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

Research output: Contribution to journalArticle

4 Citations (Scopus)

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 2012

Fingerprint

Tracheobronchomalacia
Lung Transplantation
Bronchiectasis
Respiratory Insufficiency
Cartilage
Transplantation
Transplants
Lung
Survival
Therapeutics

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 journalArticle

Rodrigo Burguete, S. ; Levine, Stephanie M ; Restrepo, Marcos ; Angel, Luis F. ; Levine, Deborah J ; Coalson, Jacqueline J. ; Peters, Jay I. / Lung transplantation for Williams-Campbell syndrome with a probable familial association. In: Respiratory Care. 2012 ; Vol. 57, No. 9. pp. 1505-1508.
@article{088e22db53e34a2c872c4d1d47e640a8,
title = "Lung transplantation for Williams-Campbell syndrome with a probable familial association",
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.",
keywords = "Bronchial cartilage deficiency, Bronchiectasis, Lung transplantation, Williams-Campbell syndrome",
author = "{Rodrigo Burguete}, S. and Levine, {Stephanie M} and Marcos Restrepo and Angel, {Luis F.} and Levine, {Deborah J} and Coalson, {Jacqueline J.} and Peters, {Jay I}",
year = "2012",
month = "9",
doi = "10.4187/respcare.01484",
language = "English (US)",
volume = "57",
pages = "1505--1508",
journal = "Respiratory Care",
issn = "0020-1324",
publisher = "Daedalus Enterprises Inc.",
number = "9",

}

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

UR - http://www.scopus.com/inward/record.url?scp=84866127546&partnerID=8YFLogxK

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 -