What Is Hypergonadotropic Hypogonadism?

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Hypergonadotropic hypogonadism (HH) is characterized by deficiencies in sex hormones caused by disorders in their production in the sex organs. This condition causes a range of symptoms, including low energy, late puberty, infertility, low sex drive, and absence of menstruation, among others. Here’s a quick overview of the causes, management, and prognosis of HH.

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Types of Hypogonadism

At its core, hypogonadism is defined by insufficient production of the female and male sex hormones, estrogen and testosterone, respectively. There are two types of hypogonadism: HH, or primary hypogonadism, and secondary, or central, hypogonadism.

Primary hypogonadism is when the hormone deficiency is due to problems in the sex glands, or gonads—the female ovaries and male testes. This type can be congenital, the result of surgery or cancer treatments, or due to autoimmune diseases or other disorders.

Secondary hypogonadism is when hormone production is caused by problems in brain signaling. In these cases, also known as hypogonadotropic hypogonadism, the activity of the pituitary gland (which regulates sex characteristics), and the hypothalamus (the surrounding brain region that regulates pituitary function) is disrupted.

Causes and Risk Factors

Risk factors and causes for this class include:

Hypergonadotropic Hypogonadism Symptoms

Levels of the sex hormones vary between sexes—insufficient estrogen produces some different symptoms than insufficient testosterone. Sex hormones also naturally decrease as you get older. Since HH affects these levels, the symptoms of the condition vary based on your age and sex.

Symptoms in Children

HH in children and adolescents can cause:

  • Delayed onset of puberty
  • Lack of facial or body hair development
  • Shortness of stature
  • Underdeveloped testicles and penis
  • Absence of irregular menstrual periods
  • Infertility
  • Low sex drive

Symptoms in Adults

In adults, HH produces different symptoms based on whether estrogen or testosterone levels are being affected. In cisgender men and women, this leads to:

  • Low or absent sex drive
  • Fatigue and low energy
  • Difficulty concentrating
  • Hair loss
  • Hot flashes

Additionally, insufficient levels of the female sex hormone, estrogen, cause:

  • Abnormal or absent menstruation
  • Early menopause
  • Infertility

If there’s not enough testosterone in the system, cisgender men may experience:

Causes

The reductions in hormone levels due to hypergonadotropic hypogonadism occur due to disorders in the gonads (sex organs), themselves. As a result, the testes and ovaries secrete insufficient gonadotropic-releasing hormone (GnRH), hindering testosterone and estrogen production, respectively.

This happens due to a range of conditions, including:

  • Part of autoimmune polyglandular endocrinopathy, including Addison’s disease (the adrenal glands produce too little of certain hormones) and type 2 diabetes (the body can’t efficiently use sugar as fuel)
  • Radiation therapy or chemotherapy for cancer
  • Turner syndrome, a genetic disorder affecting cisgender girls and women
  • Klinefelter's syndrome, a genetic disorder in cisgender boys and men
  • Too much iron in the bloodstream (hemochromatosis)
  • Undescended testicles
  • Diseases of the liver and kidney
  • Surgery on the reproductive organs

Diagnosis

If HH is suspected, there are several steps to ensure a correct diagnosis. Here’s a breakdown:

  • Physical examination: In addition to an assessment of symptoms and a medical history, a physical examination of overall health is essential to the process. Cisgender women may also have a pelvic examination.
  • Hormone level tests: Low levels of two gonadotropins in the blood, follicle-stimulating hormone (FSH) and luteinizing hormone (LH), are signs of HH. In cisgender women and girls, estrogen levels will be measured, whereas testosterone is tracked in cisgender men.
  • Blood tests: To isolate causes of hypogonadism, your blood may also be tested for signs of erythrocytosis (high red blood cells, or RBCs, in the bloodstream) or elevated iron. Genetic tests may also be performed to assess for chromosomal issues (called a karyotype) or congenital conditions.
  • Other tests: Diagnosis may also involve assessments of the pituitary gland and thyroid function, sperm counts, chromosomal analysis, and level of prolactin hormone (the hormone involved in milk production).    

Treatment

The primary way of treating hypergonadotropic hypogonadism is with therapies that aim to replace and replenish hormone levels. Here’s a quick breakdown:

  • Estrogen replacement therapy: For cisgender women and girls with HH, estrogen is prescribed, sometimes alongside progesterone (another sex hormone). This treatment, also used for menopause, comes in the form of skin patches, creams, vaginal rings, or pills. Though effective, use does raise the risk of uterine cancer, blood clots, and stroke.
  • Testosterone replacement therapy: In cisgender men and boys, testosterone can be replaced directly. As with estrogen therapy, it can be delivered in a range of ways, including injections, creams, nasal sprays pills, patches, or even via a pellet implanted in the skin. However, this therapy can affect fertility.
  • Other therapies: For women with low sex drive as a result of HH, low doses of testosterone or dehydroepiandrosterone (DHEA), a precursor hormone and one of the most widely circulating steroids, may be indicated. Additionally, alternative formulations for men may include another hormone, dihydrotestosterone, or involve the application of human chorionic gonadotropin (hCG), which can spur testosterone production.

Prognosis

While HH can be chronic, therapies for the condition can successfully manage it. Most people who get treatment lead full and active lives, though symptoms may return if you stop taking your medications. Throughout the course of your treatment, be sure to be open with your doctor about how you’re feeling and let them know if anything seems off.  

Coping

Critical for living with HH, first and foremost, is knowing when you need help. Call your doctor if you experience:

  • Breast enlargement and/or milky breast discharges (in men)
  • Hot flashes (in women)
  • Low sex drives
  • Impotence or erectile dysfunction
  • Loss of body hair
  • Lack of menstruation
  • Problems becoming pregnant

Since HH can affect sexual function and delay puberty, it can also have a significant impact on mental health. Difficult as it may be, try to be open with your provider about your sex life if it’s affected. For some, individualized or even group counseling can help.

When to Call 911

The biggest concern isn’t HH itself so much as the side effects of the medications you’re taking. Get emergency help if you experience sudden headaches or vision problems. In addition, watch out for signs of allergic shock, including swelling, shortness of breath, hives, dizziness, and fainting, as well as nausea and vomiting.

A Word From Verywell

While the symptoms of hypergonadotropic hypogonadism can be challenging, this condition can be effectively managed and treated. Don’t be afraid to enlist the support of your loved ones, along with your healthcare providers. Working together, you’ll be primed to survive—and, indeed thrive—with HH.

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5 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. National Institutes of Health. Hypogonadism.

  2. Cleveland Clinic. Low sex drive (hypogonadism): symptoms, treatment. Updated November 20, 2020.

  3. Viswanathan V, Eugster E. Etiology and treatment of hypogonadism in adolescents. Pediatr Clin North Am. 2011;58(5):1181-1200. doi:10.1016/j.pcl.2011.07.009

  4. Cleveland Clinic. Hormone therapy for menopause: types, benefits & risks. Updated June 28, 2021.

  5. Thirumalai A, Berkseth K, Amory J. Treatment of hypogonadism: current and future therapies. F1000Res. 2017;6:68. doi:10.12688/f1000research.10102.1