This is a Multisystem condition.

Pompe's Disease

What is GSD II?

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 II GSD are severely affected with a dilated esophagus (megaesophagus) and an enlarged heart.

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

This form of glycogen storage disease can cause progressive weight loss, vomiting/regurgitation, and cardiac abnormalities. Breathing difficulties secondary to heart failure and/or megaesophagus are also common.

When do signs and symptoms develop?

Onset of signs is typically around 6 months and rapidly progresses until death around 1.5 years.

How do vets diagnose this condition?

Genetic and laboratory testing used to diagnose this disease. Imaging modalities (radiographs, ultrasound) are used to diagnose secondary complications.

How is this condition treated?

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

What actions should I take if my dog is affected?

  • Focus on palliative therapies to increase your dog's comfort and quality of life.
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