Medication Summary
The goals of pharmacotherapy are to correct hormonal imbalances, to prevent complications, and to reduce morbidity.
Hormone replacement agents
Class Summary
Estrogen is used to induce negative feedback at gonadotrophic regulatory centers, which, in turn, reduces release of gonadotropins. Progestins are used to prevent endometrial hyperplasia in patients with an intact uterus. Testosterone is used in males to restore sexual function and to replace the benefits of endogenous testosterone.
Conjugated estrogens (Premarin)
Restore estrogen levels to concentrations that induce negative feedback at gonadotrophic regulatory centers, which, in turn, reduces FSH release from the pituitary gland. Vaginal estrogen creams may alleviate vaginal dryness and urogenital symptoms, but systemic absorption is not usually sufficient to provide systemic effect.
Estradiol (Climara Transdermal, Estrace, Estraderm Transdermal, Gynodiol)
Restores estrogen levels to concentrations that induce negative feedback at gonadotrophic regulatory centers, which, in turn, reduces FSH release from pituitary. Vaginal estrogen creams may alleviate vaginal dryness and urogenital symptoms, but systemic absorption is not usually sufficient to provide systemic effect.
Medroxyprogesterone (Cycrin, Provera)
Progestins stop endometrial cell proliferation, allowing organized sloughing of cells after withdrawal; typically does not stop acute bleeding episode but produces a normal bleeding episode following withdrawal.
Testosterone (Delatestryl injection, AndroGel, Androderm, Testoderm TTS)
Promotes the growth and development of the male sex organs and maintains secondary sex characteristics in androgen-deficient males
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Human G protein-coupled receptor 54 (GPR54) receptor model. Mutations identified in patients with idiopathic hypogonadotropic hypogonadism are indicated.
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This is a frequently sampled serum luteinizing hormone (LH) profile in a male patient with Kallmann syndrome (KS), compared with that in a healthy individual. A lack of LH pulsatility is seen in the former.
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Magnetic resonance imaging (MRI) scan of pituitary macroadenoma.
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Adolescent male with Klinefelter syndrome who has female-type distribution of pubic hair, as well as testicular dysgenesis.
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Hypoplastic right hemiscrotum in a patient with an undescended right testis.
