Familial male-limited precocious puberty
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Familial male-limited precocious puberty, often abbreviated as FMPP, also known as familial sexual precocity or gonadotropin-independent testotoxicosis,[1] is a form of gonadotropin-independent precocious puberty in which boys experience early onset and progression of puberty.[2] Signs of puberty can develop as early as an age of 1 year.Script error: No such module "Unsubst".
The spinal length in boys may be short due to a rapid advance in epiphyseal maturation. It is an autosomal dominant[1] condition with a mutation of the luteinizing hormone (LH) receptor. As FMPP is a gonadotropin-independent form of precocious puberty, gonadotropin-releasing hormone agonists (GnRH agonists) are ineffective. Treatment is with drugs that suppress or block the effects of gonadal steroidogenesis, such as cyproterone acetate, ketoconazole, spironolactone, and testolactone.[3] Alternatively, the combination of the androgen receptor antagonist bicalutamide and the aromatase inhibitor anastrozole may be used.[4]
Robert King Stone, personal physician to American president Abraham Lincoln, described the first case of FMPP in 1852.[5]
See also
- Follicle-stimulating hormone insensitivity
- Gonadotropin-releasing hormone insensitivity
- Hypergonadism, hyperandrogenism, and precocious puberty
- Inborn errors of steroid metabolism
- Leydig cell hypoplasia (or LH insensitivity)
References
External links
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