X-linked dominant chondrodysplasia, Chassaing-Lacombe type

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ORPHA:163966OMIM:300863Q87.8
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Overview

X-linked dominant chondrodysplasia, Chassaing-Lacombe type, is an extremely rare genetic bone disorder that primarily affects the development of cartilage and bone (chondrodysplasia). This condition follows an X-linked dominant inheritance pattern, meaning the genetic change responsible is located on the X chromosome. Because of this inheritance pattern, the condition tends to be lethal in males before or shortly after birth, while affected females may survive but show significant skeletal abnormalities. The disease is characterized by severe problems with bone and cartilage formation that become apparent before birth or at the time of delivery. Key features can include very short limbs (micromelia), a small chest, abnormal vertebrae, and distinctive facial features. The rib cage may be underdeveloped, which can lead to serious breathing difficulties. Other findings may include mineralization defects of the bones and overall short stature. Because this condition is so rare, there is no specific cure or targeted treatment available. Management is supportive and focuses on addressing breathing problems, skeletal complications, and other symptoms as they arise. A team of specialists is typically needed to provide the best possible care. Genetic counseling is strongly recommended for affected families to understand recurrence risks and reproductive options.

Also known as:

Key symptoms:

Very short limbs at birthSmall and narrow chestBreathing difficultiesAbnormal bone developmentShort statureDistinctive facial featuresAbnormal spine or vertebraePoor bone mineralizationShortened fingers and toesFlat facial profileUnderdeveloped rib cage

Clinical phenotype terms (24)— hover any for plain English
Thin ribsHP:0000883Hypoplastic iliac wingHP:0002866
Inheritance

X-linked dominant

Carried on the X chromosome; a single copy can cause the condition

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 X-linked dominant chondrodysplasia, Chassaing-Lacombe type.

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No actively recruiting trials found for X-linked dominant chondrodysplasia, Chassaing-Lacombe type at this time.

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No specialists are currently listed for X-linked dominant chondrodysplasia, Chassaing-Lacombe type.

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 X-linked dominant chondrodysplasia, Chassaing-Lacombe type.

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Community

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Latest news about X-linked dominant chondrodysplasia, Chassaing-Lacombe type

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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.What is the specific genetic change causing this condition in our family?,What is the expected outlook for my child based on the severity of their symptoms?,What respiratory support or monitoring will my child need?,Are there any clinical trials or research studies we could participate in?,What specialists should be part of our care team?,What are the chances of this condition occurring again in a future pregnancy?,What therapies or interventions can help improve my child's quality of life?

Common questions about X-linked dominant chondrodysplasia, Chassaing-Lacombe type

What is X-linked dominant chondrodysplasia, Chassaing-Lacombe type?

X-linked dominant chondrodysplasia, Chassaing-Lacombe type, is an extremely rare genetic bone disorder that primarily affects the development of cartilage and bone (chondrodysplasia). This condition follows an X-linked dominant inheritance pattern, meaning the genetic change responsible is located on the X chromosome. Because of this inheritance pattern, the condition tends to be lethal in males before or shortly after birth, while affected females may survive but show significant skeletal abnormalities. The disease is characterized by severe problems with bone and cartilage formation that be

How is X-linked dominant chondrodysplasia, Chassaing-Lacombe type inherited?

X-linked dominant chondrodysplasia, Chassaing-Lacombe type follows a x-linked dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does X-linked dominant chondrodysplasia, Chassaing-Lacombe type typically begin?

Typical onset of X-linked dominant chondrodysplasia, Chassaing-Lacombe type is neonatal. Age of onset can vary across affected individuals.