Respiratory Flashcards

1
Q

COPD general management?

A
  1. Smoking cessation = NRT, varenicicline or bupropion
  2. Annual influenze vaccination
  3. One-off pneumococcal vaccination
  4. Pulmonary rehab for functionally disabled (MRC >=3)
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2
Q

COPD medication management?

A
  1. SABA/SAMA 1st line
  2. Next determine if asthmatic features/steroid responsiveness
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3
Q

Asthmatic/steroid responsive features?

A
  1. Any previous, secure Dx of asthma or atopy
  2. Raised eosinophils
  3. Substantial FEV1 variation (>400ml)
  4. Substantial diurnal variation in PEF (at least 20%)
    (Don’t recommend formal reversibility testing)
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4
Q

No asthmatic features/steroid responsiveness?

A
  1. LABA + ICS
  2. Triple therapy = LAMA + LABA + ICS
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5
Q

Oral theophylline mushkies?

A
  1. Only after trials of SA and LAs or to people who cannot use inhaled therapy
  2. Dose reduced if macrolide or fluoroquinolone Abx co-prescribed
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6
Q

COPD oral prophylactic antibiotic therapy mushkies?

A
  1. Azithromycin in select patients
  2. Not smoke, optimised Rx, continue to have exacerbations
  3. Need CT thorax to exclude bronchiectasis ad sputum culture to rule out atypicals + TB
  4. LFT and ECG as azithromycin can prolong QT interval
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7
Q

COPD and mucolytics?

A

Considered in pts with chronic productive cough and continued if symptoms improve

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8
Q

Cor pulmonale mushkies?

A
  1. Features = peripheral oedema, raised JVP, systolic parasternal heave, loud P2
  2. Use loop diuretic for oedema, consider LTOT
  3. ACEi, CCB and alpha blocker not recommended
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9
Q

Factors which improve survival in stable COPD?

A
  1. Smoking cessation
  2. LTOT if fit criteria
  3. Lung volume reduction surgery in some
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10
Q

Acute bronchitis definition?

A

Self-limiting chest infection as a result of inflammation of the trachea and major bronchi and is therefore associated with oedematous large airways and the production of sputum. The disease course usually resolves before 3 weeks, however, 25% of patients will still have a cough beyond this time. Although there is uncertainty in the literature regarding the exact proportion of pathogens giving rise to acute bronchitis, it is accepted that viral infection is the leading cause. Around 80% of episodes occur in autumn or winter.

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11
Q

Acute bronchitis presentation?

A
  1. Cough = productive/non-productive
  2. Sore throat
  3. Rhinorrhoea
  4. Wheeze
  5. Some = low grade fever
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12
Q

Differentiating acute bronchitis from pneumonia?

A
  1. Hx = sputum, wheeze and breathlessness may be absent in bronchitis but is usually present in pneumonia
  2. Ex = No focal chest signs in acute bronchitis other than wheeze
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13
Q

Acute bronchitis Dx?

A

Clinical (but CRP also helpful)

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14
Q

Acute bronchitis Rx?

A
  1. Usually conservative
  2. Abx if = systemically very unwell, pre-existing co-morbidities, CRP 20-100 (delayed prescription) or CRP >100
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15
Q

Acute bronchitis Abx?

A

Doxycycline 1st line (Amoxicillin 2nd line)

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16
Q

Smoking cessation general points?

A
  1. NRT or Varenicline or Bupropion
  2. Target stop date –> 2 more weeks of NRT, 3-4 more weeks of V/B prescription given –> further prescription only given to those who have demonstrated their quit attempt is continuing
  3. If unsuccessful do not offer repeat prescription within 6 months unless special circumstances
  4. Do not offer combination of them
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17
Q

NRT s/e?

A
  1. N&V
  2. Headaches
  3. Flu-like symptoms
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18
Q

NRT for high level of dependence/have found single forms of NRT inadequate in the past?

A

Combination of nicotine patch and another form of NRT

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19
Q

Varenicline MOA?

A

Nicotinic receptor partial agonist

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20
Q

Varenicline mushkies?

A
  1. Should be started 1 week before the patients target date to stop
  2. Recommended course of Rx is 12 weeks
  3. More effective than bupropion
  4. Nausea is most common s/e, others = headache, insomnia, abnormal dreams
  5. Caution in pts with Hx of depression or self harm
  6. C/I in pregnancy and breastfeeding
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21
Q

Bupropion MOA?

A

Norepinephrine and dopamine reuptake inhibitor, and nicotinic antagonist

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22
Q

Bupropion mushkies?

A
  1. Start 1-2 weeks before target date to stop
  2. 1/1000 risk of seizures
  3. C/I in epilepsy, pregnancy and breast feeding
  4. Having an eating disorder is a relative contraindication
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23
Q

Pregnant women referral for smoking cessation crieteria?

A
  1. All women who smoke
  2. Stopped smoking in last 2 weeks
  3. CO reading of 7ppm or above (all pregnant women should be tested using carbon monoxide detectors)
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24
Q

Pregnant women smoking cessation interventions?

A
  1. CBT, motivational interviewing, structured self help and support
  2. NRT can be used if above measures fail, should remove patches before bed
  3. Varenicline and Bupropion are contraindicated
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25
Q

RhA respiratory problems?

A
  1. Pulmonary fibrosis, pleural effusion
  2. Pulmonary nodules, bronchiolitis obliterans
  3. Complications of drug therapy
  4. Pleurisy
  5. Atypical infections
  6. Caplan’s syndrome = massive fibrotic nodules with occupational coal dust exposure
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26
Q

Most common COPD exacerbation causes?

A
  1. Haemophilus influenzae (most common)
  2. S. pneumoniae
  3. M. catarrhalis
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27
Q

Acute exacerbation of COPD Rx?

A
  1. Increase bronchodilator frequency and consider nebuliser
  2. Prednisolone 30mg 5 days
  3. Oral Abx if purulent sputum or clinical signs of pneumonia = Amoxicillin/Clarithromycin/Doxycycline
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28
Q

Acute asthma classification?

A
  1. Moderate
  2. Severe
  3. Life threatening
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29
Q

Moderate acute asthma?

A
  1. PEFR 50-75%
  2. RR < 25
  3. HR < 110
  4. Speech normal
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30
Q

Severe acute asthma?

A
  1. PEFR 33-50%
  2. RR > 25
  3. HR > 110
  4. Can’t complete sentences
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31
Q

Life-threatening acute asthma?

A
  1. PEFR < 33%
  2. SpO2 < 92%
  3. Normal pCO2
  4. Silent chest, cyanosis, or feeble respiratory effort
  5. Bradycardia, dysrhythmia or hypotension
  6. Exhaustion, confusion or coma
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32
Q

Near-fatal asthma?

A

Raised pCO2 and/or requiring mechanical ventilation with raised inflation pressures

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33
Q

Acute asthma when is ABG indicated?

A

SpO2 < 92%

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34
Q

Acute asthma who needs admission?

A
  1. All pts with life-threatening
  2. Severe if they fail to respond to initial treatment
  3. Others = previous near-fatal, pregnancy, attack occurring despite using oral corticosteroid and presentation at night
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35
Q

Acute asthma Rx?

A
  1. O2
  2. SABA e.g. salbutamol
  3. Prednisolone 40mg 5 days
  4. Ipratropium
  5. IV MgSO4
  6. IV Aminophylline
  7. ITU/HDU = I&V, ECMO
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36
Q

Asthma criteria for discharge?

A
  1. Been stable on their discharge medication (no nebulisers or oxygen) for 12-24 hours
  2. Inhaler technique checked and recorded
  3. PEF > 75% of best or predicted
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37
Q

Lung abscess pathophysiology?

A
  1. Most commonly secondary to aspiration pneumonia
  2. Others = haematogenous (IE), direct (empyema), bronchial obstruction (tumour)
  3. Typically polymicrobial
  4. Monomicrobial = S. aureus, K. penumonia, P. aeruginosa
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38
Q

Lung abscess features?

A

Subacute, fever, night sweats, weight loss, foul smelling sputum, possible clubbing

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39
Q

Lung abscess Ix?

A
  1. Sputum and blood cultures
  2. CXR = fluid-filled space within an area of consolidation, air-fluid level seen
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40
Q

Lung abscess Rx?

A
  1. IV Abx
  2. If not resolving percutaneous drainage may be required and in very rare cases surgical resection
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41
Q

Asthma management?

A
  1. SABA
  2. SABA + Low dose ICS
  3. SABA + Low dose ICS + LTRA
  4. SABA + Low dose ICS + LABA +/- LTRA
  5. SABA + Low dose ICS MART +/- LTRA
  6. SABA +/- Med dose ICS MART +/- LTRA
  7. SABA +/- LTRA + high dose ICS/theophylline/specialist
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42
Q

When to move straight to step 2 asthma management?

A
  1. Not controlled on previous step
  2. Newly diagnosed asthma with symptoms >=3/week or night-time waking
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43
Q

What is MART?

A
  1. Combines ICS + LABA used for both daily maintenance and relief of symptoms
  2. Only available for ICS and LABA combinations in which LABA has a fast-acting component
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44
Q

Inhaled corticosteroid doses?

A
  1. <=400 mcg budesonide = low dose
  2. 400-800 mcg budesonide = moderate dose
  3. > 800 mcg budesonide = high dose
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45
Q

Primary pneumothorax?

A

No underlying lung disease

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46
Q

Primary pneumothorax Rx?

A
  1. If <2cm and pt not SOB then consider discharge
  2. Otherwise, attempt aspiration
  3. If fails (>2cm or still SOB) then insert chest drain
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47
Q

Secondary pneumothorax Rx?

A
  1. > 50 y/o and >2cm and/or SOB then chest drain
  2. Otherwise attempt aspiration if 1-2cm, if fails (>1cm) then chest drain inserted. All admitted for at least 24 hours
  3. <1cm = give O2 and admit for 24 hours
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48
Q

Pneumothorax discharge advice?

A
  1. Stop smoking
  2. Flying = 1 week post CXR
  3. Lifelong scuba diving ban
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49
Q

Obstructive lung disease PFT?

A
  1. FEV1 = significantly reduced
  2. FVC = reduced or normal
  3. FEV1/FVC = reduced
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50
Q

Obstructive lung disease examples?

A
  1. Asthma
  2. COPD
  3. Bronchiectasis
  4. Bronchiolitis obliterans
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51
Q

Restrictive lung disease PFT?

A
  1. FEV1 = reduced
  2. FVC = significantly reduced
  3. FEV1/FVC = normal or increased
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52
Q

Restrictive lung disease?

A
  1. Pulmonary fibrosis, asbestosis, sarcoidosis
  2. ARDS, Infant RDS
  3. Neuromuscular disorders, kyphoscoliosis e.g. ankylosing spondylitis
  4. Obesity
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53
Q

CRB-65?

A

Used in primary care

  1. Confusion = AMTS < 8/10
  2. RR > 30
  3. BP <90 Systolic or <60 Diastolic
  4. Age >=65

0 = home
1-2 = consider hospital
3 = urgent hospital

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54
Q

Point of care CRP test for pneumonia?

A
  1. <20 = do not routinely offer Abx
  2. 20-100 = consider delayed Abx
  3. CRP > 100 = offer Abx
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55
Q

CURB-65?

A

Secondary care setting

Confusion < 8/10 AMTS
Urea > 7
RR > 30
BP <90 systolic or <60 diastolic
Age >= 65

0-1 = consider home
>=2 = consider hospital
>=3 = consider hospital

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56
Q

Pneumonia Ix?

A
  1. CXR
  2. Intermediate/high risk = blood cultures, sputum cultures, pneumococcal and legionella urinary antigen tests
  3. CRP monitoring to determine response to treatment
57
Q

Low severity CAP Rx?

A
  1. Amoxicillin 5d 1st line
  2. If pen allergic then macrolide or tetracycline
58
Q

Moderate/high severity CAP Rx?

A
  1. Amoxicillin + Macrolide 7-10d
59
Q

Pneumonia discharge criteria?

A

Don’t d/c if in past 24h they have had 2 or more of the following
1. Temp > 37.5
2. RR > 24
3. HR > 100
4. SBP < 90
5. SpOt < 90%
6. Abnormal mental status
7. Inability to eat without assistance

60
Q

Pneumonia resolution timeline?

A
  1. 1 week = fever resolved
  2. 4 weeks = chest pain and sputum production reduced
  3. 6 weeks = cough and breathlessness reduced
  4. 3m = most symptoms resolved but fatigue may still be present
  5. 6m = most back to normal
61
Q

Pneumonia repeat CXR timing?

A

6 weeks

62
Q

Causes of bi-hilar lymphadenopathy?

A
  1. Sarcoidosis
  2. TB
  3. Lymphoma/malignancy
  4. Pneumoconiosis e.g. berylliosis
  5. Fungi e.g. histoplasmosis, coccidioidomycosis
63
Q

Mesothelioma definition?

A

Cancer of the mesothelial layer of the pleural cavity that is strongly associated with asbestos exposure. In a small percentage of cases, other mesothelial layers such as those in the abdomen may be affected.

64
Q

Mesothelioma features?

A
  1. Clubbing
  2. Chest wall pain
  3. 30% present as painless pleural effusion
  4. Only 20% have pre-existing asbestosis
  5. History of asbestos exposure in 85-90%, latent period of 30-40 years
65
Q

Mesothelioma which lung affected more often?

A

Right

66
Q

Mesothelioma Ix?

A
  1. CXR = pleural effusion/pleural thickening
  2. CT
  3. Effusion = MC&S, biochem, cytology (cytology only helpful in 20-30% cases)
  4. Local anaesthetic thoracoscopy to Ix cytology negative exudative effusions as it has a high diagnostic yield (around 95%)
  5. If area of pleural nodularity seen on CT then image-guided pleural biopsy may be used
67
Q

Mesothelioma Rx?

A
  1. Symptomatic
  2. Industrial compensation
  3. Chemo, surgery if operable
  4. Prognosis poor, median survival 12m
68
Q

Predominant cough without lung function abnormalities?

A

Chronic cough syndromes and pertussis

69
Q

Prominent dizziness, light headedness and peripheral tingling?

A

Dysfunctional breathing

70
Q

Recurrent severe asthma attacks without objective confirmatory experience

A

Vocal cord dysfunction

71
Q

Predominantly nasal symptoms without lung function abnormalities?

A

Rhinitis

72
Q

Asthma diagnosis in children < 5 y/o?

A

Clinical

73
Q

Asthma diagnosis 5-16 y/o?

A
  1. Spirometry with a bronchodilator reversibility (BDR) test
  2. FeNO test should be requested if there is normal spirometry or obstructive spirometry with a negative bronchodilator reversibility (BDR) test
74
Q

Asthma diagnosis >=17 y/o?

A
  1. All should have FeNO test
  2. All should have spirometry with a bronchodilator reversibility (BDR) test
  3. Asked if their symptoms are better on days away from work/during holidays. If so, patients should be referred to a specialist as possible occupational asthma
75
Q

FeNO positive levels?

A

> 40 in adults, >35 in children

76
Q

Spirometry FEV1/FVC ratio in obstructive disease e.g. asthma?

A

<70%

77
Q

Asthma reversibility testing?

A
  1. Adults = FEV1 improvement by 12% and increase in volume by 200ml
  2. Children = FEV1 improvement by 12%
78
Q

When should COPD diagnosis be considered?

A

> 35 y/o smokers/ex-smokers with exertional SOB, chronic cough, or regular sputum production

79
Q

Suspected COPD Ix?

A
  1. FBC = exclude secondary polycythaemia
  2. BMI calculation
  3. CXR = hyperinflation, bullae, flat hemidiaphragm, exclude lung cancer
  4. Post-bronchodilator spirometry to demonstrate airflow obstruction: FEV1/FVC ratio <70%
80
Q

How is COPD severity categorised?

A

Using FEV1 % predicted
1. > 80% = Mild
2. 50-79% = Moderate
3. 30-49% = Severe
4. <30% = Very severe

81
Q

Idiopathic pulmonary fibrosis epidemiology?

A

50-70 y/o, 2M:1F

82
Q

IPF features?

A
  1. Progressive exertional dypsnoea
  2. Bibasal fine end-inspiratory crepitations on auscultation
  3. Dry cough
  4. Clubbing
83
Q

IPF Dx?

A
  1. Spirometry = restrictive (FEV1 normal/decreased, FVC decreased, FEV1/FVC increased)
  2. Reduced TLCO = impaired gas exchange
  3. Imaging = ground glass on CXR, CT needed for diagnosis
  4. ANA +ve in 30%, RhF +ve in 10%
84
Q

IPF Rx?

A
  1. Pulmonary rehabilitation
  2. Supplementary O2
  3. Lung transplant
  4. Pirfenidone (antifibrotic) may be useful in select patients
85
Q

IPF Prognosis?

A

Life expectancy 3-4 years

86
Q

ABG triad for chronic CO2 retention?

A
  1. Normal pH
  2. high pCO2
  3. High HCO3
87
Q

OSA consequences?

A
  1. Daytime somnolescence
  2. Compensated respiratory acidosis
  3. HTN
88
Q

Sleepiness assessment?

A
  1. Epworth Sleepiness Scale = questionnaire completed by patient +/- partner
  2. Multiple Sleep Latency Test (MSLT) = measures the time to fall asleep in a dark room (using EEG criteria)
89
Q

OSA Dx?

A

Sleep studies (polysomnography) = ranging from monitoring of pulse oximetry at night to full polysomnography where a wide variety of physiological factors are measured including EEG, respiratory airflow, thoraco-abdominal movement, snoring and pulse oximetry

90
Q

OSA Rx?

A
  1. Weight loss
  2. CPAP 1st line for moderate/severe
  3. Intra-oral devices e.g. mandibular advancement may be used if CPAP is not tolerated or for patients with mild OSAHS where there is no daytime sleepiness
  4. DVLA should be informed if OSAHS is causing excessive daytime sleepiness
91
Q

COPD reason for using ICS?

A

Reduced exacerbations

92
Q

Lung cancer risk factors?

A
  1. Smoking (x10)
  2. Asbestos (x5)
  3. Arsenic, radon, nickel, chromate
  4. Aromatic hydrocarbon
  5. IPF
93
Q

When is BiPAP indicated for AECOPD?

A

Respiratory acidosis/rising PaCO2 resistant to best medical management

94
Q

Pleural effusion Ix?

A
  1. Imaging
  2. Pleural aspiration
95
Q

Pleural effusion imaging?

A
  1. PA CXR
  2. US
  3. Contrast CT
96
Q

Pleural aspiration method?

A
  1. 21G needle and 50ml syringe
  2. Fluid sent for pH, protein, LDH, cytology, microbiology
97
Q

Transudate protein level?

A

< 30 g/L

98
Q

Exudate protein level?

A

> 30 g/l

99
Q

When should Light’s criteria be used?

A

If protein level 25-35 g/L

100
Q

Light’s criteria?

A

Exudate is likely if at least 1 of the following are present:
1. Pleural fluid protein divided by serum protein >0.5
2. Pleural fluid LDH divided by serum LDH >0.6
3. Pleural fluid LDH more than two-thirds the upper limits of normal serum LDH

101
Q

Pleural fluid with low glucose?

A

TB, RhA

102
Q

Pleural fluid with raised amylase?

A

Pancreatitis, oesophageal perforation

103
Q

Pleural fluid with heavy blood staining?

A

Mesothelioma, PE, TB

104
Q

Pleural infection mushkies?

A
  1. All patients with a pleural effusion in association with sepsis or a pneumonic illness require diagnostic pleural fluid sampling
  2. If the fluid is purulent or turbid/cloudy a chest tube should be placed to allow drainage
  3. If the fluid is clear but the pH is less than 7.2 in patients with suspected pleural infection a chest tube should be placed
105
Q

Recurrent pleural effusion management?

A
  1. Recurrent aspiration
  2. Pleurodesis
  3. Indwelling pleural catheter
  4. Drug management to alleviate symptoms e.g. opioids to relieve dyspnoea
106
Q

4 commonest causes of anterior mediastinum mass?

A

4 Ts
Teratoma
Thymic Mass
Thyroid Mass
Terrible lymphadenopathy

107
Q

Cavitating pneumonia in upper lobes in diabetics and alcoholics?

A

Klebsiella

108
Q

Klebsiella definition?

A

Klebsiella pneumoniae is a Gram-negative rod that is part of the normal gut flora. It can cause a number of infections in humans including pneumonia (typically following aspiration) and urinary tract infections.

109
Q

Klebsiella pneumonia features?

A
  1. More common in alcoholics and diabetics
  2. May occur following aspiration
  3. Red-currant jelly sputum
  4. Often affects upper lobes
110
Q

Klebsiella pneumonia prognosis?

A
  1. Commonly causes lung abscess formation and empyema
  2. Mortality is 30-50%
111
Q

Bronchiectasis definition?

A

Permanent dilatation of the airways secondary to chronic infection or inflammation

112
Q

Bronchiectasis Rx?

A
  1. Physical training
  2. Postural drainage
  3. Abx for exacerbations + long term Abx in severe cases
  4. Bronchodilators in selected cases
  5. Immunisations
  6. Surgery in selected cases e.g. localised disease
113
Q

Most common organisms isolated from bronchiectasis?

A
  1. H. influenzae
  2. P. aeruginosa
  3. Klebsiella spp
  4. S. pneumoniae
114
Q

Acute oxygen therapy management of COPD patients?

A
  1. Prior to availability of blood gases, use a 28% Venturi mask at 4 l/min and aim for an oxygen saturation of 88-92% for patients with risk factors for hypercapnia but no prior history of respiratory acidosis
  2. Adjust target range to 94-98% if the pCO2 is normal
115
Q

NIV key indications?

A
  1. COPD with respiratory acidosis 7.25-7.35
  2. T2RG secondary to chest wall deformity, neuromuscular disease or OSA
  3. Cardiogenic pulmonary oedema unresponsive to CPAP
  4. Weaning from tracheal intubation
116
Q

Recommended initial settings for bi-level pressure support in COPD?

A
  1. Expiratory Positive Airway Pressure (EPAP): 4-5 cm H2O
  2. Inspiratory Positive Airway Pressure (IPAP): RCP advocate 10 cm H20 whilst BTS suggest 12-15 cm H2O
  3. Back up rate: 15 breaths/min
  4. Back up inspiration:expiration ratio: 1:3
117
Q

Pleural plaque mushkies?

A

Benign and do not undergo malignant change. They, therefore don’t require any follow-up. They are the most common form of asbestos-related lung disease and generally occur after a latent period of 20-40 years

118
Q

Pleural thickening mushkies?

A

Asbestos exposure may cause diffuse pleural thickening in a similar pattern to that seen following an empyema or haemothorax. The underlying pathophysiology is not fully understood.

119
Q

Asbestosis mushkies?

A

The severity of asbestosis is related to the length of exposure. This is in contrast to mesothelioma where even very limited exposure can cause disease. The latent period is typically 15-30 years. Asbestosis typically causes lower lobe fibrosis. As with other forms of lung fibrosis the most common symptoms are shortness-of-breath and reduced exercise tolerance. It is treated conservatively - no interventions offer a significant benefit

120
Q

Goodpasture’s syndrome?

A
  1. Haemoptysis
  2. Glomerulonephritis
  3. Systemically unwell: fever, nausea
121
Q

GPA?

A
  1. Upper respiratory = epistaxis, sinusitis, nasal crusting
  2. Lower respiratory = dyspnoea, haemoptysis
  3. Glomerulonephritis
  4. Saddle-shape nose deformity
122
Q

Sarcoidosis definition?

A

Multisystem disorder of unknown aetiology characterised by non-caseating granulomas. It is more common in young adults and in people of African descent

123
Q

Sarcoidosis features?

A
  1. Acute = erythema nodosum, bihilar lymphadenopathy, swinging fever, polyarthralgia
  2. Insidious = dyspnoea, non-productive cough, malaise, wight loss
  3. Skin = lupus pernio
  4. Hypercalcaemia = macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)
124
Q

Syndromes associated with Sarcoidosis?

A
  1. Lofgren’s syndrome = an acute form of the disease characterised by bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia. It usually carries an excellent prognosis
  2. Mikulicz syndrome = enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis or lymphoma
  3. Heerfordt’s syndrome = parotid enlargement, fever and uveitis secondary to sarcoidosis
125
Q

SABA?

A

Salbutamol

126
Q

SAMA?

A

Ipratropium

127
Q

LABA?

A

Salmeterol

128
Q

LAMA?

A

Tiotropium

129
Q

Finger clubbing and > 40 y/o?

A

CXR

130
Q

2ww lung cancer referral indications?

A
  1. CXR findings that suggest lung cancer
  2. > 40 with unexplained haemoptysis
131
Q

A1AT deficiency spirometry picture?

A

Obstructive

132
Q

A1AT deficiency definition?

A

A common inherited condition caused by a lack of a protease inhibitor (Pi) normally produced by the liver. The role of A1AT is to protect cells from enzymes such as neutrophil elastase. It classically causes emphysema (i.e. chronic obstructive pulmonary disease) in patients who are young and non-smokers.

133
Q

A1AT genetics?

A
  1. Chromosome 14
  2. Autosomal recessive/co-dominant inheritance
134
Q

A1AT deficiency features?

A
  1. Lungs = panacinar emphysema, most marked in lower lobes
  2. Liver = cirrhosis and HCC in adults, cholestasis in children
135
Q

A1AT deficiency Ix?

A
  1. A1AT concentrations
  2. Spirometry = obstructive picture
136
Q

A1AT deficiency Rx?

A
  1. No smoking
  2. Supportive = bronchodilators, physiotherapy
  3. IV A1AT protein concentrates
  4. Surgery = lung volume reduction surgery, lung transplantation
137
Q

Which smoking cessation drug is C/I in epilepsy?

A

Bupropion as reduces seizure threshold

138
Q

Tram lines on CXR?

A

Bronchiectasis

139
Q

Large amounts of purulent sputum?

A

Bronchiectasis