Rotor's syndrome

Alternative eponyms

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A rare idiopathic form of hyperbilirubinaemia.

Description

A rare idiopathic form of hyperbilirubinaemia affecting both sexes, with onset shortly after birth or in childhood. It is characterised by nonhaemolytic jaundice, attacks of intermittent epigastric discomfort and occasionally abdominal pain, and fever. Pathological findings include low-grade pigment deposition, dissociation of liver cells, occasional necrotic foci, and fibrin precipitation. Aetiology unknown. Possibly, autosomal recessive inheritance. It is due to a defect in the excretion of unconjugated bilirubin into the biliary craniculi with the bilirubin being absorbed into the blood and excreted in the urine. Primarily reported in patients from the Philippines.

Rotor’s syndrome is similar to the Dubin-Johnson syndrome, except that the gall bladder is usually visualised on an oral cholecystogram and there is no secondary appearance of the dye during the performance of bromsulphaphtalien. May be the same condition as hepatic storage disease reported in Japan and France.

Bibliography

  • A. B. Rotor, L. Manahan, Angel Florentin:
    Familial non-hemolytic jaundice with direct van den Bergh reaction.
    Acta medica Philippina, Manila, 1948, 5: 37-49.

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