The prevalence of bronchiectasis in patients with alpha-1 antitrypsin deficiency : initial report of EARCO
© 2023. Institut National de la Santé et de la Recherche Médicale (INSERM)..
BACKGROUND: Although 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.
RESULTS: Of 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.
CONCLUSIONS: The 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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:18 |
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| Enthalten in: |
Orphanet journal of rare diseases - 18(2023), 1 vom: 12. Aug., Seite 243 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Stockley, Robert A [VerfasserIn] |
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| Links: |
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| Themen: |
Alpha 1-Antitrypsin |
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| Anmerkungen: |
Date Completed 14.08.2023 Date Revised 18.11.2023 published: Electronic Citation Status MEDLINE |
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| doi: |
10.1186/s13023-023-02830-2 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM360728448 |
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| 041 | |a eng | ||
| 100 | 1 | |a Stockley, Robert A |e verfasserin |4 aut | |
| 245 | 1 | 4 | |a The prevalence of bronchiectasis in patients with alpha-1 antitrypsin deficiency |b initial report of EARCO |
| 264 | 1 | |c 2023 | |
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| 500 | |a Date Completed 14.08.2023 | ||
| 500 | |a Date Revised 18.11.2023 | ||
| 500 | |a published: Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2023. Institut National de la Santé et de la Recherche Médicale (INSERM). | ||
| 520 | |a BACKGROUND: Although 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 | ||
| 520 | |a RESULTS: Of 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 | ||
| 520 | |a CONCLUSIONS: The 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Research Support, Non-U.S. Gov't | |
| 650 | 4 | |a Alpha-1 antitrypsin deficiency | |
| 650 | 4 | |a Bronchiectasis | |
| 650 | 4 | |a Emphysema | |
| 650 | 4 | |a Prevalence | |
| 650 | 7 | |a alpha 1-Antitrypsin |2 NLM | |
| 650 | 7 | |a SERPINA1 protein, human |2 NLM | |
| 700 | 1 | |a Pye, Anita |e verfasserin |4 aut | |
| 700 | 1 | |a De Soyza, Joshua |e verfasserin |4 aut | |
| 700 | 1 | |a Turner, Alice M |e verfasserin |4 aut | |
| 700 | 1 | |a Miravitlles, Marc |e verfasserin |4 aut | |
| 700 | 0 | |a EARCO study investigators |e verfasserin |4 aut | |
| 700 | 1 | |a Torres-Duran, María |e investigator |4 oth | |
| 700 | 1 | |a Tanash, Hanan |e investigator |4 oth | |
| 700 | 1 | |a Rodríguez-García, Carlota |e investigator |4 oth | |
| 700 | 1 | |a López-Campos, José Luis |e investigator |4 oth | |
| 700 | 1 | |a Chlumsky, Jan |e investigator |4 oth | |
| 700 | 1 | |a Guimaraes, Catarina |e investigator |4 oth | |
| 700 | 1 | |a Rodríguez-Hermosa, Juan Luis |e investigator |4 oth | |
| 700 | 1 | |a Corsico, Angelo |e investigator |4 oth | |
| 700 | 1 | |a Martinez-González, Cristina |e investigator |4 oth | |
| 700 | 1 | |a Hernández-Pérez, José María |e investigator |4 oth | |
| 700 | 1 | |a Bustamante, Ana |e investigator |4 oth | |
| 700 | 1 | |a Parr, David G |e investigator |4 oth | |
| 700 | 1 | |a Casas-Maldonado, Francisco |e investigator |4 oth | |
| 700 | 1 | |a Hecimovic, Ana |e investigator |4 oth | |
| 700 | 1 | |a Janssens, Wim |e investigator |4 oth | |
| 700 | 1 | |a Lara, Beatriz |e investigator |4 oth | |
| 700 | 1 | |a Barrecheguren, Miriam |e investigator |4 oth | |
| 700 | 1 | |a González, Cruz |e investigator |4 oth | |
| 700 | 1 | |a Stolk, Jan |e investigator |4 oth | |
| 700 | 1 | |a Clarenbach, Christian F |e investigator |4 oth | |
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