Glycogen Storage Disease Type IA, Von Gierke Disease, GSD IA
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. Dogs with Type I GSD have very severe symptoms and are markedly hypoglycemic.
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Signs and symptoms
Puppies with this disease often present severely hypoglycemic: lethargic, weak, and possibly seizing.
Signs typically develop after only a few weeks, and most puppies with this disease die before two months of age. -
Diagnosis
Genetic and laboratory testing used to diagnose this disease.
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Treatment
There is no treatment except supportive care for the clinical signs the affected dog develops.
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What to do if your dog is at risk
Actions
- Focus on palliative therapies to increase your dog's comfort and quality of life.
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Genetic Information
This mutation was first described in the Maltese.
This mutation is inherited in an autosomal recessive manner; that is, a dog requires two copies of the mutation to show signs of the disease.
Gene names:
G6PC ‐ chr
Inheritance type:
recessive
Citations:
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Breeds affected
This health condition affects the following breeds
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