ICD-10 · PDGM grouping
E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
All 38 codes in the E71 family, with the PDGM clinical group, comorbidity subgroup and primary-position rule CMS assigns, read from the CMS grouper crosswalk v07.2.26 and the CDC FY2027 code file.
Codes in family
38
Can lead a claim
38
Cannot lead a claim
0
What CMS assigns to E71
All 38 codes group to MMTA - Other. None of the 38 is assigned a comorbidity subgroup. Every code can lead a claim.
Every code in E71
Descriptions are CDC’s FY2027 wording; where CMS’s grouper abbreviates one, its form is shown beneath.
| Code | Description | Primary position | Clinical group | Comorbidity subgroup |
|---|---|---|---|---|
| E71.0 | Maple-syrup-urine disease | Can lead a claim | A | None |
| E71.1 Other disorders of branched-chain amino-acid metabolism | ||||
| E71.11 Branched-chain organic acidurias | ||||
| E71.110 | Isovaleric acidemia | Can lead a claim | A | None |
| E71.111 | 3-methylglutaconic aciduria | Can lead a claim | A | None |
| E71.118 | Other branched-chain organic acidurias | Can lead a claim | A | None |
| E71.12 Disorders of propionate metabolism | ||||
| E71.120 | Methylmalonic acidemia | Can lead a claim | A | None |
| E71.121 | Propionic acidemia | Can lead a claim | A | None |
| E71.128 | Other disorders of propionate metabolism | Can lead a claim | A | None |
| E71.19 | Other disorders of branched-chain amino-acid metabolism | Can lead a claim | A | None |
| E71.2 | Disorder of branched-chain amino-acid metabolism, unspecifiedCMS: Disorder of branched-chain amino-acid metabolism, unsp | Can lead a claim | A | None |
| E71.3 Disorders of fatty-acid metabolism | ||||
| E71.30 | Disorder of fatty-acid metabolism, unspecified | Can lead a claim | A | None |
| E71.31 Disorders of fatty-acid oxidation | ||||
| E71.310 | Long chain/very long chain acyl CoA dehydrogenase deficiency | Can lead a claim | A | None |
| E71.311 | Medium chain acyl CoA dehydrogenase deficiency | Can lead a claim | A | None |
| E71.312 | Short chain acyl CoA dehydrogenase deficiency | Can lead a claim | A | None |
| E71.313 | Glutaric aciduria type II | Can lead a claim | A | None |
| E71.314 | Muscle carnitine palmitoyltransferase deficiency | Can lead a claim | A | None |
| E71.318 | Other disorders of fatty-acid oxidation | Can lead a claim | A | None |
| E71.32 | Disorders of ketone metabolism | Can lead a claim | A | None |
| E71.39 | Other disorders of fatty-acid metabolism | Can lead a claim | A | None |
| E71.4 Disorders of carnitine metabolism | ||||
| E71.40 | Disorder of carnitine metabolism, unspecified | Can lead a claim | A | None |
| E71.41 | Primary carnitine deficiency | Can lead a claim | A | None |
| E71.42 | Carnitine deficiency due to inborn errors of metabolism | Can lead a claim | A | None |
| E71.43 | Iatrogenic carnitine deficiency | Can lead a claim | A | None |
| E71.44 Other secondary carnitine deficiency | ||||
| E71.440 | Ruvalcaba-Myhre-Smith syndrome | Can lead a claim | A | None |
| E71.448 | Other secondary carnitine deficiency | Can lead a claim | A | None |
| E71.5 Peroxisomal disorders | ||||
| E71.50 | Peroxisomal disorder, unspecified | Can lead a claim | A | None |
| E71.51 Disorders of peroxisome biogenesis | ||||
| E71.510 | Zellweger syndrome | Can lead a claim | A | None |
| E71.511 | Neonatal adrenoleukodystrophy | Can lead a claim | A | None |
| E71.518 | Other disorders of peroxisome biogenesis | Can lead a claim | A | None |
| E71.52 X-linked adrenoleukodystrophy | ||||
| E71.520 | Childhood cerebral X-linked adrenoleukodystrophy | Can lead a claim | A | None |
| E71.521 | Adolescent X-linked adrenoleukodystrophy | Can lead a claim | A | None |
| E71.522 | Adrenomyeloneuropathy | Can lead a claim | A | None |
| E71.528 | Other X-linked adrenoleukodystrophy | Can lead a claim | A | None |
| E71.529 | X-linked adrenoleukodystrophy, unspecified type | Can lead a claim | A | None |
| E71.53 | Other group 2 peroxisomal disorders | Can lead a claim | A | None |
| E71.54 Other peroxisomal disorders | ||||
| E71.540 | Rhizomelic chondrodysplasia punctata | Can lead a claim | A | None |
| E71.541 | Zellweger-like syndrome | Can lead a claim | A | None |
| E71.542 | Other group 3 peroxisomal disorders | Can lead a claim | A | None |
| E71.548 | Other peroxisomal disorders | Can lead a claim | A | None |
Sources: CMS HH PPS Grouper Software v07.2.26, effective 2026-10-01, for the clinical group, comorbidity subgroup, code-first conventions and primary-position flags; CDC NCHS ICD-10-CM FY2027 code descriptions for the family title and code wording. This is the crosswalk Medicare runs a claim through. Grouping also depends on admission source, timing, the OASIS functional items and the rest of the diagnosis list.
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