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One- to Four-Year Follow-Up of Endobronchial Lung Volume Reduction in Alpha-1-Antitrypsin Deficiency Patients: A Case Series
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Respiratory Medicine and Allergology.
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Respiratory Medicine and Allergology.
2014 (English)In: Respiration, ISSN 0025-7931, E-ISSN 1423-0356, Vol. 88, no 4, 320-328 p.Article in journal (Refereed) Published
Abstract [en]

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. Objectives: Most studies of this method have excluded patients with alpha(1)-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, symptomsseverely 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. Conclusion: In carefully selected AAT deficiency patients with severe emphysema, ELVR can be safely performed with encouraging long-lasting results.

Place, publisher, year, edition, pages
2014. Vol. 88, no 4, 320-328 p.
Keyword [en]
alpha(1)-Antitrypsin deficiency, Bronchoscopy, Emphysema, Hyperinflation, Lung function, Lung volume reduction, Single lung transplantation
National Category
Respiratory Medicine and Allergy
URN: urn:nbn:se:uu:diva-240955DOI: 10.1159/000365662ISI: 000343819400010PubMedID: 25227936OAI: oai:DiVA.org:uu-240955DiVA: diva2:777513
Available from: 2015-01-08 Created: 2015-01-08 Last updated: 2015-02-27Bibliographically approved

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