Silver-Russell syndrome due to a point mutation

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ORPHA:397590OMIM:618908Q87.1
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Overview

Silver-Russell syndrome (SRS), also known as Russell-Silver syndrome, is a rare genetic condition that affects growth and development. It is one of the most well-known causes of intrauterine growth restriction, meaning babies with this condition are smaller than expected before and after birth. This specific form of Silver-Russell syndrome is caused by a point mutation — a small change in a single spot within the DNA — rather than the more common epigenetic or chromosomal changes seen in other forms of SRS. Children with this condition are typically born small for their gestational age and continue to grow slowly after birth. They often have distinctive facial features, including a triangular face, a prominent forehead, and a small, pointed chin. Body asymmetry, where one side of the body is slightly larger than the other, is also common. Many children experience feeding difficulties, low appetite, and low blood sugar (hypoglycemia) in early life. Treatment focuses on managing symptoms and supporting growth. Growth hormone therapy is a key part of treatment and can significantly improve height outcomes when started early. Nutritional support, monitoring for low blood sugar, and physical therapy for any limb length differences are also important. With proper care, many individuals with Silver-Russell syndrome lead fulfilling lives, though they may remain shorter than average. A team of specialists is usually needed to address the various aspects of this condition.

Key symptoms:

Being born small for gestational age (low birth weight)Poor growth after birth (failure to thrive)Short stature that persists into childhoodTriangular-shaped faceProminent or broad foreheadSmall, pointed chinBody asymmetry (one side of the body larger than the other)Feeding difficulties and poor appetiteLow blood sugar (hypoglycemia) in infancyThin arms and legs with relatively normal-sized headCurved pinky finger (clinodactyly)Delayed bone ageSweating excessivelyDigestive problems including refluxLearning difficulties in some children

Clinical phenotype terms (36)— hover any for plain English
Abnormal scrotum morphologyHP:0000045Short 5th fingerHP:0009237
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

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 Silver-Russell syndrome due to a point mutation.

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No actively recruiting trials found for Silver-Russell syndrome due to a point mutation at this time.

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No specialists are currently listed for Silver-Russell syndrome due to a point mutation.

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 Silver-Russell syndrome due to a point mutation.

Search all travel grants →NORD Financial Assistance ↗

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 specific genetic change (point mutation) does my child have, and how does it affect their condition?,When should growth hormone therapy be started, and what results can we realistically expect?,How should we manage feeding difficulties and prevent low blood sugar episodes at home?,Will my child need any orthopedic treatment for body asymmetry or limb length differences?,What is the chance that future children could also have this condition?,Are there any clinical trials or new treatments being studied for Silver-Russell syndrome?,What developmental or educational support services should we arrange for my child?

Common questions about Silver-Russell syndrome due to a point mutation

What is Silver-Russell syndrome due to a point mutation?

Silver-Russell syndrome (SRS), also known as Russell-Silver syndrome, is a rare genetic condition that affects growth and development. It is one of the most well-known causes of intrauterine growth restriction, meaning babies with this condition are smaller than expected before and after birth. This specific form of Silver-Russell syndrome is caused by a point mutation — a small change in a single spot within the DNA — rather than the more common epigenetic or chromosomal changes seen in other forms of SRS. Children with this condition are typically born small for their gestational age and co

At what age does Silver-Russell syndrome due to a point mutation typically begin?

Typical onset of Silver-Russell syndrome due to a point mutation is neonatal. Age of onset can vary across affected individuals.