Clinical Characteristics and Management of Angioedema Attacks in Polish Adult Patients with Hereditary Angioedema Due to C1-Inhibitor Deficiency.
C1-inhibitor deficiency
bradykinin-mediated angioedema
emergency
hereditary angioedema
treatment
Journal
Journal of clinical medicine
ISSN: 2077-0383
Titre abrégé: J Clin Med
Pays: Switzerland
ID NLM: 101606588
Informations de publication
Date de publication:
29 Nov 2021
29 Nov 2021
Historique:
received:
31
10
2021
revised:
24
11
2021
accepted:
26
11
2021
entrez:
10
12
2021
pubmed:
11
12
2021
medline:
11
12
2021
Statut:
epublish
Résumé
Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is a rare disease characterized by recurrent swellings. This study aims to determine (i) the clinical characteristics of the HAE patient population from Poland, and (ii) real-life patients' treatment practices. A cross-sectional study involved 138 adult HAE patients (88 females, 50 males) treated in six regional HAE centers in Poland. Consecutive patients during routine follow-up visits underwent a structured medical interview on the clinical characteristics of the course and treatment of HAE attacks within the last six months. A total of 118 of 138 patients was symptomatic. They reported in total 2835 HAE attacks predominantly peripheral and abdominal, treated with plasma-derived C1-INH (61.4%), icatibant (36.7%) and recombinant C1-INH (1.9%). An amount of 116 patients carried the rescue medication with them while traveling, and 74 patients self-administrated on demand treatment. There were twice as many symptomatic women (
Identifiants
pubmed: 34884311
pii: jcm10235609
doi: 10.3390/jcm10235609
pmc: PMC8658320
pii:
doi:
Types de publication
Journal Article
Langues
eng
Subventions
Organisme : Jagiellonian University
ID : N41/DBS/000500
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