A comparison of Glycosade® and Uncooked Cornstarch (UCCS) for the dietary management of hepatic glycogen storage diseases (GSD)
- Conditions
- glycogenosismetabolic disease1002742410021605
- Registration Number
- NL-OMON45944
- Lead Sponsor
- Vitaflo International Ltd
- Brief Summary
Trial is onging in other countries
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Completed
- Sex
- Not specified
- Target Recruitment
- 12
See the study protocol, §6.1:;Patients with GSD I, III, VI or IX under the care of a metabolic centre meeting the following inclusion criteria:
* Diagnosed by either genetic mutation analysis or enzymology study (this includes patients with only a single mutation but who also have supportive enzymology consistent with the condition). For those whom mutation analysis is not completed this will be assessed during the study.
* Adults and children aged 2 years or older.
* Established on UCCS for at least 6 months to full dietary requirements as defined by local clinical practice.
* Freely given informed written consent from participant or parent/caregiver.
See the study protocol, §6.2;* Evidence of any other medical condition which in the opinion of the Investigator makes it undesirable for the subject to participate in the trial or which would jeopardise compliance with the protocol.;* Women pregnant / breastfeeding at the start of the study or planning to become pregnant during its duration. ;Note: Women who become pregnant unexpectedly during this study can continue on the study product if they wish.
Study & Design
- Study Type
- Interventional
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method <p>The primary outcome for this study will be an increase in the duration of<br /><br>normal blood sugar levels and / or delaying the development of significant<br /><br>ketosis during dietary management with Glycosade® compared to dietary<br /><br>management with UCCS (Part 1) as defined by:<br /><br><br /><br>a. Period of normal blood glucose levels (plasma glucose * 3.6 mmol/l)<br /><br>increased by 60 minutes,<br /><br><br /><br>OR<br /><br><br /><br>b. A delay of 30 minutes in the development of significant ketosis in patients<br /><br>with GSD III, VI, IX as defined by:<br /><br>* BOHB * 1.0 mmol/L (participants < 14 years)<br /><br>* BOHB * 0.4 mmol/L (participants * 14 years)</p><br>
- Secondary Outcome Measures
Name Time Method <p>The secondary outcomes for this study will be:<br /><br><br /><br>1. The insulin area under the curve during dietary management with UCCS<br /><br>compared to dietary management with Glycosade®.<br /><br>2. The area under the curve in lactate, BOHB, during dietary management with<br /><br>UCCS compared to dietary management with Glycosade®.<br /><br>3. Tolerability & palatability of both Glycosade® and UCCS. </p><br>