Glycogen Storage Disease Type 1b 1c Cn380151
A genetic disorder affecting sugar metabolism, causing low blood sugar and increased infection risk due to immune system dysfunction. It requires lifelong nutritional support and careful monitoring.
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
Recurrent hypoglycemia
Fatigue or weight changes
(metabolic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Hepatomegaly
(gastrointestinal)Neutropenia
(hematologic)Inflammatory bowel disease-like symptoms
(gastrointestinal)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 supplementation regimen
Similar to GSD type 1a, frequent feeding and uncooked cornstarch maintain blood glucose levels. The glucose-6-phosphate transporter defect causes severe hypoglycemia requiring vigilant glucose monitoring and supplementation throughout day and night.
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Granulocyte colony-stimulating factor for neutropenia
GSD type 1b causes neutropenia and neutrophil dysfunction leading to recurrent infections. G-CSF stimulates neutrophil production and improves immune function, reducing infection frequency and severity.
Additional treatment options
Allopurinol for hyperuricemia
Uric acid elevation occurs similarly to type 1a due to lactic acidosis. Allopurinol prevents gout attacks and renal complications by reducing uric acid production.
medicationInfection surveillance and immune function monitoring
Due to neutrophil dysfunction in type 1b, regular monitoring of white blood cell counts, inflammatory markers, and prompt evaluation of fever is essential. Periodic assessment of immune function helps guide G-CSF dosing.
monitoringInflammatory bowel disease management
GSD type 1b can cause IBD-like intestinal inflammation requiring additional treatment. Aminosalicylates, corticosteroids, or immunomodulators may be needed for bowel symptom control.
medicationWhen 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 1b 1c Cn380151?
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 1b 1c Cn380151. 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