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, Jay I. Peters

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4 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)1505-1508
Number of pages4
JournalRespiratory care
Volume57
Issue number9
DOIs
StatePublished - Sep 1 2012

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Keywords

  • Bronchial cartilage deficiency
  • Bronchiectasis
  • Lung transplantation
  • Williams-Campbell syndrome

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

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