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Russell-Silver syndrome

Silver-Russell syndrome; Silver syndrome; RSS; Russell-Silver syndrome

Russell-Silver syndrome (RSS) is a disorder present at birth involving poor growth. One side of the body may also appear to be larger than the other.

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Causes

One in 10 children with this syndrome has a problem involving chromosome 7. In other people with the syndrome, it may affect chromosome 11.

Most of the time, it occurs in people with no family history of the disease.

The estimated number of people who develop this condition varies greatly. Males and females are equally affected.

Symptoms

Symptoms can include:

Exams and Tests

The condition is usually diagnosed by early childhood. The health care provider will perform a physical exam.

There are no specific laboratory tests to diagnose RSS. Diagnosis is usually based on the judgment of your child's provider. However, the following tests may be done:

Treatment

Growth hormone replacement may help if this hormone is lacking. Other treatments include:

Many specialists may be involved in treating a person with this condition. They include:

Outlook (Prognosis)

Older children and adults do not show typical features as clearly as infants or younger children. Intelligence may be normal, although the person may have a learning disability. Birth defects of the urinary tract may be present.

Possible Complications

People with RSS may have these problems:

When to Contact a Medical Professional

Call your child's provider if signs of RSS develop. Make sure your child's height and weight are measured during each well-child visit. The provider may refer you to:

Related Information

Short stature

References

Haldeman-Englert CR, Saitta SC, Zackai EH. Chromosome disorders. In: Gleason CA, Juul SE, eds. Avery's Diseases of the Newborn. 10th ed. Philadelphia, PA: Elsevier; 2018:chap 20.

Wakeling EL, Brioude F, Lokulo-Sodipe O, et al. Diagnosis and management of Silver-Russell syndrome: first international consensus statement. Nat Rev Endocrinol. 2017;13(2):105-124. PMID: 27585961 pubmed.ncbi.nlm.nih.gov/27585961/.

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Review Date: 10/27/2020  

Reviewed By: Anna C. Edens Hurst, MD, MS, Associate Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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