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Compare/E74.4 vs E88.4

E74.4 vs E88.4

E74.4 (Disorders of pyruvate metabolism and gluconeogenesis) compared with E88.4 (Mitochondrial metabolism disorders), from the official CMS tabular data. Do not report these codes together: the official tabular list marks them Excludes1.

Can these codes be reported together?

No — do not report together. E88.4 carries an Excludes1 note covering E74.4: “disorders of pyruvate metabolism (E74.4)”

Sole exception: when the two conditions are documented as unrelated to each other (ICD-10-CM Official Guidelines, Section I.A.12.a).

Side by side

E74.4Disorders of pyruvate metabolism and gluconeogenesisE88.4Mitochondrial metabolism disorders
Billing statusBillableNon-billable header

Report instead: E88.40, E88.41, E88.42, E88.43, E88.49

ClassificationE00-E89 — Endocrine, Nutritional and Metabolic Diseases (E00-E89)E00-E89 — Endocrine, Nutritional and Metabolic Diseases (E00-E89)
DefinitionDisorders of pyruvate metabolism and gluconeogenesis is a billable ICD-10-CM diagnosis code (E74.4).Mitochondrial metabolism disorders is a non-billable ICD-10-CM category code (E88.4).
Includes
Deficiency of phosphoenolpyruvate carboxykinase
Deficiency of pyruvate carboxylase
Deficiency of pyruvate dehydrogenase
Use additional code
codes for associated conditions

Derived from the official CMS ICD-10-CM tabular data (FY2026). Not billing advice; verify sequencing rules for the encounter. All data sources