Rotor syndrome

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Rotor syndrome
Bilirubin.png
Bilirubin
ICD-10 E80.6
ICD-9 277.4
OMIM 237450
DiseasesDB 11671
MeSH D006933

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


Rotor syndrome is a rare, benign autosomal recessive disorder of unknown origin.

Presentation

It has many things in common with Dubin-Johnson syndrome except that in Rotor Syndrome, the liver cells are not pigmented. The main symptom is a non-itching jaundice. There is a rise in bilirubin in the patient's serum, mainly of the conjugated type.

Rotor syndrome DJS
appearance of liver normal histology and appearance liver has black pigmentation
gallbladder visualization gallbladder can be visualized by oral cholecystogram gallbladder cannot be visualized
total urine coproporphyrin content high with <70% being isomer 1 normal with >80% being isomer 1 (normal urine contains more of isomer 3 than isomer 1)

Treatment

The liver is otherwise normal, and there is no need to treat this condition. It can be differentiated from Dubin Johnson syndrome by measuring the difference in urinary poryphrins. In Rotor syndrome there is an associated marked increase in urinary excretion of coproporyhrin I and III with < 80% being the I isomer.

Eponym

Rotor syndrome is named after the Filipino internist, Arturo Belleza Rotor (1907-1988).[1]

See also

References

  1. synd/2296 at Who Named It

External links

de:Rotor-Syndrom

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