The Global Registry for Hereditary Angioedema due to C1-Inhibitor Deficiency.
Attacks
C1-inhibitor
Database
Global
Hereditary angioedema
Prophylaxis
Registry
Journal
Clinical reviews in allergy & immunology
ISSN: 1559-0267
Titre abrégé: Clin Rev Allergy Immunol
Pays: United States
ID NLM: 9504368
Informations de publication
Date de publication:
Aug 2021
Aug 2021
Historique:
accepted:
25
02
2021
pubmed:
2
4
2021
medline:
15
12
2021
entrez:
1
4
2021
Statut:
ppublish
Résumé
Hereditary angioedema (HAE) is a rare condition, mostly due to genetic deficiency of complement C1 inhibitor (C1-INH). The rarity of HAE impedes extensive data collection and assessment of the impact of certain factors known to affect the course of this disabling and life-threatening disease. Establishing a global registry could assist to overcome such issues and provides valuable patient data from different countries. The HAE Global Registry is a disease-specific registry, with web-based electronic support, where data are provided by physicians and patients through a dedicated application. We collected data between January 1, 2018, and August 31, 2020. Data on 1297 patients from 29 centers in 5 European countries were collected. At least one attack was recorded for 497 patients during the study period. Overall, 1182 patients were diagnosed with HAE type 1 and 115 with type 2. At the time of database lock, 389 patients were taking long-term prophylactic medication, 217 of which were on danazol. Most recorded attacks affected the abdomen, were generally moderate in severity, and occurred in patients who were not on prophylactic treatment (70.6%, 6244/8848). The median duration of attacks was 780 min (IQR 290-1740) in patients on prophylactic medication and 780 min (IQR 300-1920) in patients not on continuous prophylactic medication. In conclusion, the establishment of a registry for C1-INH-HAE allowed collection of a large amount of data that may help to better understand the clinical characteristics of this disease. This information may enhance patient care and guide future therapeutic decisions.
Identifiants
pubmed: 33791951
doi: 10.1007/s12016-021-08855-4
pii: 10.1007/s12016-021-08855-4
pmc: PMC8282542
doi:
Substances chimiques
Complement C1 Inhibitor Protein
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
77-83Informations de copyright
© 2021. The Author(s).
Références
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