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

S. Rodrigo Burguete, Stephanie M. Levine, Marcos Restrepo, Luis F Angel, Deborah J. Levine, Jacqueline J. Coalson, Jay I. Peters

Resultado de la investigación: Articlerevisión exhaustiva

6 Citas (Scopus)


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.

Idioma originalEnglish (US)
Páginas (desde-hasta)1505-1508
Número de páginas4
PublicaciónRespiratory care
EstadoPublished - sept 2012

ASJC Scopus subject areas

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


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