GSD Type VI: When Initial Dietary Management Fails to Maintain Overnight Glucose and Ketone Targets

This protocol covers the next step in Glycogen storage disease type VI when first-line dietary management has not achieved adequate overnight metabolic control. It addresses the specific question of what to do after the standard initial approach has fallen short of its targets.

Prior Therapy — Targets Not Met

The initial regimen — avoidance of fasting with small frequent feedings, high protein diet, and bedtime corn starch — did not sustain blood glucose between 70 and 100 mg/dL or blood ketones in the range 0.0–0.2 mmol/L through the overnight period. Failure to reach these metabolic targets is the trigger for this next-line protocol.

Next-Line Approach — Partial Overview

This protocol introduces a different formulation of corn starch — one with modified release characteristics derived from a specific starch source — that is used in eligible patients to extend overnight metabolic protection beyond what standard corn starch achieves.

Full eligibility criteria, regimen details, and clinical monitoring guidance are available in the complete structured protocol below.

Treatment Targets

Blood glucose between 70 and 100 mg/dL; blood ketones in the range 0.0–0.2 mmol/L.

Instant Access to Structured Evidence-Based Regimens

References

The use of an extended release corn starch from waxy maize (Glycosade®) has proven to be beneficial in children over 5 years and adults to extend the time to overnight hypoglycemia.

The blood glucose (BG) should range between 70 and 100 mg/dL, and the target range for blood ketones is 0.0–0.2 mmol/L.

DOI: 10.1038/s41436-018-0364-2

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