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Síndrome de Dubin-Johnson

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  • Overview
  • Theory
  • Diagnosis
  • Management
  • Follow up
  • Resources
Evidence last reviewed: 15 Mar 2026
Topic last updated: 24 Aug 2023

Summary

Definition

History and exam

Key diagnostic factors

  • presença de fatores de risco
  • icterícia intermitente
  • ausência de prurido
Full details

Other diagnostic factors

  • doença, infecção ou estresse
  • icterícia desencadeada pela gravidez
  • icterícia desencadeada por medicamentos
  • dor abdominal
  • fadiga
  • hepatomegalia
Full details

Risk factors

  • história familiar
  • idade entre 10 e 30 anos
  • etnia judaica iraniana ou judaica marroquina
  • sexo masculino
Full details

Diagnostic investigations

1st investigations to order

  • bilirrubina sérica
  • fosfatase alcalina sérica
  • aminotransferases hepáticas séricas (aspartato aminotransferase e alanina aminotransferase)
  • albumina sérica
  • gama-glutamiltransferase sérica
  • ácidos biliares séricos
  • coagulograma
Full details

Investigations to consider

  • razão entre coproporfirina urinária I e III
  • cintilografia hepatobiliar com ácido hidroxi-iminodiacético (HIDA) marcado com 99mTc (colecintigrafia)
  • biópsia hepática
  • análise de mutação no gene ABCC2
Full details

Emerging tests

  • metabólitos urinários de leucotrieno

Treatment algorithm

ACUTE

todos os pacientes

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.

Peer reviewer acknowledgements

BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.

Disclosures

Peer reviewer affiliations and disclosures pertain to the time of the review.

References

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

Key articles

Dubin IN, Johnson FB. Chronic idiopathic jaundice with unidentified pigment in the liver cells: a new clinicopathologic entity with report of 12 cases. Medicine. 1954 Sep;33(3):155-97. Abstract

Dubin IN. Chronic idiopathic jaundice: a review of 50 cases. Am J Med. 1958 Feb;24(2):268-92. Abstract

Kwo PY, Cohen SM, Lim JK. ACG clinical guideline: evaluation of abnormal liver chemistries. Am J Gastroenterol. 2017 Jan;112(1):18-35. Abstract

Machida I, Wakusawa S, Sanae F, et al. Mutational analysis of the MRP2 gene and long-term follow-up of Dubin-Johnson syndrome in Japan. J Gastroenterol. 2005 Apr;40(4):366-70. Abstract

Reference articles

A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.

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