respiratory Flashcards
How is a pneumothorax managed?
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
what is ambulatory care for pneumothorax ?
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
when can you fly after a pneumothorax?
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
when can you scuba dive post pneumothorax?
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.’
What is sarcoidosis?
a multisystem disorder of unknown aetiology characterised by non-caseasting granulomas.
what are the features of sarcoidosis?
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)
how is sarcoidosis investigated?
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
how is sarcoidosis managed?
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
what factors in sarcoidosis are associated with poor prognosis?
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
how are pleural effusions classified?
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
what are causes of transudate pleural effusions?
heart failure (most common transudate cause)
hypoalbuminaemia
liver disease
nephrotic syndrome
malabsorption
hypothyroidism
Meigs’ syndrome
what are causes of exudate pleural effusions?
Infection - pneumonia, TB, subphrenic abscess
Connective tissue disease - RA, SLE
Neoplasia - lung Ca, mesothelioma, mets
pancreatitis
PE
Dressler’s syndrome
yellow nail syndrome
when should LTOT be offered to patients?
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
what is the general management of COPD?
> 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
what is the medical management of COPD?
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
what features suggest a patient with COPD has asthmatic/steroid responsive features?
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
when is theophylline used in COPD?
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
when is prophylactic abx therapy indicated in COPD ?
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
when should a rescue pack for COPD be provided to patients?
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
when are PDE-4 inhibitors recommended in COPD?
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
which type of lung cancer is not necessarily associated with smoking?
Adenocarcinoma
what are the features of squamous cell lung Ca?
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.
what are the features of small cell lung cancer?
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.
what are risk factors for lung cancer?
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
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
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
what are predisopsing factors for OSA?
obesity
macroglossia: acromegaly, hypothyroidism, amyloidosis
large tonsils
Marfan’s syndrome
what are the symptoms of OSA?
daytime somnolence
compensated respiratory acidosis
hypertension
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
how is OSA managed?
weight loss
CPAP
intraoral devices
the DVLA should be informed if OSAHS is causing excessive daytime sleepiness
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.
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
what is the most common organism found in bronchiectasis?
Haemophilus influenzae (most common)
other common organisms
Pseudomonas aeruginosa
Klebsiella spp.
Streptococcus pneumoniae
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)
what is bronchiectasis?
Bronchiectasis describes a permanent dilatation of the airways secondary to chronic infection or inflammation
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.
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
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
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
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.
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.
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.
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.
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
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
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
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.
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)
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
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.
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
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
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.
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.
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.
what medication should be avoided in Churg-Strauss syndrome?
Leukotriene receptor antagonists may trigger eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
Montelukast
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
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)
what are the investigations for lung cancer?
CXR (10% of CXR are reported as normal)
CT
Bronchoscopy
PET scanning
Bloods - may have raised platelets
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.
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
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)
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
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.
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
What leads to a right shift in the oxygen dissociation curve?
Raised [H+] (acidic)
Raised pCO2
Raised 2,3-DPG
Raised temperature
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
causes of lower total gas transfer?
Causes of a lower TLCO
pulmonary fibrosis
pneumonia
pulmonary emboli
pulmonary oedema
emphysema
anaemia
low cardiac output
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
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
what would be seen on spirometry in obstructive lung disease?
FEV1 - significantly reduced
FVC - reduced or normal
FEV1% (FEV1/FVC) - reduced
what would be seen in spirometry of restrictive lung disease?
FEV1 - reduced
FVC - significantly reduced
FEV1% (FEV1/FVC) - normal or increased
Causes of obstructive lung disease?
Asthma
COPD
Bronchiectasis
Bronchiolitis obliterans
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
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
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
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)
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
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
What is Hereford’s syndrome?
associated with sarcoidosis
Heerfordt’s syndrome (uveoparotid fever) there is parotid enlargement, fever and uveitis secondary to sarcoidosis
diet recommendations for cystic fibrosis?
high calorie diet, including high fat intake
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
what age group is lung carcinoid seen in ?
40-50 years
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)
