Takenouchi-Kosaki syndrome

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ORPHA:487796OMIM:616737Q87.8
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8Treatment centers

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Overview

Takenouchi-Kosaki syndrome is a very rare genetic condition caused by a change (mutation) in a gene called CDC42. This gene plays an important role in how cells grow, divide, and communicate with each other. When CDC42 does not work properly, it affects the development of many parts of the body, especially the brain, heart, immune system, and blood cells. The syndrome is sometimes referred to as TKS for short. People with Takenouchi-Kosaki syndrome are usually identified in infancy or early childhood. Common features include intellectual disability, delayed development, distinctive facial features, and problems with the heart. Many individuals also have issues with their blood cells, such as a condition called thrombocytopenia (low platelet count), which can cause easy bruising or bleeding. Some people have hearing loss, vision problems, or a smaller-than-usual head size (microcephaly). There is currently no cure for Takenouchi-Kosaki syndrome. Treatment focuses on managing each person's specific symptoms. This may include therapies to support development, heart surgery if needed, and regular monitoring by a team of specialists. Because the condition is so rare, care is highly individualized and often guided by clinical geneticists working alongside other specialists.

Also known as:

Key symptoms:

Intellectual disability or learning difficultiesDelayed development (sitting, walking, talking later than expected)Distinctive facial features (wide-set eyes, flat nasal bridge, low-set ears)Low platelet count (thrombocytopenia), causing easy bruising or bleedingCongenital heart defectsSmall head size (microcephaly)Hearing lossVision problemsShort statureImmune system problems (frequent infections)Feeding difficulties in infancyLymphedema (swelling due to fluid buildup)Skin abnormalities

Clinical phenotype terms (50)— hover any for plain English
Abnormal sternum morphologyHP:0000766Abnormality of the endocrine systemHP:0000818Abnormal periventricular white matter morphologyHP:0002518Increased mean platelet volumeHP:0011877
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

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 Takenouchi-Kosaki syndrome.

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No actively recruiting trials found for Takenouchi-Kosaki syndrome at this time.

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Search ClinicalTrials.gov ↗Join the Takenouchi-Kosaki syndrome community →

No specialists are currently listed for Takenouchi-Kosaki 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 Takenouchi-Kosaki syndrome.

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Community

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Latest news about Takenouchi-Kosaki 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 specific mutation was found in the CDC42 gene, and what does it mean for my child's health?,Which specialists should be part of my child's care team, and how often should we see each one?,What signs of bleeding or infection should prompt an emergency visit?,Are there any clinical trials or research studies we could participate in?,What therapies (speech, physical, occupational) would benefit my child most right now?,Should other family members be tested, and what is the chance of this happening again in a future pregnancy?,What educational and community support services are available for my child?

Common questions about Takenouchi-Kosaki syndrome

What is Takenouchi-Kosaki syndrome?

Takenouchi-Kosaki syndrome is a very rare genetic condition caused by a change (mutation) in a gene called CDC42. This gene plays an important role in how cells grow, divide, and communicate with each other. When CDC42 does not work properly, it affects the development of many parts of the body, especially the brain, heart, immune system, and blood cells. The syndrome is sometimes referred to as TKS for short. People with Takenouchi-Kosaki syndrome are usually identified in infancy or early childhood. Common features include intellectual disability, delayed development, distinctive facial fea

How is Takenouchi-Kosaki syndrome inherited?

Takenouchi-Kosaki syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Takenouchi-Kosaki syndrome typically begin?

Typical onset of Takenouchi-Kosaki syndrome is neonatal. Age of onset can vary across affected individuals.