picture of bilirubin formation and metabolism
Hemolysis of the RBCs gives rise to unconjugated bilirubin.
It is carried by the carrier protein to the liver.
In the liver, conjugation takes place with the help of a glucoronyl tranferase enzyme.
In Gilbert’s syndrome, there is a deficiency of glucoronyl transferase enzyme.
Common, mild liver condition in which the liver doesn’t properly process bilirubin.
nonhemolytic condition and is an autosomal dominant character
Liver glucuronyltransferase activity is low due to a mutation in the bilirubin-UDP-glucuronosyl-transferase gene.
This heterozygous group with raised bilirubin.
This is inherited in an autosomal recessive pattern.
There is an increase in unconjugated bilirubin usually <3 mg/dL
This is a benign condition and occurs in 2% of the population
Bilirubin is produced by the breakdown of red blood cells.
The hyperbilirubinemia is due to the reduced activity of the Glucuronyltransferase enzyme.
There is no role of hemolysis and hyperbilirubinemia is due to the deficiency of the enzyme.
Glucuronyl transferase enzymes typically have 10 to 35% reduced activity.
Glucuronyl Transferase enzyme is needed for the conjugation of the bilirubin.
There is an increase in the bilirubin monoglucuronide (unconjugated bilirubin).
Conjugation renders the bilirubin water-soluble, which is excreted into the bile and duodenum.
When these patients are given phenobarbital, that normalizes bilirubin and hepatic bilirubin clearance.
