Overnight Feeding Study in Glycogen Storage Disease Type 1

Study Overview
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Brief Summary
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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.

Official Title
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New Approaches for Over-night Feeding in Glycogen Storage Disease Type 1 (GSD 1)

Conditions
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Glycogen Storage Disease Type 1 (GSD 1)

Intervention / Treatment
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* Other: overnight nutrition regime


Participation Criteria
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Eligibility Criteria
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Inclusion criteria: Glycogen storage disease type 1 (type 1a and 1b) Stable nighttime glucose control without frequent or severe hypoglycaemia under current dietary treatment, according to capillary glucose measurements. Exclusion criteria: Unstable night-time glucose control during the last 8 weeks with frequent (> 4x weekly) hypoglycaemia during night-time, or a hospitalisation due to unstable glucose control during the last 4 weeks, or any episode of severe hypoglycaemia (requiring assistance or loss of consciousness) during the last 4 weeks. Pregnancy or breast feeding Drug or alcohol abuse Acute gastrointestinal problems (e.g. acute gastroenteritis) Known malignancy (e.g. hepatocellular carcinoma)

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Non-Randomized
Intervention Model: Crossover Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: uncooked corn starch<br>Patients receive uncooked corn starch before bed-time | Other: overnight nutrition regime<br>* Patients receive the specified overnight nutrition regimen<br>|
| Experimental: modified corn starch<br>Patients receive modified corn starch before bed-time | Other: overnight nutrition regime<br>* Patients receive the specified overnight nutrition regimen<br>|
| Experimental: other carbohydrate (starch) containing meal<br>Patients receive a carbohydrate (starch) containing meal before bed-time | Other: overnight nutrition regime<br>* Patients receive the specified overnight nutrition regimen<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | 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) |