Bronchiectasis Flashcards

(102 cards)

1
Q

What is the primary definition of bronchiectasis?

A

The permanent and irreversible dilation, or ectasia, of the large airways or bronchi.

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2
Q

What diagnostic imaging modality is considered the most accurate for diagnosing bronchiectasis?

A

High-resolution computed tomography (HRCT).

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3
Q

What are the three morphological classifications of bronchiectasis based on radiographic patterns?

A

Cylindrical, varicoid (or varicose), and cystic.

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4
Q

The proposed pathogenic model in bronchiectasis involving airway epithelial dysfunction, chronic infections, inflammation, and airway injury is known as the _____.

A

vicious vortex (or vicious cycle)

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5
Q

What is the main inflammatory cell type that infiltrates the airways in bronchiectasis, releasing proteolytic enzymes like neutrophil elastase?

A

Neutrophils.

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6
Q

According to the ERS guidelines, what is the minimum bundle of etiological tests suggested for all adults with a new diagnosis of bronchiectasis?

A

Differential blood count, serum immunoglobulins (IgG, IgA, IgM), and testing for allergic bronchopulmonary aspergillosis (ABPA).

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7
Q

What is the primary clinical manifestation that drives progression, healthcare costs, and mortality in bronchiectasis?

A

Exacerbations.

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8
Q

According to ERS guidelines, long-term antibiotic treatment should be considered for adults with bronchiectasis who have how many exacerbations per year?

A

Three or more exacerbations per year.

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9
Q

For a bronchiectasis patient with ≥3 exacerbations/year and chronic P. aeruginosa infection, what is the suggested first-line long-term antibiotic treatment according to ERS guidelines?

A

An inhaled antibiotic.

INDICATIONS to long term ABO (provided with >3 exacerbations/year):

  • Chronic P.aeruginossa infection + bronchiectasis: INHALED ABO
  • Chronic PA (p. aeruginosa) infection + bronchiectasis + inc.

exacerbation despite inhaled ABO: MACROLIDES (AZ, Erythromycin)

  • Non-pseudomonas + bronchiectasis + contraindication with macrolides or not effective or not tolerated: ORAL ABO accdg. To susceptibility & tolerance
  • Non pseudo + bronchiec + Oral ABO prophylaxis is contraindicated: INHALED ABO
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10
Q

For a bronchiectasis patient with ≥3 exacerbations/year not infected with P. aeruginosa, what class of oral antibiotics is suggested as the first-line long-term treatment by ERS guidelines?

A

Macrolides (e.g., azithromycin, erythromycin).

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11
Q

What is the term for bronchiectasis for which no known predisposing cause is identified after a thorough evaluation?

A

Idiopathic bronchiectasis.

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12
Q

Which mucoactive agent, an inhaled dry powder, has shown promise in airway clearance for bronchiectasis patients but may be difficult to tolerate?

A

Mannitol.

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13
Q

What was the outcome of trials using recombinant human deoxyribonuclease (rhDNase) in patients with idiopathic bronchiectasis?

A

It failed to reduce exacerbations and showed a trend toward more frequent and severe exacerbations.

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14
Q

In bronchiectasis, transmural inflammation causes damage, making airways susceptible to chronic colonization by microorganisms such as Aspergillus, NTM, and particularly _____.

A

Pseudomonas aeruginosa

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15
Q

The Bronchiectasis Severity Index (BSI) and the FACED score are tools used for what purpose?

A

To assess long-term prognosis and predict mortality and hospitalization risk in non-CF bronchiectasis.

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16
Q

What does the ‘F’ in the FACED prognostic score for bronchiectasis stand for?

A

FEV1 (Forced Expiratory Volume in 1 second).

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17
Q

What is the most common immunodeficiency associated with bronchiectasis?

A

Common Variable Immunodeficiency (CVID).

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18
Q

The diagnosis of Common Variable Immunodeficiency (CVID) is characterized by reduced serum levels of IgG, IgA, and/or IgM, along with what other key finding?

A

Reduced or absent antibody production in response to a specific antigen challenge (vaccination).

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19
Q

What is the classic triad of symptoms associated with Kartagener syndrome, a subtype of Primary Ciliary Dyskinesia (PCD)?

A

Situs inversus, chronic sinusitis, and bronchiectasis.

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20
Q

What is the diagnostic criterion for Allergic Bronchopulmonary Aspergillosis (ABPA) in a patient with asthma or CF, regarding total serum IgE levels?

A

A total serum IgE level greater than 1000 IU/mL.

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21
Q

When is surgical resection considered a management option for bronchiectasis?

A

In carefully selected patients with severe, localized disease refractory to medical therapy or for complications like life-threatening hemoptysis.

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22
Q

What is the general recommendation from the ERS guidelines regarding the use of inhaled corticosteroids (ICS) in bronchiectasis patients who do not have a clear history of asthma or COPD?

A

There is insufficient evidence to recommend for or against their use, and further trials are needed.

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23
Q

According to epidemiological data, the prevalence of non-CF bronchiectasis increases substantially with _____, reaching over 800 cases per 100,000 for ages ≥75 years.

A

age

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24
Q

The ERS guidelines suggest that in bronchiectasis patients with chronic P. aeruginosa infection who cannot tolerate inhaled antibiotics, what is the next therapeutic option?

A

Long-term treatment with macrolides.

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25
In a patient with bronchiectasis not infected with *P. aeruginosa*, if macrolides are contraindicated or ineffective for long-term prophylaxis, what is the next suggested option by ERS guidelines?
A different long-term oral antibiotic, based on susceptibility and tolerance.
26
What is the typical distribution of bronchiectasis in patients with cystic fibrosis or post-tuberculosis infection?
Upper zone predominant.
27
What is the typical distribution of bronchiectasis in patients with Primary Ciliary Dyskinesia (PCD) or idiopathic cases?
Middle and/or lower zone predominant.
28
Central, large-airway bronchiectasis is a characteristic finding in which condition?
Allergic Bronchopulmonary Aspergillosis (ABPA).
29
In the diagnostic workup for Primary Ciliary Dyskinesia (PCD), what is the traditional 'gold standard' test that examines the ultrastructure of cilia?
Transmission electron microscopy (TEM).
30
What is a major limitation of using transmission electron microscopy (TEM) to diagnose Primary Ciliary Dyskinesia (PCD)?
Approximately 30% of PCD patients have normal or near-normal ciliary ultrastructure.
31
What clinical scoring tool was developed to help clinicians screen for Primary Ciliary Dyskinesia (PCD) in patients with chronic productive cough?
The Primary Ciliary Dyskinesia Rule (PICADAR) Situs inversus, neonatal respiratory distress, congenital cardiac defect, chronic rhinitis, ear symptoms Majority of mutations in PCD are either deletion or nonsense mutations of the following ciliary proteins ● DNAI1 ● DNAI2 ● DNAH5 PCD patients with normal or near-normal cilia ultrastructure ● Mutation of DNAH11.
32
Quality of life impairment in bronchiectasis, as measured by the SGRQ, is equivalent to what other severe respiratory disease?
Severe Chronic Obstructive Pulmonary Disease (COPD).
33
What effect did a meta-analysis of oral mucolytics, such as N-acetylcysteine, show in patients with bronchiectasis?
It was effective at reducing exacerbations, although the trial was small and requires confirmation.
34
In patients with bronchiectasis, using hypertonic saline 7% for airway clearance showed reductions in what key outcome?
Healthcare utilization.
35
When testing a patient for CVID, assessing antibody responses to the 23-valent pneumococcal vaccine (Pneumovax) evaluates B cell responses that are independent of what?
T cell help.
36
What is a key difference in the pathogenesis of bronchiectasis in CVID compared to PCD?
CVID involves an acquired or genetic defect in the immune system (antibody production), while PCD involves a genetic defect in ciliary structure and function.
37
If a patient with asthma fulfills all criteria for ABPA except for the presence of bronchiectasis on HRCT, what is the diagnosis?
Seropositive ABPA (ABPA-S).
38
What is the purpose of airway hygiene and hyperosmotic agents like hypertonic saline in the management of bronchiectasis?
To aid in mucus clearance from the airways.
39
A study of high-dose budesonide combined with formoterol for bronchiectasis showed that the combination therapy allowed for a lower dose of _____ while improving dyspnea and QOL.
inhaled corticosteroid (ICS)
40
The 'vicious vortex' of bronchiectasis leads to airway infiltration by neutrophils and macrophages, which release pro-inflammatory cytokines such as tumor necrosis factor-α and _____.
interleukin-8 (IL-8)
41
In addition to age and FEV1, the BSI score for bronchiectasis incorporates what factor related to exacerbations?
Prior hospitalizations and frequency of exacerbations.
42
The ERS guidelines for bronchiectasis are based on systematic literature reviews and were developed with input from a committee that disclosed all potential _____.
conflicts of interest
43
In the ERS bronchiectasis guidelines, a 'conditional recommendation' implies what about the course of action for clinicians and patients?
Most patients should receive the recommended action, but different choices may be appropriate for individual patients, requiring shared decision-making.
44
Why is it important to obtain sputum cultures in patients with bronchiectasis, particularly those with frequent exacerbations?
To identify colonizing organisms (like *P. aeruginosa* or NTM) and guide targeted antimicrobial therapy.
45
In CVID, recurrent infections with encapsulated bacteria, such as *H. influenzae* and _____, are a key driver of bronchiectasis development.
S. pneumoniae
46
Some longitudinal studies have described up to a 30% mortality at 1-year follow-up after a patient with bronchiectasis suffers what event?
An exacerbation.
47
The economic burden of bronchiectasis has been estimated to be similar to that of which other major chronic respiratory disease?
COPD.
48
Why were specific guidelines for non-CF bronchiectasis necessary, rather than just extrapolating from cystic fibrosis management?
Clinical trials and experience have shown that treatment responses are different between the two conditions.
49
A meta-analysis of surgical interventions for bronchiectasis showed that complete alleviation of symptoms was seen in approximately what percentage of adult patients?
71.5%.
50
What two factors were reported as unfavorable prognostic factors for surgical outcomes in bronchiectasis?
Extent of residual bronchiectasis and *P. aeruginosa* infection.
51
What is a potential adverse event that requires a tolerance test before initiating therapy with inhaled mannitol or hypertonic saline in bronchiectasis?
Bronchospasm or a significant decrease in FEV1.
52
The ERS guidelines suggest that in a patient with bronchiectasis and chronic *P. aeruginosa* infection who still has frequent exacerbations despite inhaled antibiotics, what is a potential next step?
Adding or switching to long-term macrolides.
53
While chest radiographs may suggest bronchiectasis through findings like thickened bronchial walls ('tram tracks'), what is the definitive diagnostic test?
High-resolution computed tomography (HRCT).
54
The PICADAR scoring system uses seven clinical parameters to estimate the probability of what underlying cause of bronchiectasis?
Primary Ciliary Dyskinesia (PCD).
55
For which cause of bronchiectasis would genetic testing for mutations in the CFTR gene be indicated?
Cystic Fibrosis (CF).
56
What is the significance of chronic *P. aeruginosa* colonization in a patient with bronchiectasis?
It is associated with more frequent and severe exacerbations, accelerated lung function decline, and increased mortality.
57
What is the rationale for using long-term macrolide therapy in non-*P. aeruginosa* bronchiectasis?
Macrolides have both anti-bacterial and anti-inflammatory/immunomodulatory properties that can reduce exacerbations.
58
What non-pharmacological intervention is considered a cornerstone of bronchiectasis management to facilitate sputum clearance?
Airway clearance techniques (e.g., chest physiotherapy, oscillating PEP devices).
59
What role does pulmonary rehabilitation play in the management of bronchiectasis?
It improves exercise capacity, reduces dyspnea, and enhances quality of life, similar to its role in COPD.
60
In addition to reduced immunoglobulins, a diagnosis of CVID requires demonstrating impaired response to what?
Vaccines (specific antigen challenge).
61
If a bronchiectasis patient has a history of situs inversus, what underlying condition should be strongly suspected?
Primary Ciliary Dyskinesia (PCD).
62
According to the ERS guidelines, additional etiological tests beyond the minimum bundle may be appropriate in patients with bronchiectasis who have what characteristics?
Specific clinical features suggesting an underlying cause, or severe or rapidly progressive disease.
63
In the 'vicious vortex' of bronchiectasis, chronic infection and inflammation lead to permanent airway injury and dilation, which in turn results in poor _____.
mucociliary clearance
64
Besides *P. aeruginosa*, what other type of organism is a common colonizer in bronchiectatic airways and requires specific culture techniques for identification?
Nontuberculous mycobacteria (NTM).
65
The justification for the ERS recommendation on mucoactive agents notes that while there's a small improvement in time to first exacerbation, many patients will not experience a _____.
significant benefit
66
What is the primary reason that treatment approaches for bronchiectasis cannot simply be extrapolated from cystic fibrosis bronchiectasis?
Randomized clinical trials have demonstrated that treatment responses are different.
67
In addition to chronic cough and sputum, what are four other common symptoms of bronchiectasis?
Breathlessness, rhinosinusitis, fatigue, and haemoptysis.
68
What is Mounier-Kuhn syndrome characterized by?
Tracheobronchomegaly (markedly dilated trachea and central bronchi) associated with bronchiectasis.
69
What is Williams-Campbell syndrome?
A congenital disorder characterized by a deficiency of cartilage in the 4th to 6th order bronchi, leading to distal airway collapse and severe bronchiectasis.
70
In the BSI prognostic score for bronchiectasis, what does the 'S' represent?
Severity, which incorporates factors like prior hospitalization and exacerbation frequency.
71
The ERS guidelines suggest that an experienced surgeon and a(n) _____ should be involved if surgical treatment for bronchiectasis is being considered.
expert respiratory physician
72
What is a major component of the morbidity in bronchiectasis, aside from respiratory symptoms?
Impaired quality of life, which can be as severe as in advanced COPD or IPF.
73
A 22 year old female consulted your clinic due to recurrent lower respiratory tract infection since childhood. CT scan showed dilatation of the central airway with incidental finding of an extraordinary dilatation of the trachea measuring 22 x 24 mm. The most likely diagnosis for this condition is? Choose the best answer Answer A. Williams-Campbell Syndrome B. Swyer-James-MacLeod Syndrome C. Mounier-Kuhn Syndrome D. Ehler-Danlos Syndrome
Mounier-Kuhn Syndrome; Chapter 69, page 952
74
This is considered as the most common immunodeficiency associated with bronchiectasis? Autosomal Dominant Hyper Ig-E Syndrome B. Common Variable Immunodeficiency C. Chronic Granulomatous Disease D. Wiskott-Aldrich Syndrome
CVID
75
A 50 year old asthmatic female is a known case of bronchiectasis with history of recurrent infection and expectoration of thick brown mucous. On work up, she was found to have Allergic Bronchopulmonary Aspergillosis. Which of the following is considered an essential criteria in diagnosing her condition? Choose the best answer Answer A. Total eosinophil count > 500 cells/µl in patients not on recent corticosteroids B. Pulmonary opacification & bronchiectasis consistent with ABPA C. Presence of IgG against A. fumigatus D. Total IgE >1000 IU/ml
D. Total IgE >1000 IU/ml
76
55 year old male with previous history of pulmonary tuberculosis came to your clinic due to cough for more than a year with hemoptysis for the past three weeks. Chest radiograph revealed hyperinflated lungs with extensive bilateral ring shadows in the right middle lobe and atelectasis in the right lower lobe. The diagnostic test that you would request next is? Choose the best answer Answer A. Sputum culture B. Bronchoscopy C. CT scan D. Pulmonary Function Test
C
77
A 65 year old male, non-smoker, had pulmonary tuberculosis five years ago. For the past three years, he had recurrent cough with occasional blood tinged phlegm. On work up, he was diagnosed to have bronchiectasis. Which of the following is considered as the main mechanism of bronchiectasis secondary to tuberculosis? Choose the best answer Answer A. Local retractile forces dilate the airways B. Primary weakness of airways C. Secondary loss of structural integrity D. Intense inflammation originating in the lumen
A. Local retractile forces dilate the airways
78
Which of the following is considered the most common risk factor for aspiration resulting to bronchiectasis? Choose the best answer Answer A. Post surgery B. Gastroesophageal reflux C. Cerebro-vascular accident D. Alcohol abuse
GER
79
8. A patient diagnosed with bronchiectasis came to your clinic for second opinion. He was recently given intermittent macrolide antibiotics by a general practitioner claiming that it has the ability to inhibit disease activity in bronchiectasis. You assured the patient that taking the medication could help his condition. Which of the following is the mechanism by which intermittent macrolide therapy may help in bronchiectasis? Choose the best answer Answer A. Neutrophil degradation of fibronectin B. Accelerate mucus clearance C. Inhibition of neutrophil elastase D. Decreasing the chloride diffusion gradient across airway mucosa
Deceasing the chloride diffusion gradient across airway mucosa
80
A young adult male is a known case of bronchiectasis referred to you due to recurrent infection. On work up, he had an abnormal alleles for the CTFR gene and was found to have a concomitant aspergillus lung disease. Which of the following is the most likely organism involved in his condition? Choose the best answer Answer A. Adenovirus B. Mycobacterium avium complex C. Pseudomonas aeruginosa D. Moraxella catarrhalis
C. Pseudomonas aeruginosa Regardless of the underlying cause, P. aeruginosa is repeatedly identified as a critical driver of poor outcomes in severe bronchiectasis: * It is one of the two most frequently isolated chronic pathogens in adult bronchiectasis (along with Haemophilus influenzae). * Its presence signals severe disease and poor prognosis, playing a major role in pathogenesis and accelerating clinical decline. * Chronic P. aeruginosa infection is specifically associated with worse quality of life, increased frequency of exacerbations, more hospital admissions, and an approximately threefold increased risk of mortality
81
The most common idiopathic inflammatory disorder associated with bronchiectasis: Choose the best answer Answer A. Ankylosing spondylitis B. Rheumatoid arthritis C. Sarcoidosis D. Sjogren syndrome
C
82
A young adult male was referred to your clinic due to recurrent productive cough. On past medical history, he had recurrent episodes of bronchitis at a young age with noted chronic rhinitis leading to ear infection. Chest radiograph showed situs invertus with signs of cystic distortion of the distal airways attributed to bronchiectasis. Which of the following is the most likely diagnosis for this patient? (Chapter 69, page 950) Choose the best answer Answer A. Autosomal Dominant Hyper IgE Syndrome B. Young Syndrome C. Marfan’s Syndrome D. Primary Ciliary Dyskinesia
D A. Autosomal Dominant Hyper IgE Syndrome (AD-HIES): This primary immunodeficiency is characterized by recurrent infections (especially S. aureus), leading to bronchiectasis and pneumatoceles, but it is not specifically associated with situs invertus. * B. Young Syndrome: This condition is defined by bronchiectasis, sinusitis, and infertility due to obstructive azoospermia, and is only seen in men. While the patient is male and has sinopulmonary issues, Young syndrome is not characterized by situs invertus. * C. Marfan’s Syndrome: This heritable connective tissue disorder is associated with bronchiectasis and other lung findings like emphysema, but situs inversus is a distinguishing feature of PCD related to ciliary function, not Marfan syndrome related to fibrillin mutation
83
Your patient with long-time bronchiectasis comes to you with shortness of breath, cough productive of copious purulent phlegm, and fever. This is her third exacerbation this year. During her previous exacerbations, sputum cultures grew Pseudomonas aeruginosa. What is the first line treatment recommendation for her? (ERS Bronchiectasis 2017) A. Oral antibiotics based on pathogen susceptibility and patient tolerance B. Macrolides C. Inhaled antibiotic D. Macrolides + inhaled antibiotic
C. Inhaled antibiotic
84
How many days of antibiotic treatment are recommended for patients with acute exacerbation of bronchiectasis? (ERS Bronchiectasis 2017) A. 7 days B. 14 days C. 21 days D. 3 months
14
85
A 48-year-old Filipino patient is referred to you for evaluation of bronchiectasis. Pertinent in the history is asthma that is difficult to control without systemic steroids. Wheezing can be heard on auscultation, but the complete physical exam is otherwise unremarkable. He is a non-smoker. He claims to have been relatively healthy previous to this. What diagnostic test would be most helpful in clinching the etiology of the bronchiectasis? (Murray Chapter 69 p947) A. Sweat chloride test B. Nasal nitric oxide test C. RF/autoantibodies D. Total IgE
D
86
Which of the following is the recommended sequential airways treatment administration in an adult patient with bronchiectasis? (ERS Bronchiectasis 2017) A. Short/long-acting bronchodilators, then mucolytic/physiotherapy, then airway clearance, then inhaled antibiotics B. Airway clearance, then short/long-acting bronchodilators, then inhaled antibiotics, then mucolytic/physiotherapy adjuncts C. Mucolytic/physiotherapy adjuncts, then inhaled antibiotics, then short/long-acting bronchodilators, then airway clearance D. Inhaled antibiotics, then airway clearance, then mucolytic/physiotherapy, then short/long-acting bronchodilators
A. Short/long-acting bronchodilators, then mucolytic/physiotherapy, then airway clearance, then inhaled antibiotics
87
What is an inaccurate statement regarding the typical clinical patterns of bronchiectasis? Choose the best answer A.Bronchiectasis involving dependent zones tend to entail frequent infections. B.Bronchiectasis localized to the upper lobe tend to be “dry”. C.Honeycombing seen in chest x-rays and chest CT scans heralds the presence of concomitant interstitial lung diseases. D.Hemoptysis may be the only presenting symptom.
C
88
Pulsion bronchiectasis is seen in Choose the best answer A.Williams Campbell syndrome B.Relapsing polychondritis C.Marfan syndrome D.Allergic bronchopulmonary aspergillosis
D Pulsion Bronchiectasis: This type of bronchiectasis implies that intense inflammation originating in the lumen leads to permanent airway dilation Williams-Campbell syndrome (A): This is an example of bronchiectasis due to weakness of the airways, arising from the absence of cartilaginous rings in the fourth- to sixth-generation subsegmental bronchi. * Relapsing polychondritis (B): This is characterized by inflammation, weakness, and deformity of cartilaginous structures in the airways, which contributes to bronchiectasis. This falls under the category of weakness of the airways. * Marfan syndrome (C): This is a heritable connective tissue disorder that may be associated with bronchiectasis due to weakness of the airways, likely stemming from mutations in the fibrillin 1 (FBN1) gene
89
The vicious cycle in the development of bronchiectasis is thought to be due to the following pathology of the airway: Choose the best answer A.Traction B.Collapsibility C.Chronic bacterial colonization D.All of the above
C
90
Irreversible damage leading to bronchiectasis as a result of a discrete episode of pneumonia in childhood has been ascribed to the following etiology: Choose the best answer A.Bordetella pertussis B.Influenza C.Streptococcus pneumonia D.Any of the above
Any rreversible damage leading to bronchiectasis as a result of a discrete episode of pneumonia in childhood has been ascribed to all the listed pathogens: * Bordetella pertussis: Specific pathogens believed to cause bronchiectasis include Bordetella pertussis. * Influenza: Irreversible damage leading to bronchiectasis has been ascribed to influenza. * Streptococcus pneumoniae: Specific pathogens believed to cause bronchiectasis include mucoid strains of S. pneumoniae. More generally, a commonly held view is that many cases of bronchiectasis in adults are associated with childhood bouts of pertussis, measles, or other infections, including adenovirus. Such childhood infections, if inadequately treated, presumably produced irreversible damage leading to bronchiectasis
91
Which statement accurately describes the association of cystic fibrosis (CF) and bronchiectasis? Choose the best answer A.Majority of those with childhood-onset CF who survive into adulthood will manifest with bronchiectasiss B.Among adult patients with nontuberculous bronchiectasis, the majority of patients will have an abnormality in the CFTR gene. C.Disease of the conducting airways is acquired prenatally. D.Bronchiectatic cysts occupy <20% of the cross-sectional area of the late-stage CF lung.
A.Majority of those with childhood-onset CF who survive into adulthood will manifest with bronchiectasiss C is wrong because he genetic defect (abnormal CFTR alleles) is inherited, but the resulting structural airway disease (bronchiectasis) is acquired through a post-natal "vicious cycle". The cycle begins when mucus abnormality and impaired mucociliary clearance lead to persistent infections and inflammation, which subsequently cause the irreversible dilation of the airways
92
he preferred mucus-mobilizing agent in the management of bronchiectasis due to cystic fibrosis
Dornase alfa
93
Which statement accurately describes the role of resectional surgery in bronchiectasis? Choose the best answer A. Systematic studies have shown that it is effective in the majority of patients. B.It is the initial treatment of choice in patients with massive hemoptysis. C.Disease limited to the right middle lobe or lingula are amenable to resectional surgery. D.Studies have shown better results and less morbidity with video-assisted thoracoscopic technique compared to lateral thoracotomy.
C
94
Type of bronchiectasis described as failure of the involved airways to taper progressively in their distal course
Cylindrical Type of bronchiectasis described as failure of the involved airways to taper progressively in their distal course (p.853). a. Cylindrical - FAILURE OF THE INVOLVED AIRWAY TO TAPER PROGRESSIVELY IN THEIR DISTAL COURSE. THE BRONCHIAL WALLS ARE SMOOTH OR REGULAR. – MURRAY 7TH EDITION PAGE 941 b. Varicoid - VARICOID BRONCHIECTASIS IS AN ALLUSION TO VARICOSE VEINS AND IS MARKED BY IRREGULAR DILATION, NARROWING, AND OUTPOUCHING OF THE AIRWAYS. PDE EXAMPLE SA MONUIER KAHN SYNDROME, WILLIAM CAMPBELL DISEASE c. Saccular – SAME AS CYSTIC d. Cystic- SACCULAR BRONCHIECTASIS, ALSO KNOWN AS CYSTIC BRONCHIECTASIS, INCLUDES FOCAL OR CYSTIC DISTORTION OF THE DISTAL AIRWAYS.
95
30-year old, female came in with a history of early onset-recurrent lower respiratory infections. High-resolution CT showed diffuse, poorly defined flocculent centrilobular opacities in the lower lobes. What’s the most probable diagnosis of the patient? (p.862
PCD Cystic Fibrosis UPPER LOBE ANG INVOLVEMENT (OTHER UPPER LOBE INVOLVEMENT: CYSTIC FIBROSIS, TUBERCULOSIS, SARCOIDOSIS, SILICOSIS). RULED OUT NA AGAD PAG BABAE ANG PATIENT. YOUNG SYNDROME AND CYSTIC FIBROSIS PANG LALAKE BOTH PRESENTATION IS AZOOSPERMIA CYSTIC FIBROSIS – CONGESTINAL ABSCENSE OF VAS DEFERENS YOUNG SYNDROME – OBSTRUCTION IN DISTAL EPIDIDYMIS AUTOSOMAL RECESSIVE 2 b. Yellow Nail Syndrome TRIAD OF YELLOW, THICK, DYSTROPHIC NAILS; CHRONIC LYMPHEDEMA OF THE FACE, HANDS AND LOWER EXTREMETIES AND PLEURAL EFFUSION (EXUDATIVE – MOST PROMINENT FINDINGS) c. Primary Ciliary Dyskinesia – FEMALE, REDUCED FERTILITY, KARTAGENER SYNDROME SITUS INVERSUS OR PARTIALIS, LOWER LUNG ZONE PREDOMINANT KAYA ITO ANG SAGOT PERO PDE DIN MIDDLE LOBE. PAGE 947 AND 950 MURRAY 7TH EDITION NASAL NITRIC OXIDE TEST – SCREENING TEM – GOLD STANDARD FOR CONFIRMATION d. Young’s Syndrome – SEEN ONLY IN MEN. AZOOSPERMIA
96
52-year old, female came in due to fever and purulent sputum. History revealed that she was previously treated for PTB and had recurrent pneumonia. HCRT showed bronchiectasis involving the lower lobes, right middle lobe and the lingular segment of the left upper lobe. She was given Piperacillin-Tazobactam, based on the available culture results. What type of antibiotic administration approach was employed in this case? (p.871) A. Initial empiric antibiotic B. Initial directed antibiotic C. Episodic, targeted antibiotics D. Rotating antibiotics
C
97
The following are pathogens that can cause bronchiectasis except for? a. Bordetella pertussis b. Staphylococcus aureus c. Histoplasma capsulatum d. Rubeola e. All can cause bronchiectasis
AOTA Pathogens that can cause bronchiectasis ● Bordetella pertussis ● Streptococcus pneumoniae ● Staphylococcus aureus ● Klebsiella pneuoniae ● Adenoviruses ● Rubeola (measles) ● Influenza Chronic granulomatous pathogens related to bronchiectasis ● Mycobacterium tuberculosis ● Histoplasma capsulatum ● NTM: Mycobacterium avium complex (MAC)
98
What is the most prominent cellular defect in common variable immunodeficiency disease (CVID) that is associated with bronchiectasis?
Failure of immature B cells to differentiate into memory B cells and plasma cells
99
Which of the following statements is INCORRECT about mounier-kuhn syndrome? a. Underlying defect is the atrophy and absence of elastic fibers and smooth muscles of the large aiways b. Diagnosis in men is CT scan transverse tracheal diameter of >25mm and sagittal tracheal diameter of >27mm c. Diagnosis in women is CT scan transverse tracheal diameter of >21mm and sagittal tracheal diameter of >23mm d. Presents during the 4 th decade of life e. Management includes PEEP support and/or stenting
D May present in EARLY CHILDHOOD OR 4 THDECADE
100
The following are lung disorders associated with marfan syndrome EXCEPT a. Distal acinar emphysema b. Spontaneous pneumothorax c. Upper lobe hypoplasia d. Cystic degeneration e. Apical fibrosis
C Must be middle lobe hypoplasia
101
The following statements are true of yellow nail syndrome EXCEPT for a. Triad of yellow, thick, dystrophic nails; chronic lymphedema of the face, hands, and lower extremities; and pleural effusion b. Bilateral transudative pleural effusion is the most prominent pulmonary findings c. Women are more commonly affected d. Recurrent lower respiratory tract infection is common
Bilateral transudative pleural effusion is the most prominent pulmonary findings --- - Bilateral EXUDATIVE pleural effusion is the most prominent pulmonary findings
102
Vicious Vortex?
1. airway epithelial and ciliary dysfunction, and mucus hypersecretion; (2) chronic infections that induce further mucus hypersecretion; (3) inflammation, resulting in permanent airway injury and dilation; and (4) resultant bronchiectatic airways that are poor in mucociliary clearance, perpetuating the chronic infection, inflammation, and airway epithelial cell and ciliary dysfunction