Chapter 4

E00–E89: Endocrine, Nutritional and Metabolic Diseases

Endocrine, Nutritional and Metabolic Diseases diagnostic classification updated for 2026.

Chapter Instructions

Excludes1(Not coded here. Use a different code.)
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
Internal Codes
  • E70.33

    Albinism with hematologic abnormality

  • E70.330

    Chediak-Higashi syndrome

  • E70.331

    Hermansky-Pudlak syndrome

  • E70.338

    Other albinism with hematologic abnormality

  • E70.339

    Albinism with hematologic abnormality, unspecified

  • E70.39

    Other specified albinism

  • E70.4

    Disorders of histidine metabolism

  • E70.40

    Disorders of histidine metabolism, unspecified

  • E70.41

    Histidinemia

  • E70.49

    Other disorders of histidine metabolism

  • E70.5

    Disorders of tryptophan metabolism

  • E70.8

    Other disorders of aromatic amino-acid metabolism

  • E70.81

    Aromatic L-amino acid decarboxylase deficiency

  • E70.89

    Other disorders of aromatic amino-acid metabolism

  • E70.9

    Disorder of aromatic amino-acid metabolism, unspecified

  • 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

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