KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome

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ORPHA:457193OMIM:616268Q87.8
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Overview

KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome is a rare genetic condition caused by changes (mutations) in the KAT6A or KAT6B genes. These genes play an important role in how the body reads and uses its DNA during development. When one of these genes does not work properly, it can affect brain development, facial features, and the heart. Children with this syndrome typically have intellectual disability that ranges from mild to severe, along with speech and language delays. Facial features may include a broad or prominent forehead, widely spaced eyes, a flat nasal bridge, a thin upper lip, and low-set or unusually shaped ears. Many children also have heart defects present at birth, such as holes between heart chambers or other structural problems. Other common features include low muscle tone (hypotonia), feeding difficulties in infancy, and delayed motor milestones like sitting and walking. There is currently no cure for this condition. Treatment focuses on managing individual symptoms and supporting development. This may include speech therapy, occupational therapy, physical therapy, special education services, and surgical repair of heart defects when needed. Early intervention programs can help children reach their full potential. Regular follow-up with a team of specialists is important to monitor growth, development, and any medical complications that may arise over time.

Also known as:

Key symptoms:

Intellectual disabilitySpeech and language delaysLow muscle tone (floppiness)Heart defects present at birthFeeding difficulties in infancyDelayed motor milestones like sitting and walkingBroad or prominent foreheadWidely spaced eyesFlat nasal bridgeThin upper lipLow-set or unusually shaped earsShort stature or slow growthBehavioral challengesRecurrent infectionsVision or eye problems

Clinical phenotype terms (39)— hover any for plain English
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome.

View clinical trials →

No actively recruiting trials found for KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome community →

No specialists are currently listed for KAT6-related intellectual disability-craniofacial anomalies-cardiac defects 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 KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome

1 articles
NewsCLINICALTRIALS.GOVFeb 20, 2026
New recruiting trial: FACE.S-4-KIDS : A Deep Phenotyping Database of Craniofacial Anomalies During Development With 4 Pilot Projects
FACE.S-4-KIDS is an ambitious database project addressing the scientific question of the variable expression of craniofacial disorders in humans, to reach a sou
See all news about KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome

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 specific genetic change does my child have, and how does it affect the expected course of the condition?,Does my child have any heart defects that need treatment or monitoring?,What therapies should we start right away, and how often should they occur?,Are there any clinical trials or research studies my child might be eligible for?,What developmental milestones should I watch for, and when should I be concerned?,How often should my child see each specialist, and what routine tests are recommended?,What is the chance of this condition occurring again in future pregnancies?

Common questions about KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome

What is KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome?

KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome is a rare genetic condition caused by changes (mutations) in the KAT6A or KAT6B genes. These genes play an important role in how the body reads and uses its DNA during development. When one of these genes does not work properly, it can affect brain development, facial features, and the heart. Children with this syndrome typically have intellectual disability that ranges from mild to severe, along with speech and language delays. Facial features may include a broad or prominent forehead, widely spaced eyes, a

How is KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome inherited?

KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome typically begin?

Typical onset of KAT6-related intellectual disability-craniofacial anomalies-cardiac defects syndrome is neonatal. Age of onset can vary across affected individuals.