-Duhin-Johnson syndrome.

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.

1. Jaundice.
2. Good general condition.

*Investigations:-

1. Bilirubin: mild elevation of direct bilirubin
2. Biopsy (dark pigment inside hepatocytes i.e the liver is black in colour), it had been proved to be a metabolite of catecholamines.
3. +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.
4. No evidence of obstruction.
5. No evidence of LCF.

*Treatment:-

No treatment (almost normal life span).

Dr Ibrahim

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