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Russell-Silver syndrome

Alternative names:

Russell-Silver dwarfism; Silver syndrome

Definition:

A condition of unknown origin, but which is characterized by extremely short stature and frequent asymmetry.

Causes, incidence, and risk factors:

Genetic problems are thought to play a part in this syndrome although no pattern of inheritance has been determined. Children with Russell-Silver syndrome appear sporadically in families with no prior history of the condition.

Children with Russell-Silver syndrome generally achieve less than 5 feet at adult height. Side-to-side asymmetry is fairly common. This asymmetry may occur anywhere including the face. Other findings include excessive sweating, a small triangular face which makes the skull look large by comparison, inward curved the fingers, and pigmented skin lesions called cafe-au-lait spots.


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