Rare male infertility due to hypothalamic-pituitary-gonadal axis disorder

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Overview

Rare male infertility due to hypothalamic-pituitary-gonadal (HPG) axis disorder is a condition in which the hormonal signaling system that controls male reproductive function does not work properly. The HPG axis is a chain of communication between three parts of the body: the hypothalamus (a region in the brain), the pituitary gland (a small gland at the base of the brain), and the gonads (the testes in men). When this axis is disrupted, the testes may not receive the right hormonal signals to produce sperm or testosterone adequately, leading to infertility and sometimes other signs of low testosterone such as reduced sex drive, fatigue, or incomplete puberty. This condition can be caused by genetic mutations affecting one or more parts of the HPG axis. Some forms are present from birth and may be noticed when a boy does not go through puberty on time, while other forms may appear later in adulthood when a man has difficulty fathering children. The severity varies widely depending on the underlying cause. Treatment typically focuses on hormone replacement therapy to restore normal testosterone levels and, in many cases, gonadotropin therapy (injections of hormones like FSH and hCG) to stimulate sperm production. With appropriate treatment, some men with this condition can achieve fertility. Early diagnosis and management by a specialized team are important for the best outcomes.

Also known as:

Key symptoms:

Inability to father children (infertility)Delayed or absent pubertySmall testesLow sex driveErectile dysfunctionReduced facial or body hairFatigue or low energyDecreased muscle massMood changes or depressionBreast tissue enlargement (gynecomastia)Low sperm count or absent sperm in semenReduced bone density

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Rare male infertility due to hypothalamic-pituitary-gonadal axis disorder.

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No actively recruiting trials found for Rare male infertility due to hypothalamic-pituitary-gonadal axis disorder at this time.

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No specialists are currently listed for Rare male infertility due to hypothalamic-pituitary-gonadal axis disorder.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What is the specific cause of my HPG axis disorder, and is genetic testing recommended?,What are my chances of achieving fertility with gonadotropin therapy?,How long will it take before we know if fertility treatment is working?,Should I consider sperm banking or assisted reproductive technologies?,What are the long-term health risks of low testosterone, and how will treatment help?,Are there any clinical trials or newer treatments I should know about?,Should my family members be tested for the same genetic condition?

Common questions about Rare male infertility due to hypothalamic-pituitary-gonadal axis disorder

What is Rare male infertility due to hypothalamic-pituitary-gonadal axis disorder?

Rare male infertility due to hypothalamic-pituitary-gonadal (HPG) axis disorder is a condition in which the hormonal signaling system that controls male reproductive function does not work properly. The HPG axis is a chain of communication between three parts of the body: the hypothalamus (a region in the brain), the pituitary gland (a small gland at the base of the brain), and the gonads (the testes in men). When this axis is disrupted, the testes may not receive the right hormonal signals to produce sperm or testosterone adequately, leading to infertility and sometimes other signs of low tes