• Resp Care · Sep 2012

    Case Reports

    Lung transplantation for Williams-Campbell syndrome with a probable familial association.

    • S Rodrigo Burguete, Stephanie M Levine, Marcos I Restrepo, Luis F Angel, Deborah J Levine, Jacqueline J Coalson, and Jay I Peters.
    • 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
    • Resp Care. 2012 Sep 1;57(9):1505-8.

    AbstractWilliams-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.Copyright 2012 Daedalus Enterprises

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