Abstract
Williams-Campbell syndrome is a rare disorder characterized by deficiency of subsegmental bronchial cartilage and development of airway collapse and bronchiectasis that may subsequently progress to respiratory failure and death. There are only 2 published reports suggesting a familial association, and only one report of lung transplantation being used as a therapeutic modality. Due to postoperative airway complications, transplantation has not been recommended for this disease. We report the first lung transplant with prolonged survival, approaching 10 years, in a patient with Williams-Campbell syndrome, and provide further evidence to support a familial association.
Original language | English (US) |
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Pages (from-to) | 1505-1508 |
Number of pages | 4 |
Journal | Respiratory care |
Volume | 57 |
Issue number | 9 |
DOIs | |
State | Published - Sep 2012 |
Keywords
- Bronchial cartilage deficiency
- Bronchiectasis
- Lung transplantation
- Williams-Campbell syndrome
ASJC Scopus subject areas
- Pulmonary and Respiratory Medicine
- Critical Care and Intensive Care Medicine