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[Hereditary angioedema by defict of C1 esterase. Our experience in 8 cases]
Insights
Hereditary angioedema patients with low C1-inhibitor levels experienced digestive issues and swelling. Antifibrinolytic drugs like tranexamic acid showed excellent results with fewer side effects for managing these angioedema attacks.
Area of Science:
- Immunology
- Genetics
Context:
- Hereditary angioedema (HAE) is a rare genetic disorder.
- Characterized by recurrent episodes of swelling.
- Often associated with deficiencies in C1-esterase inhibitor.
Purpose:
- To analyze hereditary angioedema cases with low C1-esterase inhibitor.
- To investigate clinical manifestations and treatment outcomes.
Summary:
- Eight HAE patients with low C1-inhibitor were studied.
- Low C4 complement component was observed.
- Triggers included trauma and emotional stress.
- Digestive symptoms and pharyngolaryngeal edema were noted.
- Antifibrinolytic agents (Epsilon aminocaproic acid, tranexamic acid) were effective.
- Tranexamic acid had fewer side effects than Epsilon aminocaproic acid.
Impact:
- Highlights the clinical spectrum of HAE.
- Demonstrates the efficacy of antifibrinolytic therapy.
- Suggests tranexamic acid as a preferred treatment option due to safety profile.
Abstract:
Eight cases of hereditary angioedema, all of them with low values of C1-sterase inhibitor are analyzed. In 7 cases the C3 and C4 components of the complement were assessed; the results showed marked descent of C4. The 8 patients came from 4 different families; only 2 of them were males. Six patients presented digestive disorders, reporting colic pain, nausea and vomiting. In 1 of them the abdominal picture was the only evidence of the disease. In 5 patients the angioedema episodes occurred following traumatisms and in 3 because of emotional states. The duration of the attacks varied from several hours to six days. There was a familial history in all cases. Three of the patients had repeated episodes of pharyngolaryngeal angioedema, two of them requiring emergency tracheotomy because of suffocating crisis. Six patients were treated with Epsilon aminocaproic acid (16 to 20 gr daily) or with tranexamic acid (1 to 3 gr. daily). In 4 cases the results were excellent with either of these antifibrinolytic drugs. No side effects were observed in the tranexamic acid therapy whilst they were frequent in the treatment with Epsilon aminocaproic acid.