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