D53.0 vs E79.1
D53.0 and E79.1 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 D53.0
Lesch-Nyhan syndrome (E79.1)
D53.0
Protein deficiency anemia
Billable
- Chapter 3
- Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
- Section
- D50-D53
Use when the record says
- Amino-acid deficiency anemia
- Orotaciduric anemia
Indexed under
- Amino-acid disorder › anemia
- Anemia › amino-acid-deficiency (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound)
- Anemia › orotaciduric (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound)
- Anemia › protein-deficiency (essential) (general) (hemoglobin deficiency) (infantile) (primary) (profound)
- Orotaciduria, oroticaciduria › anemia (congenital) (hereditary) (pyrimidine deficiency)
E79.1
Lesch-Nyhan syndrome
Billable
- Chapter 4
- Endocrine, nutritional and metabolic diseases
- Section
- E70-E88
Use when the record says
- HGPRT deficiency
Indexed under
- Lesch-Nyhan syndrome
- Deficiency, deficient › hypoxanthine-
- Arthropathy › gouty › in › Lesch-Nyhan syndrome
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