NDLI: Alpha-1 antitrypsin deficiency in Canada: regional disparities in diagnosis and management.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Bradi, Ana C. Audisho, Nadeen Casey, Deborah K. Chapman, Kenneth R.
Spatial Coverage	Canada
Description	Country affiliation: Canada Author Affiliation: Bradi AC ( 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.)
Abstract	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.
File Format	HTM / HTML
ISSN	15412555
Issue Number	Suppl 1
Volume Number	12
e-ISSN	15412563
Journal	COPD: Journal of Chronic Obstructive Pulmonary Disease
Language	English
Publisher	Taylor & Francis
Publisher Date	2015-05-01
Publisher Place	Great Britain (UK)
Access Restriction	Subscribed
Subject Keyword	Discipline Pulmonary Medicine Delayed Diagnosis Statistics & Numerical Data Healthcare Disparities Alpha 1-antitrypsin Deficiency Adult Aged Canada Epidemiology Disease Progression Female Follow-up Studies Humans Liver Cirrhosis Diagnosis Etiology Therapy Lung Diseases Male Middle Aged Registries Retrospective Studies Treatment Outcome Complications Journal Article Research Support, Non-u.s. Gov't
Content Type	Text
Resource Type	Article
Subject	Pulmonary and Respiratory Medicine

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