1. ICD-10-CM Codes
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2. E00-E89 Endocrine, nutritional and metabolic diseases
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3. E70-E88 Metabolic disorders
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4. Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism E71

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

• E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
  • E71.0 Maple-syrup-urine disease
  • E71.1 Other disorders of branched-chain amino-acid metabolism
    • E71.11 Branched-chain organic acidurias
      • E71.110 Isovaleric acidemia
      • E71.111 3-methylglutaconic aciduria
      • E71.118 Other branched-chain organic acidurias
    • E71.12 Disorders of propionate metabolism
      • E71.120 Methylmalonic acidemia
      • E71.121 Propionic acidemia
      • E71.128 Other disorders of propionate metabolism
    • E71.19 Other disorders of branched-chain amino-acid metabolism
  • E71.2 Disorder of branched-chain amino-acid metabolism, unspecified
  • E71.3 Disorders of fatty-acid metabolism
    • E71.30 Disorder of fatty-acid metabolism, unspecified
    • E71.31 Disorders of fatty-acid oxidation
      • E71.310 Long chain/very long chain acyl CoA dehydrogenase deficiency
      • E71.311 Medium chain acyl CoA dehydrogenase deficiency
      • E71.312 Short chain acyl CoA dehydrogenase deficiency
      • E71.313 Glutaric aciduria type II
      • E71.314 Muscle carnitine palmitoyltransferase deficiency
      • E71.318 Other disorders of fatty-acid oxidation
    • E71.32 Disorders of ketone metabolism
    • E71.39 Other disorders of fatty-acid metabolism
  • E71.4 Disorders of carnitine metabolism
    • E71.40 Disorder of carnitine metabolism, unspecified
    • E71.41 Primary carnitine deficiency
    • E71.42 Carnitine deficiency due to inborn errors of metabolism
    • E71.43 Iatrogenic carnitine deficiency
    • E71.44 Other secondary carnitine deficiency
      • E71.440 Ruvalcaba-Myhre-Smith syndrome
      • E71.448 Other secondary carnitine deficiency
  • E71.5 Peroxisomal disorders
    • E71.50 Peroxisomal disorder, unspecified
    • E71.51 Disorders of peroxisome biogenesis
      • E71.510 Zellweger syndrome
      • E71.511 Neonatal adrenoleukodystrophy
      • E71.518 Other disorders of peroxisome biogenesis
    • E71.52 X-linked adrenoleukodystrophy
      • E71.520 Childhood cerebral X-linked adrenoleukodystrophy
      • E71.521 Adolescent X-linked adrenoleukodystrophy
      • E71.522 Adrenomyeloneuropathy
      • E71.528 Other X-linked adrenoleukodystrophy
      • E71.529 …… unspecified type
    • E71.53 Other group 2 peroxisomal disorders
    • E71.54 Other peroxisomal disorders
      • E71.540 Rhizomelic chondrodysplasia punctata
      • E71.541 Zellweger-like syndrome
      • E71.542 Other group 3 peroxisomal disorders
      • E71.548 Other peroxisomal disorders