Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

RECRUITING

Clinical Phase

NA

Total Enrollment

20 participants

Study Classification

INTERVENTIONAL

Study Start Date

2025-04-24

Study Completion Date

2027-03-31

Brief Summary

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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.

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Detailed Description

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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

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Glycogen Storage Disease Type I

Study Design

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Allocation Method

RANDOMIZED

Intervention Model

CROSSOVER

Primary Study Purpose

TREATMENT

Blinding Strategy

NONE

Study Groups

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Diet with additional fructose intake

Group Type EXPERIMENTAL

Fructose

Intervention Type DIETARY_SUPPLEMENT

40g fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g)

Diet with additional fructose and galactose intake

Group Type EXPERIMENTAL

Fructose and galactose

Intervention Type DIETARY_SUPPLEMENT

10g galactose (mostly from lactose) plus 40g of fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g)

Interventions

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Fructose

40g fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g)

Intervention Type 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)

Intervention Type DIETARY_SUPPLEMENT

Eligibility Criteria

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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

Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No