hypogonadotropic hypogonadism


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Related to hypogonadotropic hypogonadism: hypergonadotropic hypogonadism, primary hypogonadism

hypogonadism

 [hi″po-go´nad-izm]
decreased functional activity of the gonads, with retardation of growth, sexual development, and secondary sex characters. The American Association of Clinical Endocrinologists has published “Clinical Practice Guidelines for the Evaluation and Treatment of Hypogonadism in Adult Male Patients.” These are available online at their web site: http://www.aace.com.
hypergonadotropic hypogonadism that associated with secretion of high levels of gonadotropins, as in Klinefelter's syndrome. Called also hypergonadotropic eunuchoidism.
hypogonadotropic hypogonadism that due to lack of gonads or of gonadotropin secretion. Called also hypogonadotropic eunuchoidism.

hy·po·gon·a·do·trop·ic hy·po·go·nad·ism

[MIM*146110]
defective gonadal development or function, or both, resulting from inadequate secretion of pituitary gonadotropins.

hypogonadotropic hypogonadism

Hypogonadotropic eunuchoidism, Kallman syndrome A rare condition with a highly variable hereditary pattern, characterized by secondary hypogonadism–↓ gonadotropin-releasing hormone due to hypothalamic or pituitary dysfunction with testicular failure, and anosmia–due to hypoplasia or aplasia of the olfactory bulbs and tracts, 2º to a defect in the migration of olfactory neurons, and neurons producing GRH–gonadotropin-releasing hormone; ↓ FSH and LH impairs sperm and androgen production Clinical Delayed puberty, micropenis, eunuchoid features, cryptorchidism, midline defects–eg, cleft lip and palate, unilateral renal agenesis, horseshoe kidney, nerve deafness and hearing loss, color blindness, skeletal abnormalities; synkinesia, spatial attention defects, spastic paraplegia, cerebellar dysfunction, horizontal nystagmus, pes cavus, mental retardation Management Androgens to induce anatomic maturation; gonadotropins or LH-releasing factor for spermatogenesis. See Kalig-1.

hypogonadotropic hypogonadism

A usually congenital condition in which there is a severe deficiency of the gonadotropin-releasing hormone (GnRH) of the pituitary gland. As a rulethe penis remains infantile and the testes do not descend into the scrotum (cryptorchidism). No sexual development occurs at puberty. In rare cases the condition may develop in men who had previous normal secondary sexual development and a normal puberty. This leads to loss of libido, impotence and infertility. In these cases, treatment with gonadotropin-releasing hormone can reverse all the features of the disorder.
References in periodicals archive ?
These results clearly indicate that the initial pattern of hypogonadotropic hypogonadism was reversed after anastrazole administration.
In summary, these results, based on single-point sampling of LH, testosterone, and estradiol concentrations before and after the administration of the aromatase inhibitor anastrazole, indicate alleviation of hypogonadotropic hypogonadism in these patients.
Food and Drug Administration (FDA) in May 2014 for replacement therapy in adult men with conditions associated with deficiency or absence of endogenous testosterone, including primary hypogonadism (congenital or acquired) or hypogonadotropic hypogonadism (congenital or acquired).
Hypergonadotropic hypogonadism in 3 patients (6%), hypogonadotropic hypogonadism in 1 female patient (2%), and small testicular volume in respect to age in 3 male patients (8.
The group was able to demonstrate that mutations in the same POLR3A gene localized on chromosome 10 were responsible for three clinically different forms of leukodystrophies: Tremor-Ataxia with Central Hypomyelination (TACH) first described in Quebec cases, Leukodystrophy with Oligodontia (LO), and 4H syndrome or Hypomyelination, Hypodontia and Hypogonadotropic Hypogonadism syndrome.
A family with hypogonadotropic hypogonadism and mutations in the gonadotropin releasing hormone receptor.
Based on the presence and absence of sense of smell, idiopathic hypogonadotropic hypogonadism (IHH) forms two major subtypes: Kallmann Syndrome (KS) with anosmia/hyposmia and normosomic IHH (nIHH).