A novel starch for the treatment of glycogen storage diseases
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To determine whether a new starch offers better short-term metabolic control than uncooked cornstarch in patients with glycogen storage diseases (GSDs).
A short-term double-blind cross-over pilot study comparing uncooked physically modified cornstarch (WMHM20) with uncooked cornstarch in patients with GSD types Ia, Ib and III. Twenty-one patients (ages 3–47, 9 female) were given 2 g/kg cornstarch or WMHM20 mixed in water. Blood glucose, lactate and insulin, and breath hydrogen and 13CO2 enrichment were measured, at baseline and after each load. The hourly biochemical evaluations terminated when blood glucose was ≤3.0 mmol/L, when the study period had lasted 10 h or when the patient wished to end the test. The alternative starch was administered under similar trial conditions a median of 10 days later.
The median starch load duration was 9 h for WMHM20 versus 7 h for cornstarch. Glucose decreased more slowly (p=0.05) and lactate was suppressed faster (p=0.17) for WMHM20 compared with cornstarch. Peak hydrogen excretion was increased (p=0.05) when cornstarch was taken.
These data indicate longer duration of euglycaemia and better short-term metabolic control in the majority of GSD patients with WMHM20 compared to cornstarch.
KeywordsCornstarch Glycogen Storage Disease Glycogen Storage Disease Type Breath Hydrogen Hydrogen Excretion
continuous nocturnal pump feed
glycogen storage disease
Pee Dee Belemnite
Waxy Maize (Heat Modified) 20
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