ICD10BillableValid for clinical use

ICD-10 Code E71.110: Isovaleric acidemia

E71.110 is a billable ICD-10 diagnosis code used to classify Isovaleric acidemia in medical records and claims. You may see this code in hospital records, discharge summaries, insurance claims, encounter documentation, referrals, or other healthcare billing and coding records. ICD-10 codes are diagnosis classification codes used in healthcare records, reporting, coding workflows, and billing support. This code sits within the broader ICD-10 area for Endocrine, nutritional and metabolic diseases (E00-E89).

Reviewed by HealthAssure Clinical TeamUpdated 21 May 2026

What is this code?

ICD-10 codes are diagnosis classification codes used in healthcare records, reporting, coding workflows, and billing support. This code sits within the broader ICD-10 area for Endocrine, nutritional and metabolic diseases (E00-E89).

When is it used?

  • May be used when a clinician documents isovaleric acidemia in a patient's medical record.
  • May appear in hospital records, claims, referrals, and clinical documentation.
  • This code can be used as a clinically usable diagnosis entry in standardized coding workflows.

What it does not mean

  • A code alone does not explain severity, treatment plan, or outcome.
  • A medical code should not be treated as a substitute for a doctor's diagnosis or advice.

Code hierarchy

chapter
4Endocrine, nutritional and metabolic diseases (E00-E89)
block
E70-E88Metabolic disorders
category
E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
category
E71.1Other disorders of branched-chain amino-acid metabolism
category
E71.11Branched-chain organic acidurias
currentE71.11

Where you may see this code

You may see this code in hospital records, discharge summaries, insurance claims, encounter documentation, referrals, or other healthcare billing and coding records.

Related specialists

EndocrinologistGeneral Physician

Coding guidelines

Common synonyms

Isovaleric acidemia

Frequently asked questions

Code details

CodeE71.110
SystemICD10
Display nameIsovaleric acidemia
ChapterEndocrine, nutritional and metabolic diseases (E00-E89)
BlockMetabolic disorders

Flags

BillableYes
Valid clinical useYes

Source

SourceICD-10
Version2026-annual
Releaseannual
Year2026

Index terms

Matched terms
AcidemiaDisorder(of)Disorder(of)Isovaleric acidemia

About this content

This page is prepared by HealthAssure's clinical team using official coding standards from ICD-10. AI tools assist with drafting explanations, which are then reviewed and verified by healthcare professionals for accuracy. This content is for informational purposes and does not replace professional medical advice. Meet our team.

ICD-10 Code E71.110 for Isovaleric acidemia | HealthAssure