E71.0 is the ICD-10 code for Maple-syrup-urine disease. It is a four-character subcategory of E71 (Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism), the most specific level in the WHO edition. It belongs to the block E70–E90 (Metabolic disorders) in Chapter IV, Endocrine, nutritional and metabolic diseases.
- Code type
- Subcategory (4 characters)
- Chapter
- IV · E00–E90
- Classification
- WHO ICD-10, 2019 version
E71.0 in national editions
Countries report with their own edition of ICD-10. Most keep the WHO code and add more specific codes beneath it.
E71.0«Maple syrup urine disease»
DE710Ahornsirup-urin-sygdom (MSUD)
E71.0Vaahterasiirappitauti
E71.0Ahornsirup- (Harn-) Krankheit
E71.0Maladie des urines à odeur de sirop d'érable
E71.0maple-syrup urine disease
E71.0Maple-syrup-urine disease
Based on the World Health Organization’s International Statistical Classification of Diseases and Related Health Problems, 10th revision (ICD-10), 2019 version, and the national editions published by each country’s health authority. This page is a reference, not coding advice; follow your national coding rules when you report.
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