Unconjugated bilirubin is increased in all, except –
Correct Answer: Dubin Johnson syndrome
Description: Dubin Jhonson syndrome is a cause of congenital conjugated hyperbilirubinemia.
Congenital conjugated hyperbilirubinemia
1)Dubin Jhonson syndrome
DJS is a type of congenital conjugated hyperbilirubinemia.
It is autosomal recessive.
Conjugated bilirubin is increased because of defective biliary excretion of bilirubin glucuronides due to mutation in canalicular multidrug resistance protein 2 (MRP 2).
Liver function tests are normal
A cardinal feature of DJS is the accumulation in the lysosome of centrilobular hepatocytes of dark, coarsely granular pigment. As a result, the liver is black in appearance. This pigment is thought to be derived from epinephrine metabolites that are not excreted normally.
There is increased urinary excretion of coproporphyrine I (normal coproporphyrine III is more excreted), but total coproporphyrine level is normal.
Gall bladder is not visualized on oral cholecystography.
After iv administration, there is reflux of conjugated sulfobromophthalein (Bromsulphalein, BSP) from liver to circulation.
2)Rotor syndrome
Rotor syndrome is a type of congenital conjugated hyperbilimbinemia.
It is autosomal recessive.
It is due to decreased biliary excretion of conjugated bilirubin and also due to decrease hepatic uptake & storage of bilirubin.
Differentiating features of rotor syndrome (from DJS) -
Liver is not pigment
Coproporphyrine I is increased in urine but total coproporphyrine level is also increased.
Gall bladder is visualized
There is no reflux of conjugated BSP.
Category:
Pediatrics
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