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

Connecticut Children's Medical Center10 enrolled

Overview

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 Type

OBSERVATIONAL

Enrollment

Conditions

Glycogen Storage Disease Type IA Glycogen Storage Disease Type I Glycogen Storage Disease Type IB Glycogen Storage Disease Xi

Interventions

Eligibility

Sex: ALLMax age: 60 Years

Medical Language ↔ Plain English

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

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

Time frame: Hourly x 8 hours

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

Time frame: Hourly x 8 hours

Central Contacts

Malaya Mount, MS, RD, CDN

CONTACT

860-837-6700 mmount@connecticutchildrens.org

Malaya Mount