questions 2

Dubin-Johnson / Rotor's Syndrome

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Topic updated on 05/29/17 8:08pm

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


  RATE CONTENT
2.0
AVERAGE 2.0 of 3 RATINGS

Qbank (1 Questions)

TAG
(M1.GI.24) A 14-year-old boy who recently immigrated to the US is brought to the pediatrician by his mother because she has noticed a recent color change in his eyes as demonstrated in Figure A. She is concerned because he has never had significant medical care in his life. Otherwise the boy has no complaints and states that he feels fine. The pediatrician obtains labs which demonstrate: total bilirubin: 5 mg/dL, direct bilirubin 4.5 mg/dL, indirect bilirubin 0.5 mg/dL, AST: 15, ALT: 20, GGT: 10. A liver biopsy is subsequently obtained which is demonstrated in Figure B. Which of the following is the most likely diagnosis for this patient? Topic Review Topic
FIGURES: A   B        

1. Dubin-Johnson syndrome
2. Gilbert's syndrome
3. Rotor syndrome
4. Crigler–Najjar syndrome
5. Hepatitis C

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