Summary
Definition
History and exam
Key diagnostic factors
- presencia de factores de riesgo
- ictericia intermitente
- falta de prurito
Other diagnostic factors
- enfermedad, infección o estrés
- ictericia desencadenada por el embarazo
- ictericia desencadenada por un medicamento
- dolor abdominal
- fatiga
- hepatomegalia
Risk factors
- antecedentes familiares
- 10 a 30 años de edad
- Judíos iraníes y judíos marroquíes
- sexo masculino
Diagnostic investigations
1st investigations to order
- bilirrubina sérica
- fosfatasa alcalina en suero
- aminotransferasas hepáticas séricas conjugadas (aspartato aminotransferasa y alanina aminotransferasa)
- albúmina sérica
- gamma glutamil transferasa sérica
- ácidos biliares séricos
- perfil de coagulación
Investigations to consider
- proporción entre coproporfirina tipo I y III en orina
- exploración con ácido iminodiacético hepatobiliar (HIDA) marcado con 99mTc (gammagrafía biliar)
- biopsia hepática
- análisis mutacional del gen ABCC2
Emerging tests
- metabolitos de los leucotrienos en orina
Treatment algorithm
todos los pacientes
Contributors
Authors
Anil Dhawan, MBBS, MD, FRCPCH
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
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
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.
Differentials
- Síndrome de Rotor
- Síndrome de Gilbert
- Síndrome de Crigler-Najjar (tipo I y II)
More DifferentialsGuidelines
- ACR appropriateness criteria®: abnormal liver function tests
More Guidelines
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