Excludes1

  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)
  • 5-alpha-reductase deficiency (E29.1)

Excludes2

  • Ehlers-Danlos syndromes (Q79.6-)
  1. E88 Other and unspecified metabolic disorders

    Use additional codes for associated conditions

    Excludes1

    • histiocytosis X (chronic) (C96.6)
    1. E88.0 Disorders of plasma-protein metabolism, not elsewhere classified

      1. E88.01 Alpha-1-antitrypsin deficiency

        Inclusion term(s):

        • AAT deficiency
      2. E88.02 Plasminogen deficiency

        Inclusion term(s):

        • Dysplasminogenemia
        • Hypoplasminogenemia
        • Type 1 plasminogen deficiency
        • Type 2 plasminogen deficiency
      3. E88.09 Other disorders of plasma-protein metabolism, not elsewhere classified

        Inclusion term(s):

        • Bisalbuminemia
    2. E88.1 Lipodystrophy, not elsewhere classified

      Inclusion term(s):

      • Lipodystrophy NOS
    3. E88.2 Lipomatosis, not elsewhere classified

      Inclusion term(s):

      • Lipomatosis NOS
      • Lipomatosis (Check) dolorosa [Dercum]
    4. E88.3 Tumor lysis syndrome

      Inclusion term(s):

      • Tumor lysis syndrome (spontaneous)
      • Tumor lysis syndrome following antineoplastic drug chemotherapy
    5. E88.4 Mitochondrial metabolism disorders

      1. E88.40 Mitochondrial metabolism disorder, unspecified

      2. E88.41 MELAS syndrome

        Inclusion term(s):

        • Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes
      3. E88.42 MERRF syndrome

        Inclusion term(s):

        • Myoclonic epilepsy associated with ragged-red fibers
      4. E88.43 Disorders of mitochondrial tRNA synthetases

      5. E88.49 Other mitochondrial metabolism disorders

    6. E88.8 Other specified metabolic disorders

      1. E88.81 Metabolic syndrome and other insulin resistance

      2. E88.89 Other specified metabolic disorders

        Inclusion term(s):

        • Launois-Bensaude adenolipomatosis
    7. E88.9 Metabolic disorder, unspecified

    8. E88.A Wasting disease (syndrome) due to underlying condition

      Inclusion term(s):

      • Cachexia due to underlying condition
Abbreviations used here:

NEC Not elsewhere classifiable
This abbreviation in the Tabular List represents “other specified”. When a specific code is not available for a condition, the Tabular List includes an NEC entry under a code to identify the code as the “other specified” code.

NOS Not otherwise specified
This abbreviation is the equivalent of unspecified.