Abstract
Increased adrenal androgens are often observed in girls with CPP. The hyperandrogenism is not affected by the therapy with GnRh analog (GnRHA). We performed an i.v. ACTH stimulation test (0.25mg) in 42 girls with CPP (Tanner 2-3), before (29/42) and during (13/42) therapy with GnRHA. The stimulated levels of 17OH pregnenolone (17OHPreg), 17OH progesterone (17OHP) and the ratio 17OHPreg/17OHP were analyzed and compared to normal values for age and pubertal stage (Lashansky et al JCEM 73: 674, 1991). The results revealed 3 patterns of response: a) Enzymatic deficiency (D)-6/42 girls: non-classical (NC)21OHD - 6, NC3βHSDD - 2; b) Exaggerated “adrenarche” i.e. hyper-response to ACTH: 17OHPreg >24nmol/l and 17OHPreg/17OHP <7 - 19/42 girls; c) Normal response - 17/42 girls. Also an ACTH was performed in 12 CPP girls post GnRHA therapy in full puberty. On long term follow up 8/12 had developed early PCOS (ages: 14-16) and revealed abnormal responses: NC3βHSDD was diagnosed in 1 girl and exaggerated “adrenarche” response was found in 7. The remaining 4/12 without PCOS revealed a normal response. According to our data, associated hyperandrogenism is found in a significant number of girls with CPP (59.5% in this report) and remains sustained throughout puberty and thereafter. Hyperandrogenism can be the trigger for the onset of CPP in these patients and can explain the relatively high incidence of early PCOS in girls with CPP.
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Lazar, L., Kauli, R., Bruchis, S. et al. HYPERANDROGENISM AS A CAUSE OF EARLY POLYCYSTIC OVARY SYNDROME (PCOS) IN GIRLS WITH CENTRAL PRECOCIOUS PUBERTY (CPP). Pediatr Res 33 (Suppl 5), S14 (1993). https://doi.org/10.1203/00006450-199305001-00065
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DOI: https://doi.org/10.1203/00006450-199305001-00065