1p21.3 microdeletion syndrome

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ORPHA:293948Q93.5
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Overview

1p21.3 microdeletion syndrome is a rare genetic condition caused by a small missing piece (deletion) of chromosome 1 at a specific location called 1p21.3. This syndrome is sometimes also referred to as chromosome 1p21.3 deletion syndrome. Because this region of the chromosome contains genes important for brain development and function, the condition primarily affects learning, speech, and behavior. People with this syndrome typically experience intellectual disability that ranges from mild to moderate, along with significant delays in speech and language development. Many children are late to reach developmental milestones such as sitting, walking, and talking. Behavioral challenges, including features of autism spectrum disorder, are commonly reported. Some individuals may also have distinctive facial features, though these can be subtle and vary from person to person. There is currently no cure for 1p21.3 microdeletion syndrome. Treatment focuses on managing symptoms and supporting development through early intervention programs, speech therapy, occupational therapy, and behavioral support. With appropriate therapies and educational accommodations, many individuals can make meaningful progress in their development and quality of life. The DPYD gene and other genes in this region are thought to play a role in the features seen in this condition.

Also known as:

Key symptoms:

Intellectual disability (mild to moderate)Delayed speech and language developmentDelayed motor milestones like walkingBehavioral difficultiesFeatures of autism spectrum disorderLow muscle tone (feeling floppy as a baby)Subtle distinctive facial featuresLearning difficulties in schoolAttention problemsAnxiety or emotional challengesFeeding difficulties in infancyShort stature in some casesSeizures in some individuals

Clinical phenotype terms (25)— hover any for plain English
Self-mutilationHP:0000742
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for 1p21.3 microdeletion syndrome.

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No actively recruiting trials found for 1p21.3 microdeletion syndrome at this time.

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No specialists are currently listed for 1p21.3 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 1p21.3 microdeletion syndrome.

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Community

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Latest news about 1p21.3 microdeletion syndrome

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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, and how often?,Should my child be screened for seizures or other medical complications?,What educational supports and accommodations should we request at school?,Are there any clinical studies or research programs we could participate in?,What is the chance of this happening again in a future pregnancy?

Common questions about 1p21.3 microdeletion syndrome

What is 1p21.3 microdeletion syndrome?

1p21.3 microdeletion syndrome is a rare genetic condition caused by a small missing piece (deletion) of chromosome 1 at a specific location called 1p21.3. This syndrome is sometimes also referred to as chromosome 1p21.3 deletion syndrome. Because this region of the chromosome contains genes important for brain development and function, the condition primarily affects learning, speech, and behavior. People with this syndrome typically experience intellectual disability that ranges from mild to moderate, along with significant delays in speech and language development. Many children are late to

At what age does 1p21.3 microdeletion syndrome typically begin?

Typical onset of 1p21.3 microdeletion syndrome is infantile. Age of onset can vary across affected individuals.