Angel-shaped phalango-epiphyseal dysplasia

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ORPHA:63442OMIM:105835Q78.8
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Overview

Angel-shaped phalango-epiphyseal dysplasia, often shortened to ASPED, is a very rare inherited bone disorder that mainly affects the growth plates and small bones of the fingers and toes. The name comes from the distinctive 'angel-wing' shape that the middle finger bones (called the middle phalanges) take on when seen on X-ray. This unusual shape happens because the growth plates at both ends of these small bones develop abnormally during childhood. ASPED belongs to a group of conditions called skeletal dysplasias, meaning the skeleton does not form or grow in the typical way. People with ASPED usually have short, stubby fingers and toes, and the joints in the hands and feet may look different from usual. Some individuals also have delayed bone age, meaning their bones mature more slowly than expected for their age. Hip problems, including a condition called hip dysplasia, have also been reported in some patients. Because ASPED is so rare, the full range of symptoms is still being understood. Most people with ASPED do not have intellectual disability and can live full, active lives. Treatment focuses on managing symptoms, monitoring bone and joint health, and addressing any complications as they arise. There is no cure at this time, but regular follow-up with a specialist can help manage the condition effectively.

Also known as:

Key symptoms:

Short, stubby fingers and toesAbnormally shaped middle finger bones (angel-wing appearance on X-ray)Delayed bone maturation (bones develop more slowly than usual)Hip joint problems or hip dysplasiaShort stature in some individualsJoint stiffness or limited movement in the fingersBroad or widened fingertipsAbnormal growth plates in the hands and feet

Clinical phenotype terms (11)— hover any for plain English
Short middle phalanx of the 5th fingerHP:0004220Short middle phalanx of fingerHP:0005819Short 1st metacarpalHP:0010034Hip osteoarthritisHP:0008843
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 Angel-shaped phalango-epiphyseal dysplasia.

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No actively recruiting trials found for Angel-shaped phalango-epiphyseal dysplasia at this time.

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No specialists are currently listed for Angel-shaped phalango-epiphyseal 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 Angel-shaped phalango-epiphyseal dysplasia.

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Community

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What X-rays or other tests do you recommend to fully assess my (or my child's) bone health right now?,Should other family members be tested for ASPED, and how is it inherited in our family?,What signs of hip problems should I watch for, and how often should the hips be monitored?,Are there any physical activities or sports that should be avoided or encouraged?,Would physical therapy or occupational therapy be helpful, and what should we focus on?,Are there any clinical trials or research studies we could participate in?,What is the long-term outlook, and what complications are most important to watch for as my child grows?

Common questions about Angel-shaped phalango-epiphyseal dysplasia

What is Angel-shaped phalango-epiphyseal dysplasia?

Angel-shaped phalango-epiphyseal dysplasia, often shortened to ASPED, is a very rare inherited bone disorder that mainly affects the growth plates and small bones of the fingers and toes. The name comes from the distinctive 'angel-wing' shape that the middle finger bones (called the middle phalanges) take on when seen on X-ray. This unusual shape happens because the growth plates at both ends of these small bones develop abnormally during childhood. ASPED belongs to a group of conditions called skeletal dysplasias, meaning the skeleton does not form or grow in the typical way. People with ASP

How is Angel-shaped phalango-epiphyseal dysplasia inherited?

Angel-shaped phalango-epiphyseal dysplasia follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Angel-shaped phalango-epiphyseal dysplasia typically begin?

Typical onset of Angel-shaped phalango-epiphyseal dysplasia is childhood. Age of onset can vary across affected individuals.