[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [Conditions](https://durgical.com/en/conditions/) › Rotor syndrome

[Conditions](https://durgical.com/en/conditions/) · [Metabolism: heme, bilirubin and iron](https://durgical.com/en/conditions/)

# Rotor syndrome

A benign inherited condition with mild conjugated jaundice, because the liver poorly takes bilirubin back up from the blood.

Updated 2026-11-01

## Structures involved

Tap one to see what it is and open it in 3D.

- [Liver](https://durgical.com/en/anatomy/liver/)
- [Gallbladder](https://durgical.com/en/anatomy/gallbladder/)

## In biochemistry

- [Bilirubin](https://durgical.com/en/biochemistry/molecules/bilirubin/)

## What it is

Mild conjugated and unconjugated hyperbilirubinemia from combined absence of the OATP1B1 and OATP1B3 transporters (SLCO1B1 and SLCO1B3 genes), without liver pigment.

## Who gets it

Very rare; its prevalence is unknown. It appears in childhood or adolescence.

## Causes

- Digenic autosomal recessive inheritance: variants in both SLCO1B1 and SLCO1B3

## Risk factors

- Family history

## Pathophysiology

Some conjugated bilirubin leaves the hepatocyte into the blood and neighbouring hepatocytes take it back up via OATP1B1/1B3. Without these transporters conjugated bilirubin stays in the blood. The same transporters take up statins and methotrexate, whose toxicity may increase.

## Symptoms and signs

- Mild jaundice, sometimes only in the sclerae
- Usually no other symptoms

## Red flags

- Abnormal liver or cholestatic enzymes (look for another cause)

## Diagnosis

**Clinical**
Mild persistent jaundice, mainly conjugated, with no other [liver](https://durgical.com/en/anatomy/liver/) disease.

**Tests**
Raised total bilirubin with a large direct fraction, normal transaminases and alkaline phosphatase; high total urinary coproporphyrins (2–5 times) with isomer I around 65 %; genetic testing.

**Imaging**
Hepatobiliary scintigraphy shows poor liver uptake; the [gallbladder](https://durgical.com/en/anatomy/gallbladder/) does fill. Ultrasound is normal.

## Treatment

**Conservative**
No treatment is needed

**Follow-up**
Caution with drugs that rely on OATP1B1/1B3 (statins, methotrexate)

## Complications

- Greater toxicity of some drugs

## Prognosis

Excellent.

## Test yourself

Think of the answer, then tap the question to see it.

## Which transporters are missing in Rotor syndrome?

OATP1B1 and OATP1B3, which take bilirubin back up into the liver.

## Does the liver have black pigment in Rotor syndrome?

No; dark pigment is characteristic of Dubin-Johnson.

## Which drugs can be more toxic in Rotor syndrome?

Those the liver takes up through OATP1B1/1B3, such as statins and methotrexate.

[Add to my cards (3)](https://durgical.app/estudiar/?add=/en/conditions/rotor-syndrome/)

## Related conditions

- [Dubin-Johnson syndrome](https://durgical.com/en/conditions/dubin-johnson-syndrome/)

Educational information, not medical advice. For any symptom, see a health professional.

Sources: [Rotor syndrome: MedlinePlus Genetics](https://medlineplus.gov/genetics/condition/rotor-syndrome/) (MedlinePlus, US National Library of Medicine, 2026); [SLCO1B1 gene: MedlinePlus Genetics](https://medlineplus.gov/genetics/gene/slco1b1/) (MedlinePlus, US National Library of Medicine, 2026); [Bilirubin blood test: MedlinePlus Medical Encyclopedia](https://medlineplus.gov/ency/article/003479.htm) (MedlinePlus, US National Library of Medicine, 2026).

[← All conditions](https://durgical.com/en/conditions/)

## **Spotted a mistake?** We are always improving for the community. Tell us what is wrong and our team will review it. Report a mistake

Representative model, for educational purposes only.

---
Source: https://durgical.com/en/conditions/rotor-syndrome/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/conditions/rotor-syndrome/