Congenital adrenal hypoplasia: two new cases.
Two male adolescents with the X-linked form of congenital adrenal hypoplasia are described. Both grew slowly during childhood and adolescence and did not undergo pubertal development because of hypogonadotropic hypogonadism associated with the congenital adrenal hypoplasia. The severely delayed bone age in childhood is probably due to the adrenal androgen deficiency and suggests a role of these hormones in the prepubertal skeletal maturation. The failure of gonadotropin secretion still remains unexplained. A hypothalamic defect has been suggested, but further studies are necessary to clarify this hypothesis.
D000293:Adolescent; D000309:Adrenal Insufficiency / Q000151:congenital* / Q000188:drug therapy; D003900:Desoxycorticosterone / Q000627:therapeutic use; D006128:Growth / Q000187:drug effects; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D007006:Hypogonadism / Q000151:congenital; D008297:Male; D011627:Puberty
D003900:Desoxycorticosterone; D006854:Hydrocortisone