Genetic systemic disease with glomerulopathy as a major feature

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ORPHA:567556
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Overview

Genetic systemic disease with glomerulopathy as a major feature (Orphanet code 567556) is a broad category of inherited conditions where the kidneys — specifically the tiny filtering units called glomeruli — are damaged as a central part of the disease. The glomeruli act like microscopic sieves that clean your blood. When they are harmed by a genetic defect, protein and blood can leak into the urine, and the kidneys gradually lose their ability to filter waste properly. This group of diseases is 'systemic,' meaning the genetic problem can also affect other organs beyond the kidneys, such as the eyes, ears, liver, or nervous system, depending on the specific underlying condition. Symptoms often include foamy or bloody urine, swelling in the legs and face, high blood pressure, and fatigue. Over time, kidney function may decline, sometimes leading to chronic kidney disease or kidney failure. Because this is a category covering multiple specific genetic diseases, the exact symptoms, severity, and organs involved vary widely from person to person. Treatment depends on the specific genetic cause identified. There is no single cure for most conditions in this group. Management typically focuses on protecting kidney function, controlling blood pressure, reducing protein loss, and treating complications in other organs. Some specific subtypes may have targeted therapies available. Early diagnosis through genetic testing is very important because it guides treatment decisions and helps families understand their risk.

Key symptoms:

Foamy or frothy urine (protein leaking into urine)Blood in the urine, making it pink, red, or brownSwelling in the legs, ankles, feet, or around the eyesHigh blood pressureFatigue and low energyDecreased urine outputFrequent urinary tract infectionsHearing loss (in some subtypes)Vision problems or eye abnormalities (in some subtypes)Slow growth or poor weight gain in childrenNausea or loss of appetite as kidney function declinesMuscle cramps or weakness

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 Genetic systemic disease with glomerulopathy as a major feature.

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No actively recruiting trials found for Genetic systemic disease with glomerulopathy as a major feature at this time.

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No specialists are currently listed for Genetic systemic disease with glomerulopathy as a major feature.

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 Genetic systemic disease with glomerulopathy as a major feature.

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Community

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

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.Which specific genetic condition do I have, and what gene is responsible?,How quickly is my kidney function likely to decline, and what can I do to slow it down?,Are there any clinical trials or new treatments available for my specific genetic subtype?,Should my family members be tested for the same genetic condition?,What dietary changes should I make to protect my kidneys?,At what point would I need to consider dialysis or a kidney transplant?,Are there other organs I should be monitoring, such as my eyes or hearing?

Common questions about Genetic systemic disease with glomerulopathy as a major feature

What is Genetic systemic disease with glomerulopathy as a major feature?

Genetic systemic disease with glomerulopathy as a major feature (Orphanet code 567556) is a broad category of inherited conditions where the kidneys — specifically the tiny filtering units called glomeruli — are damaged as a central part of the disease. The glomeruli act like microscopic sieves that clean your blood. When they are harmed by a genetic defect, protein and blood can leak into the urine, and the kidneys gradually lose their ability to filter waste properly. This group of diseases is 'systemic,' meaning the genetic problem can also affect other organs beyond the kidneys, such as th