OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type

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ORPHA:953
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Overview

Acromesomelic dysplasia, Brahimi-Bacha type, is an extremely rare inherited skeletal disorder that was previously classified under its own Orphanet code (953) but has since been marked as obsolete, meaning it has been reclassified or merged with another recognized form of acromesomelic dysplasia. Acromesomelic dysplasias are a group of conditions that cause short stature due to abnormal growth of the bones, particularly affecting the middle (mesomelic) and end (acromelic) portions of the limbs — meaning the forearms, lower legs, hands, and feet are disproportionately short compared to the rest of the body. The Brahimi-Bacha type was described in a very small number of families and shares many features with other forms of acromesomelic dysplasia, including short fingers and toes, limited joint movement, and noticeably short forearms and lower legs. Because this classification is now obsolete, patients originally given this diagnosis may be reclassified under another form of acromesomelic dysplasia, such as the Maroteaux type, the Hunter-Thompson type, or the Grebe type, depending on their specific genetic findings and clinical features. There is no cure for acromesomelic dysplasias. Treatment focuses on managing symptoms, supporting mobility, and monitoring growth. Orthopedic care and physical therapy are the main approaches to help maintain function and quality of life.

Key symptoms:

Short stature (noticeably shorter than average height)Disproportionately short forearmsDisproportionately short lower legsShort fingers and toes (brachydactyly)Short hands and feetLimited range of motion in jointsBowing of the forearm bonesAbnormal shape of the vertebrae (spine bones)Waddling gait or unusual walking patternJoint stiffnessBroad hands

Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type.

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No actively recruiting trials found for OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type at this time.

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No specialists are currently listed for OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type.

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 OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type.

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Since this diagnosis is now considered obsolete, what is the current correct name for my condition?,Has genetic testing been done to identify the specific gene causing this condition?,What specialists should be part of my or my child's care team?,Are there any treatments or clinical trials that could help with growth or joint problems?,What accommodations should we request at school or work?,What is the expected adult height, and how will this affect daily life?,If we plan to have more children, what is the chance they will also be affected?

Common questions about OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type

What is OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type?

Acromesomelic dysplasia, Brahimi-Bacha type, is an extremely rare inherited skeletal disorder that was previously classified under its own Orphanet code (953) but has since been marked as obsolete, meaning it has been reclassified or merged with another recognized form of acromesomelic dysplasia. Acromesomelic dysplasias are a group of conditions that cause short stature due to abnormal growth of the bones, particularly affecting the middle (mesomelic) and end (acromelic) portions of the limbs — meaning the forearms, lower legs, hands, and feet are disproportionately short compared to the rest

How is OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type inherited?

OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type typically begin?

Typical onset of OBSOLETE: Acromesomelic dysplasia, Brahimi-Bacha type is neonatal. Age of onset can vary across affected individuals.