[MCQ] Chronic Obstructive Pulmonary Disease- Part 1

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Chronic Obstructive Pulmonary Disease- Part 1

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

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Question 1

COPD includes ?

A	Emphysema
B	Chronic bronchitis
C	Small airways disease
D	All of the above

Question 1 Explanation:

COPD includes emphysema, chronic bronchitis and small airways disease all characterized by airflow limitation that is not fully reversible

Question 2

Asthma, chronic bronchitis and emphysema are variations of the same basic disease is hypothesised by ?

A	American hypothesis
B	British hypothesis
C	British hypothesis
D	Dutch hypothesis

Question 2 Explanation:

Dutch hypothesis suggests that asthma, chronic bronchitis & emphysema are variations of the same basic disease which is modulated by environmental & genetic factors to produce these pathologically distinct entities. British hypothesis states that asthma & COPD are fundamentally different disease

Question 3

Obstructive lung disease is diagnosed if ?

A	FEV1 / FVC < 0.7
B	FEV1 / FVC > 0·7
C	FVC / FEV1 < 0·7
D	FVC / FEV1 > 0·7

Question 3 Explanation:

COPD is characterized by an FEV1 value that is <80 % of the predicted normal value and an FEV1/ FVC ratio of <0.70.

Question 4

Which allele is associated with normal 1 antitrypsin levels ?

A	S
B	M
C	Z
D	Null

Question 4 Explanation:

Protease inhibitor (PI or SERPINA1) locus encodes 1AT. M allele is associated with normal 1AT levels. S allele is associated with slightly reduced 1AT levels.

Question 5

Which allele is associated with markedly reduced 1 antitrypsin levels ?

A	S
B	M
C	Z
D	Null

Question 5 Explanation:

Z allele is associated with markedly reduced 1AT levels. Null alleles lead to absence of any 1AT. Individuals with two Z alleles or one Z and one null allele are referred to as PiZ, which is the most common form of severe  1AT deficiency.

Question 6

α1 antitrypsin is mainly produced in ?

A	Lung
B	Liver
C	Kidney
D	Spleen

Question 6 Explanation:

1-antitrypsin deficiency is a genetic disorder, clinically characterised by liver disease & early-onset emphysema. 1 AT is mainly produced in liver. Its main function is to protect lung against proteolytic damage from neutrophil elastase.

Question 7

α1 antitrypsin is the prototypic member of which superfamily of proteins ?

A	Glutamine protease inhibitor
B	Methionine protease inhibitor
C	Threonine protease inhibitor
D	Serine protease inhibitor

Question 8

Mutation that causes severe α1-antitrypsin deficiency arises in which gene ?

A	SERPINA 1
B	SERPINA 2
C	SERPINA 3
D	SERPINA 4

Question 8 Explanation:

Most frequent mutation that causes severe 1-AT deficiency arises in SERPINA1 gene & gives rise to Z allele. This mutation reduces concentrations in serum of 1 AT by retaining polymerised molecules within hepatocytes

Question 9

Risk for emphysema increases when level of serum 1-AT is ?

A	< 11 µmol/L
B	< 21 µmol/L
C	< 31 µmol/L
D	< 41 µmol/L

Question 9 Explanation:

An amount of 1-AT below the serum protective threshold of 11 µmol/L increases risk for emphysema.

Question 10

SERPINA1 gene was formerly known as ?

A	PR
B	PI
C	PX
D	PZ

Question 10 Explanation:

SERPINA1 gene was formerly known as protease inhibitor (PI).

Question 11

α1 antitrypsin deficiency is inherited as ?

A	Autosomal co-dominant
B	Autosomal recessive
C	X linked
D	None of the above

Question 11 Explanation:

1-antitrypsin deficiency is inherited as an autosomal co-dominant disorder, for which more than 100 alleles have been identified.

Question 12

SERPINA1 gene is located on ?

A	Long arm of chromosome 12
B	Long arm of chromosome 13
C	Long arm of chromosome 14
D	Long arm of chromosome 15

Question 12 Explanation:

SERPINA1 gene is located on long arm of chromosome 14. It spans 12·2 kb & is organised into four coding (2, 3, 4, 5) & three non-coding (1a, 1b, 1c) exons.

Question 13

Normal α1-antitrypsin concentrations in serum is ?

A	5 - 13 mol/L
B	13 - 20 mol/L
C	20 - 53 mol/L
D	65 - 90 mol/L

Question 14

Normal α 1-antitrypsin concentrations in serum is ?

A	15 - 50 mg/dL
B	50 - 150 mg/dL
C	150 - 350 mg/dL
D	150 - 350 mg/dL

Question 14 Explanation:

Normal alleles are typified by 1-antitrypsin concentrations in serum of 20 - 53 µmol/L. Typically, deficient alleles lead to 1 AT levels <20 µmol/L

Question 15

Dysfunctional variants leading to abnormal function of α1 antitrypsin can cause ?

A	Emphysema
B	Cirrhosis liver
C	Bleeding diathesis
D	All of the above

Question 16

Dysfunctional variants leading to abnormal function of α1 antitrypsin can cause ?

A	c-ANCA-positive vasculitis
B	Cirrhosis liver
C	Bleeding diathesis
D	All of the above

Question 16 Explanation:

Dysfunctional variants lead to abnormal function of 1 antitrypsin with reduced binding to neutrophil elastase (as in the F variant) or, as with Pittsburgh, structural abnormality that causes the protein to serve as a thrombin inhibitor rather than as an antielastolytic protein, causing a bleeding diathesis.

Question 17

Testing for α1 antitrypsin concentration in serum is done by ?

A	Nephelometry
B	Rocket immunoelectrophoresis
C	Radial immunodiffusion
D	All of the above

Question 17 Explanation:

Testing for 1-AT deficiency in serum is done by nephelometry, rocket immunoelectrophoresis or radial immunodiffusion. Clinical laboratory test used most frequently to screen for 1AT deficiency is measurement of the immunologic level of 1AT in serum

Question 18

α1 antitrypsin plays a major role in inactivating ?

A	Macrophage elastase
B	Neutrophil elastase
C	Lymphocyte elastase
D	Eosinophil elastase

Question 18 Explanation:

1 AT is the prototypic member of serine protease inhibitor (serpin) superfamily of proteins, which have a major role in inactivating neutrophil elastase to maintain protease-antiprotease balance

Question 19

α1 antitrypsin deficiency, conformational instability of which structure of serpins leads to mutations and polymerisation ?

A	α-sheet
B	β-sheet
C	γ-sheet
D	δ-sheet

Question 19 Explanation:

Conformational instability of the -sheet structure of the serpins underlies their susceptibility to mutations and polymerisation.

Question 20

α1 antitrypsin deficiency can predispose to which of the following liver disorders ?

A	Chronic hepatitis
B	Cirrhosis
C	Hepatoma
D	All of the above

Question 21

α1 antitrypsin deficiency can predispose to which of the following skin disorders ?

A	Pemphigus
B	Panniculitis
C	Psoriasis
D	All of the above

Question 21 Explanation:

1-AT deficiency can predispose to lung disease (emphysema, bronchiectasis), liver disorders (chronic hepatitis, cirrhosis, hepatoma), skin disease (panniculitis), and vasculitis (anticytoplasmic antibody-positive vasculitis - Wegener’s granulomatosis).

Question 22

Emphysema associated with  1 antitrypsin deficiency, distinctive features include ?

A	Early onset (fourth and fifth decade)
B	Panacinar pathology
C	Disproportionate involvement of lung bases
D	All of the above

Question 22 Explanation:

Emphysema associated with 1-AT deficiency is early onset (IV & V decades), panacinar pathology, & disproportionate emphysematous involvement of lung bases (compared with more apical distribution seen in 1 AT replete COPD)

Question 23

In α1 antitrypsin deficiency, retained 1 antitrypsin aggregates are found in which orgenelle of hepatocytes as inclusions ?

A	Endoplasmic reticulum
B	Mitochondria
C	Golgi apparatus
D	All of the above

Question 24

Which amino acid is essential in the reactive center of 1- antitrypsin to inhibits neutrophil elastase ?

A	Serine
B	Methionine
C	Threonine
D	Lysine

Question 24 Explanation:

Inhibition is initiated by docking of protease with serpin. Alpha1-antitrypsin with a methionine at its reactive center inhibits neutrophil elastase.

Question 25

Which of the following increase the risk of COPD in PiZ subjects ?

A	Asthma
B	Male gender
C	Cigarette smoking
D	All of the above

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See all quizzes of Obstructive Pulmonary Disease at here:

Chronic Obstructive Pulmonary Disease- Part 1| Chronic Obstructive Pulmonary Disease- Part 2 | Chronic Obstructive Pulmonary Disease- Part 3

1.COPD includes ?
A. Emphysema
B. Chronic bronchitis
C. Small airways disease
D. All of the above

2. Asthma, chronic bronchitis and emphysema are variations of the same basic disease is hypothesised by ?
A. American hypothesis
B. British hypothesis
C. Canadian hypothesis
D. Dutch hypothesis

3.Obstructive lung disease is diagnosed if ?
A. FEV1 / FVC < 0.7
B. FEV1 / FVC > 0·7
C. FVC / FEV1 < 0·7
D. FVC / FEV1 > 0·7

4. Which allele is associated with normal 1 antitrypsin levels ?
A. S
B. M
C. Z
D. Null

5. Which allele is associated with markedly reduced 1 antitrypsin levels ?
A. S
B. M
C. Z
D. Null

6. 1AT deficiency.   1 antitrypsin is mainly produced in ?
A. Lung
B. Liver
C. Kidney
D. Spleen

7. 1 antitrypsin is the prototypic member of which superfamily of proteins ?
A. Glutamine protease inhibitor
B. Methionine protease inhibitor
C. Threonine protease inhibitor
D. Serine protease inhibitor
8. Mutation that causes severe 1-antitrypsin deficiency arises in which gene ?
A. SERPINA 1
B. SERPINA 2
C. SERPINA 3
D. SERPINA 4

9. Risk for emphysema increases when level of serum 1-AT is ?
A. < 11 µmol/L
B. < 21 µmol/L
C. < 31 µmol/L
D. < 41 µmol/L

10. SERPINA1 gene was formerly known as ?
A. PR
B. PI
C. PX
D. PZ

11. 1 antitrypsin deficiency is inherited as ?
A. Autosomal co-dominant
B. Autosomal recessive
C. X linked
D. None of the above
12. SERPINA1 gene is located on ?
A. Long arm of chromosome 12
B. Long arm of chromosome 13
C. Long arm of chromosome 14
D. Long arm of chromosome 15

13. Normal 1-antitrypsin concentrations in serum is ?
A. 5 – 13 mol/L
B. 13 – 20 mol/L
C. 20 – 53 mol/L
D. 65 – 90 mol/L
14. Normal 1-antitrypsin concentrations in serum is ?
A. 15 – 50 mg/dL
B. 50 – 150 mg/dL
C. 150 – 350 mg/dL
D. 350 – 550 mg/dL

15. Dysfunctional variants leading to abnormal function of 1 antitrypsin can cause ?
A. Emphysema
B. Cirrhosis liver
C. Bleeding diathesis
D. All of the above
16. Dysfunctional variants leading to abnormal function of 1 antitrypsin can cause ?
A. c-ANCA-positive vasculitis
B. Cirrhosis liver
C. Bleeding diathesis
D. All of the above

17. Testing for 1 antitrypsin concentration in serum is done by ?
A. Nephelometry

B. Rocket immunoelectrophoresis
C. Radial immunodiffusion
D. All of the above

18.    1 antitrypsin plays a major role in inactivating ?
A. Macrophage elastase
B. Neutrophil elastase
C. Lymphocyte elastase
D. Eosinophil elastase

19. 1 antitrypsin deficiency, conformational instability of which structure of serpins leads to mutations and
polymerisation ?
A. -sheet
B. -sheet
C. -sheet
D. -sheet

20. 1 antitrypsin deficiency can predispose to which of the following liver disorders ?
A. Chronic hepatitis
B. Cirrhosis
C. Hepatoma
D. All of the above

21. 1 antitrypsin deficiency can predispose to which of the following skin disorders ?
A. Pemphigus
B. Panniculitis
C. Psoriasis
D. All of the above
22. Emphysema associated with  1 antitrypsin deficiency, distinctive features include ?
A. Early onset (fourth and fifth decade)
B. Panacinar pathology
C. Disproportionate involvement of lung bases
D. All of the above

Obstructive Pulmonary Disease

[MCQ] Chronic Obstructive Pulmonary Disease- Part 1

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Chronic Obstructive Pulmonary Disease- Part 1

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