Primary hypomagnesemia with hypercalciuria and nephrocalcinosis

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Overview

Primary hypomagnesemia with hypercalciuria and nephrocalcinosis (often called FHHNC, which stands for Familial Hypomagnesemia with Hypercalciuria and Nephrocalcinosis) is a rare inherited kidney disorder. In this condition, the kidneys lose their ability to properly reabsorb magnesium and calcium from the urine back into the blood. This leads to abnormally low magnesium levels in the blood (hypomagnesemia), too much calcium in the urine (hypercalciuria), and a buildup of calcium deposits in the kidneys (nephrocalcinosis). Over time, these calcium deposits can damage the kidneys and lead to progressive kidney failure. Symptoms often begin in childhood and can include frequent urinary tract infections, excessive thirst and urination, kidney stones, muscle cramps, seizures, and fatigue. Some patients also develop eye problems, particularly abnormalities of the cornea or lens. The disease tends to progress over years, and many affected individuals eventually develop chronic kidney disease that may require dialysis or kidney transplantation. Treatment focuses on replacing magnesium through oral or intravenous supplements and managing complications. Unfortunately, magnesium supplementation often cannot fully correct the blood levels because the kidneys continue to waste magnesium. Thiazide diuretics are sometimes used to try to reduce calcium loss in the urine. Close monitoring of kidney function is essential, and some patients will need kidney replacement therapy. There is currently no cure for this condition, and research continues to find better ways to slow kidney damage.

Also known as:

Key symptoms:

Low magnesium levels in the bloodExcessive calcium in the urineCalcium deposits in the kidneys (nephrocalcinosis)Kidney stonesFrequent urinary tract infectionsExcessive thirstFrequent urinationMuscle cramps and spasmsSeizuresFatigue and weaknessFailure to thrive or poor growth in childrenEye problems such as severe nearsightedness or corneal abnormalitiesProgressive kidney failureBone pain or rickets-like bone changesTingling or numbness in hands and feet

Inheritance

Autosomal recessive

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Primary hypomagnesemia with hypercalciuria and nephrocalcinosis.

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No actively recruiting trials found for Primary hypomagnesemia with hypercalciuria and nephrocalcinosis at this time.

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No specialists are currently listed for Primary hypomagnesemia with hypercalciuria and nephrocalcinosis.

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 Primary hypomagnesemia with hypercalciuria and nephrocalcinosis.

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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 my (or my child's) current kidney function, and how quickly is it expected to change?,Which gene mutation is causing this condition, and does it affect the eyes as well?,What is the best magnesium supplement and dosing schedule for this condition?,How often should blood tests, urine tests, and kidney imaging be done?,At what point should we start planning for dialysis or kidney transplantation?,Are there any clinical trials or new treatments being studied for this disease?,Should other family members be tested for this condition or carrier status?

Common questions about Primary hypomagnesemia with hypercalciuria and nephrocalcinosis

What is Primary hypomagnesemia with hypercalciuria and nephrocalcinosis?

Primary hypomagnesemia with hypercalciuria and nephrocalcinosis (often called FHHNC, which stands for Familial Hypomagnesemia with Hypercalciuria and Nephrocalcinosis) is a rare inherited kidney disorder. In this condition, the kidneys lose their ability to properly reabsorb magnesium and calcium from the urine back into the blood. This leads to abnormally low magnesium levels in the blood (hypomagnesemia), too much calcium in the urine (hypercalciuria), and a buildup of calcium deposits in the kidneys (nephrocalcinosis). Over time, these calcium deposits can damage the kidneys and lead to pro

How is Primary hypomagnesemia with hypercalciuria and nephrocalcinosis inherited?

Primary hypomagnesemia with hypercalciuria and nephrocalcinosis follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Primary hypomagnesemia with hypercalciuria and nephrocalcinosis typically begin?

Typical onset of Primary hypomagnesemia with hypercalciuria and nephrocalcinosis is childhood. Age of onset can vary across affected individuals.