Home > Encyclopedia of Genetic Disorders > Russell-Silver syndrome

Definition

Russell-Silver syndrome (RSS) is one of the recognized forms of intrauterine growth retardation (IUGR) diseases. It was first independently described by H. K. Silver in 1953 and by A. Russell in 1954.

Description

Russell-Silver syndrome is one of more than 300 recognized forms of genetic disorders that lead to short stature. It is characterized by:

• the presence of a triangular shaped face
• an incurving fifth finger (clinodactyly)
• low birth weight and length (intrauterine growth retardation, or IUGR)
• a poor appetite in the first few years of life

This disorder is alternately known as Russell syndrome, Silver syndrome, or Silver-Russell syndrome....