[MCQ] Cystic Fibrosis - Part 2

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Cystic Fibrosis- Part 2

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Congratulations - you have completed Cystic Fibrosis- Part 2. You scored %%SCORE%% out of %%TOTAL%%. Your performance has been rated as %%RATING%%

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Question 1

Which of the following can occur in CF ?

A	Thickened biliary secretions
B	Focal biliary cirrhosis
C	Bile-duct proliferation
D	All of the above

Question 1 Explanation:

In CF, defective hepatic ductal salt (Cl–) & water secretion causes thickened biliary secretions, focal biliary cirrhosis, and bile-duct proliferation in ~ 25 - 30% of patients. Chronic cholecystitis and cholelithiasis also occur

Question 2

In CF, sweat emerging on the skin surface contains ?

A	High level of salt
B	Low level of salt
C	Normal level of salt
D	Any of the above

Question 2 Explanation:

In CF, sweat emerging on the skin surface contains a high level of salt

Question 3

Elevated electrolyte levels in sweat can be due to ?

A	Cystic fibrosis
B	Fucosidosis
C	Glycogen storage disease type 1
D	All of the above

Question 4

Elevated electrolyte levels in sweat can be due to ?

A	Mucopolysaccharidosis
B	Hypothyroidism
C	Vasopressin-resistant diabetes insipidus
D	Vasopressin-resistant diabetes insipidus

Question 5

Elevated electrolyte levels in sweat can be due to ?

A	Adrenal insufficiency
B	Familial cholestasis
C	Familial hypoparathyroidism
D	All of the above

Question 6

Recovery of which bacteria in bronchoalveolar lavage fluid supports the diagnosis of CF ?

A	Klebsiella pneumoniae
B	Proteus mirabilis
C	Escherichia coli
D	Pseudomonas aeruginosa

Question 6 Explanation:

Recovery of Pseudomonas aeruginosa in BAL fluid supports the diagnosis of CF

Question 7

In lower respiratory tract, the first symptom of CF is ?

A	Cough
B	Dyspnoea
C	Pain chest
D	Hemoptysis

Question 7 Explanation:

In the lower respiratory tract, the first symptom of CF is cough.

Question 8

Which of the following is often the first organism recovered from lung secretions in newly diagnosed CF patients ?

A	P. aeruginosa
B	Haemophilus influenzae
C	Aspergillus fumigatus
D	Klebsiella

Question 8 Explanation:

Haemophilus influenzae and S. aureus are often the first organisms recovered from lung secretions in newly diagnosed CF patients.

Question 9

Infection with which of the following is rare in CF ?

A	Haemophilus influenzae
B	Mycobacterium tuberculosis
C	Mycobacterium tuberculosis
D	Mycobacterium tuberculosis

Question 9 Explanation:

Mycobacterium tuberculosis is rare in patients with CF

Question 10

First lung-function abnormalities seen in CF children is ?

A	Decreased forced vital capacity (FVC)
B	Decreased FEV1
C	Decreased FEV25-50
D	Increased ratios of RV / TLC

Question 10 Explanation:

First lung-function abnormality seen in CF children is increased ratios of residual volume to total lung capacity reflecting small-airways disease.

Question 11

Earliest chest x-ray change in CF lungs is that of ?

A	Hyperinflation
B	Bronchial cuffing
C	Bronchiectasis
D	Pleural effusion

Question 11 Explanation:

Earliest chest x-ray change in CF lungs is hyperinflation, reflecting small-airways obstruction.

Question 12

Which part of the lung displays earliest & most severe changes in CF ?

A	Right upper lobe
B	Left upper lobe
C	Right lower lobe
D	Left lower lobe

Question 12 Explanation:

Right upper lobe displays the earliest and most severe changes in CxR in CF

Question 13

Which of the following can occur in CF ?

A	Pneumothorax
B	Pneumothorax
C	Clubbing of digits
D	All of the above

Question 13 Explanation:

Pneumothorax occurs in >10% of CF patients. Hemoptysis is common. Clubbing of digits, respiratory failure and cor pulmonale point to advanced CF.

Question 14

Distal intestinal obstruction syndrome (DIOS) consists of all except ?

A	Left lower quadrant pain
B	Loss of appetite
C	Emesis
D	Palpable mass

Question 14 Explanation:

In children & young adults, meconium ileus equivalent or distal intestinal obstruction syndrome (DIOS) occurs consisting of right lower quadrant pain, loss of appetite, emesis & often a palpable mass

Question 15

Which of the following does not occur with increased frequency in CF ?

A	Diabetes mellitus
B	Appendicitis
C	Cholelithiasis
D	Cholelithiasis

Question 15 Explanation:

DIOS can be confused with appendicitis, whose frequency is not increased in CF. Gastrointestinal malignancy is increased in incidence in CF. CF patients have an increased incidence of osteoarthropathy, renal stones, and osteoporosis, particularly following transplant

Question 16

Which of the following can also cause pulmonary disease and azoospermia ?

A	Chédiak-Higashi syndrome
B	Young’s syndrome
C	Felty’s syndrome
D	Job’s Syndrome

Question 16 Explanation:

Young’s syndrome can cause pulmonary disease and azoospermia. > 95% of male patients with CF are azoospermic, reflecting obliteration of the vas deferens due to defective liquid secretion

Question 17

All of the following are useful tests for CF except ?

A	Sweat chloride concentration
B	Semen Analysis
C	Serum lipids
D	Nasal Potential-Difference Measurements

Question 17 Explanation:

A combination of clinical criteria & abnormal CFTR function ( sweat tests, nasal PD measurements, and CFTR mutation analysis) are required for diagnosis of CF

Question 18

What value of sweat Cl– concentration is typical in CF adults ?

A	> 40 meq/L
B	> 50 meq/L
C	> 60 meq/L
D	> 70 meq/L

Question 18 Explanation:

Typically in adults a sweat Cl– concentration of >70 meq/L is pathognomonic for CF.

Question 19

Normal mean nasal potential-difference is ?

A	– 4.7 mV
B	– 24.7 mV
C	– 44.7 mV
D	– 64.7 mV

Question 19 Explanation:

Normal mean nasal potential-difference is – 24.7 mV. In CF, nasal transepithelial PD is raised

Question 20

In CF, information of nasal potential-difference measurements can be augmented by the use of ?

A	Amiloride
B	Chloride-free solution
C	Isoproterenol
D	All of the above

Question 20 Explanation:

Nasal potential-difference measurements (including responses to amiloride, chloride-free solution, and isoproterenol) may demonstrate abnormal CFTR function more reliably than the sweat test.

Question 21

What is the strength of hypertonic saline that is recommended for inhalation in CF ?

A	2 %
B	3 %
C	5 %
D	7 %

Question 21 Explanation:

Inhaled hypertonic saline (7%) has demonstrated efficacy in CF patients by restoring mucus clearance and pulmonary function.

Question 22

In CF patients who have undergone lung transplantation, death principally results from ?

A	Massive hemoptysis
B	Secondary infection
C	Secondary infection
D	HIV complications

Question 22 Explanation:

Only effective treatment for respiratory failure in CF is lung transplantation. 2-year survival for lung transplantation exceeds 60%. Transplant-patient deaths result principally from obliterative bronchiolitis.

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See all quizzes of Cystic Fibrosis at here:

Cystic Fibrosis- Part 1| Cystic Fibrosis- Part 2

1.Which of the following can occur in CF ?
A. Thickened biliary secretions
B. Focal biliary cirrhosis
C. Bile-duct proliferation
D. All of the above

2. In CF, sweat emerging on the skin surface contains ?
A. High level of salt
B. Low level of salt
C. Normal level of salt

D. Any of the above

3. Elevated electrolyte levels in sweat can be due to ?
A. Cystic fibrosis
B. Fucosidosis
C. Glycogen storage disease type 1
D. All of the above
4. Elevated electrolyte levels in sweat can be due to ?
A. Mucopolysaccharidosis
B. Hypothyroidism
C. Vasopressin-resistant diabetes insipidus
D. All of the above
5. Elevated electrolyte levels in sweat can be due to ?
A. Adrenal insufficiency
B. Familial cholestasis
C. Familial hypoparathyroidism
D. All of the above
6. Recovery of which bacteria in bronchoalveolar lavage fluid supports the diagnosis of CF ?
A. Klebsiella pneumoniae
B. Proteus mirabilis
C. Escherichia coli
D. Pseudomonas aeruginosa

7. In lower respiratory tract, the first symptom of CF is ?
A. Cough
B. Dyspnoea
C. Pain chest
D. Hemoptysis

8. Which of the following is often the first organism recovered from lung secretions in newly diagnosed CF patients ?
A. P. aeruginosa
B. Haemophilus influenzae
C. Aspergillus fumigatus
D. Klebsiella

9. Infection with which of the following is rare in CF ?
A. Haemophilus influenzae
B. Mycobacterium tuberculosis

C. P. aeruginosa
D. Aspergillus fumigatus

10. First lung-function abnormalities seen in CF children is ?
A. Decreased forced vital capacity (FVC)
B. Decreased FEV
1
C. Decreased FEV
25-50
D. Increased ratios of RV / TLC

11. Earliest chest x-ray change in CF lungs is that of ?
A. Hyperinflation
B. Bronchial cuffing
C. Bronchiectasis
D. Pleural effusion

12. Which part of the lung displays earliest & most severe changes in CF ?
A. Right upper lobe
B. Left upper lobe
C. Right lower lobe
D. Left lower lobe

13. Which of the following can occur in CF ?
A. Pneumothorax
B. Cor pulmonale
C. Clubbing of digits
D. All of the above

14. Distal intestinal obstruction syndrome (DIOS) consists of all except ?
A. Left lower quadrant pain
B. Loss of appetite
C. Emesis
D. Palpable mass

15. Which of the following does not occur with increased frequency in CF ?
A. Diabetes mellitus
B. Appendicitis
C. Cholelithiasis

D. Malabsorption

16. Which of the following can also cause pulmonary disease and azoospermia ?
A. Chédiak-Higashi syndrome
B. Young’s syndrome
C. Felty’s syndrome
D. Job’s Syndrome
17. All of the following are useful tests for CF except ?
A. Sweat chloride concentration
B. Semen Analysis
C. Serum lipids
D. Nasal Potential-Difference Measurements

18. What value of sweat Cl– concentration is typical in CF adults ?
A. > 40 meq/L
B. > 50 meq/L
C. > 60 meq/L
D. > 70 meq/L
19. Normal mean nasal potential-difference is ?
A. – 4.7 mV
B. – 24.7 mV
C. – 44.7 mV
D. – 64.7 mV

20. In CF, information of nasal potential-difference measurements can be augmented by the use of ?
A. Amiloride
B. Chloride-free solution
C. Isoproterenol
D. All of the above

21. What is the strength of hypertonic saline that is recommended for inhalation in CF ?
A. 2 %
B. 3 %
C. 5 %

D. 7 %

22. In CF patients who have undergone lung transplantation, death principally results from ?
A. Massive hemoptysis
B. Secondary infection
C. Obliterative bronchiolitis
D. HIV complications

Cystic Fibrosis

[MCQ] Cystic Fibrosis – Part 2

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Cystic Fibrosis- Part 2

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[MCQ] Chronic Obstructive Pulmonary Disease- Part 1

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