E71.110 ICD-10-CM Code: Isovaleric acidemia
HCC Buddy Code Card
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E71.110
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceIsovaleric acidemia
Isovaleric acidemia is a rare inherited metabolic disorder where the body cannot properly break down certain amino acids, leading to a buildup of isovaleric acid in the blood and urine. This can cause serious health problems including developmental delays, seizures, and organ damage if not managed properly.
Buddy Insight
Isovaleric acidemia is a rare autosomal recessive organic acidemia caused by deficiency of isovaleryl-CoA dehydrogenase, an enzyme in the leucine degradation pathway.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
MappedHCC 23
RAF 0.194
ACA/HHS
MappedHCC 28
Varies by metal level
ESRD/PACE
MappedHCC 23
RAF 0.036
RXHCC
MappedHCC 43
RAF 0.063
Code Book Path
Inclusion Terms
OfficialICD-10-CM does not list inclusion terms for E71.110 in this effective period.
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for E71.110 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for E71.110 in this effective period.
Excludes 1
OfficialICD-10-CM does not list Excludes 1 notes for E71.110 in this effective period.
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for E71.110 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for E71.110 in this effective period.
Code Also
OfficialICD-10-CM does not list Code Also instructions for E71.110 in this effective period.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.
Is E71.110 an HCC code?
Yes. E71.110 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.
HCC Category Mapping
Each model's RAF is its CMS base weight for that model's standard population, so weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.
Work E71.110 in the Code Book — tabular path, V28 RAF, and MEAT checklist →
MEAT Criteria for E71.110
For E71.110to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.
- MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
- EEvaluate: test results, medication response, or physical findings reviewed by the provider
- AAssess: explicit mention in the assessment or plan with acknowledgment of status
- TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis
Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E71.110 during that encounter, not just copy-forwarded from a problem list.
Coder workflow notes
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What This Code Means
E71.110 is the ICD-10-CM diagnosis code for isovaleric acidemia. Isovaleric acidemia is a rare inherited metabolic disorder where the body cannot properly break down certain amino acids, leading to a buildup of isovaleric acid in the blood and urine. This can cause serious health problems including developmental delays, seizures, and organ damage if not managed properly. E71.110 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).
Under the older CMS-HCC V24 model, E71.110 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.194. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.
This is a specific metabolic disorder code that requires documentation of the diagnosis; ensure the provider has documented 'isovaleric acidemia' specifically rather than just 'organic acidemia' or 'metabolic disorder'. Because E71.110 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.
HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for E71.110 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •This is a specific metabolic disorder code that requires documentation of the diagnosis; ensure the provider has documented 'isovaleric acidemia' specifically rather than just 'organic acidemia' or 'metabolic disorder'
- •This condition often requires ongoing management and may have associated complications (such as developmental delay, seizures, or cardiomyopathy) that should be coded separately if documented
Clinical Significance
Isovaleric acidemia is a rare autosomal recessive organic acidemia caused by deficiency of isovaleryl-CoA dehydrogenase, an enzyme in the leucine degradation pathway. Patients accumulate isovaleric acid, producing a characteristic sweaty feet odor. The acute neonatal form can cause life-threatening metabolic acidosis, while the chronic intermittent form triggers crises during illness or protein loading. Long-term management includes leucine-restricted diet and glycine/carnitine supplementation.
Documentation Requirements
- ✓Documentation must include confirmed diagnosis through elevated isovalerylglycine on urine organic acid analysis or enzyme/genetic testing, the clinical phenotype (acute neonatal vs.
- ✓chronic intermittent), current metabolic status and leucine levels, and ongoing treatment including dietary restriction, glycine supplementation, and carnitine supplementation.
- ✓Emergency management protocols for metabolic crises should be documented.
- ✓Any developmental or neurological complications from prior crises should be noted.
Commonly Confused Codes
- •E71.111 (Methylmalonic acidemia) and E71.118 (Other organic acidemias) are distinct organic acidemias with different enzyme deficiencies.
- •E71.0 (Maple syrup urine disease) also involves branched-chain amino acid metabolism but a different enzyme.
- •E71.19 (Other disorders of branched-chain amino-acid metabolism) is less specific.
- •E87.2 (Acidosis) may be coded additionally during acute metabolic crises.