E74.09
Other glycogen storage disease
Clinical Classification Guidelines
Inclusion Terms
- Andersen disease
- Glycogen storage disease, types 0, IV, VI-XI
- Hers disease
- Liver phosphorylase deficiency
- Muscle phosphofructokinase deficiency
- Tauri disease
Medical Intelligence & Overview
Other glycogen storage disease, classified under ICD-10 code E74.09, refers to a group of rare inherited disorders characterized by abnormal storage and processing of glycogen, a form of stored sugar that the body uses for energy. These conditions can affect various organs, particularly the liver and muscles, leading to a range of health issues. The classification includes several specific types and related disorders, such as Andersen disease, Hers disease, and liver phosphorylase deficiency. Recognizing the features of these diseases is important for diagnosis and management, though they remain relatively uncommon.
Causes & Symptoms
Clinical Causes: Genetic mutations affecting enzymes involved in glycogen synthesis or breakdown Inheritance patterns are typically autosomal recessive, meaning both copies of a gene must be affected Mutations leading to enzyme deficiencies such as branched-chain alpha-glucan storage disease (Andersen disease) or liver phosphorylase deficiency
Key Symptoms: Muscle weakness and fatigue during exertion Enlarged liver (hepatomegaly) Hypoglycemia (low blood sugar), especially between meals Growth retardation in some cases Delayed motor development in children Symptoms may vary depending on the specific type and organs involved
Diagnostic & Treatment
Diagnosis Path: Detailed medical history and physical examination Blood tests revealing abnormal levels of enzymes and metabolites related to glycogen metabolism Liver function tests Muscle biopsy to analyze glycogen storage and enzyme activity Genetic testing to identify specific mutations Imaging studies such as ultrasound for organ enlargement
Treatment Protocols: Dietary modifications, including frequent carbohydrate-rich meals to maintain blood sugar levels Avoiding prolonged fasting Supplementation with specific nutrients or enzyme replacement therapies in select cases Monitoring and managing liver and muscle health regularly Physical therapy to maintain muscle strength and function Supportive care tailored to individual needs
Clinical Advice & FAQs
Billing Guidance
Is E74.09 a billable ICD-10 code?
Yes, E74.09 is a specific, billable code that can be used to indicate a diagnosis for reimbursement purposes.
Documentation
How do I report E74.09?
Clinical documentation must specify the nature of Other glycogen storage disease and any associated comorbidities for accurate reporting.
Cite this Clinical Reference