[MCQ] Hyperbilirubinemias- Part 2

I. Start the exam by click the “Start” button

Hyperbilirubinemias- Part 1

Please wait while the activity loads.
If this activity does not load, try refreshing your browser. Also, this page requires javascript. Please visit using a browser with javascript enabled.

If loading fails, click here to try again

Start

Congratulations - you have completed Hyperbilirubinemias- Part 1. You scored %%SCORE%% out of %%TOTAL%%. Your performance has been rated as %%RATING%%

Your answers are highlighted below.

Question 1

What proportion of bilirubin is derived from degradation of the hemoglobin of senescent red blood cells ?

A	~ 30 - 50 %
B	~ 50 - 70 %
C	~ 70 - 90 %
D	~ 100 %

Question 1 Explanation:

About 70 - 90% of bilirubin is derived from degradation of the hemoglobin of senescent red blood cells.

Question 2

Glutathione-S-transferase is related to which of the following steps in bilirubin metabolism ?

A	Hepatocellular uptake
B	Intracellular binding
C	Conjugation
D	Biliary excretion

Question 2 Explanation:

After hepatocellular uptake, bilirubin is kept in solution by binding to glutathione-S-transferases formerly called ligandins

Question 3

Bilirubin-UDP-glucuronosyltransferase is related to which of the following steps in bilirubin metabolism ?

A	Hepatocellular uptake
B	Intracellular binding
C	Conjugation
D	Biliary excretion

Question 3 Explanation:

Bilirubin is conjugated with one or two glucuronic acid moieties by a specific UDPglucuronosyltransferase to form bilirubin mono- and diglucuronide, respectively.

Question 4

Aqueous insolubility of bilirubin is due to which of the following ?

A	Internal phosphate bonding
B	Internal hydrogen bonding
C	Internal sulphate bonding
D	All of the above

Question 4 Explanation:

Conjugation of bilirubin with glucuronic acid moieties disrupts internal hydrogen bonding that limits aqueous solubility of bilirubin & the resulting glucuronide conjugates are highly soluble in water.

Question 5

UDP-glucuronosyltransferases (UGT) that conjugate bilirubin belong to which UGT family ?

A	UGT1
B	UGT2
C	UGT3
D	UGT4

Question 5 Explanation:

UDP-glucuronosyltransferases (UGT) that conjugate bilirubin belong to the UGT1 family. Exon A1 and the four common exons, collectively designated the UGT1A1 gene, encode the physiologically critical enzyme bilirubin-UDP-glucuronosyltransferase (UGT1A1)

Question 6

Human UGT1 gene complex is on which of the following chromosomes ?

A	2
B	4
C	6
D	8

Question 6 Explanation:

Human UGT1 gene complex is on chromosome 2. It contains at least 13 substrate-specific first exons (A1, A2, etc.). Since four of these are pseudogenes, nine UGT1 isoforms with differing substrate specificities are expressed. Mutations in a first exon affect only a single isoform. Those in exons 2 - 5 affect all enzymes encoded by the UGT1 complex

Question 7

Multidrug resistance - associated protein 2 (MRP2) is related to which of the following steps in bilirubin metabolism ?

A	Hepatocellular uptake
B	Intracellular binding
C	Conjugation
D	Biliary excretion

Question 7 Explanation:

Bilirubin mono- and diglucuronides are excreted across canalicular plasma membrane into bile canaliculus by ATP-dependent transport process mediated by a canalicular membrane protein called multidrug resistance-associated protein 2 (MRP2). Mutations of MRP2 result in Dubin-Johnson syndrome.

Question 8

Which of the following is false about urobilinogen ?

A	A Made from unconjugated bilirubin in gut
B	Water-soluble
C	Colorless
D	Undergoes enterohepatic cycling

Question 8 Explanation:

"Conjugated bilirubin” is converted by bacterial metabolism in gut to water-soluble colorless urobilinogen. Urobilinogen undergoes enterohepatic cycling. Urobilinogen not taken up by liver reaches systemic circulation, from which some is cleared by kidneys.

Question 9

Which of the following interrupts bilirubin enterohepatic cycling ?

A	Aluminum hydroxide
B	Magnesium sulphate
C	Calcium phosphate
D	Calcium carbonate

Question 9 Explanation:

Oral administration of calcium phosphate with or without the lipase inhibitor orlistat may be an efficient means to interrupt bilirubin enterohepatic cycling.

Question 10

In response to hemolytic stress, bone marrow is capable of increasing erythrocyte production by ?

A	Two fold
B	Four fold
C	Six fold
D	Eight fold

Question 10 Explanation:

In response to hemolytic stress, bone marrow is capable of a sustained eight fold increase in erythrocyte production.

Question 11

Hemolysis alone cannot result in a sustained hyperbilirubinemia of more than ?

A	~2 mg/dL
B	~4 mg/dL
C	~6 mg/dL
D	~8 mg/d

Question 11 Explanation:

Hemolysis alone cannot result in a sustained hyperbilirubinemia of more than ~ 4 mg/d

Question 12

Direct-reacting fraction is how much of total serum bilirubin in isolated hemolysis ?

A	<= 10 %
B	<= 12 %
C	<= 15 %
D	<= 18 %

Question 12 Explanation:

Hemolysis results in pure unconjugated hyperbilirubinemia, with direct-reacting fraction being <=15% of total serum bilirubin.

Question 13

Ineffective erythropoiesis is seen in all except ?

A	Thalassemia major
B	Congenital erythropoietic porphyria
C	Crigler-Najjar syndrome
D	Lead poisoning

Question 13 Explanation:

In thalassemia major, megaloblastic anemias due to folate or vitamin B12 deficiency, congenital erythropoietic porphyria, lead poisoning, and congenital & acquired dyserythropoietic anemias, the fraction of total bilirubin production derived from ineffective erythropoiesis is increased, reaching as much as 70% of the total producing modest degrees of unconjugated hyperbilirubinemia.

Question 14

Which of the following produces hyperbilirubinemia due to decreased hepatic bilirubin uptake ?

A	Pregnanediol
B	Chloramphenicol
C	Gentamicin
D	Cholecystographic contrast agents

Question 14 Explanation:

Apart from Gilbert’s syndrome, flavaspidic acid, novobiocin, rifampin and cholecystographic contrast agents produce defects in bilirubin uptake. Pregnanediol, novobiocin, chloramphenicol, and gentamicin produce unconjugated hyperbilirubinemia by inhibiting UGT1A1 activity.

Question 15

Fetal bilirubin is cleared by ?

A	Fetal liver
B	Fetal kidney
C	Placenta
D	All of the above

Question 15 Explanation:

Bilirubin produced by the fetus is cleared by the placenta and eliminated by the maternal liver.

Question 16

Most neonates develop mild unconjugated hyperbilirubinemia between days ?

A	1 and 3 after birth
B	2 and 5 after birth
C	5 and 7 after birth
D	7 and 10 after birth

Question 16 Explanation:

Immediately after birth, due to incompletely developed hepatic physiologic processes like low levels of UGT1A1, undeveloped intestinal flora that convert bilirubin to urobilinogen, most neonates develop mild unconjugated hyperbilirubinemia between days 2 and 5 after birth

Question 17

What are the peak levels of physiologic neonatal jaundice ?

A	3 - 5 mg/dL
B	5 - 10 mg/dL
C	10 - 15 mg/dL
D	15 - 20 mg/dL

Question 17 Explanation:

Peak levels of physiologic neonatal jaundice are typically 5 - 10 mg/dL.

Question 18

Bilibubin levels of physiologic neonatal jaundice return to normal adult concentrations within ?

A	1 week
B	2 weeks
C	3 weeks
D	4 weeks

Question 18 Explanation:

Bilibubin levels of physiologic neonatal jaundice return to normal adult concentrations within 2 weeks as mechanisms required for bilirubin disposition mature

Question 19

Which of the following is false for bilirubin encephalopathy, or kernicterus ?

A	Rapidly rising unconjugated bilirubin concentration
B	Immature blood-brain barrier
C	Deposition in the basal ganglia
D	None of the above

Question 19 Explanation:

A rapidly rising unconjugated bilirubin concentration, or absolute levels >20 mg/dL, exposed the infant to the at risk of bilirubin encephalopathy, or kernicterus

Question 20

Which of the following drugs may produce unconjugated hyperbilirubinemia by inhibiting UGT1A1 activity ?

A	Pregnanediol
B	Chloramphenicol
C	Gentamicin
D	All of the above

Question 20 Explanation:

Pregnanediol, novobiocin, chloramphenicol, and gentamicin may produce unconjugated hyperbilirubinemia by inhibiting UGT1A1 activity

Question 21

‘Breast milk jaundice’ in neonates is due to presence of what in breast milk ?

A	Immunoglobulins
B	Proteins
C	Fatty acids
D	Carbohydrates

Question 21 Explanation:

Bilirubin conjugation may be inhibited by certain fatty acids that are present in breast milk but not serum of mothers whose infants have excessive neonatal hyperbilirubinemia (breast milk jaundice)

Question 22

Lucey-Driscoll syndrome is related to ?

A	UGT1A1 inhibitor in breast milk
B	UGT1A1 inhibitor in maternal serum
C	Low UGT1A1 levels at birth
D	All of the above

Question 22 Explanation:

In transient familial neonatal hyperbilirubinemia (Lucey-Driscoll syndrome) there is a UGT1A1 inhibitor in maternal serum

Question 23

Which of the following about Crigler-Najjar Syndrome, Type I is false ?

A	Unconjugated hyperbilirubinemia of ~ 20 - 45 mg/dL
B	Appears in the neonatal period
C	Persists for life
D	Bilirubin is found in the urine

Question 23 Explanation:

No bilirubin is found in the urine

Question 24

Which of the following about Crigler-Najjar Syndrome, Type I is false ?

A	Normal serum aminotransferases
B	Normal alkaline phosphatase
C	No evidence of hemolysis
D	None of the above

Once you are finished, click the button below. Any items you have not completed will be marked incorrect. Get Results

There are 24 questions to complete.

←

List

→

Return

Shaded items are complete.

1	2	3	4	5
6	7	8	9	10
11	12	13	14	15
16	17	18	19	20
21	22	23	24	End

Return

See all quizzes of Hyperbilirubinemias at here:

Hyperbilirubinemias- Part 1 | Hyperbilirubinemias- Part 2

1.Which of the following is false about Crigler-Najjar Syndrome
Type I ?
A. Unconjugated hyperbilirubinemia
B. Absent UGT1A1 activity in liver
C. Kernicterus common
D. Responds to phenobarbital

2. Causative mutation is in the bilirubin-specific exon A1 of which
variety of Crigler-Najjar Syndrome Type I ?
A. Crigler-Najjar Syndrome Type IA
B. Crigler-Najjar Syndrome Type IB
C. Crigler-Najjar Syndrome Type IC
D. Crigler-Najjar Syndrome Type ID

3. Which of the following is false about Crigler-Najjar Syndrome
Type I ?
A. Rare (estimated prevalence = 0.6 – 1.0 per million)
B. Autosomal recessive pattern of inheritance
C. Estrogen glucuronidation is defective
D. Liver transplantation not helpful

4. Crigler-Najjar Syndrome Type I (CN-I) differs from Crigler-Najjar
Syndrome, Type II (CN-II) is which of the following ?
A. Average bilirubin concentrations are lower in CN-II
B. CN-II is infrequently associated with kernicterus
C. CN-II responds to phenobarbital
D. All of the above

5. Which of the following is false about Gilbert’s Syndrome ?
A. Conjugated hyperbilirubinemia
B. UGT1A1 activity – 10-35 % of normal
C. Phenobarbital normalizes serum bilirubin
D. Aggravated by alcohol use

6. Gilbert’s Syndrome is a close clinical entity to which of the
following ?
A. Crigler-Najjar Syndrome Type I
B. Crigler-Najjar Syndrome Type II
C. Lucey-Driscoll syndrom
D. Benign recurrent intrahepatic cholestasis (BRIC)

7. Which of the following drugs is glucuronidated specifically by
bilirubin-UDP-glucuronosyltransferase ?
A. Estradiol benzoate
B. Acetaminophen
C. Tolbutamide
D. Irinotecan

8. Which of the following drugs used in HIV patients inhibits
UGT1A1 ?
A. Zidovudine
B. Indinavir
C. Enfuvirtide
D. All of the above

9. Which of the following are examples of familial defects in
hepatic excretory function ?
A. Dubin-Johnson Syndrome
B. Rotor Syndrome
C. Benign Recurrent Intrahepatic Cholestasis
D. All of the above
10. Which of the following does not manifest as predominantly
conjugated hyperbilirubinemia ?
A. Dubin-Johnson Syndrome
B. Crigler-Najjar Syndrome
C. Rotor Syndrome
D. Benign Recurrent Intrahepatic Cholestasis

11. In Dubin-Johnson Syndrome, degree of hyperbilirubinemia
may be increased by ?
A. Intercurrent illness
B. Oral contraceptive use
C. Pregnancy
D. All of the above

12. Liver is grossly black in appearance in which of the following ?
A. Dubin-Johnson syndrome
B. Rotor syndrome
C. Progressive familial intrahepatic cholestasis
D. Benign recurrent intrahepatic cholestasis (BRIC)

13. Which of the following is false about Dubin-Johnson Syndrome ?
A. Conjugated hyperbilirubinemia
B. Bilirubinuria present
C. Liver grossly black
D. Pruritus common

14. Dark, coarsely granular pigment in hepatocytes in Dubin
Johnson Syndrome disappears during ?
A. Enteric fever
B. Viral hepatitis
C. Malaria
D. All of the above

15. Mutation in which of the following genes produce the Dubin
Johnson phenotype ?
Harrison’s 18th Ed. 2536
A. ABCC2
B. NTCP
C. MRP2
D. FIC1

16. In urine from Dubin-Johnson Syndrome patients, which is the
predominant coproporphyrin isomer ?
A. Coproporphyrin isomer I
B. Coproporphyrin isomer II
C. Coproporphyrin isomer III
D. Coproporphyrin isomer IV

17. Which of the following is an autosomal recessive disorder ?
A. Crigler-Najjar Syndrome
B. Gilbert’s Syndrome
C. Rotor Syndrome
D. All of the above
18. Rotor Syndrome is clinically similar to ?
A. Dubin-Johnson Syndrome
B. Crigler-Najjar Syndrome
C. Gilbert’s Syndrome
D. Lucey-Driscoll syndrome

19. Which of the following is false about Rotor Syndrome ?
A. Conjugated hyperbilirubinemia
B. Gallbladder visualized on oral cholecystography
C. Liver normal in appearance
D. Total urinary coproporphyrin excretion normal

20. Which of the following is false about Benign Recurrent
Intrahepatic Cholestasis (BRIC) ?
A. Recurrent attacks of pruritus and jaundice
B. Normal serum aminotransferase levels
C. Elevations in alkaline phosphatase
D. Does not lead to cirrhosis

21. Mutation in which of the following genes produce the Benign
Recurrent Intrahepatic Cholestasis (BRIC) ?
A. ABCC2
B. NTCP
C. MRP2
D. FIC1
22. Gene FIC1 is mainly expressed in ?
A. Liver
B. Small intestine
C. Kidney
D. Heart

23. Byler disease is also known as ?
A. Progressive Familial Intrahepatic Cholestasis type 1
B. Progressive Familial Intrahepatic Cholestasis type 2
C. Progressive Familial Intrahepatic Cholestasis type 3
D. Progressive Familial Intrahepatic Cholestasis type 4

24. Mutation of MDR3 gene results in ?
A. Progressive Familial Intrahepatic Cholestasis type 1
B. Progressive Familial Intrahepatic Cholestasis type 2
C. Progressive Familial Intrahepatic Cholestasis type 3
D. Progressive Familial Intrahepatic Cholestasis type 4

Hyperbilirubinemias

[MCQ] Hyperbilirubinemias- Part 2

I. Start the exam by click the “Start” button

Hyperbilirubinemias- Part 1

Share this:

Related

[MCQ] Hyperbilirubinemias- Part 1

[MCQ] Acute viral hepatitis- Part 1

You may also like

Leave a Comment Cancel Reply