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E71.19 ICD-10-CM Code: Other disorders of branched-chain amino-acid metabolism

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

E71.19

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

Other disorders of branched-chain amino-acid metabolism

A group of rare inherited metabolic disorders affecting how the body breaks down branched-chain amino acids, not classified elsewhere.

Buddy the Bee presenting code insight

Buddy Insight

This residual code captures branched-chain amino acid metabolism disorders not classified in more specific E71.

CMS-HCC V28

N/A

Not mapped

CMS-HCC V24

HCC 23

RAF 0.194

ACA/HHS

HCC 28

Varies by metal level

ESRD/PACE

HCC 23

RAF 0.036

RXHCC

HCC 43

RAF 0.063

Code Book Path

Official
E71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
E71.1Other disorders of branched-chain amino-acid metabolism
E71.19Other disorders of branched-chain amino-acid metabolism

Inclusion Terms

Official
  • Hyperleucine-isoleucinemia
  • Hypervalinemia

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E71.19 in this effective period.

Related Child Codes

Official
E71.11Branched-chain organic acidurias
E71.12Disorders of propionate metabolism

Includes

Official

ICD-10-CM does not list Includes notes for E71.19 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E71.19 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E71.19 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E71.19 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E71.19 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
Document the specific metabolic abnormality identified, plasma amino acid levels, urine organic acid profiles, enzyme activity results or genetic testing, and clinical manifestations.
Explain why a more specific code cannot be assigned based on available diagnostic information.

MEAT Support

HCC Buddy guidance
Document the specific metabolic abnormality identified, plasma amino acid levels, urine organic acid profiles, enzyme activity results or genetic testing, and clinical manifestations.
Explain why a more specific code cannot be assigned based on available diagnostic information.

Audit Caution

HCC Buddy guidance
This is an 'other specified' code, not an unspecified code.
Use when a specific branched-chain disorder is documented but does not have a dedicated code.
Do not use when documentation supports a more specific code assignment.

Common Mistakes

HCC Buddy guidance
E71.2 (Disorder of branched-chain amino-acid metabolism, unspecified) when specificity is lacking entirely
E71.0 (Maple-syrup-urine disease) when branched-chain ketoacid dehydrogenase is the specific enzyme involved
E71.111-E71.128 for specific identified organic acidurias.

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 E71.19 an HCC code?

Yes. E71.19 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

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 E71.19 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E71.19

For E71.19to 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 E71.19 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E71.19 is the ICD-10-CM diagnosis code for other disorders of branched-chain amino-acid metabolism. A group of rare inherited metabolic disorders affecting how the body breaks down branched-chain amino acids, not classified elsewhere. E71.19 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, E71.19 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 catch-all code for branched-chain amino acid disorders not specified elsewhere. Because E71.19 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 E71.19 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • This is a catch-all code for branched-chain amino acid disorders not specified elsewhere
  • Use only when a more specific E71 code cannot be assigned based on documentation

Clinical Significance

This residual code captures branched-chain amino acid metabolism disorders not classified in more specific E71.1 subcategories, including rare enzyme deficiencies affecting leucine, isoleucine, or valine catabolism. These conditions can present with variable clinical severity from asymptomatic biochemical abnormalities to severe metabolic crises. Ongoing metabolic monitoring and dietary management are typically required.

Documentation Requirements

  • Document the specific metabolic abnormality identified, plasma amino acid levels, urine organic acid profiles, enzyme activity results or genetic testing, and clinical manifestations.
  • Explain why a more specific code cannot be assigned based on available diagnostic information.

Commonly Confused Codes

  • E71.2 (Disorder of branched-chain amino-acid metabolism, unspecified) when specificity is lacking entirely
  • E71.0 (Maple-syrup-urine disease) when branched-chain ketoacid dehydrogenase is the specific enzyme involved
  • E71.111-E71.128 for specific identified organic acidurias.

Child Codes

Code Hierarchy

Because E71.19 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.

Work E71.19 in HCC Buddy

Open E71.19 in the Code Book for the full Index-to-Tabular path, MEAT checklist, and V28 HCC mapping, or in the Encoder to code from a keyword search. Pro includes 14 days to try everything.