Thin ribs-tubular bones-dysmorphism syndrome

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ORPHA:1506Q87.5
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8Treatment centers

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Overview

Thin ribs-tubular bones-dysmorphism syndrome is an extremely rare genetic condition that affects the development of bones and facial features. It is sometimes referred to by its Orphanet designation (ORPHA:1506). The hallmark features of this syndrome include unusually thin ribs, abnormally shaped long bones (called tubular bones, such as those in the arms and legs), and distinctive facial features (dysmorphism). Affected individuals may have a narrow chest due to the thin ribs, which can lead to breathing difficulties, especially in newborns and infants. Facial features may include a prominent forehead, widely spaced eyes, a flat nasal bridge, and other subtle differences in facial structure. Some individuals may also experience growth delays and short stature. Because this condition is so rare, with only a handful of cases described in the medical literature, the full range of symptoms and the best approaches to treatment are not yet well understood. Management is primarily supportive and focuses on addressing specific symptoms such as respiratory problems, feeding difficulties, and orthopedic concerns. A team of specialists is typically needed to provide comprehensive care. There is currently no cure, and treatment is aimed at improving quality of life and managing complications as they arise.

Also known as:

Key symptoms:

Unusually thin ribs visible on X-rayNarrow chestBreathing difficultiesAbnormally shaped long bones in the arms and legsDistinctive facial featuresProminent foreheadWidely spaced eyesFlat nasal bridgeShort stature or growth delaysFeeding difficulties in infancyLow muscle toneDelayed development

Clinical phenotype terms (8)— hover any for plain English
Slender long boneHP:0003100
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 Thin ribs-tubular bones-dysmorphism syndrome.

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No actively recruiting trials found for Thin ribs-tubular bones-dysmorphism syndrome at this time.

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No specialists are currently listed for Thin ribs-tubular bones-dysmorphism 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 Thin ribs-tubular bones-dysmorphism syndrome.

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Community

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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 is my child's chest and rib involvement, and what does that mean for breathing?,What kind of respiratory support might my child need now or in the future?,Are there genetic tests that could help identify the exact cause of this condition?,What specialists should be part of my child's care team?,What should I watch for at home that would require emergency medical attention?,Are there any clinical trials or research studies we could participate in?,What is the expected outlook for my child's growth and development?

Common questions about Thin ribs-tubular bones-dysmorphism syndrome

What is Thin ribs-tubular bones-dysmorphism syndrome?

Thin ribs-tubular bones-dysmorphism syndrome is an extremely rare genetic condition that affects the development of bones and facial features. It is sometimes referred to by its Orphanet designation (ORPHA:1506). The hallmark features of this syndrome include unusually thin ribs, abnormally shaped long bones (called tubular bones, such as those in the arms and legs), and distinctive facial features (dysmorphism). Affected individuals may have a narrow chest due to the thin ribs, which can lead to breathing difficulties, especially in newborns and infants. Facial features may include a prominen

How is Thin ribs-tubular bones-dysmorphism syndrome inherited?

Thin ribs-tubular bones-dysmorphism syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Thin ribs-tubular bones-dysmorphism syndrome typically begin?

Typical onset of Thin ribs-tubular bones-dysmorphism syndrome is neonatal. Age of onset can vary across affected individuals.