Specific disorders of bilirubin

Question 1

Disease associated with unconjugated pre-hepatic bilirubin

Answer 1

Hemolytic disease

Question 2

Laboratory findings in hemolytic disease

Answer 2

Increased B1, normal B2

Question 3

Defect in hemolytic disease

Answer 3

Increased RBC destruction leading to increased bilirubin production

Question 4

Diseases with unconjugated hepatic bilirubin

Answer 4

Gilbert syndrome, Lucey-Driscoll syndrome, Physiologic jaundice of the newborn, Crigler-Najjar syndrome

Question 5

Laboratory findings in unconjugated hepatic diseases

Answer 5

Increased B1, normal B2

Question 6

Diseases with mixed hepatic bilirubin elevation

Answer 6

Viral hepatitis, Cirrhosis, Hepatic carcinoma

Question 7

Laboratory findings in mixed hepatic diseases

Answer 7

Increased B1 and B2

Question 8

Diseases with conjugated/mixed hepatic bilirubin elevation

Answer 8

Dubin-Johnson syndrome, Rotor syndrome

Question 9

Laboratory findings in Dubin-Johnson and Rotor syndromes

Answer 9

Normal/increased B1, increased B2

Question 10

Characteristic finding that shows dark pigmentation in hepatocytes

Answer 10

Dubin-Johnson syndrome

Question 11

Characteristic finding that shows positive cytosolic inclusion bodies

Answer 11

Rotor syndrome

Question 12

Disease associated with conjugated post-hepatic bilirubin

Answer 12

Biliary obstruction

Question 13

Laboratory findings in biliary obstruction

Answer 13

Normal B1, increased B2

Question 14

Defect that is associated with transport defect coupled with UDPGT deficiency

Answer 14

Gilbert syndrome

Question 15

Defect that is associated with circulating inhibitor of bilirubin conjugation

Answer 15

Lucey-Driscoll syndrome

Question 16

Defect seen in Physiologic jaundice of the newborn

Answer 16

Transient UDPGT deficiency

Question 17

Defect in Crigler-Najjar syndrome

Answer 17

Total or partial UDPGT deficiency

Question 18

Consequence of total UDPGT deficiency in Crigler-Najjar syndrome

Answer 18

Stillbirth (homozygous)

Question 19

Diseases involving a conjugation defect requiring phototherapy

Answer 19

Lucey-Driscoll syndrome, Physiologic jaundice of the newborn, Crigler-Najjar syndrome

Question 20

Defect in viral hepatitis, cirrhosis, and hepatic carcinoma

Answer 20

Decreased conjugation and bile flow interference

Question 21

Defect in Dubin-Johnson and Rotor syndromes

Answer 21

Secretory defect

Question 22

Cause of biliary obstruction

Answer 22

Extrahepatic cholestasis (cholelithiasis, tumors, strictures)