Experience | Patient+ | News | Products | Other
This is a PatientPlus article. PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people.
Rotor's Syndrome
Post your experienceSee others (1 there)
This a very rare syndrome of idiopathic conjugated hyperbilirubinaemia, usually presenting in infancy or childhood.
It can be distinguished from Dubin-Johnson syndrome because in this syndrome there is an absence of hepatic pigmentation. There are also differences in urinary coproporphyrin excretion, hepatic clearance of bromosulphopthalein and oral cholecystography.
There is an impairment of excretion of organic anions from hepatocytes into the canalicular lumen. This causes defective excretion of conjugated bilirubin, its reabsorption into the blood and excretion in the urine.
It occurs sporadically in families and is thought to be inherited in an autosomal recessive fashion.1
There are no available population-based figures for its prevalence or incidence:
- It appears to be commonest in The Philippines, where it was originally described by Arturo Belleza Rotor and co-workers in 1948.2
- It is exceedingly rare in the UK and worldwide.
- Chronic jaundice without evidence of haemolysis.
- Attacks of intermittent epigastric discomfort and abdominal pain may occur but are rare.
- There may be episodic fever.
- Dubin-Johnson syndrome
- Viral hepatitis
- Drug-induced cholestasis (including anaesthetic reactions)
- Autoimmune hepatitis
- Wilson's disease
- Haemochromatosis
- Alpha-1 antitrypsin deficiency
- Benign recurrent cholestasis
- Any cause of obstruction of small or large bile ducts in children
- Sarcoidosis
- Amyloidosis
- LFTs are normal.
- Serum and urinary bilirubin is elevated and is largely in conjugated (direct) form.
- Bilirubin levels are in range 50–130 μmol/l.3
- Ultrasound usually shows no abnormality.
- Oral cholecystogram is normal.
- Total coproporphyrin excretion is greatly elevated in both Rotor and Dubin-Johnson syndromes:
- The ratio of coproporphyrin I to III in urine allows differentiation of these 2 conditions.
- Around 90% as coproporphyrin I in Dubin-Johnson but much lower proportion in Rotor.1
- The plasma disappearance of injected bromosulphopthalein is delayed, with no secondary rise (which is seen in Dubin-Johnson).4
- Hepatic biopsy will show pigment deposition in Dubin-Johnson syndrome, but not in Rotor syndrome.
The condition is largely benign and does not require any active intervention in most cases.
Other hepatic disease can damage the liver preferentially in patients with the condition, so it is best to avoid alcohol, hepatotoxic drugs, exposure to viral hepatitis etc.
Can occasionally progress to liver failure if there is another cause of hepatic compromise.
Usually good with benign course unless there is co-existent liver disease.
Document references
- OMIM; On-line Mendelian Inheritance in Man. Hyperbilirubinaemia, Rotor Type.; Genetic information on the syndrome.
- Whonamedit.com; Rotor's syndrome. Basic detail on syndrome and those associated with its discovery and characterisation.
- Internal Medicine. Chief Ed. Stein JH. 5th edition. Mosby, USA.
- Paré P;Conjugated hyperbilirubinaemia, First Principles of Gastroenterology Ch.14. On-line adapted textbook.
Internet and further reading
- OMIM - Rotor Syndrome
- Weisiger RA; Hyperbilirubinaemia, conjugated. eMedicine, January 2007.
- Limdi JK, Hyde GM; Evaluation of abnormal liver function tests. Postgrad Med J. 2003 Jun;79(932):307-12. [abstract]
DocID: 1586
Document Version: 21
DocRef: bgp1284
Last Updated: 14 Oct 2008
Review Date: 14 Oct 2010
The authors and editors of this article are employed to create accurate and up to date content reflecting reliable research evidence, guidance and best clinical practice. They are free from any commercial conflicts of interest. Find out more about updating.
Experience | Patient+ | News | Products | Other
Related pages in Patient UK
Your Experience (^ top of page)
Please add your experience about this condition / medicine
View Patient Experience for 'Rotor Syndrome' (1 there)PatientPlus articles related to this topic (^ top of page)
Patient UK Newspaper (^ top of page)
Latest Health News
Related Products (^ top of page)
Medical equipment
Books
Other - Useful resources (^ top of page)
Pictures, diagrams, photos, images, etc.Evidence based medicine
Online textbooks and journals
UK Guidelines
Online Videos
Medline
Other good health sites
Want to search some more? Use the Google Search box below to search our site.
Disclaimer: Patient UK has no control over the content of any external links above. Inclusion does not imply endorsement by Patient UK.
Want to advertise on this site? Find out how >>
Here you can follow a link to view existing patient experiences on this subject, or to add your own
This will offer you the usual PDF options i.e. document navigation, search, zoom and formatted print
Note: this is the best way to print the document
Note: this will open in a new window
Note: this will open in a new window
Here you can follow a link to view existing patient experiences on this subject, or to add your own
This will offer you the usual PDF options i.e. document navigation, search, zoom and formatted print
Note: this is the best way to print the document
Note: this will open in a new window
Note: this will open in a new window



