E71.0
BillableMaple-syrup-urine disease
Maple-syrup-urine disease
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
- Ehlers-Danlos syndromes (Q79.6-)
Also Known As / Clinical Terms
SNOMED CT
- Mild maple syrup urine disease23156007
- BCKD - branched-chain 2-ketoacid dehydrogenase deficiency27718001
- Branched chain ketoacid dehydrogenase deficiency27718001
- Branched chain ketoaciduria27718001
- Branched-chain alpha-keto acid dehydrogenase deficiency27718001
- Ketoacid decarboxylase deficiency27718001
- Ketoacidaemia27718001
- Ketoacidemia27718001
- MSUD - maple syrup urine disease27718001
- Maple syrup urine disease27718001
- Oxo-acid decarboxylase deficiency27718001
- Congenital infantile lactic acidosis due to LAD deficiency29914000
- Cytochrome-b reductase deficiency29914000
- DLD - Dihydrolipoamide dehydrogenase deficiency29914000
- Deficiency of diaphorase29914000
- Deficiency of dihydrolipoamide dehydrogenase29914000
- Deficiency of lipoamide reductase (NADH)29914000
- Diaphorase deficiency29914000
- Dihydrolipoamide dehydrogenase deficiency29914000
- Dihydrolipoyl dehydrogenase deficiency29914000
- Lactic acidosis due to LAD deficiency29914000
- Lipoamide dehydrogenase deficiency29914000
- Maple syrup urine disease with lactic acidosis29914000
- Maple syrup urine disease, type III29914000
- Thiamin-responsive maple syrup urine disease31368008
- Classical maple syrup urine disease54064006
- Severe branched chain ketoacid dehydrogenase deficiency54064006
- Maple syrup urine disease, E3 deficiency71702000
- Maple syrup urine disease, multiple dehydrogenase form71702000
- Intermediate branched chain ketoaciduria405287008
- Intermediate maple syrup urine disease405287008
- Intermittent branched chain ketoaciduria405288003
- Intermittent branched-chain ketoaciduria405288003
- Intermittent maple syrup urine disease405288003
- Partial branched chain ketoacid dehydrogenase deficiency405288003
UMLS
- BCKD - branched-chain 2-ketoacid dehydrogenase deficiencyC0024776
- BCKD DeficiencyC0024776
- BCKD deficiencyC0024776
- Branched Chain KetoaciduriaC0024776
- Branched Chain alpha Keto Acid Dehydrogenase DeficiencyC0024776
- Branched chain ketoacid dehydrogenase deficiencyC0024776
- Branched chain ketoaciduriaC0024776
- Branched-Chain KetoaciduriaC0024776
- Branched-Chain KetoaciduriasC0024776
- Branched-Chain alpha-Keto Acid Dehydrogenase DeficiencyC0024776
- Branched-chain alpha-keto acid dehydrogenase deficiencyC0024776
- Branched-chain ketoaciduriaC0024776
- Keto Acid Decarboxylase DeficiencyC0024776
- Ketoacid decarboxylase deficiencyC0024776
- KetoacidaemiaC0024776
- KetoacidemiaC0024776
- Ketoaciduria, Branched-ChainC0024776
- Ketoacidurias, Branched-ChainC0024776
- MAPLE SYRUP URINE DISC0024776
- MSUDC0024776
- MSUD (Maple Syrup Urine Disease)C0024776
- MSUD - maple syrup urine diseaseC0024776
- MSUD MAPLE SYRUP URINE DISC0024776
- Maple Syrup Urine DiseaseC0024776
- Maple syrup diseaseC0024776
- Maple syrup urine diseaseC0024776
- Maple syrup urine disease (disorder)C0024776
- Maple-syrup-urine diseaseC0024776
- Oxo-acid decarboxylase deficiencyC0024776
- branched chain ketoaciduriaC0024776
- branched-chain ketoaciduriaC0024776
- disease maple syrup urineC0024776
- ketoacidemiaC0024776
- maple syrup diseaseC0024776
- maple syrup urine diseaseC0024776
- msuC0024776
- msudC0024776
Frequently Asked Questions
What is ICD-10 code E71.0?
ICD-10-CM code E71.0 represents "Maple-syrup-urine disease". It 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.
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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.