E74.09

Billable

Other glycogen storage disease

HCC Category Mapping

V28HCC 50 — Glycogen/Amino-Acid/Other Metabolic Disorders

0.289

V24HCC 23 — Other Significant Endocrine and Metabolic Disorders

0.230

ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders

0.000

RxHCCHCC 43 — Other Significant Endocrine and Metabolic Disorders

0.000

What This Code Means

A rare inherited disorder where the body cannot properly break down and store glycogen (a form of sugar), leading to abnormal accumulation in muscles and organs.

Coding Tips

•Specify the type of glycogen storage disease if documented (e.g., Pompe disease, Cori disease) as these have more specific codes
•Review documentation for organ involvement (liver, muscle, heart) as this may affect severity coding

Clinical Significance

Other glycogen storage disease captures GSD types that do not have individual ICD-10 codes, such as Type IV (Andersen disease/branching enzyme deficiency), Type VI (Hers disease), Type VII (Tarui disease), and Type IX (phosphorylase kinase deficiency). These conditions vary in severity from mild hepatomegaly to progressive liver failure. Accurate coding ensures risk adjustment captures the complexity of managing these rare metabolic diseases.

Documentation Requirements

✓Specific GSD type identified by the provider (e.g., Type IV, VI, VII, IX)
✓Enzyme assay or genetic testing results confirming the specific enzyme deficiency
✓Organ involvement documented (liver, muscle, or both)
✓Physical exam findings relevant to the specific GSD type
✓Current management plan including dietary interventions and monitoring schedule

Commonly Confused Codes

E74.00 — Glycogen storage disease, unspecified: use only when no specific type is identified E74.01 — von Gierke disease: Type I has its own code E74.02 — Pompe disease: Type II has its own code E74.03 — Cori disease: Type III has its own code E74.04 — McArdle disease: Type V has its own code E74.05 — LAMP2 deficiency: Danon disease has its own code

Code Hierarchy

└E74Other disorders of carbohydrate metabolism └E74.0Glycogen storage disease └E74.09Other glycogen storage disease

└E74.09Other glycogen storage disease