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Glycogen storage disease due to phosphorylase kinase deficiency

ORPHA:370

Definition

Glycogen storage disease (GSD) due to phosphorylase kinase deficiency is a group of inborn errors of glycogen metabolism that is clinically and genetically heterogeneous. This group comprises GSD due to liver phosphorylase kinase (PhK) deficiency, GSD due to muscle PhK deficiency and GSD due to liver and muscle PhK deficiency.

Prevalence
1-9 / 100 000
Inheritance
Autosomal recessive, X-linked recessive
Age of onset
All ages