Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Fructose, Fructose and galactose.
- Who it may be relevant to
- Registry conditions: Glycogen Storage Disease Type I. Basic parameters: from 18 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- Switzerland
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
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Official title
Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I (GSD-DIET)
Overview
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.
Detailed description
To assess relaxed restriction of fructose and fructose/galactose intake on secondary metabolic alterations in GSDI, (i) by looking at the traditional parameters for assessing metabolic control in clinical chemistry (lactate, triglycerides, uric acid), and (ii) by using a broad analytical approach relying on targeted metabolomics/lipidomics.
It is hypothesized that relaxed restrictions on the intake of fructose and/or galactose as part of the diet in everyday life may lead to an increase in blood lactate levels (=primary outcome), triglycerides, and uric acid to a certain degree compared to baseline. However, this increase is expected to remain within a range that is not clinically relevant for adult patients, especially when fructose/galactose intake is not excessive and stays within the usual daily allowances for healthy individuals, as planned in this study.
Interventions
- Dietary supplement Fructose
40g fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g) - Dietary supplement Fructose and galactose
10g galactose (mostly from lactose) plus 40g of fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g)
Primary outcome measures
- Change in lactate during the dietary intervention compared to baseline under the usual diet [Time frame: 4 Weeks]
Secondary outcome measures (4)
- Change in plasma triglycerides during the dietary intervention compared to baseline (as parameters traditionally measured together with lactate to estimate overall metabolic control) [Time frame: 4 Weeks]
- Change in plasma uric acid during the dietary intervention compared to baseline (as parameters traditionally measured together with lactate to estimate overall metabolic control) [Time frame: 4 Weeks]
- Plasma metabolite changes in targeted metabolomics during the dietary intervention compared to baseline measurements. [Time frame: 4 Weeks]
- Plasma metabolite changes in targeted lipidomics during the dietary intervention compared to baseline measurements. [Time frame: 4 Weeks]
Eligibility criteria
Inclusion criteria
- 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 criteria
- 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
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Allocation
- Randomized
- Model
- Crossover
- Masking
- Open label
- Primary purpose
- Treatment
Study locations
Switzerland · 2 centers
- Department of Diabetes, Endocrinology, Nutritional Medicine and Metabolism, Inselspital, B — Bern
- Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zuric — Zurich
Identifiers
NCT: NCT06852612 · 2024-02154