Excludes1

  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • Ehlers-Danlos syndrome (Q79.6-)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan's syndrome (Q87.4)
  • 5-alpha-reductase deficiency (E29.1)
  1. E80 Disorders of porphyrin and bilirubin metabolism

    Includes:

    • defects of catalase and peroxidase
    1. E80.0 Hereditary erythropoietic porphyria

      Inclusion term(s):

      • Congenital erythropoietic porphyria
      • Erythropoietic protoporphyria
    2. E80.1 Porphyria cutanea tarda

    3. E80.2 Other and unspecified porphyria

      1. E80.20 Unspecified porphyria

        Inclusion term(s):

        • Porphyria NOS
      2. E80.21 Acute intermittent (hepatic) porphyria

      3. E80.29 Other porphyria

        Inclusion term(s):

        • Hereditary coproporphyria
    4. E80.3 Defects of catalase and peroxidase

      Inclusion term(s):

      • Acatalasia [Takahara]
    5. E80.4 Gilbert syndrome

    6. E80.5 Crigler-Najjar syndrome

    7. E80.6 Other disorders of bilirubin metabolism

      Inclusion term(s):

      • Dubin-Johnson syndrome
      • Rotor's syndrome
    8. E80.7 Disorder of bilirubin metabolism, unspecified

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.