Glycogen Storage Disease Type I C0017920
A group of inherited metabolic disorders where the body cannot properly process stored sugar. This causes growth problems, organ enlargement, and metabolic crises. Management focuses on diet and symptom control.
Key facts at a glance
- ICD-10
- E74.00
- 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
(gastrointestinal)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
Continuous Glucose Monitoring and Frequent Feeding
Glycogen storage disease type I requires strict dietary management to prevent life-threatening hypoglycemia. Patients typically need overnight nasogastric feeds or uncooked cornstarch every 3-4 hours to maintain blood glucose levels above 70 mg/dL.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Metabolic Laboratory Monitoring
Regular monitoring of blood glucose, lactate, uric acid, and lipid levels is essential to prevent complications. Patients typically require blood tests every 1-3 months to assess metabolic control and organ function.
Additional treatment options
Uric Acid Management
Allopurinol may be prescribed to manage hyperuricemia and prevent gout and kidney stone formation. This addresses secondary metabolic complications of the disorder.
medicationEmergency Management Plan
Families and patients receive education on recognizing and treating hypoglycemia emergencies. This includes glucagon administration training and always carrying fast-acting glucose sources.
lifestyleLiver Transplantation
In severe cases with poor metabolic control or development of hepatic adenomas, liver transplantation may be considered. This procedure restores glucose-6-phosphatase activity and improves metabolic stability.
surgicalWhen 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 Glycogen Storage Disease Type I C0017920?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalMedication Comparisons
Compare medications used to treat Glycogen Storage Disease Type I C0017920. 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