Autosomal recessive distal osteolysis syndrome

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ORPHA:2776OMIM:259610M89.5
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Overview

Autosomal recessive distal osteolysis syndrome is an extremely rare inherited bone disorder in which the bones at the ends (distal parts) of the fingers and toes gradually break down and dissolve over time. This process is called osteolysis, which literally means 'bone dissolving.' Because the condition follows an autosomal recessive inheritance pattern, a child must inherit a faulty copy of the responsible gene from both parents to develop the disease. The main features of this syndrome include progressive shortening and loss of the fingertips and toes, which may become painless or sometimes painful. The nails may also become abnormal or lost as the underlying bone disappears. In some cases, other bones in the hands and feet can be affected. The condition typically becomes noticeable in childhood, and the bone loss may slowly progress over the years. Because this condition is so rare, there is no specific cure or targeted treatment available. Management focuses on protecting the hands and feet from injury, managing pain if present, and using supportive devices or surgery when needed to maintain function. Regular monitoring by specialists is important to track the progression of bone changes and address complications early.

Also known as:

Key symptoms:

Gradual dissolving or shortening of finger bonesGradual dissolving or shortening of toe bonesLoss or deformity of fingernailsLoss or deformity of toenailsShortened fingersShortened toesSwelling of fingers or toesPain in the hands or feetReduced grip strengthDifficulty with fine motor tasks like buttoning clothesProgressive bone loss visible on X-rays

Clinical phenotype terms (9)— hover any for plain English
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 Autosomal recessive distal osteolysis syndrome.

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No actively recruiting trials found for Autosomal recessive distal osteolysis syndrome at this time.

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No specialists are currently listed for Autosomal recessive distal osteolysis syndrome.

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 Autosomal recessive distal osteolysis syndrome.

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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.How quickly is the bone loss expected to progress in my case or my child's case?,Are there any treatments that can slow down the bone dissolving process?,Should we pursue genetic testing, and what might it tell us?,What adaptive devices or therapies can help maintain hand and foot function?,What is the risk that our other children could also have this condition?,Are there any clinical trials or research studies we could participate in?,How often should we have X-rays or follow-up visits to monitor the condition?

Common questions about Autosomal recessive distal osteolysis syndrome

What is Autosomal recessive distal osteolysis syndrome?

Autosomal recessive distal osteolysis syndrome is an extremely rare inherited bone disorder in which the bones at the ends (distal parts) of the fingers and toes gradually break down and dissolve over time. This process is called osteolysis, which literally means 'bone dissolving.' Because the condition follows an autosomal recessive inheritance pattern, a child must inherit a faulty copy of the responsible gene from both parents to develop the disease. The main features of this syndrome include progressive shortening and loss of the fingertips and toes, which may become painless or sometimes

How is Autosomal recessive distal osteolysis syndrome inherited?

Autosomal recessive distal osteolysis syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Autosomal recessive distal osteolysis syndrome typically begin?

Typical onset of Autosomal recessive distal osteolysis syndrome is childhood. Age of onset can vary across affected individuals.