Dietary Treatment Strategies and Metabolic Control in Glycogen Storage Disease Type I
Trial Parameters
Brief Summary
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
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