1. Lung Transplantation for Williams-Campbell Syndrome With a Probable Familial Association
- Author
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Jay I. Peters, Stephanie M Levine, Marcos I. Restrepo, Jacqueline J. Coalson, Luis F. Angel, S Rodrigo Burguete, and Deborah Levine
- Subjects
Adult ,Male ,Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Pediatrics ,medicine.medical_treatment ,Disease ,Critical Care and Intensive Care Medicine ,Article ,medicine ,Humans ,Lung transplantation ,Williams–Campbell syndrome ,Bronchiectasis ,Lung ,business.industry ,Syndrome ,General Medicine ,respiratory system ,medicine.disease ,respiratory tract diseases ,Surgery ,Transplantation ,Cartilage ,medicine.anatomical_structure ,Respiratory failure ,Respiratory Insufficiency ,business ,Airway ,Cartilage Diseases ,Lung Transplantation - 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.
- Published
- 2012
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