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.
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
Non-compliance with routine dietary treatment
Pregnancy or lactation
Liver transplant
Recurrent hospitalisations due to metabolic decompensation within the last 12 months
Insel Gruppe AG, University Hospital Bern
Lead sponsor
University of Zurich
Collaborator
University Hospital Freiburg
Collaborator
University Hospital Heidelberg
Collaborator
Medical University Innsbruck
Collaborator