9p13 microdeletion syndrome

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ORPHA:324313Q93.5
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Overview

9p13 microdeletion syndrome is a very rare genetic condition caused by a small missing piece (microdeletion) of chromosome 9 in the region known as 9p13. Because this deletion removes one or more genes that are important for normal development, it can affect multiple body systems. The syndrome is sometimes referred to as chromosome 9p13 microdeletion syndrome or interstitial deletion 9p13. Children born with this condition typically show developmental delay, intellectual disability that ranges from mild to moderate, and speech and language difficulties. Many affected individuals also have distinctive facial features, which may include a broad forehead, widely spaced eyes, a flat nasal bridge, and low-set ears. Some children may have behavioral challenges such as attention difficulties or features on the autism spectrum. Growth problems, including short stature, and various birth defects involving the heart, kidneys, or skeleton have also been reported in some cases. Because this syndrome is so rare, there is no single standard treatment. Management focuses on addressing each individual's specific symptoms through early intervention programs, speech therapy, occupational therapy, physical therapy, and special education support. Regular monitoring by a team of specialists helps ensure that medical complications are identified and treated promptly. Research into the specific genes within the 9p13 region continues to improve our understanding of this condition.

Also known as:

Key symptoms:

Developmental delayIntellectual disability (mild to moderate)Speech and language delaysDistinctive facial featuresShort stature or growth problemsLow muscle tone (floppiness in infancy)Behavioral difficulties such as attention problemsFeeding difficulties in infancyHeart defects in some casesKidney or urinary tract abnormalitiesSkeletal differencesWidely spaced eyesSmall head size (microcephaly) in some casesSeizures in some individuals

Clinical phenotype terms (32)— hover any for plain English
BruxismHP:0003763Abnormal external ear cartilage morphologyHP:3000022
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for 9p13 microdeletion syndrome.

View clinical trials →

No actively recruiting trials found for 9p13 microdeletion syndrome at this time.

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Search ClinicalTrials.gov ↗Join the 9p13 microdeletion syndrome community →

No specialists are currently listed for 9p13 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 9p13 microdeletion syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about 9p13 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

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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 deletion, and which genes are affected?,Was this deletion inherited or did it happen for the first time in my child?,What screening tests should be done for the heart, kidneys, and other organs?,What early intervention therapies do you recommend starting right away?,How often should my child have developmental assessments?,Are there any clinical studies or registries we should consider joining?,What is the chance of this happening again in a future pregnancy?

Common questions about 9p13 microdeletion syndrome

What is 9p13 microdeletion syndrome?

9p13 microdeletion syndrome is a very rare genetic condition caused by a small missing piece (microdeletion) of chromosome 9 in the region known as 9p13. Because this deletion removes one or more genes that are important for normal development, it can affect multiple body systems. The syndrome is sometimes referred to as chromosome 9p13 microdeletion syndrome or interstitial deletion 9p13. Children born with this condition typically show developmental delay, intellectual disability that ranges from mild to moderate, and speech and language difficulties. Many affected individuals also have dis

At what age does 9p13 microdeletion syndrome typically begin?

Typical onset of 9p13 microdeletion syndrome is neonatal. Age of onset can vary across affected individuals.