Intellectual disability-brachydactyly-Pierre Robin syndrome

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ORPHA:364577OMIM:608670Q87.0
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Overview

Intellectual disability-brachydactyly-Pierre Robin syndrome is an extremely rare genetic condition that affects multiple parts of the body. The syndrome combines three main features: intellectual disability (difficulty with learning, reasoning, and problem-solving), brachydactyly (unusually short fingers and toes), and Pierre Robin sequence (a combination of a small lower jaw, a tongue that falls back into the throat, and often a cleft palate — an opening in the roof of the mouth). Because of the small jaw and tongue positioning, newborns may have serious breathing and feeding difficulties that require immediate attention. Children with this condition typically show delays in reaching developmental milestones such as sitting, walking, and speaking. The severity of intellectual disability can vary from mild to moderate. Additional features may include distinctive facial characteristics and other skeletal abnormalities. Treatment is supportive and focuses on managing each symptom individually. This may include surgery for cleft palate, airway management for breathing problems, speech therapy, special education services, and occupational therapy. A team of specialists is usually needed to provide comprehensive care throughout the person's life.

Key symptoms:

Intellectual disability or learning difficultiesShort fingers and toes (brachydactyly)Small lower jaw (micrognathia)Tongue falling back into the throat (glossoptosis)Cleft palate (opening in the roof of the mouth)Breathing difficulties in newbornsFeeding difficulties in infancyDelayed speech and language developmentDelayed motor milestones like sitting and walkingDistinctive facial featuresShort statureSkeletal abnormalities

Clinical phenotype terms (43)— hover any for plain English
Pierre-Robin sequenceHP:0000201Exaggerated cupid's bowHP:0002263Long upper lipHP:0011341Short palpebral fissureHP:0012745MicroglossiaHP:0000171Small earlobeHP:0000385
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

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 Intellectual disability-brachydactyly-Pierre Robin syndrome.

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No actively recruiting trials found for Intellectual disability-brachydactyly-Pierre Robin syndrome at this time.

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No specialists are currently listed for Intellectual disability-brachydactyly-Pierre Robin 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 Intellectual disability-brachydactyly-Pierre Robin syndrome.

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Community

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Caregiver Resources

NORD Caregiver Resources

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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.How severe is my child's airway involvement, and what monitoring or interventions are needed?,When should cleft palate surgery be planned, and what can we expect from the recovery?,What early intervention therapies should we start, and how often?,Is genetic testing available to confirm the diagnosis and help with family planning?,What is the expected level of intellectual disability, and how can we best support learning?,Are there any clinical trials or research studies we should know about?,What specialists should be part of our child's care team, and how often should we see them?

Common questions about Intellectual disability-brachydactyly-Pierre Robin syndrome

What is Intellectual disability-brachydactyly-Pierre Robin syndrome?

Intellectual disability-brachydactyly-Pierre Robin syndrome is an extremely rare genetic condition that affects multiple parts of the body. The syndrome combines three main features: intellectual disability (difficulty with learning, reasoning, and problem-solving), brachydactyly (unusually short fingers and toes), and Pierre Robin sequence (a combination of a small lower jaw, a tongue that falls back into the throat, and often a cleft palate — an opening in the roof of the mouth). Because of the small jaw and tongue positioning, newborns may have serious breathing and feeding difficulties tha

How is Intellectual disability-brachydactyly-Pierre Robin syndrome inherited?

Intellectual disability-brachydactyly-Pierre Robin syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Intellectual disability-brachydactyly-Pierre Robin syndrome typically begin?

Typical onset of Intellectual disability-brachydactyly-Pierre Robin syndrome is neonatal. Age of onset can vary across affected individuals.