Glucose 6 Phosphate Transport Defect C0268146
A metabolic disorder affecting how glucose is transported and processed in the body. It can cause low blood sugar, liver enlargement, and growth delays. Management focuses on frequent feeding and special diets.
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
Fasting hypoglycemia
Neutropenia
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hepatomegaly
(metabolic)Inflammatory bowel disease
(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 Feeding and Complex Carbohydrate Diet
Continuous glucose availability through frequent meals rich in complex carbohydrates and use of uncooked cornstarch to provide sustained glucose release. Nasogastric or gastrostomy feeds may be required in infancy.
Uric Acid Management
Allopurinol therapy to prevent hyperuricemia and reduce risk of uric acid nephropathy and gout, common complications due to impaired purine metabolism in G6PT deficiency.
Additional treatment options
Metabolic and Biochemical Monitoring
Regular monitoring of blood glucose, lactate, triglycerides, uric acid, and liver function tests to assess disease control and early detection of complications including hepatic adenomas.
monitoringAvoidance of Fasting Protocols
Strict avoidance of fasting periods exceeding recommended intervals, with night-time feeding strategies or continuous glucose monitoring to prevent life-threatening hypoglycemia.
lifestyleHepatic Imaging Surveillance
Periodic liver ultrasound or MRI imaging to monitor for hepatic adenomas, hepatocellular carcinoma risk, and progression of hepatomegaly commonly associated with G6PT deficiency.
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 Glucose 6 Phosphate Transport Defect C0268146?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Glucose 6 Phosphate Transport Defect C0268146. 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