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 | |
| State | Published - Sep 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
Lung transplantation for Williams-Campbell syndrome with a probable familial association. / Rodrigo Burguete, S.; Levine, Stephanie M; Restrepo, Marcos; Angel, Luis F.; Levine, Deborah J; Coalson, Jacqueline J.; Peters, Jay I.
In: Respiratory Care, Vol. 57, No. 9, 09.2012, p. 1505-1508.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Lung transplantation for Williams-Campbell syndrome with a probable familial association
AU - Rodrigo Burguete, S.
AU - Levine, Stephanie M
AU - Restrepo, Marcos
AU - Angel, Luis F.
AU - Levine, Deborah J
AU - Coalson, Jacqueline J.
AU - Peters, Jay I
PY - 2012/9
Y1 - 2012/9
N2 - 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.
AB - 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.
KW - Bronchial cartilage deficiency
KW - Bronchiectasis
KW - Lung transplantation
KW - Williams-Campbell syndrome
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UR - http://www.scopus.com/inward/citedby.url?scp=84866127546&partnerID=8YFLogxK
U2 - 10.4187/respcare.01484
DO - 10.4187/respcare.01484
M3 - Article
C2 - 22348466
AN - SCOPUS:84866127546
VL - 57
SP - 1505
EP - 1508
JO - Respiratory Care
JF - Respiratory Care
SN - 0020-1324
IS - 9
ER -