[Pseudo- and central precocious puberty due to adrenogenital syndrome].
We describe three patients in whom at the age of 4-7 yrs the diagnosis adrenogenital syndrome (21-hydroxylase deficiency) was made. There was no salt-loss. Two boys presented with precocious pubic hair development and increased growth velocity and bone maturation. The third patient was a severely virilized girl raised as a boy until she was 4 yrs old. Following an extensive psychiatric evaluation, it was decided to raise her as a girl and feminoplasty was performed. Clinical and biochemical evidence of central precocious puberty was present in one boy at the time of diagnosis at age 7 and developed under hydrocortisone substitution therapy in the two other patients. The precocious puberty was treated in two patients with the anti-androgen cyproterone acetate. In one boy suppression of pituitary gonadotropin secretion was obtained by LH-RH analogue (Buserelin) treatment.
D000312:Adrenal Hyperplasia, Congenital / Q000150:complications* / Q000188:drug therapy; D002064:Buserelin / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003534:Cyproterone / Q000031:analogs & derivatives / Q000627:therapeutic use; D017373:Cyproterone Acetate; D012734:Disorders of Sex Development / Q000209:etiology / Q000601:surgery; D005260:Female; D005835:Genitalia / Q000601:surgery; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D008297:Male; D011629:Puberty, Precocious / Q000188:drug therapy / Q000209:etiology* / Q000601:surgery
D017373:Cyproterone Acetate; D003534:Cyproterone; D002064:Buserelin; D006854:Hydrocortisone
Pseudo-en centrale pubertas praecox ten gevolge van het adrenogenitaal syndroom.