<prompt> Which of the following enzymes is deficient in Criggler Najer syndrome?UDP glucoronyl transferase 1UDP glucoronyl transferase 2Bilirubin synthaseHeme synthase  <response>: Ans: a UDP-Glucronyl trans... Ref: Vasudevan, 4th ed, 256; 5th ed,p. 268; Harper, 23th ed,p. 371In Crigler Najar syndrome there is severe deficiency of UDP-Glucronyl transferase-1.UBG: UrobilinogenUB: UrobilinSB: StercobilinTests for bile pigments:Bilirubin: Fouchets, gmelins, van den berg.Bilinogens: UBG Ehrlich test.Bilins: UB SB Schlesingers testFew points about jaundice in children:Physiological jaundice:Occurs 24 hrs after birth. Two phases:Phase I - lasts 3-5 days. Bilirubin level will not exceed 15 mg dl., Phase II - lasts for 2 weeks, upto 1 month in pre term infantsMost important point in physiological jaundice:Total S. bilirubin level 15 mg dl.Rise not more than 0.5 mg hr or 5 mg dl per day.Onset after 24 hrs.Direct S. bilirubin 2 mg dl.Breast feeding jaundice:Rise in serum bilirubin for the first few days only.Breast milk jaundice:Due to inhibition of conjugation by substances like FFAand pregnanediol.Rise of bilirubin occurs by 7th day.Reaches maximum concentration of 10-30 mg dl by 2-3 weeks.Temporary interruption improves condition. 