D55 vs E74.4
D55 and E74.4 can never be reported together. ICD-10-CM declares them mutually exclusive with an Excludes1 note, meaning the two conditions cannot occur in the same patient — so exactly one of them is correct for any given encounter.
The official note, declared on E74.4
disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-)
D55
Anemia due to enzyme disorders
Not billable
- Chapter 3
- Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
- Section
- D55-D59
E74.4
Disorders of pyruvate metabolism and gluconeogenesis
Billable
- Chapter 4
- Endocrine, nutritional and metabolic diseases
- Section
- E70-E88
Use when the record says
- Deficiency of phosphoenolpyruvate carboxykinase
- Deficiency of pyruvate carboxylase
- Deficiency of pyruvate dehydrogenase
Indexed under
- Deficiency, deficient › phosphoenolpyruvate carboxykinase
- Disorder › gluconeogenesis (of)
- Disorder › pyruvate metabolism (of)
- Deficiency, deficient › pyruvate › carboxylase
- Deficiency, deficient › pyruvate › dehydrogenase
These two codes sit in different chapters of the Tabular List, which is usually the clearest signal that they describe different underlying processes rather than different degrees of the same one.
Excludes1 is not always absolute: where two conditions covered by the note are genuinely unrelated, CMS guidance permits reporting both. Document the reasoning rather than assuming it. More on Excludes1 vs Excludes2 · FY2027