Dietary strategies for managing Glycogen Storage Disease Type I
Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I (GSD-DIET)
This study tests if allowing adults with Glycogen Storage Disease Type I to eat more foods with fructose and galactose can improve their quality of life without causing health problems.
Quick facts
| Phase | Not applicable |
|---|---|
| Study type | Interventional |
| Enrollment | 20 (estimated) |
| Ages | 18 Years and up |
| Sex | All |
| Sponsor | Insel Gruppe AG, University Hospital Bern Academic / other |
| Locations | 2 sites (Bern and 1 other locations) |
| Trial ID | NCT06852612 on ClinicalTrials.gov |
What this trial studies
This project evaluates the metabolic effects of dietary interventions that allow controlled intake of fructose and galactose in patients with Glycogen Storage Disease Type I (GSDI). The study aims to determine if relaxed dietary restrictions can be safely implemented for adults, potentially improving their quality of life by expanding food choices. It will assess traditional metabolic control parameters and utilize advanced metabolomics and lipidomics techniques to analyze the impact of these dietary changes. The hypothesis is that moderate increases in blood lactate, triglycerides, and uric acid levels will remain clinically insignificant for patients adhering to the new dietary guidelines.
Who should consider this trial
Good fit: Ideal candidates include adults aged 18 and older with a confirmed diagnosis of GSDI who currently restrict fructose intake.
Not a fit: Patients who are non-compliant with dietary treatment, pregnant, or have severe chronic conditions may not benefit from this study.
Why it matters
Potential benefit: If successful, this study could significantly enhance the quality of life for adults with GSDI by allowing greater dietary flexibility.
How similar studies have performed: While dietary interventions in metabolic disorders have been explored, this specific approach to relaxing fructose and galactose restrictions in GSDI is relatively novel.
Eligibility criteria
Show full inclusion / exclusion 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
Where this trial is running
Bern and 1 other locations
- Department of Diabetes, Endocrinology, Nutritional Medicine and Metabolism, Inselspital, Bern University Hospital — Bern, Switzerland (Recruiting)
- Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich — Zurich, Switzerland (Not_yet_recruiting)
Study contacts
- Principal investigator: Michel Hochuli, MD, PhD — Department of Diabetes, Endocrinology, Nutritional Medicine and Metabolism
- Study coordinator: Michel Hochuli, MD, PhD
- Email: michel.hochuli@insel.ch
- Phone: +41 31 664 03 18
How to participate
- Review the eligibility criteria above with your treating physician.
- Visit the official trial page on ClinicalTrials.gov for the most current contact information and recruitment status.
- Contact the listed study coordinator or principal investigator to request pre-screening. Pre-screening is free and never obligates you to enroll.