Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation

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ORPHA:453504OMIM:616580Q87.8
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Overview

Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation is an extremely rare genetic condition that affects multiple body systems at the same time. It is sometimes referred to by its Orphanet designation (ORPHA:453504) and falls under the broader category of syndromic neurodevelopmental disorders. The condition is caused by a specific type of genetic change (a point mutation) that disrupts normal development before and after birth. Children born with this syndrome typically show delays in brain development, which can lead to intellectual disability and delayed milestones such as sitting, walking, and talking. The face may have unusual features (craniofacial dysmorphism), which can include a broad forehead, widely spaced eyes, a flat nasal bridge, or other distinctive facial characteristics. Heart defects are another hallmark of this condition and can range from mild to serious structural problems that may need surgical repair. Skeletal anomalies, such as abnormal curvature of the spine, short stature, or differences in the hands and feet, are also commonly seen. Because this syndrome affects so many body systems, treatment is supportive and tailored to each person's specific needs. There is currently no cure, but early intervention with therapies, cardiac monitoring, and orthopedic care can improve quality of life. A team of specialists is usually needed to manage the different aspects of the condition.

Key symptoms:

Intellectual disability or learning difficultiesDelayed speech and language developmentDelayed motor milestones such as sitting and walkingUnusual facial features such as a broad forehead or widely spaced eyesHeart defects present at birthShort stature or growth delaysAbnormal curvature of the spineDifferences in the shape or size of the hands or feetLow muscle tone (feeling floppy)Feeding difficulties in infancyBehavioral challengesSeizures in some casesJoint looseness or stiffness

Clinical phenotype terms (50)— hover any for plain English
Increased nuchal translucencyHP:0010880Cystic hygromaHP:0000476Shallow orbitsHP:0000586
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 Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation.

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No actively recruiting trials found for Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation at this time.

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Search ClinicalTrials.gov ↗Join the Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation community →

No specialists are currently listed for Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation.

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 Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific genetic mutation was found, and what does it mean for my child's health?,How severe is my child's heart defect, and will surgery be needed?,What therapies should we start right away to support my child's development?,How often should my child be seen by each specialist?,Are there any clinical trials or research studies my child might be eligible for?,What should I watch for at home that would require emergency medical attention?,Is there a risk that future children could have the same condition?

Common questions about Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation

What is Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation?

Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation is an extremely rare genetic condition that affects multiple body systems at the same time. It is sometimes referred to by its Orphanet designation (ORPHA:453504) and falls under the broader category of syndromic neurodevelopmental disorders. The condition is caused by a specific type of genetic change (a point mutation) that disrupts normal development before and after birth. Children born with this syndrome typically show delays in brain development, which can lead to inte

How is Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation inherited?

Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation typically begin?

Typical onset of Neurodevelopmental disorder-craniofacial dysmorphism-cardiac defect-skeletal anomalies syndrome due to a point mutation is neonatal. Age of onset can vary across affected individuals.