Central Precocious Puberty: From Diagnosis to Treatment

Abstract

Central precocious puberty (CPP) results from premature reactivation of the gonadotropic axis. CPP is much more common in girls than in boys and is idiopathic in most cases. In boys, precocious puberty is more likely to be linked to hypothalamic lesions (≈40 %). Recent studies have implicated the inactivation of MKRN3 gene in “idiopathic” paternally transmitted familial CPP, but such defects do not underlie maternally transmitted CPP and are rarely involved in sporadic forms. Precocious puberty may have consequences for growth and psychosocial development. If a non-progressive form of PP is suspected, it is recommended to wait a few months and then to reassess the patient to avoid unnecessary treatment. Gonadotropin-releasing hormone agonists (GnRHa) are the standard treatment for progressive CPP. Such treatment results in the regression or stabilization of pubertal symptoms and decreases in growth velocity and bone age advancement and improves adult height. After the cessation of GnRHa therapy, generally at an age of about 11 years in girls and 12 years in boys, biological and clinical signs of puberty reappear within months, with most girls achieving menarche, with menstrual ovulation cycles, during the following year. PP associated with the presence of a hypothalamic lesion may progress to gonadotropin deficiency. The available data indicate that long-term GnRHa treatment does not seem to cause or aggravate obesity or have repercussions for body composition, bone mineral density, and fertility. However, data concerning psychosocial outcomes are scarce.