E71.313 ICD-10-CM Code: Glutaric aciduria type II
E71.313 is not a CMS-HCC payment code. MEAT criteria · RAF Calculator · HCC coding software
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E71.313
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceGlutaric aciduria type II
A rare inherited metabolic disorder where the body cannot properly process certain amino acids and fatty acids, resulting in the accumulation of glutaric acid in the body.
Buddy Insight
Glutaric aciduria type II (multiple acyl-CoA dehydrogenase deficiency or MADD) is a severe inborn error of metabolism affecting multiple mitochondrial dehydrogenases simultaneously, impairing both fatty acid oxidation and amino acid metabolism.
CMS-HCC V28
N/A—
Not mapped
CMS-HCC V24
HistoricalHistorical
Not used for CY2026 payment
ACA/HHS
Context neededHCC 028
Coefficient needs member context
ESRD/PACE
Context neededHCC 23
Coefficient needs member context
RXHCC
MappedHCC 43
Code-level coefficient reference
Code Book Path
Inclusion Terms
Official- Glutaric aciduria type II A
- Glutaric aciduria type II B
- Glutaric aciduria type II C
Excludes 2
Official- Ehlers-Danlos syndromes (Q79.6-)Inherited from E70-E88, E71.3
- carnitine deficiency due to inborn error of metabolism (E71.42)Inherited from E70-E88, E71.3
Related Codes
Includes
OfficialNo Includes notes are included in this display for E71.313. Check the code and parent instructions in the Code Book.
Excludes 1
Official- glutaric aciduria (type 1) NOS (E72.3)
- transitory endocrine and metabolic disorders specific to newborn (P70-P74)Inherited from E00-E89, E70-E88, E71.3
- androgen insensitivity syndrome (E34.5-)Inherited from E00-E89, E70-E88, E71.3
- congenital adrenal hyperplasia (E25.0)Inherited from E00-E89, E70-E88, E71.3
- hemolytic anemias attributable to enzyme disorders (D55.-)Inherited from E00-E89, E70-E88, E71.3
- Marfan syndrome (Q87.4-)Inherited from E00-E89, E70-E88, E71.3
- 5-alpha-reductase deficiency (E29.1)Inherited from E00-E89, E70-E88, E71.3
- peroxisomal disorders (E71.5)Inherited from E00-E89, E70-E88, E71.3
- Refsum's disease (G60.1)Inherited from E00-E89, E70-E88, E71.3
- Schilder's disease (G37.0)Inherited from E00-E89, E70-E88, E71.3
Code First
OfficialNo Code First sequencing instructions are included in this display for E71.313. Check the code and parent instructions in the Code Book.
Use Additional
OfficialNo Use Additional Code instructions are included in this display for E71.313. Check the code and parent instructions in the Code Book.
Code Also
OfficialNo Code Also instructions are included in this display for E71.313. Check the code and parent instructions in the Code Book.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
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 E71.313 an HCC code?
E71.313 is not in the CMS-HCC V28 or V24 community payment model. E71.313 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders)); the applicable result needs member context. E71.313 also appears in the HHS-HCC commercial risk model (HCC 028 (HHS-HCC 028 child, RAF varies by metal level)), where the result depends on member context and is not a Medicare Advantage payment mapping.
- Code
- E71.313
- Description
- Glutaric aciduria type II
- HCC (V28)
- No CMS-HCC V28 mapping
- RAF reference coefficient
- —
- Billable
- Yes
- Payment year
- 2026
HCC Category Mapping
These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. 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 context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.
Work E71.313 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →
MEAT review for E71.313
For E71.313, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.
- 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
Coder workflow notes
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What This Code Means
E71.313 is the ICD-10-CM diagnosis code for glutaric aciduria type ii. A rare inherited metabolic disorder where the body cannot properly process certain amino acids and fatty acids, resulting in the accumulation of glutaric acid in the body. E71.313 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).
E71.313 has no mapping under the CMS-HCC V28 or V24 community payment models. E71.313 has a separate mapping under the CMS-HCC ESRD model (HCC 23 (Other Significant Endocrine and Metabolic Disorders)) and the Part D RxHCC model (HCC 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders)); the applicable result needs member context. E71.313 also appears in the HHS-HCC commercial risk model (HCC 028 (HHS-HCC 028 child, RAF varies by metal level)), where the result depends on member context and is not a Medicare Advantage payment mapping. Do not assign V28 risk adjustment value from this page; verify the applicable model and payment year before using this code for risk adjustment.
Glutaric aciduria type II is distinct from type I (which has a different code); confirm the type is documented as type II.
HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E71.313 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •Glutaric aciduria type II is distinct from type I (which has a different code); confirm the type is documented as type II
- •Look for associated complications such as hypoglycemia, cardiomyopathy, or developmental delays that may require additional coding
Clinical Significance
Glutaric aciduria type II (multiple acyl-CoA dehydrogenase deficiency or MADD) is a severe inborn error of metabolism affecting multiple mitochondrial dehydrogenases simultaneously, impairing both fatty acid oxidation and amino acid metabolism. The neonatal-onset form presents with severe hypoglycemia, metabolic acidosis, and often congenital anomalies, while later-onset forms may respond to riboflavin therapy. This is distinct from glutaric aciduria type I, which involves a different enzyme and clinical presentation.
Documentation Requirements
- ✓Document the specific form (neonatal severe, neonatal mild, or late-onset), acylcarnitine profile showing multiple elevated species, urine organic acid results, genetic testing for ETFA/ETFB/ETFDH mutations, and riboflavin responsiveness status.
- ✓Record any congenital anomalies and current treatment regimen.
Excludes 1, Do NOT code together
- glutaric aciduria (type 1) NOS (E72.3)