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COG8-CDG

ORPHA:95428· ICD-10 E77.8

Definition

The CDG (Congenital Disorders of Glycosylation) syndromes are a group of autosomal recessive disorders affecting glycoprotein synthesis. CDG syndrome type IIh is characterised by severe psychomotor retardation, failure to thrive and intolerance to wheat and dairy products.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Childhood