Glycogen Storage Disease Type I (GSD-I) | Von Gierke Disease

Complications of Glycogen storage disease type I

Without proper management, the following complications may occur:

Acute hypoglycemia

Untreated hypoglycemia for a long time causes cerebral edema and brain damage, which may end in death.

Prolonged elevation

Prolonged elevation of uric acid leads to:

• Proteinuria: high protein level in the urine
• Hematuria: the presence of blood in the urine
• Chronic kidney dysfunction

Chronic metabolic acidosis

Prolonged lactic acid deposition leads to:

• Osteopenia: low bone mass, which makes bones very weak
• Rickets: soft and weak bone
• Skeletal bone deformities

Hyperlipidemia

Cholesterol and triglycerides accumulate in the arteries, which may cause atherosclerosis or stroke.

Liver adenomas

They are benign liver tumors, which may become malignant.