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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: 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.To determine efficacy and safety of a new uncooked starch (WMHMS) compared to UCCS over 16 weeks treatment with each.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?

SUBMITTER: Bhattacharya K 

PROVIDER: S-EPMC4340332 | biostudies-literature | 2015

REPOSITORIES: biostudies-literature

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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.

Bhattacharya Kaustuv K   Mundy Helen H   Lilburn Maggie F MF   Champion Michael P MP   Morley David W DW   Maillot François F  

Orphanet journal of rare diseases 20150215


<h4>Background</h4>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.<h4>Objective</h4>To determine efficacy and safety of a new uncooked starch (WMHMS) compared to UCCS over 16 weeks treatment with each.<h4>Method</h4>A double-blind cross-over study of 10 adults (aged 16 - 38 years, six male) with GSD Ia and Ib. After an ind  ...[more]

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