Familial primary hyperparathyroidism

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Overview

Familial primary hyperparathyroidism (FPHPT) is an inherited condition in which one or more of the parathyroid glands — four small glands located in the neck near the thyroid — become overactive and produce too much parathyroid hormone (PTH). This hormone normally controls calcium levels in the blood. When too much PTH is made, calcium levels rise above normal, a condition called hypercalcemia. Over time, high calcium can cause a wide range of problems throughout the body. Unlike the more common sporadic (non-inherited) form of primary hyperparathyroidism, the familial form runs in families and is caused by changes (mutations) in specific genes. It can appear as an isolated condition (familial isolated hyperparathyroidism, or FIHP) or as part of broader genetic syndromes such as Multiple Endocrine Neoplasia type 1 (MEN1) or type 2A (MEN2A), or hyperparathyroidism-jaw tumor syndrome (HPT-JT). Common symptoms include fatigue, bone pain, kidney stones, frequent urination, excessive thirst, muscle weakness, depression, and difficulty concentrating — sometimes summarized as 'bones, stones, abdominal moans, and psychic groans.' Some people have no obvious symptoms and are diagnosed only through routine blood tests showing high calcium. Treatment typically involves surgery to remove the overactive parathyroid gland(s), which is often curative. However, because the condition is genetic, multiple glands may be affected, and the disease can recur, so lifelong monitoring is important. Medications such as cinacalcet may be used when surgery is not possible or not desired.

Key symptoms:

Fatigue and low energyKidney stonesBone pain or bone thinning (osteoporosis)Frequent urinationExcessive thirstMuscle weaknessDepression or mood changesDifficulty concentrating or memory problemsNausea or loss of appetiteAbdominal pain or constipationJoint achesHigh blood pressureHeart rhythm irregularitiesFractures from minor injuries

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

1 available

CINACALCET

CINACALCET· Aurobindo Pharma Limited

Hypercalcemia in adult patients with primary HPT for whom parathyroidectomy would be indicated on the basis of serum calcium levels, but who are unable to undergo parathyroidectomy

No actively recruiting trials found for Familial primary hyperparathyroidism at this time.

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

Search ClinicalTrials.gov ↗Join the Familial primary hyperparathyroidism community →

Specialists

3 foundView all specialists →
LP
Laura C Hernández Ramírez, MD, PhD
Mexico City, Mexico City
Specialist

Rare Disease Specialist

PI on 1 active trial
BB
Barbara B Biesecker
CLARKS SUMMIT, PA
Specialist
PI on 16 active trials

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 Familial primary hyperparathyroidism.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Familial primary hyperparathyroidism

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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 gene mutation is causing my hyperparathyroidism, and does it put me at risk for other health problems?,Should my family members be tested for this genetic condition?,How many of my parathyroid glands are affected, and what type of surgery do you recommend?,What is the chance that my hyperparathyroidism will come back after surgery?,How often will I need blood tests and follow-up appointments after treatment?,Are there any other organs or glands I should be screened for tumors in?,If I choose not to have surgery right now, what are the risks and what monitoring do I need?

Common questions about Familial primary hyperparathyroidism

What is Familial primary hyperparathyroidism?

Familial primary hyperparathyroidism (FPHPT) is an inherited condition in which one or more of the parathyroid glands — four small glands located in the neck near the thyroid — become overactive and produce too much parathyroid hormone (PTH). This hormone normally controls calcium levels in the blood. When too much PTH is made, calcium levels rise above normal, a condition called hypercalcemia. Over time, high calcium can cause a wide range of problems throughout the body. Unlike the more common sporadic (non-inherited) form of primary hyperparathyroidism, the familial form runs in families a

How is Familial primary hyperparathyroidism inherited?

Familial primary hyperparathyroidism follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

Which specialists treat Familial primary hyperparathyroidism?

3 specialists and care centers treating Familial primary hyperparathyroidism are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.

What treatment and support options exist for Familial primary hyperparathyroidism?

1 patient support program are currently tracked on UniteRare for Familial primary hyperparathyroidism. See the treatments and support programs sections for copay assistance, eligibility, and contact details.