ICD-10-CM 2027
ICD-10 code for Phosphoenolpyruvate carboxykinase deficiency
Disorders of pyruvate metabolism and gluconeogenesis
From the official ICD-10-CM alphabetic index entry “Deficiency, deficient › phosphoenolpyruvate carboxykinase”. Page updated September 29, 2026.
About coding Phosphoenolpyruvate carboxykinase deficiency
The ICD-10-CM code for Phosphoenolpyruvate carboxykinase deficiency is E74.4 (Disorders of pyruvate metabolism and gluconeogenesis).
Within E74 (other disorders of carbohydrate metabolism), E74.4 is specifically for disorders of pyruvate metabolism and gluconeogenesis. Related codes cover glycogen storage disease (E74.0), disorders of fructose metabolism (E74.1), disorders of galactose metabolism (E74.2), other disorders of intestinal carbohydrate absorption (E74.3), other specified disorders of carbohydrate metabolism (E74.8) and disorder of carbohydrate metabolism, unspecified (E74.9).
Don't report E74.4 together with the conditions in its Excludes1 note: disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-) and Leigh's syndrome (G31.82).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
Don't confuse with (Excludes1 for E74.4)
Common questions about Phosphoenolpyruvate carboxykinase deficiency ICD-10 codes
What is the ICD-10 code for Phosphoenolpyruvate carboxykinase deficiency?
- E74.4 — Disorders of pyruvate metabolism and gluconeogenesis.
Is E74.4 billable?
- Yes. E74.4 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E74.4 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E74.4 as a principal diagnosis.
Is E74.4 a CC or MCC?
- E74.4 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.