E71.0 — Maple-syrup-urine disease
E71.0 is a billable, specific ICD-10-CM code for maple-syrup-urine disease. It is valid for submission on a claim.
Other codes that can never be billed with E71.0
These codes carry an Excludes1 note pointing at E71.0. The conflict binds both ways, but ICD-10-CM only writes it down on one side — so you would not find this by reading E71.0 alone.
- E72Other disorders of amino-acid metabolism
Risk adjustment
E71.0 does not map to an HCC and does not risk-adjust under CMS-HCC V28. That is normal — most ICD-10 codes do not. It still needs to be coded correctly; it just will not move a RAF score.
How E71.0 is indexed
Coders do not find codes by browsing the tabular list — they look them up in the alphabetic index, under the word the physician wrote. These are the index entries that lead here, so you can see which chart wordings map to E71.0.
- Maple-syrup-urine disease
- Disease, diseased›maple-syrup-urine
- Menkes' disease or syndrome›meaning maple-syrup-urine disease
- Syndrome›maple-syrup-urine
- Disorder›metabolism NOS›amino-acid›branched chain›maple syrup urine disease
Related codes at this level
Codes that share E71. If E71.0 is not quite right, the correct code is usually one of these.
Questions about E71.0
- Is E71.0 a billable ICD-10-CM code?
- Yes. E71.0 is a billable, specific ICD-10-CM code and is valid for submission on a claim.
- Where does E71.0 sit in the tabular list?
- Endocrine, nutritional and metabolic diseases (E00-E89), in the block Metabolic disorders (E70-E88).