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

ConditionGlycogen Storage Disease Type I
Trial statusRecruiting
Trial phaseNot applicable
Trial typeInterventional
Biological sexAll
Age18+
SponsorInsel Gruppe AG, University Hospital Bern

About this trial

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 criteria

This trial does not accept healthy volunteers

Qualifiers

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

Disqualifiers

Non-compliance with routine dietary treatment

Pregnancy or lactation

Liver transplant

Recurrent hospitalisations due to metabolic decompensation within the last 12 months

Trial design

Design model

Crossover

Treatments tested in this trial

  • Fructose

    Dietary supplement

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

  • Fructose and galactose

    Dietary supplement

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

Treatment groups

20 Participants
are divided into 2 treatment groups
Group A: Diet with additional fructose intakeExperimental treatment 1 intervention
Group B: Diet with additional fructose and galactose intakeExperimental treatment 1 intervention

Trial outcomes

Primary outcomes

1

Change in lactate during the dietary intervention compared to baseline under the usual diet

Lactate belongs to the parameters of secondary metabolic disturbance traditionally used to estimate metabolic control in GSDI in routine clinical practice (lactate, triglycerides, uric acid). Redundant measurements of lactate are performed in blood as well as in collected urine.

Time frame
4 Weeks

Secondary outcomes

1

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
2

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
3

Plasma metabolite changes in targeted metabolomics during the dietary intervention compared to baseline measurements.

Time frame
4 Weeks
4

Plasma metabolite changes in targeted lipidomics during the dietary intervention compared to baseline measurements.

Time frame
4 Weeks

Other outcomes

Sponsors and contacts

Click on the lead sponsor to view all of their trials.

Insel Gruppe AG, University Hospital Bern

Lead sponsor

University of Zurich

Collaborator

University Hospital Freiburg

Collaborator

University Hospital Heidelberg

Collaborator

Medical University Innsbruck

Collaborator

Source ClinicalTrials.gov
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