About this trial
The goal of this study is to determine if the Lactate Plus meter is accurate compared to lab lactate levels, and to determine if the Accu chek guide glucometer is accurate compared to lab serum glucose levels in patients with Glycogen Storage Disease Types Ia, Ib and XI.
To determine this, patient's will have a one-time planned admission to Connecticut Children's for approximately 8 hours and receive hourly blood draws as well as finger-sticks.
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
Patients with diagnosed/confirmed (by liver biopsy or genetic testing) Glycogen Storage Disease Type Ia/Ib (ICD 10 code: E74.01) or XI (74.09).
For pediatric participants: Ability of child's parent/legal guardian to understand and the willingness to sign a written informed consent document
For adolescents: assent to participate
For adults: Ability to understand and the willingness to sign a written informed consent
Disqualifiers
Patients with Glycogen storage disease unspecified
Patients not meeting inclusion criteria
Patients unable to provide consent
Patients who decline to be in the study
Trial population
Patients birth to age 60 years with diagnosed Glycogen Storage Disease Type Ia/Ib or XI.
Trial design
Case-only
Prospective
Treatments tested in this trial
Lactate Meter
Other interventionHourly blood lactate levels
Accu Chek
DeviceHourly blood glucose levels
Treatment groups
Trial outcomes
Primary outcomes
To determine if the lactate Plus meter (both capillary and serum sample) readings are within 20% of lab serum lactate reading 95% of the time, in a population of patients with glycogen storage disease type Ia/Ib/XI
Secondary outcomes
To determine if the Accu-Chek Guide glucometer (capillary sample) readings are within 20% of lab serum glucose reading 95% of the time, in a population of patients with glycogen storage disease type Ia/Ib/XI
Sponsors and contacts
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Connecticut Children's Medical Center
Lead sponsor
Global Center for Glycogen Storage Disease held at the Jewish Community Foundation
Collaborator