Central Precocious Puberty: From Diagnosis to Treatment
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 (
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Central Precocious Puberty: From Diagnosis to Treatment Juliane Léger and Jean-Claude Carel
3.1
Introduction
Precocious puberty (PP) is defined as the onset of clinical signs of puberty before the age of 8 years in girls and 9.5 years in boys. However, the onset of puberty may be subject to the effects of environmental (secular trends, adoption, absence of the father, and possible exposure to estrogenic endocrine-disrupting chemicals), nutritional (body mass index), and constitutional (genetics, ethnicity) factors [1–4], with implications for the definition of precocious puberty. PP may be caused by central or peripheral mechanisms [1]. Premature sexual maturation is a frequent cause for referral. Clinical evaluation is generally sufficient to reassure the patients and their families, but premature sexual maturation may reveal severe conditions and thorough evaluation is therefore required to identify its cause and potential for progression, so that appropriate treatment can be proposed. The clinical expression of precocious puberty is polymorphic. In addition to progressive central PP, with a progressive deterioration of adult height prognosis in the absence of treatment, there are very slowly progressive forms which do not modify predicted final height [5–7]. The heterogeneity of precocious puberty, in terms of its clinical presentation and definition, can be explained by the gradual nature of the transition to puberty. Indeed, the pulsatile secretion of LH begins before the onset of clinical signs of puberty, and an increase in the amplitude of the LH peaks is the key biological sign of pubertal maturation of the gonadotrophic pituitary axis. GnRH stimulation tests indirectly reveal pulsatile endogenous GnRH secretion, as this secretion determines the response
J. Léger, MD (*) • J.-C. Carel Department of Paediatric Endocrinology and Diabetology, INSERM UMR 1141, DHU PROTECT, Reference Center for Rare Endocrine Growth Diseases, Robert Debré Hospital, Denis Diderot Paris 7 University, 48 Bd Sérurier, F-75019 Paris, France e-mail: [email protected] © International Society of Gynecological Endocrinology 2017 C. Sultan, A.R. Genazzani (eds.), Frontiers in Gynecological Endocrinology, ISGE Series, DOI 10.1007/978-3-319-41433-1_3
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to exogenous GnRH. The available data indicate that there is no clear boundary between prepubertal and pubertal status, accounting for the frequency of “marginal” forms of precocious puberty.
3.2
tiologies and Mechanisms Underlying Premature E Sexual Development
Central precocious puberty (CPP), which is much more common in girls than in boys [8], results from premature reactivation of the hypothalamo-pituitary-gonadal axis and pulsatile GnRH secretion with a hormonal pattern similar to that of normal puberty. Premature sexual development results from the action of sex steroids or compounds with sex steroid activity on target organs. CPP may be due to hypothalamic lesions, but is idiopathic in most cases, particularly in girls (Table 3.1) [1]
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