Williams-Campbell syndrome complicated with pulmonary hypertension and Type 2 respiratory failure: An adult case report.
Journal: The Clinical Respiratory Journal
Published:
Abstract
Williams-Campbell syndrome, is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi, leading to distal airway collapse and bronchiectasis, which typically affects the fourth- to sixth-order bronchi. This article reported a 31-year-old female patient who was diagnosed with Williams-Campbell syndrome with pulmonary hypertension and Type 2 respiratory failure due to extensive cystic bronchiectasis. CT of the thorax showed the affected bronchi had characteristic ballooning on inspiration and collapse on expiration.
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