Dubin-Johnson syndrome
Resumo
Definição
História e exame físico
Principais fatores diagnósticos
- intermittent jaundice
- lack of pruritus
Outros fatores diagnósticos
- illness, infection, or stress
- pregnancy-triggered jaundice
- medication-triggered jaundice
- abdominal pain
- fatigue
- hepatomegaly
Fatores de risco
- family history
- age 10 to 30 years
- Iranian Jewish or Moroccan Jewish ethnicity
- male sex
Investigações diagnósticas
Primeiras investigações a serem solicitadas
- serum bilirubin
- serum alkaline phosphatase
- serum liver aminotransferases (aspartate aminotransferase and alanine aminotransferase)
- serum albumin
- serum gamma-glutamyl transferase
- serum bile acids
- clotting profile
Investigações a serem consideradas
- urinary coproporphyrin I to III ratio
- 99mTc hepatobiliary imino-diacetic acid (HIDA) scan (cholescintigraphy)
- liver biopsy
- mutational analysis of the ABCC2 gene
Novos exames
- urinary leukotriene metabolites
Algoritmo de tratamento
all patients
Colaboradores
Autores
Anil Dhawan, MBBS, MD, FRCPCH
Professor of Paediatric Hepatology
King's College Hospital
London
UK
Declarações
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
- Rotor syndrome
- Gilbert syndrome
- Crigler-Najjar syndrome (type I & II)
More DifferentialsGuidelines
- ACR appropriateness criteria®: abnormal liver function tests
More Guidelines
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