Dubin-Johnson syndrome
Резюме
Определение
Анамнез и осмотр
Ключевые диагностические факторы
- intermittent jaundice
- lack of pruritus
Другие диагностические факторы
- illness, infection, or stress
- pregnancy-triggered jaundice
- medication-triggered jaundice
- abdominal pain
- fatigue
- hepatomegaly
Факторы риска
- family history
- age 10 to 30 years
- Iranian Jewish or Moroccan Jewish ethnicity
- male sex
Диагностические исследования
Исследования, которые показаны в первую очередь
- serum bilirubin
- serum alkaline phosphatase
- serum liver aminotransferases (aspartate aminotransferase and alanine aminotransferase)
- serum albumin
- serum gamma-glutamyl transferase
- serum bile acids
- clotting profile
Исследования, проведение которых нужно рассмотреть
- urinary coproporphyrin I to III ratio
- 99mTc hepatobiliary imino-diacetic acid (HIDA) scan (cholescintigraphy)
- liver biopsy
- mutational analysis of the ABCC2 gene
Неотложные исследования
- urinary leukotriene metabolites
Алгоритм лечения
all patients
Составители
Авторы
Anil Dhawan, MBBS, MD, FRCPCH
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
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
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
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Отличия
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