How is metabolic control assessed in GSD Ia?

Good control requires fasting lactate <2.5 mmol/L, uric acid within normal range, triglycerides <600 mg/dL, and absence of symptomatic hypoglycemia (glucose <3.5 mmol/L).

What is uncooked cornstarch therapy?

Uncooked cornstarch (1.6-2.5 g/kg/dose every 4-6 hours for infants; 1.7-2.5 g/kg every 6 hours for children) provides slow glucose release to prevent fasting hypoglycemia.

What hepatic complications occur in GSD I?

Hepatic adenomas develop in 75% of adults; risk of malignant transformation to hepatocellular carcinoma requires annual surveillance ultrasound from age 16.

How does lactic acidosis differ in GSD Ia vs Ib?

GSD Ia causes severe hyperlactatemia from blocked gluconeogenesis; GSD Ib causes neutropenia and neutrophil dysfunction in addition to metabolic features.

What are long-term renal complications of GSD I?

Focal segmental glomerulosclerosis, hyperfiltration, and progressive CKD develop from chronic lactic acid-induced injury and abnormal substrate flux through the kidney.

Glycogen Storage Disease Type I (von Gierke) Metabolic Monitor

Monitors metabolic stability in Glycogen Storage Disease Type I using glucose, lactate, uric acid, triglycerides, and liver size, with cornstarch dosing optimization.

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Frequently Asked Questions

How is metabolic control assessed in GSD Ia?

What is uncooked cornstarch therapy?

What hepatic complications occur in GSD I?

How does lactic acidosis differ in GSD Ia vs Ib?

What are long-term renal complications of GSD I?