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Lung transplantation for Williams-Campbell syndrome.

Publication ,  Journal Article
Palmer, SM; Layish, DT; Kussin, PS; Oury, T; Davis, RD; Tapson, VF
Published in: Chest
February 1998

Williams-Campbell syndrome is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi leading to distal airway collapse and bronchiectasis. We report the first case of lung transplantation in a patient with end-stage lung disease secondary to Williams-Campbell syndrome. Although the patient did not have proximal airway collapse prior to transplantation, his posttransplant course was complicated by the development of bronchomalacia of the right and left mainstem bronchi. The patient experienced recurrent pulmonary infections and died of bacterial pneumonia 1 year after transplantation. Autopsy revealed cartilage deficiency in both right and left mainstem bronchi. A hypothesis may be made that a combination of proximal cartilage deficiency and posttransplant airway ischemia led to the development of bronchomalacia after lung transplantation. Thus, in contrast to previous reports, the cartilage deficiency in Williams-Campbell syndrome can involve both proximal and distal airways. Consequently, bilateral sequential lung transplantation may not be an effective therapeutic option in patients with this syndrome.

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Published In

Chest

DOI

ISSN

0012-3692

Publication Date

February 1998

Volume

113

Issue

2

Start / End Page

534 / 537

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Syndrome
  • Respiratory System
  • Respiratory Insufficiency
  • Recurrence
  • Pulmonary Emphysema
  • Pseudomonas aeruginosa
  • Pseudomonas Infections
  • Postoperative Complications
  • Pneumonia, Bacterial
 

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Palmer, S. M., Layish, D. T., Kussin, P. S., Oury, T., Davis, R. D., & Tapson, V. F. (1998). Lung transplantation for Williams-Campbell syndrome. Chest, 113(2), 534–537. https://doi.org/10.1378/chest.113.2.534
Palmer, S. M., D. T. Layish, P. S. Kussin, T. Oury, R. D. Davis, and V. F. Tapson. “Lung transplantation for Williams-Campbell syndrome.Chest 113, no. 2 (February 1998): 534–37. https://doi.org/10.1378/chest.113.2.534.
Palmer SM, Layish DT, Kussin PS, Oury T, Davis RD, Tapson VF. Lung transplantation for Williams-Campbell syndrome. Chest. 1998 Feb;113(2):534–7.
Palmer, S. M., et al. “Lung transplantation for Williams-Campbell syndrome.Chest, vol. 113, no. 2, Feb. 1998, pp. 534–37. Pubmed, doi:10.1378/chest.113.2.534.
Palmer SM, Layish DT, Kussin PS, Oury T, Davis RD, Tapson VF. Lung transplantation for Williams-Campbell syndrome. Chest. 1998 Feb;113(2):534–537.
Journal cover image

Published In

Chest

DOI

ISSN

0012-3692

Publication Date

February 1998

Volume

113

Issue

2

Start / End Page

534 / 537

Location

United States

Related Subject Headings

  • Treatment Outcome
  • Syndrome
  • Respiratory System
  • Respiratory Insufficiency
  • Recurrence
  • Pulmonary Emphysema
  • Pseudomonas aeruginosa
  • Pseudomonas Infections
  • Postoperative Complications
  • Pneumonia, Bacterial