Rotor syndrome: Difference between revisions
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'''Rotor syndrome''' is a rare, benign [[autosomal recessive]] disorder of unknown origin. | '''Rotor syndrome''' is a rare, benign [[autosomal recessive]] disorder of unknown origin. | ||
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{{Endocrine, nutritional and metabolic pathology}} | {{Endocrine, nutritional and metabolic pathology}} | ||
{{Heme metabolism disorders}} | {{Heme metabolism disorders}} | ||
[[Category:Syndromes]] | [[Category:Syndromes]] | ||
Revision as of 15:47, 20 August 2012
| Rotor syndrome | |
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| 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
External links
Template:Endocrine, nutritional and metabolic pathology Template:Heme metabolism disorders