Precocious Puberty: Clinical Management
Precocious puberty has been a focus of interest for both the pediatric endocrinologist and the primary pediatrician for many years. Thirty years ago (1980s), the development and application of GnRH agonist (GnRHa) therapy to treat central precocious pube
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Henry Rodriguez and Grace C. Dougan
Abstract
Precocious puberty has been a focus of interest for both the pediatric endocrinologist and the primary pediatrician for many years. Thirty years ago (1980s), the development and application of GnRH agonist (GnRHa) therapy to treat central precocious puberty significantly changed our approach to this disorder. More recently, application of molecular biological techniques has provided us with a better understanding of the intricacies of the regulation of gonadotropin and sex-steroid production characteristic of normal pubertal development and provided us with the tools to elucidate the etiologies of previously uncharacterized disorders of precocious puberty. Keywords
Precocious puberty • Gonadotropin-releasing hormone (GnRH) • GnRH agonist (GnRHa) • Follicle-stimulating hormone (FSH) • Luteinizing hormone (LH) • Androgen • Estrogen • Central precocious puberty (CPP) • Peripheral precocious puberty • Gonadotropin-independent precocious puberty
Introduction and Normal Development
H. Rodriguez, M.D. (*) USF Diabetes Center, University of South Florida-USF Health, Tampa, FL, USA e-mail: [email protected] G.C. Dougan, M.D. University of South Florida/All Children’s Hospital, St. Petersburg, FL, USA e-mail: [email protected]
Precocious puberty has been a focus of interest for both the pediatric endocrinologist and the primary pediatrician for many years. Thirty years ago (1980s), the development and application of GnRH agonist (GnRHa) therapy to treat central precocious puberty significantly changed our approach to this disorder. More recently, application of molecular biological techniques has provided us with a better
S. Radovick and M.H. MacGillivray (eds.), Pediatric Endocrinology: A Practical Clinical Guide, Second Edition, Contemporary Endocrinology, DOI 10.1007/978-1-60761-395-4_23, © Springer Science+Business Media New York 2013
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understanding of the intricacies of the regulation of gonadotropin and sex-steroid production characteristic of normal pubertal development and provided us with the tools to elucidate the etiologies of previously uncharacterized disorders of precocious puberty.
The Hypothalamic–Pituitary–Gonadal Axis The onset of puberty requires activation of the hypothalamic–pituitary–gonadal axis. Maturation of the hypothalamic–pituitary–gonadal axis initially occurs by mid-gestation [1]. GnRH or gonadotropin-releasing hormone is a decapeptide secreted by neuroendocrine neurons residing in the supraoptic and ventromedial nuclei of the preoptic and medial basal hypothalamus. Their nerve termini are found in the lateral portions of the median eminence adjacent to the pituitary stalk. GnRH secretion by these neurons is coordinated in such a way that, when grown in culture, individual cells exhibit pulsatile secretion that becomes synchronous when the cells are placed in physical proximity to each other. These cells interestingly are one of the few cell types that originate outside of the central nervous system, in the region
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