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Rotor syndrome is a rare, benign autosomal recessive disorder of unknown origin.
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.
|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)|
Differentiating Rotor syndrome from Other Diseases
Epidemiology and Demographics
Natural History, Complications, and Prognosis
History and Symptoms
Other Diagnostic Studies
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.
Rotor syndrome is named after the Filipino internist, Arturo Belleza Rotor (1907-1988).
Template:Endocrine, nutritional and metabolic pathology Template:Heme metabolism disorders