Continuous glucose for treatment of patients with type 1 glycogen-storage disease: comparison of the effects of dextrose and uncooked cornstarch on biochemical variables
Abstract
Responses to uncooked cornstarch (UCS), dextrose (Dex), and a 3:1 mixture (UCS:Dex) were determined in seven children with type 1 glycogen-storage disease (GSD-1). UCS maintained blood glucose (BG) and serum insulin concentrations between 3.5 +/- 0.3 and 4.0 +/- 0.4 mmol/L (mean +/- SEM) and 50 +/- 7 and 79 +/- 22 pmol/L, respectively, in six of the seven patients for 4 h. Only four of seven patients completed the 4-h test after UCS:Dex (BG 2.9 +/- 0.3 mmol/L): After Dex, tests had to be stopped in all patients by 150 min after initiation (BG 2.7 +/- 0.4 mmol/L). Two methods of providing dietary glucose overnight, continuous intragastric glucose infusion (COG) and intermittent UCS at 2100 and 0200, were compared by monitoring metabolites and glucoregulatory hormones. The use of UCS in amounts equal to the calculated glucose production rate is an effective method of providing a continuous dietary source of glucose overnight to patients with GSD-1.
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Plasma lactate rises as plasma glucose falls below approximately 70 mg/dL in children with glucose-6-phosphatase deficiency.
“plasma lactate rises as PG falls below ~70 mg/dL (~3.9 mmol/L) in children with glucose-6-phosphatase deficiency23”
From Recommendations from the Pediatric Endocrine Society for evaluation and management of persistent hypoglycemia in neonates, infants, and children (2015) · cites this paper as [23]
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- Authors
- Wolfsdorf, J., Plotkin, R., Laffel, L., & Crigler, J.
- Affiliations
- Boston Children's Hospital (US)
- Venue
- The American Journal of Clinical Nutrition
- Publisher
- Elsevier BV
- Published
- 1990-12
- Volume
- 52
- Issue
- 6
- Pages
- 1043-1050
- ISSN
- 0002-9165 (ISSN-L)
- OpenAlex
- W1825888921
- Cited by
- 43
- References
- 40
- Citations by year
- 2025: 1 · 2023: 1 · 2021: 1 · 2019: 1 · 2018: 2 · 2017: 2 · 2015: 1 · 2013: 1 · 2012: 2
- FWCI
- 2.59
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- American Journal of Clinical Nutrition (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Center for Research Resources (M01RR002172, RR02172)
- Topics
- Health Sciences › Medicine › Rheumatology › Glycogen Storage Diseases and Myoclonus
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Diabetes Management and Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Keywords
- uncooked cornstarch, D-glucose, continuous glucose infusion, blood glucose, serum insulin, glucoregulatory hormones, children, dietary glucose
- MeSH terms
- Adolescent, Blood Glucose, Child, Child, Preschool, Cholesterol, Female, Glucose, Glycogen Storage Disease Type I, Humans, Hydrocortisone, Hydrogen-Ion Concentration, Insulin, Lactates, Male, Growth Hormone, Starch, Triglycerides, Uric Acid
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