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 (203 Česká republika, garant, domácí), P. HARLE (276 Německo), J. SCHOLMERICH (276 Německo), P. KUKLINEK (203 Česká republika), Jindřich LOKAJ (203 Česká republika, domácí) a RH STRAUB (276 Německo)

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.