Lowry-MacLean syndrome

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ORPHA:2409OMIM:600252Q87.8
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Overview

Lowry-MacLean syndrome is an extremely rare genetic condition that affects multiple body systems, particularly the bones, brain, and heart. It was first described by Drs. Lowry and MacLean. The syndrome is characterized by a combination of craniosynostosis (early fusion of the skull bones), intellectual disability, and heart defects. Children born with this condition often have distinctive facial features, including a flat midface, prominent forehead, and widely spaced eyes. Skeletal abnormalities are common and may include short stature, abnormally shaped bones, and joint problems. Heart defects present from birth can range in severity and may require surgical intervention. Intellectual disability is typically moderate to severe, and developmental milestones such as walking and talking are often significantly delayed. Because the condition is so rare, with only a handful of cases reported in the medical literature, there is no specific cure or targeted treatment. Management focuses on addressing individual symptoms through a team of specialists. Surgery may be needed for craniosynostosis or heart defects, while physical therapy, occupational therapy, and special education services can help support development. Regular monitoring by multiple specialists is essential to manage the various aspects of this complex condition.

Key symptoms:

Early fusion of skull bones (craniosynostosis)Intellectual disabilityHeart defects present from birthShort statureFlat midfaceWidely spaced eyesProminent foreheadDelayed development and milestonesBone and skeletal abnormalitiesLow muscle toneJoint problems or limited joint movementAbnormal shape of the headFeeding difficulties in infancyVision problems

Clinical phenotype terms (50)— hover any for plain English
Small anterior fontanelleHP:0000237Delayed eruption of primary teethHP:0000680Developmental glaucomaHP:0001087
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 Lowry-MacLean syndrome.

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No actively recruiting trials found for Lowry-MacLean syndrome at this time.

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No specialists are currently listed for Lowry-MacLean 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 Lowry-MacLean syndrome.

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Community

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Latest news about Lowry-MacLean syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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 craniosynostosis, and will surgery be needed?,What type of heart defect does my child have, and what treatment is recommended?,What developmental therapies should we start, and how often?,Are there any genetic tests that could help us understand the cause better?,What signs or symptoms should prompt us to seek emergency care?,How often should my child be seen by each specialist?,Are there any research studies or registries we should consider joining?

Common questions about Lowry-MacLean syndrome

What is Lowry-MacLean syndrome?

Lowry-MacLean syndrome is an extremely rare genetic condition that affects multiple body systems, particularly the bones, brain, and heart. It was first described by Drs. Lowry and MacLean. The syndrome is characterized by a combination of craniosynostosis (early fusion of the skull bones), intellectual disability, and heart defects. Children born with this condition often have distinctive facial features, including a flat midface, prominent forehead, and widely spaced eyes. Skeletal abnormalities are common and may include short stature, abnormally shaped bones, and joint problems. Heart defe

How is Lowry-MacLean syndrome inherited?

Lowry-MacLean syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Lowry-MacLean syndrome typically begin?

Typical onset of Lowry-MacLean syndrome is neonatal. Age of onset can vary across affected individuals.