Osteopenia-intellectual disability-sparse hair syndrome

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ORPHA:2324OMIM:259690Q87.5
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Overview

Osteopenia-intellectual disability-sparse hair syndrome is an extremely rare genetic condition that affects multiple body systems. The syndrome is sometimes referred to by its Orphanet designation (ORPHA:2324) and is characterized by a combination of weakened bones (osteopenia), learning and thinking difficulties (intellectual disability), and thin or sparse hair. Individuals with this condition typically show signs from early childhood. The hallmark features include bones that are less dense than normal, making them more fragile and prone to fractures. Children with this syndrome often experience delays in reaching developmental milestones such as walking and talking, and they may have mild to moderate intellectual disability. The sparse hair is noticeable from infancy and may affect the scalp, eyebrows, and eyelashes. Some affected individuals may also have distinctive facial features, short stature, and other skeletal abnormalities. Because this condition is so rare, there is no specific cure or targeted treatment. Management focuses on addressing individual symptoms, such as physical therapy for motor delays, educational support for learning difficulties, and monitoring bone health to prevent fractures. A team of specialists typically works together to provide the best possible care for affected individuals.

Also known as:

Key symptoms:

Weak or thin bones (osteopenia)Intellectual disability or learning difficultiesThin or sparse hair on the scalpDelayed developmental milestonesShort statureBone fractures from minor injuriesSparse eyebrows or eyelashesDistinctive facial featuresDelayed speech developmentLow muscle toneSkeletal abnormalities

Clinical phenotype terms (22)— hover any for plain English
High-frequency sensorineural hearing impairmentHP:0001757
Inheritance

Autosomal recessive

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

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Osteopenia-intellectual disability-sparse hair syndrome.

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No actively recruiting trials found for Osteopenia-intellectual disability-sparse hair syndrome at this time.

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No specialists are currently listed for Osteopenia-intellectual disability-sparse hair syndrome.

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 Osteopenia-intellectual disability-sparse hair syndrome.

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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

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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.What is the severity of my child's bone weakness, and how can we best protect against fractures?,Should my child have genetic testing, and what can it tell us about this condition?,What therapies (physical, speech, occupational) do you recommend and how often?,Are there medications that can help strengthen my child's bones?,What kind of educational support should we arrange for my child?,How often should bone density be monitored?,Is there a risk that other family members could be affected?

Common questions about Osteopenia-intellectual disability-sparse hair syndrome

What is Osteopenia-intellectual disability-sparse hair syndrome?

Osteopenia-intellectual disability-sparse hair syndrome is an extremely rare genetic condition that affects multiple body systems. The syndrome is sometimes referred to by its Orphanet designation (ORPHA:2324) and is characterized by a combination of weakened bones (osteopenia), learning and thinking difficulties (intellectual disability), and thin or sparse hair. Individuals with this condition typically show signs from early childhood. The hallmark features include bones that are less dense than normal, making them more fragile and prone to fractures. Children with this syndrome often exper

How is Osteopenia-intellectual disability-sparse hair syndrome inherited?

Osteopenia-intellectual disability-sparse hair syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Osteopenia-intellectual disability-sparse hair syndrome typically begin?

Typical onset of Osteopenia-intellectual disability-sparse hair syndrome is infantile. Age of onset can vary across affected individuals.