Glycogen storage disease IV(GSD IV)
Also known as: Glykogenspeicherkrankheit Typ IV · Glycogenosis Type IV · Glycogen Branching Enzyme Deficiency · GBED · Glykogenose Typ IV
Overview
What it is
This is an inherited metabolic disorder where a deficiency in the glycogen branching enzyme prevents the body from properly storing and using sugar. Instead, abnormal glycogen molecules build up inside vital organs, particularly the liver, muscles, and nervous system, causing progressive and irreversible tissue damage.
How it presents
Symptoms typically appear in young puppies and include extreme muscle weakness, developmental delays, and progressive liver failure. Many affected animals fail to thrive and pass away shortly after birth.
Treatment
This condition is incurable and fatal, with veterinary care limited to palliative support or timely euthanasia to prevent suffering. Associated veterinary costs for supportive care or end-of-life services typically range from 150 to 800 EUR.
How it's tested
Affected breeds
Treatment cost
Estimated range of typical treatment cost. Actual cost depends on severity, clinic and region.
Frequently asked
What is Glycogen storage disease IV?
This is an inherited metabolic disorder where a deficiency in the glycogen branching enzyme prevents the body from properly storing and using sugar. Instead, abnormal glycogen molecules build up inside vital organs, particularly the liver, muscles, and nervous system, causing progressive and irreversible tissue damage.
How is Glycogen storage disease IV tested?
Various clinical and genetic tests are used depending on the breed and presentation.
Which breeds are most affected?
This condition isn't tied to a specific breed in our database.