ReviewJournal of inherited metabolic disease2011
Dietary dilemmas in the management of glycogen storage disease type I.
Review in Journal of inherited metabolic disease, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed.
- 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.Orphanet journal of rare diseases · 2015Trial
- Health-Related Quality of Life in Pediatric Hepatic Glycogen Storage Disease: A Dual-Perspective Study.Nutrients · 2026Article
- Navigating Glycogen Storage Disease: The Spiritual and Cultural Journeys of Jordanian Mothers.Journal of religion and health · 2026Article
- Proteomics profiling of serum and liver in GSD Ia and Ib patients: insights into complication mechanisms and circulation biomarkers.Journal of translational medicine · 2026Article
- Feeding Difficulties in Children with Hepatic Glycogen Storage Diseases Identified by a Brazilian Portuguese Validated Screening Tool.Nutrients · 2025Article
- Article
- Nutrition Management in Children Less than 5 Years of Age with Glycogen Storage Disease Type I: Survey Results.Nutrients · 2024Article
- Endocrine involvement in hepatic glycogen storage diseases: pathophysiology and implications for care.Reviews in endocrine & metabolic disorders · 2024Review
- Intestinal microbiota composition of children with glycogen storage Type I patients.European journal of clinical nutrition · 2024Article
- Glycogen storage diseases: An update.World journal of gastroenterology · 2023Review
- Estimation of Health Utility Scores for Glycogen Storage Disease Type Ia.PharmacoEconomics - open · 2023Article
- Current understanding on pathogenesis and effective treatment of glycogen storage disease type Ib with empagliflozin: new insights coming from diabetes for its potential implications in other metabolic disorders.Frontiers in endocrinology · 2023Review
- Development of minimally invasiveMolecular genetics and metabolism reports · 2022Article
- Untargeted plasma metabolomics identifies broad metabolic perturbations in glycogen storage disease type I.Journal of inherited metabolic disease · 2022Observational
- Impaired Very-Low-Density Lipoprotein catabolism links hypoglycemia to hypertriglyceridemia in Glycogen Storage Disease type Ia.Journal of inherited metabolic disease · 2021Article
- The natural history of glycogen storage disease type Ib in England: A multisite survey.JIMD reports · 2021Article
- ANTHROPOMETRIC AND DIETARY ASSESSMENT OF PATIENTS WITH GLYCOGENOSIS TYPE I.Revista paulista de pediatria : orgao oficial da Sociedade de Pediatria de Sao Paulo · 2021Article
- Glycogen metabolism and glycogen storage disorders.Annals of translational medicine · 2018Review
- Insulin-resistance in glycogen storage disease type Ia: linking carbohydrates and mitochondria?Journal of inherited metabolic disease · 2018Article
- Alternative nighttime nutrition regimens in glycogen storage disease type I: a controlled crossover study.Journal of inherited metabolic disease · 2015Article
Corrections and comments
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Authors and funding
1 author.
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Abstract
Over the last 50 years, understanding the biochemical bases of glycogen storage disease type I has led to vastly improved survival and health outcomes but the management still centres around an extremely intensive dietary regimen. Patients' metabolic profiles are really determined by the whole of the diet and it can be very difficult to adjust therapy accordingly. In an iso-energetic diet with reference total energy intake, high carbohydrate intake could compromise other macro- and micro-nutrients; if carbohydrates are not restricted then total energy intake is excessive. The quality of the macronutrient such as the glycemic index of carbohydrate, the type of sugar and the proportion of medium-chain triglyceride and essential fatty acids also has a bearing on an individual's long-term metabolic control with potential clinical correlates. These factors as well as the different requirements between individuals and within individuals as they get older mean that the management of glycogen storage disease type I is particularly fraught. Regular clinical and dietary review is imperative as patients grow, ensuring adequate but not excessive low glycaemic index carbohydrate intake, appropriate dynamic biochemical profiles and suitable age appropriate eating patterns. Without diligent management, and education that empowers the patient, these individuals can struggle in adult life.
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