Document Type
Article
Publication Date
12-22-2022
Abstract
OBJECTIVE: To estimate the prevalence of diagnosed alpha-1 antitrypsin deficiency (dAATD) in Denmark as of 31 December 2018, and dAATD incidence and mortality from 1 January 2000 to 31 December 2018.
STUDY DESIGN AND SETTING: We used the Danish National Patient Registry to identify patients with dAATD based on the International Classification of Diseases, 10th Revision (ICD-10) code E88.0A and the Danish Civil Registration System (CRS) for population counts and vital status. We estimated dAATD prevalence, incidence and mortality. We compared mortality among patients with dAATD and an age-matched and sex-matched cohort extracted from the Danish CRS. We conducted a sensitivity analysis to examine whether coding changes during 2000-2018, from a general to a more specific ICD-10 code for AATD, and left truncation affected results appreciably.
RESULTS: The prevalence of dAATD was 12.9 (95% CI 11.9 to 13.8) per 100 000 persons. The age distribution was bimodal, with peaks at ages ≤12 and ≥45 years. The incidence rate per 100 000 person-years was 0.90 (95% CI 0.85 to 0.96), again with a bimodal age distribution. Mortality was higher for patients with dAATD than for the general population (mortality rate ratio (mRR) 4.7, 95% CI 4.1 to 5.3), especially for children (mRR 33.8, 95% CI 6.8 to 167.4). The sensitivity analysis indicated that dAATD prevalence might have been as high as 19.7 per 100 000 persons due to less specific ICD-10 coding for AATD early in the study period or 21.4 per 100 000 persons correcting for left truncation.
CONCLUSION: Diagnosed AATD was associated with increased mortality, especially for children. The finding for children was based on few deaths and had very wide 95% CIs.
Recommended Citation
Acquavella, John; Vágó, Emese; Sorensen, Henrik Toft; Horváth-Puhó, Erzsébet; and Hess, Gregory P., "Registry-Based Cohort Study of Alpha-1 Antitrypsin Deficiency Prevalence, Incidence and Mortality in Denmark 2000-2018" (2022). College of Population Health Faculty Papers. Paper 163.
https://jdc.jefferson.edu/healthpolicyfaculty/163
Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial 4.0 License
Language
English
Comments
This article is the author's final published version in BMJ Open Respiratory Research, Volume 9, Issue 1, December 2022, Article number e001281.
The published version of this article is available at http://doi.org/10.1136/bmjresp-2022-001281. Copyright © Author(s) (or their employer(s)) 2022. Published by BMJ.