|Ewa Nowicka, Ewa Najberg, Hanna Gregorek|
Introduction. Hereditary angioedema (HAE) is a rare disorder caused by an inherited deficiency of C1 inhibitor (C1-INH). Early diagnosis and treatment is very important because HAE can be a potentially life-threatening disease.
Aim of the study. The study objective was to demonstrate the authors’ own experience in the treatment of patients with hereditary angioedema resulting from C1 esterase inhibitor deficiency.
Material and methods. We analyzed 39 patients – 9-57 years old. The diagnosis of HAE was established by personal questionnaire, family history, clinical examination and complement measurements (C4 and C1 inhibitor concentration in serum, C1 inhibitor function).
Results. We introduced health education for patients and their families, and we prepared special identity card with important diagnostic and therapeutic information. The treatment of HAE patients was individualized based on attack frequency and the severity of oedemas. 8 patients received long-term prophylaxis with danazol, while 25 patients were treated periodically with EACA, tranexamic acid or danazol.
Conclusions. High doses of tranexamic acid or danazol were used in acute cutaneous and abdominal attacks. Serious laryngeal episodes were treated with C1-INH concentrate.
keywords: dziedziczny obrzęk naczynioruchowy, leczenie ostrych epizodów, profilaktyka, HAE, prophylaxis, treatment of acute oedemas
pages: from 200 to 209
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