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

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.

Genetic and laboratory testing are used to diagnose this disease.

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

Talk to your vet about your dog’s glycogen storage disease type IA result so you can work together to plan ongoing care and monitoring.

Schedule regular checkups and lab testing as recommended to monitor liver function and blood sugar levels.

Work with your vet to provide supportive care that includes a balanced diet and consistent feeding schedule to help maintain steady energy levels.

Keep your dog’s routine calm and predictable, and contact your vet right away if you notice signs such as weakness, lethargy, or tremors.

This variant was first described in the German Pinscher.

Penetrance: This variant is inherited in an autosomal recessive manner, meaning that a dog requires two copies of the variant to show signs of Glycogen Storage Disease Type IA (GSDIA).

Gene

G6PC1 Exon 5

Inheritance type

recessive

Clinical category

Multisystem

Citations

1. Christen et al 2021

• German Pinscher