Hypergonadotropic hypogonadism

Hypergonadotropic hypogonadism
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Synonyms	Primary hypogonadism
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Hypergonadotropic hypogonadism (HH), also known as primary hypogonadism, is a condition characterized by the reduced function of the gonads (the ovaries in females and testes in males), with a consequent decrease in the production of sex hormones. This reduction in hormone levels leads to various clinical symptoms depending on the age at onset and the degree of hormone deficiency.

Causes

Hypergonadotropic hypogonadism is primarily caused by an intrinsic problem with the gonads. This can be due to:

• Genetic disorders, such as Turner syndrome in females and Klinefelter syndrome in males.
• Damage to the gonads from infections like mumps, trauma, or radiation.
• Autoimmune disorders targeting the gonadal tissues.
• Chemotherapy or radiation therapy that affects gonadal function.

Pathophysiology

In hypergonadotropic hypogonadism, the levels of gonadotropins (FSH and LH) are elevated. This is because the pituitary gland produces more FSH and LH in response to the low levels of sex hormones. The increased levels of gonadotropins are unable to stimulate the dysfunctional gonads to produce hormones, leading to a state of hypogonadism.

Symptoms

The symptoms of hypergonadotropic hypogonadism vary based on the age of onset:

• In children, it can lead to delayed puberty and may manifest as a failure to develop secondary sexual characteristics.
• In adults, symptoms may include decreased libido, erectile dysfunction, infertility, fatigue, hot flashes, and osteoporosis.

Diagnosis

Diagnosis of hypergonadotropic hypogonadism involves:

• Clinical assessment of sexual development and symptoms.
• Blood tests showing elevated levels of FSH and LH.
• Low levels of sex hormones (estrogen in females and testosterone in males).
• Imaging studies like ultrasound or MRI may be used to assess the physical structure of the gonads.

Treatment

Treatment focuses on hormone replacement therapy to manage symptoms and prevent complications:

• Testosterone replacement therapy for males.
• Estrogen and progesterone therapy for females.
• Monitoring for potential side effects of long-term hormone therapy, such as cardiovascular risks and bone health.

Prognosis

With appropriate management, individuals with hypergonadotropic hypogonadism can lead relatively normal lives, although they often require lifelong hormone therapy and monitoring for associated health issues.

See also

• Hypogonadism
• Hypothalamic disease
• Pituitary disorders