ArticleJIMD reports2016
Safety and Efficacy of Chronic Extended Release Cornstarch Therapy for Glycogen Storage Disease Type I.
Article in JIMD reports, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed.
- Short and long-term acceptability and efficacy of extended-release cornstarch in the hepatic glycogen storage diseases: results from the Glyde study.Orphanet journal of rare diseases · 2024Trial
- Continuous Glucose Monitoring-Driven Personalization of Cornstarch Therapy in Glycogen Storage Disease: A Retrospective Analysis.Yonsei medical journal · 2026Article
- Observational
- Glucose dynamics in glycogen storage disease type IXa with novel PHKA2 variants: insights from our experience and a comprehensive review of the disease spectrum.Hormones (Athens, Greece) · 2025Review
- Managing Hypoglycaemia in Patients With Insulinoma-A Tertiary Centre Experience and Review of the Literature.Clinical endocrinology · 2025Review
- Idiopathic ketotic hypoglycemia in children: an update.Annals of pediatric endocrinology & metabolism · 2024Article
- Bedtime extended release cornstarch improves biochemical profile and sleep quality for patients with glycogen storage disease type Ia.Molecular genetics & genomic medicine · 2023Article
- Glycogen storage diseases: An update.World journal of gastroenterology · 2023Review
- Impact of hematopoietic stem cell transplantation in glycogen storage disease type Ib: A single-subject research design usingMolecular genetics and metabolism reports · 2023Article
- Glycogen storage disease type I: Genetic etiology, clinical manifestations, and conventional and gene therapies.Pediatric discovery · 2023Article
- Glycogen storage diseases with liver involvement: a literature review of GSD type 0, IV, VI, IX and XI.Orphanet journal of rare diseases · 2022Review
- Development of minimally invasiveMolecular genetics and metabolism reports · 2022Article
- Glycogen Storage Disease Type Ia: Current Management Options, Burden and Unmet Needs.Nutrients · 2021Review
- Towards enhanced understanding of idiopathic ketotic hypoglycemia: a literature review and introduction of the patient organization, Ketotic Hypoglycemia International.Orphanet journal of rare diseases · 2021Review
- [Advances on the management of renal lesion in glycogen storage disease type I].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2021Article
- A patient with glycogen storage disease type 0 and a novel sequence variant inThe Journal of international medical research · 2020Review
- Dietary Management of the Glycogen Storage Diseases: Evolution of Treatment and Ongoing Controversies.Advances in nutrition (Bethesda, Md.) · 2020Review
- Glycogen storage disease type 1a is associated with disturbed vitamin A metabolism and elevated serum retinol levels.Human molecular genetics · 2020Article
- Use of waxy maize heat modified starch in the treatment of children between 2 and 5 years with glycogen storage disease type I: A retrospective study.Molecular genetics and metabolism reports · 2019Article
- Metabolic lipid muscle disorders: biomarkers and treatment.Therapeutic advances in neurological disorders · 2019Review
Corrections and comments
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Authors and funding
9 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundGlycogen storage disease type I (GSD I) causes severe hypoglycemia during periods of fasting since both glycogenolysis and gluconeogenesis are impaired. Primary treatment in North America consists of cornstarch therapy every 3-4 h. Waxy maize extended release cornstarch was introduced for maintaining overnight glucose concentrations, but no studies have assessed long-term safety and efficacy of the product.
objectiveTo demonstrate the safety and efficacy of modified cornstarch in GSD I.
designAn open-label overnight trial of extended release cornstarch was performed. Subjects with a successful trial (optimal metabolic control 2 or more hours longer than with traditional cornstarch) were given the option of continuing into the chronic observational phase. Subjects were assessed biochemically at baseline and after 12 months.
resultsOf the 106 subjects (93 GSD Ia/13 GSD Ib), efficacy was demonstrated in 82 patients (88%) with GSD Ia and 10 patients (77%) with GSD Ib. The success rate for extending fasting was 95% for females and 78% for males. Of the patients who entered the longitudinal phase, long-term data are available for 44 subjects. Mean duration of fasting on traditional cornstarch prior to study for the cohort was 4.1 and 7.8 h on the extended release cornstarch (P < 0.001). All laboratory markers of metabolic control have remained stable in the chronically treated patients.
conclusionExtended release cornstarch appears to improve the quality of life of patients with GSD I without sacrificing metabolic control. Avoiding the overnight dose of cornstarch should enhance safety in this population.
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