Maple-syrup-urine disease
Billable / specific
E71.0 is a billable, specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes in FY2027.
Inclusion terms
Includes (from E71)
- Note: All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Excludes1
Not coded here — never report together with this code.
- transitory endocrine and metabolic disorders specific to newborn (P70-P74) (from E71)
- androgen insensitivity syndrome (E34.5-) (from E71)
- congenital adrenal hyperplasia (E25.0) (from E71)
- hemolytic anemias attributable to enzyme disorders (D55.-) (from E71)
- Marfan syndrome (Q87.4-) (from E71)
- 5-alpha-reductase deficiency (E29.1) (from E71)
Excludes2
Not included here — may be reported together when both conditions exist.
Also known as
- Disease, diseased, maple-syrup-urine
- Menkes' disease or syndrome, meaning maple-syrup-urine disease
- Syndrome, maple-syrup-urine
Parent code
- E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolismHeader