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This is a Multisystem condition.

Glycogen Storage Disease Type IA, Von Gierke Disease, GSD IA

What is GSD IA?

An early onset type of glycogen storage disease, affected puppies often go to the vet for low energy levels and poor weight gain. Glycogen is the form in which our bodies store sugar: they are long, complex molecules that are primarily stored in the liver, heart, kidney, and muscle and are those tissues’ primary source of energy. In GSDs, glycogen accumulates to abnormal levels leading to enlargement and malfunction of these vital organs. Glycogen storage diseases (GSDs) occur in dogs, humans, and other animals. There are four types of GSDs known in dogs and these match Type I, II, IIIa and VII GSDs in humans. Dogs with Type I GSD are severely affected and go to the vet as puppies, where on further examination they are also markedly hypoglycemic (low blood sugar). Due to the severity of their symptoms, Type I GSD dogs often die before they reach 2 months of age.

What are the signs & symptoms that develop in affected dogs?

Puppies with this disease often present severely hypoglycemic: lethargic, weak, and possibly seizing.

When do signs and symptoms develop?

Signs typically develop after only a few weeks, and most puppies with this disease die before two months of age.

How do vets diagnose this condition?

Genetic and laboratory testing used to diagnose this disease.

How is this condition treated?

There is no treatment except supportive care for the clinical signs the affected dog develops.