E71.0 Maple-syrup-urine disease

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

Risk adjustment (HCC)

• CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
• RxHCC V08: HCC 43 — Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders
• ESRD V24: HCC 23

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 270.3 Disturbances of branched-chain amino-acid metabolism (approximate)

Code annotations containing back-references to E71.0

Back-references to E71.0

• Type 1 Excludes: E72 Other disorders of amino-acid metabolism ("branched-chain amino-acid metabolism (E71.0-E71.2)")

Back-references to E71

• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")
• Type 1 Excludes: R79 Other abnormal findings of blood chemistry ("specific findings indicating disorder of amino-acid metabolism (E70-E72)")
• Type 1 Excludes: R79.83 Abnormal findings of blood amino-acid level ("disorders of amino-acid metabolism (E70-E72)")

Diagnosis Index entries for E71.0

• Disease, diseased › maple-syrup-urine
• Disorder (of) › metabolism NOS › amino-acid › branched chain › maple syrup urine disease
• Maple-syrup-urine disease
• Menkes' disease or syndrome › meaning maple-syrup-urine disease
• Syndrome › maple-syrup-urine

Nearby codes

• E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
• E71.0 Maple-syrup-urine disease (this code)
• E71.1 Other disorders of branched-chain amino-acid metabolism
• E71.11 Branched-chain organic acidurias
• E71.110 Isovaleric acidemia
• E71.111 3-methylglutaconic aciduria
• E71.118 Other branched-chain organic acidurias