Lung Transplantation for Williams-Campbell Syndrome With a Probable Familial Association

被引：5

作者：

Burguete, S. Rodrigo ^{[1
,2
]}

Levine, Stephanie M. ^{[1
,2
]}

Restrepo, Marcos I. ^{[1
,2
]}

Angel, Luis F. ^{[1
,2
]}

Levine, Deborah J. ^{[1
,2
]}

Coalson, Jacqueline J. ^{[1
,2
]}

Peters, Jay I. ^{[1
,2
]}

机构：

[1] Univ Texas Hlth Sci Ctr San Antonio, Dept Med, Div Pulm & Crit Care Med, San Antonio, TX 78229 USA

[2] S Texas Vet Hlth Care Syst, Vet Evidence Res Disseminat & Implementat Ctr, San Antonio, TX USA

来源：

RESPIRATORY CARE | 2012年 / 57卷 / 09期

关键词：

Williams-Campbell syndrome; bronchiectasis; lung transplantation; bronchial cartilage deficiency; CONGENITAL BRONCHIECTASIS; CARTILAGE DEFICIENCY; COMPUTED-TOMOGRAPHY; CT;

D O I：

10.4187/respcare.01484

中图分类号：

R4 [临床医学];

学科分类号：

1002 ; 100602 ;

摘要：

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.

引用

页码：1505 / 1508

页数：4

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