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Diseases

Genetic and Rare Diseases Information Center (GARD)

Russell-Silver syndrome


Other Names for this Disease
  • Silver-Russell syndrome
  • Silver-Russell dwarfism
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Inheritance

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Is Russell-Silver syndrome inherited?

Most cases of Russell-Silver syndrome (RSS) are sporadic (not inherited), which means they occur in people with no family history of RSS.[1]

Less commonly, Russell-Silver syndrome is inherited. In some families, it appears to be inherited in an autosomal dominant manner.[2] This means that having one "copy" of a genetic change in each cell is enough to cause the disorder. In some cases, an affected person inherits the genetic change from a parent. In other cases, the change occurs for the first time in an affected person. When a person with a genetic change that causes an autosomal dominant condition has children, each child has a 50% (1 in 2) chance to inherit the genetic change.

In other families, the condition is inherited in an autosomal recessive manner.[2] This means that to be affected, a person must have a change in both copies of the responsible gene in each cell. Affected people inherit one copy from each parent, who is referred to as a carrier. Carriers of an autosomal recessive condition typically do not have any signs or symptoms (they are unaffected). When 2 carriers of an autosomal recessive condition have children, each child has a:
  • 25% (1 in 4) chance to be affected
  • 50% (1 in 2) chance to be an unaffected carrier like each parent
  • 25% chance to be unaffected and not be a carrier
Last updated: 3/3/2016

References
  1. Russell-Silver syndrome. Genetics Home Reference. 2008; http://ghr.nlm.nih.gov/condition=russellsilversyndrome.
  2. Saal HM. Russell-Silver Syndrome. GeneReviews. June 2 2011; http://www.ncbi.nlm.nih.gov/books/NBK1324/.


Other Names for this Disease
  • Silver-Russell syndrome
  • Silver-Russell dwarfism
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.