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Síndrome de Dubin-Johnson

最后审阅: 20 Aug 2025
最后更新: 24 Aug 2023

小结

定义

病史和体格检查

关键诊断因素

  • presencia de factores de riesgo
  • ictericia intermitente
  • falta de prurito
完整详情

其他诊断因素

  • enfermedad, infección o estrés
  • ictericia desencadenada por el embarazo
  • ictericia desencadenada por un medicamento
  • dolor abdominal
  • fatiga
  • hepatomegalia
完整详情

危险因素

  • antecedentes familiares
  • 10 a 30 años de edad
  • Judíos iraníes y judíos marroquíes
  • sexo masculino
完整详情

诊断性检查

首要检查

  • 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
完整详情

需考虑的检查

  • 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
完整详情

新兴检查

  • metabolitos de los leucotrienos en orina

治疗流程

急症处理

todos los pacientes

撰稿人

作者

Anil Dhawan, MBBS, MD, FRCPCH
Anil Dhawan

Professor of Paediatric Hepatology

King's College Hospital

London

UK

Раскрытие информации

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

Раскрытие информации

TAFC declares that he has no competing interests.

Рецензенты

John T. Jenkins, MB, CHB, FRCP

Consultant Surgeon

St. Mark's Hospital

London

UK

Раскрытие информации

JTJ declares that he has no competing interests.

Nancy Reau, MD

Assistant Professor of Medicine

University of Chicago

Center for Liver Disease

Chicago

IL

Раскрытие информации

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.

Список литературы

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Основные статьи

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. Аннотация

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

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

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. Аннотация

Статьи, указанные как источники

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