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Glycogen storage disease due to liver phosphorylase kinase deficiency

ORPHA:264580· ICD-10 E74.0

Definition

Glycogen storage disease (GSD) due to liver phosphorylase kinase (PhK) deficiency is a benign inborn error of glycogen metabolism characterized by hepatomegaly, growth retardation, and mild delay in motor development during childhood.

Prevalence
Unknown
Inheritance
Autosomal recessive, X-linked recessive
Age of onset
Childhood