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.

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.

• Authors: Kaustuv Bhattacharya, Helen Mundy, Maggie F Lilburn, Michael P Champion, David W Morley, François Maillot
• Journal: Orphanet journal of rare diseases (Orphanet J Rare Dis) Vol. 10 Pg. 18 (Feb 15 2015) ISSN: 1750-1172 [Electronic] England
• PMID: 25758258 (Publication Type: Journal Article, Randomized Controlled Trial)

Chemical References

• Blood Glucose
• Starch

Topics

• Adolescent
• Adult
• Blood Glucose (drug effects)
• Cross-Over Studies
• Double-Blind Method
• Female
• Glycogen Storage Disease Type I (diet therapy)
• Humans
• Male
• Pilot Projects
• Starch (chemistry, pharmacology)
• Young Adult