Glycogen Storage Disease Breath Test Study

NCT03218904 | Completed

• 1 other identifier: H16-03050
• Study Type: interventional
• Target: 14
• Locations: 1 country
• Sites: 1

Brief Summary

Glycogen storage disease type I (GSD I) caused by deficiency of glucose-6-phosphatase enzyme leading to build up of a complex sugar called glycogen in liver and low blood glucose level. Nutritional treatment involves supplying carbohydrates and uncooked cornstarch. Glycosade® (modified cornstarch) has shown promise in maintaining normal blood glucose level in GSD I. But the difficulty in nutritional treatment is determining the best type of carbohydrate to be given to avoid low blood glucose. Thus, there is a need to develop a simple test to examine glucose digestion and measure the utilization of different carbohydrates in GSD I and healthy controls.

Conditions

Patient Compliance; Healthy

Keywords

Glucose Breath Test

Outcome Measures

Primary Outcomes (1)

• Glucose oxidation

  Breath sample will be collected during the study to measure the rate of oxidation of 13C glucose

  4 hours

Study Arms (2)

Glucose intake

EXPERIMENTAL

Experiment 1: study day 1- single oral dose of glucose study day 2- single oral dose of glucose with U-13C-glucose

Dietary Supplement: Glucose intake

Carbohydrates intake

EXPERIMENTAL

Experiment 2: study day 1-single oral dose of uncooked cornstarch study day 2-single oral dose of Glycosade®

Dietary Supplement: Carbohydrates intake

Interventions

Glucose intake DIETARY_SUPPLEMENT

Experiment 1: study day1- single oral dose of glucose study day 2-single oral dose of glucose with U-13C-glucose

Also known as: D-glucose (Thermo Scientific™ NERL™ Trutol™)

Glucose intake

Carbohydrates intake DIETARY_SUPPLEMENT

Experiment 2:study day1- single oral dose of uncooked cornstarch study day 2- single oral dose of Glycosade®

Also known as: Uncooked cornstarch; Glycosade®

Carbohydrates intake

Eligibility Criteria

• Age: 5 Years - 35 Years
• Sex: all
• Healthy Volunteers: Yes
• Age Groups: Child (0-17), Adult (18-64)

You may qualify if:

• Experiment 1
• Healthy adults 19 - 35 years of age
• Adults who have no medical conditions
• Adults who currently free from any concurrent illness such as fever or cold
• Experiment 2
• Healthy Controls:
• Healthy children 5 - 18 years of age
• Healthy adults 19 - 35 years of age
• Children and adults who have no medical conditions
• Children and adults who currently free from any concurrent illness such as fever or cold
• Subjects with GSD I:
• Children 5 - 18 years of age who are diagnosed with GSD I
• Adults 19 - 35 years of age who are diagnosed with GSD I
• Clinically stable children and adults with GSD I with no concurrent illness such as fever, cold, vomiting or diarrhea
• Children and adults with GSD I who have had the clinical decision made to start the extended release waxy maize cornstarch Glycosade®

You may not qualify if:

• Experiment 1
• Healthy adults above age 35 years
• Adults who have a history of cardiovascular disease, liver or kidney disease, metabolic, pulmonary, gastrointestinal or endocrine disorder
• Healthy adults but are currently ill with a fever, cold, vomiting or diarrhea
• Healthy adults with claustrophobia
• Healthy adults currently smoking or consuming more than one drink containing alcohol each day
• Adults currently or recently taking medication or antibiotics
• Experiment 2
• Healthy Controls:
• Healthy children under age 5 years
• Healthy adults above age 35 years
• Children and adults who have a history of cardiovascular disease, liver or kidney disease, metabolic, pulmonary, gastrointestinal or endocrine disorder
• Healthy children and adults but are currently ill with a fever, cold, vomiting or diarrhea
• Healthy children and adults with claustrophobia
• Children and adults currently or recently taking medication or antibiotics

Sponsors & Collaborators

• University of British Columbia: lead
• Saudi Arabian Cultural Bureau, Ottawa: collaborator
• Canadian Institutes of Health Research (CIHR): collaborator

Study Sites (1)

BC Children's Hospital Research Institute, University of British Columbia

Vancouver, British Columbia, V5Z4H4, Canada

Location

MeSH Terms

Conditions

Patient Compliance

Interventions

Glucose

Condition Hierarchy (Ancestors)

Patient Acceptance of Health Care; Treatment Adherence and Compliance; Health Behavior; Behavior

Intervention Hierarchy (Ancestors)

Hexoses; Monosaccharides; Sugars; Carbohydrates

Study Officials

• Rajavel Elango, PhD

  University of British Columbia

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NON RANDOMIZED
• Masking: NONE
• Purpose: HEALTH SERVICES RESEARCH
• Intervention Model: SEQUENTIAL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Principal Investigator

Study Record Dates

• First Submitted: July 13, 2017
• First Posted: July 17, 2017
• Study Start: March 17, 2017
• Primary Completion: October 23, 2021
• Study Completion: January 24, 2025
• Last Updated: January 27, 2025

Record last verified: 2025-01

Data Sharing

• IPD Sharing: Will not share

Locations

CA (1)