AutoICD API

E71.0

Billable

Maple-syrup-urine disease

Maple-syrup-urine disease

Status

Billable / Specific

Block

E70-E88

Parent Code

E71

ICD-11 Mapping

1 equivalent

Coding Notes

Excludes 1

Codes that cannot be used together with this code (mutual exclusion)

Excludes 2

Conditions not included here, but the patient may have both

Related Codes(5)
E71.1

Other disorders of branched-chain amino-acid metabolism

E71.2

Disorder of branched-chain amino-acid metabolism, unsp

BillableE71.3

Disorders of fatty-acid metabolism

E71.4

Disorders of carnitine metabolism

E71.5

Peroxisomal disorders

ICD-11 Equivalents(1)

ICD-11 Equivalents

View full mapping

Corresponding ICD-11 codes from the WHO crosswalk mapping

5C50.D0Maple-syrup-urine diseaseequivalentPrimary
Also Known As / Clinical Terms(122)

SNOMED CT

UMLS

Clinical Terms

  • Maple syrup urine disease, type III
  • Maple Syrup Urine Disease
  • Maple syrup urine disease (disorder)
  • Partial branched chain ketoacid dehydrogenase deficiency
  • BCKD deficiency
  • MAPLE SYRUP URINE DIS
  • Intermittent branched-chain ketoaciduria
  • Branched Chain Ketoaciduria
  • Deficiency of dihydrolipoamide dehydrogenase
  • Keto Acid Decarboxylase Deficiency
  • Ketoacidurias, Branched-Chain
  • Dihydrolipoamide dehydrogenase deficiency
  • branched-chain ketoaciduria
  • Branched-chain alpha-keto acid dehydrogenase deficiency
  • Ketoaciduria, Branched-Chain
  • Ketoacid decarboxylase deficiency
  • ketoacidemia
  • MSUD MAPLE SYRUP URINE DIS
  • Branched chain ketoacid dehydrogenase deficiency
  • Congenital infantile lactic acidosis due to LAD deficiency
  • maple syrup disease
  • msu
  • Branched Chain alpha Keto Acid Dehydrogenase Deficiency
  • Dihydrolipoyl dehydrogenase deficiency
  • Branched-Chain Ketoacidurias
  • Maple syrup urine disease, E3 deficiency
  • Intermittent branched chain ketoaciduria
  • MSUD - maple syrup urine disease
  • Oxo-acid decarboxylase deficiency
  • Cytochrome-b reductase deficiency
  • disease maple syrup urine
  • Classical maple syrup urine disease
  • Lipoamide dehydrogenase deficiency
  • Diaphorase deficiency
  • Mild maple syrup urine disease
  • DLD - Dihydrolipoamide dehydrogenase deficiency
  • Intermittent maple syrup urine disease
  • Lactic acidosis due to LAD deficiency
  • Intermediate branched chain ketoaciduria
  • Maple syrup urine disease with lactic acidosis
  • MSUD
  • Thiamin-responsive maple syrup urine disease
  • Maple syrup urine disease, multiple dehydrogenase form
  • MSUD (Maple Syrup Urine Disease)
  • Severe branched chain ketoacid dehydrogenase deficiency
  • Ketoacidaemia
  • BCKD - branched-chain 2-ketoacid dehydrogenase deficiency
  • Deficiency of diaphorase
  • Intermediate maple syrup urine disease
  • Deficiency of lipoamide reductase (NADH)
Frequently Asked Questions
What is the ICD-10 code for maple-syrup-urine disease?

The ICD-10-CM code for maple-syrup-urine disease is E71.0. The full clinical description is "Maple-syrup-urine disease". E71.0 is a billable/specific code that can be used on insurance claims and medical billing.

What does ICD-10 code E71.0 mean?

ICD-10-CM code E71.0 represents “Maple-syrup-urine disease”. It is classified under Chapter 4: Endocrine, Nutritional and Metabolic Diseases and is a billable/specific code that can be used on a claim.

Is E71.0 a billable code?

Yes, E71.0 is a billable/specific ICD-10-CM code and can be used to indicate a diagnosis on a medical claim.

What chapter is E71.0 in?

E71.0 is in Chapter 4: Endocrine, Nutritional and Metabolic Diseases (codes E00-E89).

What codes cannot be used with E71.0?

E71.0 has Excludes1 notes indicating codes that cannot be used together with it, including: transitory endocrine and metabolic disorders specific to newborn (P70-P74); androgen insensitivity syndrome (E34.5-); congenital adrenal hyperplasia (E25.0); and 3 more.

What SNOMED CT codes does E71.0 map to?

E71.0 maps to 8 SNOMED CT concepts: 27718001, 54064006, 29914000, 405287008, 405288003, and 3 more. SNOMED CT is a clinical terminology used in electronic health records.

What are the UMLS CUIs for E71.0?

E71.0 is linked to 1 UMLS Concept Unique Identifier: C0024776. The UMLS (Unified Medical Language System) integrates multiple biomedical vocabularies maintained by the U.S. National Library of Medicine.

How does E71.0 relate to ICF functioning codes?

ICF (International Classification of Functioning, Disability and Health) codes describe how conditions like maple-syrup-urine disease affect a person's functioning — body functions, activities, participation, and environmental factors. AutoICD provides ICF Core Sets for 12+ conditions and can map clinical text to ICF categories automatically. Browse the ICF directory to explore functioning codes.

What is the ICD-11 equivalent of E71.0?

E71.0 maps to the ICD-11 code: 5C50.D0 (Maple-syrup-urine disease).

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Includes SNOMED Clinical Terms® (SNOMED CT®) used by permission of SNOMED International. Includes content from the UMLS Metathesaurus, courtesy of the U.S. National Library of Medicine.