Orphanet: Dubin Johnson syndrome

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Dubin-Johnson syndrome

Disease definition

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


Classification level: Disorder
  • Synonym(s):
    • Dubin-Sprinz disease
    • Hyperbilirubinemia type 2
    • Sprinz-Nelson syndrome
  • Prevalence: Unknown
  • Inheritance: Autosomal recessive 
  • Age of onset: Adolescent, Adult
  • ICD-10: E80.6
  • OMIM: 237500
  • UMLS: C0022350
  • MeSH: D007566
  • GARD: 2793  6289
  • MedDRA: 10013800
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