2p21 microdeletion syndrome without cystinuria

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Overview

2p21 microdeletion syndrome without cystinuria is an extremely rare genetic condition caused by a small missing piece (deletion) on the short arm of chromosome 2, specifically at position 2p21. This deletion affects certain genes in that region but does not include the SLC3A1 gene, which means that unlike the related 2p21 deletion syndrome, patients with this form do not develop cystinuria (a kidney stone condition caused by excess cystine in the urine). The syndrome primarily affects brain development and can lead to intellectual disability, developmental delays, and other neurological problems. Children with this condition may show delays in reaching milestones such as sitting, walking, and talking. They may also have distinctive facial features, feeding difficulties in infancy, and low muscle tone (hypotonia). Some individuals may experience seizures. The severity of symptoms can vary depending on the exact size of the deletion and which genes are affected. There is currently no cure for this condition. Treatment focuses on managing individual symptoms and supporting development through therapies such as speech therapy, physical therapy, and occupational therapy. Early intervention programs can help children reach their full potential. Seizures, if present, are managed with anti-epileptic medications. Because this condition is so rare, management is typically guided by a team of specialists who tailor care to each patient's specific needs.

Also known as:

Key symptoms:

Intellectual disabilityDelayed speech and language developmentDelayed motor milestones like walkingLow muscle tone (floppiness)Feeding difficulties in infancySeizuresDistinctive facial featuresLearning difficultiesBehavioral challengesPoor coordinationGrowth delays

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for 2p21 microdeletion syndrome without cystinuria.

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No actively recruiting trials found for 2p21 microdeletion syndrome without cystinuria at this time.

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No specialists are currently listed for 2p21 microdeletion syndrome without cystinuria.

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 2p21 microdeletion syndrome without cystinuria.

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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.What is the exact size of my child's deletion and which genes are affected?,Was this deletion inherited or did it happen for the first time in my child?,What developmental therapies should we start right away?,Should we be watching for seizures, and what should we do if one occurs?,How often should my child have developmental and neurological check-ups?,Are there any genetic implications for future pregnancies in our family?,Are there any research studies or registries we should consider joining?

Common questions about 2p21 microdeletion syndrome without cystinuria

What is 2p21 microdeletion syndrome without cystinuria?

2p21 microdeletion syndrome without cystinuria is an extremely rare genetic condition caused by a small missing piece (deletion) on the short arm of chromosome 2, specifically at position 2p21. This deletion affects certain genes in that region but does not include the SLC3A1 gene, which means that unlike the related 2p21 deletion syndrome, patients with this form do not develop cystinuria (a kidney stone condition caused by excess cystine in the urine). The syndrome primarily affects brain development and can lead to intellectual disability, developmental delays, and other neurological proble

At what age does 2p21 microdeletion syndrome without cystinuria typically begin?

Typical onset of 2p21 microdeletion syndrome without cystinuria is infantile. Age of onset can vary across affected individuals.