Lung Volume Reduction Surgery for Patients with Alpha-1 Antitrypsin Deficiency Emphysema

James M. Donahue, Stephen D. Cassivi

Research output: Contribution to journalReview article

10 Citations (Scopus)

Abstract

Alpha-1 antitrypsin deficiency (A1AD) is a rare genetic disorder characterized by early-onset emphysema and, rarely, liver disease and vasculitis. In the lungs, unopposed and enhanced elastase activity results in accelerated parenchymal destruction leading to emphysematous changes predominantly in the lower lung fields. Medical treatment includes standard therapies for emphysema and so-called "augmentation therapy" using purified pooled plasma alpha-1 antitrypsin. Surgical options include lung transplantation and lung volume reduction surgery. The option of lung volume reduction surgery potentially provides palliation of dyspnea and a bridge to lung transplantation.

Original languageEnglish (US)
Pages (from-to)201-208
Number of pages8
JournalThoracic Surgery Clinics
Volume19
Issue number2
DOIs
StatePublished - May 2009

Fingerprint

alpha 1-Antitrypsin Deficiency
Pneumonectomy
Emphysema
Lung Transplantation
Lung
alpha 1-Antitrypsin
Inborn Genetic Diseases
Pancreatic Elastase
Vasculitis
Dyspnea
Liver Diseases
Therapeutics
Autosomal Recessive alpha-1-Antitrypsin Deficiency

Keywords

  • Alpha-1 antitrypsin deficiency
  • Emphysema
  • Lung volume reduction surgery
  • Surgery

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine

Cite this

Lung Volume Reduction Surgery for Patients with Alpha-1 Antitrypsin Deficiency Emphysema. / Donahue, James M.; Cassivi, Stephen D.

In: Thoracic Surgery Clinics, Vol. 19, No. 2, 05.2009, p. 201-208.

Research output: Contribution to journalReview article

Donahue, James M. ; Cassivi, Stephen D. / Lung Volume Reduction Surgery for Patients with Alpha-1 Antitrypsin Deficiency Emphysema. In: Thoracic Surgery Clinics. 2009 ; Vol. 19, No. 2. pp. 201-208.
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