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.

Základní údaje

Originální název

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

Název česky

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

Autoři

THON, Vojtěch; P. HARLE; J. SCHOLMERICH; P. KUKLINEK; Jindřich LOKAJ a RH STRAUB

Vydání

Allergy, 2007, 0105-4538

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30102 Immunology

Stát vydavatele

Velká Británie a Severní Irsko

Utajení

není předmětem státního či obchodního tajemství

Impakt faktor

Impact factor: 5.014

Kód RIV

RIV/00216224:14110/07:00058823

Organizační jednotka

Lékařská fakulta

UT WoS

000249953300014

Klíčová slova česky

C1 inhibitor; angioedém; dehydroepiandosteron; danazol

Klíčová slova anglicky

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

Příznaky

Mezinárodní význam, Recenzováno
Změněno: 10. 4. 2013 12:30, prof. MUDr. Vojtěch Thon, Ph.D.

Anotace

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.

Česky

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