Accuracy of Lactate Meter in GSDIa

Are Portable Lactate Monitoring Devices Accurate in Patients With Glycogen Storage Disease Ia When Compared to Blood Serum Lactates?

The goal of this observational study is to determine if home lactate meters (both capillary and serum sample) are accurate, compared to lab serum lactate in a population of patients with glycogen storage disease type 1a and to determine if the Accu Chek Guide glucometer (capillary sample) is accurate, in a population of patients with glycogen storage disease type 1a.

Study Overview

• Status: Recruiting
• Conditions: Glycogen Storage Disease Type Ia
• Intervention / Treatment: Device: blood glucose meter; blood lactate meter

Detailed Description

The goal of this observational study is to determine if home lactate meters (both capillary and serum sample) are accurate, within 20% of lab serum lactate reading 95% of the time, in a population of patients with glycogen storage disease type 1a and to determine if the Accu Chek Guide glucometer (capillary sample) is accurate, within 20% of lab serum glucose reading 95% of the time, in a population of patients with glycogen storage disease type 1a.

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

Contacts and Locations

• Study Contact: Name: Rebecca Riba-Wolman, MD; Phone Number: 860-837-6700; Email: rriba@connecticutchildrens.org
• Study Contact Backup: Name: Malaya Mount, MS, RD; Phone Number: 860-837-6700; Email: mmount@connecticutchildrens.org

Study Locations

• United States
  • Connecticut
    • Hartford, Connecticut, United States, 06107
      • Recruiting
      • Connecticut Children's Medical Center
      • Contact: Rebecca Riba-Wolman; Phone Number: 860-837-6700; Email: rriba@connecticutchildrens.org

Participation Criteria

Eligibility Criteria

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

• Sampling Method: Non-Probability Sample

Study Population

Patients of Connecticut Children's birth to age 60 years with diagnosed Glycogen Storage Disease Type Ia, that are seen in clinic between 7/2020 - 7/2024, or that present to emergency room or are admitted to Connecticut Children's for surgery, acute illness or dose titration admission.

Description

Inclusion Criteria:

• Patients with diagnosed/confirmed (by liver biopsy or genetic testing) Glycogen Storage Disease Type Ia (ICD 10 code: E74.01).
• Connecticut Children's Emergency Department visit and/or admission to Connecticut Children's during time study is active
• For pediatric participants: Ability of child's parent/legal guardian to understand and the willingness to sign a written informed consent document
• For adults: Ability to understand and the willingness to sign a written informed consent

Exclusion Criteria:

• Patients with Glycogen storage disease unspecified 74.00, or Ib
• Patients not meeting inclusion criteria

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Home lactate meter accuracy in GSDIa compared with lab lactate	Lactate level via point of care finger-stick and lab draw	Every 1-3 hours for a maximum 33 hours

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Glucometer accuracy in GSDIa compared with lab lactate	Glucose level via finger-stick and lab draw	Every 1-3 hours for a maximum 33 hours

Collaborators and Investigators

• Sponsor: Connecticut Children's Medical Center
• Collaborators: Global Center for Glycogen Storage Disease held at the Jewish Community Foundation

Study record dates

Study Major Dates

• Study Start (Actual): April 14, 2025
• Primary Completion (Estimated): March 10, 2027
• Study Completion (Estimated): December 10, 2027

Study Registration Dates

• First Submitted: February 20, 2025
• First Submitted That Met QC Criteria: February 20, 2025
• First Posted (Actual): February 24, 2025

Study Record Updates

• Last Update Posted (Actual): March 6, 2026
• Last Update Submitted That Met QC Criteria: March 5, 2026
• Last Verified: March 1, 2026

More Information

Terms related to this study

• Keywords: Blood lactate; Blood glucose; GSDIa; GSD I; Glycogen Storage Disease type Ia; Glycogen Storage Disease Ia blood glucose; Accu chek glucometer; Lactate plus
• Additional Relevant MeSH Terms: Pathologic Processes; Metabolism, Inborn Errors; Genetic Diseases, Inborn; Carbohydrate Metabolism, Inborn Errors; Hepatorenal form of glycogen storage disease
• Other Study ID Numbers: 23-137

Plan for Individual participant data (IPD)

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

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