Review of precocious puberty part II: Gonadotropin-independent precocious puberty

The majority of cases of early pubertal maturation are due to premature activation of the hypothalamic-pituitary-gonadal axis. However, it is important to identify the small subset of individuals in whom the process is independent of gonadotropin secretion becuase the causes of the disorder, treatment options, and long-term implications are very different in this group of patients. After ruling out a gonadal or adrenal neoplasm and exogenous hormone exposure as causative, diagnostic possibilities include McCune Albright syndrome and familial male precocious puberty, so-called "testotoxicosis". As the process is independent of GnRH secretion, the use of GnRH analogues is not effective. All therapeutic apr preaches for these disorders involve the use of drugs that inhibit sex steroid synthesis and/or action. In some cases reassurance and monitoring may be sufficient or even preferable because none of the currently available or recommended treatment paradigms is without potential complications.