5C58.02

ICD-11 MMS 2026-01

Dubin-Johnson syndrome

Description

Dubin-Johnson syndrome (DJS) is a benign, inherited liver disorder characterised clinically by chronic, predominantly conjugated, hyperbilirubinemia and histopathologically by black-brown pigment deposition in parenchymal liver cells.

Index terms

  • Dubin-Johnson syndrome
  • DJS - [Dubin-Johnson syndrome]
  • Dubin-Sprinz disease
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  • Hyperbilirubinaemia type 2
  • Sprinz-Nelson syndrome

Exclusions from above levels

  • http://id.who.int/icd/entity/1191191920 – Disorders of lipoprotein metabolism or certain specified lipidaemias
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 5A71.01 – Congenital adrenal hyperplasia
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  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5C58.00 – Crigler-Najjar syndrome
  • 5C58.01 – Gilbert syndrome
  • 5C58.03 – Progressive familial intrahepatic cholestasis
  • 5C58.04 – Benign recurrent intrahepatic cholestasis
  • 5C58.0Y – Other specified disorders of bilirubin metabolism or excretion
  • 5C58.0Z – Disorders of bilirubin metabolism or excretion, unspecified

Foundation entity

Dubin-Johnson syndrome – http://id.who.int/icd/entity/1691610999

Part of: 5C58.0: Disorders of bilirubin metabolism or excretion › 5C58: Inborn errors of porphyrin or heme metabolism › 05: Endocrine, nutritional or metabolic diseases