Respiratory Flashcards
1
Q
What condition is bronchiolitis obliterans associated with?
A
Rheumatoid arthritis
2
Q
What are the features of bronchiolitis obliterans?
A
- Progressive dyspnoea
- Obstructive spirometry
- Centrilobular nodules, bronchial wall thickening on CT
3
Q
What is Caplan’s syndrome?
How does it manifest?
A
Rheumatoid pneumoconiosis. Characterised by intrapulmonary nodules that appear homogeneous and well-defined on CXR
4
Q
What causes Caplan’s syndrome?
A
Occurs in patients with both RA and pneumoconiosis related to mining dust
5
Q
What is the investigation of choice for upper airway compression?
A
Flow volume loop
6
Q
How is a normal flow volume loop often described?
A
Triangle on top of a semicircle
7
Q
What is silicosis?
A
Fibrosing lung disease caused by inhalation of fine particles of crystalline silicone dioxide (silica)
8
Q
Which occupations are at risk of silicosis?
A
- Mining, quarrying
- Slate works
- Foundries
- Potteries
9
Q
What areas of the lungs does silicosis affect?
A
Upper zone fibrosis
10
Q
What are the features of silicosis on CXR?
A
- Upper zone fibrosis (diffuse upper lobe reticular shadowing
- Egg-shell calcification of the hilar lymph nodes
11
Q
What are the features of silicosis on CT?
A
- Diffuse multiple small nodues accompanied by calcifications
- Mass like scarring with calcification and volume loss
12
Q
What does egg shell calcification indicate?
A
Silicosis
13
Q
What is hypersensitivity pneumonitis also known as?
What type of hypersensitivity reaction predominates the acute phase of this condition?
A
Extrinsic allergic alveolitis = type 3 hypersensitivity reaction. Some type 4 hypersensitivity activity too.
14
Q
What type of particles cause hypersensitivity pneumonitis (AKA extrinsic allergic alveolitis)?
A
Organic particles
15
Q
Give 4 examples of EAA/hypersensitivity pneumonitis.
A
- Bird fanciers lung
- Farmers lung
- Malt workers lung
- Mushroom workers lung
16
Q
What causes bird fanciers lung
A
Avian proteins from bird droppings
17
Q
What bacteria causes farmers lung?
A
Spores from Saccharopolyspora rectivirgula (from wet hay)
18
Q
What organism causes malt workers lung?
A
Aspergillus clavatus
19
Q
What organisms are implicated in mushroom workers lung?
A
Thermophilic actinomycetes
20
Q
How does extrinsic allergic alveolitis present in the acute phase?
A
Dyspnoea
Dry cough
Fever
21
Q
How does chronic hypersensitivity pneumonitis/EAA present?
A
- Lethargy
- Dyspnoea
- Productive cough
- Anorexia and weight loss
22
Q
What areas of the lungs are fibrosed in EAA/hypersensitivity pneumonitis?
A
Upper/mid-zone fibrosis
23
Q
What would you find on bronchoalveolar lavage for EAA/hypersensitivity pneumonitis?
A
Lymphocytosis
24
Q
What investigations can you do for EAA/hypersensitivity pneumonitis?
A
- Imaging
- Bronchoalveolar lavage
- Serologic assays for specific IgG antibodies
25
Would you find eosinophilia in EAA/hypersensitivity pneumonitis?
No eosinophilia in EAA.
26
How is EAA managed?
Avoid precipitating factors
Oral glucocorticoids
27
List chemicals associated with occupational asthma
1. Isocyanataes e.g. toluene diisocyanate
2. Platinum salts
3. Soldering flux resin
4. Glutaraldehyde
5. Flour
6. Epoxy resins
7. Proteolytic enzymes
28
Which type of lung cancer is most associated with cavitating lung lesions?
Squamous cell carcinoma
29
Which cancer gives the appearance of "cherry red ball" on bronchoscopy?
Carcinoid tumour
30
Which smoking cessation drug should be avoided in depression?
Varenicline
31
Which smoking cessation drug should be avoided in epilepsy?
Bupropion
32
Gene polymorphisms encoding for what are linked to asthma?
Cytokines
Chemokines
Toll-like receptors
33
List innate immune cells involved in asthma inflammatory response.
Mast cells
Dendritic cells
Macrophages
34
List adaptive immune cells involved in asthma inflammatory response.
T helper type 2 lymphocytes
35
Which mediators are implicated in asthma?
Histamine
Leukotrienes
Cytokines (IL 4, 5, 9, and 13)
Chemokines
36
Describe some structural changes that occur in asthmatic airways.
1. Hypertrophy and hyperplasia of smooth muscle
2. Thickening of the basement membrane
3. Angiogenesis
4. Increased mucous gland size
37
Which T-helper cell is involved in eosinophilic asthma?
Th2
38
Which T-helper cells are involved in neutrophilic asthma?
Th1 or Th17
39
What physical examination findings in acute asthma attack?
Prolonged expiratory phase
Use of accessory muscles
Hyperinflation of the chest
40
Post-bronchodilator improvement in spiro/PEFR of what level suggests asthma?
>12%, >200ml
41
How to investigate for asthma?
Spiro with BD reversibility and FeNO
42
Methacholine challenge: How much decrease in FEV1 indicates hyperreactive airways (asthma)?
20%
43
Explain how NO is produced in asthma
1. nitric oxide produced by 3 types of nitric oxide synthesases.
2. inducible NOS found in eosinophils and other inflammatory cells
3. levels of NO correlate with levels of inflammation
44
List the step wise approach for long term asthma management
1. SABA
2. SABA + ICS
3. SABA + ICS + LTRA
4. SABA + ICS + LABA +/- LTRA
5. SABA + MART +/- LTRA (MART = fast acting LABA + low dose ICS)
6. SABA + MART with medium dose ICS +/- LTRA
7. SABA + high dose ICS + LABA, trial LAMA/theophylline
Specialist referral if necessary
45
What PEFR in moderate acute asthma?
>50-75%
46
PEFR in acute severe asthma?
33-50%
47
PEFR in life threatening asthma?
<33%
48
What are the features of acute severe asthma?
1. PEFR 33-50
2. HR >=110
3. RR >=25
4. Can't speak in full sentences
49
What are the features of life threatening asthma attack?
1. PEFR <33%
2. Normal pCO2
3. Tiring/exhaustion/reduced resp. effort
4. Confusion
5. Reduced GCS
6. Cyanosis
7. Hypotension
8. New arrhythmia
50
What differentiates between life-threatening asthma and near-fatal asthma?
CO2 level normal in life-threatening, raised in near-fatal
51
Should you continue routine inhalers while on acute asthma treatment?
yes, including ICS
52
What is a common complication of mechanical ventilation in asthmatics?
hypotension
53
Which childhood infections can predispose to asthma in later life?
RSV
rhinovirus
54
Which immunoglobulin in atopy/allergy?
IgE
55
How do tobacco and marijuana combined affect COPD risk?
synergistic effect
56
Give an example of indoor pollution linked to COPD
biomass smoke
57
Give an example of outdoor pollution linked to COPD
PM2.5
58
Give example of occupational exposure linked to COPD
coal dust
59
Which gene polymorphisms are linked to COPD?
1. CFTR (cystic fibrosis transmembrane conductance regulator)
2. transforming growth factor beta-1
3. serpine-2
4. antioxidant enzymes
60
How does matrix metalloproteinase dysregulation lead to COPD?
increased MMP-12 is linked to emphysema and reduced lung function
61
How is asthma linked to risk of COPD?
chronic airway inflammation and atopy increases risk of copd
62
How does bronchopulmonary dysplasia link to COPD?
chronic airflow limitation
bronchopulmonary dysplasia occurs due to preterm birth
63
Which infections predispose to COPD?
TB, RSV, HIV
64
Which vitamin deficiencies linked to COPD?
antioxidants - vit C and E
65
Which gender is more predisposed to COPD?
females
66
How are elastase levels linked to COPD?
excess elastase linked to copd
67
How does excess elastase link to COPD?
imbalance between elastases and their inhibitors (eg. alpha 1 antitrypsin) leads to premature emphysema
68
Give examples of proteases
neutrophil elastase
matrix metalloproteinases
69
Give example of antiproteases
alpha-1-antitrypsin
tissue inhibitors of metalloproteinases
70
How does protease activity link to COPD?
excess protease activity degrades extracellular matrix components, leads to destruction of lung parenchyma and emphysema
71
How does smoking lead to oxidative stress?
1. pollutants such as cigarette smoke induces production of reactive oxygen species.
2. this results in oxidative stress
3. oxidative stress causes direct cellular damage and impairs antiprotease activity, and promotes inflammation
72
How does oxidative stress link to copd pathogenesis?
1. impairs antiprotease activity
2. direct cellular damage
3. promotes inflammation
73
What spirometry result confirms COPD?
**POST-BRONCHODILATOR**
FEV1/FVC <0.7 confirms persistent airflow limitation
74
What CXR findings in COPD?
hyperinflation
bullae
75
What is FEV1 in stage 1 mild COPD?
FEV1>80%, if has symptoms of copd
76
What is FEV1 in stage 2 moderate COPD?
FEV1 50-79%
77
What is FEV1 in stage 3 severe COPD?
FEV1 30-49%
78
what is FEV1 in stage 4 very severe COPD?
FEV1 <30%
79
What vaccines for COPD?
annual flu
one off pneumococcal
80
What is general management for COPD?
smoking cessation
annual flu vax
one off pneumococcal vax
pulm rehab
81
What meds in initial mgmt of COPD?
SABA or SAMA
82
What is second line management of COPD if not controlled on SABA or SAMA and steroid responsive?
if steroid responsive: add ICS + LABA, can continue to take either LABA or LAMA
83
If not steroid responsive, what is second line management for COPD not controlled on SABA/SAMA?
LABA + LAMA.
PRN SABA only. No LAMA with SAMA.
84
What is third line mgmt of all COPD patients if not controlled with ICS or with LAMA+LABA?
triple therapy:
LAMA + LABA + ICS + SABA PRN
85
When would you reduce oral theophylline dose?
if going to give macrolide (eg clarithromycin) or fluoroquinolones (eg levofloxacin) alongside
86
Why is azithromycin given as prophylaxis in COPD (re: properties)?
Both antimicrobial and immunomodulatory effects
87
What screening prior to azithromycin, and what monitoring?
1. CT to exclude TB
2. LFT
3. ECG for QTc
4. hearing
88
Which medication can be used to prevent COPD exacerbations, and what class of meds?
phosphodiesterase-4 inhibitor - roflumilast
89
What are the clinical examination findings of Cor pulmonale?
1. peripheral oedema
2. systolic parasternal heave
3. loud P2
4. raised JVP
90
How to treat oedema as a result of Cor pulmonale?
loop diuretics eg furosemide
91
How does COPD link to Cor pulmonale?
1. chronic hypoxia leads to small pulmonary arteries constricting in order to redirect blood flow to better ventilated airways of lungs
2. increased pulm pressure (ie pulm hypertension)
3. chronic pulm htn causes increased RV pressure = cor pulmonale
92
What are the features of RV strain/cor pulmonale on CTPA?
1. flattened intraventricular septum
2. pulmonary trunk enlargement (bigger than aorta)
3. reflux of contrast into IVC
4. enlarged RV
93
What can improve survival in copd patients?
LTOT
94
What are the indications for LTOT?
evidence of chronic hypoxia
1. cyanosis
2. polycythaemia
3. peripheral oedema
95
What pO2 levels needed for LTOT?
pO2 <7.3kPa on 2 occasions at least 3 weeks apart
96
If pO2 7.3-8kPa, what features would make you consider LTOT?
1. polycythaemia
2. cyanosis
3. features of RV strain - oedema, raised JVP, pulm HTN.
97
What are the extrapulmonary complications of COPD?
1. cardiac - cor pulmonale, arrhythmias, IHD
2. depression/anxiety
3. weight loss/malnutrition
4. osteoporosis if many steroids
98
Why weight loss in copd?
increased energy expenditure and reduced appetite.
99
What is lung compliance?
Change in lung volume per unit change in airway pressure
ie. the lungs' ability to stretch and expand
100
How does pulmonary oedema affect lung compliance?
Pulmonary oedema reduces lung compliance
101
How do kyphosis and pneumonectomy affect lung compliance?
reduces
102
Give example of condition that causes low lung compliance.
fibrosis - (thick balloon)
103
Give example of condition that causes high lung compliance.
emphysema - plastic bag
104
How does age affect lung compliance?
Age increases lung compliance
105
What type of molecule is alpha-1 antitrypsin?
protease inhibitor
106
What does A1A inhibit?
neutrophil elastase
107
Where is A1A produced?
Liver
108
Where is the gene for A1A?
Chromosome 14
109
How is A1AT inherited?
Autosomal recessive/co-dominant
110
How are the alleles classified in A1AT?
Electrophoretic mobility
M = normal
S = slow
Z = very slow
111
What does heterozygous PiMZ signify in terms of emphysema risk and inheritance?
Low risk of emphysema if no-smoking
May pass on A1AD gene to children
112
What are the A1AT levels in PiSS?
50%
113
What are the A1AT levels in PiZZ?
10%
114
How does A1AT present in lungs?
Panacinar emphysema, predominantly lower lobes
115
How does A1AT affect liver in children?
Cholestasis
116
How does A1AT affect liver in adults?
Cirrhosis
Hepatocellular carcinoma
117
What is the mechanism behind ARDS?
Increased alveolar permeability leading to alveolar fluid accumulation (pulmonary oedema, non-cardiogenic)
118
How is A1AT treated?
1. No smoking
2. Supportive - bronchodilators, physio
3. IV alpha-1 antitrypsin protein concentrates
4. Lung volume reduction surgery/endobronchial valve surgery, lung transplant
119
How is A1AT investigated?
Spirometry - obstructive picture
A1AT concentrations
120
How would ARDS present?
1. Tachypnoea, Dyspnoea
2. Hypoxia
3. Bilateral crepitations
121
What are the American-European Consensus Conference criteria for diagnosing ARDS?
1. Acute onset (within 1 week)
2. Pulmonary oedema (bilateral infiltrates on CXR)
3. Non-cardiogenic (pulmonary wedge pressure required if uncertain)
4. pO2/FiO2 < 40kPa (300 mmHg)
122
What are some triggers for ARDS?
1. Large blood transfusion
2. Cardiopulmonary bypass
3. Infections, sepsis
4. Trauma
5. Covid-19
6. Acute pancreatitis
7. Smoke inhalation
123
What are the key investigations in suspected ARDS?
CXR and ABG
124
What can be used to confirm non-cardiogenic pulmonary oedema in ARDS?
Pulmonary wedge pressure
125
What is pulmonary wedge pressure and how is it measured?
Catheter wedged into tapering branch of a pulmonary artery
126
How is ARDS treated?
1. Treat underlying cause eg Abx in sepsis
2. Oxygenation/ventilation
3. Organ support, vasopressors
4. Prone positioning, muscle relaxation
127
What does pulmonary wedge pressure estimate?
Left atrial pressure.
128
Which conditions predispose to ABPA?
Atopic/asthma
CF
129
What investigations/findings for ABPA?
1. Eosinophilia
2. Flitting CXR changes
3. IgE levels raised
4. Positive IgG precipitins (not as raised as in aspergilloma)
5. Positive Radioallergosorbent (RAST) test to aspergillus
130
What is ABPA?
Allergy to aspergillus spores
131
What is a bronchocoele, and how are they linked to ABPA?
Impacted mucoid secretions within the bronchial tree.
ABPA is a cause of non-obstructive bronchocoele.
132
Where is bronchiectasis in ABPA?
proximal
133
How is ABPA treated?
1. Oral glucocorticoids
2. Can add on itraconazole as second-line
134
What is an aspergilloma?
Mycetoma (fungus ball) which colonises a pre-existing lung cavity e.g. secondary to lung cancer, CF, tuberculosis
135
What are the clinical features of an aspergilloma?
Cough
Haemoptysis
136
How would aspergilloma present on CXR?
Fungal ball cavity surrounded by crescent sign
137
What investigations for aspergilloma?
CXR
High titres of aspergillus precipitins
138
What is cryptogenic organising pneumonia?
Type of ILD
139
Where does cryptogenic organising pneumonia affect?
1. Distal bronchioles
2. Respiratory bronchioles
3. Alveolar ducts
4. Alveolar walls
140
How does cryptogenic organising pneumonia present?
Cough
SoB
Fever
Malaise
Symptoms present for weeks-months
Non-response to antibiotics
141
How does cryptogenic organising pneumonia present on imaging?
Bilateral patchy consolidation/ground glass changes
142
How does cryptogenic organising pneumonia present on lung function tests?
Restrictive picture, reduced TLCO
143
How is cryptogenic organising pneumonia treated?
Watch and wait if mild
Steroids if severe
144
What is Churg-Strauss also known as?
Eosinophilic granulomatosis with polyangiitis
145
Which ANCA is Churg-Strauss?
pANCA (only in 60% of patients)
146
What are the main clinical features of Churg-Strauss?
1. Asthma
2. Eosinophilia
3. Nerve lesions (mono or polyneuropathy)
4. Paranasal sinusitis
147
What can precipitate Churg-Strauss?
LTRAs (montelukast)
148
What antibodies in Churg-Strauss?
Anti myeloperoxidase antibodies in Churg Strauss
149
What does ANCA stand for?
Anti Neutrophil Cytoplasmic Antibody
150
What are the pathophysiological phases in Churg-Strauss?
1. Prodromal allergic phase - similar to asthma/allergy symptoms
2. Eosinophilic phase - accumulation of Eos in lungs/GI tract - causes pneumonia/gastroenteritis
3. Vasculitic phase - necrotizing small-medium vessel vasculitis
151
How does the vasculitic phase of Churg-Strauss manifest?
1. Purpura
2. Neuropathies
3. Organ damage - renal/cardiac.
152
How does Acute Mountain Sickness present?
1. Nausea
2. Headache
3. Fatigue
153
How is Acute mountain sickness prevented? Explain the mechanism of action of this drug
Acetozolamide
Carbonic anhydrase inhibitor
Causes a metabolic acidosis
Body tries to compensate by causing a respiratory alkalosis
This increases resp rate (to blow off CO2)
Increased resp rate increases oxygenation
154
How is acute mountain sickness treated?
descent
155
What is the pathophysiological mechanism behind High Altitude Pulmonary oEdema (HAPE)?
Hypobaric hypoxia causes uneven pulmonary vasoconstriction
Areas of lung with greater blood flow experience increase in capillary pressure
Causes alveolar oedema
Hypoxia also increases capillary permeability, more fluid leaks into alveoli
156
How does HAPE present?
Typical pulmonary oedema symptoms
157
How is HAPE treated?
What is the proposed mechanism of action for these drugs?
Descent
Nifedipine
Dexamethasone
Acetozolamide
PDE5 inhibitors
Drugs reduce systolic pulmonary artery pressure
158
What is High Altitude Cerebral oEdema (HACE)?
Hypoxia causes cerebral vasodilation
Elevated cerebral blood volume -> fluid leaks out
Hypoxia increases BBB permeability -> more fluid in extracellular space
159
How does HACE present?
Headache
Ataxia
Papilloedema
160
How is HACE managed?
Descent
Dexamethasone
161
What is omalizumab?
MAB to treat severe confirmed allergic IgE mediated asthma.
Binds to IgE
Add on to optimised standard therapy
SC Injection every 2-4 weeks
Can also be used in ABPA
162
List 5 ways that asbestos can affect the lung.
1. Pleural plaques
2. Pleural thickening
3. Asbestosis
4. Lung cancer
5. Mesothelioma
163
What is asbestosis?
Lung fibrosis secondary to asbestos exposure.
Severity of asbestosis is directly proportional to length of asbestos exposure
164
What is the latent period for asbestosis?
10-15 years after exposure
165
What area of lung is affected in asbestosis?
Lower lobe fibrosis
166
How does asbestosis present?
Like other ILD
Dyspnoea on exertion
Clubbing
Bilateral end inspiratory crackles
Restrictive spiro with reduced TLCO
167
How is asbestosis treated?
Conservative management. No current interventions offer a significant benefit
168
What is mesothelioma?
Malignant disease of pleura
169
How does mesothelioma present?
1. Chest pain
2. Progressive dyspnoea
3. Pleural effusion
170
What is the most dangerous form of asbestos in mesothelioma?
Crocidolite (blue) asbestos
171
How is mesothelioma treated?
Palliative chemo
May be offered surgery/radiotherapy
172
How does asbestos exposure link to smoking in the risk of developing lung cancer?
Smoking has a synergistic effect with asbestos - massively increases risk of developing lung Ca.
173
What is atelectasis?
Collapse of lung alveoli
Occurs when airways obstructed by bronchial secretions
174
When does atelectasis usually occur?
72 hours post-op
175
How does atelectasis present?
Dyspnoea
Hypoxia
176
How is atelectasis treated?
Positioning patient upright
Chest physio
177
List some causes of bilateral hilar lymphadenopathy
1. **Sarcoidosis**
2. **TB**
3. Lymphoma/malignancy
4. Pneumoconiosis e.g. berylliosis
5. Fungi e.g. histoplasmosis, coccidioidomycosis
178
List causes of bronchiectasis.
1. Post-infective
2. CF
3. Bronchial obstruction due to foreign body/tumour
4. Immune deficiency - Hypogammaglobulinaemia, selective IgA
5. ABPA
6. Ciliary dyskinetic syndromes: Kartagener, Young's syndrome
7. Yellow nail syndrome
179
What is bronchiectasis?
Permanent dilatation of the airways secondary to chronic infection or inflammation.
180
Which organisms most commonly isolated from bronchiectasis patients?
1. Haemophilus influenzae
2. Pseudomonas aeruginosa
3. Klebsiella spp.
4. Streptococcus pneumoniae
181
Where should chest drains be inserted?
What borders make up the triangle of safety?
"Triangle" of safety (square fr)
Anterior border of latissimus dorsi
Base of the axilla
Lateral edge of pectoralis major
5th intercostal space (nipple line)
182
Which type of lung cancer is associated with cavitating lung lesions?
Squamous cell carcinoma
183
What cancers are associated with cannonball mets?
Renal
Prostate
Choriocarcinoma
184
Which cancers cause calcified lung mets?
Chondrosarcomas
Osteosarcomas
185
Where is the gene for CF located?
Long arm of Chromosome 7
186
What is the most common mutation for CF patients in the UK?
Delta F508
187
How does CFTR mutation cause thickened secretions in CF?
1. Dysfunctional CFTR protein causes Cl- and H2O retention in cells
2. This leads to increased extracellular Na concentration.
3. This prompts reabsorption of Na **and** H2O **together** into cells.
4. Leads to dehydrated and thickened secretions
188
How does CF cause lung damage over time?
1. Thickened mucus causes impaired mucociliary clearance, allows bacteria to colonise
2. This leads to chronic infections and inflammation in the airways
3. Chronic inflammation and mucoid obstruction over time lead to bronchiectasis and structural changes
4. Eventually leads to respiratory failure
189
How does CF affect the GI system?
1. Pancreatic blockage - leads to malabsorption and steatorrhoea
2. Intestinal obstruction - meconium ileus/distal intestinal obstruction syndrome in adults
3. Liver involvement - blocked bile ducts leads to focal biliary cirrhosis
190
How can CF affect fertility?
In males: congenital bilateral absence of vas deferens (obstructive azoospermia)
In females: thickened cervical mucus, irregular menses
191
How does CF cause dehydration?
1. CFTR dysfunction causes reduced reabsorption of chloride and sodium from sweat.
2. Salty sweat increases dehydration and electrolyte imbalance esp in exercise
192
What respiratory symptoms in CF?
1. Chronic cough
2. Wheezing and dyspnoea
3. Recurrent respiratory infections
4. Chronic sinusitis and nasal polyps
193
What are the most common organisms which cause infections in CF patients?
1. Staph Aureus
2. Pseudomonas aeruginosa
3. Burkholderia cepacia
4. Haemophilus influenzae
194
Which vitamin deficiencies commonly found in CF?
Fat soluble vitamins
ADEK
195
What GI symptoms in CF?
1. Meconium ileus, distal intestinal obstruction syndrome in adults
2. Pancreatic insufficiency
3. Biliary cirrhosis
4. GORD
196
What is distal intestinal obstruction syndrome?
Partial or complete intestinal obstruction due to inspissated faecal material.
197
How does CF affect liver?
bile duct obstruction
leads to cirrhosis and portal hypertension and potential liver failure
198
How does GORD affect CF patients?
Increased prevalance in CF.
Exacerbates pulmonary complications
199
How does CF lead to haemoptysis and how is this treated?
Bronchial wall erosion due to chronic inflammation/infection
May need pulmonary artery embolisation
200
Give 2 endocrine manifestations of CF.
1. CF related diabetes due to reduced insulin secretion in pancreas + insulin resistance
2. Growth problems - delayed growth and puberty due to malabsorption, chronic inflammation, and increased energy expenditure
201
What diagnostic tests for CF?
1. Newborn heelprick - immunoreactive trypsinogen
2. Chloride sweat test
3. Genetic testing
202
What substance in CF newborn heelprick test?
Immunoreactive trypsinogen
203
What are three other clinical features of CF (aside from lung/GI)
1. Clubbing due to chronic hypoxia
2. Osteoporosis (reduced Vit D absorption, chronic inflammation and freq. steroids)
3. Salt loss syndrome
204
What is salt loss syndrome in CF?
Excessive NaCl loss in sweat leads to hyponatraemic dehydration
Also metabolic alkalosis, particularly in children
205
How to improve airway clearance in CF?
1. Chest physio/positioning
2. High frequency chest wall oscillation
3. Exercise
206
List some pharmacological interventions that can help in CF.
1. Mucolytics - dornase alfa, hypertonic saline
2. Bronchodilators
3. Antibiotics (inhaled in Pseudomonas colonisation), rotational prophylaxis
4. Anti-inflammatories - steroids and NSAIDs
5. CFTR modulators - ivacaftor, lumacaftor, elexacaftor
207
List CFTR modulators
ivacaftor
lumacaftor
elexacaftor
208
How are nutritional manifestations of CF managed?
1. Pancreatic enzyme supplementation
2. High fat, high calorie diet
3. Fat soluble vitamin supplements (ADEK)
209
How can CF liver disease be managed?
Regular LFT monitoring
Early UDCA
Can consider liver transplant in severe cases
210
List 4 respiratory complications of CF.
1. Bronchiectasis
2. Pneumothorax
3. Haemoptysis due to bronchial wall erosion - may need bronchial artery embolisation
4. Respiratory failure - may need supplemental oxygen/lung transplant to extend life
211
List 3 other complications of CF (not lung/GI/endo).
1. Sinusitis/nasal polyps
2. Bone disease (osteoporosis)
3. Kidney stones due to dehydration, alkaline urine, and hyperoxaluria
212
What causes nephrolithiasis in CF?
Combination of:
1. Dehydration
2. Alkaline urine
3. Hyperoxaluria
213
How do ipratropium and tiotropium work?
Anti-muscarinic
Block acetylcholine receptors
214
What class of drug is theophylline and how does it work?
Methylxanthines
Non-specific inhibitor of PDE, increases cAMP
215
List triad of systems implicated in Wegener's
1. Upper respiratory tract
2. Lower respiratory tract
3. Glomerulonephritis
216
How does Wegener's manifest in the upper respiratory tract?
1. Chronic sinusitis
2. Nasal crusting/discharge
3. Epistaxis
4. Subglottic stenosis - hoarseness/stridor
5. Otitis media, hearing loss, ear pain
Can have oral ulcers
217
How does Wegener's manifest in the lower respiratory tract?
1. Cough, dyspnoea
2. Haemoptysis
3. Pleuritis
4. Pulmonary infiltrates/nodules
218
How does Wegener's manifest in the kidneys?
1. Microscopic haematuria
2. Urinary sediment
3. Pauci-immune Rapidly progressing glomerulonephritis, can lead to CKD
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How can Wegener's affect the eye?
1. Ocular pseudotumours - proptosis, diplopia, visual loss
2. Episcleritis/scleritis
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How can Wegener's manifest in skin?
1. Leukoclastic angiitis - purpura, ulceration, vesicles
221
List neurological manifestations of Wegener's
1. Mononeuritis multiplex
2. Peripheral sensorimotor polyneuropathy
3. Cranial nerve palsies
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List investigations for Wegener's
1. cANCA positive in >90%, pANCA positive in 25%. (specifically anti-proteinase 3)
2. CXR - nodular changes, cavitating lesions
3. Endothelial crescents in Bowman's capsule on renal biopsy
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Which proteinase implicated in Wegener's
proteinase 3
224
What is the gold standard for diagnosis in Wegener's?
Histopathological confirmation of necrotising granulomatous inflammation from affected organ
225
How is Wegener's treated?
1. Steroids
2. Cyclophosphamide
3. Plasma exchange
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What prophylaxis alongside Wegener treatment?
Osteoporosis prophylaxis
Pneumocystis prophylaxis
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List 3 respiratory complications of Wegener's
1. Pulmonary haemorrhage
2. Otitis media, chronic sinusitis
3. Pulmonary fibrosis
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How can orbital pseudotumour present in Wegener's
Proptosis
Diplopia
Eye pain
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How does IPF present?
1. Progressive exertional dyspnoea
2. Bibasal fine end-inspiratory crackles
3. Clubbing
4. Dry cough
230
What are the features of IPF on HRCT?
Bilateral patchy irregularities, small, peripheral
Initially ground-glass, progressing to honeycombing
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What investigations in IPF?
1. HRCT
2. Spirometry - restrictive + reduced TLCO
3. ANA positive in 30%, RF positive in 10%
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How is IPF treated?
1. Pulmonary rehabilitation
2. Antifibrotics - nintedanib, pirfenidone
3. May need oxygen supplementation, lung transplant
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What are some respiratory complications of IPF?
1. Respiratory failure
2. Pulmonary hypertension and cor pulmonale
3. Lung infections
4. Lung Ca
234
What are some complications of IPF (not respiratory)?
1. VTE
2. GORD
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What is Loffler syndrome?
Eosinophilic pneumonia
236
What are common triggers for Loffler's syndrome?
1. Parasitic infections (Ascaris lumbricoides, Strongyloides stercoralis) - **Type 2 hypersensitivity**
2. Drug induced - NSAIDs, antibiotics, anticonvulsants
3. Idiopathic
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How is Loeffler's treated?
1. Treat underlying cause - antiparasitics if indicated
2. Steroids
Oxygen if required
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What is Kartagener's syndrome?
Primary ciliary dyskinesia
239
What is the pathophysiology of Kartagener's?
Defects in dynein arm leads to immotile cilia
240
What are the features of Kartagener's?
Dextrocardia or complete situs invertus
Bronchiectasis
Recurrent sinusitis
Subfertility
241
How does dextrocardia present in exam questions?
Quiet heart sounds
Small volume complexes in lateral leads
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What is the mechanism behind subfertility in Kartagener's?
Males: impaired sperm motility
Females: impaired ciliary motility in Fallopian tubes
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How is Kartagener's managed?
1. Chest physio
2. Mucolytics such as N-Acetylcisteine
3. Bronchodilators
4. Antibiotics (both prophylactic and treatment)
5. Sinus management (saline irrigation, topical steroids)
6. Surgery - lobectomy in localised bronchiectasis, sinus surgery if recurrent
7. Vaccinations against resp. organisms (flu/pneumococcal)
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Where is Klebsiella normally found?
Klebsiella is part of normal gut flora
245
What type of pneumonia is commonly caused by Klebsiella?
Aspiration pneumonia
246
In what groups does Klebsiella pneumonia tend to occur?
1. Alcohol excess
2. Diabetes
247
Where in lungs does Klebsiella tend to affect?
Upper lobes
248
How is Klebsiella sputum often described?
Red currant jelly sputum = klebsiella
249
What are the complications of Klebsiella pneumonia?
1. Lung abscess
2. Empyema
250
Which receptors are blocked by LTRAs (montelukast, zafirlukast)?
CysLT1
251
What is Lofgren's syndrome?
Form of sarcoidosis characterised by:
1. Bilateral hilar lymphadenopathy
2. Erythema nodosum
3. Fever
4. Polyarthralgia
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What is the prognosis of Lofgren's syndrome?
Lofgren = Excellent prognosis
