1 .For each of the disorders of direct and indirect increases in bilirubin, list

Question

1

.For each of the disorders of direct and indirect increases in bilirubin, list the mechanism by which the defect occurs in bilirubin metabolism.

Disorder

Defect in Bilirubin Metabolism

Unconjugated Hyperbilirubinemia

Hemolysis

Ineffective erythropoiesis

Neonatal physiologic jaundice

Crigler-Naijar syndrome Type I

Conjugated Hyperbilirubinemia

Dubin-Johnson syndrome

Rotor’s syndrome

Extrahepatic cholestasis

Hepatocellular injury

2

Serum from a 50 yr old man was analyzed for both prostatic acid phosphatase(PAP) and prostate specific antigen(PSA) following a digital rectal examination of the prostate. The PAP result is 6.0 ug/L (URL= 3.0 ug/L) and the PSA is 2.0 ug/L (URL=4.0 ug/L). Both tests are repeated on the same sample and the results remain unchanged. What is the most likely explanation

Disorder

Defect in Bilirubin Metabolism

Unconjugated Hyperbilirubinemia

Hemolysis

Ineffective erythropoiesis

Neonatal physiologic jaundice

Crigler-Naijar syndrome Type I

Conjugated Hyperbilirubinemia

Dubin-Johnson syndrome

Rotor’s syndrome

Extrahepatic cholestasis

Hepatocellular injury

Explanation / Answer

Dublin Johnson syndrome- impaired excretion of bilirubin because of a in a MRP2 protein .

Rotors syndrome : less efficient transport of bilirubin due to defect in transport proteins OATP1B1 and OATP1B3.

Extrahepatic cholestasis : impairment in the secretion of hepatocytes or maybe a bile obstruction by either the intrahepatic or extrahepatic bile ducts.

Hepatocellular injury : decreased uptake of bilirubin

Hemolysis : increased accumulation of bilirubin due to breakdown of hemoglobin

Ineffective erythropoiesis : moderate increase in hemolysis resulting in excessive unconjugated bilirubin

Crigler-Najjar syndrome type 1: absence of the enzyme hepatic bilirubin glucuronosyltransferase

Neonatal jaundice : degradation of the fetal hemoglobin and excess bilirubin secretion

2. The most likely explanation is for prostate cancer as both the prostatic acid phosphatase(PAP) and prostate specific antigen(PSA) are markers for prostrate cancer. Out of the two, PAP has been found to be the most sensitive marker.

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