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Costello syndrome

ORPHA:3071· ICD-10 Q87.8

Definition

A rare syndrome with intellectual disability, characterized by failure to thrive, short stature, joint laxity, soft skin, and distinctive facial features. Cardiac and neurological involvement is common and there is an increased lifetime risk of certain tumors. Costello syndrome belongs to the RASopathies, a group of conditions resulting from germline derived point mutations affecting the RAS-mitogen activated protein kinase pathway.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal dominant, Not applicable
Age of onset
Antenatal, Neonatal