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Glycogen storage disease due to glycogen branching enzyme deficiency

ORPHA:367· ICD-10 E74.0

Definition

A rare form of glycogen storage disease characterized by a continuum of clinical manifestations of variable presentation and severity, involving hepatic, neuromuscular and/or cardiac symptoms.

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
All ages