ICD-10-CM E71.0 is the diagnosis code for Maple-syrup-urine disease. This code falls under the section "Metabolic disorders" within Chapter 4 — Endocrine, nutritional and metabolic diseases (E00-E89). It is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. Medical coders and healthcare providers use this code to document and classify diagnoses in electronic health records, insurance claims, and clinical databases.
Always refer to the official ICD-10-CM Tabular List for complete coding guidelines. Ensure documentation supports the specificity of the code selected. When in doubt, consult a certified medical coder or the latest CMS guidelines.
Understanding where E71.0 sits in the ICD-10-CM classification helps ensure proper coding:
Yes, E71.0 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
E71.0 is the ICD-10-CM diagnosis code for "Maple-syrup-urine disease". It is used by healthcare providers to classify and document this condition in medical records and insurance claims.
The parent code of E71.0 is E71 ("Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism"). E71.0 provides a more specific classification within this category.
E71.0 is located in Section E70-E88 — "Metabolic disorders" within Chapter 4 of the ICD-10-CM Tabular List.
Use E71.0 when the patients documented diagnosis matches "Maple-syrup-urine disease" and the clinical documentation supports this level of specificity. Always verify with the latest ICD-10-CM guidelines and payer requirements.
The ICD-10-CM code for Maple-syrup-urine disease is E71.0.
Yes, E71.0 can be used as a primary diagnosis code since it is billable and specific.
E71.0 is in Chapter 4 of the ICD-10-CM Tabular List.
Yes, E71.0 is a valid ICD-10-CM code for the 2026 fiscal year, subject to official CMS updates.