E74.01 von Gierke disease

The ICD-10-CM code for von Gierke disease is E74.01 (FY2026). It is a billable, claim-ready diagnosis code.

Classification

Chapter

4: Endocrine, nutritional and metabolic diseases (E00-E89)

Section

E70-E88: Metabolic disorders (E70-E88)

Category E74

32 codes (24 billable)

FY2026 Status

Stable since FY2024

Also Known As

ICD-10-CM Alphabetic Index entries that lead to E74.01:

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

Inclusion Terms

• Type I glycogen storage disease

U.S. Hospital Utilization

• An estimated 450 U.S. inpatient stays in 2023 included E74.01 among the documented diagnoses.
• 110 stays listed it as the principal diagnosis.

Source: National Inpatient Sample (NIS), Healthcare Cost and Utilization Project (HCUP), Agency for Healthcare Research and Quality, 2016–2023. National survey-weighted estimates.

Official Coding Guidelines

Diabetes mellitus (TOC entry)

a. Diabetes mellitus ................................................................................................................... 39

Obesity (TOC entry)

b. Obesity .................................................................................................................................. 42

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

• CMS — ICD-10-CM Official Code Set and Guidelines, FY2026
• CDC/NCHS — ICD-10-CM Files and Browser Tool
• AHRQ — HCUP National Inpatient Sample, Frequencies by Diagnosis Code (2016–2023)