title: Endobronchial valve deployment in severe α-1 antitrypsin deficiency emphysema: a case series.
abstract: BACKGROUND
  Patients with end-stage emphysema because of α-1 antitrypsin (AAT) deficiency represent a challenging clinical management problem, and studies of volume reduction therapy to date have largely excluded these patients. We report the outcome of bronchoscopic volume reduction with the insertion of Emphasys endobronchial valves (Emphasys Medical, Redwood City, CA, USA) in six patients with end-stage emphysema because of AAT deficiency.
  
  
  METHODS
  Of 51 patients with end stage emphysema referred for transplantation, we studied six patients with AAT deficiency and utilized the BODE index and lung allocation score for survival estimation.
  
  
  RESULTS
  The forced expiratory volume in 1s improved from a median of 0.575L to 0.905L (P=0.028). There was a median reduction in total lung capacity (TLC) of 0.61L. The residual volume /TLC fell from 74.0% to 58.4%. Before treatment, four patients had a BODE index of greater than eight units, which correlates with a 4-year survival of 18%. After treatment, two patients improved their BODE index to below seven units, which correlates with an estimated 4-year survival of over 50%.
  
  
  CONCLUSIONS
  The data from this case series suggest that this intervention may provide bridging therapy to subsequent transplantation for younger AAT patients with end-stage emphysema.
authors.affiliation: Advanced Lung Disease and Lung Transplant Programme, Mater Misericordiae University Hospital, University College Dublin, Dublin, Ireland.
authors.forename: Mary Majella
authors.initials: MM
authors.lastname: Tuohy
authors.forename: Kasper Friedrich
authors.initials: KF
authors.lastname: Remund
authors.forename: Roger
authors.initials: R
authors.lastname: Hilfiker
authors.forename: Dara Thomas
authors.initials: DT
authors.lastname: Murphy
authors.forename: John Gerard
authors.initials: JG
authors.lastname: Murray
authors.forename: Jim John
authors.initials: JJ
authors.lastname: Egan
mesh_terms: D000328:Adult; D004646:Emphysema / Q000209:etiology* / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume; D006801:Humans; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D011013:Pneumonectomy / Q000009:adverse effects / Q000379:methods*; D011183:Postoperative Complications / Q000209:etiology; D015996:Survival Rate; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications* / Q000401:mortality