Excludes1 pair

E74- vs E76.3

E74 and E76.3 are not coded together. ICD-10-CM declares them mutually exclusive with an Excludes1 note — the two conditions are not expected to occur together, so exactly one of them is normally correct for a given encounter. The Official Guidelines carve out one exception: if the two conditions are clearly unrelated to each other, both may be reported.

The official note, declared on E74
mucopolysaccharidosis (E76.0-E76.3)
E74

Other disorders of carbohydrate metabolism

Not billable
Chapter 4
Endocrine, nutritional and metabolic diseases
Section
E70-E88
E76.3

Mucopolysaccharidosis, unspecified

BillableUnspecified
Chapter 4
Endocrine, nutritional and metabolic diseases
Section
E70-E88
Indexed under
  • Mucopolysaccharidosis
  • Cardiopathy › mucopolysaccharidosis
  • Mucopolysaccharidosis › cardiopathy

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