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Adrenal Steroidogenic Function in a Black and Hispanic Population with Precocious Pubarche

Department of Pediatrics, St. Luke's-Roosevelt Hospital Center New York, New York 10025
Columbia University College of Physicians and Surgeons New York, New York 10032
Endocrine Sciences Tarzana, California

Address requests for reprints to: Dr. Sharon E. Oberfield, St. Lukes-Roosevelt Hospital Center, New York, New York 10025

Adrenal steroidogenic function was evaluated in 34 children with precocious pubarche (PP; onset of pubic hair, <8 yr in girls and <9 yr in boys). The adrenal steroid response to an iv bolus of ACTH-(l–24) in the patients (aged 9 months to 9 yr) was compared to that in 16 normal controls (prepubertal, n = 9; Tanner stage II pubic hair, n = 7). The patient population consisted of 20 Hispanics (17 from the Dominican Republic), 13 black Americans, and 1 black Haitian. All patients had normal stimulated levels of 17-hydroxyprogesterone (17-OHP), 11-deoxycortisol (compound S), and desoxycorticosterone, thereby ruling out 21-hydroxylase deficiency and 11β-hydroxylase deficiency, respectively. To evaluate for the presence of 3β-hydroxysteroid dehydrogenase (3β-HSD) deficiency, the patients were classified on the basis of their 60-min ⁵-17-hydroxypregnenolone/17-OHP (⁵-17P/17-OHP) ratio [PPl (n = 13), 2 SD of Tanner I controls; PP2 (n = 17), >2 SD above Tanner I controls and 2 SD Tanner II controls; and PP3 (n = 4), >2 SD above Tanner II controls; 2.1 ± 1.0, 6.1 ± 1.7, and 16.1 ± 3.3 for PPl, PP2, and PP3, respectively. ⁵-17P/17-OHP for PPl vs. PP2, PP2 vs. PP3, and PPl vs. PP3 were significantly different (P < 0.05) by analysis of variance and multiple comparison testing using the Student-Newman-Keuls procedure. The four patients in PP3 were considered to have a possible nonclassical 3β-HSD deficiency. This diagnosis was supported by the fact that these patients had the greatest increment in ⁵-17P and dehydroepiandrosterone (DHEA) levels as well as the highest stimulated ⁵-17P/cortisol (⁵-17P/F) ratio among the patient groups.

In contrast to the ACTH-stimulated androgens there were no differences in the baseline ⁵-17P/170HP or androgens among the patient groups. Additionally, the 60-min ⁵-17P/17-OHP within the patient groups was highly correlated with the 60 minvalues for ⁵-17P, DHEA, DHEA/⁴-androstendione, and ⁵-17P/F. In the children with PP the mean bone age/chronological age (BA/CA) was 1.27 ± .27, the mean BA/height age (BA/HA) was 1.09 ± 0.25, and the mean HA/CA was 1.18 ± 0.17. No differences were noted between the patient population groups in mean BA/CA, mean BA/HA, or mean HA/CA.

Thus, in a group of 34 black and Hispanic children with PP, 4 (12%) had a diagnosis consistent with 3β-HSD deficiency, none had 21-hydroxylase or 11β-hydroxylase deficiency, and 30 (88%) had no evidence of a defect in adrenal steroidogenesis (idiopathic). Further, the 4 patients with nonclassical 3β-HSD deficiency could not be distinguished by clinical parameters from those with idiopathic PP.

Received May 11, 1989.

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