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Respiratory Flashcards

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1
Q

What is FEV1?

A

The volume of air that can be forcibly expired in 1 sec

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2
Q

What is FVC?

A

The total volume of air that can be forcibly exhaled after maximum inhalation

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3
Q

If FEV1 is reduced so the FEV1/FVC is <0.7, what does this suggest?

A

Patient can’t expire air quickly enough, obstructive - asthma, COPD

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4
Q

What would it suggest if FVC was reduced so FEV1/FVC is >0.8?

A

Restriction - pulmonary fibrosis

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5
Q

What is type 1 respiratory failure?

A

low pO2 and low normal pCO2 -PE

fibrosis causes, lung fails to fill properly

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6
Q

What is type 2 respiratory failure?

A

Low pO2 and high pCO2 - hypoventilation
Obstruction causes can’t remove CO2 properly - codp , asthma

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7
Q

What is Chronic Obstructive Pulmonary Disease, COPD?

A

COPD is a disease state that is characterised by airflow obstruction, usually is progressive and isn’t fully reversible

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8
Q

What are the risk factors of COPD?

A
  • smoking
    -air pollutants
    -Alpha 1 Antitrypsin deficiency
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9
Q

What is alpha 1 anti trypsin?

A

A protein produced in your liver that helps protect your lungs - inhibits neutrophil elastase which degrades elastin

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10
Q

What are the 3 associations of the pathology of COPD?

A
  1. Chronic bronchitis
  2. Emphysema
  3. A1AT deficiency
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11
Q

What is Chronic bronchitis?

A

Hypertrophy and hyperplasia of mucous secreting glands. Chronic inflammation cells infiltrate bronchi and bronchioles causing luminal narrowing. Restricts airflow

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12
Q

What are the 3 pathophysiology changes in chronic bronchitis?

A

1.Mucus hypersecretion
2. cilliary dsyfunction
3.narrowed lumen
All increases the chance of infection and traps air inside

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13
Q

What would chronic bronchitis present with?

A

cough for 3+ months , over 2+ years with sputum

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14
Q

What is blue bloater associated with?

A

Chronic bronchitis - it is the typical presentation

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15
Q

What is Emphysema?

A

Destruction of elastin layer in alveoli ducts/ sacs and respiratory bronchioles. Elastin usually keeps walls open during expiration. Without elastic this causes air trapping and expiratory airflow limitation. Also large air sacs are called Bullae

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16
Q

What is pink puffer associated with?

A

The typical presentation of a patient with Emphysema

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17
Q

What is A1AT deficiency?

A

Autosomal codominnat inheritance. A1AT degrades neutrophil elastae to protect excess damage to elastin layer especially in the lungs. If this is deficient then NE levels are high causing panacinar Emphysema.

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18
Q

What is the typical presentation of someone with COPD?

A

Typically older patient with a chronic cough, with purulent sputum and extensive smoking history and Contant dyspnoea.
Blue boater or pink puffer presentation or usually both.

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19
Q

What is the presentation of a blu bloater?

A
  • Chronic purulent cough
    -dyspnoea
    -cyanosis
    -obesity
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20
Q

What is the presentation of a pink puffer?

A

pursed lip breathing
Barrel shaped chest
Hyperesonance

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21
Q

What is the dyspnoea grading scale MRC?

A

used to assess the baseline disability due to dyspnoea
1 - difficulty breathing during strenuous exercise
2/3
4 - difficulty when just doing minor tasks

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22
Q

What are the investigations for COPD?

A

Pulmonary function tests - spirometry = If FEV1/FVC <0.7 suggests COPD
-Irreversible on bronchodilators-
(<12% improved FEV1)
DlCO( diffusing capacity of CO across lung) = low in COPD
CXR- May show flattened diaphragm from hyperventilation and bullae formation

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23
Q

What are the treatments for COPD?

A
  1. Smoking cessation
  2. Vaccines - influenza/pneumoccal
  3. Short acting beta 2 agonists - Salbutamol
    4.Short acting beta 2 agonist and long acting beta 2 agonist - salmeratol and long acting anticholinergic agonist -tiotropium bromide
  4. SAB2A +LAB2A + LAM3A + ICS - inhaled corticosteroids
  5. Oxygen therapy - 15hr a day for 3 weeks if severe - <88%/90%
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24
Q

What are beta agonists?

A

Bronchodilators - increased cAMP = smooth muscle relaxation

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25
What are anticholinergics?
Bronchodilators - promotes cGMP degradation = smooth muscle relaxation
26
What causes a COPD exacerbations?
Respiratory tract infections
27
How would you treat a COPD exacerbation?
Oxygen - titrate to improve hypoxaemia ( target saturation = 88-92% for COPD patient and 94-98% for normal) - nebuliser salbutamol and ipatropium bromide -ICS and Abx
28
What is Asthma?
Chronic reversible airway disease characterised by reversible airway obstruction, airway hyper responsiveness, inflamed bronchioles and mucus hyper secretion.
29
What is the epidemiology of Asthma?
-Commonly starts in childhood (3-5) -More common in developed countries
30
What are the types of asthma?
Allergic - 70% and non-allergic - 30%
31
What is allergic asthma?
IgE mediated; extrinsic Type 1 Hypersensitivity - due to environmental trigger(Pollen, smoke, mould, antigens) -consider genetics and hygiene hypothesis - often early presentation
32
What is non-allergic asthma?
Non IgE mediated; intrinsic - may present later in life, harder to treat -associated with smoking
33
What are the triggers for asthma?
infection allergens cold weather exercise drugs - BB, aspirin
34
What is atopic triad?
atopic rhinitis Asthma Eczma These 3 conditions together is known as atopy
35
What is samter's triad?
A disease characterised by the triad: - nasal polyps -asthma -aspirin sensitivity
36
What is the pathology of asthma?
Overexpressed TH2 cells in airways exposed to trigger --> TH2 cytokine release IL3,4,5,13, IgE production and eosinophils recruitment --> IgE mast cell degradation: -histamines -Leukotriene -Tryptase Esonphilia release of toxic proteins e.g MBP ---> This causes bronchial constriction and mucus hyper secretion and over time = chronic remodelling
37
What is chronic remodelling?
bronchial scarring decreases lumen size and increases mucus secretion
38
What is the presentation of someone with asthma?
Wheeze, cough, chest tightness, SOB - typically episodic with triggers and diurnal variation - often younger patient
39
What would show if you do a microscopy of mucus that had been coughed up? ASTHMA
curschmanns spirals Charcot leyden crystals
40
What are the episodes classed as?
Moderate - PEF = 50-75% Severe - PEF= 33-50% / can't finish sentences Life threatening - PEF -<33%, low consciousness Fatal - hypercapnic
41
What are the investigations for asthma?
FeNO is high and spirometry shows obstruction = FEV1/FVC<0.7 - but bronchodilator reversible = >12% FEV1 increase = asthma
42
What is FeNO test?
FeNO devices are used to diagnose asthma. FeNO devices measure fractional exhaled nitric oxide in the breath of patients. Nitric oxide is a biomarker for asthma which provides an indication of the level of inflammation in the lungs.
43
What is the treatment for Asthma?
1. SAB2A 2. SAB2A + ICS BEFORE DRUG ADDITION ASSESS TECHNIQUE AND COMPLIANCE 3. SAB2A +ICS + LTRA 4.SAB2A + ICS +LAB2A -/+ LTRA 5. Increase ICS dose
44
What is LTRA?
Leukotriene receptor antagonist - Montelukast
45
What is the treatment for asthma exacerbations?
OSHITME : - O2 -Nebulised SAB2A -ICS Hydrocortisone -IV MgSO4 - bronchodilation -IV theophylline -MgSO4 IV -escalate BIPAP - bilevel positive airway pressure +/- Abx - if infection present
46
What is the epidemiology of lung tumours?
- Bronchial carcinoma most common -M>F -1/3 of all cancer deaths
47
What are common metastases sites?
- bone - liver -adrenals -brain -lymph nodes
48
What are the types of Lung cancers?
Pleura = mesothelioma Lung parenchyma= bronchial - Small cells or non small cells (squamous, adenocarcinoma, carcinoid, large cell)
49
What is Mesothelioma?
Malignancy of the pleura
50
What is the cause of mesothelioma?
Asbestos - typically doesn't present until decades after exposure - latent period Typically males, 40-70
51
What is the presentation of mesothelioma?
Cancer Sx - Weight loss, night pain Lung Sx - shortness of breath, persisted cough, pleuritic chest pain - tumour may press on nearby structures - recurrent laryngeal (hoarse voice) - also signs of metastases-bone pain
52
What is the diagnosis for mesothelioma?
-CXR + CT - imaging first line - pleural thickening (+/- effusion) -CA - 125 (Cancer antigen 125) high level - non specifically raised in many tumours; sensitive but not specific -Lung Biopsy = diagnostic
53
What is the treatment for mesothelioma?
-Aggressive tumour BUT isn't like to metastasise as affects pleura; pleura isn't found everywhere in the body - usually palliative -If found early can try surgery, chemo/ radio ( generally resistant)
54
What is Bronchial carcinoma?
Malignancy of lung parenchyma - can be either small cell (15%) or non small cell(85%)
55
What are the risk factors of bronchial carcinoma?
- smoking -asbestos -coal -ionising radiation -lung disease already present
56
Who is affected by small cell bronchial carcinoma?
Exclusively smokers
57
What is associated by SCLC?
Paraneoplastic syndromes: -ectopic ACTH --> cushings -Ectopic ADH --> SIADH -Lambert Eaton syndrome (autoimmune disorder of nmj)
58
What is SCLC?
- fast growing- early mets -central lung lesions - most agressive
59
What are the types of non small cell bronchial carcinoma?
- squamous -Adenocarcinoma -Carcinoid tumours -Large cell
60
What is Squamous cell carcinoma?
- 25% -mostly smokers -affects central lung, lesions with central necrosis May secrete PTH -> hypercalcemia -arise from lung epithelium -locally spread mostly -late mets
61
What can cause hypertrophic pulmonary osteoarthropathy?
Squamous cell carcinoma as secretes PTH so hypercalcemia. - clubbing -arthritis -periostitis Biggest cause of secondary HPO = adenocarcinoma
62
What is adenocarcinoma?
-40% -commonly asbestos -affect peripheral lung -arise from mucus secreting glandular epithelium -Mets common -> bone, brain, adrenals, lymph nodes,liver
63
What causes carcinoid tumour?
Associated with genetics; MEN1 mutation and neurofibromatosis 1.
64
What is carcinoid tumour?
Neuroendocrine tumour secretes serotonin, arise in GIT and sometimes lung. Symptoms only appear when Liver mets present
65
What are the general symptoms of Bronchial carcinomas?
chest pain, cough, haemoptysis, cancer Sx, signs of mets (hoarse voice,horners syndrome, Pemberton sign)
66
what is the diagnosis of bronchial carcinoma?
-Imaging first line - CT,CXR -Diagnostic -bronchoscopy/ lung biopsy -MRI - staging TNM
67
What are the treatments for small cell carcinoma?
More aggressive so if early consider chemo/radio Metastasised = palliative
68
What is the treatment for non small cell carcinoma?
Less aggressive - early surgical excision Metastasised = chemo/radio Mab therapy cetuximab
69
Why are secondary lung tumours more common/likely than primary lung tumours?
Lungs oxygenate 100% blood therefore all blood comes to the lungs therefore higher mets risk. ESP: breast, kidney, bowel and bladder cancers
70
What is a Pancoast tumour?
Tumour in lung apex metastasises to necks sympathetic plexus - causing horners syndrome ; ptosis, myosis, andhidrosis
71
What is a BALT lymphoma?
A non hodgkin lymphoma originate in bronchi (bronchial associated tissue lymphoma)
72
What is a pulmonary embolism?
where the pulmonary artery circulation is blocked by a blood clot, typically a DVT
73
What are the risk factors of a PE?
anything affecting the virchows triad + FHx
74
What is the virchows triad?
3 components of thrombus formation - endothelial injury -venous statsis -hypercoaguability
75
What causes endothelial injury?
- smoking -HTN -trauma -vascular catheters
76
What causes venous stasis?
- immobility (long flight) -post surgery -varicose veins -AF
77
what causes hypercoaguability?
- acquired: pregnancy, obesity, malignancy -inherited: factor v leiden, antiphospholipid syndrome, Protein c+s deficiency
78
What is the pathology of a pulmonary embolism?
DVT embolisms and enters right side of heart via IVC -> occluding pulmonary artery small vessels causing A-a gradient to increase and a V/Q mismatch (ventilation with no perfusion) - this causes reactive bronchoconstriction therefore dyspnoea and smaller airways ->embolus incraeses pulmonary blood pressure -> pul HTN -> RV strain/ RH
79
What is A-a gradient?
The Alveolar–arterial gradient, is a measure of the difference between the alveolar concentration of oxygen and the arterial concentration of oxygen
80
What is a V/Q mismatch?
happens when part of your lung receives oxygen without blood flow or blood flow without oxygen LOW- no ventilation with perfusion High - ventilation, no perfusion
81
What is the presentation of a pulmonary embolism?
-Classically sudden onset SOB + pleuritic chest pain with swollen painful calf (DVT) and Hx of immobility -Haemoptysis -increased JVP -tachycardia and dyspnoea -signs of RHF= hypotensive, tachycardia, peripheral oedema
82
What are the investigations for PE?
Wells score = <4 - unlikely so do D-dimer = <500ng/ml PE unlikely >4 PE likely - Gold standard -CTPA - clot = PE and no clot exclude PE -ECG -CXR - usually normal (Ddx pleural effusion = visible on CXR)
83
What is D-dimer?
measures plasmin therefore measures clot burden - sensitive but not specific
84
What is the wells score?
the most widely used clinical decision tool for the diagnosis of deep vein thrombosis (DVT) - 8point maximum
85
What are the ECG characteristics?
S1Q3T3 = - S waves deep in lead 1 -Q waves deep in lead 3 -T waves inverted in lead 3 RBBB V1-3 -> RSR pattern due to right axis deviation -sinus tachycardia
86
What is the treatment for PE?
Haemodynamically stable = 1st line - DOAC - apixaban (LMWH if DOAC CI) 2nd Line - warfarin Haemodynamically unstable = Thrombolysis (clot busting) - alteplase If fails - catheter embolectomy
87
What is the prophylaxis for PE?
- compression stockings -regular walking
88
What is pneumonia?
Fluid exudation into alveoli due to inflammation from an infection - typically bacterial but can be viral.
89
What are the two forms of pneumonia?
Community acquired pneumonia -CAP Hospital acquired pneumonia -HAP
90
What is community acquired pneumonia?
In the community or <48hr in a hospital
91
What bacteria causes CAP?
-S.pneumonia - mc -H.influenza -mycoplasma pneumonia - causes atypical pneumonia -others: -S.aures -Legionella -chlamydia pneumonia
92
What is strep pneumonia?
- gram postive cocci -alpha haemolytic -optochin sensitive
93
What fungi can cause pneumonia?
Pneumocystis jiroveci - in immunocompromised patients - AIDS defining illness
94
What is HAP?
Hospital acquired pneumonia, >48hr of hospital admission
95
What bacteria causes HAP?
P. auerginosa E.coli. Klebsiella ALL of the above gram negative aerobic bacilli (MRSA)
96
How can you get pneumonia?
Typically from inhaled pathogens, can also be from aspiration.
97
What are the risk factors of pneumonia?
- immunocompromised (HIV, long term steroids) -IVDU(s.aures) respiratory pre existing disease -v.young/ old
98
What is typical pneumonia?
Bacteria invades + exudate forms inside alveoli lumen + sputum
99
What is atypical pneumonia?
Bacteria invades + exudate forms in interstitial of alveoli + dry cough
100
What is the presentation of pneumonia?
- productive cough with rusty coloured sputum (purilent) -pyrexic due to infection -pleuritic chest pain - worse with breathing and cough -tachypnoea, dyspnoea( tachycardia and hypotension) - confusion in elderly ATYPICAL - dry cough, low grade fever
101
What is the diagnosis of pneumonia?
-1st line diagnostic: CXR shows consolidation - airbronchogram -air fused bronchi made visible by specified fluid filled surrounding alveoli -Sputum sample + culture -CAP assess severity
102
How do you assess the severity of CAP?
CURB65: Confusion - abbreviated mental test Urea nitrogen >7mmol/L Respiration Rate>30/min BP - low systolic <90mmHg or diastolic <60 Age >65
103
What would happen if the score was 1?
3% mortality - Outpatient, oral Abx and discharge
104
What would happen if the patient scored 2?
Consider short hosp stay + Abx
105
What would happen if the patient scored 3 and above?
15% mortality, Hospital. --> ICU +IV abx
106
What is the treatment for pneumonia?
02 (stats 94-98%, broad spec Abx, Analgesia (NSAIDS) for pleuritic chest pain
107
What antibiotics would be used for CAP - CURB 0-2?
Amoxicillin
108
What antibiotics would be prescribed for CAP - CURB 3 to 5?
CoAmoxiclav + Clarithromycin
109
What would be needed to treat legonella?
Clarithromycin - notifiable disease
110
What is aspiration pneumonia?
- seen in presentation with stroke, bulbar palsy and myasthenia gravis -Aspiration of gastric contents into lungs can be fatal due to destructiveness of gastric acid
111
What is the epidemiology of tuberculosis?
- top infectious killer in the world - global incidence on the decline -Most commonly seen in Africa and Asia
112
What is tuberculosis?
Granulomatous caseating disease, caused by mycobacteria
113
What are the 4 species that make up mycobacterium complex?
- M. tuberculosis -M. africanum -M. microtis -M.bovis These cause TB
114
Where is tuberculosis most common?
- South Asia (India ,China, Pakistan) and Africa -1.7billion worldwide have TB (mostly latent)
115
How is TB spread?
Airborne
116
What are the risk factors of TB?
- country + travel associated - immunocompromised (HIV) - IDVDU -smoking + alcohol -Ascending age
117
What are MTC features?
- non motive and non spore forming -mycocilic acid capsule - stain acid fast bacilli with ziehl Nielsen - go red -resistant to phagocyte killing - slow growing 15-20hr
118
What is the pathology of tuberculosis?
TB phagocytosed but resist killing therefore form granuloma --> T cells recruited and central region of granuloma undergoes caseating necrosis --> forms primary Ghon focus in upper parts of the lung(increased ventilation)--> Ghon focus spreads to nearby lymph nodes - Ghon complex (in most infection contained within granulomas, but TB doesn't die - known as latent TB) --> if TB spreads systemically = Miliary TB
119
What is the presentation of tuberculosis?
If symptomatic (not latent as latent =Asx) -Characteristic night sweats and weight loss -Pyrexia, chest pain and patient looks very unwell -extrapulmonary - meningitis, skin change, TB pericarditis Sx, joint pain
120
What is the diagnosis for tuberculosis?
-Mantoux skin test - (red lump if TB) (latent or active) -Sputum culture 3x - the acid fast bacilli = bright red with ziehl Neelson stain -CXR biopsy
121
What is the treatment for TB?
RI2PE6: Rifampicin - 2months - SE: haematuria Isoniazid - 2months -SE: peripheral neuropathy Pyramidine - 6months - SE: hepatitis Ethambutol - 6months-SE: eye problems - optic neuritis Refer to public health England
122
What is an interstitial lung disease ?
interstitial lung disease (ILD) is an umbrella term used for a large group of diseases that cause scarring (fibrosis) of the lungs. The scarring causes stiffness in the lungs which makes it difficult to breathe and get oxygen to the bloodstream.
123
What is a granulomatous ILD?
Sarcoidosis
124
What is an inhalational ILD?
Hypersensitivity pneumonitis Pneumoconiosis (Asbestos/silicosis)
125
What are idiopathic pneumonias ILD?
Pulmonary fibrosis -most common ILD Non pulmonary fibrosis
126
What are conn tissue ILD?
Scleroderma, R.A
127
What are drug induced ILD?
Amiodarone, biemycin
128
What are other ILD?
Good pastures, vasculitis
129
What is pulmonary fibrosis?
-Commonest ILD, seen in older men who smoke, idiopathic - unknown cause
130
what are the risk factors of pulmonary fibrosis?
smoking, occupational (e.g dust), drugs(methotrexate), Viruses (EBV,CMV)
131
What is the pathology of pulmonary fibrosis?
pulmonary fibrosis is progressive scarring --> type 1 respiratory failure
132
What is the presentation of pulmonary fibrosis?
exertion dyspnoea, dry unproductive cough
133
What is the diagnosis for pulmonary fibrosis?
Spirometry = restriction; FEV1/FVC >0.7 BUT FVC lower than normal <0.8 Gold standard imaging = CT chest= ground glass lungs - traction bronchiolectasis
134
What is traction bronciolectasis?
dilation of bronchioles due to airway damage
135
What is the treatment for pulmonary fibrosis?
smoking cessation + vaccines Pirfenidone , nintendinib - help slow down the process of scarring Surgery- lung transplant
136
What is pneumoconiosis?
Occupationally acquired interstitial lung disease caused by inhalation of mineral dust.
137
What is silicosis?
Inhalation of silicon dioxide, egg shell calcification at hilar lymph nodes
138
What is asbestosis?
Inhalation of asbestos; affects pleura ; mesothelioma
139
What is sarcoidosis?
Multi system granulomatous disease with lung involvement. Characterised by non caseating granulomas throughout the body.
140
What is the epidemiology of sarcoidosis?
Common in women 20-40y.o African Caribbean
141
What is the presentation of sarcoidosis ?
- fever, fatigue -dry cough, dyspnoea -eye lesions (anterior uveitis), lupus permio- blue-red nodules on nose and cheeks -erythema nodosum -bilateral hilar lympadenopathy
142
What is the diagnosis for sarcoidosis?
-CXR (staging 1-4) - shows bilateral hilar adenopathy + pulmonary infiltrates - Diagnostic : biopsy shows non caseating granuloma -Serum Ca high -Serume ACE high
143
What is the treatment for sarcoidosis?
Early stages - self resolving Later stages, 2-4 (symptomatic) = corticosteroids
144
What is lofgren syndrome?
A subset of sarcoidosis Triad of : -erythema nodosum -bilateral hilar adenopathy -fever
145
What is hypersensitivity pneumonitis?
T3 hypersensitivity; immune antibody- antigen complex deposition in lung tissues causing immune hyper response.
146
What are the risk factors of hypersensitivity pneumonitis?
occupation (e.g farmer_, bird keeping
147
What are the types of of hypersensitivity pneumonitis ?
Farmers lung - (mc) - mouldy hay Pigeon fancier's lung - bird droppings Malt worker's lung Cheeseworker's lung Humidifier fever
148
What is the treatment for hypersensitivity pneumonitis?
remove allergen
149
What is good pastures syndrome?
T2 autoimmune hypersensitivity response
150
What is the pathology of goodpasture's syndrome?
Autoantibodies ANTIGBM attack lungs and kidneys - lung fibrosis, glomerulonephritis
151
What is the diagnosis of good pasture's syndrome?
lung and kidney biopsy = damage + Ig deposition Serum Anti - GBM positive
152
What is the treatment of goodpasture's syndrome?
supportive corticosteroids plasma exchange - rid of anti - GBM
153
What is Wegener's granulomatosis (Granulomatosis with polyangitis)
Granulomatosis vasculitis where small and medium blood vessels become inflamed typically affecting the ENT< lungs and kidneys. c-ANCA associated vasculitis
154
What is the presentation of GPA?
ENT = saddle shaped nose, ear infection Lungs= diffuse alveolar haemorrhage therefore haemoptysis Kidney = glomerulonephritis therefore haematuria
155
What is the diagnosis of GPA?
cANCA positive
156
What is the treatment for GPA?
Corticosteroids Immunosuppression - rituximab
157
What is Bronchiectasis?
permanent dilation of bronchioles and excessive mucus in them - usually affects lower lobes
158
What is the pathology of bronchiectasis?
irreversible dilation, loss of cilia and mucus hyper secretion, increased risk of infection as low muco-ciliary clearance
159
What are the risk factors of bronchiectasis?
-most common after an infection (TB,pneumonia) -CF,HIV
160
What is the presentation of bronchiectasis?
productive cough with lots of sputum, dyspnoea
161
What is the diagnosis of bronchiectasis?
Imaging - CXR - -High Resolution CT gold standard = dilated thickened bronchi (signet ring sign) + cysts at end of bronchioles -Spirometry = obstructive FEV1/FVC <0.7 -sputum culture. - (check infection) - H.influenza , pseudomonas aeruginosa, s.aures, s.pneumonia
162
What is the treatment for bronchiectasis?
non curative conservative - chest physio and stop smoking bronchodilators Abx for infections H.influenza and s.pneumonia = Amoxocillin Pseudomonas aeruginosa = ciprofloxacin S.aures= flucloxacillin
163
What is cystic fibrosis?
Autosomal recessive condition, a mutation in the CFTR gene on chromosome 7 which causes a defective CFTR production. Most commonly the F508 mutation.
164
What does CFTR stand for?
Cystic fibrosis transmembrane conductance regulator
165
What are the risk factors of cystic fibrosis?
FHx, caucasians - mostly diagnosed in childhood - 90% before 8 years old
166
What is the pathology of CF?
Defective CFTR gene - normally secretes CL- (actively)and Na+(+H2O) (passively) into ductal secretions therefore making them thin and watery. Now they are thicker with high retention of Na+ and Cl-. Lung - impaired mucociliary clearance as mucus is extra thick therefore stagnation causing an increase in infection risk and difficulty breathing and increased risk of bronchiectasis .
167
What is the presentation of cystic fibrosis?
Resp - thick sticky sputum cough, recurrent upper respiratory infection, bronchiectasis Neonates - Meconium ileus (earliest stool too thick to pass through bowel --> bowel obstruction) + failure to thrive GIT- thick secretions, pancreatic insufficiency, bowel obstruction Other - salty sweat, atrophy of vas deferens + epididymis (infertility)
168
What is the diagnosis for cystic fibrosis?
-Sweat test - Na+ and Cl- >60mmol/L in children -Fecal elastase negative/ low = normally elastase produced by pancreas + found in faeces; obstruction in CF may mean negative -FHx, genetic testing - F508 mutation
169
What is the treatment for CF?
Non curative : Conservative - chest phscion, no smoking Drugs - mucolytics, bronchodilators, pancreatic enzyme replacements
170
What is the treatment for CF?
Non curative : Conservative - chest phscion, no smoking Drugs - bronchodilators, pancreatic enzyme replacements, prophylactic antibiotics
171
What antibiotic is used for Staphylococcus aureus?
Flucloxacillin
172
what antibiotic is used to treat Haemophilus influenza?
Amoxicillin
173
What antibiotic is used to treat pseudomonas aeruginosa?
Ciprofloxacin
174
What is pharyngitis?
Inflammation of pharynx +/- exudate
175
What are the causes of pharyngitis?
Viral - EBV, adenoviruses Bacterial - group A haemolytic strep; streptococcus pyogenes
176
What is the presentation of pharyngitis?
sore throat + fever Viral = +cough + nasal congestion Bacterial = +exudate
177
What is sinusitis?
inflamed mucosa of nasal cavity and nasal sinuses
178
What are the causes of sinusitis?
mostly viral (10 days, non purulent discharge ) Sometimes bacterial >10 days, purulent -S.pneumonia (40%), H.influenza(30%)
179
What is Otitis media?
-inflamed middle ear, typically children -bacterial or viral
180
what is the diagnosis of otitis media?
otoscope shows inflamed erythematous tympanic membrane
181
What can be linked to Meningitis contagious spread?
Sinusitis and otitis media
182
What is epiglottitis?
Inflammation of epiglottis; obstructs airway. Mostly children <5
183
What is the most common cause of epiglottitis?
H. influenza
184
What is the presentation of epiglottitis?
Tripoding (leant forward, mouth open and tongue out=max air in), sore throat, SOB
185
What is whooping cough?
chronic cough caused by bordetella pertussis (gram neg bacillus) - mainly children 90% - <5 years old
186
What is the pathology of whooping cough?
Bordetella has a high virulence -Hemaggultuinin and fimbree adhere to urt -adenylate cyclase toxin inhibits phagocyte chemotaxis -pertussis toxin inhibits alveolar macrophages
187
What is the presentation of a whooping cough?
classic inspiratory whoop
188
What is croup/laryngobronchitis?
An occasional complication of URT infections, particularly those caused by parainfluenza and measles - most severe in children under 3
189
What is the presentation of croup/laryngobronchitis?
hoarse voice, barking cough, inspiratory stridor - high pitched wheeze when inhaling
190
What is the treatment for coup?
single dose dexamethasone
191
What is pleural effusion?
excess fluid accumulated between visceral and parietal pleural layers
192
What is transudative fluid?
Low protein - <25g/L Due to high hydrostatic pressure or low oncotic pressure - usually from liver cirrhosis, nephrotic syndrome - transparent fluid
193
What is exudative fluid?
High protein >35g/L Due to inflammation causing high vascular permeability e.g cancer, TB, pneumonia -cludy fluid
194
What is light criteria?
used to determine whether a pleural effusion is exudative or transudative -when protein level between 25-35
195
What is the presentation of pleural effusion?
Generic dyspnoea, pleuritic chest pain, cough, low breathing sounds - dull percussion(high fluid) on ipsilateral side
196
What is the diagnosis of pleural effusion?
-Gold standard - CXR - low costophrenic angles (blunting), excess fluid appears white +/- tracheal dilation -Thoracentesis (aspiration of pleural fluid) = sample pleural fluid - pH,Lactase,WCC,Microscopy, transudate or exudate
197
What is the treatment for pleural effusion?
Chest drain - if recurrent - pleurodesis Surgical fusing of pleural layers to prevent fluid building )
198
What is pneumothorax?
Excess air accumulation in pleural space, causing ipsilateral collapse
199
What is the pathology of pneumothorax?
-Can be primary - spontaneous or secondary to trauma pleural space normally a vacuum (and air) - breach in pleura (trauma)e.g subpleural bullae burst, abnormal connection (fistula) between pleural space and airways
200
What is the presentation of pneumothorax?
Typical presentation= tall thin males with connective tissue disorders (Marfans,50) +/- smokers, some kind of trauma
201
What are the symptoms of pneumothorax?
SOB, one sided sharp pleuritic chest pain, low breathing sounds - hyper resonant percussion ipsilaterally as air increase
202
What is the diagnosis of pneumothorax?
GS, 1st line - CXR - excess fluid appears black, tracheal deviation to other side
203
What is the treatment for pneumothorax?
-small self healing -Larger - needle decompression(suck out with syringe) - chest drain(more longer term treatment, one way air removal) -surgical - pleurodosis if recurrent
204
What is simple pneumothorax?
- Non medical emergency, little tracheal deviation -air can flow in and out of valve between alveoli and Plura therefore doesn't or is unlikely to worsen with every breath
205
What is tension pneumothorax?
-Medical emergency -air can flow into pleural space but can't leave therefore increase in intra pleural pressure with every breath( can even press on heart and cause cardiac Sx)
206
What is the treatment for tension pneumothorax?
Insert large bore cannula into 2nd intercostal space at midclavicular line - needle decompress first and then chest drain
207
What is pulmonary hypertension?
resting mean pulmonary arterial pressure >25mmHg measured with right heart catheterisation. Often results RHF; cor pulmonale
208
What is the pre capillary cause of pulmonary hypertension?
Pulmonary emboli, primary pulmonary HTN
209
What is the the post capillary cause of pulmonary hypertension?
LV failure
210
How does chronic hypoxia cause pulmonary hypertension?
COPD, altitude
211
What is the pathology of pulmonary hypertension?
Reactive pulmonary vasoconstriction when hypoxia ( all causes cause hypoxia due to low perfusion to tissues) --> increased pulmonary vascular resistance and pressure therefore endothelial damage --> RVH and failure
212
What is the presentation of pulmonary hypertension?
Initially - exertion dyspnoea and fatigue Then RHF signs : -increased JVP -peripheral oedema -Louder S2 than normal
213
What is the diagnosis of pulmonary hypertension?
CXR - RVH - enlarged proximal pulmonary artery ECG- RA dilation, peaked p waves Echo - RVH Diagnostic (GS) - right heart catheter (>25mmHg)
214
What is the treatment for pulmonary hypertension?
- phosphodiesterase 5 inhibitor (sildenafil) -CCB - amlodipine -Endothelin-1 antagonists =Diuretic for oedema

Phase 2a (9 decks)

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