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 studyReportar como inadecuado




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Orphanet Journal of Rare Diseases

, 10:18

First Online: 15 February 2015Received: 30 November 2014Accepted: 18 January 2015DOI: 10.1186-s13023-015-0229-6

Cite this article as: Bhattacharya, K., Mundy, H., Lilburn, M.F. et al. Orphanet J Rare Dis 2015 10: 18. doi:10.1186-s13023-015-0229-6

Abstract

BackgroundUncooked 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.

ObjectiveTo determine efficacy and safety of a new uncooked starch WMHMS compared to UCCS over 16 weeks treatment with each.

MethodA 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.

Results4 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.

ConclusionWMHMS leads to significant reduction in insulin release and reduced starch use in some GSD patients.

KeywordsGlycogen storage disease Corn-starch Glycemic index Carbohydrate Glycosade Insulin Glucose Lactate Waxy maize  Download fulltext PDF



Autor: Kaustuv Bhattacharya - Helen Mundy - Maggie F Lilburn - Michael P Champion - David W Morley - François Maillot

Fuente: https://link.springer.com/







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