Rotor syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

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## Overview

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

Rotor syndrome

Bilirubin
ICD-10 | E80.6
ICD-9 | 277.4
OMIM | 237450
DiseasesDB | 11671
MeSH | D006933

### Presentation

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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)

## Historical Perspective

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## Classification

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## Pathophysiology

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## Causes

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## Differentiating Rotor syndrome from Other Diseases

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## Epidemiology and Demographics

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## Risk Factors

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## Screening

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## Natural History, Complications, and Prognosis

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### Natural History

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### Complications

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### Prognosis

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## Diagnosis

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### Diagnostic Criteria

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### History and Symptoms

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### Physical Examination

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### Laboratory Findings

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### Imaging Findings

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### Other Diagnostic Studies

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## Treatment

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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.

### Medical Therapy

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### Surgery

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### Prevention

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## Eponym

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

## See also

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* Jaundice
* Bilirubin metabolism
* Dubin-Johnson syndrome
* Gilbert syndrome
* Crigler-Najjar syndrome

## References

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1. ↑ Template:WhoNamedIt

## External links

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* med/1065 at eMedicine \- article on eMedicine on conjugated hyperbilirubinemia

Template:Endocrine, nutritional and metabolic pathology Template:Heme metabolism disorders

de:Rotor-Syndrom

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