Respiratory Flashcards
Give three bacteria which can colonise the nares.
- Staphylococcus epidermidis
- Corynebacteria
- Staphylococcus aureus
Give four potentially dangerous pathogens which can colonise the pharynx.
- Strep pneumoniae
- Haemophilus influenzae
- Strep pyogenes
- Neisseria
Give four defences that the respiratory tract has against pathogens.
- Commensal flora
- Swallowing
- Mucociliary escalator
- Cough/sneeze reflex
Give four conditions which may cause immune dysfunction of the lung.
- Primary immunodeficiency
- Complement deficiencies
- HIV
- Immunosuppressant therapy
Give four reasons why someone’s ability to swallow may be impaired, putting them at a higher risk of respiratory infection.
- Stroke
- Motor neurone disease
- Tumour
- Surgery
Give four intrinsic causes of altered lung physiology, putting the patient at increased risk of infection.
- Cystic fibrosis
- Bronchiectasis
- Emphysema
- Interstitial lung disease
Give four extrinsic causes of altered lung physiology, putting the patient at increased risk of infection.
- Spinal disease
- Weakness
- Obesity
- Surgery
What is the normal alveolar-arterial gradient?
<2kPa
Give three causes of a raised Alveolar-arterial gradient.
- V/Q mismatch
- Diffusion limitation
- Right to left cardiac shunt
Give two causes of a low PAO2.
- Hypoventilation
- Reduced FiO2 (or Patm)
What physiological response occurs to alveolar hypoxia?
Pulmonary vasoconstriction
In ambient hypoxia, what lung condition can result from widespread pulmonary vasoconstriction?
Pulmonary oedema
Where does gas exchange begin in the respiratory system?
Respiratory bronchioles
What breaks a breath hold?
Raised CO2 in the CSF (detected by central chemoreceptors)
What do peripheral chemoreceptors usually respond to?
Large changes in PaO2.
Give five functions of the lung.
- Gas exchange
- Acid-base balance
- Defence
- Hormones
- Heat exchange
Give five ways that the lung provides defence against pathogens.
- Mucosal barrier
- Mast cells
- Macrophages
- Mucociliary clearance
- Cough reflex
What is FEV1?
The forced expiratory volume in 1 second.
Describe the FEV1 and FVC in obstructive lung disease.
FEV1 low
FVC normal
Low FEV1:FVC ratio
Describe the FEV1 and FVC in restrictive airways disease.
Low FEV1
Low FVC
FEV1:FVC normal
A disease of the airways typically causes a _____________ (obstructive/restrictive) disease.
Obstructive
A disease of the lung parenchyma typically causes a _____________ (obstructive/restrictive) disease.
Restrictive
A disease of the chest wall/pleura typically causes a _____________ (obstructive/restrictive) disease.
Restrictive
What is TLCO?
Transfer factor (also known as diffusing capacity)
Give two causes of a low TLCO.
- Thickening of alveolar-capillary membrane
- Reduced lung volumes
Give two causes of a raised TLCO.
- Increased capillary blood volume
- Pulmonary haemorrhage
Give two causes of increased pulmonary capillary volume, which may lead to an increased TLCO.
- Polycythaemia
- Left to right cardiac shunt
Is lung cancer more common in males or females?
Males
What proportion of all cancer deaths does lung cancer account for?
One third
What is the five year survival rate in lung cancer?
<5-10%
Give three general risk factors for lung cancer.
- Cigarettes
- Occupational
- Lung fibrosis
Give six occupational risk factors for lung cancer.
- Asbestos
- Radon
- Nickel
- Chromate
- Arsenic
- Uranium
What are the symptoms of lung cancer?
- Cough
- Recurrent chest infections
- Haemoptysis
- Increasing shortness of breath
- General malaise
- Weight loss
- Chest pain
- Hoarseness
- Loss of voice
Give four general signs of lung cancer.
- Cachexia
- Anaemia
- Clubbing
- Supraclavicular or axillary nodes
Give three potential chest signs that may appear in lung cancer.
- Consolidation
- Collapse
- Pleural effusion
What percentage of patients have paraneoplastic changes in lung cancer?
3-10%
Give nine paraneoplastic changes that may occur in lung cancer.
- Secretion of PTH
- SIADH
- Secretion of ACTH and other hormones
- Hypertrophic pulmonary osteo-arthropathy (HPOA)
- Myasthenic syndrome (Lambert-Eaton)
- Finger clubbing
- Migratory thrombophlebitis
- Non-infective endocarditis (Libman Sacks)
- DIC
Describe the stage of lung cancer on presentation.
Most present with late stage
Is primary or metastatic lung cancer more common?
Metastatic
What is the most common classification of lung cancer?
Carcinoma (90%)
Give six general types of lung tumour.
- Carcinoma
- Bronchial gland neoplasm
- Pleural neoplasia
- Soft tissue sarcoma/benign tumour
- Lymphoma
- Hamartoma
Briefly describe the pathology of how lung cancer develops.
- Precursor cell changes lead to dysplasia, which leads to cancer
Give three samples that can be collected in lung cancer.
- Sputum
- Bronchoalveolar lavage (BAL)
- Pleural fluid
Give six investigations to carry out in lung cancer.
- Fluid sample collection (cytology/histology)
- Biopsy
- Lobectomy/wedge/pneumonectomy
- CXR
- Bronchoscopy
- Lung function tests
Give five types of primary lung carcinoma.
- Squamous cell carcinoma
- Adenocarcinoma (bronchioloalveolar adenocarcinoma)
- Large (non-small) cell undifferentiated carcinoma
- Small cell carcinoma
- Carcinoid tumour
Primary lung carcinomas can be broadly divided into what two groups?
- Small cell lung carcinoma (SCLC)
- Non-small cell lung carcinoma (NSCLC)
What grade is a small cell lung carcinoma?
High grade
What is the major risk factor for small cell lung carcinoma?
Cigarette smoking
What stage is the cancer usually at on presentation in small cell lung carcinoma?
Has usually spread by presentation
What is the standard treatment for small cell lung carcinoma?
Chemotherapy
What grade are non-small cell lung carcinomas usually?
Variable grade
What is a major risk factor for non-small cell lung carcinomas?
Cigarette smoking
Describe the stage at presentation of non-small cell lung carcinoma.
May have metastasised
Compare the rate of growth of small cell and non-small cell lung carcinomas.
Small cell carcinomas are faster
What is the usual treatment for non-small cell lung carcinomas?
Chemotherapy may be offered, but surgery and radiotherapy are the mainstay of treatment.
Can also use new drugs blocking specific tumour cell receptors.
What age group do carcinoid tumours of the lung more commonly occur in?
Younger people
Is smoking a major risk factor for carcinoid lung tumours?
No
Describe the prognosis for carcinoid lung tumours.
All are malignant, but they are less aggressive.
Give two types of lung lymphoma.
- Hodgkin’s lymphoma
- Non-Hodgkin’s lymphoma/BALTOMA
What is a BALTOMA?
Bronchus associated lymphoid tissue lymphoma
Give an example of a sarcoma which occurs in the lung.
Epitheloid haemangio-endothelioma
What does PL1/PL2 indicate when staging lung tumours?
Pleural involvement
What does M0 mean when staging lung tumours?
No metastases
What does M1a mean when staging lung tumours?
Contralateral lung, or pleural/pericardial effusion/nodule
What does M1b mean when staging lung tumours?
Distant spread outside chest
Give three examples of non-malignant lung nodules.
- TB and other infections
- Lymph nodes
- Benign neoplasia (eg. Hamartoma)
What is a hamartoma?
Disorganised benign tumour of various tissue types.
Give three types of pleural neoplasia.
- Metastatic disease
- Pleural fibroma
- Malignant mesothelioma
Describe a pleural fibroma.
- Solitary fibrous tumour
- Most are benign
What patient demographic are malignant mesotheliomas more common in?
Males >60yrs
What is a major risk factor for malignant mesothelioma?
Asbestos exposure
What is the average survival time in a malignant mesothelioma?
8-12months
Describe the treatment for malignant mesothelioma.
Limited benefit from surgery, chemotherapy, radiotherapy
Describe the microscopic appearance of a malignant mesothelioma.
Many different microscopic appearances
Give six consequences of asbestos exposure.
- Plaques
- Persistent pleural effusion
- Pleural fibrosis
- Lung cancer
- Asbestosis (diffuse interstitial fibrosis)
- Mesothelioma
Give ten differential diagnoses of a lung nodule.
- Primary or metastatic malignancy
- Abscesses
- Granuloma
- Carcinoid tumour
- Pulmonary hamartoma
- Arterio-venous malformation
- Encysted effusion (fluid, blood, pus)
- Cyst
- Foreign body
- Skin tumour
What are ‘interstitial lung diseases’?
A general term for diseases resulting in an increased amount of connective tissue between the alveolar spaces in the lungs.
In lung fibrosis, do patients have trouble breathing in or out?
In
Are interstitial lung diseases generally obstructive or restrictive?
Restrictive
Give three general consequences of interstitial lung diseases.
- Increased stiffness
- Decreased compliance
- Loss of alveolar surface
Describe the Tco expected in interstitial lung diseases.
Reduced
Describe the VC expected in interstitial lung diseases.
Reduced
Describe the FEV1 expected in interstitial lung diseases.
Reduced
Describe the FEV1/FVC ratio expected in interstitial lung diseases.
Normal
Describe the peak expiratory flow rate expected in interstitial lung diseases.
Normal
Give four general clinical features of interstitial lung diseases.
- Dyspnoea on exertion
- Non-productive paroxysmal cough
- Abnormal breath sounds
- Abnormal CXR
Give five examples of acute interstitial lung diseases.
- Adult respiratory distress syndrome
- Drug and toxin reactions
- Gastric aspiration
- Radiation pneumonitis
- Diffuse intrapulmonary haemorrhage
Give five causes of adult respiratory distress syndrome.
- Shock
- Trauma (direct pulmonary or multisystem)
- Infections
- Gas inhalation
- Narcotic abuse
Briefly describe the pathology in adult respiratory distress syndrome.
- Polymorphs release enzymes and activate complement
- Increased capillary permeability leads to pulmonary oedema
- Diffuse alveolar damage with hyaline membranes
What is the fatality rate in adult respiratory distress syndrome?
50%
What are the clinical features of adult respiratory distress syndrome?
- Cyanosis
- Tachypnoea
- Dyspnoea
- Tachycardia
- Pulmonary oedema
- Arterial hypoxaemia
- Peripheral vasodilation
What would a CXR show in adult respiratory distress syndrome?
Bilateral pulmonary infiltrates
Give two cytotoxic drugs that can cause an acute interstitial lung disease.
- Busulphan
- Bleomycin
How does paraquat (a potent herbicide) cause an acute interstitial lung disease?
- Releases hydrogen peroxide and superoxide free radical
- Remains in high concentrations in the lungs after ingestion
Give two potential consequences of radiation pneumonitis.
- Respiratory distress syndrome
- Progressive pulmonary fibrosis
Give five examples of chronic interstitial lung diseases.
- Fibrosing alveolitis
- Pneumoconioses
- Sarciodosis
- Diffuse malignancies
- Rheumatoid diseases
What is another name for fibrosing alveolitis?
Idiopathic pulmonary fibrosis
Give two pathological processes that occur in idiopathic pulmonary fibrosis.
- Inflammatory cell infiltrate
- Pulmonary fibrosis
Describe the macroscopic appearance of the lungs in idiopathic pulmonary fibrosis.
- Abnormally large irregular spaces separated by thick fibrous septa
- End stage fibrosis = honeycomb lung
Which areas of the lung are predominantly affected by idiopathic pulmonary fibrosis?
Subpleural regions
What pattern will the pulmonary function tests show in idiopathic pulmonary fibrosis?
Restrictive
Give the symptoms of idiopathic pulmonary fibrosis.
- Dry cough
- Exertional dyspnoea
- Malaise
- Weight loss
- Arthralgia
Give three signs of idiopathic pulmonary fibrosis.
- Cyanosis
- Finger clubbing
- Fine end-inspiratory crepitations
What would be ABG show in idiopathic pulmonary fibrosis?
Low PaO2
What would be CRP be in idiopathic pulmonary fibrosis?
High
What would the immunoglobulin levels be in idiopathic pulmonary fibrosis?
High
What would the CXR show in idiopathic pulmonary fibrosis?
- Reduced lung volume
- Honeycomb lung
Give three potential complications in idiopathic pulmonary fibrosis.
- Cor pulmonale
- Respiratory failure
- Increased risk of lung cancer
What is the 5 year survival rate in idiopathic pulmonary fibrosis?
50%
What is pneumoconiosis?
Lung disease caused by inhaled dust (can be organic or inorganic).
Give four different types of reaction that can occur in pneumoconiosis.
- Inert
- Fibrous
- Allergic
- Neoplastic
Give an example of an inert reaction to pneumoconiosis.
Coal worker’s pneumoconiosis
Give three examples of fibrous reactions to pneumoconiosis.
- Progressive massive fibrosis
- Asbestosis
- Silicosis
Give an example of an allergic reaction to pneumoconiosis.
Extrinsic allergic alveolitis
Give two examples of neoplastic reactions to pneumoconiosis.
- Mesothelioma
- Lung cancer
Describe the pathology in coal worker’s pneumoconiosis.
- Coal ingested by alveolar macrophages (dust cells)
- Macrophages aggregate around bronchioles
What is anthracosis?
Presence of coal dust pigment in the lung
Describe macular coal worker’s pneumoconiosis.
Aggregates of dust laden macrophages with no significant scarring.
Describe nodular coal worker’s pneumoconiosis.
Nodules >10mm in a background of extensive macular CWP, with no significant scarring.
Give three consequences that can result from coal worker’s pneumoconiosis.
- Progressive massive fibrosis
- Emphysema
- Honeycomb lung and/or cor pulmonale
Where are silicates found?
Stone and sand
Briefly describe the pathology of silicosis.
- Silicates are toxic to macrophages
- Macrophages die and release proteolytic enzymes
- This leads to tissue destruction and fibrosis
- Nodules formed after many years of exposure
Give a disease that has a higher incidence in people with silicosis.
TB
What is asbestos?
An inconsumable silicate
What type of hypersensitivity reaction is extrinsic allergic alveolitis?
Type 3
Describe the basic pathology in extrinsic allergic alveolitis.
- Hypersensitivity leads to bronchiolitis
- Later leads to chronic inflammation and granulomas
Give the possible outcomes of extrinsic allergic alveolitis.
- Resolution
- Fibrosis
What is sarcoidosis?
Deposits of immune complexes resulting in a granulomatous disease mainly affecting the lungs and lymph nodes.
What type of hypersensitivity reaction is sarcoidosis?
Type IV
Give four symptoms of sarcoidosis.
- Dry cough
- Progressive dyspnoea
- Decreased exercise tolerance
- Chest pain
Describe Kveim test.
- Subcutaneous injection of sterile homogenised sarcoid tissue
- Induces granulomas in patients with sarcoidosis
What investigation should be used to assess sarcoidosis?
CXR
Give a potential treatment for sarcoidosis?
Corticosteroids
Give three potential affects of Rheumatoid disease on the lungs.
- Diffuse fibrosis
- Rheumatoid nodules
- Caplan’s syndrome
What is Caplan’s syndrome?
Rheumatoid arthritis + pneumoconiosis
What will the pleura show in rheumatoid disease affecting the lungs?
Fibrosis and lymphocytic aggregates
Give two diffuse malignancies that may cause a chronic interstitial lung disease.
- Lymphangitis
- Bronchioloalveolar carcinoma
Describe lymphangitis and how it affects the lungs.
Tumour spreads through lung and obstructs lymphatics
How does brochioloalveolar carcinoma cause an interstitial lung disease?
Spreads on the surface of the lung to cause local scarring.
Is asthma an obstructive or restrictive disease?
Obstructive
Give seven pathological mechanisms that occur in asthma.
- Increased irritability of the bronchi causing spasm
- Bronchial obstruction with distal overinflation or atelectasis (lung collapse)
- Overdistended lungs
- Enlarged bronchial mucous glands with excess secretions (mucous plugs in bronchi)
- Bronchial inflammation
- Bronchial wall smooth muscle hypertrophy
- Thickening of bronchial basement membrane
Give three types of extrinsic asthma.
- Atopic
- Occupational
- Allergic bronchopulmonary aspergillosis
Briefly describe the pathology in extrinsic asthma.
Environmental agents lead to IgE cross linking and mast cell degranulation.
What type of reaction occurs in extrinsic asthma?
IgE/type 1 hypersensitivity
What type of reaction occurs in occupational asthma?
Type 3 hypersensitivity
Give seven causes of intrinsic asthma.
- Aspirin
- Cold
- Infection
- Stress
- Exercise
- SO2
- Pollutants
Give two other conditions which are associated with intrinsic asthma.
- Recurrent chest infections
- Chronic bronchitis
Give three other things associated with aspirin-induced asthma.
- Recurrent rhinitis
- Nasal polyps
- Skin urticaria
What organism causes allergic bronchopulmonary aspergillosis?
Aspergillus fumigatus
What type of reaction occurs in allergic bronchopulmonary aspergillosis?
Type I and type III hypersensitivity reaction
Give the two categories that eosinophilic asthma can be divided into.
- Atopic
- Non-atopic
Give three causes of atopic asthma.
- Fungal allergy
- Common aeroallergens
- Occupation/pets/exposures
Give three types of non-eosinophilic asthma.
- Non-smoking, non-eosinophilic
- Smoking-associated
- Obesity-related
What are the symptoms of asthma?
- Cough
- Shortness of breath
- Episodic wheezing
- Chest tightness
- Secretions
Give two hallmark clinical features of asthma.
- Diurnal variation
- Reversibility
Is asthma typically worse in the morning or evening?
Morning
What is type 1 brittle asthma?
Chronic severe (bad all the time)
What is type 2 brittle asthma?
Sudden dips
Give three features of the wheeze commonly heard in asthma.
- Polyphonic
- Expiratory
- Widespread
Give two features you WOULDN’T expect to find on a clinical examination in a patient with asthma.
- Crackles
- Sputum
Give six factors with may provoke asthma.
- Allergens
- Infections
- Menstrual cycle
- Exercise
- Cold air
- Laughter/emotion
Give three simple tests to carry out in asthma.
- Blood count (eosinophils)
- Tests for atopy and allergy (Skin prick and IgE)
- CXR
Give four findings on pulmonary function tests in asthma.
- Airways obstruction
- Reduced FEV1
- Reduced FEV1/FVC ratio
- Reduction in PEFR (diurnal variation)
How can challenge agents be used to assess asthma?
There is increased responsiveness to challenge agents (eg. Mannitol, methacholine)
How can reversibility testing help to diagnose asthma?
- 12% increase in FEV1 (with increase in 200ml in volume) after bronchodilator is positive
- 400ml+ increase makes asthma highly likely
How can an exhaled NO test be used to assess asthma?
- NO given off by inflamed lungs
- Marker of eosinophilic inflammation
- This is non-specific
Give a factor that may suppress NO production by the lungs in asthma.
Smoking
Give two factors that may increase NO production by the lungs in asthma.
- Viral infections
- Rhinitis
What two factors should be considered when assessing the severity and control of asthma?
- Day to day control
- Exacerbation
What three criteria, named RCP3, are used to assess day to day control of asthma?
- Recent nocturnal waking?
- Usual asthma symptoms in day?
- Interference with ADLs?
What four things are taken into account when assessing the severity of asthma using exacerbations?
- A and E attendances
- GP
- Admissions
- ITU
What are the three elements of Samter’s triad?
- Bronchial asthma
- Nasal polyps
- Aspirin intolerance
Compare the age of onset in asthma and COPD.
ASTHMA
- Usually earlier onset
COPD
- Usually onset later in life
Compare the association with smoking in asthma and COPD.
ASTHMA
- Not associated with smoking
COPD
- Usually seen in smokers
Compare the disease progression in asthma and COPD.
ASTHMA
- Relatively stable disease
COPD
- Progressive and worsens over time
Compare the diurnal variation in asthma and COPD.
ASTHMA
- Diurnal variation
COPD
- Less diurnal variation
Compare the day-to-day variation in asthma and COPD.
ASTHMA
- Day to day variation
COPD
- Less day to day variation
Compare the seasonal changes in asthma and COPD.
ASTHMA
- Not really seasonal
COPD
- Worse in winter
Compare the sputum production in asthma and COPD.
ASTHMA
- Less sputum production
COPD
- Sputum production
What is ACOS?
Asthma COPD overlap syndrome
Give five risk factors for asthma death.
- > 3 classes of treatment
- Recent admission/frequent attender
- Previous near-fatal disease
- Brittle disease
- Psychosocial factors
Give four general methods of treating asthma.
- Avoidance of triggers
- Bronchodilators
- Anti-inflammatory drugs
- New biological drugs
How do bronchodilators work to treat asthma?
Relieve symptoms
How do anti-inflammatory drugs work to treat asthma?
Prevent exacerbations
Give four categories of bronchodilators which are used in asthma.
- Beta agonists
- Leukotriene receptor antagonists
- Theophyllines
- Long acting beta agonists
- Anticholinergics
Give a beta agonist used to treat asthma.
Salbutamol
Give two leukotriene receptor antagonists used to treat asthma.
- Montelukast
- Zafirlukast
Give a theophylline used to treat asthma.
Aminophylline
Give two long acting beta agonists used to treat asthma.
- Salmeterol
- Formoterol
Give two anticholinergics used to treat asthma.
- Ipratropium
- Tiotropium
What class of anti-inflammatory drugs are used to treat asthma?
Steroids
Give an example of a steroid used to treat asthma.
Beclometasone
Give two examples (and how they work) of new biological drugs used to treat asthma.
- Omalizumab (Anti-IgE)
- Mepolizumab (Anti-IL5)
Give the three first steps (in order) in managing acute asthma.
- High flow oxygen
- Emergency beta agonists (nebuliser)
- Brief history/examination
Describe the PEFR, RR, pulse, and speech in moderate acute asthma.
- PEFR = >50%
- RR <25
- Pulse <110
- Normal speech
Describe the PEFR, RR, pulse, and speech in severe acute asthma.
How many criteria need to be present?
- PEFR 33%-50%
- RR >25
- Pulse >110
- Inability to complete sentences
*Only one has to be present
Describe the PEFR, O2 sats/PaO2, and PaCO2 in life-threatening acute asthma.
- PEFR <33%
- O2 <92%
- PaO2 <8kPa
- Normal PaCO2
What would you expect the PaCO2 to be in acute asthma?
Low due to hyperventilation.
*If normal or high this is a bad sign
Give seven indications, other than vital signs, of life-threatening acute asthma.
- Altered consciousness level
- Exhaustion
- Arrhythmia
- Hypotension
- Silent chest
- Poor effort
- Cyanosis
Give two organs that are affected in Goodpasture’s syndrome.
- Lungs
- Kidney
What is the clinical presentation of Goodpasture’s syndrome in the kidney?
Acute glomerulonephritis
Give two effects of Goodpasture’s syndrome on the lung?
- Haemoptysis
- Diffuse pulmonary haemorrhage
What would be seen on a CXR in Goodpasture’s syndrome?
Infiltrates due to pulmonary haemorrhage (often in lower zones)
What would be seen on a kidney biopsy in Goodpasture’s syndrome?
Crescentic glomerulonephritis
How is Goodpasture’s syndrome treated?
- Vigorous immunosuppression
- Plasmapheresis
What is the cut off for pulmonary hypertension at rest?
> 25mmHg
What is the cut off for pulmonary hypertension during exercise?
> 30mmHg
Describe primary pulmonary hypertension.
Rare, occurs in the absence of chronic lung or heart disease.
Describe secondary pulmonary hypertension.
Quite common, develops as a complication of lung or heart disease.
Give five causes of primary pulmonary hypertension.
- Idiopathic
- Drugs
- HIV
- Collagen vascular disease
- Congenital systemic-to-pulmonary shunts
Give four common causes of secondary pulmonary hypertension.
- COPD
- Interstitial lung disease
- Left ventricular failure
- Chronic pulmonary thromboemboli
Describe the clinical presentation of primary pulmonary hypertension.
- Exertional dyspnoea
- Fatigue
- Dizziness
- Syncope
Describe the clinical presentation of secondary pulmonary hypertension.
- Worsening of symptoms of pre-existing condition
- Increasing breathlessness
What is the five year survival rate in primary pulmonary hypertension?
25-50%
Describe the prognosis in secondary pulmonary hypertension.
Generally implies significant underlying cardiac or lung disease with poor prognosis.
Give two investigations to carry out in pulmonary hypertension.
- Echocardiogram
- Right heart catheterisation
What is cystic fibrosis?
An inherited disorder caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Describe the inheritance pattern in cystic fibrosis.
Autosomal recessive
What is allelic heterogeneity, in terms of cystic fibrosis?
Many different mutations in the same gene can cause the disease.
What does the CFTR gene code for?
A chloride channel
Describe the pathology of cystic fibrosis.
Lack of normal CFTR causes defective electrolyte transfer across epithelial cell membranes, resulting in thick mucous secretions.
What is the most common form of presentation of cystic fibrosis?
Pulmonary disease
At what stage in cystic fibrosis does liver disease develop?
Late
Describe a neonatal presentation of cystic fibrosis.
- Failure to thrive
- Meconium ileus (bowel obstruction)
- Rectal prolapse
Describe how a child/young adult might present with cystic fibrosis.
- Cough
- Wheeze
- Recurrent infections
- Bronchiectasis
- Pneumothorax
- Haemoptysis
- Respiratory failure
- Cor pulmonale
- Pancreatic insufficiency
- Distal intestinal obstruction syndrome
- Gallstones
- Cirrhosis
- Male infertility
- Osteoporosis
- Arthritis
- Vasculitis
- Nasal polyps
- Sinusitis
- Hypertrophic pulmonary osteoarthropathy (HPOA)
Give three signs of cystic fibrosis.
- Cyanosis
- Finger clubbing
- Bilateral course crackles
What organisms commonly cause respiratory infections in cystic fibrosis patients?
- Initially, infection is caused by common bacteria which colonise the lungs
- Eventually Pseudomonas aeruginosa becomes the dominant organism
Give two macroscopic changes that may occur in cystic fibrosis.
- Widespread bronchiectasis
- Liver may be fatty/cirrhotic
Give four microscopic changes that may be seen in cystic fibrosis.
- Lungs contain thick mucous
- Acute inflammation may be seen if there is active infection in the lungs
- Periportal fibrosis in liver, may lead to cirrhosis
- Thickened bile in intrahepatic bile ducts
Give three diagnostic tests for cystic fibrosis.
- Sweat test
- Genetics
- Faecal elastase
What sweat test results would suggest cystic fibrosis?
- Sodium and chloride >60mmol/L
- Chloride usually greater than sodium
How can the faecal elastase test be used to assess cystic fibrosis?
Screening test for exocrine pancreatic dysfunction (low measurement suggests insufficiency)
What blood tests would it be useful to carry out in cystic fibrosis?
- FBC
- U and E
- LFT
- Clotting
- Vitamins A/D/E
How could the presence of an infection be detected in cystic fibrosis?
Cough swab or sputum culture
How can diabetes be picked up in cystic fibrosis patients?
Annual glucose tolerance test
Give two potential features of a CXR in cystic fibrosis.
- Hyperinflation
- Bronchiectasis
Give three potential USS findings in cystic fibrosis.
- Fatty liver
- Cirrhosis
- Chronic pancreatitis
What pattern might the spirometry results show in cystic fibrosis?
Obstructive
As well as faecal elastase, what other faecal test might be useful in cystic fibrosis?
Faecal fat analysis
Give four management principles for the chest in cystic fibrosis.
- Physiotherapy
- Antibiotics for acute infections and prophylactically
- Mucolytics
- Bronchodilators
Give four management principles for the GI system in cystic fibrosis.
- Pancreatic enzyme replacement
- Fat soluble vitamin supplements (A, D, E, K)
- Ursodeoxycholic acid for impaired liver function
- Cirrhosis may require liver transplant
Give four management principles for advanced lung disease in cystic fibrosis.
- Oxygen
- Diuretics (for Cor pulmonale)
- Non-invasive ventilation
- Lung or heart/lung transplant
What is the average life expectancy for a patient with cystic fibrosis?
35yrs
What is the most common cause of death in cystic fibrosis?
Pulmonary disease
What is a pleural effusion?
An accumulation of excess fluid within the pleural space.
What is the protein content of a transudate pleural effusion?
<25g/L
What is the protein content of an exudate pleural effusion?
> 35g/L
What is a haemothorax?
Blood in the pleural space
What is an empyema?
Pus in the pleural space
What is a chylothorax?
Chyle (lymph with fat) in the pleural space
What is the term for blood and air in the pleural space?
Haemopneumothorax
Give four general causes/mechanisms of transudate pleural effusion.
- Increased venous pressure
- Hypoproteinaemia
- Hypothyroidism
- Meigs’ syndrome
Give three causes of transudate pleural effusion due to increased venous pressure.
- Left ventricular failure
- Constrictive pericarditis
- Fluid overload
Give three causes of transudate pleural effusion due to hypoproteinaemia.
- Cirrhosis
- Nephrotic syndrome
- Malabsorption
Describe the mechanism which causes an exudate pleural effusion.
Increased leakiness of pleural capillaries.
Give six causes of exudate pleural effusion.
- Pneumonia
- TB
- Pulmonary infarction
- Pulmonary embolism
- Malignancy
- Multisystem autoimmune diseases (SLE, RA)
What are the symptoms of a small pulmonary effusion.
May be asymptomatic
Give two symptoms of a large pleural effusion.
- Breathlessness
- Pleuritic chest pain
Give seven signs of a pleural effusion.
- Decreased expansion
- Stony dull percussion note
- Diminished breath sounds
- Decreased vocal resonance
- Bronchial breathing above effusion
- Tracheal deviation (away from effusion) if large effusion
- Signs of underlying disease
Give three signs of a pleural effusion that may be seen on a CXR.
- Blunting of costophrenic angles
- Water-dense shadows with concave upper borders
- Completely flat horizontal upper borders implies that there is also a pneumothorax
When is an ultrasound useful in pleural effusion?
To identify presence of pleural fluid and to guide aspiration.
Give four tests that can be carried out on a diagnostic pleural aspiration.
- Clinical chemistry
- Bacteriology
- Cytology
- Immunology
Give four management options for a pleural effusion.
- Treat underlying cause
- Drainage
- Pleurodesis
- Surgery
When is drainage of a pleural effusion carried out?
- If symptomatic
- Not in TB
How fast should the fluid be removed when draining a pleural effusion?
Slowly
What is pleurodesis?
Adhesion of the pleural layers (obliterating the pleural space).
Give three substances that can be used for Pleurodesis.
- Tetracycline
- Bleomycin
- Talc
What is the prognosis for a parapeumonic/pulmonary embolus effusion?
Usually resolve upon treatment
What is the prognosis for a pleural effusion due to left ventricular failure?
Usually implies advanced disease and poor prognosis.
Describe the prognosis in a pleural effusion due to malignancy.
Due to metastatic disease so has very poor prognosis.
Give two causes of a turbid, yellow pleural effusion.
- Empyema
- Parapneumonic effusion
Give three causes of a haemorrhagic pleural effusion.
- Trauma
- Malignancy
- Pulmonary infarction
Give two causes of a pleural effusion with increased neutrophils.
- Parapneumonic effusion
- Pulmonary embolism
Give five causes of a pleural effusion with increased lymphocytes.
- Malignancy
- TB
- Rheumatoid arthritis
- Lupus
- Sarcoidosis
Give a cause of pleural effusion with many mesothelial cells.
Pulmonary infarction
Give a cause of a pleural effusion containing abnormal mesothelial cells.
Mesothelioma
Give a cause of a pleural effusion containing multinucleated giant cells.
Rheumatoid arthritis
Give a cause of pleural effusion which contains lupus erythematosus cells.
Lupus
Give five causes of a pleural effusion with glucose <3.3mmol/L.
- Empyema
- Malignancy
- TB
- Rheumatoid arthritis
- SLE
Give five causes of a pleural effusion with pH <7.2.
- Empyema
- Malignancy
- TB
- Rheumatoid arthritis
- SLE
Give five causes of a pleural effusion with raised LDH.
- Empyema
- Malignancy
- TB
- Rheumatoid arthritis
- SLE
Give four causes of a pleural effusion with raised amylase.
- Pancreatitis
- Carcinoma
- Bacterial pneumonia
- Oesophageal rupture
Give four causes of a pleural effusion with low complement levels.
- Rheumatoid arthritis
- SLE
- Malignancy
- Infection
Give two pulmonary diseases in which Pseudomonas aeruginosa is a likely cause of infection.
- Cystic fibrosis
- Bronchiectasis
What is the term for when Streptococcus pneumoniae gets into the blood?
Invasive pneumococcal pneumona
Describe the shape of Haemophilus.
Coccobacilli (short rod)
What are the X, V, and XV disks sometimes used on agar plates?
Growth factors - can see which ones the organism needs to grow.
Give an alternative stain to the Zeil Neelsen stain that can be used to identify Mycobacteria.
Auramine-phenol stain
Give two cultures on which Mycobacteria can be cultured.
- Lowenstein Jensen slope
- Mycobacterial growth indicator tube (MGIT)