Unconjugated Hyperbilirubinemia Workup

  • Author: Sandeep Mukherjee, MB, BCh, MPH, FRCPC; Chief Editor: Julian Katz, MD   more...
 
Updated: Jan 5, 2012
 

Laboratory Studies

  • Increased production of bilirubin, ineffective erythropoiesis (ELB production) - Marked increase in fecal urobilinogen excretion and a normal or near-normal red blood cell life span
  • Impaired conjugation of bilirubin
    • Crigler-Najjar syndrome type I: Results of liver tests, except high serum unconjugated bilirubin levels, are normal. Serum bilirubin levels range from 20-50 mg/dL. Conjugated bilirubin is absent from serum, and bilirubin is not present in urine.
    • Crigler-Najjar syndrome type II (Arias syndrome): Serum bilirubin levels range from 7-20 mg/dL. This disorder may be distinguished definitively from type I by chromatographic analysis of pigments excreted in bile. In type II, bile contains significant amounts of conjugated bilirubin, although the proportion of bilirubin monoglucuronide in bile is increased.
    • Gilbert syndrome: Hyperbilirubinemia is the only serum biochemical abnormality. Serum bilirubin concentrations range from 1-5 mg/dL. Two provocative tests, energy deprivation and nicotinic acid administration, have been used. However, a significant number of false-positive and false-negative results limit the value of these tests in patients with marginal elevation of serum bilirubin concentration. A polymerase chain reaction assay has also been introduced to identify TA repeats and may be used as a screening test.
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Imaging Studies

  • Impaired conjugation of bilirubin, Crigler-Najjar syndrome type I: The gallbladder is visualized using oral cholecystography despite very high serum bilirubin levels because excretion of nonglucuronidated organic anions is normal.
  • Japanese researchers have reported that patients with schizophrenia associated with Gilbert syndrome have specific changes of signal intensities on fluid-attenuated inversion-recovery magnetic resonance images. This suggests that schizophrenia with associated Gilbert syndrome may produce changes in the fronto-temporal cortex, limbic system, and basal ganglia.
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Other Tests

  • Impaired conjugation of bilirubin, Crigler-Najjar syndrome type I: Bile collected through duodenal aspiration is light yellow because of small amounts of unconjugated bilirubin. Bilirubin conjugates are nearly absent from the bile.
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Histologic Findings

In impaired conjugation of bilirubin, Crigler-Najjar syndrome type I, liver histology findings are normal.

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Contributor Information and Disclosures
Author

Sandeep Mukherjee, MB, BCh, MPH, FRCPC  Associate Professor, Department of Internal Medicine, Section of Gastroenterology and Hepatology, University of Nebraska Medical Center; Consulting Staff, Section of Gastroenterology and Hepatology, Veteran Affairs Medical Center

Sandeep Mukherjee, MB, BCh, MPH, FRCPC is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Merck Honoraria Speaking and teaching; Ikaria Pharmaceuticals Honoraria Board membership

Coauthor(s)

Nuri Ozden, MD  Assistant Professor, Department of Internal Medicine, Meharry Medical College

Nuri Ozden, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Specialty Editor Board

Ann Ouyang, MBBS  Professor, Department of Internal Medicine, Pennsylvania State University College of Medicine; Attending Physician, Division of Gastroenterology and Hepatology, Milton S Hershey Medical Center

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Oscar S Brann, MD, FACP  Associate Clinical Professor, Department of Medicine, University of California at San Diego; Consulting Staff, Mecklenburg Medical Group

Oscar S Brann, MD, FACP is a member of the following medical societies: American Gastroenterological Association

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

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Production of bilirubin.
Enterohepatic circulation of bilirubin.
Conjugation of bilirubin.
 
 
 
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