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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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