Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:31043OMIM:248250E83.4
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Primary hypomagnesemia with hypercalciuria and nephrocalcinosis (PHHN) — sometimes called familial hypomagnesemia with hypercalciuria and nephrocalcinosis, or FHHNC — is a rare inherited kidney disorder. In this condition, the kidneys are unable to hold onto enough magnesium and calcium, so these important minerals are lost in the urine at much higher levels than normal. Over time, the excess calcium builds up in the kidney tissue, a process called nephrocalcinosis, which can slowly damage the kidneys. The disease is caused by changes (mutations) in genes that control special channels in the kidney tubules — the tiny tubes that filter and reabsorb minerals. Without working channels, magnesium and calcium leak out instead of being kept in the body. Low magnesium levels in the blood can cause muscle cramps, spasms, and seizures. The kidneys gradually lose their filtering ability, and many patients develop chronic kidney disease over time. This specific form of PHHN is distinguished from a related condition by the absence of severe eye problems. Treatment focuses on replacing magnesium with supplements and managing kidney health. Unfortunately, supplements can slow but often cannot fully stop kidney damage. Regular monitoring by kidney specialists is essential to protect long-term kidney function.

Also known as:

Key symptoms:

Frequent urinary tract infectionsKidney stonesCalcium deposits in the kidneys (nephrocalcinosis)Muscle cramps and spasmsMuscle weaknessSeizures due to low magnesiumExcessive thirst and frequent urinationFatigue and low energyPoor growth or failure to thrive in childrenAbdominal or flank painGradually worsening kidney function (chronic kidney disease)

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 ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement.

View clinical trials →

No actively recruiting trials found for Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement at this time.

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

Search ClinicalTrials.gov ↗Join the Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement community →

No specialists are currently listed for Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement.

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 without severe ocular involvement.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvementForum →

No community posts yet. Be the first to share your experience with Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement.

Start the conversation →

Latest news about Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement

No recent news articles for Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement.

Follow this condition to be notified when news becomes available.

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.How advanced is the kidney damage right now, and how quickly might it progress?,What dose of magnesium supplement is right for my child, and how do we know if it is working?,Should we also test other family members for this condition?,Are there any dietary changes that could help protect the kidneys?,At what point would dialysis or a kidney transplant be considered?,Are there any clinical trials or new treatments we should know about?,How often should we have follow-up appointments and what tests are needed each time?

Common questions about Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement

What is Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement?

Primary hypomagnesemia with hypercalciuria and nephrocalcinosis (PHHN) — sometimes called familial hypomagnesemia with hypercalciuria and nephrocalcinosis, or FHHNC — is a rare inherited kidney disorder. In this condition, the kidneys are unable to hold onto enough magnesium and calcium, so these important minerals are lost in the urine at much higher levels than normal. Over time, the excess calcium builds up in the kidney tissue, a process called nephrocalcinosis, which can slowly damage the kidneys. The disease is caused by changes (mutations) in genes that control special channels in the

How is Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement inherited?

Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement 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 without severe ocular involvement typically begin?

Typical onset of Primary hypomagnesemia with hypercalciuria and nephrocalcinosis without severe ocular involvement is childhood. Age of onset can vary across affected individuals.