What is ICD-10 code E74.4?

E74.4 is the ICD-10-CM diagnosis code for Disorders of pyruvate metabolism and gluconeogenesis. A metabolic disorder affecting how the body produces energy from pyruvate or creates new glucose, leading to lactic acidosis, neurological problems, or low blood sugar. E74.4 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E74.4 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. E74.4 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. E74.4 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.

What is the V28 vs V24 difference for E74.4?

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. E74.4 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 should coders know when reporting E74.4?

Specify the type of pyruvate metabolism disorder if documented (pyruvate dehydrogenase deficiency, etc.) Note any complications such as lactic acidosis or neurological manifestations for additional coding Because E74.4 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.

2026 ICD-10-CM E74.4: Disorders of pyruvate

ICD-10-CM Code View

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Code lookupE74.4Effective periodFY2026 Apr Update (Apr 1-Sep 30)

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

E74.4

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

Disorders of pyruvate metabolism and gluconeogenesis

A metabolic disorder affecting how the body produces energy from pyruvate or creates new glucose, leading to lactic acidosis, neurological problems, or low blood sugar.

Buddy Insight

Disorders of pyruvate metabolism and gluconeogenesis include pyruvate dehydrogenase deficiency, pyruvate carboxylase deficiency, and phosphoenolpyruvate carboxykinase deficiency.

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

E74Other disorders of carbohydrate metabolism

E74.4Disorders of pyruvate metabolism and gluconeogenesis

Inclusion Terms

Official

Deficiency of phosphoenolpyruvate carboxykinase
Deficiency of pyruvate carboxylase
Deficiency of pyruvate dehydrogenase

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E74.4 in this effective period.

Related Child Codes

Official

E74.0Glycogen storage disease

E74.1Disorders of fructose metabolism

E74.2Disorders of galactose metabolism

E74.3Other disorders of intestinal carbohydrate absorption

E74.8Other specified disorders of carbohydrate metabolism

Includes

Official

ICD-10-CM does not list Includes notes for E74.4 in this effective period.

Excludes 1

Official

disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-)
Leigh's syndrome (G31.82)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E74.4 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E74.4 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E74.4 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Specific pyruvate metabolism or gluconeogenesis enzyme deficiency identified

Laboratory confirmation including lactate levels, pyruvate levels, and lactate-to-pyruvate ratio

Genetic testing or enzyme assay results

Documentation of neurological findings: developmental delay, seizures, or encephalopathy

MEAT Support

HCC Buddy guidance

Specific pyruvate metabolism or gluconeogenesis enzyme deficiency identified

Laboratory confirmation including lactate levels, pyruvate levels, and lactate-to-pyruvate ratio

Genetic testing or enzyme assay results

Documentation of neurological findings: developmental delay, seizures, or encephalopathy

Audit Caution

HCC Buddy guidance

Coding only the lactic acidosis or hypoglycemia without identifying the underlying pyruvate metabolism disorder

Confusing pyruvate dehydrogenase deficiency (responds to ketogenic diet) with pyruvate carboxylase deficiency (worsened by ketogenic diet)

Not recognizing that persistent lactic acidosis in a neonate may indicate a pyruvate metabolism disorder requiring further workup

Using a general metabolic disorder code when E74.4 specifically covers this category

Common Mistakes

HCC Buddy guidance

E87.2 — Acidosis: lactic acidosis is a manifestation of pyruvate disorders, not the primary diagnosis

E74.4 specifically captures pyruvate metabolism defects, while E88.89 covers other metabolic disorders

E74.00-E74.09 — Glycogen storage diseases: separate category of carbohydrate metabolism disorders

G93.49 — Other encephalopathy: neurological damage is a complication, not the underlying metabolic defect

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 E74.4 an HCC code?

Yes. E74.4 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

Code: E74.4
Description: Disorders of pyruvate metabolism and gluconeogenesis
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 E74.4 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E74.4

For E74.4 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 E74.4 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E74.4 is the ICD-10-CM diagnosis code for disorders of pyruvate metabolism and gluconeogenesis. A metabolic disorder affecting how the body produces energy from pyruvate or creates new glucose, leading to lactic acidosis, neurological problems, or low blood sugar. E74.4 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, E74.4 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.

Specify the type of pyruvate metabolism disorder if documented (pyruvate dehydrogenase deficiency, etc.). Because E74.4 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 E74.4 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•Specify the type of pyruvate metabolism disorder if documented (pyruvate dehydrogenase deficiency, etc.)
•Note any complications such as lactic acidosis or neurological manifestations for additional coding

Clinical Significance

Disorders of pyruvate metabolism and gluconeogenesis include pyruvate dehydrogenase deficiency, pyruvate carboxylase deficiency, and phosphoenolpyruvate carboxykinase deficiency. These conditions disrupt central energy metabolism, causing severe lactic acidosis, hypoglycemia, and progressive neurological damage. They are among the most serious metabolic disorders, often presenting in the neonatal period with metabolic crisis.

Documentation Requirements

✓Specific pyruvate metabolism or gluconeogenesis enzyme deficiency identified
✓Laboratory confirmation including lactate levels, pyruvate levels, and lactate-to-pyruvate ratio
✓Genetic testing or enzyme assay results
✓Documentation of neurological findings: developmental delay, seizures, or encephalopathy
✓Current treatment plan including dietary management (ketogenic diet for PDH deficiency) and cofactor supplementation

Excludes 1, Do NOT code together

disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-)
Leigh's syndrome (G31.82)

Commonly Confused Codes

•E87.2: Acidosis: lactic acidosis is a manifestation of pyruvate disorders, not the primary diagnosis
•E74.4 specifically captures pyruvate metabolism defects, while E88.89 covers other metabolic disorders
•E74.00-E74.09: Glycogen storage diseases: separate category of carbohydrate metabolism disorders
•G93.49: Other encephalopathy: neurological damage is a complication, not the underlying metabolic defect
•E16.2: Hypoglycemia, unspecified: hypoglycemia from gluconeogenesis defects should use E74.4 as primary