E71.0
Billable codeMaple-syrup-urine disease
The ICD-10 code for maple-syrup-urine disease is E71.0.
Documentation support
General coding-documentation guidance — not a substitute for payer-specific requirements or professional coding judgment.
- •As a general rule, code to the highest level of specificity supported by the documentation in the medical record.
Related codes in this category
- E71.1Other disorders of branched-chain amino-acid metabolismnon-billable header
- E71.2Disorder of branched-chain amino-acid metabolism, unspecified
- E71.3Disorders of fatty-acid metabolismnon-billable header
- E71.4Disorders of carnitine metabolismnon-billable header
- E71.5Peroxisomal disordersnon-billable header
- E71.0Maple-syrup-urine disease
ICD-9-CM equivalent
Informational only — GEM mappings are approximate, not guaranteed one-to-one equivalents. Verify before use in billing.
- 2703Approximate match
Associated MS-DRGs
E71.0 can serve as the principal diagnosis for these Medicare Severity Diagnosis-Related Groups.
Informational only — actual DRG assignment also depends on procedures, complications/comorbidities (CC/MCC), discharge status, and payer-specific rules not reflected here.