Rotor syndrome
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2008-05-22T19:16:24Z
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{{refimprove|date=December 2007}}
{{Infobox_Disease |
Name = {{PAGENAME}} |
Image = Bilirubin.svg |
Caption = [[Bilirubin]] |
DiseasesDB = 11671 |
ICD10 = {{ICD10|E|80|6|e|70}} |
ICD9 = {{ICD9|277.4}} |
ICDO = |
OMIM = 237450 |
MedlinePlus = |
eMedicineSubj = |
eMedicineTopic = |
MeshID = D006933 |
}}
'''Rotor syndrome''' is a rare, relatively benign [[autosomal recessive]] [[bilirubin]] 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 [[Blood plasma|serum]], mainly of the conjugated type.
It can be differentiated from Dubin-Johnson syndrome in the following ways:
{| class="wikitable"
| || '''Rotor syndrome''' || '''DJS'''
|-
| appearance of [[liver]] || normal histology and appearance || liver has black pigmentation
|-
| [[gallbladder]] visualization ||gallbladder can be visualized by USG || 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)
|}
==Eponym==
Rotor syndrome is named after the [[Filipino people|Filipino]] [[Internal medicine|internist]], [[Arturo Belleza Rotor]] (1907-1988).<ref>{{WhoNamedIt|synd|2296}}</ref>
== See also ==
* [[Jaundice]]
* [[Bilirubin metabolism]]
* [[Dubin-Johnson syndrome]]
* [[Gilbert syndrome]]
* [[Crigler-Najjar syndrome]]
==References==
{{reflist}}
== External links ==
* {{eMedicine|med|1065}} - article on eMedicine on conjugated hyperbilirubinemia
{{Heme metabolism disorders}}
[[Category:Syndromes]]
[[Category:Metabolic disorders]]
[[Category:Genetic disorders]]
[[Category:Autosomal recessive disorders]]
[[Category:Inborn errors of metabolism]]
[[Category:Hepatology]]
{{genetic-disorder-stub}}
[[de:Rotor-Syndrom]]
[[es:Síndrome de Rotor]]
[[it:Sindrome di Rotor]]
[[pl:Zespół Rotora]]