OBSOLETE: DDX59-related orofaciodigital syndrome

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ORPHA:369902
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Overview

DDX59-related orofaciodigital syndrome is an extremely rare genetic condition that affects the development of the mouth (oral), face (facio), and fingers and toes (digital). This condition was previously classified under Orphanet code 369902 but has since been reclassified or merged with other orofaciodigital syndrome subtypes. The DDX59 gene provides instructions for making a protein involved in important cell signaling pathways during early development. When this gene does not work properly, it can lead to a range of birth defects. People with this condition may be born with a cleft lip or cleft palate, extra fingers or toes (polydactyly), abnormally short fingers or toes, and distinctive facial features. Some individuals may also have intellectual disability or developmental delays. The brain, kidneys, and other organs can sometimes be affected as well. There is currently no cure for this condition. Treatment focuses on managing individual symptoms and may include surgery to repair cleft lip or palate, removal or correction of extra digits, speech therapy, and educational support for developmental delays. A team of specialists typically works together to provide the best care for affected individuals. Because this is such a rare condition, much of the medical knowledge comes from a small number of reported cases in the scientific literature.

Key symptoms:

Extra fingers or toes (polydactyly)Cleft lip or cleft palateAbnormally short fingers or toesWebbing or fusion of fingers or toesUnusual facial featuresSmall or underdeveloped jawTongue abnormalities such as bumps or clefts on the tongueIntellectual disability or learning difficultiesDevelopmental delaysKidney abnormalitiesBrain malformations in some casesWidely spaced eyesLow-set ears

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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: DDX59-related orofaciodigital syndrome.

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No actively recruiting trials found for OBSOLETE: DDX59-related orofaciodigital syndrome at this time.

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Search ClinicalTrials.gov ↗Join the OBSOLETE: DDX59-related orofaciodigital syndrome community →

No specialists are currently listed for OBSOLETE: DDX59-related orofaciodigital 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 OBSOLETE: DDX59-related orofaciodigital syndrome.

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Community

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Latest news about OBSOLETE: DDX59-related orofaciodigital syndrome

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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.How severe are my child's oral, facial, and digital differences, and what surgeries will be needed?,Should we have kidney and brain imaging done to check for internal abnormalities?,What developmental milestones should we watch for, and when should we be concerned?,What therapies (speech, occupational, physical) should we start and when?,What is the chance of having another child with this condition?,Are there any clinical trials or research studies we could participate in?,How often should my child see each specialist for follow-up care?

Common questions about OBSOLETE: DDX59-related orofaciodigital syndrome

What is OBSOLETE: DDX59-related orofaciodigital syndrome?

DDX59-related orofaciodigital syndrome is an extremely rare genetic condition that affects the development of the mouth (oral), face (facio), and fingers and toes (digital). This condition was previously classified under Orphanet code 369902 but has since been reclassified or merged with other orofaciodigital syndrome subtypes. The DDX59 gene provides instructions for making a protein involved in important cell signaling pathways during early development. When this gene does not work properly, it can lead to a range of birth defects. People with this condition may be born with a cleft lip or

How is OBSOLETE: DDX59-related orofaciodigital syndrome inherited?

OBSOLETE: DDX59-related orofaciodigital syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does OBSOLETE: DDX59-related orofaciodigital syndrome typically begin?

Typical onset of OBSOLETE: DDX59-related orofaciodigital syndrome is neonatal. Age of onset can vary across affected individuals.