Russell-Silver syndrome (RSS) is a disorder present at birth involving poor growth. One side of the body may also appear to be larger than the other.
Silver-Russell syndrome; Silver syndrome; RSS; Russell-Silver syndrome
One in 10 children with this syndrome has a problem involving chromosome 7. In other people with the syndrome, it may affect chromosome 11.
Most of the time, it occurs in people with no family history of the disease.
The estimated number of people who develop this condition varies greatly. Males and females are equally affected.
Symptoms can include:
Exams and Tests
The condition is usually diagnosed by early childhood. The health care provider will perform a physical exam.
There are no specific laboratory tests to diagnose RSS. Diagnosis is usually based on the judgment of your child's health care provider. 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 a person with this condition. They include:
These organizations can provide support and more information:
Older children and adults do not show typical features as clearly as infants or younger children. Intelligence may be normal, although the person may have a learning disability. Birth defects of the urinary tract may be present.
People with RSS may have these problems:
When to Contact a Medical Professional
Call your child's health care provider if signs of RSS develop. Make sure your child's height and weight are measured during each well-child visit. The provider may refer you to:
Backeljauw PF, Dattani MT, Cohen P, Rosenfeld RG. Disorders of growth hormone/insulin-like growth factor secretion and action. In: Sperling MA, ed. Pediatric Endocrinology. 4th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 10.
Wakeling EL, Brioude F, Lokulo-Sodipe O, et al. Diagnosis and management of Silver-Russell syndrome: first international consensus statement. Nat Rev Endocrinol. 2017;13(2):105-124. PMID: 27585961 www.ncbi.nlm.nih.gov/pubmed/27585961.
Review Date: 10/30/2016
Reviewed By: Anna C. Edens Hurst, MD, MS, Assistant Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.