PMID- 29175361
OWN - NLM
STAT- MEDLINE
DCOM- 20190920
LR  - 20190920
IS  - 1931-3543 (Electronic)
IS  - 0012-3692 (Print)
IS  - 0012-3692 (Linking)
VI  - 153
IP  - 5
DP  - 2018 May
TI  - The Role of Computed Tomography for the Evaluation of Lung Disease in Alpha-1 
      Antitrypsin Deficiency.
PG  - 1240-1248
LID - S0012-3692(17)33130-6 [pii]
LID - 10.1016/j.chest.2017.11.017 [doi]
AB  - Alpha-1 antitrypsin deficiency (AATD) is characterized by low serum levels of or 
      dysfunctional alpha-1 proteinase inhibitor. In the lung parenchyma, this results 
      in a loss of protection against the activity of serine proteases, particularly 
      neutrophil elastase. The resultant imbalance in protease and antiprotease 
      activity leads to an increased risk for the development of early-onset emphysema 
      and COPD. As in traditional smoke-related COPD, the assessment of the severity 
      and disease progression of lung disease in AATD is conventionally based on lung 
      function; however, pulmonary function tests are unable to discriminate between 
      emphysema and airways disease, the two hallmark pathologic features of COPD. CT 
      imaging has been used as a tool to further characterize lung structure and 
      evaluate therapeutic interventions in AATD-related COPD. Moreover, recent 
      advances in quantitative CT have significantly improved our assessment of the 
      lung architecture, which has provided investigators and clinicians with a more 
      detailed evaluation of the extent and severity of emphysema and airways disease 
      in AATD. In addition, serial CT imaging measures are becoming increasingly 
      important, as they provide a tool to monitor emphysema progression. This review 
      describes the principles of CT technology and the role of CT imaging in assessing 
      pulmonary disease progression in AATD, including the effect of therapeutic 
      interventions.
CI  - Published by Elsevier Inc.
FAU - Campos, Michael A
AU  - Campos MA
AD  - Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, University of 
      Miami School of Medicine, Miami, FL. Electronic address: Mcampos1@med.miami.edu.
FAU - Diaz, Alejandro A
AU  - Diaz AA
AD  - Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham 
      and Women's Hospital, Harvard Medical School, Boston, MA.
LA  - eng
GR  - K01 HL118714/HL/NHLBI NIH HHS/United States
GR  - R01 HL133137/HL/NHLBI NIH HHS/United States
PT  - Journal Article
PT  - Review
DEP - 20171123
PL  - United States
TA  - Chest
JT  - Chest
JID - 0231335
SB  - IM
MH  - Humans
MH  - Lung Diseases/*diagnostic imaging/*etiology
MH  - *Tomography, X-Ray Computed
MH  - alpha 1-Antitrypsin Deficiency/*complications/diagnostic imaging
PMC - PMC6026284
OTO - NOTNLM
OT  - COPD
OT  - CT
OT  - alpha-1 antitrypsin
OT  - alpha-1 antitrypsin deficiency
OT  - alpha-1 proteinase inhibitor
OT  - emphysema
EDAT- 2017/11/28 06:00
MHDA- 2019/09/21 06:00
PMCR- 2019/05/01
CRDT- 2017/11/28 06:00
PHST- 2017/06/24 00:00 [received]
PHST- 2017/10/15 00:00 [revised]
PHST- 2017/11/09 00:00 [accepted]
PHST- 2017/11/28 06:00 [pubmed]
PHST- 2019/09/21 06:00 [medline]
PHST- 2017/11/28 06:00 [entrez]
PHST- 2019/05/01 00:00 [pmc-release]
AID - S0012-3692(17)33130-6 [pii]
AID - 10.1016/j.chest.2017.11.017 [doi]
PST - ppublish
SO  - Chest. 2018 May;153(5):1240-1248. doi: 10.1016/j.chest.2017.11.017. Epub 2017 Nov 
      23.