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dc.contributorVall d'Hebron Barcelona Hospital Campus
dc.contributor.authorGuilarte Clavero, Mar
dc.contributor.authorSala Cunill, Anna
dc.contributor.authorBaeza, María Luisa
dc.contributor.authorCabañas, Rosario
dc.contributor.authorHernández, María Dolores
dc.contributor.authorIbañez, Ethel
dc.date.accessioned2022-06-16T07:25:31Z
dc.date.available2022-06-16T07:25:31Z
dc.date.issued2021-12-29
dc.identifier.citationGuilarte M, Sala-Cunill A, Baeza ML, Cabañas R, Hernández MD, Ibañez E, et al. Hereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in Spain. Allergy, Asthma Clin Immunol. 2021 Dec 29;17:137.
dc.identifier.issn1710-1492
dc.identifier.urihttp://hdl.handle.net/11351/7700
dc.descriptionBradykinin; Hereditary angioedema; Icatibant
dc.description.abstractBackground The Icatibant Outcome Survey (IOS) is an international registry monitoring the use of icatibant, a bradykinin B2 receptor antagonist indicated for the acute treatment of hereditary angioedema (HAE) attacks. Our goal was to assess disease characteristics and icatibant treatment outcomes in patients with HAE due to C1 inhibitor deficiency (HAE type 1 or 2 (HAE-1/2)) from Spain relative to other countries participating in IOS. Methods Descriptive retrospective analyses of data are reported from 10 centers in Spain vs 51 centers in 12 other participating countries (July 2009 to January 2019). Results No meaningful differences were identified between patients in Spain (n = 119) and patients across other countries (n = 907) regarding median age at symptom onset (15.0 vs 12.0 years) or diagnosis (22.3 vs 20.5 years). Overall HAE attack rates (total attacks/total years of follow-up) were 2.66 in Spain and 1.46 across other countries. Patients in Spain reported fewer severe/very severe HAE attacks before treatment (41.0% vs 45.9%; P < 0.0001) and, for icatibant-treated attacks, longer median time to treatment (2.9 vs 1.0 h), time to attack resolution (18.0 vs 5.5 h), and total attack duration (24.6 vs 8.0 h). Use of androgens for long-term prophylaxis was higher in Spain (51.2% vs 26.7%). Conclusion Patients with HAE-1/2 in Spain reported fewer severe/very severe attacks, administered icatibant later, and had longer-lasting attacks than did patients across other countries in IOS. These differences may indicate varying disease management practices (e.g., delayed icatibant treatment) and reporting. Efforts to raise awareness on the benefits of early on-demand treatment may be warranted.
dc.language.isoeng
dc.publisherBMC
dc.relation.ispartofseriesAllergy, Asthma & Clinical Immunology;17
dc.rightsAttribution 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.sourceScientia
dc.subjectMalalties congènites - Tractament
dc.subjectInflamació
dc.subjectProteïna C - Deficiència
dc.subject.meshAngioedemas, Hereditary
dc.subject.mesh/drug therapy
dc.subject.meshSurveys and Questionnaires
dc.subject.meshTime-to-Treatment
dc.titleHereditary angioedema due to C1 inhibitor deficiency: real-world experience from the Icatibant Outcome Survey in Spain
dc.typeinfo:eu-repo/semantics/article
dc.identifier.doi10.1186/s13223-021-00641-3
dc.subject.decsangioedemas hereditarios
dc.subject.decs/farmacoterapia
dc.subject.decsencuestas y cuestionarios
dc.subject.decstiempo hasta el tratamiento
dc.relation.publishversionhttps://doi.org/10.1186/s13223-021-00641-3
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.audienceProfessionals
dc.contributor.organismesInstitut Català de la Salut
dc.contributor.authoraffiliation[Guilarte M, Sala-Cunill A] Secció d’Al•lèrgia, Servei de Medicina, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Spanish Research Network on Allergy (National Allergy Network ARADyAL: Asma, Reacciones Adversas y Alérgicas), Instituto de Salud Carlos III, Madrid, Spain. Vall d’Hebron Institut de Recerca (VHIR), Barcelona, Spain. Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Baeza ML] Allergy Department, Hospital General Universitario Gregorio Marañón, Madrid, Spain. Biomedical Research Network on Rare Diseases, CIBERER-U761, Gregorio Marañón Health Research Institute, Madrid, Spain. [Cabañas R] Allergy Department, Hospital Universitario La Paz, Hospital La Paz Institute for Health Research (IdiPaz), Madrid, Spain. Biomedical Research Network on Rare Diseases, CIBERER-U754, Madrid, Spain. [Hernández MD, Ibañez E] Allergy Department, IIS-Hospital Universitari I Politècnic La Fe, Valencia, Spain
dc.identifier.pmid34965883
dc.identifier.wos000736714300001
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


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