Dubin-Johnson syndrome

Last reviewed: 2 Sep 2022
Last updated: 30 Aug 2022

Summary

Definition

History and exam

Key diagnostic factors

  • presence of risk factors
  • intermittent jaundice
  • lack of pruritus
More key diagnostic factors

Other diagnostic factors

  • illness, infection, or stress
  • pregnancy-triggered jaundice
  • medication-triggered jaundice
  • abdominal pain
  • fatigue
  • hepatomegaly
Other diagnostic factors

Risk factors

  • family history
  • age 10 to 30 years
  • Iranian Jewish or Moroccan Jewish ethnicity
  • male sex
More risk factors

Diagnostic investigations

1st investigations to order

  • serum bilirubin
  • serum alkaline phosphatase
  • serum liver aminotransferases (aspartate aminotransferase and alanine aminotransferase)
  • serum albumin
  • serum gamma-glutamyl transferase
  • serum bile acids
  • clotting profile
More 1st investigations to order

Investigations to consider

  • urinary coproporphyrin I to III ratio
  • 99mTc hepatobiliary imino-diacetic acid (HIDA) scan (cholescintigraphy)
  • liver biopsy
  • mutational analysis of the ABCC2 gene
More investigations to consider

Emerging tests

  • urinary leukotriene metabolites

Treatment algorithm

ACUTE

all patients

Contributors

Authors

Anil Dhawan, MBBS, MD, FRCPCH
Anil Dhawan

Professor of Paediatric Hepatology

King's College Hospital

London

UK

Disclosures

AD declares he has no competing interests.

Tracy A.F. Coelho, MBBS, DCH, MRCPCH, PhD
Tracy A.F. Coelho

Consultant Paediatric Gastroenterologist

University Hospital Southampton

Southampton

UK

Disclosures

TAFC declares that he has no competing interests.

Peer reviewers

John T. Jenkins, MB, CHB, FRCP

Consultant Surgeon

St. Mark's Hospital

London

UK

Disclosures

JTJ declares that he has no competing interests.

Nancy Reau, MD

Assistant Professor of Medicine

University of Chicago

Center for Liver Disease

Chicago

IL

Disclosures

NR has received reimbursement for speaking for Gilead, maker of Viread and Hepsera, and BMS, maker of Baraclude.

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