respiratory Flashcards

(85 cards)

1
Q

How is a pneumothorax managed?

A

if no or minimal symptoms - conservative care regardless of pneumothorax (minimal symptoms is defined as no significant pain, breathlessness and no physiological compromise)

if they are symptomatic - assess for high risk characteristics

high risk characteristics:
haemodynamic compromise (suggesting a tension pneumothorax)
significant hypoxia
bilateral pneumothorax
underlying lung disease
≥ 50 years of age with significant smoking history
haemothorax

if not high risk characteristics are present - either conservative management, ambulatory device, needle aspiration

If high risk characteristics - chest drain

patients with a primary spontaneous pneumothorax that is managed conservatively should be reviewed every 2-4 days as an outpatient
patients with a secondary spontaneous pneumothorax that is managed conservatively should be monitored as an inpatient
if stable, follow-up in the outpatients department in 2-4 weeks

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2
Q

what is ambulatory care for pneumothorax ?

A

an example of an ambulatory device is the Rocketµ Pleural Vent„
it includes an 8FG catheter mounted on an 18G needle and a pigtail catheter to minimize the risk of occlusion
ambulatory devices typically have a one-way valve and vent to prevent air and fluid return to the pleural space while allowing for controlled escape of air and drainage of fluid
many devices also have an indication diaphragm that signals when the catheter tip enters the pleural space and continues to fluctuate with respiration, aiding in the assessment of pneumothorax resolution

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3
Q

when can you fly after a pneumothorax?

A

absolute contraindication, the CAA suggest patients may travel 2 weeks after successful drainage if there is no residual air. The British Thoracic Society used to recommend not travelling by air for a period of 6 weeks but this has now been changed to 1 week post check x-ray

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4
Q

when can you scuba dive post pneumothorax?

A

the BTS guidelines state: ‘Diving should be permanently avoided unless the patient has undergone bilateral surgical pleurectomy and has normal lung function and chest CT scan postoperatively.’

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5
Q

What is sarcoidosis?

A

a multisystem disorder of unknown aetiology characterised by non-caseasting granulomas.

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6
Q

what are the features of sarcoidosis?

A

acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia
insidious: dyspnoea, non-productive cough, malaise, weight loss
ocular: uveitis
skin: lupus pernio
hypercalcaemia: macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)

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7
Q

how is sarcoidosis investigated?

A

ACE levels have a sensitivity of 60% and specificity of 70% and are therefore not reliable in the diagnosis of sarcoidosis although they may have a role in monitoring disease activity.

Routine bloods may show hypercalcaemia (seen in 10% if patients) and a raised ESR

A chest x-ray may show the following changes:
stage 0 = normal
stage 1 = bilateral hilar lymphadenopathy (BHL)
stage 2 = BHL + interstitial infiltrates
stage 3 = diffuse interstitial infiltrates only
stage 4 = diffuse fibrosis

spirometry - restrictive defect
tissue biopsy - non-caseating granulomas

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8
Q

how is sarcoidosis managed?

A

Indications for steroids
patients with chest x-ray stage 2 or 3 disease who are symptomatic. Patients with asymptomatic and stable stage 2 or 3 disease who have only mildly abnormal lung function do not require treatment
hypercalcaemia
eye, heart or neuro involvement

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9
Q

what factors in sarcoidosis are associated with poor prognosis?

A

insidious onset, symptoms > 6 months
absence of erythema nodosum
extrapulmonary manifestations: e.g. lupus pernio, splenomegaly
CXR: stage III-IV features
black African or African-Caribbean ethnicity

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10
Q

how are pleural effusions classified?

A

Pleural effusions may be classified as being either a transudate or exudate according to the protein concentration.

Transudate < 30g/L protein

Exudate >30g/L proetein

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11
Q

what are causes of transudate pleural effusions?

A

heart failure (most common transudate cause)
hypoalbuminaemia
liver disease
nephrotic syndrome
malabsorption
hypothyroidism
Meigs’ syndrome

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12
Q

what are causes of exudate pleural effusions?

A

Infection - pneumonia, TB, subphrenic abscess
Connective tissue disease - RA, SLE
Neoplasia - lung Ca, mesothelioma, mets
pancreatitis
PE
Dressler’s syndrome
yellow nail syndrome

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13
Q

when should LTOT be offered to patients?

A

LTOT should be offered to patients with a pO2 of < 7.3 kPa or to those with a pO2 of 7.3 - 8 kPa and one of the following:
secondary polycythaemia
nocturnal hypoxaemia
peripheral oedema
pulmonary hypertension

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14
Q

what is the general management of COPD?

A

> smoking cessation advice: including offering nicotine replacement therapy, varenicline or bupropion
annual influenza vaccination
one-off pneumococcal vaccination
pulmonary rehabilitation to all people who view themselves as functionally disabled by COPD

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15
Q

what is the medical management of COPD?

A

1st line - SABA or SAMA
if they remain breathless or have exacerbations the next step is to determine if the patient has asthmatic features suggesting steroid responsiveness

No asthmatic features
add a long-acting beta2-agonist (LABA) + long-acting muscarinic antagonist (LAMA)
if already taking a SAMA, discontinue and switch to a SABA

Asthmatic features
LABA + inhaled corticosteroid (ICS)
if patients remain breathless or have exacerbations offer triple therapy i.e. LAMA + LABA + ICS
if already taking a SAMA, discontinue and switch to a SABA
NICE recommend the use of combined inhalers where possible

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16
Q

what features suggest a patient with COPD has asthmatic/steroid responsive features?

A

any previous, secure diagnosis of asthma or of atopy
a higher blood eosinophil count - note that NICE recommend a full blood count for all patients as part of the work-up
substantial variation in FEV1 over time (at least 400 ml)
substantial diurnal variation in peak expiratory flow (at least 20%)

Interestingly NICE do not recommend formal reversibility testing as one of the criteria. In the guidelines they state that ‘routine spirometric reversibility testing is not necessary as part of the diagnostic process or to plan initial therapy with bronchodilators or corticosteroids

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17
Q

when is theophylline used in COPD?

A

NICE only recommends theophylline after trials of short and long-acting bronchodilators or to people who cannot used inhaled therapy
the dose should be reduced if macrolide or fluoroquinolone antibiotics are co-prescribed

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18
Q

when is prophylactic abx therapy indicated in COPD ?

A

azithromycin prophylaxis is recommended in select patients
patients should not smoke, have optimised standard treatments and continue to have exacerbations
other prerequisites include a CT thorax (to exclude bronchiectasis) and sputum culture (to exclude atypical infections and tuberculosis)
LFTs and an ECG to exclude QT prolongation should also be done as azithromycin can prolong the QT interval

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19
Q

when should a rescue pack for COPD be provided to patients?

A

have had an exacerbation within the last year
understand how to take the medication, and are aware of associated risks and benefits
know to when to seek help and when to ask for replacements once medication has been used

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20
Q

when are PDE-4 inhibitors recommended in COPD?

A

Phosphodiesterase-4 (PDE-4) inhibitors

oral PDE-4 inhibitors such as roflumilast reduce the risk of COPD exacerbations in patients with severe COPD and a history of frequent COPD exacerbations

NICE recommend if:
the disease is severe, defined as a forced expiratory volume in 1 second (FEV1) after a bronchodilator of less than 50% of predicted normal, and
the person has had 2 or more exacerbations in the previous 12 months despite triple inhaled therapy with a long-acting muscarinic antagonist, a long-acting beta-2 agonist and an inhaled corticosteroid

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21
Q

which type of lung cancer is not necessarily associated with smoking?

A

Adenocarcinoma

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22
Q

what are the features of squamous cell lung Ca?

A

Squamous cell lung cancers are strongly associated with smoking
They can cavitate and sometimes appear as cavitating lesions on chest x-ray. In addition, they are associated with hypercalcemia.

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23
Q

what are the features of small cell lung cancer?

A

Small cell carcinomas account for about 20% of lung cancers. They are the most aggressive type of lung cancer and have usually metastasized by the time of diagnosis
The are also associated with hyponatraemia.

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24
Q

what are risk factors for lung cancer?

A

Smoking
increases risk of lung ca by a factor of 10

Other factors
asbestos - increases risk of lung ca by a factor of 5
arsenic
radon
nickel
chromate
aromatic hydrocarbon
cryptogenic fibrosing alveoli’s

Smoking and asbestos are synergistic, i.e. a smoker with asbestos exposure has a 10 * 5 = 50 times increased risk

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25
what is the mechanism of action of bupropion and what is it used for
Norepinephrine and dopamine reuptake inhibitor, and nicotinic antagonist. Bupropion is an atypical antidepressant and smoking cessation aid. should be started 1 to 2 weeks before the patients target stop date there is a small risk of seizures contraindicated in epilepsy, pregnancy and breast feeding having an eating disorder is a relative contraindication
26
what is the mechanism of action of Vareniciline and what is it used for ?
Nicotinic receptor partial agonist Varenicline works by partially activating the nicotinic acetylcholine receptor, reducing cravings for nicotine and withdrawal symptoms. should be started one week prior to the target stop date course of treatment is 12 weeks nausea is the most common adverse effect. Other common problems include headache, insomnia, abnormal dreams varenicline should be used with caution in patients with a history of depression or self-harm. There are ongoing studies looking at the risk of suicidal behaviour in patients taking varenicline contraindicated in pregnancy and breast feeding
27
what are predisopsing factors for OSA?
obesity macroglossia: acromegaly, hypothyroidism, amyloidosis large tonsils Marfan's syndrome
28
what are the symptoms of OSA?
daytime somnolence compensated respiratory acidosis hypertension
29
how do you diagnose OSA?
Assessment of sleepiness Epworth Sleepiness Scale - questionnaire completed by patient +/- partner Multiple Sleep Latency Test (MSLT) - measures the time to fall asleep in a dark room (using EEG criteria) Diagnostic tests sleep studies (polysomnography) - ranging from monitoring of pulse oximetry at night to full polysomnography where a wide variety of physiological factors are measured including EEG, respiratory airflow, thoraco-abdominal movement, snoring and pulse oximetry
30
how is OSA managed?
weight loss CPAP intraoral devices the DVLA should be informed if OSAHS is causing excessive daytime sleepiness
31
which investigations are indicated in COPD?
post-bronchodilator spirometry to demonstrate airflow obstruction: FEV1/FVC ratio less than 70% chest x-ray: hyperinflation, bullae, flat hemidiaphragm. Also important to exclude lung cancer full blood count: exclude secondary polycythaemia body mass index (BMI) calculation ** Measuring peak expiratory flow is of limited value in COPD, as it may underestimate the degree of airflow obstruction.
32
How is COPD severity categorised?
FEV1 (of predicted value) > 80% Stage 1 - Mild - symptoms should be present to diagnose COPD in these patients 50-79% Stage 2 - Moderate 30-49% Stage 3 - Severe < 30% Stage 4 - Very severe
33
what is the most common organism found in bronchiectasis?
Haemophilus influenzae (most common) other common organisms Pseudomonas aeruginosa Klebsiella spp. Streptococcus pneumoniae
34
how is bronchiectasis managed?
physical training (e.g. inspiratory muscle training) - has a good evidence base for patients with non-cystic fibrosis bronchiectasis postural drainage antibiotics for exacerbations + long-term rotating antibiotics in severe cases bronchodilators in selected cases immunisations surgery in selected cases (e.g. Localised disease)
35
what is bronchiectasis?
Bronchiectasis describes a permanent dilatation of the airways secondary to chronic infection or inflammation
36
what are the main indications for placing a chest tube in pleural infection ?
Patients with frankly purulent or turbid/cloudy pleural fluid on sampling should receive prompt pleural space chest tube drainage. The presence of organisms identified by Gram stain and/or culture from a non-purulent pleural fluid sample indicates that pleural infection is established and should lead to prompt chest tube drainage. Pleural fluid pH < 7.2 in patients with suspected pleural infection indicates a need for chest tube drainage.
37
pleural aspiration
as above, ultrasound is recommended to reduce the complication rate a 21G needle and 50ml syringe should be used fluid should be sent for pH, protein, lactate dehydrogenase (LDH), cytology and microbiology
38
what is light's criteria ?
Light's criteria was developed in 1972 to help distinguish between a transudate and an exudate. exudates have a protein level of >30 g/L, transudates have a protein level of <30 g/L if the protein level is between 25-35 g/L, Light's criteria should be applied. An exudate is likely if at least one of the following criteria are met: pleural fluid protein divided by serum protein >0.5 pleural fluid LDH divided by serum LDH >0.6 pleural fluid LDH more than two-thirds the upper limits of normal serum LDH
39
what do the following characteristics of pleural fluid suggest: - low glucose - raised amylase - heavy blood staining
low glucose: rheumatoid arthritis, tuberculosis raised amylase: pancreatitis, oesophageal perforation heavy blood staining: mesothelioma, pulmonary embolism, tuberculosis
40
what are pleural plaques?
Pleural plaques are benign and do not undergo malignant change. They, therefore don't require any follow-up. They are the most common form of asbestos-related lung disease and generally occur after a latent period of 20-40 years.
41
What are the features of asbestosis?
The severity of asbestosis is related to the length of exposure. This is in contrast to mesothelioma where even very limited exposure can cause disease. The latent period is typically 15-30 years. Asbestosis typically causes lower lobe fibrosis. Features dyspnoea and reduced exercise tolerance clubbing bilateral end-inspiratory crackles lung function tests show a restrictive pattern with reduced gas transfer It is treated conservatively - no interventions offer a significant benefit.
42
what is mesothelioma?
Mesothelioma is a malignant disease of the pleura. Crocidolite (blue) asbestos is the most dangerous form. Possible features progressive shortness-of-breath chest pain pleural effusion Patients are usually offered palliative chemotherapy and there is also a limited role for surgery and radiotherapy. Unfortunately, the prognosis is very poor, with a median survival from diagnosis of 8-14 months.
43
what is the most common form of cancer caused by asbestos ?
Whilst mesothelioma is in some ways synonymous with asbestos, lung cancer is actually the most common form of cancer associated with asbestos exposure. It also has a synergistic effect with cigarette smoke in terms of the increased risk. Therefore, smoking cessation is very important as the risk of lung cancer in smokers who have a history of asbestos exposure is very high.
44
what are the contraindication to surgery in patients with non-small cell lung cancer?
stage IIIb or IV (i.e. metastases present) FEV1 < 1.5 litres is considered a general cut-off point* malignant pleural effusion tumour near hilum vocal cord paralysis SVC obstruction
45
how is non-small cell lung cancer managed?
only 20% suitable for surgery mediastinoscopy performed prior to surgery as CT does not always show mediastinal lymph node involvement curative or palliative radiotherapy poor response to chemotherapy
46
what are the most common causes of COPD exacerbation?
bacteria Haemophilus influenzae (most common cause) Streptococcus pneumoniae Moraxella catarrhalis *if CXR showed pneumonia then step pneumonia would be the most common causative organism respiratory viruses account for around 30% of exacerbations human rhinovirus is the most important pathogen
47
how is exacerbation of COPD managed?
Increase the frequency of bronchodilator use and consider giving via a nebuliser give prednisolone 30 mg daily for 5 days it is common practice for all patients with an exacerbation of COPD to receive antibiotics. NICE do not support this approach. They recommend giving oral antibiotics 'if sputum is purulent or there are clinical signs of pneumonia' the BNF recommends one of the following oral antibiotics first-line: amoxicillin or clarithromycin or doxycycline.
48
when is admission required in exacerbation of COPD?
severe breathlessness acute confusion or impaired consciousness cyanosis oxygen saturation less than 90% on pulse oximetry. social reasons e.g. inability to cope at home (or living alone) significant comorbidity (such as cardiac disease or insulin-dependent diabetes)
49
when is NIV indicated in exacerbation of COPD?
typically used for COPD with respiratory acidosis pH 7.25-7.35 the BTS guidelines state that NIV can be used in patients who are more acidotic (i.e. pH < 7.25) but that a greater degree of monitoring is required (e.g. HDU) and a lower threshold for intubation and ventilation should be used bilevel positive airway pressure (BiPaP) is typically used with initial settings: Expiratory Positive Airway Pressure (EPAP): 4-5 cm H2O Inspiratory Positive Airway Pressure (IPAP): RCP advocate 10 cm H20 whilst BTS suggest 12-15 cm H2O
50
how is acute asthma classified?
moderate PEFR 50-75% best or predicted Speech normal RR < 25 / min Pulse < 110 bpm Severe PEFR 33 - 50% best or predicted Can't complete sentences RR > 25/min Pulse > 110 bpm life threatening PEFR < 33% best or predicted Oxygen sats < 92% 'Normal' pC02 (4.6-6.0 kPa) Silent chest, cyanosis or feeble respiratory effort Bradycardia, dysrhythmia or hypotension Exhaustion, confusion or coma 'Near-fatal asthma', is also recognised characterised by a raised pC02 and/or requiring mechanical ventilation with raised inflation pressures.
51
how is asthma attack managed?
admission - life threatening, severe if they do not respond to treatment, previous near-fatal asthma attack, pregnancy oxygen bronchidialation - SABA Corticosteroid - 40mg of prednisone daily Ipratropium bromide IV magnesium sulphate IV aminophylline intubation ECMO
52
what are the indications for NIV?
COPD with respiratory acidosis pH 7.25-7.35 type II respiratory failure secondary to chest wall deformity, neuromuscular disease or obstructive sleep apnoea cardiogenic pulmonary oedema unresponsive to CPAP weaning from tracheal intubation
53
what is catamenial pneumothorax ?
Catamenial pneumothorax is an uncommon and rather complex clinical condition which occurs due to endometrial tissues and is generally encountered in reproductive women. The mean ages are between 32-35 years old. In most of the cases, it involves the right side presenting with shortness of breath or difficulty breathing, fatigue, and dry cough. It can produce monthly episodes of chest pain which may radiate to the shoulder.
54
what is Meigs' syndrome?
Meigs' syndrome consists of the classical triad of ascites, pleural effusion, and benign ovarian tumour e.g. ovarian fibroma and usually resolves following resection of the tumour.
55
what is eosinophilic granulomatosis with polyangitis ?
Eosinophilic granulomatosis with polyangiitis (EGPA) is now the preferred term for Churg-Strauss syndrome. It is an ANCA associated small-medium vessel vasculitis. Features asthma blood eosinophilia (e.g. > 10%) paranasal sinusitis mononeuritis multiplex pANCA positive in 60% Leukotriene receptor antagonists may precipitate the disease.
56
what medication should be avoided in Churg-Strauss syndrome?
Leukotriene receptor antagonists may trigger eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) Montelukast
57
What are the steps of asthma management?
1 - Newly-diagnosed asthma Short-acting beta agonist (SABA) 2 - Not controlled on previous step OR Newly-diagnosed asthma with symptoms >= 3 / week or night-time waking SABA + low-dose inhaled corticosteroid (ICS) 3 - SABA + low-dose ICS + leukotriene receptor antagonist (LTRA) 4- SABA + low-dose ICS + long-acting beta agonist (LABA) Continue LTRA depending on patient's response to LTRA 5- SABA +/- LTRA Switch ICS/LABA for a maintenance and reliever therapy (MART), that includes a low-dose ICS 6- SABA +/- LTRA + medium-dose ICS MART OR consider changing back to a fixed-dose of a moderate-dose ICS and a separate LABA 7- SABA +/- LTRA + one of the following options: increase ICS to high-dose (only as part of a fixed-dose regime, not as a MART) a trial of an additional drug (for example, a long-acting muscarinic receptor antagonist or theophylline) seeking advice from a healthcare professional with expertise in asthma
57
what is MART?
Maintenance and reliever therapy (MART) a form of combined ICS and LABA treatment in which a single inhaler, containing both ICS and a fast-acting LABA, is used for both daily maintenance therapy and the relief of symptoms as required MART is only available for ICS and LABA combinations in which the LABA has a fast-acting component (for example, formoterol)
58
what are the investigations for lung cancer?
CXR (10% of CXR are reported as normal) CT Bronchoscopy PET scanning Bloods - may have raised platelets
59
what is Lofgren's syndrome ?
Lofgren's syndrome is an acute form sarcoidosis characterised by bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia. It typically occurs in young females and carries an excellent prognosis.
60
causes of upper zone pulmonary fibrosis?
hypersensitivity pneumonitis (also known as extrinsic allergic alveolitis) coal worker's pneumoconiosis/progressive massive fibrosis silicosis sarcoidosis ankylosing spondylitis (rare) histiocytosis tuberculosis radiation-induced pulmonary fibrosis may develop following radiotherapy for breast or lung cancer typically seen between 6 and 12 months following completion of radiotherapy course Acronym for causes of upper zone fibrosis: CHARTS C - Coal worker's pneumoconiosis H - Histiocytosis/ hypersensitivity pneumonitis A - Ankylosing spondylitis R - Radiation T - Tuberculosis S - Silicosis/sarcoidosis
61
causes of lung fibrosis predominately effecting the lower zones?
idiopathic pulmonary fibrosis most connective tissue disorders (except ankylosing spondylitis) e.g. SLE drug-induced: amiodarone, bleomycin, methotrexate asbestosis Causes of lower zone pulmonary fibrosis: Most connective tissue diseases (e.g. rheumatoid arthritis) Asbestosis Idiopathic pulmonary fibrosis Drugs (e.g. methotrexate)
62
what are the pulmonary manifestations of rheumatoid arthritis?
pulmonary fibrosis pleural effusion pulmonary nodules bronchiolitis obliterans complications of drug therapy e.g. methotrexate pneumonitis pleurisy Caplan's syndrome massive fibrotic nodules with occupational coal dust exposure infection (possibly atypical) secondary to immunosuppression
63
What is Brinchiololitis obliterates
progressive dyspnoea obstructive pattern on spirometry centrilobular nodules, bronchial wall thickening is seen on CT symptoms of dyspnoea, non-productive cough, expiratory wheeze and obstructive pattern on spirometry strongly suggests bronchiolitis obliterans. Obstructive pattern on spirometry is evidenced by decreased ratio of FEV1 and FVC.
64
what leads to a left shift of the oxygen dissociation curve?
Shifts to Left = Lower oxygen delivery HbF, methaemoglobin, carboxyhaemoglobin Low [H+] (alkali) Low pCO2 Low 2,3-DPG Low temperature
65
What leads to a right shift in the oxygen dissociation curve?
Raised [H+] (acidic) Raised pCO2 Raised 2,3-DPG Raised temperature
66
causes of raised total gas transfer?
Causes of a raised TLCO asthma pulmonary haemorrhage (e.g. granulomatosis with polyangiitis, Goodpasture's) left-to-right cardiac shunts polycythaemia hyperkinetic states male gender, exercise
67
causes of lower total gas transfer?
Causes of a lower TLCO pulmonary fibrosis pneumonia pulmonary emboli pulmonary oedema emphysema anaemia low cardiac output
68
indications for steroids in sarcoidosis?
patients with chest x-ray stage 2 or 3 disease who are symptomatic. Patients with asymptomatic and stable stage 2 or 3 disease who have only mildly abnormal lung function do not require treatment hypercalcaemia eye, heart or neuro involvement
69
The most common infective causes of COPD exacerbations are:
bacteria Haemophilus influenzae (most common cause) Streptococcus pneumoniae Moraxella catarrhalis respiratory viruses account for around 30% of exacerbations human rhinovirus is the most important pathogen
70
what would be seen on spirometry in obstructive lung disease?
FEV1 - significantly reduced FVC - reduced or normal FEV1% (FEV1/FVC) - reduced
71
what would be seen in spirometry of restrictive lung disease?
FEV1 - reduced FVC - significantly reduced FEV1% (FEV1/FVC) - normal or increased
72
Causes of obstructive lung disease?
Asthma COPD Bronchiectasis Bronchiolitis obliterans
73
causes of restrictive lung disease?
Pulmonary fibrosis Asbestosis Sarcoidosis Acute respiratory distress syndrome Infant respiratory distress syndrome Kyphoscoliosis e.g. ankylosing spondylitis Neuromuscular disorders Severe obesity
74
What is the best investigation to assess for possible compression of the upper airways?
Flow volume loop is the investigation of choice for upper airway compression
75
what is sarcoidosis?
Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas. It is more common in young adults and in people of African descent
76
what are the features of sarcoidosis?
Features acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia insidious: dyspnoea, non-productive cough, malaise, weight loss ocular: uveitis skin: lupus pernio hypercalcaemia: macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)
77
what is Lofgren's syndrome?
associated with sarcoidosis Lofgren's syndrome is an acute form of the disease characterised by bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia. It usually carries an excellent prognosis
78
what is Mikulicz syndrome?
In Mikulicz syndrome* there is enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis or lymphoma associated with sarcoidosis
79
What is Hereford's syndrome?
associated with sarcoidosis Heerfordt's syndrome (uveoparotid fever) there is parotid enlargement, fever and uveitis secondary to sarcoidosis
80
diet recommendations for cystic fibrosis?
high calorie diet, including high fat intake
81
management of CF?
egular (at least twice daily) chest physiotherapy and postural drainage. Parents are usually taught to do this. Deep breathing exercises are also useful high calorie diet, including high fat intake* patients with CF should try to minimise contact with each other to prevent cross infection with Burkholderia cepacia complex and Pseudomonas aeruginosa vitamin supplementation pancreatic enzyme supplements taken with meals lung transplantion chronic infection with Burkholderia cepacia is an important CF-specific contraindication to lung transplantation Lumacaftor/Ivacaftor (Orkambi) is used to treat cystic fibrosis patients who are homozygous for the delta F508 mutation lumacaftor increases the number of CFTR proteins that are transported to the cell surface ivacaftor is a potentiator of CFTR that is already at the cell surface, increasing the probability that the defective channel will be open and allow chloride ions to pass through the channel pore
82
what age group is lung carcinoid seen in ?
40-50 years
83
features of lung carcinoid?
typical age = 40-50 years smoking not risk factor slow growing: e.g. long history of cough, recurrent haemoptysis often centrally located and not seen on CXR 'cherry red ball' often seen on bronchoscopy carcinoid syndrome itself is rare (associated with liver metastases)
84