Rotor syndrome: Difference between revisions

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__NOTOC__
{{SI}}
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==Overview==
'''Rotor syndrome''' is a rare, benign [[autosomal recessive]] disorder of unknown origin.
{{Infobox_Disease |
{{Infobox_Disease |
   Name          = {{PAGENAME}} |
   Name          = {{PAGENAME}} |
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   MeshID        = D006933 |
   MeshID        = D006933 |
}}
}}
{{SI}}
{{Editor Help}}
===Presentation===
 
'''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 [[Blood plasma|serum]], mainly of the conjugated type.
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.


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  | [[gallbladder]] visualization ||gallbladder can be visualized by oral cholecystogram  || gallbladder cannot be visualized
  | [[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)
  | 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==
==Classification==
==Pathophysiology==
==Causes==
==Differentiating {{PAGENAME}} from Other Diseases==
==Epidemiology and Demographics==
==Risk Factors==
==Screening==
==Natural History, Complications, and Prognosis==
===Natural History===
===Complications===
===Prognosis===
==Diagnosis==
===Diagnostic Criteria===
===History and Symptoms===
===Physical Examination===
===Laboratory Findings===
===Imaging Findings===
===Other Diagnostic Studies===


==Treatment==
==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.
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===
===Surgery===
===Prevention===


==Eponym==
==Eponym==
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==References==
==References==
<references/>
{{reflist|2}}


== External links ==
== External links ==
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{{Endocrine, nutritional and metabolic pathology}}
{{Endocrine, nutritional and metabolic pathology}}
{{Heme metabolism disorders}}


[[Category:Syndromes]]
[[Category:Metabolic disorders]]
[[Category:Inborn errors of metabolism]]
[[Category:Hepatology]]
[[Category:Hepatology]]



Latest revision as of 17:14, 19 July 2016

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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

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

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

Classification

Pathophysiology

Causes

Differentiating Rotor syndrome from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Natural History

Complications

Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

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.

Medical Therapy

Surgery

Prevention

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

de:Rotor-Syndrom

Template:WikiDoc Sources