Respiratory Flashcards

1
Q

Give two examples of type 1 respiratory failure

A
  • PE
  • Pneumonia
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2
Q

Give two examples of type 2 respiratory failure

A
  • COPD
  • Asthma
  • Emphysema
  • Neuromuscular disease
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3
Q

Define COPD

A

Progressively worsening, irreversible airflow obstruction

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4
Q

What are the subsets of COPD?

A
  • Bronchitis
  • Emphysema
  • A1AT deficiency
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5
Q

What is chronic bronchitis?

A
  • Hypertrophy + hyperplasia of mucous glands → mucus hypersecretion + ciliary dysfunction → productive cough
  • Inflammation → airway narrowing (bronchoconstriction) → limited airflow

Cough for 3+ months, over 2+ years

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6
Q

What is emphysema?

A
  • Exposure to irritants → degrades elastin in alveoli + airways → air-trapping → poor gas exchange
  • ** Loss of elastin → lose elasticity →lungs more compliant (lungs expand + hold air) → exhaling difficulty**
  • Dilation + destruction of the lung tissue (distal to terminal bronchioles)
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7
Q

A1AT deficiency inheritence pattern

A

Autosomal recessive

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8
Q

A1AT Pathology

A
  • Alpha-1 antitrypsin = degrades NE (neutrophil elastase) - protects excess damage to elastin layer (esp in lungs)
  • A1AT deficiency → Increased NE → (paracinar) emphysema + liver issues
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9
Q

Who should you suspect A1AT deficiency in?

A

Younger/middle age men with COPD Sx - but NO SMOKING HISTORY!

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10
Q

Rx for COPD

A
  • SMOKING
  • Air pollution
  • Genetic factors (A1AT deficiency)
  • Occupational exposure (chemical, vapors, fumes)
  • Advanced age
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11
Q

Differentiating factor between COPD and asthma

A

COPD = not significantly reversible with bronchodilators (e.g. salbutamol)

COPD obstructive picture = does NOT show a dramatic response to reversibilty testing with beta-2 agonist (e.g. salbutamol) during spirometry testing

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12
Q

A 65 y/o who is a long-term smoker presents with:
* Chronic SOB
* Cough
* Sputum production
* Wheeze
* Recurrent respiratory infections (particularly in winter)
Possible diagnosis?

A

COPD

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13
Q

What are the signs of COPD?

A
  • Barrel chest
  • Coarse crackles
  • Wheezing on ausculation
  • Tachypnoea
  • Weight loss
  • Hyper-resonance on percussion
  • Cor pulmonale
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14
Q

Symptoms of COPD

A
  • Cough
    • Freq. morning
    • Usually productive (sputum)
  • SOB
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15
Q

What are the 2 main pathogens that cause acute exacerbations in COPD?

A
  • S. Pneumo
  • H. influenzae
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16
Q

What does an ‘obstructive’ picture indicate on spirometry?

A

Overall lung capacity is not as bad as their ability to quickly blow air out of their lungs
FEV1/FVC ratio <0.7

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17
Q

What is the severity of ariflow obstruction graded by?

A

FEV1
- Stage 1: FEV1 >80% of predicted
- Stage 2: FEV1 50-79% of predicted
- Stage 3: FEV1 30-49% of predicted
- Stage 4: FEV1 <30% of predicted

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18
Q

Ix for COPD

A
  • Pulse oximetry (low oxygen saturation)
  • Spirometry: FEV1/FVC < 0.7 (obstructive picture)
  • Diffusing capacity of carbon monoxide (DLCO): Decreased
  • CXR: Signs of hyperinflation (flattened diaphragm, hyperexpansion)
  • ABG: May should type 2 respiratory failure
  • FBC: Anaemia, polycythaemia (rasied Hb) - in response to chronic hypoxia
  • Genetic testing: A1AT deficiency
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19
Q

What are the grades in the Modified Medical Research Council Dyspnoea Sacle (mMRCD Scale)?

A
  • Grade 5 – Unable to leave the house due to breathlessness
  • Grade 4 – Stop to catch their breath after walking 100 meters on the flat
  • Grade 3 – Breathless that slows walking on the flat
  • Grade 2 – Breathless on walking up hill
  • Grade 1 – Breathless on strenuous exercise
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20
Q

COPD does not cause which extra-pulmonary manifestation?

A

Clubbing!

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21
Q

What WBCs underpin the pathology of asthma and COPD?

A
  • Asthma = characterised by eosinophillic inflammation
  • COPD = characterised by neutrophilic inflammation
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22
Q

What is the treatment plan for COPD?

A

In order:
* Smoking cessarion + vaccines (pneumococcal + influenza)
* Step 1: Beta-2 agonists (salbutamol)
* Step 2: SABA (salbutamol) + LABA (salmeterol) + LAMA (tiotropium)
* Long term oxygen therapy at home (must be non-smoker) or the nebulisers (salbutamol and/or ipratropium)

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23
Q

What is the O2 target for someone having an COPD exacerbation?

A

88-92%

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24
Q

Name 2 complicatiosn of COPD

A
  • Cor pulmonale
  • Recurrent pneumonia
  • Depression
  • Polycythaemia
  • Respiratory failure
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25
Q

What does an ABG look like in an COPD exacerbation?

A
  • Respiratory acidosis + raised bicarbonate (HCO3-)
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26
Q

Ix to run if you suspect a COPD exacerbation

A
  • CXR (rule out pneumonia)
  • ECG (check for HF)
  • CRP (infection)
  • Sputum culture
  • FBC (rasied WBC count)
  • Bloood cultures (if septic)
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27
Q

Management of an acute eacerbation in COPD

A

Home:
* Prednisolone
* Regular inhalers/nebulisers (SABA)
* Antibiotics (if infection)

Hospital:
* Nebulsied bronchodilators (salbutamol)
* Steroids (IV hydrocortisone OR oral prenisolone)
* Antibiotics (if infection)
* Physiotherapy

More:
* NIV

Antibiotics (amoxicillin)

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28
Q

What is asthma?

A
  • Chronic inflammatory reversible airway disease - characterised by REVERSIBLE AIRWAY OBSTRUCTION
  • AIRWAY HYPERRESPNSIVENESS & INFLAMED BRONCHIOLES + mucus secretion
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29
Q

Triggers

A
  • Infection
  • Alergen
  • Cold weather
  • Exercise
  • Drugs (beta-blockers, aspirin)

Bronchoconstriction = caused by hypersensitivity of the airways

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30
Q

What conditions are in the atopic triad?

A
  • Eczema
  • Asthma
  • Hayfever (atopic rhinitis)
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31
Q

Presentation of asthma

A
  • Episodic
  • Diurnal variability (worse at night)
  • Dry cough w/ wheeze + SOB
  • **Bilateral widespread ‘polyphonic’ wheeze **
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32
Q

Name soem clinical manifestations that there is another diagnosis, that is not asthma

A
  • Wheeze related to coughs and colds more suggestive ofviral induced wheeze
  • Isolated or productive cough
  • Normal investigations
  • No response to treatment
  • Unilateral wheeze → this suggests a focal lesion or infection.
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33
Q

First line testing for asthma

A
  • Fractional exhaled nitric oxide
  • Spirometry with bronchodilator reversibility
    • FEV1/FVC <0.7
    • Shows good response to bronchodilator (>12% FEV1 increase)
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34
Q

Asthma: Name a SABA, ICS, LABA, LTRA, LAMA

A
  • SABA: Salbutamol
  • ICS: Beclomethasone
  • LABA: Salmeterol
  • LTRA: Monkelukast
  • LAMA: Tiotropium bromide
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35
Q

What is used in the long-term management of asthma?

A
  • SABA
  • SABA + ICS
  • SABA + ICS + LTRA
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36
Q

Mangement for an asthma exacerbation (mnemonic)

A

OSHITME
* O - Oxygen
* S - Salbutamol (nebulised)
* I - ICS (hydrocortisone)
* T - Theophylline
* M - Magnesium sulfate
* E - Esculate

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37
Q

Additional management for asthma

A
  • Annual flu jab
  • Annual asthma review
  • Advise exercise + avoid smoking
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38
Q

Clincial manifestations of an acute exacerbation of asthma

A
  • Progressively worsening SOB
  • Use of accessory muscles
  • Tachypnoea (fast respiratory rate)
  • Symmetrical expiratory wheeze on auscultation
  • The chest can sound ‘tight’ on auscultation - with reduced air entry
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39
Q

What is used to grade acute asthma exacerbations and what are the groupings?

A

Peak expiratory flow rate (PEFR)
* Moderate: 50-75% predicted
* Severe: 33-50% predicted
* Life-threatening: Less than 33% (silent chest - no air entry)

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40
Q

What bacterium is tuberculosis caused by?

A

Mycobacterium tuberculosis

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41
Q

What is the staining required for TB?

A

Zeihl-Neelsen stain
(bacteria turns bright red against blue background)

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42
Q

Transmssion of TB

A

Aerosol transmission

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43
Q

How does latent TB present?

A
  • No clincial disease
  • Detectable CMI to TB on tuberculin skin test (Mantoux test)
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44
Q

What is the primary (Ghon) focus?

A

Bacilli + macrophages = coalesce to form a granuloma

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45
Q

What is a Ghon complex?

A

Pimary focus + medastinal lymph nodes (enlarged)

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46
Q

What are the stages of TB?

A
  • Active TB = active TB in various areas within the body
    • Majority of cases → immune system = able to kill + clear the infection
  • Latent TB = The immune system = encapsulates sites of infection → stopping the progression of the disease
  • Secondary TB = when latent TB reactivated
  • Miliary TB = When the immune system = unable to control the disease → causes a disseminated severe disease
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47
Q

How and where may you get extrapulmonary TB?

A
  • Haematogenous dissemination
  • TB meningitis
  • Pleural TB
  • Genito-urinary TB
  • Bacilli in lymph nodes
  • Miliary TB
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48
Q

Presentation of TB

A
  • WEIGHT LOSS + NIGHT SWEATS
  • Cough +/- haemoptysis
  • Low grade fever, malaise
    Extrapulmonary TB: Lymph node TB (swelling +/- discharge)
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49
Q

Ix for TB

A

First line:
* Mantoux skin (tuberculin) test
- Tests for immune response to TB (by previous, latent or active TB)
* Sputum culture (Ziehl-Neelson test - red is positive)

Other:
* CXR
* Biopsy

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50
Q

What vaccine is used for TB?

A

Neonatal BCG vaccine (live attenuated)

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51
Q

What are the drugs used in TB? ANd how long are they given for?

A

RIPE:
* R - Rifampicin (6 months)
* I - Isoniazid (6 months)
* P - Pyrazinamide (2 months)
* E - Ethambutol (2 months)

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52
Q

Give side effects of the TB RIPE drugs

A
  • Rifampicin → red urine, hepatitis
  • Isonazid → peripheral neuropathy, hepatitis
  • Pyrazinamide → rash, arthralgia, hepatitis
  • Ethambutol → optic neuritis

rifampicin (“red-an-orange-pissin’”)
isoniazide (“I’m-so-numb-azid”)
ethambutol (“eye-thambutol”)

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53
Q

What type of granuloma forms in TB

A

Caseating granuloma

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54
Q

Give a complciation of TB

A
  • Pleural effusion
  • Pericardial effusion
  • Consolidation
  • Pneumothorax
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55
Q

If a patients presents with chronic illness, fever and weight loss, what should you suspect?

A

TB

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56
Q

Define pneumonia

A
  • Infection of lung parenchyma
  • Causes inflammation of lung tissue + sputum filling the airways + alveoli
  • Can be seen as consolidation on an CXR
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57
Q

What are the main causative organisms for pneumonia?

A
  • Streptococcus pneumoniae (50%)
  • Haemophilus influenzae (20%)
  • Pseudomonas aeruginosa in patients with CF or bronchiectasis
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58
Q

What is a viral caused pneumonia - that is also an AIDS defining illness?

A

Pneumocystis pneumonia (PCP)

59
Q

What antiobotic do you not use for atypical pneumonia, and what do you use instead?

A
  • Don’t use penicillins
  • Use a macrolide (clarithromycin)
  • Tetracycline (doxycycline)
60
Q

Give an example of an atypical pneumonia

A

Legionella pneumophilia (Legionnaire’s disease)

Can cause hyponatraemia

61
Q

How does PCP present?

A
  • Dry cough (with sputum)
  • SOB on exertion
  • Nigh sweats
62
Q

What is the treatmemt for PCP?

A

Co-trimoxazole

63
Q

Difference in presentation between typical and atypical pneumonia?

A
  • Typical pneumonia: Productive cough w/ rusty coloured sputum (purulent)
  • Atypical pneumonia: Dry cough, low grade fever
64
Q

A patient presents with:
* Productive cough w/ rusty coloured sputum
* Pleuritic chest pain
* Tachypoea
* Dullness to percussion

Possible diagnosis?

A

Typical pneumonia
* Productive cough w/ rusty coloured sputum (suggests streptococcus pneumoniae)

65
Q

Ix for pneumonia

A
  • ‘Point of care’ test in primary care
  • Pulse oximetry
  • CXR (CONSOLIDATION)
  • Sputum culture + sensitivities
  • Urinary antigen
    • Legionella spp
    • S. pneumoniae
66
Q

What does the CURB65 stand for?

A

In hospital (CURB65); Out of hospital (CRB65) - no urea

  • CConfusion (new disorientation in person, place or time)
  • UUrea > 7 mmol/L
  • RRespiratory rate ≥ 30/min
  • BBlood pressure < 90 systolic or ≤ 60 diastolic.
  • 65– Age ≥65
67
Q

What is the CURB65 core used for?

A
  • Severity assessment of pneumonia
  • Predicts mortality
  • Score 0/1: Consider treatment at home
  • Score ≥ 2: Consider hospital admission
  • Score ≥ 3: Consider intensive care assessment
68
Q

What inheritence pattern is CF?

A

Autosomal recessive

69
Q

What gene (on which chromosome) is affected in CF?

A
  • Cystic fibrosis transmembrane conductance regulatory (CFTR) gene
  • Chromosome 7
70
Q

What ion channel is affected in CF?

A

Chloride channel

71
Q

How many people are CF carriers?

A

1 in 25

72
Q

Both parents are healthy, one sibling has cystic fibrosis and a second child does not have the disease, what is the likelihood of the second child being a carrier?

A

2 in 3

73
Q

What are 3 major complications in CF?

A
  • Thick pancreatic + biliary secretions → blockage of ducts → lack of digestive enzymes
    • E.g. pancreatic lipase in the digestive tract
  • Low volume thick airway secretions → reduce airway clearance → bacterial colonisation → airway infection susceptibility
  • Congenital bilateral absence of the vas deferens
    • Patients = generally have healthy sperm → but no way of getting from the testes to the ejaculate → male infertility
74
Q

Sign of CF in neonates

A

Meconium ileus
Low weight or height on growth charts

  • Recurrent lower respiratory tract infections
  • Failure to thrive
  • Pancreatitis
75
Q

Signs of CF

A
  • **Low weight or height on growth charts **
  • Finger clubbing
  • Crackles + wheexe on ausculation
  • Abdominal distension
  • Genital abnormalities in males
76
Q

Symptoms of CF

A
  • **Chronic cough (wet sounding cough)
  • Thick sputum production
  • Recurrent respiratory tract infections
  • Steatorrhoea**
  • Abdominal pain + bloating
77
Q

A yound child presents with recurrent lower respiratory tract infections, pancreatitis and failure to thrive. Possible diagnosis?

A

Cystic fibrosis

78
Q

What are the investigations for CF?

A
  • Newborn bloodspot test (screening)
  • Sweat test (Na+ + Cl- >60mmol/L) = diagnostic
  • Genetic testing for GFTR (amniocenteses)
79
Q

What are the 2 most common colonising bacteria in CF patients?

A
  • Staphylococcus aureus → prophylactic flucloxacillin
  • Pseudomonas aeruginosa → difficiult to treat + worsens prognosis
80
Q

Which antibiotic do you use for pseudomonas aeruginosa in CF?

A

Oral ciprofloxacin

81
Q

First line treatments for CF

A
  • Chest physiotherapy
  • High calorie diet (malabsorption + increased respiratory effort)

Medication:
* Prophylactic flucloxacillin
* Bronchodilators (salbutamol)
* Vaccinations (Pneumococcal, Influenza, Varicella)
* Treat chest infections when they occur

82
Q

Complications of CF

A
  • Failure to thrive + delayed puberty
  • Depression + anxiety
  • Chronic respiratory failure
  • Cor Pulmonale
  • Diabetes mellitus
  • Acute/retention pancreatitis (pancreatic insufficiency)
  • Liver disease
83
Q

Complication of mediastinal shift in a pneumothorax or pleural effusion

A

Impaired cardiovascular function

84
Q

Define pleural effusion and the different types

A

Pleural effusion = abnormal collection of fluid in the pleural cavity
* Exudative → high protein count (>3g/dL) (cloudy)
* Transudative → lower protein count (<3g/dL) (transparent)
* Transudative or exudative = helps determine the cause

85
Q

Name an exudative cause of pleural effusion

A
  • Exudative causes = related to inflammation
  • Inflammation → results in protein leaking out of the tissues into the pleural space
    • Ex- → moving out of lung tissue
  • Exudative causes → think causes of inflammation:
    • Lung cancer
    • Pneumonia
    • Rheumatoid arthritis
    • Tuberculosis
86
Q

Name a transudative cause of pleural effusion

A
  • Transudative causes = relate to fluid moving across into the pleural space
    • Trans- → moving across
  • Transudative causes = think about causes of fluid shifting
    • Congestive heart failure
    • Hypoalbuminaemia
    • Hypothyroidism
87
Q

Pathology of pleural effusion

A
  • Excess fluid accumulates in pleural space
  • Lung expansion limited → impaired ventilation
88
Q

A patient presents with SOB, O/E, has reduced breath sounds and dullness to percussion over the effusion. Possible diagnosis?

A

Pleural effusion

89
Q

What are the signs of a pleural effusion?

A
  • Reduced breath sounds
  • Dullness to percussion over the effussion
  • Decreased or absent tactile fremitus
  • If massive: Tracheal deviated away from the effusion + mediastinal shift
90
Q

What are the symptoms of a pleural effusion?

A
  • SOB
  • Pleuritic chest pain
  • Cough
91
Q

What is empyema?

A

Infected pleural effusion
(Suspect in a patient with pneumonia with new or ongoing fever)
(Treated with chest drain + antibiotics)

92
Q

Ix for pleural effusion

A
  • 1st + GS: Postero-anterior and lateral CXR
  • Other: Thoracentesis → sample of pleural fluid (from aspiration + chest drain)
93
Q

What CXR results are seen in a pleural effusion?

A
  • Blunting of the costophrenic angle
  • Fluid in lung fissures
  • Larger effusion → meniscus
  • Massive effusion → tracheal + mediatstinal deviation
94
Q

What do you look for thoracentesis in a pleural effusion?

A
  • Protein count (exudate or transudate)
  • Cell count
  • pH
  • Microbiology testing
95
Q

Treatment for a pleural effusion

A
  • Small effusions → Conservative management + treatment of underlying cause
    • E.g. Heart failure → diuretic
  • Larger effusions → aspiration or chest drain
96
Q

What is a pneumothorax?

A

Air in the pleural space/cavity
* Separating the lung from the chest wall
* Can be primary (spontaneous) or secondary (trauma/pathology)

97
Q

What is a tension pneumothorax?

A
  • ** One-way valve** formed by damaged tissue air enters + can’t escape → intrathoracic pressure build up → impaired cardiac + respiratory function
98
Q

Who is the typical patient that presents with pneumothorax?

A
  • Young, tall, thin, young man
  • Present with sudden breathlessness + pleuritic chest pain (possibly playing sports)
99
Q

Rx for a pneumothorax

A
  • Smoking
  • Tall and slender body build
  • Age less than 40 years
  • Male
  • Recent invasive medical procedure
  • Chest trauma
  • Acute severe asthma
  • COPD
  • Tuberculosis
  • Cystic fibrosis
  • Changes in atmospheric pressure
100
Q

Pathology of a pneumothorax

A
  • Air enters through damage to chest wall/lung/gas-producing microorganisms
    • Positive pressure in the pleural space if air enters → lung partial/complete collapse
101
Q

Symptoms of pneumothorax

A
  • Sharp chest pain (one-sided)
  • Dyspnoea
  • Hypercapnia → confusion, coma
102
Q

What are the signs of a pneumothorax?

A
  • Reduced/absence of breath sounds (affected side/ipsilateral)
  • Hyperresonance to percussion
  • Tachycardia
  • Cyanosis
103
Q

Ix for a pneumothorax

A
  • 1st: Postero-anterior CXR
    • Excess fluid = appears black
    • Absence of lung markings between the lung margin + chest wall
  • Gold standard: CT Chest
    • Can detect small pneumothorax (that CXR wont pick up on)
    • Detects underlying pathology
104
Q

What is the most common ABG findining in a penumothorax?

A

Respiratory alkalosis

105
Q

Differentials for a penumothorax

A
  • Asthma, acute exacerbation
  • COPD, acute exacerbation
  • Pulmonary embolism
  • Pleural effusion
106
Q

Treatment for a pneumothorax

A
  • No SOB + less than 2cm air on CXR → no treatment required (maybe supplememtal oxygen)
  • SOB and/or more than 2cm air on CXR → percutaneous aspiration
  • If aspiration fails twice or unstable, bilateral, or secondary → **chest drain **
107
Q

Where is a chest drain inserted?

A

In the ‘triangle of safety’
* The 5th intercostal space

108
Q

What is the management sentence for chest drain for a tension pneumothorax?

A

“Insert a large bore cannula into the second intercostal space in the midclavicular line.”

109
Q

What is a tension pneumothorax?

A
  • Tension pneumothorax = caused by trauma to the chest wall → creates a one-way valve (lets air in + not out of the pleural space)
  • Tension pneumothorax = dangerous as it creates pressure inside of the thorax → pushes the mediastinum acrosskink the big vessels in the mediastinumcardiorespiratory arrest
110
Q

Difference in presentation between a pneumothorax and a tension pneumothorax

A
  • Pneumothorax: Dyspnoea + chest pain
  • Tension pneumothorax: Patients are distressed with rapid laboured respirations + cyanosis + profuse diaphoresis + tachycarida
111
Q

What is an interstitial lung disease?

A
  • Umbrella term used to describe conditions that affect the lung parenchyma (the lung tissue) → causing inflammation + fibrosis
  • Between inflammatory + fibrosing (scarring) → ILDs are within a spectrum
112
Q

Pathology of interstitial lung disease

A
  • Interstitial lung disease → thin membrane = thickenslittle carbon monoxide goes into the capillary → stays in the lung → goes into expiratory breath → transfer factor of the lung for carbon monoxide (TLCO) = reduced
  • Oxygen (+ other gas) uptake = reduced in ILD
113
Q

What is a diagnosis of interstitial lung disease a combination of?

A

Clinical features + High resolution CT thorax
(Unclear disease → take lung biopsy → confirm diagnosis on histology)

114
Q

Describe the appearance of an intersitial lung disease on a high resolution chest CT

A

Ground glass’ appearance

115
Q

Ix for interstitial lung disease

A
  • Incremental shuttle walk test
  • Six-minute walk test
  • High resolution chest CT
116
Q

What is idiopathic pulmonary fibrosis?

A

Idiopathic pulmonary fibrosis = causes scarring of the lung tissue later in life

117
Q

Pathology of idopathic pulomonary fibrosis

A
  • Myofibroblasts deposit collagen in the extracellular matrix → thickened lung tissue → cannot inflate properly → lung volume decreases over time
  • The thickened tissue leads to lower gas exchange efficiency in the lungs
118
Q

What are the two drugs for idiopathic pulmonary fibrosis?

A
  • Pirfenidone
  • Nintedanib
119
Q

What is key in the treatment of intersitial lung disease?

A
  • Removal of antigen in hypersensitivity pneumonitis
  • Removal of drug in drug-induced pulmonary fibrosis
120
Q

Who is idiopathic pulmonary fibrosis most commonly seen in?

A

Oler men (60) who smoke

121
Q

What type of respiratory failure is idiopathic pulmonary fibrosis?

A

Type 1 respiratory failure

122
Q

Sx of idiopathic pulmonary fibrosis

A
  • Exertional dyspnoea
  • Dry unproductive cough
123
Q

What are the investigations for idiopathic pulmonary fibrosis?

A
  • First line: Spirometry → restrictive
    • FEV1:FVC >0.7 BUT FVC is decreased (<0.8 normal)
  • Gold-standard: High resolution chest CT
    • Ground glass lungs + traction bronchiectasis
124
Q

Treatment of idiopathic pulmonary fibrosis

A
  • Smoking cessation + vaccines
  • Pirfenidone, ninetedanib
  • Surgery (lung transplant)
125
Q

Treatment of idiopathic pulmonary fibrosis

A
  • Smoking cessation + vaccines
  • Pirfenidone, ninetedanib
  • Surgery (lung transplant)
126
Q

What underlying disease should you treat in interstitial lung disease?

A

Connective tissue disease

127
Q

What drugs cause drug-induced pulmonary fibrosis?

A
  • Methotrexate
  • Amiodarone
  • Nitrofurantoin
  • Cyclophosphamide
128
Q

What is hypersensitivity pneumonitis and what type of hypersensitivity reaction is it?

A
  • Hypersensitivity pneumonitis = type III hypersensitivity reaction - to a environmental allergen
  • Causes parenchymal inflammation + destruction in people that are sensitive to that allergen
129
Q

What is key to investigate hypersensitivity pneumonitis?

A

Clinical history = key
* Pets
* Mould
* Occupation

130
Q

Ix for hypersensivity pneumonitis

A
  • Bronchoalveolar lavage (BAL) - raised lymphocytes
  • CT chest (ground-glass shadowing)
  • Serum IgG (positive)
  • Pulmonary function test (restrictive; mixed restrictive/obstructive)
  • TLCO (decreased)
131
Q

Types of hypersensitivity pneumonitis

A
  • Bird-fanciers lungis a reaction to bird droppings
  • Farmers lungis a reaction to mouldy spores in hay
  • Mushroom workers’ lungis a reaction to specific mushroom antigens
  • Malt workers lungis a reaction to mould on barley
132
Q

Mx of hypersensitivity pneumonitis

A
  • Identification + removal of antigen
  • Prednisolone (orally)
  • Smoking cessation, pulmonary rehabilitation, supplemental oxygen
133
Q

Name a disease that causes secondary pulmonary fibrosis

A
  • Alpha-1 antitripsin deficiency
  • Rheumatoid arthritis
  • Systemic lupus erythematosus (SLE)
  • Systemic sclerosis
134
Q

What is sarcoidosis?

A

A granulomatoid inflammatory condition
(Granulomas = nodules of inflammation full of macrophages)

135
Q

Typical patient that presents with sarcoidosis

A

20-40 black woman with a dry cough and shortness of breath. She has nodules on her shins - suggesting erythema nodosum.

May also have uritis

136
Q

What does sarcoidosis do to the lungs?

A
  • Mediastinal lymphadenopathy
  • Pulmonary fibrosis
  • Pulmonary nodules/granulomas (contain macrophages)
137
Q

First line and gold-standard Ix for sarcoidosis

A
  • First line: CXR (hilar lymphadenopathy)
  • Gold standard: Biopsy (non-caseating granuloma)
138
Q

Management of sarcoidosis

A
  • Oral steroids (prednisolone)
  • Immunosupressants (methotrexate or azathioprine)
139
Q

What is pulmonary hypertension?

A
  • Increased pressure + resistance of blood in the pulmonary arteries
  • mPAP > 25mmHg
140
Q

What are some causes of pulmonary hypertension?

A
  • Pulmonary vascular disorders → PE
  • Disease of lung and parenchyma → COPD
  • Cardiovascular → Mitral stenosis, LV HF, congenital heart disease
141
Q

What happens due to pulmonary hypertension?

A

Increased pressure + resistance in the pulmonary arteries causes:
* Strain on the right side of the heart → trying to pump through the lungs
* Also, causes a back pressure of blood into the systemic venous system

142
Q

What is the presentation of pulmonary hypertension?

A
  • Exertional dyspnoea + fatigue + syncope
  • Right-sided heart failure signs
    • Raised JVP
    • Peripheral oedema
    • Louder S2 than normal
143
Q

Ix for pulmonary hypertension

A

ECG:
* Right ventricular hypertrophy
* Right bundle branch block
* Right axis deviation

CXR:
* Dilated pulmonary arteries
* Right ventricular hypertrophy (elevated cardiac apex)

Echo = can be used to estimate pulmonary artery pressure

144
Q

Treatment for pulmonary hypertension

A
  • Sildenfil (phosphodiesterase-5 inhibitors)
  • CCB (amlodipine)
  • Treat underlying cause (PE or SLE)
  • Diuretics for oedema