Lack of dehydroepiandrosterone in type I and II hereditary
angioedema and role of danazol in steroid hormone conversion

J 2007

Lack of dehydroepiandrosterone in type I and II hereditary angioedema and role of danazol in steroid hormone conversion

THON, Vojtěch, P. HARLE, J. SCHOLMERICH, P. KUKLINEK, Jindřich LOKAJ et. al.

Basic information

Original name

Lack of dehydroepiandrosterone in type I and II hereditary angioedema and role of danazol in steroid hormone conversion

Name in Czech

Nedostatek dehydroepiandosteronu u pacientů s hereditárním angioedémem a úloha danazolu v konverzi steroidních hormonů

Authors

THON, Vojtěch (203 Czech Republic, guarantor, belonging to the institution), P. HARLE (276 Germany), J. SCHOLMERICH (276 Germany), P. KUKLINEK (203 Czech Republic), Jindřich LOKAJ (203 Czech Republic, belonging to the institution) and RH STRAUB (276 Germany)

Edition

Allergy, 2007, 0105-4538

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30102 Immunology

Country of publisher

United Kingdom of Great Britain and Northern Ireland

Confidentiality degree

není předmětem státního či obchodního tajemství

Impact factor

Impact factor: 5.014

RIV identification code

RIV/00216224:14110/07:00058823

Organization unit

Faculty of Medicine

UT WoS

000249953300014

Keywords (in Czech)

C1 inhibitor; angioedém; dehydroepiandosteron; danazol

Keywords in English

C1 INHIBITOR; C1-INHIBITOR DEFICIENCY; MONONUCLEAR CELLS; BETA ENDORPHIN; EXPRESSION; WOMEN;

Abstract

ORIG CZ

V originále

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.

In Czech

Nedostatek dehydroepiandosteronu u pacientů s hereditárním angioedémem a úloha danazolu v konverzi steroidních hormonů.

Citovat

THON, Vojtěch, P. HARLE, J. SCHOLMERICH, P. KUKLINEK, Jindřich LOKAJ and RH STRAUB. Lack of dehydroepiandrosterone in type I and II hereditary angioedema and role of danazol in steroid hormone conversion. Allergy. 2007, vol. 62, No 11, p. 1320-1325. ISSN 0105-4538.

@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 and RH STRAUB. Lack of dehydroepiandrosterone in type I and II hereditary angioedema and role of danazol in steroid hormone conversion. \textit{Allergy}. 2007, vol.~62, No~11, p.~1320-1325. ISSN~0105-4538.

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