Familial Precocious Puberty Associated with Isolated Elevation of Luteinizing Hormone

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Abstract
THE great majority of cases of central precocious puberty, with premature activation of the hypothalamic-pituitary-gonadal axis, are sporadic and idiopathic, with a female-to-male ratio of between 4:1 and 8:1.1 Familial central precocious puberty, however, is generally inherited as a male-limited autosomal dominant trait.2 In the 11 reported kindreds, transmission has usually been from affected male to affected male or through unaffected female subjects. There has been only one documented occurrence of central precocious puberty involving siblings of opposite sexes.3 We recently observed a unique familial abnormality in pubertal development, which resulted in isosexual precocious development in a boy and heterosexual . . .

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