Precocious puberty of central or peripheral origin

Specialty: Pediatrics.

• Early sexual development
• advanced sexual maturation

Why it occurs

• Idiopathic central precocious puberty (premature activation of the hypothalamic-pituitary-gonadal axis of unknown cause, more common in girls)
• Central nervous system lesions (hypothalamic hamartomas, astrocytomas, ependymomas, sequelae of irradiation or head trauma that activate the GnRH pulse generator)
• Peripheral precocious puberty or pseudoprecocious puberty (autonomous production of sex steroids independent of gonadotropins by ovarian cysts, Leydig cell tumors, congenital adrenal hyperplasia or hCG-secreting tumors)
• Exogenous exposure to endocrine disruptors (accidental ingestion or application of creams or gels with estrogen or testosterone)

Initial workup

Analytical determination of basal gonadotropins: LH (luteinizing hormone) and FSH (follicle stimulating hormone), as well as estradiol in girls and testosterone in boys, preferably using ultrasensitive immunochemiluminescence methods. Stimulation test with GnRH or GnRH analogues (assessing the LH response to differentiate central vs. peripheral origin). Bone age by x-ray of the left hand and wrist. Pelvic ultrasound in girls to assess the size and morphology of the uterus and ovaries. High-resolution brain and sella turcica magnetic resonance imaging, mandatory in all boys with central precocious puberty and in girls with onset before age 6 or rapid progression.

Standard management

• Triptorelin Acetate or Leuprorelin Acetate — extended-release GnRH analogues of choice for central precocious puberty; They act by producing a desensitization and downregulation of pituitary GnRH receptors, stopping the secretion of gonadotropins and sex hormones; intramuscular or subcutaneous dosage every 4 or 12 weeks, adjusted by weight and hormonal response
• Ketoconazole or Testolactone — steroidogenesis inhibitors indicated in specific forms of peripheral precocious puberty such as testotoxicosis
• Spironolactone and Flutamide (androgen receptor antagonists used in combination in very specific therapeutic regimens).