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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.
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.