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.
Silver-Russell syndrome; Silver syndrome
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.
Many specialists may be involved in treating this condition:
A doctor specializing in genetics can help diagnose Russell-Silver syndrome.
A gastroenterologist or nutritionist can help develop the proper diet to enhance growth.
An endocrinologist may prescribe growth hormone, if it is needed.
Genetic counselors and psychologists may also be involved.
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.
Chewing or speaking difficulty if jaw is very small
When to Contact a Medical Professional
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:
A genetic professional for a full evaluation and chromosome studies
A pediatric endocrinologist for management of your child's growth problems
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).