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. 

References:

OMIM: Hyperbilirubinemia. Rotor type. Available at http://omim.org/entry/237450?search=rotor&highlight=rotor. 

Fretzayaz, A. et al. Cholescintigraphy in the diagnosis of Rotor syndrome. J Nucl Med. 1994; 35(6): 1048-1050. 

Summary of hereditary hyperbilinaemias:

Gilbert syndrome: unconjugated hyperbilinaemia

Crigler-Najjar: unconjugated hyperbilinaemia

Dubin Johnson syndrome: conjugated hyperbilinaemia

Rotor syndrome: conjugated hyperbilinaemia