Skip navigation | ||
|
||
Medical Encyclopedia |
|
Other encyclopedia topics: | A-Ag Ah-Ap Aq-Az B-Bk Bl-Bz C-Cg Ch-Co Cp-Cz D-Di Dj-Dz E-Ep Eq-Ez F G H-Hf Hg-Hz I-In Io-Iz J K L-Ln Lo-Lz M-Mf Mg-Mz N O P-Pl Pm-Pz Q R S-Sh Si-Sp Sq-Sz T-Tn To-Tz U V W X Y Z 0-9 |
Contents of this page: | |
Alternative names
Silver-Russell syndrome; Silver syndromeDefinition Return to top
Rusell-Silver syndrome is a congenital disease (present at birth) characterized by short stature and, frequently, asymmetry in the size of the two halves or other parts of the body.Causes, incidence, and risk factors Return to top
Genetic problems are thought to cause this syndrome, although the specific gene(s) remain to be discovered. The pattern of inheritance has not been determined, but in most cases it seems to occur without any family history of the condition.
Children with Russell-Silver syndrome are born small and generally achieve less than 5 feet at adult height. Side-to-side asymmetry is fairly common. For example, a left body part may be larger than the right counterpart. This asymmetry may occur anywhere including the face.
Other characteristics include excessive sweating, a small triangular face that makes the skull look large by comparison, inward curving 5th fingers, and colored spots on the skin called cafe-au-lait spots.
Symptoms Return to top
Signs and tests Return to top
A physical examination may show:Tests include:
Treatment Return to top
There is no specific treatment for Russell-Silver syndrome. Symptoms are treated as necessary. Sometimes growth hormone replacement is recommended if a growth hormone deficiency is discovered. This has had varying results. Supportive treatment for discrepancy of leg length may prevent problems.Support Groups Return to top
Additional resources are available from Little People of America (888-572-2001) and MAGIC Foundation for Children's Growth (800-362-4423).
Expectations (prognosis) Return to top
As the child ages, many will improve in growth and appearance. There is normal intelligence.Complications Return to top
Calling your health care provider Return to top
There may be some indications of this condition at birth. Call your health care provider if your child does not seem to be growing normally and has asymmetry of body parts or in-turned 5th fingers. Update Date: 1/30/2003 Updated by: Douglas R. Stewart, M.D., Division of Medical Genetics, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
Home | Health Topics | Drug Information | Encyclopedia | Dictionary | News | Directories | Other Resources | |
Copyright | Privacy | Accessibility | Selection Guidelines U.S. National Library of Medicine, 8600 Rockville Pike, Bethesda, MD 20894 National Institutes of Health | Department of Health & Human Services |
Page last updated: 28 October 2004 |