Introduction to Dubin Johnson Syndrome

What is/are Dubin Johnson Syndrome?

Dubin Johnson Syndrome - OBJECTIVES Dubin-Johnson syndrome (DJS) is an autosomal recessive disorder in which multidrug-resistance-associated protein 2 (MRP2) deficiency causes an excretion disorder of conjugated bilirubin from hepatocytes into bile canaliculi. ^[1] BACKGROUND Dubin-Johnson syndrome (DJS) is a benign autosomal recessive liver disease involving mutations of the ABCC2 gene. ^[2] This 48th article will explore three of these conditions: foetal valproate syndrome; familial amyloidosis; and Dubin-Johnson syndrome. ^[3] Dubin-Johnson syndrome is a rare inherited disorder associated with a defect in the ability of hepatocytes to secrete conjugated bilirubin into bile. ^[4] Objective: To explore the clinical and pathological features of Dubin-Johnson syndrome. ^[5] Dubin-Johnson syndrome (DJS), an autosomal recessive disorder presenting with conjugated hyperbilirubinemia, is not associated with progression to chronic liver disease (CLD). ^[6] Dubin-Johnson syndrome (DJS) is a rare autosomal recessive disorder that typically manifests in young adulthood as jaundice with conjugated hyperbilirubinemia. ^[7]

autosomal recessive disorder

OBJECTIVES Dubin-Johnson syndrome (DJS) is an autosomal recessive disorder in which multidrug-resistance-associated protein 2 (MRP2) deficiency causes an excretion disorder of conjugated bilirubin from hepatocytes into bile canaliculi. ^[1] Dubin-Johnson syndrome (DJS), an autosomal recessive disorder presenting with conjugated hyperbilirubinemia, is not associated with progression to chronic liver disease (CLD). ^[2] Dubin-Johnson syndrome (DJS) is a rare autosomal recessive disorder that typically manifests in young adulthood as jaundice with conjugated hyperbilirubinemia. ^[3]