ReviewJournal of inherited metabolic disease2015
Dietary management in glycogen storage disease type III: what is the evidence?
Review in Journal of inherited metabolic disease, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
26 citing papers in PubMed, 48 citations in OpenAlex.
- Effects of acute nutritional ketosis during exercise in adults with glycogen storage disease type IIIa are phenotype-specific: An investigator-initiated, randomized, crossover study.Journal of inherited metabolic disease · 2021Trial
- Highly potent MyoAAV4A vector reverses GSD III pathology in aged mice and enables long-term muscle disease correction in young adult mice.Molecular therapy. Advances · 2026Article
- Association of the children's dietary inflammatory index (c-DII) with thiol/disulfide homeostasis in pediatric glycogen storage disease: a cross-sectional exploratory study.Frontiers in nutrition · 2026Article
- Avascular necrosis as an uncommon manifestation in glycogen storage disease type III: diagnostic and therapeutic challenges.Oxford medical case reports · 2025Article
- Galectin-3: a novel biomarker of glycogen storage disease type III.Cell death discovery · 2025Article
- Olfactory and gustatory perception in Brazilian PKU patients: A cross-sectional study.JIMD reports · 2024Article
- Synergism of dual AAV gene therapy and rapamycin rescues GSDIII phenotype in muscle and liver.JCI insight · 2024Article
- Glycogen storage disease type III: a mixed-methods study to assess the burden of disease.Therapeutic advances in endocrinology and metabolism · 2024Article
- Review
- French recommendations for the management of glycogen storage disease type III.European journal of medical research · 2023Review
- Glycogen storage diseases: An update.World journal of gastroenterology · 2023Review
- Improvement in hypertrophic cardiomyopathy after using a high-fat, high-protein and low-carbohydrate diet in a non-adherent child with glycogen storage disease type IIIa.Molecular genetics and metabolism reports · 2022Article
- Over 20-Year Follow-up of Patients with Hepatic Glycogen Storage Diseases: Single-Center Experience.Diagnostics (Basel, Switzerland) · 2020Article
- Glycogen storage disease type VI: clinical course and molecular background.European journal of pediatrics · 2020Observational
- Uniparental isodisomy of chromosome 1 results in glycogen storage disease type III with profound growth retardation.Molecular genetics & genomic medicine · 2019Article
- Feeding Difficulties and Orofacial Myofunctional Disorder in Patients with Hepatic Glycogen Storage Diseases.JIMD reports · 2019Article
- Glucose-free/high-protein diet improves hepatomegaly and exercise intolerance in glycogen storage disease type III mice.Biochimica et biophysica acta. Molecular basis of disease · 2018Article
- Rescue of GSDIII Phenotype with Gene Transfer Requires Liver- and Muscle-Targeted GDE Expression.Molecular therapy : the journal of the American Society of Gene Therapy · 2018Article
- Tight metabolic control plus ACE inhibitor therapy improves GSD I nephropathy.Journal of inherited metabolic disease · 2017Article
- Glycogen storage disease type III: diagnosis, genotype, management, clinical course and outcome.Journal of inherited metabolic disease · 2016Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
In childhood, GSD type III causes relatively severe fasting intolerance, classically associated with ketotic hypoglycaemia. During follow up, history of (documented) hypoglycaemia, clinical parameters (growth, liver size, motor development, neuromuscular parameters), laboratory parameters (glucose, lactate, ALAT, cholesterol, triglycerides, creatine kinase and ketones) and cardiac parameters all need to be integrated in order to titrate dietary management, for which age-dependent requirements need to be taken into account. Evidence from case studies and small cohort studies in both children and adults with GSD III demonstrate that prevention of hypoglycaemia and maintenance of euglycemia is not sufficient to prevent complications. Moreover, over-treatment with carbohydrates may even be harmful. The ageing cohort of GSD III patients, including the non-traditional clinical presentations in adulthood, raises new questions.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.