Alternative nighttime nutrition regimens in glycogen storage disease type i: A controlled crossover study
Open accessAbstract
Background: Traditional approaches for nighttime glycemic control in glycogen storage disease type I (GSDI) include continuous tube feeding, or ingestion of uncooked corn starch (CS) at bedtime. A modified corn starch (MCS) has been shown to prolong euglycemia in some patients. The aim of this study was to evaluate whether stable nighttime glucose control can be achieved with other types of slowly digested carbohydrates in adult GSDI patients. Methods: In this cross‐over study, nocturnal glucose control and fasting times were assessed with three different nocturnal nutrition regimens in five patients, using continuous glucose monitoring (CGMS) in an outpatient everyday life setting. For each patient, continuous glucose profiles were measured after ingestion of (1) CS, (2) MCS or (3) a pasta meal at bedtime, during 5 to 6 consecutive nights for each regimen. Results: Stable nocturnal glucose control was achieved for all patients with a pasta meal, with a mean duration of glycemia >3.5 mmol/l of 7.6 h (range 5.7‐10.8), and >4 mmol/l of 7 h (5.2‐9.2), similar to CS and MCS. Fasting glucose before breakfast on workdays (after 7.1 ± 0.8 h) was not significantly different between the three interventions (CS 4.1 ± 0.5 mmol/l, MCS 4.6 ± 0.7 mmol/l, pasta 4.3 ± 0.9 mmol/l). During prolonged fasting on weekends, longer duration of normoglycemia was achieved with CS or MCS than with pasta. Conclusion: Consumption of cooked pasta is a suitable and more palatable alternative to uncooked corn starch to achieve nighttime glucose control in adult patients with GSDI.
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A 3 mg intranasal glucagon dose (containing 1 mg glucagon per 10 mg powder) seems to produce the maximum effect.
“Three mg of intranasal glucagon (1 mg glucagon per 10 mg dry powder) appears to have maximal effect.1231–1235”
From American Association of Clinical Endocrinology Clinical Practice Guideline: Developing a Diabetes Mellitus Comprehensive Care Plan-2022 Update. (2022) · cites this paper as [1233]
The Results and Discussion (page numbers unavailable) examine nighttime glucose control with cornstarch and pasta in patients with glycogen storage disease type I. Searches for intranasal glucagon, a 3 mg dose, and dry-powder formulation found no passage addressing the claimed maximal effect. (LLM, conf=0.78)
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- Authors
- Hochuli, M., Christ, E., Meienberg, F., Lehmann, R., Krützfeldt, J., & Baumgartner, M. R.
- Affiliations
- University of Zurich (CH)
- University Hospital of Bern (CH)
- University Hospital of Basel (CH)
- University Hospital Zurich (CH)
- Corresponding author
- Hochuli, Michel
- ORCID
- Venue
- Journal of Inherited Metabolic Disease
- Publisher
- Wiley
- Published
- 2015-06-25
- Volume
- 38
- Issue
- 6
- Pages
- 1093-1098
- ISSN
- 0141-8955 (ISSN-L)
- 1573-2665
- OpenAlex
- W798238325
- Cited by
- 15
- References
- 21
- Citations by year
- 2026: 2 · 2025: 2 · 2024: 1 · 2022: 3 · 2021: 1 · 2020: 1 · 2019: 2 · 2018: 1 · 2017: 1 · 2016: 1
- FWCI
- 0.84
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Journal of Inherited Metabolic Disease (publishedVersion)
- PubMed (publishedVersion)
- Zurich Open Repository and Archive (University of Zurich) (acceptedVersion, other-oa)
- RERO DOC (Universität Bern) (submittedVersion)
- Bern Open Repository and Information System (University of Bern) (submittedVersion)
- Zurich Open Repository and Archive (University of Zurich) (submittedVersion, other-oa)
- Zurich Open Repository and Archive (University of Zurich)
- Funders
- Universität Zürich
- 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
- glycogen storage disease type I, modified corn starch, fasting blood glucose, continuous glucose monitoring
- MeSH terms
- Adult, Blood Glucose, Dietary Carbohydrates, Fasting, Female, Glycogen Storage Disease Type I, Humans, Hypoglycemia, Insulin, Male, Starch, Linear Models, Cross-Over Studies, Young Adult
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