Lethal recessive chondrodysplasia

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Overview

Lethal recessive chondrodysplasia is an extremely rare group of inherited skeletal disorders that affect how cartilage and bone develop in a growing baby. The term 'chondrodysplasia' means abnormal growth of cartilage, and 'lethal recessive' indicates that the condition is severe enough to cause death before or shortly after birth, and that it follows an autosomal recessive inheritance pattern. This means a child must inherit a faulty copy of the responsible gene from both parents to be affected. Babies with this condition typically show severely shortened limbs, an abnormally shaped chest that is often very narrow or small, and underdeveloped lungs. The small chest and poor lung development are the main reasons the condition is life-threatening, as the baby cannot breathe adequately. Other features may include a flattened face, soft skull bones, and overall very short stature. The condition is usually detected during pregnancy through ultrasound imaging, which may reveal very short limbs and a small chest. Because this condition is lethal in the newborn period, there are currently no curative treatments available. Medical care focuses on providing comfort and supportive measures. Genetic counseling is very important for affected families, as carrier parents have a 25% chance of having another affected child with each pregnancy. Prenatal diagnosis through genetic testing or detailed ultrasound can be offered in future pregnancies.

Also known as:

Key symptoms:

Severely shortened arms and legsVery small or narrow chestUnderdeveloped lungsDifficulty breathing or inability to breathe at birthVery short statureFlattened facial featuresSoft or poorly formed skull bonesAbnormal shape of the spineShort ribsSwollen or rounded abdomenExcess fluid around the baby (hydrops fetalis)Reduced fetal movement during pregnancy

Clinical phenotype terms (12)— hover any for plain English
Flared elbow metaphysesHP:0003950Generalized osteosclerosisHP:0005789
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 Lethal recessive chondrodysplasia.

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No actively recruiting trials found for Lethal recessive chondrodysplasia at this time.

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No specialists are currently listed for Lethal recessive chondrodysplasia.

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 Lethal recessive chondrodysplasia.

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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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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 specific type of lethal chondrodysplasia does our baby have, and has a genetic cause been identified?,What are our options for comfort care and palliative support for our baby?,What is the chance that this could happen again in a future pregnancy?,Can we have genetic testing to find out if we are carriers of a specific gene mutation?,What prenatal testing options are available for future pregnancies?,Are there support groups or counseling services for families who have experienced this diagnosis?,Should other family members be tested to see if they are carriers?

Common questions about Lethal recessive chondrodysplasia

What is Lethal recessive chondrodysplasia?

Lethal recessive chondrodysplasia is an extremely rare group of inherited skeletal disorders that affect how cartilage and bone develop in a growing baby. The term 'chondrodysplasia' means abnormal growth of cartilage, and 'lethal recessive' indicates that the condition is severe enough to cause death before or shortly after birth, and that it follows an autosomal recessive inheritance pattern. This means a child must inherit a faulty copy of the responsible gene from both parents to be affected. Babies with this condition typically show severely shortened limbs, an abnormally shaped chest th

How is Lethal recessive chondrodysplasia inherited?

Lethal recessive chondrodysplasia follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Lethal recessive chondrodysplasia typically begin?

Typical onset of Lethal recessive chondrodysplasia is neonatal. Age of onset can vary across affected individuals.