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

Launched by INSEL GRUPPE AG, UNIVERSITY HOSPITAL BERN · Feb 24, 2025

Keywords

ClinConnect Summary

This clinical trial is looking at how different dietary changes can help people with Glycogen Storage Disease Type I (GSDI), a rare condition that affects how the body processes sugar. The researchers want to see if loosening the current strict limits on foods containing fructose and galactose could improve the daily lives of adults with this condition. By allowing a wider variety of foods, they hope to enhance the quality of life for patients while still keeping their metabolism stable.

To participate in the trial, you must be an adult (18 years or older) who has been diagnosed with GSDI and currently follows a diet that restricts fructose. However, some individuals may not be eligible, such as those who have not followed their dietary treatment, are pregnant or breastfeeding, have had a liver transplant, or have certain serious health issues. Participants will need to provide written consent and can expect to be part of a study that could lead to changes in dietary recommendations for GSDI, potentially allowing for more food choices in the future.

Gender

ALL

Eligibility 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