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

doi:10.4187/respcare.01484

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

Research output: Contribution to journal › Article

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 language	English (US)
Pages (from-to)	1505-1508
Number of pages	4
Journal	Respiratory care
Volume	57
Issue number	9
DOIs	https://doi.org/10.4187/respcare.01484
State	Published - 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

Cite this

Rodrigo Burguete, S., Levine, S. M., Restrepo, M. I., Angel, L. F., Levine, D. J., Coalson, J. J., & Peters, J. I. (2012). Lung transplantation for Williams-Campbell syndrome with a probable familial association. Respiratory care, 57(9), 1505-1508. https://doi.org/10.4187/respcare.01484

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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.",

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AU - Coalson, Jacqueline J.

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10.4187/respcare.01484