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Safety and Efficacy of Long-Term Use of Extended Release Cornstarch Therapy for Glycogen Storage Disease Types 0, III,...
ISSN: 1929 - 5634
Publisher: author
Safety and Efficacy of Long-Term Use of Extended Release Cornstarch Therapy for Glycogen Storage Disease Types 0, III,...
Indexed in
Health Sciences
ARTICLE-FACTOR
1.3
Article Basics Score: 3
Article Transparency Score: 3
Article Operation Score: 3
Article Articles Score: 2
Article Accessibility Score: 2
SUBMIT PAPER ASK QUESTION
International Category Code (ICC):
ICC-0702
Publisher: Lifescience Global Inc.
International Journal Address (IAA):
IAA.ZONE/1929107195634
eISSN
:
1929 - 5634
VALID
ISSN Validator
Abstract
Background: Impaired glycogen release with fasting results in hypoglycemia in the glycogen storage diseases. A waxy-maize extended release cornstarch was introduced in the United States in 2012 to maintain glucose concentrations during the overnight period, but no studies have assessed the long-term safety and efficacy of this product in the ketotic forms of GSD. Objective: To assess long-term safety and efficacy of modified cornstarch in patients with ketotic forms of GSD. Design: An open label overnight trial of extended release cornstarch was performed. Subjects who had a successful trial (defined as optimal metabolic control lasting 2 or more hours more than with traditional cornstarch) were given the option of continuing into the long-term observational phase. Participants were assessed biochemically at baseline and after 12 months. Results: A total of 16 subjects participated in the open label trial. Efficacy was demonstrated in...
