Table 4.

Treatment of Manifestations in individuals with GSD VI

Manifestations/
Concern		TreatmentConsiderations/Other
Hypoglycemia
  • Frequent small meals
  • Uncooked cornstarch (1-1.5 g/kg) 1-4x/day
  • Protein 2-3 g/kg body weight per day
  • Glycosade 1 from waxy maize, proven beneficial in children age >5 yrs & adults to extend overnight fast duration
Doses adjusted to keep glucose concentrations at 75-100 mg/dL (4.2-5.6 mmol/L) & beta-OH-butyrate concentrations ≤0.2 mmol/L
Hepatomegaly
  • Restricted intake of simple sugars (<5 g)
  • Restricted intake of total carbohydrates (15-30 g per meal)
To ↓ liver size
Growth
restriction 		Cornstarch & protein supplementation
  • Growth normalizes w/treatment
  • Growth hormone contraindicated
Decreased
bone density
  • Cornstarch & protein supplementation
  • Calcium & Vitamin D
Primarily due to ketosis
Muscle
cramping 		Protein 2-3 grams per kg body weight per dayMuscle cramping is usually due to undertreatment & protein deficiency.
1.

Extended-release cornstarch

From: Glycogen Storage Disease Type VI

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