Respiratory Disease Flashcards
(119 cards)
1
Q
— asthma —
Describe the pathology of asthma.
A
- asthma is a chronic disorder of the conducting airways caused by an immunologic reaction
- bronchoconstriction is reversible
- atopic asthma is mediated by IgE (type I hypersensitivity)
- remodelling of the airway occurs with repeated allergen exposure, including hypertrophy and hyperplasia of smooth muscle, epithelial injury, increased vascularity, mucus gland hypertrophy, and deposition of collagen
2
Q
Describe the clinical presentation of asthma.
A
- Asthma is characterised by wheezing, dyspnoea, chest tightness, and cough.
- Symptoms are often diurnal (worst in the early morning and at night)
- There may be a history of other atopic conditions, such as eczema or allergic rhinitis
3
Q
Describe the rationale behind investigating asthma.
A
- diagnosis is based on clinical assessment, and can be supported by tests. NICE guidelines have recently advocated moving toward more objective tests.
- spirometry with bronchodilator reversibility testing (BDR)
- fractional exhaled nitric oxide test (FeNO)
4
Q
Describe the mechanism by which asthma causes remodelling of the airway.
A
Bronchoconstriction, chronic inflammation (eosinophils, by IgE hyp1), and airway hyperresponsiveness.
5
Q
Describe the stepwise management of asthma.
A
- SABA + ICS (if 3+ symptoms/week or night-time waking)
- SABA + ICS (low-dose)
- SABA + ICS + LTRA (e.g. montelukast)
- SABA + ICS + LABA (+ LTRA in adults if responsive previously)
- SABA (+ LTRA), and switch to a MART (low-dose ICS)
- SABA (+ LTRA) and increase steroid dose component of MART
- Specialist territory; options include high-dose steroid (fixed regime only, not as part of a MART), adding theophylline, and seeking advice from a professional
NB steroid dosing classification from NICE has recently changed:
- low dose: <400ug equiv.
- moderate dose: 400-800ug equiv.
- high dose: >800ug equiv.
6
Q
Describe the types of inhaler available.
A
- pressurised metered dose inhaler (pMDI): available with spacer (yellow or blue), and can be used in all ages
- breath actuated (BAI): used only in school-aged children
- dry powdered inhaler (DPI): should only be used in older children (e.g. 12+)
7
Q
Compare the types of steroid available for airway disease, and how they may be administered.
A
Firstly, it is important to note steroids are compared by considering the beclomethasone dipropionate (BDP) equivalent dose. Steroid dosing is based on the BDP dose being ‘standard’ potency (e.g. BDP = 1).
- there are three main ICS available: beclomethasone (BDP), budesonide, and fluticasone.
- budesonide = BDP
- fluticasone is twice as potent as BDP (e.g. 0.5 fluticasone = 1 BDP)
ICS inhalers are available as either monotherapies with brand names, or as combination therapies with a LABA (formoterol or salmeterol), which constitutes a MART
- BDP: clenil modulite, kelhale etc.; fostair is a MART with formeterol
- budesonide: easyhaler, pulmicort, turbohaler, symbicort (+ formeterol)
- fluticasone: flixotide, accuhaler, evohaler, seretide (+ salmeterol)
new NICE dosing is as follows:
- very low: 200ug/day BDP equiv.
- low: 400ug/day BDP equiv.
- medium: 800ug/day BDP equiv.
- high: >800ug/day BDP equiv.
8
Q
Describe the features that constitute life-threatening asthma.
A
Remember 33 92 CHASE (not CHEST!):
- PEFR <33% expected
- SpO2 <92%
- cyanosis
- hypotension
- arrhythmia/altered consciousness
- silent chest
- exhaustion
9
Q
Describe the role of CO2 concentrations in assessing severity of acute asthma.
A
- acidosis with a normal PaCO2 indicates exhaustion, and is a feature of life-threatening asthma
- acidosis with a raised PaCO2 indicates near-fatal asthma
10
Q
Describe the management of acute asthma.
A
The mnemonic ‘O SHIT ME’ may be used to remember the key drugs, but does not fully represent the order in which they should be given.
The following should be given to all patients simultaneously:
- O2: 15L non-rebreather, titrated down to a flow rate when patient is able to maintain a normal SpO2
- Salbutamol (back-to-back nebulisers intially)
- Hydrocortisone (prednisolone is now recommended)
The following should be given additionally if required and with senior input:
- Magnesium sulphate (IV over 20 minutes as a one-off dose), given before theophylline
- Theophylline (aminophylline infusion)
- Escalate care (intubation and ventilation)
11
Q
What criteria are required to diagnose a patient after an episode of acute asthma?
A
- stable on discharge medication (e.g. oral prednisolone)
- no nebulisers or O2 for 12-24hr
- inhaler technique checked and recorded
- PEFR >75% recorded
12
Q
— COPD —
Describe the pathophysiology of COPD.
A
COPD is a condition which consists of emphysema and chronic bronchitis.
- emphysema is characterised by irreversible enlargement of airspaces distal to the terminal bronchioles, plus destruction of the walls without obvious fibrosis.
– centriacinar emphysema is seen in ~95% of cases of emphysema COPD
– panacinar emphysema is typically seen in a1-antitrypsin deficiency
- chronic bronchitis: defined as persistent cough + sputum production for >3 months in >2 consecutive years. hypertrophy of tracheal and bronchial cells leads to mucus hypersecretion as a protective reaction against smoke, smog etc.
13
Q
What are the clinical differentiating signs of asthma?
A
Reversible (asthma) vs non-reversible (COPD) obstruction. Non-smoking vs smoking. Wheeze vs no wheeze. Diurnal variation (PEFR) vs not.
14
Q
Describe the clinical features of COPD.
A
- progressive dyspnoea (especially on exertion), cough with sputum production, wheeze, frequent winter ‘bronchitis’
- less common: weight loss, waking at night, ankle oedema
- on examination: poor chest expansion, hyperinflated lungs, hypercapnic flapping tremor of hands, absence of clubbing
15
Q
Describe the diagnosis of COPD.
A
- NICE recommend considering a diagnosis of COPD in patients >35 who are smokers, or ex-smokers, and have symptoms such as exertional dyspnoea, chronic cough, regular sputum production; the following investigations are recommended in patients with COPD:
– post-bronchodilator spirometry to demonstrate airflow obstruction; FEV1/FVC <70%
– CXR: hyperinflation, bullae, flat hemidiaphragm and to exclude of lung cancer
– FBC: exclude secondary polycythaemia
– BMI calculation
16
Q
Describe the pharmacological management of stable COPD.
A
- SABA (initial therapy)
- assess whether the patient has steroid responsive features:
– previous diagnosis of asthma or atopy
– high eosinophil count
– variation in FEV1 (>400ml)
– diurnal variation in PEFR (>20%) - steroid responsive: SABA + ICS + LABA
- non-responsive: SABA + LABA + LAMA
After patients have been escalated through triple therapy:
- oral theophylline
- roflumilast (PDE4 inhibitor), if disease is severe (FEV <50%) and 2+ exacerbations despite triple therapy
- prophylactic macrolide (azithromycin) with the following criteria:
– CT thorax (exclude bronchiectasis)
– sputum culture (exclude atypical infection + TB)
– ECG (exclude QTc prolongation)
– 4+ exacerbations, requiring hospital admission at least once
- mucolytic (carbocysteine)
17
Q
Describe the presentation and management of an acute exacerbation of COPD not requiring hospital admission.
A
- H influenzae is the most common pathogen overall, with others including S pneumoniae, M catarrhalis, and respiratory viruses (e.g. human rhinovirus)
- features include acute worsening of COPD symptoms (e.g. increased sputum production), hypoxia, and sometimes acute confusion
- management consists of three main aspects:
– antibiotics (amoxicillin, doxycycline, clarithromycin): NICE advocate not giving these routinely, only for production of purulent sputum or clinical signs of pneumonia
– oral steroids (30mg prednisolone for 5 days)
– increased frequency of bronchodilators (SABA, SAMA) and consider giving via nebuliser
18
Q
What are the admission criteria for a patient with an acute exacerbation of COPD?
A
- severe dyspnoea
- acute confusion or impaired consciousness
- cyanosis or SpO2 <90%
- social (e.g. unable to cope at home or living alone)
- significant comorbidity (e.g. cardiac disease)
19
Q
Describe the management of an acute exacerbation of COPD in secondary care.
A
Oxygen therapy
- oxygen: prior to the availability of ABG results, use 28% Venturi at 4l/min with a SpO2 target of 88-92%
- patients who develop type 2 respiratory failure should be given BiPAP with an EPAP of 4-5cm H2O and an IPAP of 10-15cm H2O
- severely acidotic patients (pH <7.25) may be given BiPAP but should be monitored more frequently (e.g. in HDU) and with a lower threshold to intubate and ventilate
Additional therapies
- nebulised SABA/SAMA
- steroid therapy: oral prednisolone (30mg OD 5 days, or IV hydrocortisone)
- IV theophylline if not responding to nebulisers
20
Q
Why are airway spacers sometimes used instead of straight inhalers?
A
Traps particles that cause thrush, reduces particle size/velocity.
21
Q
— Bronchiectasis —
Name the risk factors for bronchiectasis.
A
- congenital and hereditary conditions: cystic fibrosis, immunodeficiency, Kartagener’s
- infections: pneumonia, measles, pertussis, TB
- bronchial obstruction: tumour, foreign bodies
- COPD
- autoimmune conditions: RA, SLE, IBD, post-transplant
22
Q
Describe the pathophysiology of bronchiectasis.
A
- destruction of smooth muscle and elastic tissue by chronic necrotising infections, leading to permanent dilation of bronchi and bronchioles
- normal clearing mechanisms are impaired, leading to pooling of secretions distal to obstruction
- pooled secretions lead to infection, inflammation, necrosis, fibrosis, and dilatation
- obliteration of bronchioles occurs progressively (bronchiolitis obliterans)
23
Q
Describe the clinical and radiological findings of bronchiectasis.
A
- severe, persistent cough with large volumes of foul smelling and occasionally bloody sputum (haemoptysis) and dyspnoea
- coarse crackles and wheeze
- clubbing may be present
- HRCT is the imaging modality of choice and may demonstrate ‘signet rings’ and a tram-track appearance
24
Q
Describe the management of bronchiectasis.
A
- physical training, such as inspiratory muscle training, which has a good evidence base for patients with non-CF bronchiectasis
- airway clearance: options include devices (Flutter, Acapella) or nebulised saline
- long-term rotating antibiotics: azithromycin is useful for long-term prophylaxis + ciprofloxacin for Pseudomonas
- surgery/transplantation
25
--- Oxygen therapy ---
When should liberal oxygen therapy overtake conservative?
In cluster headaches, poisoning, and tension pneumothorax
26
--- additional oxygen therapy cards ---
27
--- spirometry ---
Describe the peak expiratory flow rate curves for obstructive and restrictive disease.
Obstructive - peak is lower and not as long. Restrictive - peak reaches top but not as long.
28
--- Lung cancer ---
What are the two main presentations of lung cancer?
Primary and paraneoplastic
29
Describe some of the main symptoms of lung cancer.
Cachexia, cough, dyspnoea, persistant haemoptysis, hoarseness, recurrent pneumonia
30
Describe the histological classification and features of lung cancer.
SCLC
- arises from APUD cells
- typically central in location
- associated with SIADH, Cushing's syndrome, and Lambert-Eaton
NSCLC
- adenocarcinoma
-- most common type in both smokers and non-smokers
- squamous (SCC)
-- Central location
-- Cigarettes
-- Cavitating
-- Clubbing
-- Calcium (hypercalcaemia)
- large cell: peripheral, anaplastic tumours which may secrete B-hCG
31
Describe the main benign tumours of the lung.
- hamartoma: most common benign tumour; 'popcorn' calcification is virtually diagnostic
- bronchial adenoma: resembles an intestinal carcinoid tumour, but does not secrete serotonin (instead secretes ACTH)
- additional benign tumours include cylindroma, chondroma, and lipoma
32
Describe the investigations for lung cancer.
- bloods: may show raised platelets
- CXR: often the first investigation; may be normal in ~10% of cases
- CT: investigation of choice, allows staging via TNM
- bronchoscopy: histological diagnosis
- PET-CT: lymph nodes and metastasis
33
Describe the main paraneoplastic effects of lung cancer.
SCLC
- SIADH (ADH secretion)
- Cushing's syndrome (ACTH secretion): hypertension, hyperglycaemia, hypokalemic alkalosis, muscle weakness
- Lambert-Eaton syndrome: antibodies block calcium channels that enable acetylcholine release in the neuromuscular junction
Adenocarcinoma
- gynaecomastia
- HPOA + clubbing
SCC
- hypercalcaemia
- HPOA + clubbing
- hyperthyroidism due to ectopic TSH secretion (controversial)
Pancoast tumour
- Horner's syndrome
34
Which two treatments may be used for lung cancer?
Surgery/radiotherapy
35
Describe the referral criteria for suspected lung malignancy.
Urgent referral (2-week wait pathway)
- CXR findings suggesting lung cancer
- aged 40+ with unexplained haemoptysis
Offer CXR (2-week wait) if aged 40+ with either 2+ of the following, or if ever smoked with 1+ of the following:
- cough
- fatigue
- dyspnoea
- chest pain
- weight loss
- anorexia
Consider CXR (2-week wait) if aged 40+ with any of the following:
- persistent or recurrent chest infections
- finger clubbing
- supraclavicular lymphadenopathy
- persistent cervical lymphadenopathy
- thrombocytosis
36
What is the factor that decides whether a lung biopsy should be a bronchoscope or CT guided?
Proximity to the hilum.
37
--- TB ---
What is the main pathogen that causes TB?
Mycobacterium tuberculosis
Mycobacterium bovis is contracted from unpasteurised milk
38
What are the main symptoms of TB?
Haemopytsis, night sweats, weight loss
39
What are the risk factors for TB?
- immunosuppression: HIV, IVDU, solid organ transplant, haematological malignancy, chemotherapy
- age <5
- gastric surgery: jejunoileal bypass, gastrectomy
- comorbidities: CKD/haemodialysis, diabetes, silicosis
- biologics: e.g. anti-TNFa agents
40
Describe the histology of TB infection development.
- TB enters macrophages, replicating within them and seeding multiple sites
- activation of TH1 cells produces interferon gamma (IFN-y), which differentiates macrophages into epithelioid histiocytes, which aggregate to form granulomas
- the initial granuloma is known as the Ghon focus
- if the infection is not controlled within this site, it spills over to local pulmonary lymph nodes
- Ghon focus + lymph node infection = Ghon complex
41
Describe the investigations that should be undertaken to diagnose latent TB.
The Mantoux test remains the test of choice to diagnose latent TB. It is considered positive with an induration of 5+mm, regardless of BCG history.
- close contacts aged 18-65 should be offered a Mantoux test
- a positive Mantoux test should prompt assessment for active TB
- positive Mantoux test + no active TB: offer interferon gamma release assay (IGRA) if more evidence of infection is needed to decide on treatment
- inconclusive Mantoux: refer to a TB specialist
42
Describe the investigations that should be undertaken to diagnose active TB.
- CXR -> CT thorax
- sputum samples (3 deep cough samples, ideally including 1 early morning sample) for microscopy and culture
-- these should be sent prior to treatment, or within 7 days if treatment is initiated in life-threatening disease
-- if spontaneous samples cannot be produced consider gastric lavage, induction of sputum, or bronchoscopy and lavage
- NAAT should be offered for all children and in adults whom
-- have HIV
-- care would be altered with rapid information about mycobacterial species
-- need for large contact-tracing initiative is being explored
43
Describe the treatment of both latent and active TB.
Latent
- 3 months of RI (concerns re hepatotoxicity)
- 6 months of I alone
Active
- RIPE therapy for 2 months, then
- RI therapy for 4 months (10 months with CNS involvement)
44
Describe the side effects that may be observed with the 4 main drugs used for treating TB.
Rifampicin
- potent liver enzyme inducer
- hepatitis
- orange secretions ('rifam-pissin')
- flu-like symptoms
Isoniazid
- peripheral neuropathy ('I'm-so-numb-azid'); requires co-prescription of pyridoxine (vitamin B6)
- agranulocytosis
Pyrazinamide
- hyperuricaemia (precipitates gout)
- arthralgia, myalgia
Ethambutol
- optic neuritis (E = eyes)
45
--- Pneumonia ---
Which clinical symptoms may suggest pneumonia?
Cough, high fever, chest pain, tachypnoea, pleural rub, crackles.
46
Describe the classification of pneumonia.
- community-acquired pneumonia (CAP)
- hospital-acquired pneumonia (HAP): requires being in hospital for a minimum of 48 hours
- aspiration pneumonia
- ventilator-acquired pneumonia (VAP)
47
Briefly describe the microbiology of pneumonia.
- S pneumoniae: 80% of cases
- H influenzae: most common in COPD
- M catarrhalis: elderly patients and second-most common in COPD
- S aureus: IVDU, following viral infection
- Klebsiella: malnourished, debilitated, alcoholics
- Pseudomonas: immunocompromise, CF
- Legionella: contaminated water and air conditioning units
- P jiroveci: HIV infection
- Zoonoses: Coxiella burnetti (Q fever), C psittaci (avian proteins)
48
Describe the CURB65 score and its use.
Used to monitior patients/treatment with pneumonia and indicates its prognosis.
- Confusion,
- Urea >7mmol/l,
- Respiratory Rate >30,
- Blood Pressure <90/60,
- age 65+.
49
Describe the main treatments for community acquired pneumonia (CAP), hospital acquired pneumonia (HAP), and aspiration pneumonia.
CAP
- CURB65 0-1: amoxicillin/doxycycline, or a macrolide
- CURB65 2-3: combine amoxicillin/doxycycline + macrolide
- CURB65 3+: co-amoxiclav, or cefuroxime + macrolide
HAP
- <7 days admission: co-amoxiclav, or cefuroxime
- >7 days admission, or VAP:
-- ciprofloxacin, or
-- 3rd gen cephalosporin, or
-- extended spectrum penicillin
50
What is the difference between lobar and bronchopneumonia?
Lobar is confined to one lobe. May appear patchy but sharp lines indicate one lobe only. Bronchopneumonia spreads to main bronchi.
51
How do NICE recommend describing resolution of pneumonia to patients?
- 1 week: fever resolved
- 4 weeks: chest pain + sputum production substantially reduced
- 6 weeks: cough and dyspnoea substantially reduced; NB patients need a repeat CXR at this point to exclude malignancy etc.
- 3 months: most symptoms resolved, although fatigue may still be present
- 6 months: most people will feel back to normal
52
What are the clinical signs of influenza?
Abrupt high fever, malaise, myalgia, headache, cough, prostration
53
--- Influenza ---
How should influenza be diagnosed?
PCR and nasopharyngeal swab.
54
Describe how influenza outbreaks may occur each year.
Due to antigenic shift, antigenic variation, and mixing vessels.
55
How can influenza be prevented, and how is it treated?
Vaccination. Osaltmivir, Zaramivir
56
--- Pleural Effusion ---
Describe the terminology used around pleural effusion.
- parapneumonic effusion (PPE): any pleural effusion secondary to pneumonia or a lung abscess
- complicated PPE: a PPE with evidence of infection, usually bacterial, of the fluid itself
- empyema: an effusion with visibly turbid or culture-positive pleural fluid
57
Describe the investigation of pleural effusion.
- pleural effusion can be diagnosed using radiology, pleural aspiration, or pleural biopsy; CT follow-up should be considered to exclude occult malignancy
- thoracocentesis (pleural aspiration) is key to obtain fluid for protein, LDH, pH, cytology, and microbiology
- thoracic ultrasound (TUS) is strongly advocated prior to thoracocentesis
58
Describe the interpretation of pleural fluid sampling.
- protein and LDH are key to differentiating a transudate from an exudate.
-- low protein and LDH indicate a transudate (systemic)
-- high protein and LDH indicate an exudate (local)
Put simply, transudate can be considered as a failure of organ/s.
- liver (e.g. hypoalbuminaemia)
- heart (pulmonary oedema)
- pancreatitis
Exudate occurs with death of lung cells (hence a local problem, as protein leaks out).
- infection
- malignancy
- autoimmune disease
- post-CABG, PE
59
Describe the management of pleural effusion.
- remove the fluid and treat the underlying cause
- empyema requires an immediate drain in the 5th intercostal space
60
--- Asbestos Disorders ---
Describe the development of mesothelioma.
- as opposed to benign asbestos disorders, there is no correlation between length of asbestos exposure and severity of disease
- crocidolite is the most dangerous form of asbestos
- there is a long latent period between asbestos exposure and mesothelioma development (~25-45 years)
61
Describe the investigation of suspected mesothelioma.
- first line CXR, which may show pleural effusion and/or pleural plaques
- pleural CT should follow CXR
- thoracocentesis should be performed to send fluid for microscopy, culture, and cytology
- cytology is only helpful in 20-30% of cases; if negative follow up with a local anaesthetic thoracoscopy (sensitivity of 95%)
62
Describe the management of mesothelioma.
- if operable: surgery and chemotherapy (pemextred and cisplatin)
- symptomatic therapies: talc slurry, vacuum catheters
- prognosis is dim, with a median survival of 12 months
63
Describe the benign conditions related to asbestos exposure.
- pleural plaques: do not undergo malignant change; no follow-up required
- pleural thickening: pathophysiology is not fully understood
- asbestosis: severity is related to duration of exposure; causes lower lobe fibrosis, a restrictive defect, dyspnoea, clubbing, and bilateral end-inspiratory crackles. managed conservatively.
64
--- Pneumothorax ---
What are the clinical signs of someone with pneumothorax?
Dyspnoea, hypoxia, sharp pleuritic chest pain
On examination:
- hyper-resonant percussion note,
- reduced breath sounds and chest expansion
- tachycardia, tachypnoea
Features of tension pneumothorax include
- raised JVP
- haemodynamic instability (e.g. hypotension)
- tracheal deviation away from the side of the pneumothorax
65
Describe the classification of pneumothorax.
spontaneous
- PSP: occurs without underlying lung disease, often in tall, thin, young individuals
- SSP: occurs in underlying lung disease, e.g. asthma, COPD, CF, lung cancer etc.
traumatic: penetrating or blunt chest trauma
iatrogenic: thoracocentesis, central venous catheter placement, ventilation
66
How should a spontaneous, simple pneumothorax be managed?
Place the patient into one of three categories:
- minimal symptoms: no significant pain, breathlessness, or physiological compromise
- intermediate: greater than minimal symptoms, but no high-risk characteristics
- high-risk characteristics, including significant hypoxia, bilateral disease, >50 with a significant smoking history, and/or haemothorax
Minimal symptoms
- manage conservatively
- PSP: review 2-4 daily as an outpatient
- SSP: review daily as an inpatient
- follow-up as an outpatient in 2-4 weeks, including CXR
Intermediate
- conservative care
- ambulatory device (e.g. Rocket Pleural Vent)
- needle aspiration
High risk
- if safe to intervene, insert a chest drain
- remove drain after resolution
67
Describe the management of tension pneumothorax.
- do not delay intervention with imaging
- needle decompression to convert a tension pneumothorax to a simple pneumothorax: 2nd intercostal space, midclavicular line
- chest drain: 5th intercostal space, anterior axillary line
68
Describe the discharge advice for a patient with pneumothorax.
- advise smoking cessation (reduces risk from 10% to 0.1%)
- flying is an absolute contraindication until 1 week after a normal CXR
- scuba diving should be indefinitely avoided, unless the patient has had a bilateral pleurectomy and normal lung function + CT chest
69
--- Timing of Dyspnoea ---
An immediate onset of dyspnoea is suggestive of?
Pneumothorax, pulmonary embolism.
70
An acute onset of dyspnoea is suggestive of (mins-hrs)?
Asthma, pneumonia, acute MI, cardiac tamponade
71
Subacute onset (days) of dyspnoea is suggestive of?
Vasculitis, pleural effusion
72
Chronic dyspnoea is suggestive of?
COPD, ILD, hypertension, anaemia.
73
--- URTI & ENT ---
Describe tonsilitis.
Dysphagia, dysphonia. Swelling of the tonsils. Can lead to quinsy.
74
What is quinsy? How is it treated?
Peritonsilar abcess. Should be drained.
75
Which pathogens cause the emergency epiglottitis?
S. pyogenes or S. pneumoniae
76
What is diphtheria?
A pseudo-membrane formed around the tongue. Life threatening
77
Describe the symptoms and treatment of sinusitis.
Headache, retro-orbital pain, maxillary sinus, tooth pain. Nasal drain if severe.
78
Describe the main presentation of bronchiolitis.
Young child, coughing, fever, runny nose
79
Which pathogen causes bronchiolitis?
Respiratory synctial virus (RSV)
80
How may acute bronchitis be described? What are its main symptoms? Why can't it be treated by antibiotics?
A cold that goes to the chest. Fever and productive cough. It's caused by a virus.
81
What is the main presentation of pertussis?
Whooping cough. PARYOXSMAL attacks of cough with cold like symptoms and vomiting.
82
--- Cystic Fibrosis ---
What is used to screen for cystic fibrosis in newborns?
The heel prick test
83
Describe the variable presentations of cystic fibrosis.
- respiratory: recurrent infection, productive cough, development of bronchiectasis or abscess
- pancreatic: nutrient malabsorption and maldigestion (failure to thrive, faltering growth), diabetes mellitus
- GI: meconium ileus, rectal prolapse
- hepatic: portal hypertension, gallstones
- GU: urinary incontinence (F > M), male infertility (due to absence of vas deferens)
- growth: delayed puberty, osteoporosis
- psychosocial: depression (associated with planning futures with a reduced lifespan, difficulty eating in social settings, relationship problems)
84
Describe the epidemiology and genetic basis of cystic fibrosis.
- 1 in every 2500 live births
- 1 in 25 carrier frequency
- autosomal recessive disease
- the primary defect in CF arises from the abnormal function of an epithelial Cl- channel, encoded by the CFTR gene on chromosome 7
- the CFTR mutation is classed into six categories (I - VI), but class II is the most common (70%) and arises from an F508 variant
85
Describe the bare bones management of patients with cystic fibrosis.
Patients with CF should be managed by a dedicated CF service
Respiratory
- regular spirometry for review
- chest physiotherapy, BD, for 20-30min. this is performed by parents in younger children
- airway clearance: e.g. DNAse, hypertonic saline, mannitol
Nutrition
- vitamins and a high calorie diet (150% of general population)
- oral pancreatic enzyme replacement with all food (e.g. Creon)
Prophylactic antibiotics
- daily flucloxacillin PO
- regular nebulised macrolide, such as azithromycin
86
What is the definitive treatment of CF and when should it be administered?
Lung transplant, when FEV1 < 40%.
87
Which therapies, undergoing development, are revolutionising the management and life expectancy of patients with CF?
Immune modulators, such as ivacaftor, lumacaftor, trikefta, orkambi, symkevi etc.
88
--- Ciliary Disorders ---
Which two syndromes may cause abnormal cilia?
Kartenagar's, Young's
89
--- Restrictive Disorders ---
Name the causes of restrictive pulmonary disease.
- chronic infiltrative disease, such as idiopathic pulmonary fibrosis and pneumoconiosis
- chest wall disorders (neuromuscular disease, obesity, kyphoscoliosis)
90
What is idiopathic pulmonary fibrosis?
Progressive fibrosis of the interstitium without a known cause
91
Describe the clinical and radiological features of idiopathic pulmonary fibrosis (IPF).
Progressive dyspnoea, dry cough, end-inspiratory crackles, clubbing. Honeycombing (more in bases)
92
Describe the treatment of IPF.
- supplemental oxygen
- antifibrotics (e.g. pirfenidone) in selected patients
- Nintedanib, a tyrosine kinase inhibitor
- definitive management is a lung transplant
- otherwise, prognosis is poor at about 3 years after diagnosis
93
--- Occupational Disease ---
What is the main cause of pneumoconiosis?
Exposure to asbestos, coal mining, silicon etc.
94
What is Caplan's pneumoconiosis?
A person with rheumatoid arthritis exposed to mineral dust
95
How could silicosis and baritosis be differentiated on an Xray?
Eggshell calcification / barium snowstorm
96
Hypersensitivity Pneumonitis is typically caused by?
Hobbies/occupations other than mining (i.e. farmer's lung, bird fancier's lung)
97
How does a patient with hypersensitivity pneumonitis present?
Cough, myalgia, pyrexia, hypoxia, OR progressive dyspnoea, cough, history of exposure
98
How should hypersensitivity pneumonitis be treated?
Remove exposure, oral steroids if severe
99
--- Sarcoidosis ---
Which two clinical symptoms are by far the most common for sarcoidosis?
Bilateral lymphadenopathy (enlargement of hilar lymph nodes) and erythema nodosum (rash on ankles)
100
Describe the main clinical features of sarcoidosis.
SARCOID
Skin (erythema nodosum), arthritis, respiratory (bilateral hilar lymphadenopathy), cardiac (arrhythmia), ocular (uveitis), intracranial (stroke, haemorrhage), dysfunction of liver and kidney (hypercalcaemia, raised ACE)
101
Which other differential diagnoses should be looked for in sarcoidosis?
Lymphoma, carcinoma, fungal infection, TB
102
--- Congenital ---
What is a tracheoesophageal fistula?
Communication between the trachea and oesophagus
103
What is choanal atresia?
Blockage of the nose - bilateral is an emergency
104
What is the main symptom of croup? What causes it?
Stridor, viral laryngotrachealbronchitis
105
Define pulmonary hypertension.
mPAP of > 25 mmHg, when it is usually 12-20 mmHg
106
--- Cor Pulmonale ---
Which two tests can be used for pulmonary hypertension diagnostically? What is the difference?
Echocardiogram is an estimate. Catheter of the heart is gold standard (in Glasgow only)
107
What may cause pulmonary hypertension?
(Cardiac - mitral stenosis/regurg, left ventricular systolic dysfunction, myopathy), hypoxia, PE, immunosuppression, shunt etc
108
--- ARDS ---
Give the full name for, and the other three names for, ARDS.
Adult respiratory distress syndrome, alveolitis, shock lung, diffuse alveolar damage syndrome
109
Describe the pathology of ARDS.
Injury causes inflammation, oedema in alveoli causes exudate
110
What are the clinical signs of ARDS?
Rapid onset dyspnoea, low O2 sats, exudate on Xray
111
Which three treatments could be used for ARDS?
Mechanical ventilation, fluid management, prone positioning
112
--- Airway Obstruction ---
Which three key symptoms indicate anaphylaxis?
Stridor, respiratory failure, wheeze
113
How should acute anaphylaxis be treated?
IM adrenaline, IV antihistamine, corticosteroids, bronchodilators, O2
114
What is the main cause of stridor in newborns, children, and adults?
Epiglottitis/croup, inhaled foreign body, neoplasms
115
How may stridor be investigated/treated?
Laryngoscope (but beware in acute epiglottitis), bronchoscopy, cricothyroidotomy/tracheotomy, Heimlich, tumour removal
116
--- OSA ---
What are the main symptoms of obstructive sleep apnoea?
Excessive daytime sleepiness, personality change, cognitive impairment, danger in driving
117
Which two diagnostic tools are used for obstructive sleep apnoea?
Oximetry, and the Epworth sleepiness scale
118
How is obstructive sleep apnoea managed?
CPAP, mandibular advancement device, or corrective surgery in the young
119
Give the features of sarcoidosis.
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