Rotor’s Syndrome

Sample for Rotor’s Syndrome

1. The serum of the patient is needed.
2. A urine sample can be used.

Precautions for Rotor’s Syndrome

1. Avoid the hemolyzed sample.
2. Avoid lipemic serum.
3. Avoid serum from the light.
4. Avoid urine from exposure to light.

Pathophysiology of Rotor’s Syndrome

1. This is a rare, relatively benign (mild) autosomal recessive disease of the liver.
2. This is similar to Dubin-Johnson syndrome, and both lead to increase conjugated (direct) bilirubin.

3. There is an impairment in the excretion of bilirubin from hepatocytes into the canalicular bile lumen.

4. This causes defective:
   1. Excretion of conjugated bilirubin.
   2. It’s reabsorption into the blood.
   3. Excretion in the urine.
5. The difference between Rotor’s syndrome from the Dubin-Johnson syndrome is:
   1. There is no hepatic pigmentation.
   2. Oral Cholecystography also differentiates, which is normal in this condition.
   3. Ultrasound shows no abnormality.
   4. Liver biopsy shows no pigmentation.

Signs and symptoms of Rotor’s Syndrome

1. This disease is usually seen in infancy or childhood.
2. There is chronic jaundice without any evidence of hemolysis.
3. Sometimes these patients may have intermittent epigastric discomfort and abdominal pain.
4. There may be an attack of fever.

Diagnosis of Rotor’s Syndrome

1. There is jaundice with increased conjugated bilirubinemia.
2. Liver biopsy is normal in histology.
3. The gallbladder can be visualized by cholecystography. This differentiates it from Dubin-Johnson syndrome.

Treatment of Rotor’s Syndrome

• This is a benign disease, and no active intervention is needed unless another cause of liver disease exists.

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