Glycogen breakdown is hindered, which leads to Von Gierke disease, commonly known as Type I glycogen storage disorder.
In those with Von Gierke illness, the body is unable to break down glucose for energy. A lack of an enzyme that discharges glucose from glycogen causes the illness. This causes excessive levels of glycogen to accumulate in some tissues. Type I glycogen storage disorder is another name for Von Gierke disease (GSD I). Low blood glucose results from inadequate glycogen breakdown.
- Von Gierke Disease type 1a has no specific medical needs. Antibiotics are necessary for recurrent infections.
- Supplemental iron is administered orally.
- Granulocyte colony-stimulating factor administration for type 1b illness must occur every week (GCSF).
- GCSF increases the formation of blood cells that fight infections or lessen their impact (GSD1 causes a large decline in protective blood cells like neutrophils).
- Multivitamins, calcium, and vitamin D dietary supplements are frequently necessary.
- It could be necessary to take the medication allopurinol to lower high uric acid levels. When cholesterol levels are too high, medications from the statin and fibric acid families are utilized.
- The use of medications known as angiotensin-converting enzyme (ACE) inhibitors can help to prevent renal dysfunction.
- Microalbuminuria is a symptom of renal disease that is treated by ACE inhibitors.
- Calcium buildup in the kidneys may be avoided with the aid of citrate supplements. It might also stop kidney stones from forming.
Antibiotics,Allopurinol,ACE inhibitors,Angiotensin-converting enzyme (ACE),Allopurinol
Constant hunger,Frequent need to eat often,Easy bruising,Nosebleeds,Fatigue,Irritation,Thin chest and limbs,Puffy cheeks