All of the following are examples of predominantly unconjugated hyperbilirubinemia, except
Correct Answer: Dubin-Johnson hyperbilirubinemia
Description: Congenital HyperbilirubinemiasCongenital hyperbilirubinemiasInheritanceBiochemical defectClinical featuresUnconjugated hyperbilirubinemiaCrigler Najjar syndrome type I ARAbsent UGT1A1 (Glucronyl transferase) Liver normal; Bile colourless;Kernicterus in newborn;No response to phenobarbitone Total Sr. bilirubin - > 20 - 45 mg/dlCrigler Najjar syndrome type II ADReduced activity of UGT1A1 (0-10%)Liver normal; Bile pigmented;Kernicterus in newborn rare;Response to phenobarbitone +++Total Sr. bilirubin - < 20 mg/dlGilbe syndrome ADReduced activity of UGT1A1 (10-33%)Liver normal; Bile normal colour;Kernicterus never occurs;Phenobarbitone normalizes bilirubinTotal Sr. bilirubin - < 4 mg/dlConjugated hyperbilirubinemiaDubin Johnson syndrome ARImpaired biliary excretion of bilirubin glucuronides Liver darkly pigmented Lysosomes contain polymers of epinephrine metabolites Rotor syndromeARDecreased hepatic uptake and storage and decreased bilirubin excretion Liver normal (Refer: Harrison's Principles of Internal Medicine, 18th edition, pg no: 2532-2536)
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