Modified from Grumbach MM, Kaplan SL: Recent advances in the diagnosis and management of sexual precocity, Acta Paediatr Jpn 30(Suppl):155, 1988. FSH, Follicle-stimulating hormone; GnRH, gonadotropin-releasing hormone. *If true precocious puberty develops, a GnRH agonist can be added.
analog. Therapy is provided with testolactone and antiandrogens or antiestrogen, such as tamoxifen.
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Precocious Puberty in the Female
Benign premature thelarche is the isolated appearance of unilateral or bilateral breast tissue in girls, usually at ages 6 months to 3 years. There are no other signs of puberty and no evidence of excessive estrogen effect (vaginal bleeding, thickening of the vaginal secretions, increased height velocity, or bone age acceleration). Ingestion or dermal application of estrogen-containing compounds must be excluded. Laboratory investigations are not usually necessary, but a pelvic ultrasound study rarely may be indicated to exclude ovarian disease. Girls with this condition should be re-evaluated at intervals of 6 to 12 months to ensure that apparent premature thelarche is not the beginning of progression into precocious puberty. The prognosis is excellent; if no progression occurs, no treatment other than reassurance is necessary.
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In males breast tissue is termed gynecomastia, and it may occur to some degree in 45% to 75% of normal pubertal boys (see Chapter 67). Androgens normally are converted to estrogen by aromatization; in early puberty, only modest amounts of androgens are produced, and the estrogen effect can overwhelm the androgen effects at this stage. Later in pubertal development, the androgen production is so great that there is little effect from the estrogen produced by aromatization. Gynecomastia also can suggest the possibility of Klinefelter syndrome as puberty progresses. Prepubertal gynecomastia suggests an unusual source of estrogen either from exogenous sources (oral or dermal estrogen administration is possible from contamination of food or ointments) or from endogenous sources (from abnormal function of adrenal gland or ovary or from increased peripheral aromatization).
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Amenorrhea Precocious Puberty in the Male Precocious Puberty in the Female
The isolated appearance of pubic hair before age 6 to 7years in girls or before age 9 years in boys is termed premature pubarche, usually resulting from adrenarche. It is relatively common. If the pubic hair is associated with anyother feature of virilization (clitoral or penile enlargement oradvanced bone age) or other signs (acne, rapid growth, orvoice change), a detailed investigation for a pathologic causeof virilization is indicated. Measurements of serum testosterone, 17-hydroxyprogesterone, and DHEAS are indicated toinvestigate the possibility of CAH. Ultrasound studies mayreveal a hyperplastic adrenal gland or a virilizing adrenalor ovarian tumor. Most patients with isolated pubic hair donot have progressive virilization and simply have prematureadrenarche (pubarche) resulting from premature activationof DHEA secretion from the adrenal gland. The skeletal maturation, as assessed by bone age, may be slightly advancedand the height slightly increased, but testosterone concentrations are normal. DHEA levels usually are high for prepubertal age but are consistent with Tanner (sexuality maturityrating) stages II and III.