Lung Transplantation for Williams-Campbell Syndrome With a Probable Familial Association
| Autor: | Jay I. Peters, Stephanie M Levine, Marcos I. Restrepo, Jacqueline J. Coalson, Luis F. Angel, S Rodrigo Burguete, Deborah Levine |
|---|---|
| Rok vydání: | 2012 |
| Předmět: |
Adult
Male Pulmonary and Respiratory Medicine medicine.medical_specialty Pediatrics medicine.medical_treatment Disease Critical Care and Intensive Care Medicine Article medicine Humans Lung transplantation Williams–Campbell syndrome Bronchiectasis Lung business.industry Syndrome General Medicine respiratory system medicine.disease respiratory tract diseases Surgery Transplantation Cartilage medicine.anatomical_structure Respiratory failure Respiratory Insufficiency business Airway Cartilage Diseases Lung Transplantation |
| Zdroj: | Respiratory Care. 57:1505-1508 |
| ISSN: | 1943-3654 0020-1324 |
| DOI: | 10.4187/respcare.01484 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|