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HomePediatrics: General MedicinePrecocious Pseudopuberty

Precocious Pseudopuberty

Practice Essentials

Puberty is the process of physical maturation manifested by an increase in growth rate and the appearance of secondary sexual characteristics. The Lawson Wilkins Pediatric Endocrine Society (LWPES) guidelines define precocious puberty as the appearance of any sign of secondary sexual maturation in boys younger than 9 years, in white girls younger than 7 years, and in black girls younger than 6 years.
The American Academy of Pediatrics (AAP) recommends referral for endocrine evaluation of most girls who have breast development (confirmed by palpation) before 8 years.
 Precocious puberty can be divided into 2 distinct categories. The first category, referred to as central precocious puberty (CPP), is gonadotropin-dependent and involves the premature activation of the hypothalamic-pituitary-gonadal (HPG) axis. The second category, referred to as precocious pseudopuberty or peripheral precocious puberty (PPP), is gonadotropin-independent, and the presence of sex steroids is independent of pituitary gonadotropin release.

The etiology of PPP can be categorized into either genetic or acquired disorders, and some vary by gender. Congenital or genetic causes include McCune-Albright syndrome (MAS), familial male-limited precocious puberty (FMPP), and congenital adrenal hyperplasia (CAH). Acquired causes include tumors that secrete human chorionic gonadotropin (HCG); sex steroid–excreting tumors of the adrenal gland, ovary, or testis; or exposure to exogenous sex hormones.
 

The diagnosis is made with the help of a careful history and physical examination in conjunction with the use of radiologic and laboratory evaluations.

Medical and surgical therapies are directed at treatment of the underlying cause of precocious puberty. The therapies are designed to minimize both short-term and the long-term morbidity. The initial evaluation can usually be performed on an outpatient basis. However, inpatient studies and surgical treatment may be required. 

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