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E70.49 ICD-10-CM Code: Other disorders of histidine metabolism

E70.49 is not a CMS-HCC payment code. MEAT criteria · RAF calculator · HCC Buddy coding tools

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

E70.49

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

Other disorders of histidine metabolism

Other rare metabolic disorders affecting histidine processing in the body, excluding histidinemia.

Buddy the Bee presenting code insight

Buddy Insight

Other disorders of histidine metabolism captures rare metabolic conditions affecting histidine processing that are distinct from classic histidinemia, such as urocanic aciduria (histidase deficiency variant) and formiminoglutamic aciduria (deficiency in formiminotransferase).

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
E70Disorders of aromatic amino-acid metabolism
E70.4Disorders of histidine metabolism
E70.49Other disorders of histidine metabolism

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for E70.49 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E70.49 in this effective period.

Related Child Codes

Official
E70.40Disorders of histidine metabolism, unspecified
E70.41Histidinemia

Includes

Official

ICD-10-CM does not list Includes notes for E70.49 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E70.49 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E70.49 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E70.49 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E70.49 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
Documentation should specify the exact histidine metabolism disorder identified, supporting laboratory evidence (urine organic acids, enzyme assays, or genetic testing), clinical manifestations, and management plan.
The provider should clearly distinguish this from histidinemia and from unspecified histidine disorders.
Any associated developmental, hematologic, or immunologic complications should be documented.

MEAT Support

HCC Buddy guidance
Documentation should specify the exact histidine metabolism disorder identified, supporting laboratory evidence (urine organic acids, enzyme assays, or genetic testing), clinical manifestations, and management plan.
The provider should clearly distinguish this from histidinemia and from unspecified histidine disorders.
Any associated developmental, hematologic, or immunologic complications should be documented.

Audit Caution

HCC Buddy guidance
Ensure documentation clearly specifies the histidine metabolism disorder to justify use of this other-specified code rather than the unspecified code.
Do not use this code for histidinemia, which has its own code.
Verify that clinical findings are attributable to the metabolic disorder rather than coincidental conditions.
Code all complications individually.

Common Mistakes

HCC Buddy guidance
E70.41 (Histidinemia) is the specific code for that condition.
E70.40 (Disorders of histidine metabolism, unspecified) is used when the type cannot be determined.
E70.89 (Other disorders of aromatic amino-acid metabolism) covers broader amino acid metabolism disorders.
D53.1 (Other megaloblastic anemias, not elsewhere classified) may be relevant for folate-related histidine metabolism disorders.

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 E70.49 an HCC code?

Yes. E70.49 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

Code
E70.49
Description
Other disorders of histidine metabolism
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 E70.49 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E70.49

For E70.49 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 E70.49 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E70.49 is the ICD-10-CM diagnosis code for other disorders of histidine metabolism. Other rare metabolic disorders affecting histidine processing in the body, excluding histidinemia. E70.49 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, E70.49 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.

Use this code for histidine metabolism disorders that are not histidinemia or unspecified. Because E70.49 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 E70.49 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • Use this code for histidine metabolism disorders that are not histidinemia or unspecified
  • Ensure documentation clearly describes the specific histidine metabolism abnormality to justify use of this code

Clinical Significance

Other disorders of histidine metabolism captures rare metabolic conditions affecting histidine processing that are distinct from classic histidinemia, such as urocanic aciduria (histidase deficiency variant) and formiminoglutamic aciduria (deficiency in formiminotransferase). These conditions may be associated with more significant clinical manifestations than benign histidinemia, including intellectual disability, anemia, and immune dysfunction.

Documentation Requirements

  • Documentation should specify the exact histidine metabolism disorder identified, supporting laboratory evidence (urine organic acids, enzyme assays, or genetic testing), clinical manifestations, and management plan.
  • The provider should clearly distinguish this from histidinemia and from unspecified histidine disorders.
  • Any associated developmental, hematologic, or immunologic complications should be documented.

Commonly Confused Codes

  • E70.41 (Histidinemia) is the specific code for that condition.
  • E70.40 (Disorders of histidine metabolism, unspecified) is used when the type cannot be determined.
  • E70.89 (Other disorders of aromatic amino-acid metabolism) covers broader amino acid metabolism disorders.
  • D53.1 (Other megaloblastic anemias, not elsewhere classified) may be relevant for folate-related histidine metabolism disorders.

Child Codes

Code Hierarchy

Because E70.49 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 E70.49 in HCC Buddy

Open E70.49 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 7 days to try everything, no card required.