Xp22.13p22.2 duplication syndrome

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ORPHA:284180Q99.8
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Overview

Xp22.13p22.2 duplication syndrome is a very rare genetic condition caused by having an extra copy of a small piece of the short arm of the X chromosome, specifically in the region labeled p22.13 to p22.2. This extra genetic material disrupts the normal function of genes in that region, which can affect brain development and other body systems. Because the duplication is on the X chromosome, it tends to affect males more severely than females, though females who carry the duplication can also show symptoms. The main features of this syndrome include intellectual disability, developmental delays (especially in speech and language), behavioral difficulties, and sometimes distinctive facial features. Some individuals may also experience seizures, low muscle tone, or problems with coordination. The severity of symptoms can vary widely from person to person, even within the same family, depending on the exact size of the duplication and which genes are affected. There is currently no cure for this condition. Treatment focuses on managing individual symptoms and supporting development. This typically includes speech therapy, occupational therapy, physical therapy, special education services, and behavioral support. Seizures, if present, are managed with anti-seizure medications. Early intervention services can make a meaningful difference in a child's development and quality of life.

Also known as:

Key symptoms:

Intellectual disabilityDelayed speech and language developmentDelayed motor milestones like sitting and walkingLow muscle tone (feeling floppy)Behavioral problems such as hyperactivity or aggressionSeizures or epilepsyDistinctive facial featuresLearning difficultiesPoor coordination or clumsinessAttention difficultiesAnxiety or mood problemsFeeding difficulties in infancyShort stature in some cases

Clinical phenotype terms (34)— hover any for plain English
Flared nostrilsHP:0000454
Inheritance

X-linked dominant

Carried on the X chromosome; a single copy can cause the condition

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 Xp22.13p22.2 duplication syndrome.

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No actively recruiting trials found for Xp22.13p22.2 duplication syndrome at this time.

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No specialists are currently listed for Xp22.13p22.2 duplication 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 Xp22.13p22.2 duplication syndrome.

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Community

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Caregiver Resources

NORD Caregiver Resources

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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 and location of my child's duplication, and which genes are affected?,Was this duplication inherited or did it happen for the first time in my child?,Should other family members be tested for this duplication?,What therapies and early intervention services do you recommend starting right away?,What should I watch for in terms of seizures or other new symptoms?,How often should my child have developmental assessments?,Are there any research studies or clinical trials that might be relevant for this condition?

Common questions about Xp22.13p22.2 duplication syndrome

What is Xp22.13p22.2 duplication syndrome?

Xp22.13p22.2 duplication syndrome is a very rare genetic condition caused by having an extra copy of a small piece of the short arm of the X chromosome, specifically in the region labeled p22.13 to p22.2. This extra genetic material disrupts the normal function of genes in that region, which can affect brain development and other body systems. Because the duplication is on the X chromosome, it tends to affect males more severely than females, though females who carry the duplication can also show symptoms. The main features of this syndrome include intellectual disability, developmental delay

How is Xp22.13p22.2 duplication syndrome inherited?

Xp22.13p22.2 duplication syndrome follows a x-linked dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Xp22.13p22.2 duplication syndrome typically begin?

Typical onset of Xp22.13p22.2 duplication syndrome is infantile. Age of onset can vary across affected individuals.