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gonadotropin-independent precocious puberty
Wednesday 21 April 2010
A group of children exists in whom premature sexual maturation occurs in the absence of pubertal levels of gonadotropins; that is, they have gonadotropin-independent precocious puberty.
Children with gonadotropin-independent precocity demonstrate an absence of gonadotropin pulsations, variable responses to luteinizing hormone-releasing hormone, lack of suppression of puberty in response to LHRHa, and cyclic steroidogenesis.
Tissue from testicular biopsies performed in boys with gonadotropin-independent precocity show a range from incipient pubertal development of the tubules with proliferation of Leydig cells to the appearance of normal adult testes.
Gonadotropin-independent precocious puberty is a distinct syndrome, of unknown cause, that may be familial and may have been responsible for many previously reported cases of precocious puberty.
References
Puberty without gonadotropins. A unique mechanism of sexual development. Wierman ME, Beardsworth DE, Mansfield MJ, Badger TM, Crawford JD, Crigler JF Jr, Bode HH, Loughlin JS, Kushner DC, Scully RE, et al. N Engl J Med. 1985 Jan 10;312(2):65-72. PMID: 3917301