Rotor syndrome
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| Rotor syndrome Classification and external resources | |
| Bilirubin | |
| ICD-10 | E80.6 |
| ICD-9 | 277.4 |
| OMIM | 237450 |
| DiseasesDB | 11671 |
| MeSH | D006933 |
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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
External links
Heme metabolism disorders (E80, 277.1, 277.4) | |
|---|---|
| Porphyrin | Hepatic porphyria: Acute intermittent - Cutanea tarda/Hepatoerythropoietic - Hereditary copro- - Variegate Erythropoietic: Gunther's |
| Hereditary hyperbilirubinemia | Unconjugated: Lucey-Driscoll syndrome - Gilbert's syndrome - Crigler-Najjar syndrome Conjugated: Dubin-Johnson syndrome - Rotor syndrome |
| see also porphyrin metabolism enzymes, intermediates | |
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .

