Table 1.

Laboratory Findings in Rotor Syndrome

FindingRotor SyndromeNormal
Serum bilirubinTotal 2-5 mg/dL 10.3-1.0 mg/dL 2
Conjugated: total>50%<20%
UrineBilirubinPresentNot detected
Coproporphyrins↑ 2.5-5x normal 3
Disappearance of plasma anionic compounds 4DelayedRapid
CholescintigraphySee footnote 5Normal
HistologyNormal 6Normal
Protein expression Absence of OATP1B1 and OATP1B3 7Normal

Rarely may be up to 5-10 mg/dL [Author personal observation] or up to 20 mg/dL [Chowdhury et al 2001]


For total and direct bilirubin in persons over age one year. Note: Although normal levels of total and direct bilirubin may be higher in the neonatal period and infancy, Rotor syndrome is not usually diagnosed in this age group.


Coproporphyrinuria is frequently observed in those with parenchymal liver diseases, and thus is not specific to Rotor syndrome.


Includes bromosulfophthalein and indocyanin green


Radiotracers (99mTc-HIDA/99mTc-N [2,6-dimethylphenyl-carbamoylmethyl] iminodiacetic acid, 99mTc-DISIDA/disofenin, 99mTc- BrIDA/mebrofenin) are taken up slowly by the liver and the liver is scarcely visualized; however, there is persistent visualization of the cardiac blood pool and prominent excretion by the kidneys.


Note that suspicion of hereditary jaundice is not an indication for liver biopsy.


Immunohistologic staining does not detect OATP1B1 and OATP1B3 at the sinusoidal membrane of hepatocytes. Note: Expression of MRP2, frequently absent in Dubin-Johnson syndrome (see Differential Diagnosis), is normal [Hrebícek et al 2007].

From: Rotor Syndrome

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