Respiratory Flashcards

(74 cards)

1
Q

Name 4 organisms that commonly cause respiratory infections in patients with cystic fibrosis.

A

Staph aureus

Pseudomonas aeruginosa

Burkholderia cepacia

Aspergillus

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2
Q

What is the Epworth Sleep Scale out of, and what is considered normal?

A

Out of 24; up to 10 is normal

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3
Q

What are the three key features of obstructive sleep apnoea syndrome?

A

Snoring

Apnoeas

Daytime somnolence

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4
Q

How do you distinguish obstructive sleep apnoea from central sleep apnoea?

A

CSA - periods of absence of respiratory effort, versus attempts to overcome obstructed upper airway

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5
Q

What happens in REM sleep that results in less use of respiratory ventilatory muscles and decreased sats?

A

Skeletal muscle paralysis - this also stops you acting out your dreams during REM sleep

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6
Q

Name 8 conditions associated with restless leg syndrome.

A

Iron deficiency

ADHD

Coeliac disease

COPD

Depression/panic disorders

Fibromyalgia

Parkinson’s disease

Multiple sclerosis

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7
Q

Name 5 (groups of) medications that can cause restless legs syndrome?

A

SSRIs

Neuroleptics

Lithium

Beta blockade

Dopamine antagonists

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8
Q

Following conservative treatments, what medications can you give for restless legs syndrome? Name 7.

A

Iron replacement

Levodopa

Ropinirole

Clonazepam

Tramadol

Oxycodone

Gabapentin

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9
Q

What is the pathophysiology of narcolepsy, and what are the two different types?

A

Acquired deficiency of orexin (hypocretin) in key hypothalamic neurons

Type 1 - narcolepsy with cataplexy

Type 2 - narcolepsy without cataplexy

Diagnosed by REM intrusions on PSG

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10
Q

How is narcolepsy treated? Name 3 drugs.

A

Modafinil - treats narcolepsy but not cataplexy

Methylphenidate - treats both

Dexamphetamine - treats both

Anti-cataplectic include venlafaxine and clomipramine

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11
Q

Name 7 absolute contraindications for receiving a lung transplant?

A

Recent history of malignancy

Major organ dysfunction

Chronic infection with virulent or resistant microbes that are poorly controlled pre-Tx

BMI > 35

Current or repeated non-adherence to treatment

Severely limited functional status with poor rehabilitation potential

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12
Q

Name two conditions where single lung transplant can be indicated.

A

ILD

COPD

Single lung transplant recipients will generally be older, and they will not have an infection.

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13
Q

Name 4 conditions where bilateral lung transplants can be indicated.

A

Cystic fibrosis

Bronchiectasis

Pulmonary hypertension

Severe infection

Bilateral lung transplant recipients will be young patients

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14
Q

When could COPD patients get listed for a lung transplant? Name 5 stipulations.

A

BODE 7 or greater

FEV 15-20%

3 or more exacerbations in one year

One episode of acute hypercapnic respiratory failure

Moderate to severe pH

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15
Q

When could pulmonary fibrosis patients be listed for a lung transplant? Provide 4 stipulations.

A

Decline in FVC >10% and DLCO >15% in the prior 6 months

Development of pulmonary hypertension

Hospitalisation because of respiratory decline, acute exacerbation or pneumothorax

Significant exercise associated desaturation or requirement for oxygen

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16
Q

When could pulmonary vascular disease patients be listed for a lung transplant? Provide 3 stipulations.

A

NYHA functional class III or IV despite escalating pulmonary vasodilator therapy

Refractory or progressive right heart failure

Right heart catheter measurements of mean right arterial pressure >15 mmHg, cardiac index <2 litres/minute/m2 and mean PAP >50 mmHg

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17
Q

When could cystic fibrosis patients be listed for a lung transplant? Provide 7 stipulations.

A

Frequent hospitalisation

FEV1 <30% of predicted especially if rapid downward trajectory

Increasing antibiotic dependence or resistance

Life-threatening haemoptysis or pneumothorax

Requirement for non-invasive ventilation

Development of pulmonary hypertension

PCO2 > 50 mmHg and/or pO2 <60 mmHg (need for NIV)

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18
Q

What is the greatest limitation to survival in lung transplant patients?

A

Chronic allograft lung dysfunction

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19
Q

Which hypoventilation syndrome has an association with CVD?

A

Central sleep apnoea

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20
Q

Name 4 risk factors for developing CTEPH

A

Pro-coagulant states (antithrombin, protein C and S deficiency)

Lupus anticoagulant and APS

Blood groups A, B and aB

Splenectomy

CTEPH patients require lifelong anticoagulation

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21
Q

What is the definitive treatment for CTEPH?

A

Pulmonary endarterectomy

Procedural mortality is 2-5%, and the operation should be done at an expert centre where 20/year are performed

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22
Q

What sweat chloride test result is diagnostic of cystic fibrosis?

A

> 60 mmol/L

40-60 is in between normal and abnormal

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23
Q

What is the definitive test for primary ciliary dyskinesia?

A

Examination of ciliary ultrastructure under electron microscopy

Expect to see abnormal ultrastructure and slow ciliary beat frequency

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24
Q

How is cystic fibrosis diagnosed?

A

Clinical features of CF or family history of CF

AND EITHER

2 CF-causing mutations OR

Positive sweat chloride on 2 occasions OR

Nasal potential difference tracing typical for CF

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25
What is nasal potential difference?
Test for CF Voltage correlates with movement of Na across a cell membrane; amiloride blocks epithelial Na channels; isoprotenerol stimulates CFTR CF patients have: A more negative baseline potential difference Larger inhibition after addition of amiloride Little or no change with addition of Cl free solution or isoprotenerol
26
What is the inheritance of cystic fibrosis, and what is the most commonly involved gene?
Autosomal recessive Deletion on long arm chromosome 7 Delta F508
27
What is ivacaftor?
Treatment for CF with gene mutation G551D Class 3 defect - CFTR reaches cell surface but channel opening is impaired Class 4 defect - decreased conductance Ivacaftor is a small molecule potentiator of the CFTR channel
28
What is lumacaftor?
CFTR corrector Used in combination with ivacaftor for homozygous Phe508del Small improvement in FEV1 but significant decrease in pulmonary exacerbations
29
What is the benefit of macrolides in cystic fibrosis?
Inhibits biofilm production Modifies mucous production Suppresses inflammatory markers Indicated for CF and non-CF bronchiectasis with pseudomonas or frequent exacerbators without pseudomonas
30
What constitutes significant bronchodilator reversibility on spirometry?
Improvement of 12% and/or 200ml
31
What does exhaled nitric oxide on PFTs help to indicate?
Whether or not there is inflammation present that might be responsive to steroids Correlates with Th2 inflammation and sputum eosinophilia
32
What 4 parameters are used in the GOLD ABCD assessment tool for COPD?
FEV1 (predicted) - 1 is >80, 2 is 50-79, 3 is 30-49, 4 is <30 Exacerbation Hx - A or B is 0 or 1 (not leading to hospital admission), C or D is >2 or >1 leading to hospital admission mMRC questionnaire - A or C or 0 or 1, B or D is >2 CAT questionnaire - A or C is CAT < 10, B or D is CAT >10
33
What are the 4 parameters used for the BODE index in COPD?
BMI Obstruction - FEV1 Dyspnoea - mMRC dyspnoea scale Exercise capacity - six-minute walk distance
34
Which gene is responsible for the onset of alpha-1-antitrypsin deficiency?
SERPINA1
35
What is the pathophysiology of alpha-1-antitrypsin deficiency?
Failure to inhibit neutrophil elastase in lung Build up of AAT polymers in liver cells
36
When is long term oxygen therapy indicated for COPD patients?
Non-smoking stable COPD patients who have: PaO2 < 55 PaO2 < 55-59 with evidence of pulmonary hypertension or polycythaemia Note - no evidence of improved outcomes in COPD patients with moderate hypoxia
37
Name 5 benefits of pulmonary rehabilitation.
Improved exercise capacity Reduced perceived intensity of breathlessness Improved health-related quality of life Reduced hospitalisation and hospital days Reduced anxiety and depression in COPD
38
Name 7 bacteria that can be seen in bacterial exacerbations of COPD.
H influenza Strep pneumoniae M catarrhalis Mycoplasma Chlamydia In more severe disease - Staph aureus, Pseudomonas
39
When would you consider referral for lung transplants in COPD patients? Name 5 considerations.
When the majority of the following are present... Progressive symptoms, despite maximal treatment including medication, pulmonary rehabilitation and oxygen therapy Patient is not a candidate for endoscopic or surgical lung volume reduction surgery BODE index of 5-6 PaCO2 > 50 and/or PaO2 < 60 FEV1 < 25% predicted
40
What is a DECAF score, and what are its constituent parts?
Predictor of in-hospital mortality from COPD exacerbations Dyspnoea Eosinopaenia Consolidation Acid anemia Atrial fibrillation Score of 0-1 low risk; 2 intermediate risk; 3-6 high risk
41
Which vaccinations are recommended for COPD patients?
Influenza Pneumococcal
42
When would you consider adding an ICS to treatment for COPD?
If FEV1 < 50% 2 or more hospitalisations within a year All despite LABA and LAMA therapy
43
Name 4 viruses that can cause viral exacerbations of COPD
Influenza Rhinovirus RSV Metapneumovirus
44
Name the main 3 cell types and cytokines that are involved in the pathogenesis of asthma.
Cells - Th2 cells - mast cells - eosinophils Cytokines - IL-4 - IL-5 - IL-13
45
What are the pathological processes that occur in asthma? Name 5.
Mucosal inflammation Mucoid exudate Goblet cell metaplasia Epithelial basement membrane thickening Smooth muscle hypertrophy, hyperplasia and contraction
46
How is asthma diagnosed on spirometry?
FEV1 increase >200ml and >12% from baseline post bronchodilator
47
What does an increased exhaled nitric oxide test suggest on spirometry?
Suggests steroid-responsive airway disease.
48
What are the stages of treatment for asthma?
Stage 1 - SABA as required Stage 2 - Low dose regular preventer (ICS) + SABA as required Stage 3 - Low dose combo regular preventer (LABA + ICS) + reliever as required - budesonide/formoterol (low dose) maintainence-and-reliever therapy - ICS/LABA combo (low dose) as maintenance Stage 4 - High dose combo regular preventer (LABA + ICS) + reliever as required - budesonide/formoterol maintenance (medium dose) and reliever (low dose) therapy - ICS/LABA combo (moderate-high dose) as maintenance therapy Stage 5 - specialised treatments
49
What is SMART therapy for asthma?
Single Inhaler Maintenance and Reliever Therapy (SMART) Budesonide/formoterol combination inhaler, 2 puffs BD and PRN Used in moderate-to-severe asthma
50
What is omalizumab, and what stipulation is required to use it?
Anti-IgE - mAb that binds the Fc portion of IgE to prevent if from activating the high-affinity IgE receptor on mast cells, basophils and dendritic cells Used in asthma principally Baseline IgE needs to be <1300 IU/mL, otherwise too much IgE to bind
51
What is the main adverse effect of omalizumab?
Anaphylactoid reactions
52
What is mepolizumab?
Anti IL-5 - mAb that reduces production and survival of eosinophils Used in severe asthma; reduces exacerbations and has a steroid-sparing effect
53
Name 2 drugs that can be used for the treatment of IPF?
Pirfenidone Nintedanib
54
Name 4 features on HRCT consistent with usual interstitial pneumonia
Pleural/subpleural predominance Traction bronchiectasis Reticular shadowing Honeycombing
55
Name 6 conditions associated with lymphocytic interstitial pneumonia.
Sjögren’s syndrome Rheumatoid arthritis HIV infection SLE Myasthenia Travis Chronic active hepatitis infections
56
Name 2 features on HRCT of lymphocytic interstitial pneumonia.
Discrete peribronchovascular cysts Varying degrees of ground glass opacities Note - lot of overlap with NSIP, gold standard of diagnosis is biopsy
57
What are the two different types of NSIP that can be seen?
Cellular Fibrotic
58
What features would you expect to see on HRCT with hypersensitivity pneumonitis? Name 3 features.
Upper zone predominant Fine nodules (starry sky) with airway involvement Inspiratory/expiratory scans demonstrate segmental air trapping and hence mosaic ventilation patterns
59
What are the features of cryptogenic organising pneumonia on HRCT?
Pleurally based Dense consolidation in triangular, pleural areas Air bronchograms Peribronchovascular densities
60
What is pulmonary lymphangioleiomyomatosis?
A diffuse bilateral cystic disease that is either sporadic or associated with tuberous sclerosis complex Associated with angiomyolipomas of the kidneys and meningiomas Management is with mTOR inhibitors or lung transplantation
61
Name 2 radiographic patterns on HRCT associated with smoking.
RBILD (respiratory bronchiolitis-associated interstitial lung disease) DIP (desquamative interstitial pneumonia) DIP is a later presentation and likely represents the end stage of RBILD
62
What are the features on HRCT you would expect to see with RBILD (respiratory bronchiolitis-associated interstitial lung disease)? Name 5.
No zonal predominance Bilateral Prominent ground glass Bronchial wall thickening Coexisting centrilobar emphysema
63
Name 3 epidemiological factors associated with Langerhans cell histocytosis X (LCHC).
Young adults (20-40) Smokers Male > female
64
Name 4 features/disease associations with Langerhans cell histocytosis X.
Diabetes insipidus Lytic bone lesions Lymphoma Pituitary hormone deficiencies
65
Where does CT prove to be more beneficial than PET scanning when looking for malignancy lung nodules?
When nodules are small (<0.7cm) Also with bronchoalveolar Ca and carcinoid, as these exhibit low uptake
66
Name 2 confounding factors that can result in increased nitric oxide upon exhalation testing.
URTI Air pollution exposure
67
Name 4 confounding factors that can result in decreased nitric oxide upon exhalation testing.
Steroids Leukotriene receptor antagonists Exercise Smoking
68
Name 2 bacteria that are strongly linked to sarcoidosis.
Mycobacteria Proprionibacteria
69
What are the characteristic findings on CT of sarcoidosis? Name 4.
Mediastinal and hilar lymphadenopathy Lung disease with upper lobe predominance Peribronchial irregularities Subpleural micronodules
70
What is found on histology for sarcoidosis?
Non-caseating granulomas Occasionally necrosis
71
What is the treatment for sarcoidosis.
50-60% of cases spontaneously remit Corticosteroids for severe or progressive disease Immunosuppressive treatment if steroid failure - methotrexate is slow-acting, may take up to 6 months for benefit - cyclophosphamide is treatment of choice for CNS sarcoidosis - antimalarials have a role in skin, splenic involvement and hypercalcaemia
72
What are the characteristic features of bronchiectasis on HRCT? Name 3.
Airway dilatation Bronchial wall thickening Mucous plugging of airways - with tree-in-bud pattern
73
What is the alveolar gas equation?
paO2 = FiO2 (pATM - pH2O) - (paCO2 (1 - FiO2 (1 - RER)))/RER paO2 = alveolar partial pressure of O2 FiO2 = fraction of inspired gas that is O2 (0.21) pATM = prevailing atmospheric pressure (usually 760 at sea level) pH2O = water vapour pressure (usually 47 at sea level) paCO2 = partial pressure of carbon dioxide RER = respiratory exchange ratio (0.8) Simplified version, putting the numbers in, assuming sea level: 150 - (paCO2 x 1.25)
74
What does peptostreptococcus typically cause?
Empyemas - anaerobic infection Foul tasting sputum is characteristic