Pubertal maturation of the lh stimulatory response to clomiphene citrate in congenital virilizing adrenal hyperplasia.
To determine the LH release response to clomiphene citrate in children with virilizing congenital adrenal hyperplasia (CAH), we examined six patients with glucocorticoid-treated virilizing CAH, five girls and one boy, chonological age (CA) 4 years 2 months-15 years 9 months and bone age (BA) range 8-18 years. All the patients but one, were treated with nocturnal dexamethasone. Their ages at the onset of treatment ranged from 2 years 6 months to 13 years 8 months. An additional boy (CA = 4 years 3 months and BA = 7-8 years) was studied 10 days after removal of a virilizing adrenal adenoma. Clomiphene citrate (3mg/kg/day) was administered orally in two doses/day for 8 days. Venous blood was collected on days 0, 4, 6 and 8. Two patients were also studied in consecutive years. In three patients no increase was noted over the basal levels of LH, but in the others LH rose during clomiphene administration. In two patients studied in consecutive years, there was detectable shift of LH release pattern. The basal LH levels and the sum of responses (day 4, 6 and 8) to clomiphene citrate and particularly their decimal logarithms were statistically significantly correlated with their respective BA. These results confirm that previous prolonged exposure to elevated concentrations of adrenal sex steroids, in children with treated virilizing CAH, may induce precocious maturation of the hypothalamic pubertal mechanism of LH secretion. This maturation was closely correlated with the degree of skeletal maturation.
D000312:Adrenal Hyperplasia, Congenital / Q000097:blood* / Q000188:drug therapy; D000365:Age Determination by Skeleton; D001846:Bone Development / Q000187:drug effects; D002648:Child; D002675:Child, Preschool; D002996:Clomiphene / Q000031:analogs & derivatives* / Q000494:pharmacology; D003907:Dexamethasone / Q000627:therapeutic use; D006801:Humans; D007986:Luteinizing Hormone / Q000097:blood*; D011627:Puberty
D002996:Clomiphene; D003907:Dexamethasone; D007986:Luteinizing Hormone