Familial Male-Limited Precocious Puberty

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Retrieved
2021-01-18
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Familial male-limited precocious puberty, often abbreviated as FMPP, also known as familial sexual precocity or gonadotropin-independent testotoxicosis, is a form of gonadotropin-independent precocious puberty in which boys experience early onset and progression of puberty. Signs of puberty can develop as early as an age of 1 year.

The spinal length in boys may be short due to a rapid advance in epiphyseal maturation. It is an autosomal dominant condition with a mutation of the luteinizing hormone (LH) receptor. Treatment is with drugs that suppress gonadal steroidogenesis, such as cyproterone acetate, ketoconazole, spironolactone, and testolactone. Alternatively, the combination of the androgen receptor antagonist bicalutamide and the aromatase inhibitor anastrozole may be used.

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)