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

Rotor syndrome is a rare, relatively benign autosomal recessive bilirubin disorder of unknown origin.

Contents 1 Presentation 2 Eponym 3 See also 4 References 5 External links

[edit] 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.

It can be differentiated from Dubin-Johnson syndrome in the following ways:

	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)

[edit] Eponym

Rotor syndrome is named after the Filipino internist, Arturo Belleza Rotor (1907-1988).^[1]

[edit] See also

• Jaundice
• Bilirubin metabolism
• Dubin-Johnson syndrome
• Gilbert syndrome
• Crigler-Najjar syndrome

[edit] References

[edit] External links

• med/1065 at eMedicine - article on eMedicine on conjugated hyperbilirubinemia

v • d • e 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