[MCQ] Cystic Fibrosis- Part 1

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Cystic Fibrosis- Part 1

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Question 1

The median survival for patients with CF is about ?

A	27 years
B	37 years
C	47 years
D	57 years

Question 1 Explanation:

Due to improvements in therapy, the median survival is >37.4 years for patients with CF

Question 2

CF is characterized by ?

A	Bronchiolectasis
B	Exocrine pancreatic insufficiency
C	Intestinal dysfunction
D	All of the above

Question 2 Explanation:

CF is characterized by chronic bacterial infection of airways that leads to bronchiectasis and bronchiolectasis, exocrine pancreatic insufficiency and intestinal dysfunction, abnormal sweat gland function, and urogenital dysfunction.

Question 3

Cystic fibrosis (CF) is characterized by all except ?

A	Polygenic disorder
B	Autosomal recessive
C	Bronchiolectasis
D	Exocrine pancreatic insufficiency

Question 3 Explanation:

Cystic fibrosis (CF) is a monogenic autosomal recessive disorder.

Question 4

CFTR stands for ?

A	CF transmembrane resistance regulator
B	CF transmembrane conductance regulator
C	CF transport regulator
D	CF transcription regulator

Question 4 Explanation:

CFTR stands for CF transmembrane conductance regulator.

Question 5

CFTR gene is located on chromosome ?

A	4
B	7
C	12
D	13

Question 5 Explanation:

CF is due to mutations in the gene located on the long arm of chromosome 7 that encodes CF transmembrane conductance regulator (CFTR) protein.

Question 6

Which class of CF is least severe ?

A	I
B	II
C	III
D	IV

Question 6 Explanation:

Mutations in CFTR gene fall into five major classes. Classes I - III CFTR gene mutations are “severe” with pancreatic insufficiency and high sweat NaCl values. Class IV mutations can be “mild” associated with pancreatic sufficiency & intermediate/normal sweat NaCl values.

Question 7

Which of the following is abnormal in Class IV CFTR gene mutation ?

A	Defective protein synthesis
B	Defective processing
C	Defective regulation
D	Defective conduction

Question 7 Explanation:

Class I - Defective protein synthesis, Class II - Defective processing, Class III - Defective regulation, Class IV - Defective conduction, Class V - Reduced functioning CFTR protein

Question 8

The most common mutation in CF is termed as ?

A	 F508
B	N1303K
C	G85E
D	G91R

Question 8 Explanation:

Most common mutation in CF is termed F508. CFTR with F508 mutation lacks a phenylalanine (F) residue at position 508. Other clinically important mutations - N1303K, G85E, and G91R lead to misfolded CFTR protein that is prematurely degraded.

Question 9

CF patients homozygous for  F508 have normal sweat electrolytes in the presence of which second mutation ?

A	R553Q
B	R554Q
C	R555Q
D	R556Q

Question 9 Explanation:

Patients homozygous for F508 mutation have normal sweat electrolyte concentrations if a second mutation - R553Q is also present

Question 10

The CFTR protein contains how many amino acids ?

A	1280
B	1380
C	1480
D	1580

Question 10 Explanation:

CFTR protein is a single polypeptide chain with 1480 amino acids. It functions as a cyclic AMP - regulated Cl– channel and as a regulator of other ion channels

Question 11

Fully processed form of CFTR is found in ?

A	Plasma membrane
B	Nuclear membrane
C	Cytoplasm
D	Rough endoplasmic reticulum

Question 11 Explanation:

Fully processed CFTR is found in the plasma membrane in normal epithelia

Question 12

Cellular function ascribed to CFTR is ?

A	Conducts chloride across cell membrane
B	Down-regulates transepithelial sodium transport
C	Regulates Ca++-activated chloride & K+ channels
D	All of the above

Question 12 Explanation:

CFTR conducts chloride across the cell membrane & is regulated by protein kinase A (PKA) in a cAMP-dependent fashion. Other cellular functions of CFTR are down-regulation of transepithelial sodium transport, regulation of calcium-activated chloride & potassium channels.

Question 13

Diagnostic biophysical hallmark of CF airway epithelia is ?

A	Lowered transepithelial electric potential difference
B	Raised transepithelial electric potential difference
C	Plasma membrane fenestration
D	Plasma membrane rigidity

Question 13 Explanation:

Diagnostic biophysical hallmark of CF airway epithelia is raised transepithelial electric potential difference which reflects both the rate of active ion transport and epithelial resistance to ion flow

Question 14

Cystic fibrosis (CF) is characterized by all except ?

A	Increased airway Na+ absorption
B	Increased airway Cl- secretion
C	Reduced salt & water content of mucus
D	Decreased volume of periciliary liquid

Question 14 Explanation:

Capacity to initiate cyclic AMP mediated Cl– secretion is diminished in CF airway epithelia due to absence/dysfunction of CFTR Cl– channel. Accelerated Na+ absorption in CF reflects absence of CFTR inhibitory effects on Na+ channels.

Question 15

In a normal cell, CFTR is synthesized in ?

A	Golgi apparatus
B	Rough endoplasmic reticulum
C	Mitochondria
D	Nucleus

Question 15 Explanation:

CFTR is synthesized in rough endoplasmic reticulum (RER),glycosylated in Golgi apparatus & functions as a Cl- channel & regulator of other ion channels when located in plasma membrane.

Question 16

Which of the following is best related to airway surfaces in CF ?

A	Edematous
B	Atrophic
C	Hypertrophic
D	Dehydrated

Question 16 Explanation:

Due to faulty regulation of Na+ absorption and inability to secrete Cl– via CFTR reduce the volume of liquid on airway surfaces and appear “dehydrated”.

Question 17

Which of the following is mostly infected in CF ?

A	Mucus layer
B	Epithelia
C	Airway wall
D	All of the above

Question 17 Explanation:

Infection in CF airways involves mucus layer rather than epithelial or airway wall

Question 18

Which of the following is false about mucus layer in CF airways ?

A	Mucus stasis
B	Mucus hypoxemia
C	Mucus dehydration
D	All of the above

Question 18 Explanation:

O 2 tension is very low in CF mucus (mucus hypoxemia). Mucus stasis is due to dehydration of mucus and periciliary liquid layers. It produces adhesion of mucus to airway surface causing failure to clear mucus from airways by ciliary and cough-dependent mechanisms

Question 19

CF airways are predisposed to chronic infection by ?

A	Staphylococcus aureus
B	Pseudomonas aeruginosa
C	Strict anaerobes
D	All of the above

Question 20

CF patients fail to secrete which of the following in pancreatic duct ?

A	Na+
B	HCO3–
C	Water
D	All of the above

Question 20 Explanation:

In CF, failure to secrete Na+, HCO3– and water leads to retention of enzymes in pancreas & destruction of virtually all pancreatic tissue

Question 21

Cholelithiasis occurs with increased frequency in ?

A	Cystic fibrosis (CF)
B	Crohn’s disease (CD)
C	Wilson Disease
D	All of the above

Question 21 Explanation:

In addition to the above three illnesses, cholelithiasis occurs with increased frequency in polyglandular autoimmune syndrome Type I.

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See all quizzes of Cystic Fibrosis at here:

Cystic Fibrosis- Part 1| Cystic Fibrosis- Part 2

1.The median survival for patients with CF is about ?
A. 27 years
B. 37 years
C. 47 years
D. 57 years

2. CF is characterized by ?
A. Bronchiolectasis
B. Exocrine pancreatic insufficiency
C. Intestinal dysfunction
D. All of the above

3. Cystic fibrosis (CF) is characterized by all except ?
A. Polygenic disorder
B. Autosomal recessive
C. Bronchiolectasis
D. Exocrine pancreatic insufficiency

4. CFTR stands for ?
A. CF transmembrane resistance regulator
B. CF transmembrane conductance regulator
C. CF transport regulator
D. CF transcription regulator

5. CFTR gene is located on chromosome ?
A. 4
B. 7

C. 12
D. 13

6. Which class of CF is least severe ?
A. I
B. II
C. III
D. IV

7. Which of the following is abnormal in Class IV CFTR gene mutation ?
A. Defective protein synthesis
B. Defective processing
C. Defective regulation
D. Defective conduction

8. The most common mutation in CF is termed as ?
A.  F508
B. N1303K
C. G85E
D. G91R

9. CF patients homozygous for  F508 have normal sweat electrolytes in the presence of which second mutation ?
A. R553Q
B. R554Q
C. R555Q
D. R556Q

10. The CFTR protein contains how many amino acids ?
A. 1280
B. 1380
C. 1480
D. 1580

11. Fully processed form of CFTR is found in ?
A. Plasma membrane

B. Nuclear membrane
C. Cytoplasm
D. Rough endoplasmic reticulum

12. Cellular function ascribed to CFTR is ?
A. Conducts chloride across cell membrane
B. Down-regulates transepithelial sodium transport
C. Regulates Ca++-activated chloride & K+ channels
D. All of the above

13. Diagnostic biophysical hallmark of CF airway epithelia is ?
A. Lowered transepithelial electric potential difference
B. Raised transepithelial electric potential difference
C. Plasma membrane fenestration
D. Plasma membrane rigidity

14. Cystic fibrosis (CF) is characterized by all except ?
A. Increased airway Na+ absorption
B. Increased airway Cl– secretion
C. Reduced salt & water content of mucus
D. Decreased volume of periciliary liquid

15. In a normal cell, CFTR is synthesized in ?
A. Golgi apparatus
B. Rough endoplasmic reticulum
C. Mitochondria
D. Nucleus

16. Which of the following is best related to airway surfaces in CF ?
A. Edematous
B. Atrophic
C. Hypertrophic
D. Dehydrated

17. Which of the following is mostly infected in CF ?
A. Mucus layer
B. Epithelia

C. Airway wall
D. All of the above

18. Which of the following is false about mucus layer in CF airways ?
A. Mucus stasis
B. Mucus hypoxemia
C. Mucus dehydration
D. All of the above

19. CF airways are predisposed to chronic infection by ?
A. Staphylococcus aureus
B. Pseudomonas aeruginosa
C. Strict anaerobes
D. All of the above
20. CF patients fail to secrete which of the following in pancreatic duct ?
A. Na+
B. HCO3–
C. Water
D. All of the above

21. Cholelithiasis occurs with increased frequency in ?
A. Cystic fibrosis (CF)
B. Crohn’s disease (CD)
C. Wilson Disease
D. All of the above

Cystic Fibrosis

[MCQ] Cystic Fibrosis- Part 1

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Cystic Fibrosis- Part 1

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[MCQ] Cystic Fibrosis – Part 2

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