Glycosade v UCCS in the Dietary Management of Hepatic GSD (Glyde)

A Comparison of Glycosade® and Uncooked Cornstarch (UCCS) for the Dietary Management of Hepatic Glycogen Storage Diseases (GSD)

To compare efficacy of Glycosade® with uncooked corn starch (UCCS for the dietary management of hepatic glycogen storage diseases (GSD).

Study Overview

• Status: Completed
• Conditions: Glycogen Storage Disease
• Intervention / Treatment: Dietary supplement: Medical Food - Glycosade

Detailed Description

'Glyde' is a prospective, randomised, double-blind, crossover trial to compare the effects of two different starches used in the dietary management of GSD.

The study will recruit 64 patients from six centres in the UK, USA, France and The Netherlands. The planned duration per participant is 2 years.

Participants will be randomised into two equally sized groups:

Arm A- Glycosade intake given first, followed by UCCS 1-6 weeks later Arm B- UCCS intake given first, followed by Glycosade 1-6 weeks later.

The objective of this trial is to establish whether Glycosade® improves outcomes for patients with GSD compared to UCCS therapy by comparing the duration of normal blood sugars, lactate and ketone levels of patients with hepatic GSD (Types I, III, VI and IX) post equivalent intakes of UCCS and Glycosade®.

The first part consists of two (blinded) 12 hour starch load tests administered randomly within a maximum 6 week period. Participants will be randomised to receive either UCCS or Glycosade® for the initial intervention period crossing over to the other product for the second intervention period (part 1).

The second part (unblinded) is a 24 month open follow up whereby participant's will continue on the product considered most suitable by the clinician and patient.

• Study Type: Interventional
• Enrollment (Actual): 61
• Phase: Not Applicable

Contacts and Locations

Study Locations

• France
  • Clamart, France, 92140 Cedex
    • Hôpital Antoine Béclère
• Netherlands
  • Groningen, Netherlands, 9700RB
    • Universitair Medisch Centrum Groningen
• United Kingdom
  • London, United Kingdom
    • Evelina Children's Hospital
  • London, United Kingdom
    • Great Ormond Street Hospital for Children
  • Greater London
    • London, Greater London, United Kingdom, WC1N 3BG
      • National Hospital For Neurology and Neurosurgery
• United States
  • Connecticut
    • Hartford, Connecticut, United States, 06106
      • Connecticut Children's Medical Center

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 1 year to 71 years (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: No

Description

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.
• Aged 2 years or older (5 years or older in the USA)
• Established on full intake of uncooked corn starch therapy for at least 6 months

Exclusion Criteria:

• Women who are pregnant or breastfeeding at the start of the study or planning to become pregnant during the study will be excluded.
• children less than 2 years of age

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Treatment
• Allocation: Randomized
• Interventional Model: Crossover Assignment
• Masking: Triple

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Active Comparator: Glycosade; Participants will be randomised to receive the medical food Glycosade as a starch load with a maximum dose of 100g. Glycosade to be taken as one dose.	Dietary supplement: Medical Food - Glycosade; double blind randomised crossover of 2 starches - glycosade and UCCS
Placebo Comparator: Uncooked corn starch; Participants will be randomised to receive uncooked corn starch as a starch load with a maximum dose of 100g. Uncooked corn starch to be taken as one dose.	Dietary supplement: Medical Food - Glycosade; double blind randomised crossover of 2 starches - glycosade and UCCS

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
To assess if Glycosade® will result in improved blood glucose, lactate and ketone levels in a larger group of patients with GSD I, III, VI & IX as measured by duration of normal blood glucose levels or prevention of ketosis	24 hours

Secondary Outcome Measures

Outcome Measure	Time Frame
1. The insulin area under the curve during dietary management with UCCS compared to dietary management with Glycosade®.	24 hours
2. The area under the curve in lactate, BOHB, during dietary management with UCCS compared to dietary management with Glycosade®.	24 hours

Collaborators and Investigators

• Sponsor: Vitaflo International, Ltd
• Collaborators: University College London Hospitals; University Medical Center Groningen; University of Florida; Great Ormond Street Hospital for Children NHS Foundation Trust; Hôpital Necker-Enfants Malades
• Investigators: Principal Investigator: Helen Mundy, MRCP MRCPCH, Guys and St Thomas NHS Foundation Trust

Study record dates

Study Major Dates

• Study Start (Actual): February 22, 2016
• Primary Completion (Actual): August 14, 2020
• Study Completion (Actual): August 14, 2020

Study Registration Dates

• First Submitted: December 12, 2014
• First Submitted That Met QC Criteria: December 12, 2014
• First Posted (Estimated): December 17, 2014

Study Record Updates

• Last Update Posted (Actual): February 16, 2024
• Last Update Submitted That Met QC Criteria: February 15, 2024
• Last Verified: February 1, 2024

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Metabolic Diseases; Genetic Diseases, Inborn; Carbohydrate Metabolism, Inborn Errors; Metabolism, Inborn Errors; Glycogen Storage Disease
• Other Study ID Numbers: MCTW-GLY-03/14-18

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO