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ICD-10-CM Codes E00-E89 E70-E88 E71

ICD-10-CM Diagnosis Code E71

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

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Codes Hierarchy:

    • 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  X-linked adrenoleukodystrophy, 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