E71.0

Maple-syrup-urine disease

Billable FY2026 2025-10-01 → 2026-09-30

Exclusions 7 never code together

Excludes1

androgen insensitivity syndrome (E34.5-)E34.5-
congenital adrenal hyperplasia (E25.0)E25.0
hemolytic anemias attributable to enzyme disorders (D55.-)D55.-
Marfan syndrome (Q87.4-)Q87.4-
5-alpha-reductase deficiency (E29.1)E29.1

Excludes2

Excludes1

transitory endocrine and metabolic disorders specific to newborn (P70-P74)P70-P74

Excludes1 — never code together. The excluded condition and this one are mutually exclusive.

Excludes2 — not included here, but the patient may have both. Code both when documented.

Instructional notes 1

Notes

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.E05.8 E07.0 E16-E31 E34.-

Index entries leading here 5

Disorder (of) — see also Disease
- metabolism NOS E88.9
  - amino-acid E72.9
    - branched chain E71.2
      - maple syrup urine disease E71.0
Disease, diseased — see also Syndrome
- maple-syrup-urine E71.0
Syndrome — see also Disease
- maple-syrup-urine E71.0
Maple-syrup-urine disease E71.0
Menkes' disease or syndrome E83.09
- meaning maple-syrup-urine disease E71.0

Alongside this code 5 same parent — E71

Additional references

AHA Coding Clinic® Licence required

Official coding advice for this code. Published by the American Hospital Association. Not included in this installation.

CMS22HCC23 ESRD21HCC23 ESRD24HCC23 RX08HCC43

Presence is a property of the code. Untick a model to hide it everywhere.

Present FY2024–FY2027.