PMID- 32800189
OWN - NLM
STAT- MEDLINE
DCOM- 20201110
LR  - 20201110
IS  - 1557-8216 (Electronic)
IS  - 0272-5231 (Linking)
VI  - 41
IP  - 3
DP  - 2020 Sep
TI  - Alpha-1 Antitrypsin Deficiency Associated COPD.
PG  - 339-345
LID - S0272-5231(20)30029-0 [pii]
LID - 10.1016/j.ccm.2020.05.003 [doi]
AB  - Alpha-1 antitrypsin deficiency (AATD) was the first genetic risk factor for 
      chronic obstructive pulmonary disease (COPD) described. In the more than 50 years 
      since its description, the disease continues to provide insights into more common 
      forms of COPD. Although AATD is caused by a single genetic variant, the clinical 
      manifestations of disease include panacinar emphysema, airway 
      hyperresponsiveness, and bronchiectasis. With improved molecular understanding of 
      the mechanisms of disease pathogenesis and progression, new therapies in addition 
      to intravenous augmentation therapy are on the horizon.
CI  - Copyright (c) 2020 Elsevier Inc. All rights reserved.
FAU - Strange, Charlie
AU  - Strange C
AD  - Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Medical 
      University of South Carolina, 96 Jonathan Lucas Street, MSC630, Charleston, SC 
      29425, USA. Electronic address: strangec@musc.edu.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Clin Chest Med
JT  - Clinics in chest medicine
JID - 7907612
SB  - IM
MH  - Humans
MH  - Pulmonary Disease, Chronic Obstructive/*etiology/pathology
MH  - alpha 1-Antitrypsin Deficiency/*complications/pathology
OTO - NOTNLM
OT  - Alpha-1
OT  - Antiprotease
OT  - Antitrypsin
OT  - Emphysema
OT  - Genetic
OT  - Protease
COIS- Conflict of interest Dr C. Strange is a medical director of AlphaNet, a 
      not-for-profit disease management company for AATD. Dr C. Strange consults on 
      AATD with AstraZeneca, CSL Behring, Dicerna, Grifols, and Vertex. He has grants 
      paid to the Medical University of South Carolina from Adverum, CSA Medical, CSL 
      Behring, Grifols, MatRx, Novartis, Nuvaira, Pulmonx, Takeda, and Vertex.
EDAT- 2020/08/18 06:00
MHDA- 2020/11/11 06:00
CRDT- 2020/08/18 06:00
PHST- 2020/08/18 06:00 [entrez]
PHST- 2020/08/18 06:00 [pubmed]
PHST- 2020/11/11 06:00 [medline]
AID - S0272-5231(20)30029-0 [pii]
AID - 10.1016/j.ccm.2020.05.003 [doi]
PST - ppublish
SO  - Clin Chest Med. 2020 Sep;41(3):339-345. doi: 10.1016/j.ccm.2020.05.003.
+Error%3A+CEFetchPApplication%3A%3Aproxy_stream()%3A+%0A%3Chtml%3E%3Chead%3E%0A%3Cmeta+http-equiv%3D%22content-type%22+content%3D%22text%2Fhtml%3Bcharset%3Dutf-8%22%3E%0A%3Ctitle%3E503+Server+Error%3C%2Ftitle%3E%0A%3C%2Fhead%3E%0A%3Cbody+text%3D%23000000+bgcolor%3D%23ffffff%3E%0A%3Ch1%3EError%3A+Server+Error%3C%2Fh1%3E%0A%3Ch2%3EThe+service+you+requested+is+not+available+at+this+time.%3Cp%3EService+error+-27.%3C%2Fh2%3E%0A%3Ch2%3E%3C%2Fh2%3E%0A%3C%2Fbody%3E%3C%2Fhtml%3E