Observational studyRespiratory research2022
Clinical and functional characteristics of individuals with alpha-1 antitrypsin deficiency: EARCO international registry.
Observational study in Respiratory research, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. It is linked to trial NCT04180319 (A Pan-European Multi-Centre Observational Study To Determine The Natural History Of Patients With Alpha-1 Antitrypsin), which is not on this map. Cited by 34 papers, 2 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
A Pan-European Multi-Centre Observational Study To Determine The Natural History Of Patients With Alpha-1 Antitrypsin
Who cites it
34 citing papers in PubMed, 2 syntheses or guidelines pooled it, 45 citations in OpenAlex.
- The Relationship Between Gas Transfer Measurements, Lung Volumes, and Spirometric Indices in Alpha-1 Antitrypsin Deficiency and Non-AATD COPD: A Systematic Review.International journal of chronic obstructive pulmonary disease · 2026Pooled it
- Pulmonary Function Decline in Alpha-1 Antitrypsin Deficiency: A Systematic Review and Meta-Analysis.International journal of chronic obstructive pulmonary disease · 2026Pooled it
- Disparities in α1-Antitrypsin Deficiency Testing and Treatment in US Adults.JAMA network open · 2026Observational
- Community-BasedBiomedicines · 2026Article
- Dissociation of the Hepatic and Pulmonary Axes in Alpha-1 Antitrypsin Deficiency: Independent Trajectories of Organ-Specific Disease.Biomolecules · 2026Observational
- The Association of Alpha-1 Antitrypsin Deficiency and Arterial Aneurysms: An Update and Review.Journal of clinical and translational hepatology · 2026Review
- Alpha-1 Antitrypsin Deficiency-Associated Chronic Obstructive Pulmonary Disease.Medicina (Kaunas, Lithuania) · 2026Review
- Bronchiectasis in severe αERJ open research · 2026Article
- Characteristics of bronchiectasis in patients with different genotypes of severe αERJ open research · 2026Article
- Argo Delphi consensus statement on red flags and clinical gateways towards rare disease diagnosis.Scientific reports · 2025Article
- The phenotypic state of biallelicERJ open research · 2025Article
- Lung disease in never-smokers with severe αERJ open research · 2025Article
- Is αERJ open research · 2025Article
- Clinical characteristics of AATD-related COPD patients vary with age at diagnosis: data from the EARCO international registry.BMC pulmonary medicine · 2025Article
- Alpha-1 Antitrypsin Deficiency and Bronchial Asthma: Current Challenges.Biomolecules · 2025Review
- Alpha-1 antitrypsin deficiency-associated liver disease: From understudied disorder to the poster child of genetic medicine.Hepatology communications · 2025Review
- Characterization of the Mmalton carrier's cohort within the EARCO (European Alpha- 1 Antitrypsin Research Collaboration) registry.BMC pulmonary medicine · 2025Article
- Densitometric and Functional Progression in Patients with Alpha-1 Antitrypsin Deficiency Genotype SZ.Journal of clinical medicine · 2025Article
- Characterization of alpha-1 antitrypsin in a Pi∗ZZ patient with emphysema: a case report.Respiratory medicine case reports · 2025Article
- Can Quality of Life Tests Be Useful in Patients Affected by Alpha-1 Antitrypsin Deficiency?Journal of clinical medicine · 2024Article
Corrections and comments
- Erratum issued
Authors and funding
23 authors at 20 institutions in 10 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAlpha-1 antitrypsin deficiency (AATD) is a rare disease that is associated with an increased risk of pulmonary emphysema. The European AATD Research Collaboration (EARCO) international registry was founded with the objective of characterising the individuals with AATD and investigating their natural history.
methodsThe EARCO registry is an international, observational and prospective study of individuals with AATD, defined as AAT serum levels < 11 μM and/or proteinase inhibitor genotypes PI*ZZ, PI*SZ and compound heterozygotes or homozygotes of other rare deficient variants. We describe the characteristics of the individuals included from February 2020 to May 2022.
resultsA total of 1044 individuals from 15 countries were analysed. The most frequent genotype was PI*ZZ (60.2%), followed by PI*SZ (29.2%). Among PI*ZZ patients, emphysema was the most frequent lung disease (57.2%) followed by COPD (57.2%) and bronchiectasis (22%). Up to 76.4% had concordant values of FEV1(%) and KCO(%). Those with impairment in FEV1(%) alone had more frequently bronchiectasis and asthma and those with impairment in KCO(%) alone had more frequent emphysema and liver disease. Multivariate analysis showed that advanced age, male sex, exacerbations, increased blood platelets and neutrophils, augmentation and lower AAT serum levels were associated with worse FEV1(%).
conclusionsEARCO has recruited > 1000 individuals with AATD from 15 countries in its first 2 years. Baseline cross sectional data provide relevant information about the clinical phenotypes of the disease, the patterns of functional impairment and factors associated with poor lung function. Trial registration www. CLINICALTRIALS: gov (ID: NCT04180319).
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.