Akademik

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.

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

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