Last reviewed: 4 Jul 2021
Last updated: 10 Dec 2019

Summary

Definition

History and exam

Risk factors

  • glucose-6-phosphate dehydrogenase deficiency
  • beta-thalassaemia

Diagnostic investigations

1st investigations to order

  • serum total bilirubin
  • serum conjugated bilirubin
  • urinary bilirubin
  • serum aminotransferases, alkaline phosphatase, and gamma-GT
  • haemoglobin
  • absolute reticulocyte count
  • blood smear
  • plasma haptoglobin

Investigations 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

Contributors

Authors

Tracy A.F. Coelho, MBBS, DCH, MRCPCH
Tracy A.F. Coelho

Consultant Paediatric Gastroenterologist

University Hospital Southampton

Southampton

UK

Disclosures

TAFC declares that he has no competing interests.

Anil Dhawan, MBBS, MD, FRCPCH
Anil Dhawan

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.

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