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Serum and urinary steroids in girls with precocious pubarche and/or hirsutism due to mild 3-beta-hydroxysteroid dehydrogenase deficiency.
Sólyom, J; Halász, Z; Hosszú, E; Gláz, E; Vihko, R; Orava, M; Homoki, J; Wudy, S A; Teller, W M.
Afiliación
  • Sólyom J; 2nd Department of Pediatrics, Semmelweis University Medical School, Budapest, Hungary.
Horm Res ; 44(3): 133-41, 1995.
Article en En | MEDLINE | ID: mdl-7590644
ABSTRACT
To obtain data on the correlation of serum and urinary steroids in nonclassical 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) deficiency, 9 girls with precocious pubarche and 33 adolescent girls with mild to severe hirsutism were studied. Urinary steroid profiles were analyzed by capillary gas chromatography. Serum 17-OH-pregnenolone (17-OHPreg) and 17-OH-progesterone (17-OHP) were determined by RIA after column-chromatographic separation. One out of 9 girls with precocious pubarche and 4/33 girls with hirsutism had elevated ratios of 17-OHPreg to 17-OHP after ACTH stimulation in serum and elevated urinary excretion of 5-ene steroids under basal conditions. These patients were defined to have decreased adrenal 3 beta-HSD activity. Basal and ACTH-stimulated serum 17-OHPreg levels in patients with mild 3 beta-HSD deficiency overlapped those of healthy controls and peripubertally virilized female patients without enzyme deficiency. Post-ACTH 17-OHPreg/17-OHP ratios in serum discriminated patients with and without 3 beta-HSD deficiency using a cutoff value of 13 instead of mean + 2 SD for age-related control values (6.7 and 11.6 for girls with Tanner stage II-III and IV-V, respectively). Sums of urinary 5-ene steroids in patients with 3 beta-HSD deficiency overlapped those in patients without enzyme deficiency. Results showed that an abnormal post-ACTH serum 17-OHPreg/17-OHP ratio may not be associated with elevated urinary 5-ene steroid excretion, and vica versa. In conclusion, patients with simultaneous elevation of post-ACTH serum 17-OHPreg/17-OHP ratio and basal urinary 5-ene steroid excretion are supposed to have mild 3 beta-HSD deficiency.
Asunto(s)
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Pubertad Precoz / Esteroides / Hirsutismo / 3-Hidroxiesteroide Deshidrogenasas Límite: Adolescent / Child / Female / Humans Idioma: En Revista: Horm Res Año: 1995 Tipo del documento: Article País de afiliación: Hungria
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Pubertad Precoz / Esteroides / Hirsutismo / 3-Hidroxiesteroide Deshidrogenasas Límite: Adolescent / Child / Female / Humans Idioma: En Revista: Horm Res Año: 1995 Tipo del documento: Article País de afiliación: Hungria