Accuracy of Lactate Meter in GSDIa

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.

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