Adrenal steroidogenic function in a black and Hispanic population with precocious pubarche.
Adrenal steroidogenic function was evaluated in 34 children with precocious pubarche (PP; onset of pubic hair, less than 8 yr in girls and less than 9 yr in boys). The adrenal steroid response to an iv bolus of ACTH-(1-24) in the patients (aged 9 months to 9 7/12 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 11 beta-hydroxylase deficiency, respectively. To evaluate for the presence of 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD) deficiency, the patients were classified on the basis of their 60-min delta 5-17-hydroxypregnenolone/17-OHP (delta 5-17P/17-OHP) ratio [PP1 (n = 13), less than or equal to 2 SD of Tanner I controls; PP2 (n = 17), greater than 2 SD above Tanner I controls and less than or equal to 2 SD Tanner II controls; and PP3 (n = 4), greater than 2 SD above Tanner II controls; 2.1 +/- 1.0, 6.1 +/- 1.7, and 16.1 +/- 3.3 for PP1, PP2, and PP3, respectively. delta 5-17P/17-OHP for PP1 vs. PP2, PP2 vs. PP3, and PP1 vs. PP3 were significantly different (P less than 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 3 beta-HSD deficiency. This diagnosis was supported by the fact that these patients had the greatest increment in delta 5-17P and dehydroepiandrosterone (DHEA) levels as well as the highest stimulated delta 5-17P/cortisol (delta 5-17P/F) ratio among the patient groups. In contrast to the ACTH-stimulated androgens there were no differences in the baseline delta 5-17P/170HP or androgens among the patient groups. Additionally, the 60-min delta 5-17P/17-OHP within the patient groups was highly correlated with the 60 min-values for delta 5-17P, DHEA, DHEA/delta 4-androstendione, and delta 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.(ABSTRACT TRUNCATED AT 400 WORDS)
Oberfield
S E
SE
Department of Pediatrics, St. Luke's-Roosevelt Hospital Center, New York, New York 10025.
Mayes
D M
DM
Levine
L S
LS
D015096:3-Hydroxysteroid Dehydrogenases / Q000172:deficiency; D000311:Adrenal Glands / Q000187:drug effects / Q000201:enzymology / Q000378:metabolism*; D000324:Adrenocorticotropic Hormone; D000728:Androgens / Q000097:blood; D044383:Black People; D002648:Child; D002675:Child, Preschool; D005260:Female; D006197:Hair / Q000254:growth & development; D006630:Hispanic or Latino; D006801:Humans; D006889:Hydroxycorticosteroids / Q000097:blood; D007223:Infant; D008297:Male; D011372:Progestins / Q000097:blood; D011629:Puberty, Precocious / Q000201:enzymology / Q000378:metabolism*; D013256:Steroids / Q000097:blood*; D001741:Black or African American
D000728:Androgens; D006889:Hydroxycorticosteroids; D011372:Progestins; D013256:Steroids; D000324:Adrenocorticotropic Hormone; D015096:3-Hydroxysteroid Dehydrogenases