The specific features of adrenal steroidogenesis in patients with congenital adrenal cortical dysfunction due to 21-hydroxylase deficiency
https://doi.org/10.14341/probl200551422-26
Abstract
Forty-two patients with classical forms of congenital adrenal cortical dysfunction (CACD) (22 patients with salt-loosing form and 20 patients with simple virile form) caused by 21-hydroxylase deficiency and 29 female with an obscure form of the disease were examined. The patients with classical CACD received therapy with gluco- and mineralocorticoids. Radioimmunoassay and enzyme immunoassay were employed to estimate the levels of 17-OH-progesterone and the activities of plasma renin and serum corticotropin, aldosterone, dehydroepiandrosterone, dehydroepiandrosterone sulfate, and androstenedione. High performance liquid chromatography (HPLC) was used to measure the blood levels ofcortisol, cortisone, corticosterone (B), 11-deoxycorticosterone (DOC), and 11-deoxycortisol (S). There were increases in the levels of В and DOC in patients with the simple form at the decompensation stage and in those of DOC in patients with the simple form at the compensation stage, which is indicative of activation of the renin-angiotensin system. There were also rises in В and DOC in 35.8 and 33.0% of the patients with obscure CACD, respectively, as well as a significant elevation of the content of S. A study of the profiles of corticosteroids by HPLC in patients with 21-hydroxylase deficiency-related CACD is an additional method for assessing the compensation of the disease in patients with classical CACD and for diagnosing the obscure form of the disease.
About the Authors
I. P. SerebryakovaSaint-Petersburg medical academy of postgraduate education
Russian Federation
L. I. Velikanova
Saint-Petersburg medical academy of postgraduate education
Russian Federation
N. V. Vorokhobina
Saint-Petersburg medical academy of postgraduate education
Russian Federation
P. A. Silnitskiy
Saint-Petersburg medical academy of postgraduate education
Russian Federation
E. G. Strelnikova
Saint-Petersburg medical academy of postgraduate education
Russian Federation
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Review
For citations:
Serebryakova I.P., Velikanova L.I., Vorokhobina N.V., Silnitskiy P.A., Strelnikova E.G. The specific features of adrenal steroidogenesis in patients with congenital adrenal cortical dysfunction due to 21-hydroxylase deficiency. Problems of Endocrinology. 2005;51(4):22-26. (In Russ.) https://doi.org/10.14341/probl200551422-26
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