Summary
Definition
History and exam
Key diagnostic factors
- jaundice
- dark-colored urine
Other diagnostic factors
- fatigue
- abdominal pain
Risk factors
- glucose-6-phosphate dehydrogenase deficiency
- beta-thalassemia
Diagnostic tests
1st tests to order
- serum total bilirubin
- serum conjugated bilirubin
- urinary bilirubin
- serum aminotransferases, alkaline phosphatase, and gamma-GT
- hemoglobin
- absolute reticulocyte count
- blood smear
- plasma haptoglobin
Tests to consider
- serum bile acids (total)
- urinary coproporphyrins
- liver biopsy
- ultrasound of the liver and biliary tree
- oral cholecystogram
- cholescintigraphy with 99mTc-HIDA
- molecular genetic testing
- sulphobromophthalein (BSP) plasma retention test
Treatment algorithm
all patients
Contributors
Authors
Tracy A.F. Coelho, MBBS, DCH, MRCPCH
Consultant Paediatric Gastroenterologist
University Hospital Southampton
Southampton
UK
Disclosures
TAFC declares that he has no competing interests.
Anil Dhawan, MBBS, MD, FRCPCH
Professor of Paediatric Hepatology
King's College Hospital
London
UK
Disclosures
AD 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.
William A. Petri, Jr., MD, PhD, FACP
Chief and Professor of Medicine
Division of Infectious Diseases and International Health
University of Virginia Health System
Charlottesville
VA
Disclosures
WAP 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.
James Neuberger, BM, BCh
Consultant Physician
Liver Unit
Queen Elizabeth Hospital
Birmingham
UK
Disclosures
JN declares that he has no competing interests.
References
Key articles
Rotor AB, Manahan L, Florentin A. Familial non-hemolytic jaundice with direct Van Den Bergh reaction. Acta Med Phil. 1948;5:37-49.
Vest MF, Kaufmann HJ, Fritz E. Chronic non-haemolytic jaundice with conjugated bilirubin in the serum and normal histology: a case study. Arch Dis Child. 1960;36:600-4.Full text Abstract
Kwo PY, Cohen SM, Lim JK. ACG clinical guideline: evaluation of abnormal liver chemistries. Am J Gastroenterol. 2017 Jan;112(1):18-35.Full text 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
- Dubin-Johnson syndrome
- Gilbert syndrome
- Crigler-Najjar syndrome (type I and II)
More DifferentialsGuidelines
- ACG clinical guideline: evaluation of abnormal liver chemistries
More Guidelines
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