Duhin-Johnson syndrome.
It is Autosomal recessive disease characterized by Partial defect in excretion of conjugated bilirubin into the bile.*Clinical picture:-It is usually asymptomatic.
- Jaundice.
- Good general condition.
*Investigations:-
- Bilirubin: mild elevation of direct bilirubin
- Biopsy (dark pigment inside hepatocytes i.e the liver is black in colour), it had been proved to be a metabolite of catecholamines.
- +ve Bromosulphthalein (BSP) Test: Normally 90% of this dye is removed from blood within 45 minutes after I.V injection in normal liver. In Dubin-Johnson syndrome there is a late rise in plasma SSP after an initial fall due to reflux of the dye from the liver reflecting hepatic excretion defect.
- No evidence of obstruction.
- No evidence of LCF.
*Treatment:-
No treatment (almost normal life span).