Accuracy of Home Lactate Meter and Accu-chek Glucometer in Patients With Glycogen Storage Disease

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.

Study Overview

• Status: Recruiting
• Conditions: Glycogen Storage Disease Type I; Glycogen Storage Disease Type IA; Glycogen Storage Disease Type IB; Glycogen Storage Disease Xi
• Intervention / Treatment: Other: Lactate Meter; Device: Accu Chek

• Study Type: Observational
• Enrollment (Estimated): 10

Contacts and Locations

• Study Contact: Name: Malaya Mount, MS, RD, CDN; Phone Number: 860-837-6700; Email: mmount@connecticutchildrens.org
• Study Contact Backup: Name: Malaya Mount; Phone Number: 3608889293

Study Locations

• United States
  • Connecticut
    • Hartford, Connecticut, United States, 06106
      • Recruiting
      • Connecticut Children's
      • Contact: Malaya Mount, MS, RD, CDN; Phone Number: 860-837-6700; Email: mmount@connecticutchildrens.org

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Patients birth to age 60 years with diagnosed Glycogen Storage Disease Type Ia/Ib or XI.

Description

Inclusion Criteria:

1. 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).
2. For pediatric participants: Ability of child's parent/legal guardian to understand and the willingness to sign a written informed consent document
3. For adolescents: assent to participate
4. For adults: Ability to understand and the willingness to sign a written informed consent

Exclusion Criteria:

1. Patients with Glycogen storage disease unspecified
2. Patients not meeting inclusion criteria
3. Patients unable to provide consent
4. Patients who decline to be in the study

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
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	Hourly x 8 hours

Secondary Outcome Measures

Outcome Measure	Time Frame
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	Hourly x 8 hours

Collaborators and Investigators

• Sponsor: Connecticut Children's Medical Center
• Collaborators: Global Center for Glycogen Storage Disease held at the Jewish Community Foundation
• Investigators: Principal Investigator: Malaya Mount, MS, RD, CDN, Connecticut Children's; Principal Investigator: Rebecca Riba-Wolman, MD, Connecticut Children's

Study record dates

Study Major Dates

• Study Start (Estimated): August 1, 2026
• Primary Completion (Estimated): March 1, 2027
• Study Completion (Estimated): December 1, 2027

Study Registration Dates

• First Submitted: March 5, 2026
• First Submitted That Met QC Criteria: March 5, 2026
• First Posted (Actual): March 10, 2026

Study Record Updates

• Last Update Posted (Actual): June 1, 2026
• Last Update Submitted That Met QC Criteria: May 29, 2026
• Last Verified: May 1, 2026

More Information

Terms related to this study

• Keywords: blood glucose; lactate
• Additional Relevant MeSH Terms: Urogenital Diseases; Male Urogenital Diseases; Kidney Diseases; Urologic Diseases; Female Urogenital Diseases; Female Urogenital Diseases and Pregnancy Complications; Metabolism, Inborn Errors; Genetic Diseases, Inborn; Metabolic Diseases; Carbohydrate Metabolism, Inborn Errors; Renal Tubular Transport, Inborn Errors; Glycogen Storage Disease; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Nutritional and Metabolic Diseases; Fanconi Syndrome; Glycogen Storage Disease Type I; Hepatorenal form of glycogen storage disease; Glycogen Storage Disease IB
• Other Study ID Numbers: 25-061

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: Yes
• product manufactured in and exported from the U.S.: No