致慢性呼吸衰竭并伴有反复肺部感染。目前尚未确立有效且实用的治疗方法。一名31岁男性通过吸气和呼气计算机断层扫描结果被诊断为威廉姆斯-坎贝尔综合征，他反复出现肺部感染，且呼吸困难呈进行性加重。给予家庭无创正压通气（NPPV）治疗后，呼吸衰竭显著改善，肺部感染发作次数减少。对于患有严重呼吸衰竭和反复肺部感染的威廉姆斯-坎贝尔综合征成年患者，NPPV可能具有优势。
