E71

Non-Billable

Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

E71 should not be used for reimbursement purposes as there are multiple sub-diagnoses that fall under this code. Please refer to the sub-diagnoses below.

Related Diagnoses

  • E71.0Maple-syrup-urine disease
  • E71.1Other disorders of branched-chain amino-acid metabolism
    • E71.11Branched-chain organic acidurias
      • E71.110Isovaleric acidemia
      • E71.1113-methylglutaconic aciduria
      • E71.118Other branched-chain organic acidurias
    • E71.12Disorders of propionate metabolism
    • E71.19Other disorders of branched-chain amino-acid metabolism
  • E71.2Disorder of branched-chain amino-acid metabolism, unspecified
  • E71.3Disorders of fatty-acid metabolism
    • E71.30Disorder of fatty-acid metabolism, unspecified
    • E71.31Disorders of fatty-acid oxidation
      • E71.310Long chain/very long chain acyl CoA dehydrogenase deficiency
      • E71.311Medium chain acyl CoA dehydrogenase deficiency
      • E71.312Short chain acyl CoA dehydrogenase deficiency
      • E71.313Glutaric aciduria type II
      • E71.314Muscle carnitine palmitoyltransferase deficiency
      • E71.318Other disorders of fatty-acid oxidation
    • E71.32Disorders of ketone metabolism
    • E71.39Other disorders of fatty-acid metabolism
  • E71.4Disorders of carnitine metabolism
    • E71.41Primary carnitine deficiency
    • E71.42Carnitine deficiency due to inborn errors of metabolism
    • E71.40Disorder of carnitine metabolism, unspecified
    • E71.43Iatrogenic carnitine deficiency
    • E71.44Other secondary carnitine deficiency
      • E71.440Ruvalcaba-Myhre-Smith syndrome
      • E71.448Other secondary carnitine deficiency
  • E71.5Peroxisomal disorders
    • E71.50Peroxisomal disorder, unspecified
    • E71.51Disorders of peroxisome biogenesis
      • E71.510Zellweger syndrome
      • E71.511Neonatal adrenoleukodystrophy
      • E71.518Other disorders of peroxisome biogenesis
    • E71.52X-linked adrenoleukodystrophy
      • E71.520Childhood cerebral X-linked adrenoleukodystrophy
      • E71.521Adolescent X-linked adrenoleukodystrophy
      • E71.522Adrenomyeloneuropathy
      • E71.528Other X-linked adrenoleukodystrophy
      • E71.529X-linked adrenoleukodystrophy, unspecified type
    • E71.53Other group 2 peroxisomal disorders
    • E71.54Other peroxisomal disorders
      • E71.540Rhizomelic chondrodysplasia punctata
      • E71.541Zellweger-like syndrome
      • E71.542Other group 3 peroxisomal disorders
      • E71.548Other peroxisomal disorders
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