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Rotor syndrome

Last reviewed: 1 May 2025
Last updated: 10 Dec 2024

Summary

Definition

History and exam

Key diagnostic factors

  • jaundice
  • dark-colored urine
Full details

Other diagnostic factors

  • fatigue
  • abdominal pain
Full details

Risk factors

  • glucose-6-phosphate dehydrogenase deficiency
  • beta-thalassemia
Full details

Diagnostic investigations

1st investigations 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
Full details

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
Full details

Treatment algorithm

ONGOING

all patients

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

Divulgaciones

AD declares that he has no competing interests.

Revisores por pares

John T. Jenkins, MB, CHB, FRCP

Consultant Surgeon

St. Mark's Hospital

London

UK

Divulgaciones

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

Divulgaciones

WAP declares that he has no competing interests.

Nancy Reau, MD

Assistant Professor of Medicine

University of Chicago

Center for Liver Disease

Chicago

IL

Divulgaciones

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

Divulgaciones

JN declares that he has no competing interests.

Referencias

Nuestros equipos internos de evidencia y editoriales colaboran con colaboradores expertos internacionales y revisores pares para garantizar que brindemos acceso a la información más clínicamente relevante posible.

Artículos principales

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.Texto completo  Resumen

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

Artículos de referencia

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