What is ICD-10 code E71.0?

E71.0 is the ICD-10-CM diagnosis code for Maple-syrup-urine disease. A rare inherited metabolic disorder where the body cannot properly break down certain amino acids, causing a buildup of maple syrup-scented urine and potentially severe neurological complications if untreated. E71.0 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E71.0 is part of the FY2026 ICD-10-CM code set published by the CMS National Center for Health Statistics and used on Medicare, Medicaid, and commercial claims in the United States.

Yes. E71.0 is a billable ICD-10-CM code that can be reported on professional and institutional claims as a primary or secondary diagnosis when the provider documentation supports the specific condition described. Billable codes in ICD-10-CM are coded to the highest level of specificity available for the documented diagnosis. E71.0 is current for fiscal year 2026 and remains in the active CMS ICD-10-CM code set published by the National Center for Health Statistics.

Which HCC does E71.0 map to under V28?

E71.0 does not map to a CMS-HCC V28 payment category. Check the current CMS-HCC V28 model file for payment year 2026 rather than an older V24 tip sheet.

What does E71.0 pay in RAF?

E71.0 has no separately stated V28 community RAF weight. If it is not a V28 payment HCC, it adds nothing to a community RAF score.

What is the V28 vs V24 difference for E71.0?

CMS phased in the V28 risk adjustment model over payment years 2024 (33%), 2025 (67%), and 2026 (100%), replacing V24. V28 consolidates HCC categories, removes some lower-acuity conditions, and recalibrates RAF weights across the entire model. E71.0 was a payment HCC under V24 (Other Significant Endocrine and Metabolic Disorders) but the V28 recalibration removed it from the risk adjustment model. Both models can still appear in legacy data and in payer-specific lookback periods.

What documentation supports E71.0?

This is a specific diagnosis code; do not use the unspecified code E71.2 if maple-syrup-urine disease is documented Ensure documentation specifies the type (classic, intermediate, or intermittent) if available for more precise coding Because E71.0 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score. See the MEAT criteria checklist at /meat-criteria for what Monitor, Evaluate, Assess, or Treat looks like in a note. MEAT is generic documentation evidence, not a payer-specific rule.

2026 ICD-10-CM E71.0: Maple-syrup-urine disease

E71.0 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · HCC Buddy coding tools

ICD-10-CM Code View

HCC Buddy Code Card

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Code lookupE71.0Effective periodFY2026 Apr Update (Apr 1-Sep 30)

FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E71.0

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Maple-syrup-urine disease

A rare inherited metabolic disorder where the body cannot properly break down certain amino acids, causing a buildup of maple syrup-scented urine and potentially severe neurological complications if untreated.

Buddy Insight

CMS-HCC V28

N/A

—

Not risk-adjusted for this model/period.

Not mapped

CMS-HCC V24

Mapped

HCC 23

Other Significant Endocrine a...

RAF 0.194

ACA/HHS

Mapped

HCC 28

HHS-HCC 28

Varies by metal level

ESRD/PACE

Mapped

HCC 23

Other Significant Endocrine a...

RAF 0.036

RXHCC

Mapped

HCC 43

Other Significant Endocrine a...

RAF 0.063

Code Book Path

Official

E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

E71.0Maple-syrup-urine disease

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for E71.0 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E71.0 in this effective period.

Related Child Codes

Official

E71.1Other disorders of branched-chain amino-acid metabolism

E71.2Disorder of branched-chain amino-acid metabolism, unspecified

E71.3Disorders of fatty-acid metabolism

E71.4Disorders of carnitine metabolism

E71.5Peroxisomal disorders

Includes

Official

ICD-10-CM does not list Includes notes for E71.0 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E71.0 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E71.0 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E71.0 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E71.0 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Documentation must include confirmed diagnosis through elevated branched-chain amino acids on plasma amino acid analysis, the specific type (classic, intermediate, intermittent, or thiamine-responsive), enzyme activity testing or genetic analysis results, and current leucine levels with target ranges.

Dietary management details including branched-chain amino acid-free medical formula, leucine intake limits, and emergency sick-day protocol should be documented.

Any history of metabolic crises should be noted.

MEAT Support

HCC Buddy guidance

Dietary management details including branched-chain amino acid-free medical formula, leucine intake limits, and emergency sick-day protocol should be documented.

Any history of metabolic crises should be noted.

Audit Caution

HCC Buddy guidance

Distinguish between the clinical variants (classic, intermediate, intermittent, thiamine-responsive) as management intensity varies significantly.

Do not delay coding during acute metabolic crises while awaiting confirmatory testing if clinical suspicion is high.

Code any neurological sequelae from prior metabolic decompensations separately.

Ensure the dietary management plan is documented to demonstrate ongoing active management of this chronic condition.

Common Mistakes

HCC Buddy guidance

E71.110-E71.118 (Organic acidemias) are related but distinct metabolic disorders.

E70.0 (Classical phenylketonuria) is another amino acid metabolism disorder requiring dietary restriction but involves a different pathway.

E71.19 (Other disorders of branched-chain amino-acid metabolism) covers less specific conditions.

E71.2 (Disorder of branched-chain amino-acid metabolism, unspecified) should not be used when maple syrup urine disease is confirmed.

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is E71.0 an HCC code?

Yes. E71.0 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

Code: E71.0
Description: Maple-syrup-urine disease
HCC (V28): No CMS-HCC V28 mapping
RAF: —
Billable: Yes
Payment year: 2026

HCC Category Mapping

V24HCC 23, Other Significant Endocrine and Metabolic Disorders

0.194

ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders

0.036

RxHCCHCC 43, Other Significant Endocrine and Metabolic Disorders

0.063

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 E71.0 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E71.0

For E71.0 to 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 E71.0 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E71.0 is the ICD-10-CM diagnosis code for maple-syrup-urine disease. A rare inherited metabolic disorder where the body cannot properly break down certain amino acids, causing a buildup of maple syrup-scented urine and potentially severe neurological complications if untreated. E71.0 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, E71.0 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 diagnosis code; do not use the unspecified code E71.2 if maple-syrup-urine disease is documented. Because E71.0 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 E71.0 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•This is a specific diagnosis code; do not use the unspecified code E71.2 if maple-syrup-urine disease is documented
•Ensure documentation specifies the type (classic, intermediate, or intermittent) if available for more precise coding

Clinical Significance

Maple syrup urine disease is a serious autosomal recessive metabolic disorder caused by deficiency of the branched-chain alpha-keto acid dehydrogenase complex, preventing proper breakdown of leucine, isoleucine, and valine. The classic form presents in neonates with poor feeding, lethargy, a distinctive sweet maple syrup odor in urine, and can rapidly progress to seizures, coma, and death without emergency treatment. Lifelong dietary restriction of branched-chain amino acids is required.

Documentation Requirements

✓Documentation must include confirmed diagnosis through elevated branched-chain amino acids on plasma amino acid analysis, the specific type (classic, intermediate, intermittent, or thiamine-responsive), enzyme activity testing or genetic analysis results, and current leucine levels with target ranges.
✓Dietary management details including branched-chain amino acid-free medical formula, leucine intake limits, and emergency sick-day protocol should be documented.
✓Any history of metabolic crises should be noted.

Commonly Confused Codes

•E71.110-E71.118 (Organic acidemias) are related but distinct metabolic disorders.
•E70.0 (Classical phenylketonuria) is another amino acid metabolism disorder requiring dietary restriction but involves a different pathway.
•E71.19 (Other disorders of branched-chain amino-acid metabolism) covers less specific conditions.
•E71.2 (Disorder of branched-chain amino-acid metabolism, unspecified) should not be used when maple syrup urine disease is confirmed.