PMID- 26238635
OWN - NLM
STAT- MEDLINE
DCOM- 20160509
LR  - 20191210
IS  - 1098-9048 (Electronic)
IS  - 1069-3424 (Linking)
VI  - 36
IP  - 4
DP  - 2015 Aug
TI  - Treatment of Alpha-1 Antitrypsin Deficiency.
PG  - 470-7
LID - 10.1055/s-0035-1555608 [doi]
AB  - Alpha-1 antitrypsin deficiency (AATD) is a rare genetic disease that creates 
      multiple unique phenotypes of chronic obstructive pulmonary disease. While 
      bronchospasm, cough, dyspnea, and sputum production all occur with AATD, the 
      phenotypic differences require a computed tomographic (CT) scan to decipher. The 
      availability of augmentation therapy in the United States since 1989 has 
      generated both controversy and evidence that informs the science of usual chronic 
      obstructive pulmonary disease (COPD). Because of the predominance of emphysema in 
      AATD, much of the best evidence concerning biomarkers of emphysema progression 
      comes from this population. Imaging measurement of emphysema progression, impact 
      of emphysema phenotypes on hyperinflation and dynamic hyperinflation, and 
      correlation with traditional spirometric measures of COPD progression are 
      required to understand the impact of AAT therapies. These studies are important 
      for better understanding of usual COPD pathogenesis. Significantly, there are no 
      adequately powered research studies to determine if augmentation therapy is 
      helpful for the non-emphysema phenotypes of AATD. Specifically, phenotypes of 
      chronic bronchitis, asthma predominant disease, and bronchiectasis will require 
      targeted research studies to define optimal therapy.
CI  - Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
FAU - Strange, Charlie
AU  - Strange C
AD  - Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Medical 
      University of South Carolina, Charleston, South Carolina.
FAU - Beiko, Tatsiana
AU  - Beiko T
AD  - Division of Pulmonary, Critical Care, Allergy and Sleep Medicine, Medical 
      University of South Carolina, Charleston, South Carolina.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
DEP - 20150803
PL  - United States
TA  - Semin Respir Crit Care Med
JT  - Seminars in respiratory and critical care medicine
JID - 9431858
RN  - 0 (Trypsin Inhibitors)
RN  - 0 (alpha 1-Antitrypsin)
SB  - IM
MH  - Disease Management
MH  - Disease Progression
MH  - Humans
MH  - Outcome Assessment, Health Care
MH  - *Pulmonary Disease, Chronic Obstructive/diagnosis/etiology
MH  - *Pulmonary Emphysema/diagnosis/etiology
MH  - Spirometry/methods
MH  - Tomography, X-Ray Computed/methods
MH  - Trypsin Inhibitors/pharmacology
MH  - alpha 1-Antitrypsin/*pharmacology
MH  - *alpha 1-Antitrypsin Deficiency/complications/diagnosis/physiopathology/therapy
EDAT- 2015/08/05 06:00
MHDA- 2016/05/10 06:00
CRDT- 2015/08/05 06:00
PHST- 2015/08/05 06:00 [entrez]
PHST- 2015/08/05 06:00 [pubmed]
PHST- 2016/05/10 06:00 [medline]
AID - 10.1055/s-0035-1555608 [doi]
PST - ppublish
SO  - Semin Respir Crit Care Med. 2015 Aug;36(4):470-7. doi: 10.1055/s-0035-1555608. 
      Epub 2015 Aug 3.