E74.01 von Gierke disease

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

Applicable to

• Type I glycogen storage disease

Applies from E74 Other disorders of carbohydrate metabolism

Excludes1 (never code together)

• diabetes mellitus (E08-E13)
• hypoglycemia NOS (E16.2)
• increased secretion of glucagon (E16.3)
• mucopolysaccharidosis (E76.0-E76.3)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 50 — Amyloidosis, Porphyria, and Other Specified Metabolic Disorders
• CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
• RxHCC V08: HCC 43 — Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders
• ESRD V24: HCC 23

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 271.0 Glycogenosis (approximate)

Code annotations containing back-references to E74.01

Back-references to E74.0

• Code First: G73.7 Myopathy in diseases classified elsewhere ("glycogen storage disease (E74.0-)")
• Code First: I43 Cardiomyopathy in diseases classified elsewhere ("glycogen storage disease (E74.0-)")
• Code First: N16 Renal tubulo-interstitial disorders in diseases classified elsewhere ("glycogen storage disease (E74.0-)")

Back-references to E74

• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")
• Type 1 Excludes: R79 Other abnormal findings of blood chemistry ("specific findings indicating disorder of carbohydrate metabolism (E73-E74)")

Diagnosis Index entries for E74.01

• Deficiency, deficient › glucose-6-phosphatase
• Disease, diseased › Gierke's (glycogenosis I)
• Disease, diseased › glycogen storage › glucose-6-phosphatase deficiency
• Disease, diseased › glycogen storage › type I
• Disease, diseased › glycogen storage › Von Gierke's
• Disease, diseased › van Creveld-von Gierke (glycogenosis I)
• Disease, diseased › von Gierke's (glycogenosis I)
• Gierke's disease (glycogenosis I)
• Van Creveld-von Gierke disease
• Von Gierke's disease

Nearby codes

• E74 Other disorders of carbohydrate metabolism
• E74.0 Glycogen storage disease
• E74.00 Glycogen storage disease, unspecified
• E74.01 von Gierke disease (this code)
• 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