E71.53 ICD-10-CM Code: Other group 2 peroxisomal disorders
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E71.53
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceOther group 2 peroxisomal disorders
Rare inherited metabolic disorders affecting peroxisomes (cellular structures) that cause various combinations of neurological, developmental, and metabolic problems.
Buddy Insight
Other group 2 peroxisomal disorders include single-enzyme peroxisomal defects such as adult Refsum disease (phytanic acid oxidase deficiency), D-bifunctional protein deficiency, and acyl-CoA oxidase deficiency.
CMS-HCC V28
00
RAF 0
CMS-HCC V24
MappedHCC 23
RAF 0.230
ACA/HHS
00
RAF 0
ESRD/PACE
MappedHCC 23
RAF 0.0
RXHCC
MappedHCC 43
RAF 0.0
Code Trumping
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Code Book Path
Inclusion Terms
OfficialICD-10-CM does not list inclusion terms for E71.53 in this effective period.
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for E71.53 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for E71.53 in this effective period.
Excludes 1
Official- Schilder's disease (G37.0)
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for E71.53 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for E71.53 in this effective period.
Code Also
OfficialICD-10-CM does not list Code Also instructions for E71.53 in this effective period.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
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 E71.53 an HCC code?
Yes. E71.53 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.
HCC Category Mapping
RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. 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 E71.53 in the Code Book — tabular path, V28 RAF, and MEAT checklist →
MEAT Criteria for E71.53
For E71.53to 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 E71.53 during that encounter, not just copy-forwarded from a problem list.
Coder workflow notes
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What This Code Means
E71.53 is the ICD-10-CM diagnosis code for other group 2 peroxisomal disorders. Rare inherited metabolic disorders affecting peroxisomes (cellular structures) that cause various combinations of neurological, developmental, and metabolic problems. E71.53 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, E71.53 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.230. 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 code represents a category of peroxisomal disorders; document the specific diagnosis if known (e.g., Refsum disease, hyperoxaluria type 1). Because E71.53 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 E71.53 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.
Coding Tips
- •This code represents a category of peroxisomal disorders; document the specific diagnosis if known (e.g., Refsum disease, hyperoxaluria type 1)
- •Ensure documentation includes clinical manifestations and metabolic findings to support the diagnosis and guide appropriate management
Clinical Significance
Other group 2 peroxisomal disorders include single-enzyme peroxisomal defects such as adult Refsum disease (phytanic acid oxidase deficiency), D-bifunctional protein deficiency, and acyl-CoA oxidase deficiency. These conditions affect specific peroxisomal metabolic pathways rather than peroxisome biogenesis as a whole. Clinical presentations vary from progressive neuropathy and retinitis pigmentosa in Refsum disease to severe neonatal-onset neurological disease in other subtypes.
Documentation Requirements
- ✓Document the specific peroxisomal enzyme deficiency, biochemical markers (phytanic acid, pristanic acid, or very long chain fatty acid levels as appropriate), genetic testing results, clinical manifestations, and management plan.
- ✓Specify why a more specific peroxisomal code is not applicable.