clinical symposia - respiratory strand Flashcards
What are symptoms of asthma?
Wheeze, shortness of breath, chest tightness, cough (particularly at night or in early morning), difficulty sleeping, chest pain, vomiting, tremor/hoarse voice
What structures are affected by asthma? How?
- inflammation of the airway
- hyper-reactive smooth muscle
- increased basal tone (increased contraction of vessels)
- mucus hypersecretion
- mucosal oedema (build up of tissue fluid in the mucosa)
Is asthma a restrictive or obstructive pulmonary disease?
Obstructive
The lungs are unable to expel air effectively during exhalation
What would you predict the FEV1/FVC ratio to be?
Reduced
As with all obstructive lung disease
Is there likely to be any significant airway reversibility?
Yes
Although the airway narrowing is recurring, treatment can reverse this.
How is asthma monitored?
Spirometry
Used to measure narrowing of the airways before and after medication.
What signs of asthma may be elicited?
- FEV1/FVC ratio (reduced, measured through spirometry)
- FeNO test (measures levels of NO in breath showing lung inflammation)
- Reduced peak flow compared to other individuals of the same age, sex and height
Why may someone with asthma present with a hoarse voice or a tremor?
- overuse of beta agonists
- overuse of steroids
(Beta agonists can cause a tremor and steroids can cause oral candida, especially if technique is poor)
What is a feature of life-threatening asthma?
Paradoxical pulse
this is a large decrease in systolic pressure and pulse wave amplitude during inspiration
What is immediate treatment for an asthma attack?
- oxygen - aim for saturation of 94-98%
- salbutamol 5mg via an oxygen driven nebuliser
- ipratropium bromide 0.5mg
- prednisolone tablets 40-50mg or IV hydrocortisone
- no sedatives
- chest x-ray
What triggers asthma?
- tobacco smoke
- allergens such a dust mites and pets
- pests
- mould
How can asthma be managed?
Treatment such as relievers or preventers
What are examples of reliever treatment for asthma?
Beta-2 adrenoceptor agonists such as salbutamol
- stimulation of airway B2 adrenoreceptors results in relaxation of bronchiole smooth muscle
Antimuscarinics such as ipratropium bromide
- inhibit muscrarinic receptors on smooth muscle causing relaxation of bronchiole smooth muscle
What is an example of a preventer treatment for asthma?
Glucocorticoids
- bind to cytosolic receptors and affect gene transaction and transcription
- production of potent anti-inflammatory agents and reduce airway hyper-resposonsiveness
Describe the difference between asthma and COPD
- nearly all COPD patients smoke
- symptoms under 35 more common in asthma
- chronic productive cough more common in COPD
- breathlessness is persistent and progressive in COPD
- variability is more common in asthma
What are the two types of asthma?
- extrinsic (childhood, family history, episodic, positive skin conditions)
- intrinsic (adulthood, negative skin test, no clear precipitating factors)
What are risk factors of asthma?
- genes
- maternal smoking
- pollution
- obesity
What reduces asthma risk?
- breast feeding
- early exposure to animals
what kind of pulmonary disease is COPD
obstructive pulmonary disease
is there any reversibility in COPD
no
is COPD of slow or fast progression?
slowly progressive
significant airflow obstruction may be present before person aware
how does COPD affect the airways
- chronic inflammation
- large and small airway narrowing
what are 2 the symptoms of COPD
- abnormal mucous (due to irritation/ infection)
- cough (smokers cough)
- breathlessness (dyspnoea)
- sputum production (green)
- wheeze
what is there main risk factor for COPD?
smoking
what is the main disease also associated with COPD
emphysema
how does emphysema effect the lungs?
- abnormal, permanent enlargement of airspaces distal to the terminal bronchiole
- accompanied by destruction of their walls
what is the main symptoms of emphysema and why is this
significant breathlessness due to airway collapse in exhalation
what are the risk factors for emphysema and COPD?
- smoking
- pollution
- family history (Alpha1-antitrypsin deficiency )
what is alpha1-antitrypsin?
glycoprotein produced in liver
protease inhibitor which balances out the action of neutrophil elastase
(some neutrophil elastase needed to clear out debris in alveoli , not too much)
what is alpha1-antitrypsin defiancey?
autosomal recessive deficiency in chromsome 14
co-dominant alleles
what can 2 things other than alpah1-antitrypsin effect the amount of neutrophil elastase?
smoking
infection
why is abnormal amounts of neutrophil elastase bad?
can get trapped in the lungs and cause liver disease
What is bronchiectasis?
A disease characterised by a structural abnormality of irreversibly dilated bronchi and large amounts of sputum
What are the types of bronchiectasis?
- cylindrical bronchiectasis (airways don’t taper. They are dilated right out to the periphery)
- varicose bronchiectasis (tubes get smaller and larger like veins in varicose veins
- cystic bronchiectasis (fluid in bronchioles)
What would you see on the chest x-ray of an individual with bronchiectasis?
- dilated airways
- signet ring sign showing that the bronchus is larger than the pulmonary artery
What conditions are associated with bronchiectasis?
- cystic fibrosis
- rheumatoid arthritis
- immunodeficiency
- childhood infections such as pneumonia, TB and measles
- primary ciliary dyskinesia
What are 6 causes of bronchiectasis?
- Post infection (measles, whooping cough, TB, pneumonia)
- Immune deficiency
- Allergic bronchopulmonary hypogammagobulinemia ( increased levels of a certain immunoglobulin causing inflammation)
- Defective clearance eg in CF or PCK
- Aspirations of toxic substances
- Mechanical eg tumour (distal bronchiectasis), lymph node (middle lymph node syndrome) or foreign body
What are symptoms of bronchiectasis?
- persistent purulent sputum (purulent = contains puss)
- haemoptysis
- breathlessness
- wheeze
- sinusitis and nasal symptoms
- weight loss
- pleurisy (inflammation of the pleura)
- nasal polyps
What are signs of bronchiectasis?
- clubbing
- coarse crackles
- wheeze
Some people display no signs and bronchiectasis can only be seen on a CT scan
What is clubbing?
Tissue at the base of the nail is thickened so the normal angle between the nail base and adjacent ski is obliterated
What are signs of clubbing?
- fluctuation and softening of the nail bed (boggy)
- loss of angle between the nail bed and fold
- increased convexity of the nail fold
What conditions are associated with clubbing?
- bronchiectasis
- lung cancer
- cystic fibrosis
What investigations can be carried out into bronchiectasis?
- history
- genetics
- ciliary beat frequency/ nasal NO
- sperm analysis
- sputum (MC&AFB, fungal)
- autoantibody screen
- aspergillosis antibodies
- immunological review
What are treatments for bronchiectasis?
- physiotherapy/ airway clearance
- mucolytics/ hypertonic saline
- prompt oral antibiotics
- IV antibiotics as required
- nebulised antibiotics
- annual influenza vaccine
- immunoglobulin replacement
what kind of disease inheritance is CF?
autosomal recessive
what was the first mutation discovered for the CFTR protein?
mutation in DF508
what does mutation in DF508 cause?
- deletion of 3 nucleotides spanning positions 507-508 of CFTR gene
- = loss ignite codon for AA Phe
why is the class of mutation in the CFTR protein key?
dictate the therapy undertaken
where are CFTR proteins expressed?
airway epithelial cells
on what chromosome is the CFTR gene?
chromosome 7
what kind of protein is the does the CFTR gene code for and what’s its function?
ion transport channel
regulate chloride bicarbonate and Na transport
what are the effects of the DF508 mutation on the CFTR protein?
- protein produced is abnormal
- broken down in airway epithelium
- no CFTR expression
what does the breakdown of the CFTR protein lead to?
cant secrete chloride and bicarbonate –> lose inhibitory effect of Na transport –> increased Na absorption –> increased water absorption –> dehydration of airway surface liquid
what is mucocillary clearance?
protective mechanism that protects the lung from invade particles
particles stick to mucous, propelled to back f throat and swallowed
what is ciliopathy?
where cilia don’t function properly
what does airway surface dehydration cause?
-mucocillary disfunction - abnormal mucous Clearence
apart from mucocillary disfunction what else can cause abnormal mucous clearance?
abnormal mucous and production of DNA from degenerating neutrophils = make secretions very sticky - accumulate in airways
what causes mucocillary disfunction ?
- predisposition to infection
- CF also associated with increased inflammatory response which itself causes lung damage
is the CFTR gene found all around the body? if so, where?
yes pancreas liver digestive tract reproductive tract skin nasal cavity lungs
why is CF considered a multi system disease?
protein found all around the body
can cause:
liver disease, artists, pancreatic insufficiency, male infertility ect
how does airway dehydration effect the airway liquid and what is the significance of this?
abnormal pH
leads to abnormalities in defensive protein used in infection
what are the consequences of airway dehydration?
airway inflammation airway infection viscous secretions lung destruction bronchiectasis
what is the main test we use to diagnose CF
sweat test
-Chloride >60mmol/l (milder cases 30-60
why do CF victims have a higher salt content in their sweat and how can we recogniser this ?
- Defective Na/Cl absorption means high sweat salt content
high amount of Na and Cl as duct impeccable to water absorption
white crystals form when they sweat
what investigations can we do for CF
- genetics- dictate treatment
- pancreatic function(pancreatic malabsorption, test for faecal elastase, low levels = CF)
- sperm (obstructive azoospermia)
- CT scan for bronchiectasis
what differnt therapies do we use for CF?
- nebuliser antibiotics
- oral/ IV antibiotics
- DNase
- hypertonic saline
- pancreatic enzymes
- insulin ( endocrine disfunction of pancreas too)
- CFTR modulators
- Ursodeocycholic acid – reduce liver disease
what do nebuliser antibiotics do?
fine mist inhaled- eradicate bugs that come up and treat chronic infection such as pseudomonas
what are the symptoms of CF?
- Pulmonary exacerbation
- Increased cough
- Increased sputum production
- Change in sputum colour
- Fever / malaise
why may we get a collapsed upper lobe in CF?
due to thick secretions
what are the signs of CF
- Clubbing
- Coarse crackles
- Wheeze
- Lost weight - infections and pancreatic malabsorption
- 30% adult have CF diabetes
- Vision due to vitamin A deficiency
is family history important in CF?
CRITICAL!!
how many different classes of infections do we have for CF and which are the most common?
7 classes
2 and 3 most common
what is the effect of class 2 mutations on CFTR gene?
protein so abnormal not expressed - EG. DF508
what is the effect of class 3 mutations on CFTR gene?
Called acting mutations - only partially functioned but still expressed
what drug do we use for class 3 CFTR mutations
CFTR potentilator
can we use drugs for class 2 CFTR mutations and if so what?
harder as protein not expressed:
- Tezacaftor/ Lumacaftor + ivacaftor used to increase expression, but this not potent
- Triple therapy addition of : elaxacaftor to tezacaftor(stabilise and express protein) and invacaftor (mature protein) - significantly effective
What are the key symptoms of pneumonia?
- progressive breathlessness
- ankle swelling
- cough with green sputum
What is the FEV1 and indicator of?
FEV1 is a sensitive indicator of lung function
What patients are suitable for LTOT (long term oxygen therapy)?
- patients with COPD who have a PO2 <7.3kPa when stable
- PO2 > 7.3 kPa and <8 kPa when stable and one or more of the following
— secondary polycythaemia
— nocturnal hypoxaemia
— peripheral oedema
— pulmonary hypertension
What is a type 1 respiratory failure?
Hypoxia
- PCO2 normal
- V/Q mismatch
What is a type 2 respiratory failure?
Hypoxia and hypercapnoea
- inadequate alveolar ventilation
- PCO2 high
What does hypoxic drive mean in the context of COPD?
The patient needs low oxygen in order to breathe
What are the types of COPD?
- pink puffer: maintain normal blood gas at the expense of breathlessness and often with skeletal muscle wastage
- blue bloater: less breathlessness , but at the expense of abnormal blood gas tensions and right heart failure
What are treatments for COPD?
- beta agonists
- anticholinergic agents
- inhaled steroids
- antibiotics
- mucolytics
- flu vaccination
- non-invasive ventilation
- LTOT
- pulmonary ventilation
- lung volume reduction surgery
what are the two types of lung cancer?
small cell and non-small cell
what symptoms are there of lung cancer?
cough breathlessness wheeze stridor (noisy breathing) haemoptysis swallowing difficulties hoarse voice chest pain anorexia bone pain neurology weight loss
what are the signs of lung cancer?
weight loss clubbing tachypnoea signs of collapse lymphodenopathy SVC obstruction horner's syndrome metastatic spread
wat is horner’s syndrome?
interruption of sympathetic nerve supply to the eye
- miosis (constricted pupil)
- partial ptosis (droopy eyelid)
- anhidrosis (loss of hemifacial sweating)
what is SVC obstruction?
superior vena cava obstruction
- large tumours can surround the trachea and block the SVC
- can cause engorgements - little veins on skin as like with a blocked river, tributaries will form
what investigations can you do for lung cancer?
chest x ray CT scan PET scan bronchoscopy EBUS perataneous fine needle aspiration mediastinoscopy VATS
what will a chest x ray do with lung cancer?
can usually (70-80% of cases) see the tumour clearly
what will a CT scan do with lung cancer?
good for confirming diagnosis and staging (can see if there is metastasis)
also in screening for early tumours
what will a PET scan do with lung cancer?
positron emission tomography
- system detects gamma rays emitted by a tracer
- FDG (flurodeoxyglucose)
- indicates tissue metabolic activity –> can see if benign or malignant and if it has metastasised
what will a bronchoscopy do with lung cancer?
70% of tumours arise in large bronchi and so can be seen and biopsied by bronchoscopy
- can take brushing of cells
- can also go in with forceps and grab a chunk of tumour
what is EBUS and for lung cancer?
endobronchial ultrasound
- ultrasound at end of bronchoscope
- can put needle in lymph nodes to get direct samples
what is perataneous fine needle aspiration?
for tumours not reached through bronchoscope
CT guided through chest wall to get sample
what is mediastinoscopy?
looks at mediastinum
what is VATS?
video assisted thoracoscopic surgery
- telescope between lung and parietal pleura to see outside of lung (good for mesotheliomas)
what is treatment for small cell lung cancer?
chemotherapy and radiotherapy
what is treatment for non-small cell lung cancer?
surgery and radiotherapy
chemotherapy for certain tumours
can you have targeted drug treatment for lung cancer?
advanced cancers
epidermal growth factor receptors in response to chemotherapy in non-small cell
what is the survival of lung cancer?
one of the lowest survival rates of cancers due to late diagnosis in 2/3 patients
what are the causes of lung cancer?
90% due to smoking (increase risk by 8-20 fold) asbestos exposure radioactive materials (radon gas) pyrene, arsenic, nickel, napthalenes family history
what is a paraneoplastic syndrome?
syndrome that is a consequence of cancer in the body due to production of chemical signalling molecules (eg hormones) by tumour cells or the immune response to tumour
what is ectopic cushings syndrome caused by?
ectopic ACTH and ACTH-like substance
what is hyponatremial reversible confusion caused by?
antidiuretic hormone
what is hypercalcaemia caused by?
parathyroid hormone-related protein
what is pulmonary embolus state caused by?
hypercoaguable state
what is a pleural effusion?
build up of fluid between parietal and visceral pleura
what is an exudate pleural effusion?
greater amounts of protein
malignancy and infection
what is a transudate pleural effusion?
lesser amounts of protein
heart failure and nephronic syndrome
what is an empyema?
inflammatory cells and pus in pleura
what is a hydropnuemothorax?
pneumothorax and fluid
what is a heamothorax?
blood in pleura
what is chylothorax?
lymph in pleura
what is pneumonia?
an acute lower respiratory infection associated with recently developed radiological signs
how can the flu lead to pneumonia?
can develop into secondary Bacterial Staphylococcus infection
who does pneumonia affect the most?
the very young and elderly
what are the symptoms of pneumonia?
progressive breathlessness fever with shivering attacks left sided sharp pain general lethargy chest pain - pleurisy
what is pleurisy?
sharp pain which is worse on inspiration caused by inflammation of the pleura (basically them rubbing together like sandpaper)
what are the signs of pneumonia?
hot, flushed with periferal dilation chest is dull to percussion and increased breath sounds crackles pleural rub hypotension
why may pneumonia cause hypotension?
inflammatory stimulus
decreasing peripheral arterial resistance
cardiac output increases
give some organisms that could cause pneumonia?
bacteria - streptococcus pneumoniae, haemophilus influenza, staphylococcus aureus, gm neg bacteria influenza chlamydia pnuemoniae and psttaci legionella pnuemophila mycoplasma pneumoniae
how do you treat pneumonia?
antibiotics/antivirals O2 analgesia for chest pains fluids physiotherapy nutrition stop smoking
what is the scoring system for predicting complications of pneumonia?
CURBA Confusion Urea Respiratory rate Blood pressure Age
what test is used for confusion in CURBA?
abbreviated mental test
what age is risk in CURBA?
over 65
what CURBA score would be ok for home treatement?
short stay in hospital?
defo hospital as likely severe?
0/1
2
3 or more
what complication could arise from pneumonia?
empayema - pleural effusion of bacteria
what is the treatment for empayema?
pleural aspiration and drain (may require VATS/ surgery)
TPA to break up empayema for drainage