E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

Codes

• 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

    • E71.120Methylmalonic acidemia
    • E71.121Propionic acidemia
    • E71.128Other disorders 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

  • E71.40Disorder of carnitine metabolism, unspecified
  • E71.41Primary carnitine deficiency
  • E71.42Carnitine deficiency due to inborn errors of metabolism
  • 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