WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome

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ORPHA:466943OMIM:616708Q87.0
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1Active trials1Specialists8Treatment centers

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Overview

WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome is a rare genetic condition caused by changes (mutations) in the WAC gene. This syndrome is sometimes called DeSanto-Shinawi syndrome. It affects multiple parts of the body, but the most noticeable features involve facial appearance, learning and development, and behavior. Children with this condition typically have distinctive facial features that may include a broad forehead, widely spaced eyes, a thin upper lip, and a short nose. Developmental delays are common, meaning children may take longer to reach milestones like sitting, walking, and talking. Many children have mild to moderate intellectual disability and may struggle with speech and language. Behavioral issues are also a key part of this syndrome and can include attention deficit hyperactivity disorder (ADHD), anxiety, aggression, and autism-like behaviors. Other features can include low muscle tone (hypotonia), feeding difficulties in infancy, growth problems, and occasionally seizures. There is currently no cure for this condition. Treatment focuses on managing individual symptoms through therapies such as speech therapy, occupational therapy, physical therapy, and behavioral support. Early intervention services can make a meaningful difference in a child's development and quality of life.

Also known as:

Key symptoms:

Distinctive facial features such as broad forehead and widely spaced eyesDelayed speech and language developmentDelayed motor milestones like walkingMild to moderate intellectual disabilityLow muscle tone (floppiness)Attention deficit hyperactivity disorder (ADHD)AnxietyAggressive behaviorAutism-like behaviorsFeeding difficulties in infancyShort stature or growth delaysSeizures in some casesSleep problemsThin upper lip and short nose

Clinical phenotype terms (50)— hover any for plain English
Square faceHP:0000321Gastrointestinal dysmotilityHP:0002579Delayed fine motor developmentHP:0010862
Inheritance

Autosomal dominant

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

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

1 event
Nov 2024Further Delineation of the De Santo Shinawi Syndrome Phenotype Using a Series of Individuals Carrying a Pathogenic Variant of the WAC Gene

University Hospital, Clermont-Ferrand

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome.

1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

1 recruitingView all trials with filters →
Other1 trial
Further Delineation of the De Santo Shinawi Syndrome Phenotype Using a Series of Individuals Carrying a Pathogenic Variant of the WAC Gene
Actively Recruiting
PI: Florian CHERIK (University Hospital, Clermont-Ferrand) · Sites: Clermont-Ferrand, Auvergne

Specialists

1 foundView all specialists →
FC
Florian CHERIK
Specialist
PI on 1 active trial

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 WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome.

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Community

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Latest news about WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome

1 articles
ResearchBIORXIVApr 12, 2026
Preprint: Complementary vertebrate Wac models exhibit phenotypes relevant to DeSanto-Shinawi Syndrome
Scientists created animal models (using fish and mice) that mimic DeSanto-Shinawi Syndrome, a rare genetic disorder caused by mutations in the WAC gene. These a
See all news about WAC-related facial dysmorphism-developmental delay-behavioral abnormalities 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 was found in my child's WAC gene, and what does it mean?,What therapies should we start right away, and how often should they occur?,Should my child be monitored for seizures or other medical complications?,What educational supports or accommodations should we request at school?,Are there any medications that could help with behavioral challenges?,What is the long-term outlook for my child's independence and quality of life?,Should other family members be tested for this genetic change?

Common questions about WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome

What is WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome?

WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome is a rare genetic condition caused by changes (mutations) in the WAC gene. This syndrome is sometimes called DeSanto-Shinawi syndrome. It affects multiple parts of the body, but the most noticeable features involve facial appearance, learning and development, and behavior. Children with this condition typically have distinctive facial features that may include a broad forehead, widely spaced eyes, a thin upper lip, and a short nose. Developmental delays are common, meaning children may take longer to reach mi

How is WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome inherited?

WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome typically begin?

Typical onset of WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome is infantile. Age of onset can vary across affected individuals.

Are there clinical trials for WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome?

Yes — 1 recruiting clinical trial is currently listed for WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome?

1 specialists and care centers treating WAC-related facial dysmorphism-developmental delay-behavioral abnormalities syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.