Dysostosis with combined reduction defects of upper and lower limbs

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:294957
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Dysostosis with combined reduction defects of upper and lower limbs is an extremely rare skeletal disorder that is present at birth. In this condition, parts of the bones in both the arms and legs do not form properly during development in the womb. The term "dysostosis" refers to abnormal bone formation, and "reduction defects" means that certain bones are partially or completely missing. This affects both the upper limbs (arms, hands, fingers) and lower limbs (legs, feet, toes), which distinguishes it from conditions where only one set of limbs is involved. Because the bones of the arms and legs are shortened, missing, or malformed, children born with this condition may have noticeably shorter or differently shaped limbs. The severity can vary widely — some individuals may have mild shortening of certain bones, while others may have more significant absence of limb segments. Fingers and toes may also be affected, with some being fused, missing, or unusually short. There is currently no cure for this condition. Treatment focuses on improving function and quality of life. This may include orthopedic surgery to correct bone alignment, prosthetic devices to assist with mobility and hand function, and physical and occupational therapy to help maximize independence. A team of specialists typically works together to create a personalized care plan for each affected individual.

Key symptoms:

Missing or shortened bones in the armsMissing or shortened bones in the legsAbsent or underdeveloped fingersAbsent or underdeveloped toesFused fingers or toesAbnormally shaped hands or feetLimb length differences between left and right sidesDifficulty gripping or holding objectsDifficulty walking or standingJoint stiffness or limited range of motionAbnormal bone shape visible on X-ray

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Dysostosis with combined reduction defects of upper and lower limbs.

View clinical trials →

No actively recruiting trials found for Dysostosis with combined reduction defects of upper and lower limbs at this time.

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

Search ClinicalTrials.gov ↗Join the Dysostosis with combined reduction defects of upper and lower limbs community →

No specialists are currently listed for Dysostosis with combined reduction defects of upper and lower limbs.

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 Dysostosis with combined reduction defects of upper and lower limbs.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Dysostosis with combined reduction defects of upper and lower limbsForum →

No community posts yet. Be the first to share your experience with Dysostosis with combined reduction defects of upper and lower limbs.

Start the conversation →

Latest news about Dysostosis with combined reduction defects of upper and lower limbs

No recent news articles for Dysostosis with combined reduction defects of upper and lower limbs.

Follow this condition to be notified when news becomes available.

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.What specific bones are affected in my child, and how severe are the differences?,Is genetic testing recommended, and what might it tell us about the cause?,What surgeries or procedures might be needed as my child grows?,When should we start physical and occupational therapy?,Would prosthetic devices be helpful, and when should we begin fitting them?,Are there any other organs or systems we should check for problems?,What is the chance that future children could be affected?

Common questions about Dysostosis with combined reduction defects of upper and lower limbs

What is Dysostosis with combined reduction defects of upper and lower limbs?

Dysostosis with combined reduction defects of upper and lower limbs is an extremely rare skeletal disorder that is present at birth. In this condition, parts of the bones in both the arms and legs do not form properly during development in the womb. The term "dysostosis" refers to abnormal bone formation, and "reduction defects" means that certain bones are partially or completely missing. This affects both the upper limbs (arms, hands, fingers) and lower limbs (legs, feet, toes), which distinguishes it from conditions where only one set of limbs is involved. Because the bones of the arms and

At what age does Dysostosis with combined reduction defects of upper and lower limbs typically begin?

Typical onset of Dysostosis with combined reduction defects of upper and lower limbs is neonatal. Age of onset can vary across affected individuals.