Dietary strategies for managing Glycogen Storage Disease Type I

Is this trial a fit for you?

A plain-English reading of the study's rules. Only the study team can confirm whether you qualify.

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.

Why it matters

Who can join: the full rules

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 it's running

• 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

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How to take part

1. Review the eligibility criteria above with your treating physician.
2. Check the official listing on ClinicalTrials.gov for the latest contact details and recruitment status.
3. Contact the study coordinator or principal investigator to ask for pre-screening. Pre-screening is free and never obligates you to enroll.