Causes, diagnosis, and treatment of central precocious puberty

Author(s)

Latronico, Ana Claudia ^[1] ; Brito, Vinicius Nahime ^[1] ; Carel, Jean-Claude ^{[2, 3, 4, 5]}

Affiliation

^[1] Univ Sao Paulo, Fac Med, Hosp Clin, Disciplina Endocrinol & Metabol, BR-05403900 Sao Paulo - Brazil

^[2] Univ Paris Diderot, Sorbonne Paris Cite, Paris - France

^[3] Hop Robert Debre, Dept Pediat Endocrinol & Diabetol, F-75019 Paris - France

^[4] Ctr Reference Malad Endocriniennes Rares Croissan, Paris - France

^[5] INSERM, U1141, Paris - France

• Document type: Journal article
• Source: LANCET DIABETES & ENDOCRINOLOGY; v. 4, n. 3, p. 265-274, MAR 2016.
• Web of Science Citations: 47

Abstract

Central precocious puberty results from the premature activation of the hypothalamic-pituitary-gonadal axis. It mimics physiological pubertal development, although at an inappropriate chronological age (before 8 years in girls and 9 years in boys). It can be attributable to cerebral congenital malformations or acquired insults, but the cause in most cases in girls remains unknown. MKRN3 gene defects have been identified in familial disease, with important basic and clinical results. Indeed, genetic analysis of this gene should be included in the routine clinical investigation of familial and idiopathic cases of central precocious puberty. Gonadotropin-releasing hormone agonists are the gold-standard treatment. The assessment and management of this disease remain challenging for paediatric endocrinologists. In this Series paper, we describe current challenges involving the precise diagnosis and adequate treatment of this disorder. (AU)

• FAPESP's process: 13/03236-5 - New approaches and methodologies in molecular-genetic studies of growth and pubertal development disorders
• Grantee: Alexander Augusto de Lima Jorge
• Support Opportunities: Research Projects - Thematic Grants