PMID- 32396717
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20210212
IS  - 2372-952X (Print)
IS  - 2372-952X (Electronic)
IS  - 2372-952X (Linking)
VI  - 7
IP  - 3
DP  - 2020 Jul
TI  - Alpha-1 Antitrypsin Deficiency: Have We Got the Right Proteinase?
PG  - 163-171
LID - 10.15326/jcopdf.7.3.2019.0151 [doi]
AB  - Alpha-1 antitrypsin deficiency (AATD) has traditionally been associated with the 
      development of early onset panlobular emphysema thought to reflect the direct 
      interstitial damage caused by neutrophil elastase. Since this enzyme is highly 
      sensitive to irreversible inhibition by alpha-1 antitrypsin (AAT), the logic of 
      intravenous augmentation therapy has remained unquestioned and efficacy is 
      supported by both observational studies and formal clinical trials. However, 
      evidence suggests that although AAT augmentation modulates the progression of 
      emphysema, it only slows it down. This raises the issue of whether our long-held 
      beliefs of the cause of the susceptibility to develop emphysema in deficient 
      individuals are correct. There are several aspects of our understanding of the 
      disease that might benefit from a radical departure from traditional thought. 
      This review addresses these concepts and alternative pathways that may be central 
      to progression of emphysema.
CI  - JCOPDF (c) 2020.
FAU - Stockley, Robert A
AU  - Stockley RA
AD  - Lung Investigation Unit, University Hospitals, Birmingham National Health Service 
      Foundation Trust, Queen Elizabeth Hospital, Edgbaston, Birmingham, United 
      Kingdom.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Chronic Obstr Pulm Dis
JT  - Chronic obstructive pulmonary diseases (Miami, Fla.)
JID - 101635411
PMC - PMC7857712
OTO - NOTNLM
OT  - alpha-1 antitrypsin deficiency
OT  - emphysema
OT  - oxidants
OT  - proteinases
OT  - small airways disease
COIS- This review is independently conceived and written. However, RAS serves on 
      multiple advisory boards for companies with interest in treatments for AATD 
      including Grifols, Kamada, CSL Behring, Vertex, Mereo BioPharma, Inhibrx, Z 
      Factor, pH Pharma and Shire.
EDAT- 2020/05/13 06:00
MHDA- 2020/05/13 06:01
PMCR- 2020/04/30
CRDT- 2020/05/13 06:00
PHST- 2020/05/13 06:00 [pubmed]
PHST- 2020/05/13 06:01 [medline]
PHST- 2020/05/13 06:00 [entrez]
PHST- 2020/04/30 00:00 [pmc-release]
AID - 10.15326/jcopdf.7.3.2019.0151 [doi]
PST - ppublish
SO  - Chronic Obstr Pulm Dis. 2020 Jul;7(3):163-171. doi: 
      10.15326/jcopdf.7.3.2019.0151.