2025 – PAGE 62 – ADOLESCENT MEDICINE
GONADOTROPIN-INDEPENDENT PRECOCIOUS PUBERTY
In gonadotropin-independent precocious puberty (AKA peripheral precocious pseudopuberty or pseudoprecocious puberty), there is some sex-steroid production going on in the body but not because of pituitary hormone production. The hormones usually come from the adrenals, ovaries or testes. If gonadotropes (LH and FSH) levels are normal and everything in the history sounds like it’s on a proper timeline except for one abnormality, such as breast development followed by the onset of menstruation but without pubic hair, then consider this category of precocious puberty. Specific causes of non-pituitary related precocious puberty include tumors, congenital adrenal hyperplasia, McCune-Albright syndrome and Leydig cell hyperplasia (boys).
PRECOCIOUS PUBERTY IN GIRLS
In girls, precocious puberty is defined as having breasts + vaginal bleeding OR accelerated growth. Get LH, FSH, estrogen, and progesterone. This condition is often idiopathic when brain hormones have started early (elevated LH and FSH). If there are elevated gonadal hormones but low brain hormones (meaning a gonadotropin-independent precocious puberty), this could be BAD (tumor). Look for ovarian tumors with a pelvic ultrasound. You may consider getting an MRI to look for a pituitary tumor if neurologic signs are present and/or central hormones are elevated. Treat CENTRAL PRECOCIOUS PUBERTY with a GnRH Analogue (gonadoTROPIN releasing hormone analogue) called leuprolide. It’s counterintuitive, but it eventually results in the suppression of LH and FSH release and therefore results in suppression of ovarian (or testicular) steroidogenesis.
PRECOCIOUS PUBERTY IN BOYS
In boys, precocious puberty can be caused by elevated LH alone causing elevated gonadal androgens. HCG can act on the same LH receptors; therefore, an HCG-SECRETING TUMOR can also cause it. Look for increased testicular size/volume.
PEARL/SHORTCUT: In order to choose an answer that has “PUBERTY” in it (CENTRAL PRECOCIOUS PUBERTY or TRUE PUBERTY), there must be evidence of testicular enlargement (>4 ml/2.5 cm). So, if the testes are small (<4 ml/2.5 cm), but there is evidence of extra hair, penile enlargement, and/or a growth spurt, there is a non-central and non-gonadal problem → think late onset Congenital Adrenal Hyperplasia or a VIRILIZING TUMOR or EXOGENOUS STEROIDS!
ADRENAL ANDROGENS
Adrenal androgens cause body odor, acne, and hair development. Etiology of ACNE → androgens. The term adrenarche = hair.
PREMATURE ADRENARCHE
Premature adrenarche is common in girls. Parents bring them to the office quickly because they are concerned about their hairy/mannish princess. It’s usually not a big deal. In boys it’s VERY concerning, but boys are unfortunately NOT brought to the office often enough because parents think boys are supposed to be hairy! It’s serious in boys because it can be due to CONGENITAL ADRENAL HYPERPLASIA (CAH).
PEARL: If workup suggests an adrenal source, choose CAH over adrenal tumor as your answer.
CONGENITAL ADRENAL HYPERPLASIA (CAH) INTRO
In congenital adrenal hyperplasia (CAH), there is a cortisol and aldosterone manufacturing problem in the adrenal glands. Absence of negative feedback (due to low cortisol levels) results in high levels of ACTH being released from the pituitary glands → Results in an increase in cortisol precursors → Resulting in more ANDROGENS. It is diagnosed by measuring 17-hydroxyprogesterone and noting HIGH levels. More details in ENDOCRINOLOGY under Congenital Adrenal Hyperplasia.