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| Name | Class |
|---|---|
| University of Zurich | OTHER |
| University Hospital Freiburg | OTHER |
| University Hospital Heidelberg | OTHER |
| Medical University Innsbruck |
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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.
To assess relaxed restriction of fructose and fructose/galactose intake on secondary metabolic alterations in GSDI, (i) by looking at the traditional parameters for assessing metabolic control in clinical chemistry (lactate, triglycerides, uric acid), and (ii) by using a broad analytical approach relying on targeted metabolomics/lipidomics.
It is hypothesized that relaxed restrictions on the intake of fructose and/or galactose as part of the diet in everyday life may lead to an increase in blood lactate levels (=primary outcome), triglycerides, and uric acid to a certain degree compared to baseline. However, this increase is expected to remain within a range that is not clinically relevant for adult patients, especially when fructose/galactose intake is not excessive and stays within the usual daily allowances for healthy individuals, as planned in this study.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Diet with additional fructose intake | Experimental |
| |
| Diet with additional fructose and galactose intake | Experimental |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Fructose | Dietary Supplement | 40g fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g) |
|
| Measure | Description | Time Frame |
|---|---|---|
| Change in lactate during the dietary intervention compared to baseline under the usual diet | Lactate belongs to the parameters of secondary metabolic disturbance traditionally used to estimate metabolic control in GSDI in routine clinical practice (lactate, triglycerides, uric acid). Redundant measurements of lactate are performed in blood as well as in collected urine. | 4 Weeks |
| Measure | Description | Time Frame |
|---|---|---|
| Change in plasma triglycerides during the dietary intervention compared to baseline (as parameters traditionally measured together with lactate to estimate overall metabolic control) | 4 Weeks | |
| Change in plasma uric acid during the dietary intervention compared to baseline (as parameters traditionally measured together with lactate to estimate overall metabolic control) |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Michel Hochuli, MD, PhD | Contact | +41 31 664 03 18 | michel.hochuli@insel.ch |
| Name | Affiliation | Role |
|---|---|---|
| Michel Hochuli, MD, PhD | Department of Diabetes, Endocrinology, Nutritional Medicine and Metabolism | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Department of Diabetes, Endocrinology, Nutritional Medicine and Metabolism, Inselspital, Bern University Hospital | Recruiting | Bern | 3010 | Switzerland |
Individual participant data (IPD) will be made available upon reasonable request to the principal investigator.
IPD will be made available starting 12 months after publication of trial results.
Ethics approval, as applicable under Swiss legislation, will need to be obtained by those requesting the data. Additionally, a data transfer and processing agreement must be in place to ensure compliance with data protection regulations.
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| ID | Term |
|---|---|
| D005953 | Glycogen Storage Disease Type I |
| ID | Term |
|---|---|
| D006008 | Glycogen Storage Disease |
| D002239 | Carbohydrate Metabolism, Inborn Errors |
| D008661 | Metabolism, Inborn Errors |
| D030342 | Genetic Diseases, Inborn |
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| ID | Term |
|---|---|
| D005632 | Fructose |
| D005690 | Galactose |
| ID | Term |
|---|---|
| D006601 | Hexoses |
| D009005 | Monosaccharides |
| D000073893 | Sugars |
| D002241 | Carbohydrates |
| D007661 |
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| OTHER |
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| Fructose and galactose | Dietary Supplement | 10g galactose (mostly from lactose) plus 40g of fructose (free and bound, max amount of fructose from added free sugar/saccharose 25g) |
|
| 4 Weeks |
| Plasma metabolite changes in targeted metabolomics during the dietary intervention compared to baseline measurements. | 4 Weeks |
| Plasma metabolite changes in targeted lipidomics during the dietary intervention compared to baseline measurements. | 4 Weeks |
| Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich | Not yet recruiting | Zurich | 8091 | Switzerland |
|
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |
| Ketoses |