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Clinical Characteristics and Management of Angioedema Attacks in Polish Adult Patients with Hereditary Angioedema Due to C1-Inhibitor Deficiency
- Source :
- Journal of Clinical Medicine; Volume 10; Issue 23; Pages: 5609, Journal of Clinical Medicine, Vol 10, Iss 5609, p 5609 (2021), Journal of Clinical Medicine
- Publication Year :
- 2021
- Publisher :
- Multidisciplinary Digital Publishing Institute, 2021.
-
Abstract
- 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 (n = 78) as there were men (n = 40). Women treated their HAE attacks significantly more often than men. Older patients (≥65 years) reported a longer delay in diagnosis, and practiced the self-administration of rescue medication less frequently in comparison to other patients. Clinical features of the surveyed population are similar to other European, but not Asian, HAE patient groups. Self-administration still remains an unmet medical need. Some distinct HAE patients may require special attention due to the severe course of the disease (females) or a delay in diagnosis (the elderly).
- Subjects :
- Pediatrics
medicine.medical_specialty
C1 inhibitor deficiency
bradykinin-mediated angioedema
Population
Disease
Article
chemistry.chemical_compound
Icatibant
hereditary angioedema
C1-inhibitor deficiency
treatment
emergency
medicine
education
education.field_of_study
Angioedema
Adult patients
business.industry
General Medicine
medicine.disease
chemistry
Hereditary angioedema
Medicine
medicine.symptom
business
Rare disease
Subjects
Details
- Language :
- English
- ISSN :
- 20770383
- Database :
- OpenAIRE
- Journal :
- Journal of Clinical Medicine; Volume 10; Issue 23; Pages: 5609
- Accession number :
- edsair.doi.dedup.....0c3e340cac972a780683d4b8f0e7df3c
- Full Text :
- https://doi.org/10.3390/jcm10235609