title: Respiratory failure of Williams-Campbell syndrome is effectively treated by noninvasive positive pressure ventilation.
abstract: 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.
authors.affiliation: Division of Respiratory Medicine, Department of Internal Medicine, Shiga University of Medical Science, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Wada
authors.forename: Ruriko
authors.initials: R
authors.lastname: Seto
authors.forename: Hideto
authors.initials: H
authors.lastname: Yamada
authors.forename: Taishi
authors.initials: T
authors.lastname: Nagao
authors.forename: Takashi
authors.initials: T
authors.lastname: Hajiro
authors.forename: Yasutaka
authors.initials: Y
authors.lastname: Nakano
mesh_terms: D000328:Adult; D055091:Bronchomalacia / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D008297:Male; D011175:Positive-Pressure Respiration / Q000379:methods; D012131:Respiratory Insufficiency / Q000175:diagnosis* / Q000628:therapy*; D013577:Syndrome; D016896:Treatment Outcome