Advanced search
Publication Cover
International Journal of Chronic Obstructive Pulmonary Disease Volume 15, 2020 - Issue
Submit an article Journal homepage
147
Views
6
CrossRef citations to date
0
Altmetric
Original Research

Alpha-1 Antitrypsin Augmentation Therapy Improves Survival in Severely Deficient Patients with Predicted FEV1 Between 10% and 60%: A Retrospective Analysis of the NHLBI Alpha-1 Antitrypsin Deficiency Registry

Franck F Rahaghi1 Department of Pulmonary and Critical Care, Cleveland Clinic Florida, Weston, FL, USACorrespondence[email protected]
,
Richard Monk2 Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Medical University of South Carolina, Charleston, SC, USA
,
Viswanathan Ramakrishnan3 Department of Public Health Sciences, Medical University of South Carolina, Charleston, SC, USA
,
Tatsiana Beiko2 Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Medical University of South Carolina, Charleston, SC, USA
&
Charlie Strange2 Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Medical University of South Carolina, Charleston, SC, USAORCID Iconhttps://orcid.org/0000-0002-8109-8067
ORCID Icon
Pages 3193-3199 | Published online: 03 Dec 2020

References

  • LaurellCB, ErikssonS. The electrophoretic alpha1-globulin pattern of serum in alpha1-antitrypsin deficiency. 1963. Copd. 2013;10(Suppl 1):3–8. doi:10.3109/15412555.2013.77195623527532
  • WewersMD, CasolaroMA, SellersSE, et al. Replacement therapy for alpha 1-antitrypsin deficiency associated with emphysema. N Engl J Med. 1987;316(17):1055–1062. doi:10.1056/NEJM1987042331617043494198
  • StockleyRA, MiravitllesM, VogelmeierC. Augmentation therapy for alpha-1 antitrypsin deficiency: towards a personalised approach. Orphanet J Rare Dis. 2013;8:149. doi:10.1186/1750-1172-8-14924063809
  • SandhausRA, StollerJK. Introduction to the 50th anniversary of the description of alpha-1 antitrypsin deficiency. COPD. 2013;10(Suppl sup1):1–2. doi:10.3109/15412555.2013.765840
  • ChapmanKR, StockleyRA, DawkinsC, WilkesMM, NavickisRJ. Augmentation therapy for alpha1 antitrypsin deficiency: a meta-analysis. Copd. 2009;6(3):177–184. doi:10.1080/1541255090290596119811373
  • McElvaneyNG, StollerJK, BuistAS, et al. Baseline characteristics of enrollees in the National Heart, Lung and Blood Institute Registry of alpha 1-antitrypsin deficiency. Chest. 1997;111(2):394–403. doi:10.1378/chest.111.2.3949041988
  • StollerJK, TomashefskiJ, CrystalRG, et al. Mortality in individuals with severe deficiency of alpha1-antitrypsin: findings from the National Heart, Lung, and Blood Institute Registry. Chest. 2005;127(4):1196–1204.15821195
  • The Alpha-1-Antitrypsin Deficiency Registry Study Group. Survival and FEV1 decline in individuals with severe deficiency of alpha1-antitrypsin. Am J Respir Crit Care Med. 1998;158(1):49–59. doi:10.1164/ajrccm.158.1.97120179655706
  • HankinsonJL, OdencrantzJR, FedanKB. Spirometric reference values from a sample of the general U.S. population. Am J Respir Crit Care Med. 1999;159(1):179–187. doi:10.1164/ajrccm.159.1.97121089872837
  • CrapoRO, MorrisAH, ClaytonPD, NixonCR. Lung volumes in healthy nonsmoking adults. Bull Eur Physiopathol Respir. 1982;8(3):419–425.
  • CrapoRO, MorrisAH. Standardized single breath normal values for carbon monoxide diffusing capacity. Am Rev Respir Dis. 1981;123(2):185–189.7235357
  • FletcherC, PetoR. The natural history of chronic airflow obstruction. Br Med J. 1977;1(6077):1645–1648. doi:10.1136/bmj.1.6077.1645871704
  • BhavaniS, TsaiCL, PerusichS, et al. Clinical and immunological factors in emphysema progression: 5-year prospective LES-COPD study. Am J Respir Crit Care Med. 2015;192(10):1171–1178. doi:10.1164/rccm.201504-0736OC26241705
  • BudweiserS, HarlacherM, PfeiferM, JorresRA. Co-morbidities and hyperinflation are independent risk factors of all-cause mortality in very severe COPD. Copd. 2014;11(4):388–400. doi:10.3109/15412555.2013.83617424111878
  • CelliBR, CoteCG, MarinJM, et al. The body-mass index, airflow obstruction, dyspnea, and exercise capacity index in chronic obstructive pulmonary disease. N Engl J Med. 2004;350(10):1005–1012. doi:10.1056/NEJMoa02132214999112
  • ParrDG, StoelBC, StolkJ, StockleyRA. Validation of computed tomographic lung densitometry for monitoring emphysema in alpha1-antitrypsin deficiency. Thorax. 2006;61(6):485–490. doi:10.1136/thx.2005.05489016537666
  • McElvaneyNG, BurdonJ, HolmesM, et al. Long-term efficacy and safety of α1 proteinase inhibitor treatment for emphysema caused by severe α1 antitrypsin deficiency: an open-label extension trial (RAPID-OLE). Lancet Respir Med. 2017;5(1):51–60. doi:10.1016/S2213-2600(16)30430-127916480
  • EllisP, HolmK, ChoateR, et al. Comparison of outcomes in augmentation naïve and augmented patients with alpha-1 antitrypsin deficiency related lung disease. Eur Respir J. 2019;54:PA3383.

Related research

People also read lists articles that other readers of this article have read.

Recommended articles lists articles that we recommend and is powered by our AI driven recommendation engine.

Cited by lists all citing articles based on Crossref citations.
Articles with the Crossref icon will open in a new tab.