ICD-10/E74.00

E74.00 ICD 2026 Code: Glycogen storage disease, unspecified

E74.00 is the authoritative medical code for Glycogen storage disease, unspecified. This classification is used in medical billing and clinical recording to specify the clinical criteria for glycogen storage disease, unspecified (ICD-10-CM E74.00), ensuring healthcare documentation aligns with 2026 federal coding standards.

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Official Registry Overview & Definition

Glycogen storage disease, unspecified is a billable ICD-10-CM diagnosis code E74.00. Excludes1 (not coded here): diabetes mellitus E08-E13; hypoglycemia NOS E16.2; increased secretion of glucagon E16.3; mucopolysaccharidosis E76.0-E76.3; androgen insensitivity syndrome E34.5-; congenital adrenal hyperplasia E25.0; hemolytic anemias attributable to enzyme disorders D55.-; Marfan syndrome Q87.4-; 5-alpha-reductase deficiency E29.1. Excludes2 (not included here): Ehlers-Danlos syndromes Q79.6-.

Official Tabular Instructional Notes

Sequencing, inclusion, and exclusion notes published for E74.00 in the official ICD-10-CM tabular list.

Excludes1 — Not Coded Here

Conditions that can never be reported with this code; the two are mutually exclusive.

  • diabetes mellitus E08-E13
  • hypoglycemia NOS E16.2
  • increased secretion of glucagon E16.3
  • mucopolysaccharidosis E76.0-E76.3
  • androgen insensitivity syndrome E34.5-
  • congenital adrenal hyperplasia E25.0
  • hemolytic anemias attributable to enzyme disorders D55.-
  • Marfan syndrome Q87.4-
  • 5-alpha-reductase deficiency E29.1

Excludes2 — Not Included Here

Conditions not covered by this code, but which may be reported alongside it when both are present.

  • Ehlers-Danlos syndromes Q79.6-

Frequently Asked Questions (FAQ) & Clinical Guidance

What can't be coded together with E74.00?

Per official ICD-10-CM Excludes1 instructions, E74.00 must not be reported together with: diabetes mellitus (E08-E13); hypoglycemia NOS (E16.2); increased secretion of glucagon (E16.3); mucopolysaccharidosis (E76.0-E76.3).

Can E74.00 be reported alongside related conditions?

Per official ICD-10-CM Excludes2 instructions, E74.00 and the following are not considered part of each other and may both be reported when both conditions are present: Ehlers-Danlos syndromes (Q79.6-).

Nearest Codes in This Family

Official ICD-10-CM classifications closest to E74.00 in its code family, with their registry titles.

  • E74 — Other disorders of carbohydrate metabolism
  • E74.0 — Glycogen storage disease
  • E74.01 — von Gierke disease
  • E74.02 — Pompe disease
  • E74.03 — Cori disease
  • E74.04 — McArdle disease
  • E74.05 — Lysosome-associated membrane protein 2 [LAMP2] deficiency
  • E74.09 — Other glycogen storage disease
  • E74.1 — Disorders of fructose metabolism
  • E74.10 — Disorder of fructose metabolism, unspecified

View all 31 codes in the E74 family

Indexed Clinical Terms (6)

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code.

  • Aglycogenosis
  • Disease, diseased, glycogen storage
  • Disease, diseased, glycogen storage, generalized
  • Disorder (of), tubulo-interstitial (in), glycogen storage disease
  • Myopathy, in (due to), glycogen storage disease
  • Nephritis, nephritic (albuminuric) (azotemic) (congenital) (disseminated) (epithelial) (familial) (focal) (granulomatous) (hemorrhagic) (infantile) (nonsuppurative, excretory) (uremic), tubulo-interstitial (in), due to, glycogen storage disease