8q21.11 microdeletion syndrome

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ORPHA:284160OMIM:614230Q93.5
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Overview

8q21.11 microdeletion syndrome is a very rare genetic condition caused by a small missing piece (deletion) of chromosome 8 in the region known as 8q21.11. Because this deletion removes one or more genes that are important for normal development, it can affect how the brain and body grow and function. The syndrome is sometimes referred to as 8q21.11 deletion syndrome or chromosome 8q21.11 microdeletion. People with this condition typically show intellectual disability or developmental delay, meaning they may take longer to reach milestones like sitting, walking, and talking. Speech and language development are often significantly affected. Some individuals may also have distinctive facial features, behavioral challenges, and problems with muscle tone (either too floppy or too stiff). Seizures have been reported in some cases. The severity of symptoms can vary from person to person depending on the exact size of the deletion and which genes are missing. Because this syndrome is so rare, there is no specific cure or targeted treatment. Management focuses on supportive therapies such as speech therapy, occupational therapy, physical therapy, and special education services. Seizures, if present, are treated with standard anti-seizure medications. Early intervention programs can help children reach their full potential. A team of specialists typically works together to address the various needs of affected individuals.

Also known as:

Key symptoms:

Intellectual disabilityDelayed speech and language developmentDelayed motor milestones such as sitting and walkingLow muscle tone (floppiness)Distinctive facial featuresBehavioral difficultiesSeizures or epilepsyLearning difficultiesShort stature or growth delayFeeding difficulties in infancyAttention problemsPoor coordination

Clinical phenotype terms (39)— hover any for plain English
Exaggerated cupid's bowHP:0002263SclerocorneaHP:0000647
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for 8q21.11 microdeletion syndrome.

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No actively recruiting trials found for 8q21.11 microdeletion syndrome at this time.

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No specialists are currently listed for 8q21.11 microdeletion 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 8q21.11 microdeletion syndrome.

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Community

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

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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 is the exact size of my child's deletion and which genes are affected?,Was this deletion inherited or did it happen for the first time in my child?,What therapies should we start right away to support development?,Should we be watching for seizures, and what should we do if one happens?,How often should my child have follow-up appointments with specialists?,Are there any clinical trials or research studies we could participate in?,What is the long-term outlook for my child's independence and quality of life?

Common questions about 8q21.11 microdeletion syndrome

What is 8q21.11 microdeletion syndrome?

8q21.11 microdeletion syndrome is a very rare genetic condition caused by a small missing piece (deletion) of chromosome 8 in the region known as 8q21.11. Because this deletion removes one or more genes that are important for normal development, it can affect how the brain and body grow and function. The syndrome is sometimes referred to as 8q21.11 deletion syndrome or chromosome 8q21.11 microdeletion. People with this condition typically show intellectual disability or developmental delay, meaning they may take longer to reach milestones like sitting, walking, and talking. Speech and languag

How is 8q21.11 microdeletion syndrome inherited?

8q21.11 microdeletion syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does 8q21.11 microdeletion syndrome typically begin?

Typical onset of 8q21.11 microdeletion syndrome is infantile. Age of onset can vary across affected individuals.