Medical Care
Hypogonadotropic hypogonadism
Treatment of hypogonadotropic hypogonadism depends on the gender and age of the patient as well as their desire for current fertility.
Females with delayed puberty secondary to hypogonadotropic hypogonadism are treated with estrogen to promote development of secondary sexual characteristics. Adult women with hypogonadotropic hypogonadism who desire fertility undergo ovulation induction with injectable gonadotropins. Clomiphene citrate is typically not effective for inducing ovulation in these patients. Women who do not desire pregnancy are treated with estrogen to prevent osteoporosis. Cyclic progesterone/progestins are added to decrease the risk of endometrial cancer. [21]
Women with hypogonadotropic hypogonadism secondary to anorexia nervosa or exercise can resume normal menses by gaining and maintaining weight equal at least to 15% of ideal body weight. Mild cases of anorexia nervosa may be treated on an outpatient basis under the care of a primary care physician, psychiatrist, psychologist, and/or nutritionist. Severe cases may require hospital admission for aggressive psychiatric rehabilitation and medical management. Mortality associated with anorexia has been reported to be as high as 15%. [22, 23]
Males with delayed puberty secondary to hypogonadotropic hypogonadism are treated with testosterone to promote the development of secondary sexual characteristics. Likewise, adult men with IHH who do not desire fertility are treated with testosterone to restore libido and secondary sexual characteristics.
Adult men with IHH who desire fertility can be treated with a subcutaneous pump that delivers pulses of GnRH. Alternatively, maintenance treatment clomiphene citrate therapy improves both sexual function and sperm production in men with IHH. Clomiphene citrate does not appear to increase testosterone secretion or sperm production in men with Kallmann syndrome.
Pituitary dysfunction
Men and women with LH deficiency secondary to pituitary dysfunction require treatment depending on the presenting symptoms and associated hormonal disorders. Most commonly, these patients have a pituitary adenoma and hyperprolactinemia. Men and women who desire fertility are best treated medically with a dopamine agonist (eg, bromocriptine, cabergoline), which inhibits prolactin secretion. Most patients with macroadenomas (>1 cm in diameter) are treated with a dopamine agonist to decrease the chance of further growth. Women with hyperprolactinemia who do not desire fertility but have amenorrhea are treated with oral contraceptives or cyclic estrogen and progestin as long as they do not have a macroadenoma. [17] Panhypopituitarism can result in life-threatening adrenal crisis (see Addison Disease). Patients with this condition require lifelong treatment with replacement thyroid and adrenal hormones in addition to the medical treatment discussed above.
Surgical Care
Most conditions that result in LH deficiency are not amenable to surgical therapy. One notable exception is the pituitary adenoma. Surgical therapy is required for large pituitary adenomas, those that continue to enlarge despite dopamine agonist treatment or those that impact the visual field irrespective of size. Most commonly, this type of microsurgery is performed using a transsphenoidal approach. This surgery has a risk of panhypopituitarism or persistent nasal leakage of cerebral spinal fluid.
Consultations
Kallmann syndrome
These patients are treated by endocrinologists (pediatric, reproductive, or medical) or urologists, depending on their gender and age. Genetic counseling is important, as this condition is often hereditable.
Hypogonadotropic hypogonadism
Women with this condition are most commonly cared for by reproductive endocrinologists (obstetrician-gynecologists with subspecialty training). Some patients with stress-related hypogonadotropic hypogonadic amenorrhea require psychological therapy as well, particularly in the cases of anorexia nervosa or bulimia, which can be fatal.
Men with hypogonadotropic hypogonadism are usually treated by urologists who specialize in infertility and impotence, since these are common presenting symptoms.
Pituitary dysfunction
Women with isolated hyperprolactinemia or anovulation related to pituitary dysfunction are treated by reproductive endocrinologists. Men with infertility related to pituitary dysfunction are often cared for by urologists who specialize in infertility.
Men or women with panhypopituitarism are cared for by medical endocrinologists. Those with pituitary tumors that are symptomatic (headaches, visual disturbances) or enlarging, despite medical therapy, should be referred to neurosurgeons with special expertise in transsphenoidal surgery.
Diet and Activity
No specific dietary recommendations have been made for the conditions associated with LH deficiency.
No specific activity recommendations have been made for the conditions that cause LH deficiency.
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MRI of pituitary macroadenoma.
