Dubin Johnson Syndrome

Dubin Johnson syndrome is an autosomal recessive condition which results in a conjugated hyperbilinaemia.

It is caused by a mutation in the CMOAT gene resulting in the absence of a functional multidrug resistance protein 2 from the hepatocyte canalicular membrane.

  • Jaundice 
  • Abdominal pain 
  • Dark urine 

Other features of Dubin Johnson syndrome 
  • Increased urinary excretion of coproporphyrin isomer I - more isomer I excreted than isomer III (in normal patients, more isomer III is excreted)
  • Black pigmentation of liver 
  • Prolonged retention of sulfobromophthalein 

It is benign and no specific treatment is required. 

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Secret collector of interesting anonymised ECGs. Fan of the Bath Photomarathon. Lover of cream teas. [Sarah Hudson] (Your Picture)