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dc.creatorZanichelli A., Farkas H., Bouillet L., Bara N., Germenis A.E., Psarros F., Varga L., Andrási N., Boccon-Gibod I., Castiglioni Roffia M., Rutkowski M., Cancian M.en
dc.date.accessioned2023-01-31T11:38:16Z
dc.date.available2023-01-31T11:38:16Z
dc.date.issued2021
dc.identifier10.1007/s12016-021-08855-4
dc.identifier.issn10800549
dc.identifier.urihttp://hdl.handle.net/11615/80951
dc.description.abstractHereditary 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. © 2021, The Author(s).en
dc.language.isoenen
dc.sourceClinical Reviews in Allergy and Immunologyen
dc.source.urihttps://www.scopus.com/inward/record.uri?eid=2-s2.0-85103369590&doi=10.1007%2fs12016-021-08855-4&partnerID=40&md5=fbc6402097f55259b6eb2545333a7bcb
dc.subjectcomplement component C1en
dc.subjectcomplement component C1s inhibitoren
dc.subjectdanazolen
dc.subjectgestagenen
dc.subjectlanadelumaben
dc.subjectstanozololen
dc.subjecttranexamic aciden
dc.subjectcomplement component C1s inhibitoren
dc.subjectadulten
dc.subjectageden
dc.subjectangioneurotic edemaen
dc.subjectArticleen
dc.subjectcomplement deficiencyen
dc.subjectdisease durationen
dc.subjectdisease registryen
dc.subjectdisease severityen
dc.subjectdrug useen
dc.subjectEuropeen
dc.subjectfemaleen
dc.subjecthumanen
dc.subjectinformation processingen
dc.subjectmajor clinical studyen
dc.subjectmaleen
dc.subjectmedical decision makingen
dc.subjectmiddle ageden
dc.subjectonline systemen
dc.subjectpathogenesisen
dc.subjectpatient careen
dc.subjectprophylaxisen
dc.subjectsoftwareen
dc.subjectvery elderlyen
dc.subjectyoung adulten
dc.subjectangioneurotic edemaen
dc.subjectgeneticsen
dc.subjectregisteren
dc.subjectAngioedemas, Hereditaryen
dc.subjectComplement C1 Inhibitor Proteinen
dc.subjectEuropeen
dc.subjectHumansen
dc.subjectRegistriesen
dc.subjectSpringeren
dc.titleThe Global Registry for Hereditary Angioedema due to C1-Inhibitor Deficiencyen
dc.typejournalArticleen


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