Gilberts syndrome disease all are true, except -: (A) Conjugated hyperbilirubinemia, (B) Fasting hypoglycaemia, (C) Normal liver Histology, (D) Liver enzymes normal
Answer is A. 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