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- Title
A pilot longitudinal study of the use of waxy maize heat modified starch in the treatment of adults with glycogen storage disease type I: a randomized double-blind cross-over study.
- Authors
Bhattacharya, Kaustuv; Mundy, Helen; Lilburn, Maggie F.; Champion, Michael P.; Morley, David W.; Maillot, François
- Abstract
Background: Uncooked corn-starch (UCCS) has been the mainstay of therapy for the hepatic glycogen storage diseases (GSD) but is not always effective. A new starch (WMHMS) has demonstrated a more favourable short-term metabolic profile. Objective: To determine efficacy and safety of a new uncooked starch (WMHMS) compared to UCCS over 16 weeks treatment with each. Method: A double-blind cross-over study of 10 adults (aged 16 - 38 years, six male) with GSD Ia and Ib. After an individualised fast, subjects were randomised to take a 50 g starch-load of either WMHMS or UCCS. Starch-loads terminated when blood glucose was < 3.0 mmol/L or the subject felt subjectively hypoglycaemic. Anonymous biochemical profiles were assessed by 2 investigators and a starch administration schedule recommended. Each starch was delivered in coded sachets and intake was monitored for the following 16 weeks. After a washout period, the protocol was repeated with the alternative product. Results: 4 subjects failed to establish therapy on the cross-over limb. Data from 7 paired starch load showed: longer median fasting duration with WMHMS (7.5 versus 5 hours; p = 0.023), slower decrease in the glucose curve (0.357 versus 0.632 mmol/hr p = 0.028) and less area under insulin curves for the first 4 hours (p = 0.03). Two of six subjects took 50% or less WMHMS compared to UCCS and one took more. Plasma triglycerides, cholesterol and uric acid were unchanged after each study phase. Conclusion: WMHMS leads to significant reduction in insulin release and reduced starch use in some GSD patients.
- Subjects
CORNSTARCH; GLYCOGEN storage disease; GLYCOGEN phosphorylase; GLYCEMIC index; CARBOHYDRATE content of food
- Publication
Orphanet Journal of Rare Diseases, 2015, Vol 10, Issue 1, p1
- ISSN
1750-1172
- Publication type
Article
- DOI
10.1186/s13023-015-0229-6