PMID- 25938286
OWN - NLM
STAT- MEDLINE
DCOM- 20160125
LR  - 20150505
IS  - 1541-2563 (Electronic)
IS  - 1541-2563 (Linking)
VI  - 12 Suppl 1
DP  - 2015 May
TI  - Alpha-1 antitrypsin deficiency in Canada: regional disparities in diagnosis and 
      management.
PG  - 15-21
LID - 10.3109/15412555.2015.1021908 [doi]
AB  - BACKGROUND: Since 1999, as part of the Alpha1 International Registry (AIR), the 
      Canadian Alpha-1 Antitrypsin Deficiency (AATD) Registry has maintained 
      demographic and medical information volunteered by AATD individuals. METHODS: We 
      undertook a retrospective chart review to describe the characteristics of 
      registry participants. Inclusion criteria were ZZ phenotype or other severe 
      deficiency and written consent. We reviewed baseline medical records and annual 
      follow-ups, conducted by mail. RESULTS: The number of registrants ranged from 8.7 
      per million in British Columbia and Ontario to 1.3 per million in Quebec. 
      Similarly, the rate of augmentation therapy use ranged from 7.7 per million in 
      British Columbia to 0.1 per million in Quebec. 290 patients (146 males), most 
      PiZZ, were enrolled by 2013. Patients with lung disease reported symptoms onset 
      at (mean +/- SD) 40 +/- 11 years but were diagnosed as AATD at 47 +/- 10 years. Typical 
      patients were ex-smokers with respiratory symptoms, severely reduced FEV1, an 
      accelerated rate of FEV1 decline, and minimal bronchodilator response. A subgroup 
      diagnosed by liver disease or familial screening was younger and had better 
      preserved lung function but a similar rate of FEV1 decline. There were 63 deaths, 
      of which 29 were lung-related and 6 were liver-related. Average age at death was 
      60.5 +/- 11.2 years. DISCUSSION: Most patients experience a diagnostic delay of 
      seven years after symptom onset, a period during which lung health may 
      deteriorate further. There is marked regional variation in the rate of diagnosis 
      and specific therapy usage for AAT in Canada.
FAU - Bradi, Ana C
AU  - Bradi AC
AD  - Asthma & Airway Centre, University Health Network, Toronto Western Hospital , 
      Toronto, Ontario , Canada and Division of Respiratory Medicine, Department of 
      Medicine, University of Toronto , Toronto, Ontario , Canada.
FAU - Audisho, Nadeen
AU  - Audisho N
FAU - Casey, Deborah K
AU  - Casey DK
FAU - Chapman, Kenneth R
AU  - Chapman KR
LA  - eng
GR  - Canadian Institutes of Health Research/Canada
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - England
TA  - COPD
JT  - COPD
JID - 101211769
SB  - IM
MH  - Adult
MH  - Aged
MH  - Canada/epidemiology
MH  - Delayed Diagnosis/*statistics & numerical data
MH  - Disease Progression
MH  - Female
MH  - Follow-Up Studies
MH  - Healthcare Disparities/*statistics & numerical data
MH  - Humans
MH  - Liver Cirrhosis/diagnosis/epidemiology/etiology/therapy
MH  - Lung Diseases/diagnosis/epidemiology/etiology/therapy
MH  - Male
MH  - Middle Aged
MH  - Registries
MH  - Retrospective Studies
MH  - Treatment Outcome
MH  - *alpha 1-Antitrypsin Deficiency/complications/diagnosis/epidemiology/therapy
EDAT- 2015/05/06 06:00
MHDA- 2016/01/26 06:00
CRDT- 2015/05/05 06:00
PHST- 2015/05/05 06:00 [entrez]
PHST- 2015/05/06 06:00 [pubmed]
PHST- 2016/01/26 06:00 [medline]
AID - 10.3109/15412555.2015.1021908 [doi]
PST - ppublish
SO  - COPD. 2015 May;12 Suppl 1:15-21. doi: 10.3109/15412555.2015.1021908.