Synonyms FMPP, Familial Gonadotrophin-Independent Sexual Precocity, Familial Male-Limited Precocious Puberty + [20] |
Introduction A very rare gonadotropin-independent familial form of male-limited precocious puberty generally presenting between 2-5 years of age as accelerated growth, early development of secondary sexual characteristics and reduced adult height. Caused by an activating mutation of the Lutropin-Choriogonadotropic Hormone Receptor gene (LHCGR, 2p21) which leads to increased levels of sex steroids in the context of low luteinising hormone. This receptor's chronic activation leads to precocious testosterone production by Leydig cells. No effect is observed in female carriers due to the dual luteinising hormone (LH)/ follicle stimulating hormone (FSH) signal necessary to promote ovarian stimulation. Transmission is autosomal dominant. Mothers may act as silent carriers, with each son having a 50% chance of displaying this disorder. |
Target |
Mechanism GnRHR agonists |
Originator Org. |
Active Indication |
Inactive Indication |
Drug Highest PhaseApproved |
First Approval Ctry. / Loc. KR |
First Approval Date04 Nov 1996 |
Target |
Mechanism aromatase inhibitors |
Active Org. |
Originator Org. |
Active Indication |
Inactive Indication |
Drug Highest PhaseApproved |
First Approval Ctry. / Loc. US |
First Approval Date27 Dec 1995 |
Target |
Mechanism AR antagonists |
Active Org. |
Originator Org. |
Active Indication |
Inactive Indication |
Drug Highest PhaseApproved |
First Approval Ctry. / Loc. GB |
First Approval Date23 Feb 1995 |
Start Date19 Dec 2006 |
Sponsor / Collaborator |
Start Date02 Jan 2006 |
Sponsor / Collaborator |
Start Date22 Nov 2004 |
Sponsor / Collaborator |