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In this clinical cross-over study, we will compare the efficacy of different oral nutrition regimens for night-time glucose control in adult GSD 1 patients. Three different over-night nutrition regimens (=interventions) will be compared in each patient sequentially, (1) uncooked corn starch (UCSS, "Maizena"), (2) modified corn-starch, (3) other carbohydrate (starch) containing meal. During each intervention, glucose profiles will be continuously monitored by continuous glucose monitoring (CGMS). The duration of each intervention is 3d (mimimum) to 6d (maximum), depending on the quality of night-time glucose control and the technical quality of glucose sensor readings. Between the interventions, the patients follow their normal prescribed diet.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| uncooked corn starch | Experimental | Patients receive uncooked corn starch before bed-time |
|
| modified corn starch | Experimental | Patients receive modified corn starch before bed-time |
|
| other carbohydrate (starch) containing meal | Experimental | Patients receive a carbohydrate (starch) containing meal before bed-time |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| overnight nutrition regime | Other | Patients receive the specified overnight nutrition regimen |
|
| Measure | Description | Time Frame |
|---|---|---|
| Maximum fasting time over-night (h) | Maximum fasting time will be measured during each dietary intervention for an average period of 3d (minimum) to 6d (maximum) |
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Inclusion criteria:
Exclusion criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Giatgen Spinas, Prof MD | University Hospital Zurich, Division of Endocrinology, Diabetes and Clinical Nutrition | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University Hospital Zurich, Division of Endocrinology, Diabetes and Clinical Nutrition | Zurich | Canton of Zurich | 8091 | Switzerland |
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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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| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |