Dubin-Johnson syndrome

Overview
Dubin-Johnson syndrome is an autosomal recessive disease which presents shortly after birth with an increase of conjugated bilirubin without elevation of liver enzymes (ALT, AST).

Presentation
There is a lack of canalicular multi-drug resistant protein which causes dysfunction on bilirubin transfer to bile canaliculi.

An isoform of this protein is localized to the lateral hepatocyte membrane, allowing transport of glucuronide and glutathione conjugates back into the blood.

Analysis of urine pophyrins show a normal level of coproporphirin but the I isomer accounts for 80% ot the total(normally 25%)

Liver will present with dark brown appearance due to pigment accumulation.

Prognosis
Prognosis is good and there's no need to treat this syndrome.