ArticleInternational journal of chronic obstructive pulmonary disease2016
Cause-specific mortality in individuals with severe alpha 1-antitrypsin deficiency in comparison with the general population in Sweden.
Article in International journal of chronic obstructive pulmonary disease, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers, 2 of them syntheses that pooled it.
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Who cites it
23 citing papers in PubMed, 2 syntheses or guidelines pooled it, 39 citations in OpenAlex.
- Disease burden associated with alpha-1 antitrypsin deficiency: systematic and structured literature reviews.European respiratory review : an official journal of the European Respiratory Society · 2022Pooled it
- Severe αRespiratory research · 2022Pooled it
- Prevalence of alpha-1-antitrypsin deficiency carriers in a population with and without colonic diverticula. A multicentre prospective case-control study: the ALADDIN study.International journal of colorectal disease · 2019Trial
- Alpha-1 antitrypsin deficiency associated with increased risk of venous thromboembolism: a nationwide cohort study in Denmark.Research and practice in thrombosis and haemostasis · 2026Article
- Alpha-1 antitrypsin deficiency and cardiovascular disease: a review.Respiratory research · 2026Review
- αERJ open research · 2025Article
- Epidemiology and outcomes of alpha-1 antitrypsin deficiency in Sweden 2002-2020: A population-based cohort study of 2286 individuals.Journal of internal medicine · 2025Article
- Hospital Admission Trends in Alpha-1-Antitrypsin Deficiency: A Sex-Based Analysis from the Spanish National Discharge Database, 2016-2022.Journal of clinical medicine · 2024Article
- αERJ open research · 2023Article
- Heterozygous Alpha-1 Antitrypsin Deficiency Causing Pulmonary Emboli and Pulmonary Bullae.Cureus · 2021Article
- α1-Antitrypsin deficiency and the risk of COVID-19: an urgent call to action.The Lancet. Respiratory medicine · 2021Article
- Do gene-environment interactions play a role in COVID-19 distribution? The case of Alpha-1 Antitrypsin, air pollution and COVID-19.Multidisciplinary respiratory medicine · 2021Article
- Alpha 1 Antitrypsin Therapy in Patients with Alpha 1 Antitrypsin Deficiency: Perspectives from a Registry Study and Practical Considerations for Self-Administration During the COVID-19 Pandemic.International journal of chronic obstructive pulmonary disease · 2021Review
- Alpha-1-antitrypsin deficiency (carrier) as possible risk factor for development of colonic diverticula. A multicentre prospective case-control study: the ALADDIN study.Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and Ireland · 2020Article
- Decreased Risk of Ischemic Heart Disease in Individuals with Severe Alpha 1-Antitrypsin Deficiency (PiZZ) in Comparison with the General Population.International journal of chronic obstructive pulmonary disease · 2020Article
- Association between diverticular disease requiring surgical intervention and mortality in the postlung transplant population - a retrospective cohort study.Transplant international : official journal of the European Society for Organ Transplantation · 2019Article
- Liver disease in adults with severe alpha-1-antitrypsin deficiency.Journal of gastroenterology · 2019Article
- Update on αBreathe (Sheffield, England) · 2018Review
- Effect of Pingchuan Guben decoction on patients with chronic obstructive pulmonary disease: Results from a randomized comparative effectiveness research trial.Experimental and therapeutic medicine · 2017Article
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Corrections and comments
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Authors and funding
4 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSevere alpha 1-antitrypsin deficiency (PiZZ) predisposes to morbidity and mortality due to early-onset emphysema and liver disease. The risk of death from other causes, including cardiovascular disease and cancer, has not been well investigated. We aimed to analyze cause-specific mortality in PiZZ individuals compared with the general Swedish population.
methodsData on 1,561 PiZZ individuals from the Swedish National AAT Deficiency Register, prospectively followed from 1991 to 2014, were analyzed. Causes of death according to the Swedish National Causes of Death Register for the study group were compared with those for the general Swedish population matched for age, sex, and calendar year, with the excess mortality expressed as standardized mortality ratios (SMRs) with 95% confidence intervals (CIs).
resultsThere were 524 deaths during the follow-up period. PiZZ individuals had excess all-cause mortality compared with the Swedish general population (SMR 3.6, 95% CI 3.3-3.9). SMR for ischemic heart disease (IHD) was 0.5 (95% CI 0.3-0.8) and was similar for never and ever-smokers, and in males and females. SMR for lung cancer was 0.9 (95% CI 0.4-1.7). PiZZ individuals had increased mortality compared with the general population for the following diseases: respiratory disease, SMR 48.4 (95% CI 43.0-54.5); primary liver carcinoma, SMR 90.0 (95% CI 59.3-130.9); complicated colon diverticulitis, SMR 20.8 (95% CI 6.7-48.6); and pulmonary embolism, SMR 6.9 (95% CI 3.3-12.7).
conclusionPiZZ individuals had a reduced mortality risk of IHD. Mortality due to respiratory, hepatic disease, diverticulitis, and pulmonary embolism was markedly increased compared with the age- and sex-matched Swedish population.
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