TY - JOUR T1 - Lung Transplantation for Williams-Campbell Syndrome With a Probable Familial Association JF - Respiratory Care SP - 1505 LP - 1508 DO - 10.4187/respcare.01484 VL - 57 IS - 9 AU - S Rodrigo Burguete AU - Stephanie M Levine AU - Marcos I Restrepo AU - Luis F Angel AU - Deborah J Levine AU - Jacqueline J Coalson AU - Jay I Peters Y1 - 2012/09/01 UR - http://rc.rcjournal.com/content/57/9/1505.abstract N2 - 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. ER -