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- Title
Lung Transplantation for Williams-Campbell Syndrome With a Probable Familial Association.
- Authors
Burguete, S. Rodrigo; Levine, Stephaniem.; Restrepo, Marcosi.; Angel, Luis F.; Levine, Deborah J.; Coalson, Jacqueline J.; Peters, Jay I.
- 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.
- Subjects
ADRENOCORTICAL hormones; HORMONE therapy; ANTIBIOTICS; BRONCHODILATOR agents; LUNG transplantation; BRONCHIECTASIS; OXYGEN therapy; RESPIRATORY insufficiency; GENETICS; DIAGNOSIS; THERAPEUTICS
- Publication
Respiratory Care, 2012, Vol 57, Issue 9, p1505
- ISSN
0020-1324
- Publication type
Case Study
- DOI
10.4187/respcare.01484