Treatment of Glycogen Storage Disease Type I in Infants

GSD type I demands prompt, protocol-driven metabolic management beginning in infancy. The initial treatment strategy in this age group is specific and evidence-based.

Clinical Scenario

GSD I most commonly presents as hypoglycemia and/or hepatomegaly in infants. Early recognition of this presentation in infancy is what directs the treatment approach.

Treatment Approach

First-line management centres on a specialized dietary formula approach. The complete formula specification and feeding protocol are detailed in the full regimen.

Treatment Goal

The primary metabolic target is to maintain blood glucose at a defined safe level.

Blood glucose > 70 mg/dl

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1038/gim.2014.128

GSD I most commonly presents as hypoglycemia and/or hepatomegaly in infants.

In infancy, a soy-based, sugar-free formula or a formula that is free of sucrose, fructose, and lactose is fed on demand every 2–3 hours.

The formula may be offered every 4 hours by mouth and/or by tube, or the formula may be infused continually at a rate to provide adequate glucose to maintain the BG level at more than 70 mg/dl or 4 mmol/l.

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