Epiphyseal stippling-osteoclastic hyperplasia syndrome

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ORPHA:1952OMIM:167220Q77.8
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Overview

Epiphyseal stippling-osteoclastic hyperplasia syndrome is an extremely rare skeletal disorder that affects how bones grow and develop. The condition is characterized by two main features: epiphyseal stippling, which means there are small, dot-like calcium deposits (called punctate calcifications) in the growing ends of bones visible on X-rays, and osteoclastic hyperplasia, which refers to an overactivity of the cells that normally break down and remodel bone (called osteoclasts). This combination leads to abnormal bone development and can cause skeletal deformities, short stature, and joint problems. Because the growing ends of bones (epiphyses) are affected, children with this condition may experience difficulties with limb growth and movement. The syndrome falls under the broader category of skeletal dysplasias classified as ICD-10 code Q77.8 (other osteochondrodysplasias with defects of growth of tubular bones and spine). Due to the extreme rarity of this condition, the treatment approach is mainly supportive and symptom-based, focusing on managing skeletal complications, improving mobility, and monitoring growth. There is currently no cure or disease-specific therapy available. Orthopedic interventions and physical therapy are typically the mainstays of management.

Also known as:

Key symptoms:

Abnormal bone development visible on X-raysSmall dot-like calcium deposits at the ends of bonesShort stature or growth problemsJoint stiffness or limited range of motionSkeletal deformitiesLimb shortening or asymmetryDelayed motor milestonesBone pain or discomfortAbnormal bone remodeling

Clinical phenotype terms (10)— hover any for plain English
Abnormal calvaria morphologyHP:0002683Coronal cleft vertebraeHP:0003417Lethal skeletal dysplasiaHP:0005716Epiphyseal stipplingHP:0010655Abnormal bone ossificationHP:0011849Coarse metaphyseal trabecularizationHP:0100670
Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Epiphyseal stippling-osteoclastic hyperplasia syndrome.

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No actively recruiting trials found for Epiphyseal stippling-osteoclastic hyperplasia syndrome at this time.

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No specialists are currently listed for Epiphyseal stippling-osteoclastic hyperplasia syndrome.

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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 Epiphyseal stippling-osteoclastic hyperplasia syndrome.

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Community

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Latest news about Epiphyseal stippling-osteoclastic hyperplasia syndrome

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What is the expected course of this condition for my child specifically?,How often should we have X-rays or other imaging to monitor bone development?,Are there any genetic tests that could help identify the exact cause?,What therapies or interventions can help my child's mobility and development?,Should we be concerned about any other organs being affected?,Are there any clinical trials or research studies we could participate in?,What signs or symptoms should prompt us to seek emergency care?

Common questions about Epiphyseal stippling-osteoclastic hyperplasia syndrome

What is Epiphyseal stippling-osteoclastic hyperplasia syndrome?

Epiphyseal stippling-osteoclastic hyperplasia syndrome is an extremely rare skeletal disorder that affects how bones grow and develop. The condition is characterized by two main features: epiphyseal stippling, which means there are small, dot-like calcium deposits (called punctate calcifications) in the growing ends of bones visible on X-rays, and osteoclastic hyperplasia, which refers to an overactivity of the cells that normally break down and remodel bone (called osteoclasts). This combination leads to abnormal bone development and can cause skeletal deformities, short stature, and joint pr

At what age does Epiphyseal stippling-osteoclastic hyperplasia syndrome typically begin?

Typical onset of Epiphyseal stippling-osteoclastic hyperplasia syndrome is neonatal. Age of onset can vary across affected individuals.