Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I
Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I (GSD-DIET)
Insel Gruppe AG, University Hospital Bern
20 participants
Apr 24, 2025
INTERVENTIONAL
Conditions
Summary
The present project will specifically assess metabolic effects of dietary interventions with controlled intake of fructose and fructose/galactose in GSDI, with the aim to provide evidence whether relaxed dietary restrictions of fructose and galactose may be justified in treatment recommendations at least for adults, which would considerably enlarge food choice in everyday life of the patients with an expected positive impact on the quality of life of patients with this rare disorder.
Eligibility
Inclusion Criteria4
- Genetically and/or enzymatically confirmed diagnosis of GSDI (GSDIa or GSDIb)
- Male or female ≥ 18y
- Restriction of fructose intake in usual dietary treatment
- Written informed consent
Exclusion Criteria6
- Non-compliance with routine dietary treatment
- Pregnancy or lactation
- Liver transplant
- Recurrent hospitalisations due to metabolic decompensation within the last 12 months
- Severe chronic kidney disease with glomerular filtration rate (GFR) < 30 ml/min
- For GSDIb: Severe, uncontrolled symptomatic inflammatory bowel disease
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Interventions
40g fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g)
10g galactose (mostly from lactose) plus 40g of fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g)
Locations(1)
View Full Details on ClinicalTrials.gov
For the most up-to-date information, visit the official listing.
NCT06852612