Acrocapitofemoral dysplasia

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ORPHA:63446OMIM:607778Q78.8
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Overview

Acrocapitofemoral dysplasia (ACFD) is a very rare inherited bone disorder that affects the way the skeleton grows and develops. The name comes from the parts of the body most affected: 'acro' refers to the hands and feet, 'capito' refers to the head of the upper arm bone, and 'femoral' refers to the top of the thigh bone. This condition is caused by changes (mutations) in the IHH gene, which plays an important role in how bones form and grow. People with ACFD are typically much shorter than average (short stature) because the long bones of the arms and legs do not grow to their full length. The bones in the hips and shoulders are often cone-shaped or abnormally formed, which can cause pain and limit movement. The hands and feet may also look shorter and broader than usual. Symptoms are usually noticed in early childhood when growth delays become apparent. There is currently no cure for ACFD. Treatment focuses on managing symptoms and improving quality of life. This may include physical therapy, pain management, and in some cases orthopedic surgery to address joint problems. A team of specialists including a medical geneticist, orthopedic surgeon, and endocrinologist typically work together to care for people with this condition.

Key symptoms:

Short stature (much shorter than average height)Short arms and legs relative to body sizeShort, broad hands and feetCone-shaped or abnormally formed hip jointsAbnormal shoulder joint developmentHip pain or stiffnessLimited range of motion in the hips and shouldersWaddling or unusual walking patternDelayed bone growth seen on X-raysShortened finger and toe bones

Clinical phenotype terms (21)— hover any for plain English
Small nailHP:0001792Broad nailHP:0001821Flared iliac wingHP:0002869Ovoid vertebral bodiesHP:0003300Abnormal femoral neck morphologyHP:0003367Cone-shaped metacarpal epiphysesHP:0006059
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 Acrocapitofemoral dysplasia.

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No actively recruiting trials found for Acrocapitofemoral dysplasia at this time.

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No specialists are currently listed for Acrocapitofemoral dysplasia.

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 Acrocapitofemoral dysplasia.

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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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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 specific changes were found in my (or my child's) IHH gene, and what do they mean for the future?,How often should we have X-rays or other imaging to monitor bone and joint health?,Is physical therapy recommended, and what type of exercises are safe?,At what point would you recommend considering surgery for hip or shoulder problems?,Are there any clinical trials or research studies we could participate in?,Should other family members be tested for this condition?,What signs should prompt us to seek urgent medical attention?

Common questions about Acrocapitofemoral dysplasia

What is Acrocapitofemoral dysplasia?

Acrocapitofemoral dysplasia (ACFD) is a very rare inherited bone disorder that affects the way the skeleton grows and develops. The name comes from the parts of the body most affected: 'acro' refers to the hands and feet, 'capito' refers to the head of the upper arm bone, and 'femoral' refers to the top of the thigh bone. This condition is caused by changes (mutations) in the IHH gene, which plays an important role in how bones form and grow. People with ACFD are typically much shorter than average (short stature) because the long bones of the arms and legs do not grow to their full length. T

How is Acrocapitofemoral dysplasia inherited?

Acrocapitofemoral dysplasia follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Acrocapitofemoral dysplasia typically begin?

Typical onset of Acrocapitofemoral dysplasia is childhood. Age of onset can vary across affected individuals.