GSD type I most commonly presents as hypoglycemia and/or hepatomegaly in infants. Keeping blood glucose consistently above the target threshold is the central challenge in this age group, and the approach must be stepped up when initial measures fall short.
This protocol addresses infants with GSD type I whose blood glucose cannot be reliably maintained above 70 mg/dl on the first-line dietary approach alone.
Infants managed with a soy-based, sugar-free formula or a formula free of sucrose, fructose, and lactose, offered on demand every 2–3 hours, who still fail to maintain blood glucose above 70 mg/dl meet the threshold for escalation to this protocol.
When frequent formula feeds are insufficient, the recognised next approach involves continuous enteral feeding delivered via a nasogastric tube or a surgically placed gastrostomy tube. The complete regimen — including feeding parameters, timing of feeds, and guidance on the introduction of an alternative carbohydrate source later in infancy — is in the full structured protocol.
Target: blood glucose > 70 mg/dlDOI: 10.1038/gim.2014.128
GSD I most commonly presents as hypoglycemia and/or hepatomegaly in infants.
Another option is to use overnight gastric feedings (OGFs).
In general, the rate of the continuous tube feeding is calculated to provide a glucose infusion rate of 8–10 mg glucose/kg/min during infancy and 4–8 mg glucose/kg/min in older children.
Raw, uncooked cornstarch may be introduced between 6 and 12 months of age.
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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