Metaphyseal dysplasia without hypotrichosis

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ORPHA:1838OMIM:250460
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Overview

Metaphyseal dysplasia without hypotrichosis is an extremely rare inherited bone disorder that primarily affects the growing ends (metaphyses) of long bones. The name distinguishes it from a related condition called metaphyseal dysplasia with hypotrichosis (McKusick type or cartilage-hair hypoplasia), because in this form, patients do not have the thin or sparse hair (hypotrichosis) that is seen in the related condition. The condition affects how bones grow and develop during childhood, leading to abnormalities visible on X-rays at the metaphyses — the flared portions of long bones near the growth plates. Patients may experience short stature, bowing of the legs, a waddling gait, and joint problems. The severity can vary from mild skeletal changes found incidentally on X-rays to more noticeable limb shortening and deformity. Because this condition is so rare, the treatment landscape is limited and primarily supportive. There is no cure, and management focuses on orthopedic care, physical therapy, and monitoring growth. Surgical correction may be considered for significant limb deformities. Regular follow-up with specialists is important to track bone development and address complications as they arise.

Also known as:

Key symptoms:

Short statureBowing of the legsWaddling gaitWidened or irregular ends of long bones on X-rayJoint stiffness or limited range of motionLimb shorteningKnock knees or bow legsMild to moderate skeletal deformityNormal hair growth (distinguishing it from related conditions)Possible mild joint pain

Inheritance

Autosomal dominant

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Metaphyseal dysplasia without hypotrichosis.

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No actively recruiting trials found for Metaphyseal dysplasia without hypotrichosis at this time.

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No specialists are currently listed for Metaphyseal dysplasia without hypotrichosis.

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 Metaphyseal dysplasia without hypotrichosis.

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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.How severe is my child's bone involvement, and what changes should I watch for?,Will my child need surgery for leg bowing or other bone deformities?,How will this condition affect my child's final adult height?,Should we pursue genetic testing, and what would the results mean for our family?,What physical activities are safe, and are there any that should be avoided?,How often should we schedule follow-up X-rays and orthopedic visits?,Is there a risk that other family members could be affected?

Common questions about Metaphyseal dysplasia without hypotrichosis

What is Metaphyseal dysplasia without hypotrichosis?

Metaphyseal dysplasia without hypotrichosis is an extremely rare inherited bone disorder that primarily affects the growing ends (metaphyses) of long bones. The name distinguishes it from a related condition called metaphyseal dysplasia with hypotrichosis (McKusick type or cartilage-hair hypoplasia), because in this form, patients do not have the thin or sparse hair (hypotrichosis) that is seen in the related condition. The condition affects how bones grow and develop during childhood, leading to abnormalities visible on X-rays at the metaphyses — the flared portions of long bones near the gro

How is Metaphyseal dysplasia without hypotrichosis inherited?

Metaphyseal dysplasia without hypotrichosis follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Metaphyseal dysplasia without hypotrichosis typically begin?

Typical onset of Metaphyseal dysplasia without hypotrichosis is childhood. Age of onset can vary across affected individuals.