Kernicterus is invariably associated with
Correct Answer: Crigler-Najjar syndrome type-I
Description: Inherited Unconjugated HyperbilirubinemiaCrigler Najjar Type - 1 Crigler Najjar Type - 2Persistence of unconjugated bilirubin > 20mg/dl after 1st week in the absence of hemolysis suggests CN-1.KERNICTERUS -usual UDPGT activity reduced.Autosomal recessive.Rx - Phototherapy.Heme oxygenase inhibition by metalloporphyrin therapy.Cure - ohotropic liver transplantation.Can be differentiated from CN1 by marked decrease in serum bilirubin with phenobarbitone.KERNICTERUS -unusual.Inducible phenobarbitone response on UGTA1 promoter.Orlistat intestinal lipase inhibitor reduces bilirubin in both CN-1 & CN -2Inherited Conjugated HyperbilirubinemiaDubin Johnson syndrome Rotor syndrome Absent MRP2 protein - multiple drug resistant protein is responsible defectCholangiography fails to visualize gall bladderTotal urinary coproporphyrin is normal||| coproporphyrin 1 excretion||| coproporphyrin 3 excretionX ray -Gall bladder abnormalLiver histology-Black pigment.Additional deficiency of organic anion uptakeTotal urinary coproporphyrin is increasedX ray gall bladder - normalNo black pigment(Refer: Nelson's Textbook of Paediatrics, 19thedition, pg no:604)
Category:
Pathology
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