Resumo
Definition
History and exam
Key diagnostic factors
- presença de fatores de risco
- icterícia intermitente
- ausência de prurido
Other diagnostic factors
- doença, infecção ou estresse
- icterícia desencadeada pela gravidez
- icterícia desencadeada por medicamentos
- dor abdominal
- fadiga
- hepatomegalia
Risk factors
- história familiar
- idade entre 10 e 30 anos
- etnia judaica iraniana ou judaica marroquina
- sexo masculino
Diagnostic tests
1st tests to order
- bilirrubina sérica
- fosfatase alcalina sérica
- aminotransferases hepáticas séricas (aspartato aminotransferase e alanina aminotransferase)
- albumina sérica
- gama-glutamiltransferase sérica
- ácidos biliares séricos
- coagulograma
Tests 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
Emerging tests
- metabólitos urinários de leucotrieno
Treatment algorithm
todos os 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.
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 (tipos I e II)
More DifferentialsGuidelines
- ACR appropriateness criteria®: abnormal liver function tests
More Guidelines
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