title: Lung transplantation for Williams-Campbell syndrome with a probable familial association.
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.
authors.affiliation: Division of Pulmonary Diseases and Critical Care Medicine, Department of Medicine, University of Texas Health Science Center at San Antonio, Texas 78229-3900, USA. burguete@uthscsa.edu
authors.forename: S Rodrigo
authors.initials: SR
authors.lastname: Burguete
authors.forename: Stephanie M
authors.initials: SM
authors.lastname: Levine
authors.forename: Marcos I
authors.initials: MI
authors.lastname: Restrepo
authors.forename: Luis F
authors.initials: LF
authors.lastname: Angel
authors.forename: Deborah J
authors.initials: DJ
authors.lastname: Levine
authors.forename: Jacqueline J
authors.initials: JJ
authors.lastname: Coalson
authors.forename: Jay I
authors.initials: JI
authors.lastname: Peters
mesh_terms: D000328:Adult; D001987:Bronchiectasis / Q000601:surgery*; D002356:Cartilage / Q000002:abnormalities*; D002357:Cartilage Diseases / Q000151:congenital / Q000235:genetics / Q000601:surgery*; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D012131:Respiratory Insufficiency / Q000601:surgery*; D013577:Syndrome