Kosaki overgrowth syndrome

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ORPHA:477831OMIM:616592Q87.3
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Overview

Kosaki overgrowth syndrome is a rare genetic condition caused by changes in the PDGFRB gene. It belongs to a group of disorders called overgrowth syndromes, meaning that children with this condition tend to grow larger or taller than expected. The syndrome was first described by Japanese geneticist Kosaki and colleagues, and it is sometimes referred to as PDGFRB-related overgrowth syndrome. The condition affects multiple parts of the body. Children typically show signs from birth or early infancy, including being larger than average, having distinctive facial features, and showing differences in how their muscles and connective tissues develop. Some children also experience intellectual disability or developmental delays, meaning they may take longer to reach milestones like talking or walking. Right now, there is no cure for Kosaki overgrowth syndrome. Treatment focuses on managing individual symptoms, such as physical therapy for movement difficulties, educational support for learning challenges, and regular monitoring by a team of specialists. Because this condition is very rare, most of what doctors know comes from a small number of reported cases, and research is still ongoing.

Also known as:

Key symptoms:

Overgrowth — being larger or taller than expected for ageDistinctive facial features such as a broad forehead or widely spaced eyesIntellectual disability or learning difficultiesDevelopmental delays, such as late talking or walkingMuscle tone problems (muscles may be too loose or too stiff)Connective tissue differences affecting joints or skinEnlarged head size (macrocephaly)Feeding difficulties in infancyBehavioral challenges

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 Kosaki overgrowth syndrome.

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

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No specialists are currently listed for Kosaki overgrowth 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 Kosaki overgrowth syndrome.

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Community

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Latest news about Kosaki overgrowth syndrome

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Caregiver Resources

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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 change was found in the PDGFRB gene, and what does it mean for my child's health?,What therapies should we start right away, and how often should my child receive them?,Are there any other health problems we should watch for as my child grows?,Should other family members be tested for this gene change?,Are there any clinical trials or research studies we could participate in?,What educational supports should we request for my child at school?,How often should my child be seen by the specialist team, and which specialists are most important?

Common questions about Kosaki overgrowth syndrome

What is Kosaki overgrowth syndrome?

Kosaki overgrowth syndrome is a rare genetic condition caused by changes in the PDGFRB gene. It belongs to a group of disorders called overgrowth syndromes, meaning that children with this condition tend to grow larger or taller than expected. The syndrome was first described by Japanese geneticist Kosaki and colleagues, and it is sometimes referred to as PDGFRB-related overgrowth syndrome. The condition affects multiple parts of the body. Children typically show signs from birth or early infancy, including being larger than average, having distinctive facial features, and showing differences

How is Kosaki overgrowth syndrome inherited?

Kosaki overgrowth syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Kosaki overgrowth syndrome typically begin?

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