Diseases Flashcards
What is ACOS?
Asthma / COPD overlap syndrome
Define asthma
A chronic inflammatory disease of both large and small airways. Airway inflammation is the basic underlying process
What is the asthma triad?
- reversible airflow obstruction
- airway inflammation
- airway hyperresponsiveness
What are the hallmarks of remodelling in asthma?
- thickening of the basement membrane
- collagen deposition in the submucosa
- hypertrophy in the smooth muscle
Describe the inflammatory cascade in asthma
- inherited or acquired factors; viral, allergen or chemical
- eosinophilic inflammation
- mediators - TH2 cytokines
- twitch smooth muscle (hyper reactivity)
What would be prescribed for twitchy smooth muscle (hyper reactivity) ? (asthma)
- bronchodilators
- beta 2 agonists
- muscarinic antagonists
What would be prescribed for mediators / TH2 cytokines? (asthma)
- antileukotrienes or antihistamines
- anti IgE
- anti interleukin 5
What would be prescribed for eosinophilic inflammation? (asthma)
- anti-inflammatory medication
- corticosteroids
- cromones
- theophylline
Describe the triggers of asthma
- allergens
- others such as exercise, viral infection, smoke, cold, chemicals, drugs (NSAIDs, beta blockers0
Describe the clinical signs of asthma
- episodic symptoms and signs
- diurinal variability - nocturnal/ early morning
- non productive cough, wheeze
- triggers
- associated atopy, increased IgE (rhinitis, conjunctivitis, eczema)
- blood eosinophilia >4%
- responsive to steroid or beta-agonists
- family history of asthma
- wheezing due to turbulent airflow
Describe the diagnosis of asthma
- history and examination
- diruninal variation of peak flow rate
- reduced forced expiratory ratio (FEV.FVC <75%)
- reversibility to inhaled salbutamol
- provocation testing = bronchospasm
Describe COPD
- a multi-component disease process
- mucociliary dysfunction
- inflammation
- tissue damage
- leads to obstruction of airflow
Describe chronic bronchitis
- chronic neutrophilic inflammation
- mucus hypersecretion
- mucociliary dysfunction
- altered lung microbiome
- smooth muscle spasm hypertrophy
- partially reversible
Describe emphysema
- alveolar destruction
- impaired gas exchange
- loss of bronchial support
- irreversible
Describe the COPD clinical syndrome
- chronic symptoms, not episodic
- smoking
- non atopic
- daily productive cough
- progressive breathlessness
- frequent infective exacerbations
- chronic bronchitis, wheezing
- emphysema, reduced breath sounds
Describe the chronic cascade in COPD
- progressive fixed airflow obstruction
- impaired alveolar gas exchange
- respiratory failure ; decrease PaO2 and increased PaCO2
- pulmonary hypertension
- right ventricular hypertrophy / failure
- death
- stopping smoking arrests further decline in lung volume
Describe ACOS
- COPD with blood eosinophilia >4%
- responds better to ICS with exacerbation reductions
- more reversible to salbutamol
- difficult from asthmatic smokers who have airway remodelling (reduced FVC)
The physiology of hypoventilation leads to what?
Hypoxaemia and then to hypercarbia
What guidelines are used in the treatment of asthma?
Sign guidelines
Describe the challenge test
- use mannitol or histamine
- airways will narrow to a certain extent
Describe the benefit of flu vaccines
- reduce flu rates
- reduce admissions
- reduce severity of flu
- opportunistic vaccination
What are the challenges in primary care of asthma
- non-attendance
- SABA overuse
- who to refer to secondary care
When would you refer for additional investigation and specialist advice for asthma?
- diagnosis unclear
- suspected occupational asthma (symptoms that improve when patient is not at-work, adult onset asthma and workers in high risk occupations)
- poor response to asthma treatment
- severe / life threatening asthma attack
Describe the ‘red flags’ and indicators of other diagnoses in reference to asthma
- prominent systemic features (myalgia, fever, weight loss)
- unexpected clinical findings (eg. crackles, clubbing, cyanosis, cardiac disease, monophonic wheeze or stridor)
- present non-variable breathlessness
- chronic sputum production
- unexplained restrictive spirometry
- chest x-ray shadowing
- marked blood eosinophilia
Name some important mimics of asthma
- COPD
- hypersensitivity pneumonitis
- inducible laryngeal obstruction
- dysfunctional breathing
Describe inducible laryngeal obstruction
- difficulty breathing in rather than out
- feeling of something stuck in the throat
- triggered by exercise, perfume, strong smells, flowers, change in temperature
- not an allergic phenomenon
Describe the effects of smoking
- reduces ciliary beat frequency
- sputum retention
- increased infection
- steroids are much less effective in smokers
- macrolide antibiotics are not effective in smokers
Describe allergic borncho-pulmonary aspergillosis
- an allergic response to aspergillus
- mucus plugging
- proximal bronchiectasis
- total IgE >1000, elevated aspergillus IgE
- treatment = steroids and itraconazole
Describe allergic asthma
- usually childhood onset
- atopic triad; asthma, eczema, rhinitis
- typically allergic to; HDM, grass, cats and dogs
- dermatographism
- treatment options; Montelukast, antihistamines, allergen avoidance, omalizumab (monoclonal antibody to IgE
Describe eosinophilic asthma
- usually adult onset
- female preponderance
- usually more steroid resistant
- often stuck on prednisolone
- anti-allergen therapy not effective
- anti IL5 therapy; mepolizumab, benralizumab
A combination of severe asthma is recognised by what criteria?
- previous near fatal asthma eg. previous ventilation or respiratory acidosis
- previous admission for asthma, especially in the last year
- requiring three or more classes of asthma medication
- heavy use of a beta 2 agonist
- repeated attendances at ED for asthma care, especially in the last year
AND adverse behavioural or physiological features
Name the clinical signs of life-threatening asthma
- altered conscious level
- exhaustion
- arrhythmia
- hypotension
- cyanosis
- silent chest
- poor respiratory effort
- PEF <33% best or predicted
- SpO2 <92%
- PaO2 <8 kPa
- normal PaCO2
What are the co-morbidities associated with COPD?
- heart failure
- ischaemic heart disease
- obesity
- interstitial lung disease
- bronchiectasis
Describe the pharmacological management of COPD
- bronchodilators
- inhaled corticosteroids
- combination therapies
- oral therapies
Describe the use of bronchodilators in stable COPD
- LABA and LAMA significantly improve lung function, dyspnoea, health status and reduce exacerbation rates
- LAMA have a greater effect on exacerbation and decrease hospitalisations
- LABA/LAMA combination increases FEV1 and reduces symptoms compared with monotherapy
- theophylline exerts a small bronchodilator effect in COPD
Describe inhaled corticosteroids in COPD
- an ICS combined with LABA is more effective than the individual components in improving lung function and health status
- regular treatment with ICDS increases the risk of pneumonia, especially in those with severe disease
- triple inhaled therapy improves lung function, symptoms and health status
When would you consider use of ICS treatment in COPD?
- one moderate exacerbation of COPD per year
- blood eosinophils 100-300 cells / ul
What factors go against use of ICS in COPD
- repeated pneumonia events
- blood eosinophils <100 cells/ ul
- history of mycobacterial infection
What are the key principles in treating COPD?
- accurate diagnosis
- treat co-morbidities
- smoking cessation
- vaccinations
- pulmonary rehab
- LABA/LAMA
- trial of triple if high eos or frequent exacerbator
Who do you refer to secondary care in terms of COPD?
- diagnosis uncertain
- rapidly declining FEV1
- for consideration of; LVRS, bronchoscopic values, lung transplant
Describe exacerbations in COPD
- an exacerbation of COPD is defined as an acute worsening of respiratory symptoms that results in additional therapy
- the symptoms are not specific to COPD - relevant differential diagnoses should be considered
- exacerbation of COPD can be precipitated by several factors
- the goal of treatment is to minimise the negative impact of the current exacerbation
Name the differential diagnoses of COPD exacerbation
- pneumonia
- pneumothorax
- pleural effusion
- pulmonary embolism
- pulmonary oedema
- cardiac arrhythmias
Describe exacerbation in COPD
- increased short acting bronchodilators are recommended as initial management
- systemic steroids can improve lung function, oxygenation and shorten recovery time. Duration of therapy should not be more than 5-7 days
- antibiotics, when indicated, can shorten recovery time, reduce the risk of early relapse, treatment failure. Duration of therapy should be 5-7 days
Describe the approach in clinic in COPD
- confirm the diagnosis of COPD
- determine if there are additional diagnoses or exacerbating factors, particularly compliance
- optimise therapy
- consider surgical options
- discuss anticipatory care and end of life management
Name the surgical options for COPD
- bullectomy
- lung volume reduction surgery
- endobronchial valves and coils
- lung transplant
Describe bullectomy
- for bullae that occupy more than 50% of the thoracic cavity
- not without challenge
- need some other lung expand to take its place
- giant bullae are often associated with cannabis use
Describe volume reduction surgery
- effective for more heterogenous bullous emphysema
- surgical removal of the upper lobe(s)
- rarely done these days
- must have been through PR
Describe endobronchial valves/coils
- new technology
- one way valves of self collapsing coils inserted via a bronchoscope
- block ventilation to bullae, and areas of poor V/Q matching
Describe non-invasive ventilation
- two levels of pressure
- expiratory positive
- expiratory positive airway pressure; lowers the work of breathing, overcomes intrinsic PEEP, reduces pCO2
- inspiratory positive airways pressure; increases tidal volume and minute volume, reduces pCO2
What are the important considerations of non-invasive ventilation
- will the patient tolerate the mask
- does the patient have an ACP to avoid NIC
- what will we do if NIV fails
Describe the clinical presentation of pulmonary neoplasia
- local effects; obstruction of the airway, invasion of chest wall, ulceration
- metastases; nodes, bones, liver, brain
- systemic effects; weight loss, ectopic hormone production
Describe small cell cancer
- rapidly progressive disease
- early metastases
- rarely suitable for surgery, most of the time at first presentation, it has spread beyond the primary site
- good initial response to chemotherapy
- the rapid growth of the tumour makes it more susceptible to cytotoxic chemotherapy, often backed up with radiotherapy
Describe non-small cell cancer
- includes squamous and adenocarcinomas
- curative options are surgery or radical radiotherapy
- palliative chemotherapy and new targeted treatment
- account for the majority of lung cancers
Describe cytotoxic chemotherapy
- rarely curative but longer survival
- better response in small cell cancer
- major side effects
- intravenous infusions every 3-4weeks
- outpatient visits
- more detailed imaging
- whole body treatment
- targets rapidly dividing cells
- blood brain barrier; prophylactic cranial irradiation
Name some chemotherapy side effects
- nausea and vomiting
- tiredness
- bone marrow suppression
- opportunistic infection
- anaemia
- hair loss
- pulmonary fibrosis
Describe radiotherapy
- ionising radiation
- usually xrays
- external beam
- radical; curative treatment
- palliative; delaying tactic, useful for metastases
- well tolerated
Name some cons of radiotherapy
- maximum cumulative dose
- collateral damage- spinal cord, oesophagus, adjacent lung tissue
- only goes where you point the beam
- not suitable for subclinical metastases
Describe stereotatic ablative radiotherapy (SABR)
- many more beams
- each beam is less powerful;
- less collateral damage
- total dose delivered to tumour is higher and more effective
- 4D scanning required
Describe endobronchial therapy
- stent insertion for stridor
- photodynamic therapy
- other laser therapy
Describe histological diagnosis of pulmonary neoplasia
- bronchoscopy and biopsy of the tumour if seen
- biopsy or needle aspiration metastases (especially mediastinal or supraclavicular lymph nodes)
- endobronchial ultrasound guided specimens
- we need a microscope diagnosis and also sufficient tissue for identification of molecular predictor or response to treatment
What is pleural effusion?
- abnormal collection of fluid
- common presentation of numerous diseases
- does not always require drainage or sampling
- large unilateral effusions should raise concern
Describe the workup for pleural effusion
- history and examination
- PA CXR
- pleural aspirate
- biochemistry (transudate or exudate)
- cytology
- culture
- other tests; contrasted enhanced CT chest, repeat pleural tap, pleural biopsy (blind or thorascopy)
Describe the different appearances of pleural effusion and what they suggest
- straw coloured = cardiac failure, hypalbuminaemia
- bloody = trauma, malignancy, infection, infarction
- turbid / milky = empyema, chylothorax, smells
- foul smelling = anaerobic empyema
- food particles = oesophageal rupture
- bilateral = LVF (heart failure), PTE, drugs, systemic path
What does transudate mean?
- failure of an organ
- protein <30g/L
- heart failure; liver cirrhoisis, hypoalbuminaemia, atelectasis, peritoneal dialysis
- does not always have a benign aetiology
What does exudate mean?
- protein >30g/L
- malignancy
- infection including TB
- pulmonary infarction
- asbestos
- always look for serious pathology
When would glucose levels be low?
- infection
- TB
- rheumatoid arthritis
- malignancy
- oesophageal rupture
What do cytology and cell counts look for?
- mostly looking for malignant cells
- lymphocytes - think TB, malignancy, although any long standing effusion will eventually become lymphocytic
- neutrophils suggest an acute process
Why can a biopsy be negative?
- technique is wrong, biopsy does not contain pleura
- the involvement of pleural disease is discontinuous
- the effusion is ancillary to malignancy but not malignant
Describe mesothlioma
- ASBESTOS
- uncommon malignant tumour of the lining of the lung or very occasionally of the lining of the abdominal cavity
- likelihood of developing mesothelioma increases with the degree and the length of time exposed to asbestos
- often takes 30-40 years to develop
- may cause breathlessness, chest pain, weight loss, fever, sweating and cough
Name the asbestos fibre types
- chrysotile (white asbestos, most common)
- amosite (brown asbestos)
- crocidolite (most dangerous)
Name the investigations for mesothelioma
- imaging; pleural nodularity, circumferential pleural thickening, local invasion, lung entrapment
- pleural fluid aspiration; low cytological yield, avoid repeated aspiration
- biopsy; thoracoscopy or CT/US guided
Name the mesothelioma treatments
- pleurodeses effusions
- radiotherapy
- surgery
- chemotherapy
- palliative care
- report death to fiscal
Describe the treatment of malignant pleural effusion
- palliate symptoms
- repeated pleural taps
- drain and or pleurodesis (talc slurry or during thoracoscopy)
- long term pleural catheters
- surgical options (abrasion, pluerectomy)
What are the complications of talc slurry?
- minor pleuritic pain and fever
- pneumonia
- respiratory failure
- talc pneumonitis/ ARDS
- secondary empyema
- local tumour implantation at port site in mesothelioma
Describe long term pleural catheters
- designed to allow patients to control their effusion and therefore their symptoms
- inserted mostly in patients with malignant effusions
- may need an overnight stay
- drain is designed to remain in place for lifer though some people will stop producing pleural fluid
- vacuum in drainage bottle that provides suction to drain pleural fluid
- initially people will need to drain daily for a week or so
- never drain more than 1 litre per day
What are the complications of long term pleural catheters?
- incorrect placement
- bleeding
- infection
- flying can be tricky
- bath or swim can be done but not recommended
- 10 inserted each year
Name investigations for pneumothorax investigations
- chest xray; measured at hilar level not apex
- CT chest; useful to differentiate bullous lung disease or small pneumothoraxes
What is the management of a pneumothorax?
- oxygen even if no drain
- no treatment if asymptomatic and small
- aspiration 1st line in PSP; avoids chest drain, time consuming, may fail
- chest drain
- may need suction
- surgical intervention
What are the surgical intervention indicators for pneumothorax?
- second ipsilateral ptx
- first contralateral ptx
- bilateral spontaneous ptx
- persistent air leak
- risk professions (pilots, drivers) after first ptx
Describe the presentation of pneumothorax
- PSP may be asymptomatic even if moderately sized
- SSP usually symptomatic even if small
- acute onset pleuritic chest pain
- SOB, hypoxia
- sings; tachycardia, hype-resonant percussion note, reduced expansion, quiet breath sounds on auscultation
Describe tension pneumothorax
- emergency, can lead to cardiac arrest
- one way valve, progressively increasing pressure in pleural space
- pushes other chest organs to opposite side of affected side
- acute respiratory distress
- signs; deviated trachea, hypotension, raised JVP, reduced air entry on affected side
What is the treatment for tension pneumothorax?
- needle decompression
- usually a large bore venflon
- second intercostal space anteriorly, mid-clavicular line
Describe pleural infection
- increasing incidence especially extremes of age
- significant mortality (up to 20%)
- does not necessarily follow pneumonia
- can rapidly coagulate and organise to form fibrous peels even with antibiotics
- do not let the sun set on a potentially infected pleural space
Name some risks of pleural infections
- diabetes mellitus
- immuno-suppression including corticosteroids
- gastro-oesophageal reflux
- alcohol misuse
- intravenous drug abuse
- many patients have no apparent risk factors
Name the types of pleural infections
- simple parapneumonic effusion
- complicated parapneumonic effusion
- empyema
- quickly sample pleural fluid to identify parapneumonic effusions that require urgent tube drainage
Describe the management of pleural infection
- antibiotics (often for several weeks)
- drain effusion needed
- early discussion with surgeons if persistent sepsis
- nutrition
- VTE prophylaxis
- reassess patients who don’t improve
- IV antibiotics for a week and then oral antibiotics for 6 weeks
What shape of bacteria is m. TB?
rod shaped gram positive bacillus
How is TB transmitted?
Through the aerosol route, bacillus is small enough to travel in the droplet
Who gets TB?
- immigrants
- people who have had recent contact with someone who has TB
- socially deprived
- immunosuppressed
Name the symptoms of TB
- weight loss
- night fevers / sweats
- loss of appetite
- coughing
- haemoptysis
- breathlessness
- fatigue
- chills
What are the two types of TB?
- primary
- reactivated
What drugs are given for treatment of active TB?
- 4 drugs for 2 months; rifampicin, isoniazid, pyrazinamide, ethambutol
- 2 drugs for a further 4 months; rifampicin, isoniazid
What drugs are given for treatment of latent TB?
- 2 drugs for 3 months; rifampicin, isoniazid
- or 1 drug for 6 months; isoniazid
What is the initial intracellular primary niche of MTB?
the macrophage
At which anatomical location is the distinction between upper and lower respiratory tract infections?
Above the vocal cords is upper respiratory tract infections
Below the vocal cords is lower respiratory tract infections
What do viral throat swab tests test for?
- influenza A
- influenza B
- RSV
- metapneumovirus
- rhinovirus (particularly bad in asthmatic patients)
- coronavirus
- parainfluenza
- adenovirus
- enterovirus
- parechovirus
Describe strep throat
- exudate (goo)
- pus
- sore throat
- dysphagia
- dysphonia
- majority caused by virus
- do not recommend bacterial swabs or antibiotics
Describe tonsillitis
- swollen tonsils
- erythematous
- dysphagia
- recurrent = tonsillectomy
- tonsils are lymphoid tissue
- patients with bacterial infection will benefit from antibiotics
What is the fever pain score?
- used to help guide whether antibiotics are recommended or not
- fever during previous 24 hours
- purulence
- attended rapidly
- severely inflamed tonsils
- no cough or coryza
Describe quinsy
- complication of tonsillitis
- pre-tonsillar abscess
- can be drained but must be aware of the internal carotid artery
- airway obstruction - ludwigs angina
- IV antibiotics and surgical drainage
- retropharyngeal abscess
- sepsis
- can be described as ‘hot potato’
- patients present being unable to swallow
Describe epiglottitis
- emergency situation
- seen predominantly in infants
- infection means it swells = airway obstruction
- historically associated with influenza B
- now most cases are usually strep pneumonia, pyrogenes or staph aureus
- airway must be secured with and ET tube
- urgent IV antibiotics - ceftriaxone, vancomycin or clindamycin
Describe coryza (common cold)
- acute viral infection of the nasal passages
- often accompanied by sore throat
- sometimes a mild fever
- spread by droplets and fomites
- complications can include sinusitis or acute bronchitis
Describe sinusitis
- frontal headache
- retro-orbital pain
- maxillary sinus pain
- tooth ache
- discharge
- sinuses drain into the nose
Describe acute sinusitis
- preceded by a common cold
- purulent nasal discharge
- most viral aetiology
- usually self limited
- resolves in ten days
- some need antibiotics
- nasal decongestant; oxymetazoline
- nasal steroids
- pseudo-ephedrine
Describe diphtheria
- obstructs airway
- life threatening due to toxin production
- characteristic pseudo membrane
Name some lower respiratory tract infections
- acute bronchitis
- acute exacerbation of COPD
- pneumonia
- influenza
- fungal infection
Describe acute bronchitis
- the cold which ‘goes to the chest’
- often preceded by common cold
- clinical features; productive cough, fever, normal chest examination, normal chest x-ray, may have a transient wheeze
- usually viral symptoms
- cough really burns in the centre of the chest
- treatment; usually self limiting and analgesia recommended. Can lead to significant morbidity in patients with chronic lung disease
Describe an acute exacerbation of COPD
- may be preceded by an URT infection
- increased sputum production, increased sputum purulence, more wheezy, breathlessness
- on examination; respiratory distress, wheeze, coarse crackles, may be cyanosed (in advanced disease- worsening ankle oedema)
- management in primary care; antibiotics (doxycycline, amoxicillin), bronchodilator inhalers, short course of steroids in some cases
- refer to hospital if; evidence of respiratory failure or not coping at home
In which disease would red hepatisation of the lung occur?
In pneumonia
Name the symptoms of pneumonia
- malaise
- anorexia
- sweats
- rigors (uncontrollable shaking)
- myalgia
- headache
- arthralgia
- confusion
- cough
- pleurisy
- haemoptysis
- dyspnoea
- preceding URTI
- abdominal pain
- diarrhoea
Name the signs of pneumonia
- fevers
- rigors
- herpes labialis
- tachypnoea
- crackles
- rub
- cyanosis
- hypotension
When is a sputum sample sent?
If there is persistent or recurrent infection
Name the investigations for pneumonia
- blood culture
- serology
- arterial gases
- full blood count
- urea
- liver function
- chest x-ray
What is CURB65?
- severity score for community acquired pneumonia
- c= new onset of confusion
- u = urea >7
- r = respiratory rate >30/min
- b = blood pressure systolic <90 or diastolic <61
- 65 years or older
Name some other severity markers for pneumonia
- temperature <35 or >40
- cyanosis PaO2<8kPa
- WBC <4 or >30
- multi-lobar involvement
Describe the management o community acquired pneumonia
- antibiotics; doxycycline, amoxicillin
- oxygen; maintain SaO2 94-98% or 89-92%
- fluids
- bed rest
- no smoking
What disease is associated with contact with birds?
Psittacosis
Describe legionella
- chest symptoms may be minimal
- GI disturbance is common
- confusion is common
- levofloxacin
- can catch from stagnant water
When are IV antibiotics given?
- when oral route isn’t available
- sensitivities; drug resistant organisms (pseudomonas)
- deep seated infection- abscesses, bone endocarditis, meningitis
- first rapid- rapid increase in plasma concentrations
Name the complications of pneumonia
- respiratory failure
- pleural effusion
- empyema
- death
Describe the clinical presentation of influenza
- fever; high, abrupt onset
- malaise
- myalgia
- headache
- cough; initially dry but becomes productive
- prostration
- generally feeling unwell
Name the complications of flu
- primary influenzal pneumonia; high mortality, seen in young adults, seen most during pandemic years
- secondary bacterial pneumonia; more common in infants, elderly and debilitated, pre-existing disease and pregnant women, most common cause of death in fatal influenza
- bronchitis
- otitis media
- influenza during pregnancy may also be associated with perinatal mortality, prematurity, smaller neonatal size and lower birth weight
What disease should be thought of if there is shadowing in the upper zone cavities?
Tuberculosis
Name some risk factors for developing chronic pulmonary infection
- abnormal host response; immunodeficiency, immunosuppression
- abnormal innate host defence; damages bronchial mucosa, abnormal cilia, abnormal secretions
- repeated insult; aspiration, indwelling material
Name some drugs or treatments which can be immunosuppressive
- steroids
- azathioprine
- methotrexate
- cyclophosphamide
- monoclonal antibodies
- chemotherapy
Name some forms of chronic infection
- intrapulmonary abscess
- empyema
- chronic bronchial sepsis
- bronchiectasis
- cystic fibrosis
Describe the presentation of intrapulmonary abscesses
- indolent presentation
- weight loss common
- lethargy; tiredness, weakness
- cough +/- sputum
- high mortality if not treated
- usually a preceding illness of some sort; pneumonic infection, post viral, foreign body
Describe the features of a simple parapneumonic effusion
- clear fluid
- ph >7.2
- LDH <1000
- glucose >2.2
Describe complicated parapneumonic effusion
- ph <7.2
- LDH >1000
- glucose < 2.2
- requires chest tube drainage
What is the D sign?
Empyemas are sticky and keep shape despite gravity (unlike effusion) so can appear as a d shape on scans
Describe bronchiectasis
- localised, irreversible dilation of the bronchial tree,
- involved bronchi are dilate, inflamed and easily collapsible
- airflow obstruction
- impaired clearance of secretions
- recurrent sputum production
Describe the presentation of bronchiectasis
- recurrent chest infections
- recurrent antibiotic prescriptions
- no response to antibiotics
- short lived response to antibiotics
What would CT scans show for bronchiectasis?
- dilation of the airways, thickening of bronchial walls, lack of tapering airways
- airways is larger in diameter than the accompanying pulmonary artery
Name the causes of bronchiectasis
- 50% idiopathic
- bronchial obstruction
- cystic fibrosis
- youngs syndrome
- kartanagers syndrome
- ABPA
- immunodeficiency
rheumatoid arthritis - bronchopulmonary sequestrian
- mounier-khun syndrome
- yellow nail syndrome
- traction bronchiectasis associated with pulmonary fibrosis
What diseases feature failure of the mucociliary escalator?
- cystic fibrosis
- youngs syndrome
- kartanagers syndrome
Name the treatment options for bronchiectasis
- stop smoking
- flu vaccine
- pneumococcal vaccine
- reactive antibiotics; send sputum sample
- when colonised with persistent bacteria; oral macrolide antibiotics, nebulised gentamicin, colomycin , pulsed IV abx. alternating oral antibiotics
How would you treat acute exacerbations of bronchiectasis?
- 2 weeks of antibiotics appropriate to the most recent positive sputum sample
- send sputum every time
- alter antibiotics if the sputum culture shows resistant organism
- aggressively eradicate pseudomonas aeriginosa
How many people carry the gene for cystic fibrosis?
1 in 25 people carry the gene
What type of genetic disorder is CF?
Recessively inherited gene disorder
Specifically, which molecule is affected by CF?
Cystic fibrosis trans-membrane conductance regulator
What happens if there is no CFTR protein present or an abnormal CFTR protein?
This means there is no negative reinforcement of ENAC and Na+ can move into the cell freely. This causes water to flow out of the cell meaning secretions are much thicker as the is less water
Name the consequences of CF
- salty sweat
- intestinal blockage
- fibrotic pancreas
- failure to thrive
- recurrent bacterial lung infections
- congenital bilateral absence of vas deferens
- filled sinuses
- gallbladder and liver disease
Describe class 1 cf
- no CFTR synthesis
- die in utero, not compatible with life
Describe class 2 CF
- delta f508
- CFTR trafficking defect, cftr is made but not the correct shape
- most common defect
- die in 30s and 40s
Describe class 3 CF
- dysregulation of CFTR
- channel created but will not open
Describe class 4 CF
- defective chloride conductance or channel gating
- channel half open
Describe class 5 CF
- reduced CFTR transcription and synthesis
In CF patients, if staph aureus was colonised, what treatment would be given?
- oral flucloxacillin
- oral septrin
In CF patients, if pseudomonas was colonised what treatment would be given?
- oral azithromycin
- nebulised colomycin
- nebulised tobramycin
- nebulised aztreoanam
- inhaled tobramycin
Describe endocrine failure
- destruction of pancreatic islet cells, patty replacement of pancreatic tissues
- annual OGTT,CGMS
- usually need insulin as they have insulin production failure
Describe exocrine failure
- sludged up ducts, failure of secretion of lipases (amylases), digestive failure
- give creon
- patients hate taking it
Describe DIOS
- thick mucus blocks up the large and small intestine
- symptoms similar to constipation
- treatment; gastrograffin, laxido, fluids
- prevention; laxido, hydration, keep moving
What composes exacerbation management in CF patients?
- antibiotics
- physiotherapy; autogenic drainage, ACBT, with and without a physiotherapist
- adequate hydration
- increased dietary input; dietician, fridge in room
Name the oral antibiotics used for CF patients
- augmentin
- flucloxacillin
- minocycline
- septrin
- fusidin
- ciprofloxacin
Describe OHPAT
- treatment at home for CF patients
- start antibiotics as an inpatient, finish at home
- 2 weeks antibiotics at home
- well tolerated
- safe
- saves money
- keeps patients at home
Describe Ivacaftor
- CFTR potentiator, opens the channel
- improves chloride flow through the CFTR
- tablet, twice a day
- cant have grapefruit juice
Describe symkevi
- tezecaftor and ivacaftor
Describe lung transplantation in CF patients
- bilateral lung transplantation
- consider once FEV1 <40%
- patients with M abscesses no eligible
- psychological assessment is key
