Condition

Rotor syndrome

Endocrine, Nutritional and Metabolic Diseases

Rotor syndrome, also called Rotor type hyperbilirubinemia, is a rare inherited disorder of bilirubin handling. It is inherited in an autosomal recessive pattern and is regarded as relatively benign. People with it have a chronic rise in bilirubin that is mostly the conjugated form, which produces jaundice without the red blood cell breakdown seen in hemolytic jaundice. The source places the fault in the liver cells, which store conjugated bilirubin poorly so that it leaks back into the blood plasma. It is a distinct but similar disorder to Dubin-Johnson syndrome, which also raises conjugated bilirubin but arises by a different mechanism.

Facts
Classification
Body System
Digestive System 1
ICD-10 Chapter
IV: Endocrine, Nutritional and Metabolic Diseases 2
Communicable or Non-communicable
Non-Communicable 1
Transmission Route
Non-Transmissible 3
Causative Agent
Causative Agent (category)
Genetic / Hereditary 1
Prevalence Band
Rare (Under 1 in 2000) 4
Sources
1. Rotor syndrome (Wikipedia)
  • Lead section
    autosomal recessive bilirubin disorder
  • Lead section [body-system]
    impaired hepatocellular storage of conjugated bilirubin
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2. Orphadata (Orphanet): Orphadata cross-references, ORPHAcode 3111
Orphadata (Orphanet)Orphanet cross-reference to ICD-10, chapter derived from the code
Quote, Orphanet cross-reference to ICD-10, chapter derived from the code
"Reference": "E80.6", "Source": "ICD-10"
View the Source
3. MedlinePlus Genetics (U.S. National Library of Medicine): Rotor syndrome - MedlinePlus Genetics
MedlinePlus Genetics (U.S. National Library of Medicine)Description and Inheritance
Quote, Description and Inheritance
This condition is inherited in an autosomal recessive pattern .
View the Source
4. Orphadata (Orphanet): Orphadata epidemiology, ORPHAcode 3111
Orphadata (Orphanet)Orphanet epidemiology, ORPHAcode 3111
Quote, Orphanet epidemiology, ORPHAcode 3111
"PrevalenceClass": "\u003c1 / 1 000 000", "PrevalenceGeographic": "Worldwide", "PrevalenceQualification": "Class only", "PrevalenceType": "Point prevalence"
View the Source
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