Dubin-Johnson Syndrome Differential Diagnoses

Updated: Dec 15, 2022
  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: BS Anand, MD  more...
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Diagnostic Considerations

Consider Dubin-Johnson syndrome (DJS) when other common causes of conjugated hyperbilirubinemia have been ruled out, and the concentration of direct bilirubin is elevated in relation to their total bilirubin concentration. [39]

Initially, patients with jaundice must be determined to have directly reacting hyperbilirubinemia, other causes of which, aside from Dubin-Johnson syndrome, include the following:

  • Rotor syndrome: This condition, especially with normal liver transaminase levels and normal family history, may be the cause of the jaundice. Impaired bilirubin (re)uptake occurs as a result of deficiency of two basolateral/sinusoidal hepatocellular membrane proteins. [9]

  • Obstructive cholestasis with stones in the cystic duct: These can demonstrate hepatic uptake but delayed or no visualization of the gallbladder on nuclear medicine studies

Virtually all hepatic diseases that cause directly reacting hyperbilirubinemia, including the following, are accompanied by elevated transaminase levels as well:

  • Viral hepatitis

  • Autoimmune hepatitis

  • Alcoholism and other hepatotoxic exposure

  • Sepsis

  • Hepatic storage diseases

  • Infiltrative diseases such as hepatoblastoma

  • Hepatocellular carcinoma

  • Metastatic liver disease