Hereditary angioneurotic edema (HAE) is a complement-related clinical disorder with a deficiency of the C1 esterase inhibitor protein. Eight patients with severe attacks of the disease were treated with the adrenal “androgen” dehydroepiandrosterone sulphate (DS). Steroid therapy for 3–28 months resulted in dramatic improvement in their clinical state and a moderate increase in the serum concentration of C1 inhibitor. There was a significant increase in the serum level of either unconjugated dehydroepiandrosterone (D) or of DS during treatment.
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Koó, E., Fehér, K.G., Fehér, T. et al. Effect of dehydroepiandrosterone on hereditary angioedema. Klin Wochenschr 61, 715–717 (1983). https://doi.org/10.1007/BF01487618
- Hereditary angioedema
- C1 esterase inhibitor deficiency
- Dehydroepiandrosterone therapy