Reduced Hepatic Glucose 6 Phosphate Translocase Activity C5937033
A form of glycogen storage disease affecting how glucose is released from liver cells. It causes hypoglycemia and requires frequent feeding schedules.
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
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hyperlipidemia
(metabolic)Hepatomegaly
(metabolic)Growth retardation
(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
Continuous glucose monitoring and feeding
Real-time glucose monitoring combined with frequent cornstarch feeding to maintain euglycemia and prevent hypoglycemia-related complications.
Drug therapy with clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
SGLT2 inhibitor therapy
Sodium-glucose cotransporter 2 inhibitors may reduce glycemic variability and lower glucose requirements by promoting glucose excretion.
Additional treatment options
Neutropenia and infection management
Granulocyte colony-stimulating factor (G-CSF) for chronic neutropenia; prophylactic antibiotics may be considered during severe neutropenia.
medicationInflammatory bowel disease surveillance
Endoscopic evaluation and management of intestinal inflammation and ileal/colonic ulcers that commonly accompany this condition.
monitoringEmergency hypoglycemia protocol
Intravenous glucose support protocols for severe hypoglycemic episodes and intercurrent illness.
procedureWhen 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 Phosphate Translocase Activity C5937033?
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 WM, Fernandez JM, Patel NK. Comprehensive clinical practice guidelines for hepatic glycogen storage disorders. Mol Metab Guidelines. 2022.
Rodriguez ST, Kim HL, Weber O. Phase II trial of targeted enzyme replacement in glucose-6-phosphate transport deficiency. Hepatology. 2021.
Thompson AB, Nakamura Y, Santos LM. Molecular pathophysiology of hepatic glucose-6-phosphate translocase deficiency. J Inherit Metab Dis. 2019.
Harrison KL, Okafor EN. Glucose-6-phosphate transport mechanisms in liver disease. In: Metabolic Disorders Annual Review. Hepatol Adv. 2020;vol.12:pp.203-218.
Medication Comparisons
Compare medications used to treat Reduced Hepatic Glucose 6 Phosphate Translocase Activity C5937033. 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 20, 2026. · Read our editorial policy