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Your GSD0 coordinator is
Abbie Maguire


The enzyme glycogen synthase is needed for the body to make glycogen. A deficiency results in very low amounts of glycogen stored in the liver. A person between meals can develop very low blood sugar levels, known as hypoglycemia. Early in infancy children usually have no symptoms. The hypoglycemia typically develops once night feeding stops. Before breakfast children may have drowsiness, look pale, have vomiting, fatigue and sometimes convulsions. If a GP investigates a child lacking energy, hypoglycemia is often discovered through morning urine samples.

Other names

Glycogen synthase deficiency


Liver and muscle forms

Inheritance Autosomal recessive

Less than 1 in 1,000,000

UK diagnosed Under 20  (UK diagnosed explained)

Before breakfast drowsiness, tiredness, looking pale, vomiting.

Secondary symptoms

Quick to tire, muscle cramps


Regular snacks, cornstarch to reduce overnight hypoglycemia.


Good with adherence to recommended feeding.

How can we help?


Diagnosis & Follow up

Incidence, Diagnosis, Treatment


Information & Support

Support, Coordinator, Links


Activity & Exercise

More content planned.

Research & Development

More content planned.

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