ICD-10-CM › E00-E89 › E70-E88 › E71 Disorders of branched-chain... › E71.0
Maple-syrup-urine disease
ValidICD-10-CM diagnosis code.
Valid for claims with a date of service in the current code year.
What has happened to this code
Every change the publisher made, from 2007 to now. Entries marked draft are from the pre-implementation releases: ICD-10 took effect on 1 October 2015, so nothing before FY2016 was ever valid on a claim.
- FY2007 Added to the draft code set draft Maple-syrup-urine disease
Official coding rules
Published by CDC for FY2027. A rule attached to a category binds every code beneath it, so the rules inherited from this code's ancestors are shown with the code they come from.
Inherited from the E70-E88 section Metabolic disorders (E70-E88)
Excludes1 - never coded here
- androgen insensitivity syndrome (E34.5-)
- congenital adrenal hyperplasia (E25.0)
- hemolytic anemias attributable to enzyme disorders (D55.-)
- Marfan syndrome (Q87.4-)
- 5-alpha-reductase deficiency (E29.1)
Excludes2 - not included here
- Ehlers-Danlos syndromes (Q79.6-)
How CDC's index refers to this code
Wording from the official alphabetic index. Much of it appears nowhere in the code description, which is why a search for the everyday phrase so often finds nothing.
- Maple-syrup-urine disease
- Syndrome > maple-syrup-urine
- Disease, diseased > maple-syrup-urine
- Menkes' disease or syndrome > meaning maple-syrup-urine disease
- Disorder > metabolism NOS > amino-acid > branched chain > maple syrup urine disease
Codes in the same group
| Code | Description | Status |
|---|---|---|
| E71.1 | Other disorders of branched-chain amino-acid metabolism | Not billable |
| E71.2 | Disorder of branched-chain amino-acid metabolism, unspecified | Valid |
| E71.3 | Disorders of fatty-acid metabolism | Not billable |
| E71.4 | Disorders of carnitine metabolism | Not billable |
| E71.5 | Peroxisomal disorders | Not billable |