hypogonadism

Inadequate gonadal function, as manifested by deficiencies in gametogenesis and/or the secretion of gonadal hormones; results in atrophy or deficient development of secondary sexual characteristics and, when occurring in prepubertal males, in altered body habitus characterized by a short trunk and long limbs.

- familial hypogonadotropic h. [MIM*312100 & MIM*307300] a group of disorders characterized by failure of sexual development, owing to inadequate secretion of pituitary gonadotropins; perhaps X-linked, but probably autosomal dominant and recessive modes of inheritance also exist.

- hypergonadotropic h. defective gonadal development or function of the gonads, resulting from elevated levels of gonadotropins.

- hypogonadotropic h. defective gonadal development or function, or both, resulting from inadequate secretion of pituitary gonadotropins. SYN: hypogonadotropic eunuchoidism, secondary h..

- male h. SYN: eunuchoidism.

- primary h. defective gonadal development or function, or both, due to abnormality or loss of the gonad itself.

- secondary h. SYN: hypogonadotropic h..

- h. with anosmia failure of sexual development secondary to inadequate secretion of pituitary gonadotrophins, associated with anosmia due to agenesis of the olfactory lobes of the brain. Autosomal dominant [MIM*147950], autosomal recessive [MIM*244200], and X-linked recessive [MIM*308700] forms exist; the X-linked form is caused by mutation in the Kallmann gene (KAL1) on Xp. SYN: Kallmann syndrome.

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hy·po·go·nad·ism -'gō-.nad-.iz-əm n

1) functional incompetence of the gonads esp. in the male with subnormal or impaired production of hormones and germ cells

2) an abnormal condition (as Klinefelter's syndrome) involving gonadal incompetence

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n.

impaired function of the testes or ovaries, causing absence or impairment of the secondary sexual characteristics.

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hy·po·go·nad·ism (hi″po-goґnad-iz-əm) a condition resulting from abnormally decreased gonadal function, with retardation of growth, sexual development, and secondary sex characters; see hypo-orchidism, hypo-ovarianism, and eunuchoidism. Called also hypogenitalism.