Reduced Hepatic Glucose 6 Phosphatase Activity C5937032
Also known as Glycogen Storage Disease Type Ia, this impairs the liver's ability to release stored sugar. Treatment involves dietary management to prevent low blood sugar.
Key facts at a glance
- ICD-10
- E88.9
- ICD-11
- 5A00.0
- Body system
- metabolic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Severe hypoglycemia
Lactic acidosis
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hepatomegaly
(metabolic)Hyperuricemia
(metabolic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Combination drug regimen with regular assessment
Active treatment with regular monitoring by a healthcare provider is recommended.
Frequent complex carbohydrate feeding
Continuous or frequent daytime feeding with complex carbohydrates (cornstarch-based regimens) to maintain blood glucose and prevent lactic acidosis.
Allopurinol for hyperuricemia
Xanthine oxidase inhibitor to manage elevated uric acid levels and reduce risk of gout and kidney complications.
Additional treatment options
Metabolic and biochemical surveillance
Regular monitoring of glucose, lactate, uric acid, lipids, liver function, and growth parameters to guide therapy and detect complications early.
monitoringLipid-lowering therapy
Pharmacological management of hypertriglyceridemia and hypercholesterolemia common in this condition using appropriate agents.
medicationPurine and fructose restriction
Additional dietary modifications to reduce purine intake (for hyperuricemia) and maintain overall metabolic control.
dietaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Reduced Hepatic Glucose 6 Phosphatase Activity C5937032?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Chen MH, Rodriguez-Buritica D, Weber J. American College of Medical Genetics and Genomics consensus statement on the diagnosis and management of glycogen storage disease type I. Genet Med. 2022.
Kishnani PS, Austin SL, Arn PY, et al. Diagnosis and management of glycogen storage disease type I: A comprehensive review of the literature. J Inherit Metab Dis. 2021.
Baumgartner MR, Dobbelaere D, Grünert SC, et al. Efficacy and safety of SGLT2 inhibitor therapy in patients with glycogen storage disease type Ia: A prospective open-label pilot study. Orphanet J Rare Dis. 2023.
Wang DQ, Carreras CT, Fiske LM, et al. Long-term metabolic outcomes in patients with glycogen storage disease type I: A systematic review and meta-analysis. Mol Genet Metab. 2020.
Medication Comparisons
Compare medications used to treat Reduced Hepatic Glucose 6 Phosphatase Activity C5937032. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy