Actively Recruiting
Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I (GSD-DIET)
Led by Insel Gruppe AG, University Hospital Bern · Updated on 2026-05-12
20
Participants Needed
2
Research Sites
N/A
Total Duration
On this page
Sponsors
I
Insel Gruppe AG, University Hospital Bern
Lead Sponsor
U
University of Zurich
Collaborating Sponsor
AI-Summary
What this Trial Is About
Researchers are evaluating the metabolic effects of dietary changes involving controlled intake of fructose and fructose/galactose in adults with Glycogen Storage Disease Type I (GSDI). This study aims to determine whether relaxing dietary restrictions on these sugars is safe and could improve food choices and quality of life for patients with this rare disorder. The research assesses traditional metabolic control markers and uses advanced metabolomics and lipidomics for a broad analysis. The study involves two dietary intervention groups: one with added fructose intake (40g, including up to 25g from added sugars) and another with added fructose plus galactose (10g galactose mostly from lactose plus 40g fructose). These interventions are compared against the participants' usual diet over a 4-week period to observe changes in metabolic markers. Participants will undergo assessments of blood lactate, triglycerides, uric acid, and plasma metabolites before and after the dietary interventions. Researchers will monitor these parameters to evaluate metabolic control and safety. The main outcome is the change in blood lactate levels during the intervention compared to baseline. The study lasts at least 4 weeks, with regular monitoring to understand the impact of dietary changes on metabolic health in adult GSDI patients.
CONDITIONS
Brief Title
Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I
Who Can Participate
Eligibility Criteria
You may qualify if you...
- Genetically and/or enzymatically confirmed diagnosis of GSDI (GSDIa or GSDIb)
- Male or female aged 18 years or older
- Currently following a diet that restricts fructose intake
- Able to provide written informed consent
You will not qualify if you...
- Not following routine dietary treatment
- Pregnant or breastfeeding
- History of liver transplant
- Recurrent hospitalizations due to metabolic problems in the last 12 months
- Severe chronic kidney disease with glomerular filtration rate below 30 ml/min
- For GSDIb: Severe, uncontrolled symptomatic inflammatory bowel disease
AI-Screening
AI-Powered Screening
Complete this quick 3-step screening to check your eligibility
Your Study Journey
Duration - 2 to 4 weeks
Participants are screened for eligibility to participate in the trial.
1 visit (in-person)
Duration - 4 weeks
Participants follow different dietary treatment strategies involving additional fructose or fructose and galactose intake to assess metabolic control.
Weekly visits for up to 4 weeks
Trial Site Locations
Total: 2 locations
1
Department of Diabetes, Endocrinology, Nutritional Medicine and Metabolism, Inselspital, Bern University Hospital
Bern, Switzerland, 3010
Actively Recruiting
2
Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich
Zurich, Switzerland, 8091
Not Yet Recruiting
Research Team
M
Michel Hochuli, MD, PhD
How is the study designed?
Study Type
INTERVENTIONAL
Masking
NONE
Allocation
RANDOMIZED
Model
CROSSOVER
Primary Purpose
TREATMENT
Number of Arms
2
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