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@article{837042,
author = {Thon, Vojtěch and Harle, P. and Scholmerich, J. and Kuklinek, P. and Lokaj, Jindřich and Straub, RH},
article_number = {11},
keywords = {C1 INHIBITOR; C1-INHIBITOR DEFICIENCY; MONONUCLEAR CELLS; BETA ENDORPHIN; EXPRESSION; WOMEN;},
language = {eng},
issn = {0105-4538},
journal = {Allergy},
title = {Lack of dehydroepiandrosterone in type I and II hereditary angioedema and role of danazol in steroid hormone conversion},
volume = {62},
year = {2007}
}
TY - JOUR
ID - 837042
AU - Thon, Vojtěch - Harle, P. - Scholmerich, J. - Kuklinek, P. - Lokaj, Jindřich - Straub, RH
PY - 2007
TI - Lack of dehydroepiandrosterone in type I and II hereditary angioedema and role of danazol in steroid hormone conversion
JF - Allergy
VL - 62
IS - 11
SP - 1320-1325
EP - 1320-1325
SN - 01054538
KW - C1 INHIBITOR
KW - C1-INHIBITOR DEFICIENCY
KW - MONONUCLEAR CELLS
KW - BETA ENDORPHIN
KW - EXPRESSION
KW - WOMEN;
N2 - Background: Hereditary angioedema (HAE) is successfully treated with danazol, a therapeutic steroid compound. To investigate hormones of the hypothalamic-pituitary-adrenal (HPA) and hypothalamic-pituitary-gonadal (HPG) axis in patients with HAE with and without danazol. Methods: We included 16 patients with type I HAE, nine patients with type II HAE, and 16 healthy subjects. Serum levels of adrenocorticotropic hormone (ACTH), cortisol, androstenedione, dehydroepiandrosterone (DHEA), free testosterone, and 17 beta-oestradiol were measured. Results: Serum levels of ACTH were markedly decreased in patients with type II HAE compared to the other groups (P < 0.001). Serum cortisol was similar between groups but danazol treatment decreased cortisol levels, particularly in women (P = 0.019). Serum levels of DHEA were significantly decreased in all patients with type I and II HAE compared to controls (P < 0.05), which was only partly dependent on prior danazol therapy as patients without danazol had also decreased serum levels of DHEA (P < 0.05). Furthermore, free testosterone serum levels were markedly increased in patients under danazol (P < 0.005) and the ratio of 17 beta-oestradiol/free testosterone was significantly decreased in these patients (P < 0.005). Conclusions: This study demonstrated decreased DHEA in patients with type I and II HAE independent of danazol therapy, which was particularly evident in women. It also demonstrates that danazol induced a marked up-regulation of free testosterone in relation to precursors and downstream 17 beta-oestradiol. In HAE, there seems to be a primary lack of the adrenal androgen DHEA.
ER -
THON, Vojtěch, P. HARLE, J. SCHOLMERICH, P. KUKLINEK, Jindřich LOKAJ a RH STRAUB. Lack of dehydroepiandrosterone in type I and II hereditary angioedema and role of danazol in steroid hormone conversion. \textit{Allergy}. 2007, roč.~62, č.~11, s.~1320-1325. ISSN~0105-4538.
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