Peripheral precocious puberty

Robert Krysiak; Bogdan Marek; Bogusław Okopień

Vol 60, No 6 (2009)

Review paper

Submitted: 2013-02-15

Published online: 2009-12-30

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Peripheral precocious puberty

Robert Krysiak, Bogdan Marek, Bogusław Okopień

Endokrynol Pol 2009;60(6):503-514.

Vol 60, No 6 (2009)

Reviews — Postgraduate Education

Submitted: 2013-02-15

Published online: 2009-12-30

Abstract

The term precocious puberty is defined as the appearance of secondary sex characteristics before the age of 8 in girls and 9 in boys. There are two major forms of premature sexual maturation: gonadotrophin-dependent (central, or ’true’ precocious puberty) and gonadotrophin- independent precocious puberty. The latter, also called peripheral precocious puberty, is characterized by increased production of sex steroids, causing the typical physical changes of puberty, in the absence of reactivation of the hypothalamic-pituitary axis. It may result from several different disorders including testotoxicosis, McCune-Albright syndrome, congenital adrenal hyperplasia, adrenal and gonadal tumours. The accumulation of knowledge regarding the pathogenesis of symptoms and the development of safe, effective treatment modalities have led to earlier intervention in patients with peripheral precocious puberty to prevent the decline in their psychosocial wellbeing, adult height and quality of life. We review the ethiopathogenesis, clinical picture, diagnosis and treatment of various disorders causing peripheral precocious puberty and provide the reader with current recommendations concerning approach to the patient with this health problem.

Abstract

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Keywords

testotoxicosis; McCune-Albright syndrome; congenital adrenal hyperplasia; adrenal and gonadal tumours; etiopathogenesis; symptoms; diagnosis; treatment