Rotor syndrome

Rotor syndrome is an autosomal recessive disorder which results in a conjugated hyperbilinaemia. It is very similar to Dubin Johnson syndrome.

The differences between Rotor syndrome and Dubin Johnson are: 
  • Liver in Dubin Johnson syndrome has black pigmentation while liver in Rotor syndrome does not
  • Dubin-Johnson has nearly 90% of its urinary coproporphyrin excreted as coproporphyrin I while Rotor syndrome has around 60% excreted as coproporphyrin I 
  • Sulfobromophthalein excretion is also different between the 2 but due to reports of fatal anaphylactic reactions to sulfobromophthalein this test is no longer advised. 


Summary of hereditary hyperbilinaemias:
Gilbert syndrome: unconjugated hyperbilinaemia
Crigler-Najjar: unconjugated hyperbilinaemia
Dubin Johnson syndrome: conjugated hyperbilinaemia
Rotor syndrome: conjugated hyperbilinaemia

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