Gilberts syndrome disease all are true, except –
Correct Answer: Conjugated hyperbilirubinemia
Description: Ans. is 'a' i.e., Conjugated heperbilirubinemia HEREDITARY UNCONJUGATED HYPERBILIRUBINEMIAFeatureCrigler Najjar syndromeGilbert's syndrome Type IType II Total serum bilirubin, mol/L (mg/dl)310-755 (usually >345)100-430 (usually <345)Typically < 70 mol/L in the absence of fasting or hemolysisRoutine liver testNormalNormalNormalResponse to phenobarbitoneNoneDecrease bilirubin by >25%Decrease bilirubin to normalKernicterusUsualRareNoneHepatic histologyNormalNormalUsually normal increased lipofuscin pigment in someBile colorPale or colorlessPigmentedNormal dark colorBilirubin fractions> 90% uncojugated (57%) mono conjugateslargest fractionMainly diconjugates but monoconjugates also increaseBilirubin UDP - Glucuronosyl transferase activityTypically absenttraces in someMarkedly reduced0 to 10% of normalReduced, typically10-33% of normalInheritanceAutosomalrecessiveAutosomalrecessiveAutosomalrecessive
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