Wayne K S, Taussig L M
Am Rev Respir Dis. 1976 Jul;114(1):15-22. doi: 10.1164/arrd.1976.114.1.15.
Two siblings in whom respiratory symptoms developed immediately after birth subsequently were found to have bronchiectasis with strikingly similar distribution of lesions (mainly lower lobes). Inspiratory and expiratory bronchograms performed on one of the siblings demonstrated marked ballooning and collapse of proximal bronchi during tidal breathing. The clinical courses and roentgenographic findings, in the absence of other underlying abnormalities, suggest that the basic disease process was the absence of bronchial cartilage (Williams-Campbell syndrome). This would be the first reported familial occurrence of this syndrome. The familial pattern and the neonatal onset of symptoms support the theory of a congenital basis for this variety of bronchiectasis.
两名出生后立即出现呼吸道症状的兄弟姐妹，随后被发现患有支气管扩张，病变分布极为相似（主要在肺下叶）。对其中一名兄弟姐妹进行的吸气和呼气支气管造影显示，在潮式呼吸期间近端支气管明显扩张和塌陷。在没有其他潜在异常的情况下，临床病程和X线表现提示基本疾病过程是支气管软骨缺如（威廉姆斯-坎贝尔综合征）。这将是该综合征首次报道的家族性病例。家族模式和症状的新生儿期发作支持了这种类型支气管扩张的先天性基础理论。
