NCT06852612

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)

• Status: Recruiting
• Phase: N/A
• Study type: Interventional
• Enrollment: 20 (estimated)
• Sponsor: Insel Gruppe AG, University Hospital Bern · Academic / Other
• Sex: All
• Age: 18 Years
• Healthy volunteers: Not accepted

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.

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.

Conditions

• Glycogen Storage Disease Type I

Interventions

Type	Name	Description
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)

Timeline

• Start date: 2025-04-24
• Primary completion: 2027-03-01
• Completion: 2027-03-01
• First posted: 2025-02-28
• Last updated: 2025-04-24

Locations

1 site across 1 country: Switzerland