Gilbert syndrome true is -
Ans. is 'c' i.e., Phenobarbitone improves the condition* Gilbert syndrome is a milder form of hyperbilirubinemia caused by a reduced activity of UDP-glucuronyl transferase. It is caused by mutation of UGT1A1 gene.* UDP glucuronyl transferase is involved in conjugating bilirubin to form bilirubin diglucuronide. Hence it presents as unconjugated hyperbilirubinemia.* Total bilirubin is mildly increased. But the ratio of unconjugated to conjugated bilirubin is high.* It presents as a milder form of jaundice. It is not life threatening* Starvation and stress are found to cause aggravation of the milder jaundice* If jaundice is disturbing, treatment can be tried with enzyme inducers like phenobarbitone. Phenobarbitone induces UDP glucuronyl transferase and helps in the reduction of bilirubin levels.* As some drugs also get metabolised by UDP glucuronyl transferase, detection of this condition is of clinical significance. For example, an antineoplastic drug, irinotecan is metabolised by UDP glucuronyl transferase. Hence in Gilbert's syndrome, Irinotecan has to be avoided. As UDP glucuronyl transferase defect increases the half-life of Irinotecan, it results in life threatening neutropenia.
Conjugated hyperbilirubinemia
Starvation improves the condition
Phenobarbitone improves the condition
Life threatening
New Born Infants