HJV or HAMP-related hemochromatosis

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ORPHA:79230OMIM:613313E83.1
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Overview

HJV or HAMP-related hemochromatosis, also called juvenile hemochromatosis or type 2 hemochromatosis, is a rare inherited condition where the body absorbs too much iron from food. Unlike the more common adult form of hemochromatosis, this type usually begins causing serious problems in teenagers and young adults. The extra iron builds up in organs like the heart, liver, and hormone-producing glands, slowly damaging them over time. The most common problems caused by this iron overload include heart disease, liver damage, and hormonal issues such as delayed puberty or infertility. Without treatment, the heart damage in particular can become life-threatening at a young age. The condition is caused by changes (mutations) in one of two genes: HJV (also called HFE2) or HAMP. Both genes play an important role in controlling how much iron the body absorbs. The good news is that treatment is available and works well when started early. The main treatment is called phlebotomy, which simply means regularly removing blood from the body to lower iron levels. This is similar to donating blood. When iron overload is caught and treated early, many people can live a relatively normal life. However, damage that has already happened to organs before treatment begins may not fully reverse, which is why early diagnosis is so important.

Also known as:

Key symptoms:

Extreme tiredness and low energyHeart problems such as irregular heartbeat, heart failure, or chest painLiver damage or cirrhosisDelayed puberty or failure to go through puberty normallyInfertility or loss of sex driveJoint pain, especially in the hands and fingersSkin that looks bronze or gray in colorDiabetes caused by iron damage to the pancreasAbdominal painWeakness and muscle achesHormonal imbalances affecting the thyroid or adrenal glands

Clinical phenotype terms (15)— hover any for plain English
Congenital hepatic fibrosisHP:0002612ArthropathyHP:0003040Generalized hyperpigmentationHP:0007440Abnormality of iron homeostasisHP:0011031Abnormality of endocrine pancreas physiologyHP:0012093Elevated transferrin saturationHP:0012463
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Juvenile

Begins in the teen years

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for HJV or HAMP-related hemochromatosis.

View clinical trials →

No actively recruiting trials found for HJV or HAMP-related hemochromatosis at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the HJV or HAMP-related hemochromatosis community →

No specialists are currently listed for HJV or HAMP-related hemochromatosis.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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

No travel grants are currently matched to HJV or HAMP-related hemochromatosis.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about HJV or HAMP-related hemochromatosis

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Caregiver Resources

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Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.How much iron has already built up in my organs, and has any damage occurred?,How often will I need phlebotomy, and what should I expect during and after each session?,Should my siblings and parents be tested for this condition?,Will my heart and liver damage improve with treatment, or is some of it permanent?,What dietary changes should I make to help manage my iron levels?,Are there any clinical trials or new treatments I should know about?,How will this condition affect my fertility, and are there options if I want to have children?

Common questions about HJV or HAMP-related hemochromatosis

What is HJV or HAMP-related hemochromatosis?

HJV or HAMP-related hemochromatosis, also called juvenile hemochromatosis or type 2 hemochromatosis, is a rare inherited condition where the body absorbs too much iron from food. Unlike the more common adult form of hemochromatosis, this type usually begins causing serious problems in teenagers and young adults. The extra iron builds up in organs like the heart, liver, and hormone-producing glands, slowly damaging them over time. The most common problems caused by this iron overload include heart disease, liver damage, and hormonal issues such as delayed puberty or infertility. Without treatm

How is HJV or HAMP-related hemochromatosis inherited?

HJV or HAMP-related hemochromatosis follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does HJV or HAMP-related hemochromatosis typically begin?

Typical onset of HJV or HAMP-related hemochromatosis is juvenile. Age of onset can vary across affected individuals.