Dubin-Johnson Syndrome

Published on 21/04/2017 by admin

Filed under Pathology

Last modified 21/04/2017

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Grossly Evident Pigment
Gross photograph of liver core biopsies embedded in the paraffin block show dark regions image corresponding to the pigment within centrizonal hepatocytes.

image
Cytoplasmic Pigment
H&E section shows coarse granular pigment deposition in centrizonal hepatocytes.
image
PAS Stain With Diastase Stain
PAS stain with diastase digestion accentuates the coarse pigment granules in the cytoplasm of centrizonal hepatocytes.
image
Fontana-Masson Stain
Fontana-Masson stain highlights the coarse pigment within centrizonal hepatocytes.

TERMINOLOGY

Definitions

• Defect in hepatocellular secretion of conjugated bilirubin

ETIOLOGY/PATHOGENESIS

Genetic Disorder

• Autosomal recessive

• Mutations in ABCC2 ( CMOAT / MRP2) gene, which codes for ATP-dependent organic anion transport localized to canalicular membrane
image Results in impaired biliary canalicular transport of organic anions including conjugated bilirubin
image Impaired glutathione excretion reduces bile salt-independent bile flow

CLINICAL ISSUES

Epidemiology

• Incidence

image Rare
• Age

image Develop jaundice in teenage years
• Sex

image M = F
• Ethnicity

image Prevalence highest among Moroccan and Iranian Jews (1:1,300)

Presentation

• Most patients asymptomatic
• Can present as chronic or intermittent jaundice or with mild right upper quadrant abdominal pain
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