Respiratory Flashcards
WHat is alpha 1 antitrypsin deficiency?
Common inherited condition caused by a lack of protease inhibitor (Pi) normally produced in the liver
Where is alpha 1 antitripsyin located?
Chromosome 14
Inheritance of alpha 1 antitrypsin deficiency
Autosomal recessive / co dominant fashion
WHat does heterozygous PiMZ mean?
If non smoker low risk of developing emphysema but may pass on A1AT gene to children
What does homozygous PiSS mean?
50% normal A1AT levels
What does homozygous PiZZ mean?
10% normal A1AT levels
Patients who manifest A1AT disease usually have what phenotype?
PIZZ
How does A1AT deficiency manifest?
Paraacinar emphysema, marked in lower lobes
Liver cirrhosis and HCC in adults
Cholestasis in children
Investigations for ALAT defiency
A1AT concentrations
Spirometry
What does spirometry show in ALAT deficiency?
Obstructive picture
Treatment of ALAT1 deficiency
No smoking
Supportive; bronchodilators and physio
IV aplha1-antitrypsin protein concentrations
Lung volume reduction surgery
Lung transplantation
Management of high altitude cerebral oedema (HACE)
Descent
Dexamethasone
Presentation of acute mountain sickness
Headache
Fatigue
Nausea
Prevention and treatment of acute mountain sickness
Gain altitude more than 500 meters
Acetozolamide
Presentation of HAPE
Classic pulmonary oedema features
Presentation of HACE
Headache
Ataxia
Papilloedema
Management of HAPE
Descent
Nifedipine
Dexamethasone
Acetozolamide
Oxygen if available
Investigations of suspected COPD
Post bronchodilator spirometry to demonstrate airflow obstruction (FEV1/FVC ratio < 70%)
CXR
FBC; exclude secondary polycythaemia
BMI calculation
CXR findings to support COPD
Hyperinflation
Bullae
Flat hemidiaphragm
Stage 1 COPD FEV1
FEV > 80%
Stage 2 (mod) COPD FEV1
FEV 50-79%
Stage 3 (severe) COPD FEV1
FEV 30-49%
Stage 4 (very severe) COPD FEV1
FEV < 30%
What is idiopathic pulmonary fibrosis?
Chronic lung condition characterised by progressive fibrosis of the interstitium of the lungs
Presentation of IPF
Progressive exertional dyspnoea
Bibasal fine end inspiratory creps on auscultation
Dry cough
Clubbing
Diagnosis of IPF
Spirometry
Impaired gas exchange; reduced transfer factor (TLCO)
CXR
High resolution CT
Serology
What spirometry is seen in IPF?
Restrictive picture (FEV1 normal/decreased, FVC decreased, FEV1/FVC increased)
CXR findings in IPF
Bilateral interstitial shadowing (ground class later progressing to honeycombing)
Serology in IPF
ANA + in 30%
RF + in 10%
Treatment of IPF
Pulmonary rehab
Oxygen
Lung transplant
Life expectancy IPF
3-4 years
Post bronchodilator FEV1/FVC found in any severity COPD
< 0.7
What is bronchiectasis?
Permenant dilatation of airways secondary to chronic infection or inflammation
Causes of bronchiectasis
Post infective; TB, measles, pertussis, pneumonia
CF
Bronchial obstruction; foriegn body, cancer
Immune deficiency; Selective IgA, hyppogammaglobuninaemia
Allergic bronchopulmonary aspergillosis
Ciliary dyskinetic syndrome (kartageners syndrome, youngs syndrome)
Yellow nail syndrome
CXR findings in bronchiectasis
Tramlines
CT findings in bronchiectasis
Tram tracks
Signet ring signs
What does a normal CO2 in a patient with severe acute asthma attack indicate?
Life threatning asthma
Classification of moderate asthma
PEFR 50-75% best or predicted
Speech normal
RR < 25 / min
HR < 100bpm
Classification of severe asthma
PEFR 33-50%
Cant complete sentences
RR > 25
HR > 100bpm
Classification of life threatning asthma
PEFR < 33 % best or predicted
O2 sats < 92%
Normal PCO2
Silent chest
Cyanosis
Feeble respiratory effort
Bradycardia
Dysrhytmia
Hypotension
Exhaustion
Confusion
Coma
Treatment of acute asthma exacerbation
O2 therapy if needed
SABA
Prednisolone PO
Ipratropium bromide neb
IV magnesium sulphate
Criteria for admission in acute asthma exacerbation
Life threatning asthma
Previous near fatal asthma attack
Pregnancy
Presentation at night
Attack occurring despite using corticosteriod
Do pleural plaques undergo malignant change?
No
Do pleural plaques require follow up?
No
What is the most common form of asbestos related lung disease?
Pleural plaques
What is the severity of asbestosis related to?
Length of exposure
What does asbestosis tend to cause?
Lower lobe fibrosis
Presentation of asbestosis
SOB
Reduced exercise tolerance
Clubbing
Bilateral end inspiratory crackles
What do lung function tests show in asbestosis?
Restrictive pattern with reduced gas transfer
Management of asbestosis
Conservative
What is a mesothelioma?
Malignant disease of the pleura
What is the most dangerous form of asbestos?
Crocidolite (blue)
Presentation of mesothelioma
Progressive SOB
Chest pain
Pleural effusion
Prognosis of mesothelioma
Very poor, 8-14 months
What is the most common form of cancer associated with asbestos exposure?
Lung cancer
What is extrinsic allergic alveolitis assosiated with?
Upper/mid zone lung fibrosis
What is extrinsic allergic alveolitis?
A condition caused by hypersensitivity induced lung damage due to a variety of inhaled organic particles.
Examples of extrinsic allergic alveolitis
Bird fanciers lung; avian proteins from bird droppings
Farmers lungs
Malt workers
Mushroom workers
Presentation of extrinsic allergic alveolitis
SOB
Dry cough
Fever
Lethargy
Productive cough (long term)
Anorexia
Investigations of extrinsic allergic alveolitis
Imaging; upper/mid zone fibrosis
Bronchoalveolar lavage; lymphocytosis
Serologic assays for specific IgG antibodies
NO eosinophilia
Management of extrinsic allergic alveolitis
Avoid precipitating factors
Oral glucocorticoids
Smoking cessation drugs
NRT (nicoteine replacement therapy)
Varenciline
Bupropion
S/Es of NRT
N/V
Headache
Flu like symptoms
S/Es varenciline
Nausea
Headache
Imsomnia
Abnormal dreams
Used in caution with patients with hx of depression or self harm
Contraindications of varenciline
Pregnancy
Breast feeding
What is there a small risk of when using bupropion?
Seizures
Contraindications of bupropion
Epilepsy
Pregnancy
Breast feeding
Eating disorder is a relative contraindication
Treatment of smoking cessation in pregnant women
CBT
Motivational interviewing
Structured self help
Can use NRT - mixed evidence and have to remove patches before going to bed
Is performing serial peak flows in someone with acute life threatning asthma helpful?
No
What is near fatal asthma characterised by?
Raised PCO2
Requiring mechanical ventilation with raised inflation pressures
What does a pH of < 7.35 represent in acute asthma?
Carbon dioxide retention in a tiring patient
Admission criteria for acute asthma
Life threatning asthma
Failing to respond to initial treatment
Previous near fatal asthma
Pregnancy
An attack occuring despite already using oral corticosteriods
Presentation at night
Management of acute asthma attack
O2 if hypoxic
High dose inhaled SABA (Life threatning - nebulised is recommended)
Corticosteriod (40mg PO pred)
What should be given if initital management for asthma does not work?
Nebulised ipratropium bromide
IV mag sulphate
IV aminophyline (senior staff)
Criteria for discharge post admission for asthma
Stable on their discharge medication (i.e. no oxygen or nebs) for 12-24 hrs
Inhaler technique checked or recorded
PEF >75% best or predicted
OP treatment for newly diagnosed asthma
SABA
OP treatment for asthma which isnt controlled by SABA alone or newly diagnosed asthma with symptoms >3 times / week or night time waking
SABA + low dose ICS
OP treatment for asthma after SABA + ICS
Add leukotrine receptor antagonist (LTRA)
OP treatment for asthma after SABA+ICS+LTRA
SABA + low dose ICS + LABA
Continue LTRA depending on patients response
OP Tx for asthma after SABA + ICS + LABA
SABA +/- LTRA
Switch ICS/LABA for a maintenance and reliever therapy (MART) which includes a low dose ICS
OP Tx asthma after SABA + low dose MART
SABA +/- LRTA + medium dose ICS MART
OR change back to a fixed dose of moderate dose ICS and a seperate LABA
OP Tx of asthma after SABA +/- LRTA and ICS moderate dose
One of the following options
- Increase ICS to high dose
- A trial of an additional drug (e.g. long acting anti-muscarinic receptor antagonist or theophylline)
- expert therapy
