Russell-Silver syndrome is a disorder present at birth involving poor growth. One side of the body also will appear to be larger than the other.
Up to 10% of patients with this syndrome have a defect involving chromosome 7. In other patients, there the syndrome may affect chromosome 11, 15, 17, or 18.
In most patients, the cause is unknown. Most cases occur in people with no family history of the disease.
The estimated number of people who develop this condition varies greatly. Some say it affects about 1 in 3,000 people. Other reports say it affects 1 in 100,000 people. Males and females are equally affected.
The condition is usually diagnosed by early childhood. The doctor will perform a physical exam. Signs include:
There are no specific laboratory tests to diagnose Russell-Silver syndrome. Diagnosis is uaullay based on the judgment of your child's pediatrician. However, the following tests may be done:
Growth hormone replacement may help if this hormone is lacking. Other treatments include:
Many specialists may be involved in treating this condition:
Older children and adults do not show typical features as clearly as infants or younger children. Intelligence may be normal, although the patient may have a learning disability.
Call your health care child's provider if signs of Russell-Silver syndrome develop. Make sure your child's height and weight is measure during each well child visit. The doctor may refer you to:
Silver-Russell syndrome; Silver syndrome
Updated by: A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, and David R. Eltz. Previously reviewed by Chad Haldeman-Englert, MD, Wake Forest University School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC (12/1/2010).
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