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Snap Shot
  • A 22-year-old motorcycle accident victim with unknown past medical history is brought into the ED with severe head injuries. He is stabilized and brought to the surgical ICU where he is deemed to be brain dead by both the intensivist and neurosurgery staff. The organ transplant team is contacted and determine that he is an eligible kidney donor. However, he is not eligible for liver donation. Upon entering the abdomen during harvest, the team notices that his liver is black. 
Introduction
  • Hereditary conjugated hyperbilirubinemia 
    • ↓ hepatic excretion of conjugated bilirubin
  • Two types
    • Dubin-Johnson syndrome (DJS) 
      • grossly black liver due to impaired excretion of epinephrine metabolites
      • benign
      • autosomal recessive
    • Rotor's syndrome
      • even milder than Dubin-Johnson
      • does not cause black liver
Presentation
  • Symptoms
    • most patients asymptomatic
  • Physical exam
    • may become jaundiced or icteric during pregnancy or with oral contraceptives
Evaluation
  • Labs 
    • may show direct hyperbilirubinemia (usually 2-5 mg/dL) and resulting increased total bilirubin in DJS
    • coproporphyrin III:coproporphyrin I ratio 1:3-4 (opposite of this is normal)
    • total urine coproporphyrin levels
      • elevated in Rotor's
      • normal in DJS
  • Gross pathology
    • dark black liver in DJS
Treatment
  • Medical management
    • no treatment is needed
 

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