ArticleOrphanet journal of rare diseases2023
The prevalence of bronchiectasis in patients with alpha-1 antitrypsin deficiency: initial report of EARCO.
Article in Orphanet journal of rare diseases, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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18 citing papers in PubMed, 20 citations in OpenAlex.
- Quantitative CT of emphysema, wall thickness and mucus plugs in alpha-1-antitrypsin deficiency: relationship to clinical outcomes.European radiology · 2026Article
- Alpha-1 Antitrypsin Deficiency-Associated Chronic Obstructive Pulmonary Disease.Medicina (Kaunas, Lithuania) · 2026Review
- Alpha-1 Antitrypsin Deficiency Beyond COPD and Emphysema: A Narrative Review.Medical sciences (Basel, Switzerland) · 2026Review
- The Role of Alpha-1 Antitrypsin in the Pathophysiology and Treatment of Inflammatory Lung Diseases.Journal of inflammation research · 2026Review
- Article
- Bronchiectasis in severe αERJ open research · 2026Article
- Quantitative Analysis of Bronchiectasis in Alpha-1 Antitrypsin Deficiency.Canadian respiratory journal · 2026Article
- Characteristics of bronchiectasis in patients with different genotypes of severe αERJ open research · 2026Article
- Searching for alpha-1 antitrypsin deficiency in patients with bronchiectasis: reducing idiopathic cases.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2025Article
- Considerations in the Diagnosis and Management of Bronchiectasis in Older Adults.Clinics in geriatric medicine · 2025Review
- Pi*M Palermo Mutation in Bronchiectasis due to Alpha-1 Antitrypsin Deficiency: A Rare Genetic Cause.Medeniyet medical journal · 2025Article
- Rationale and Design of the Alpha-1 Biomarkers Consortium Study.Chronic obstructive pulmonary diseases (Miami, Fla.) · 2025Article
- Prevalence of SERPINA1 mutations in a bronchiectasis cohort: implications of extended screening for alpha-1 antitrypsin deficiency.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2025Article
- Bronchiectasis Occurs Independently of Chronic Obstructive Pulmonary Disease in Alpha-1 Antitrypsin Deficiency.Chronic obstructive pulmonary diseases (Miami, Fla.) · 2024Article
- Antitrypsin deficiency: still more to learn about the lung after 60 years.ERJ open research · 2024Review
- Alpha-1 antitrypsin deficiency associated with rareRespiratory medicine case reports · 2024Article
- Nine controversial questions about augmentation therapy for alpha-1 antitrypsin deficiency: a viewpoint.European respiratory review : an official journal of the European Respiratory Society · 2023Article
- Personalised indication of augmentation therapy for emphysema associated with severe alpha-1 antitrypsin deficiency: a case series.Therapeutic advances in respiratory diseaseArticle
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Authors and funding
6 authors at 4 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAlthough bronchiectasis has been recognised as a feature of some patients with Alpha1-Antitrypsin deficiency the prevalence and characteristics are not widely known. We wished to determine the prevalence of bronchiectasis and patient characteristics. The first cohort of patients recruited to the EARCO (European Alpha1 Research Collaboration) International Registry data base by the end of 2021 was analysed for radiological evidence of both emphysema and bronchiectasis as well as baseline demographic features.
resultsOf the first 505 patients with the PiZZ genotype entered into the data base 418 (82.8%) had a reported CT scan. There were 77 (18.4%) with a normal scan and 38 (9.1%) with bronchiectasis alone. These 2 groups were predominantly female never smokers and had lung function in the normal range. The remaining 303 (72.5%) ZZ patients all had emphysema on the scan and 113 (27%) had additional evidence of bronchiectasis.
conclusionsThe data indicates the bronchiectasis alone is a feature of 9.1% of patients with the PiZZ genotype of Alpha1-antitrypsin deficiency but although emphysema is the dominant lung pathology bronchiectasis is also present in 27% of emphysema cases and may require a different treatment strategy.
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