Familial male-limited precocious puberty

Abstract

Familial male-limited precocious puberty (FMPP), also known as testotoxicosis, is a rare, dominantly inherited disorder that causes gonadotropin-independent sexual precocity in boys. Early signs of puberty, including virilization, growth acceleration, and skeletal advancement, develop in affected boys, usually by 3 years of age. Activating mutations of the human luteinizing hormone (LH) receptor result in increased testosterone production by the Leydig cells despite low LH levels. Diagnosis, molecular analysis, and therapy for FMPP are discussed. © Humana Press Inc., Totowa, NJ.