Category E71
Non-Billable Category
FY2027 Dataset
E71: Disord of branched-chain amino-acid metab & fatty-acid metab
Parent Chapter: Chapter 4: Endocrine, nutritional and metabolic diseases
Notice on Coding Specificity: Category E71 is a 3-character heading and cannot be reported directly on healthcare claims. You must assign one of the specific child codes listed below.
Specific Child Codes in Category E71
| Code | Official Descriptor | Status | Action |
|---|---|---|---|
| E71.0 | Maple-syrup-urine disease | Billable Code | View Code |
| E71.110 | Isovaleric acidemia | Billable Code | View Code |
| E71.111 | 3-methylglutaconic aciduria | Billable Code | View Code |
| E71.118 | Other branched-chain organic acidurias | Billable Code | View Code |
| E71.120 | Methylmalonic acidemia | Billable Code | View Code |
| E71.121 | Propionic acidemia | Billable Code | View Code |
| E71.128 | Other disorders of propionate metabolism | Billable Code | View Code |
| E71.19 | Other disorders of branched-chain amino-acid metabolism | Billable Code | View Code |
| E71.2 | Disorder of branched-chain amino-acid metabolism, unsp | Billable Code | View Code |
| E71.30 | Disorder of fatty-acid metabolism, unspecified | Billable Code | View Code |
| E71.310 | Long chain/very long chain acyl CoA dehydrogenase deficiency | Billable Code | View Code |
| E71.311 | Medium chain acyl CoA dehydrogenase deficiency | Billable Code | View Code |
| E71.312 | Short chain acyl CoA dehydrogenase deficiency | Billable Code | View Code |
| E71.313 | Glutaric aciduria type II | Billable Code | View Code |
| E71.314 | Muscle carnitine palmitoyltransferase deficiency | Billable Code | View Code |
| E71.318 | Other disorders of fatty-acid oxidation | Billable Code | View Code |
| E71.32 | Disorders of ketone metabolism | Billable Code | View Code |
| E71.39 | Other disorders of fatty-acid metabolism | Billable Code | View Code |
| E71.40 | Disorder of carnitine metabolism, unspecified | Billable Code | View Code |
| E71.41 | Primary carnitine deficiency | Billable Code | View Code |
| E71.42 | Carnitine deficiency due to inborn errors of metabolism | Billable Code | View Code |
| E71.43 | Iatrogenic carnitine deficiency | Billable Code | View Code |
| E71.440 | Ruvalcaba-Myhre-Smith syndrome | Billable Code | View Code |
| E71.448 | Other secondary carnitine deficiency | Billable Code | View Code |
| E71.50 | Peroxisomal disorder, unspecified | Billable Code | View Code |
| E71.510 | Zellweger syndrome | Billable Code | View Code |
| E71.511 | Neonatal adrenoleukodystrophy | Billable Code | View Code |
| E71.518 | Other disorders of peroxisome biogenesis | Billable Code | View Code |
| E71.520 | Childhood cerebral X-linked adrenoleukodystrophy | Billable Code | View Code |
| E71.521 | Adolescent X-linked adrenoleukodystrophy | Billable Code | View Code |
| E71.522 | Adrenomyeloneuropathy | Billable Code | View Code |
| E71.528 | Other X-linked adrenoleukodystrophy | Billable Code | View Code |
| E71.529 | X-linked adrenoleukodystrophy, unspecified type | Billable Code | View Code |
| E71.53 | Other group 2 peroxisomal disorders | Billable Code | View Code |
| E71.540 | Rhizomelic chondrodysplasia punctata | Billable Code | View Code |
| E71.541 | Zellweger-like syndrome | Billable Code | View Code |
| E71.542 | Other group 3 peroxisomal disorders | Billable Code | View Code |
| E71.548 | Other peroxisomal disorders | Billable Code | View Code |
| E71 | Disord of branched-chain amino-acid metab & fatty-acid metab | Non-Billable | View Code |
| E71.1 | Other disorders of branched-chain amino-acid metabolism | Non-Billable | View Code |
| E71.11 | Branched-chain organic acidurias | Non-Billable | View Code |
| E71.12 | Disorders of propionate metabolism | Non-Billable | View Code |
| E71.3 | Disorders of fatty-acid metabolism | Non-Billable | View Code |
| E71.31 | Disorders of fatty-acid oxidation | Non-Billable | View Code |
| E71.4 | Disorders of carnitine metabolism | Non-Billable | View Code |
| E71.44 | Other secondary carnitine deficiency | Non-Billable | View Code |
| E71.5 | Peroxisomal disorders | Non-Billable | View Code |
| E71.51 | Disorders of peroxisome biogenesis | Non-Billable | View Code |
| E71.52 | X-linked adrenoleukodystrophy | Non-Billable | View Code |
| E71.54 | Other peroxisomal disorders | Non-Billable | View Code |