title: One- to four-year follow-up of endobronchial lung volume reduction in alpha-1-antitrypsin deficiency patients: a case series.
abstract: BACKGROUND
  Lung volume reduction surgery can improve lung function and working capacity in severe heterogeneous emphysema. Endobronchial lung volume reduction (ELVR) performed by one-way valves inserted via a flexible bronchoscope can result in a moderate but significant improvement in lung function and exercise tolerance, eliminating the surgical risks.
  
  
  OBJECTIVE
  Most studies of this method have excluded patients with α1-antitrypsin (AAT) deficiency, but small series of cases with positive short-term outcome have been reported. The sustainability of results has been questioned and we here present our experience in AAT-deficient patients treated with ELVR followed up for up to 4 years.
  
  
  METHODS
  From August 2008 to January 2012, 15 patients were treated with ELVR. Inclusion criteria were homozygotic AAT deficiency, age <80 years, residual volume of 140% or more, forced expiratory volume in 1 s (FEV1) 15-45% of predicted, severe heterogeneous emphysema, symptoms severely restricting daily life, informed consent and absence of other serious diseases.
  
  
  RESULTS
  One patient coughed up valves after 2 months, 1 developed pneumothorax and had valve displacement and subsequent removal, and 1 improved from an FEV1 of 0.62 to 0.84 liters, but after 4 months developed repeated and severe pneumonia and the valves had to be removed. Thus, 12 patients remained and were followed up for at least 1 year. In these patients, FEV1 increased (mean: 54%), the quality of life was much improved, and 2 patients could be taken off oxygen therapy. During the 4-year follow-up, patients demonstrated no significant deterioration in lung function.
  
  
  CONCLUSIONS
  In carefully selected AAT deficiency patients with severe emphysema, ELVR can be safely performed with encouraging long-lasting results.
authors.affiliation: Department of Respiratory Medicine and Allergy, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Gunnar
authors.initials: G
authors.lastname: Hillerdal
authors.forename: Stephanie
authors.initials: S
authors.lastname: Mindus
mesh_terms: D000368:Aged; D001999:Bronchoscopy / Q000379:methods; D017079:Exercise Tolerance; D005260:Female; D006801:Humans; D008176:Lung Volume Measurements / Q000379:methods; D008297:Male; D008875:Middle Aged; D011013:Pneumonectomy / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods; D011014:Pneumonia / Q000209:etiology*; D011030:Pneumothorax / Q000209:etiology*; D011183:Postoperative Complications / Q000175:diagnosis / Q000523:psychology / Q000628:therapy; D011475:Prosthesis Failure / Q000209:etiology*; D019919:Prosthesis Implantation / Q000009:adverse effects / Q000295:instrumentation / Q000379:methods; D011656:Pulmonary Emphysema / Q000209:etiology / Q000503:physiopathology; D011788:Quality of Life; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013525:Surgical Instruments / Q000009:adverse effects*; D016896:Treatment Outcome; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery