Clinical Characteristics and Management of Angioedema Attacks in Polish Adult Patients with Hereditary Angioedema Due to C1-Inhibitor Deficiency

Autor:	Robert Pawłowicz, Aldona Juchacz, Katarzyna Piotrowicz-Wójcik, Jacek Gocki, Joanna Jamróz-Brzeska, Krzysztof Kuziemski, Małgorzata Bulanda, Grzegorz Porebski
Jazyk:	angličtina
Rok vydání:	2021
Předmět:	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
Zdroj:	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
ISSN:	2077-0383
DOI:	10.3390/jcm10235609
Popis:	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).
Databáze:	OpenAIRE
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