E75 vs E77.1
E75 and E77.1 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 E75
mucolipidosis, types I-III (E77.0-E77.1)
E75
Disorders of sphingolipid metabolism and other lipid storage disorders
Not billable
- Chapter 4
- Endocrine, nutritional and metabolic diseases
- Section
- E70-E88
E77.1
Defects in glycoprotein degradation
Billable
- Chapter 4
- Endocrine, nutritional and metabolic diseases
- Section
- E70-E88
Use when the record says
- Aspartylglucosaminuria
- Fucosidosis
- Mannosidosis
- Sialidosis [mucolipidosis I]
Indexed under
- Aspartylglucosaminuria
- Fucosidosis
- Mannosidosis
- Sialidosis
- Defect, defective › degradation, glycoprotein
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