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The University of Southampton
Wessex Imprinting GroupInformation for patients

Silver Russell Syndrome (SRS)

Silver-Russell syndrome (SRS) or Russell-Silver syndrome (RSS) is a growth disorder occurring in approximately 1/3,000 to 1/100,000 births.

This is a condition characterised by poor growth before and after birth (birth weight usually less than 2.5 kg).

Other characteristics of SRS include:


Genetic Causes

In approximately 60% of cases, SRS has been demonstrated to be due to a change in the expression (activation) of imprinted genes on chromosome 11 (for more information on imprinting and genetics please see the ‘Other imprinting disorders’ page). This is usually due to a problem with the ‘silencing’ of the copy of a gene from the father (paternal hypomethylation) on chromosome 11.

In approximately 10% of cases, SRS is caused by a maternal uniparental disomy (UPD) on chromosome 7. This is where the person receives two copies of chromosome 7 from the mother instead of the usual one from each parent.

Useful links for SRS:

References for Silver Russell Syndrome

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