E71.0Billable
Maple-syrup-urine disease
- Chapter
- 4. Endocrine, nutritional and metabolic diseases
- Category
- E71
- Effective date
- October 1, 2026
Related codes in category E71
E71Disord of branched-chain amino-acid metab & fatty-acid metabE71.1Other disorders of branched-chain amino-acid metabolismE71.11Branched-chain organic aciduriasE71.110Isovaleric acidemiaYesE71.1113-methylglutaconic aciduriaYesE71.118Other branched-chain organic aciduriasYesE71.12Disorders of propionate metabolismE71.120Methylmalonic acidemiaYesE71.121Propionic acidemiaYesE71.128Other disorders of propionate metabolismYesE71.19Other disorders of branched-chain amino-acid metabolismYesE71.2Disorder of branched-chain amino-acid metabolism, unspYes
Frequently asked questions
What is ICD-10 code E71.0?
E71.0 is an ICD-10-CM diagnosis code for Maple-syrup-urine disease.
Is E71.0 a billable code?
Yes. E71.0 is billable and specific enough to be used on a claim.
What ICD-10-CM chapter is E71.0 in?
E71.0 falls under Chapter 4: Endocrine, nutritional and metabolic diseases.
When did E71.0 become effective?
E71.0 has been effective since October 1, 2026, per the CMS/CDC ICD-10-CM annual release.
What other codes are related to E71.0?
E71.0 belongs to category E71, which includes 12 other codes — see the related codes list on this page.
ICD-10-CM data maintained by CDC/CMS (public domain). Always verify codes with authoritative sources before making coding or billing decisions.