Clinical Respiratory Medicine Flashcards
embryonic stage of devlelopment
trachea and main bronchi appear
pseudoglandular stage of development
all conducting airways form
epithelial cells differentiate
canalicular stage of development
respiratory airways form
blood-gas barrier thins
surfactant appears
saccular stage of development
saccules (terminal sacs) appear
alveolar stage of development
saccules form alveoli
lung development into childhood
alveoli continue to multiply
stages of lung morphogenesis
- Embryonic
- Pseudo-glandular
- Canalicular
- Saccular
- Alveolar
stage of lung development at which extra-uterine life is possible
late canalicular / early saccular
Tracheo-bronchomalacia pathophysiology
Cartilage in airways doesn’t form properly in early development.
leads to “floppy” airways that are only held open by muscle
usually resolves naturally without treatment
Symptoms of tracheo-bronchomalacia
Barking cough
Dyspnoea on exertion
Recurrent /early croup
Frequent infections
Congenital Pulmonary Airway Malformation (CPAM)
aka Pulmonary adenomatoid malformation
overgrowth of terminal bronchioles creates areas of cystic tissue that cannot function normally
may resolve spontaneously in utero.
most cases are asymptomatic.
sometimes other areas of the lung are compressed (causing difficulty breathing) or other organs are compressed.
surgery may be required
Infant respiratory distress syndrome/ Surfactant Deficiency Disorder/ Hyaline Membrane Disease
surfactant deficiency
treated with surfactant replacement
fetal origins of COPD
in utero nicotine exposure
fetal infection
poor maternal nutrition
Low birth weight/ prematurity
pediatric origins of COPD
infection
growth
environmental pollutants
Remodelling
definition + examples
The alteration of structure following external influence
e.g lung hypoplasia caused by pre-natal nicotine exposure.
chronic inflammation in asthma causing airway narrowing
Specific features to be included in the clinical history of asthma
childhood asthma inhalers taken (+ doses) occupation (past and present) smoking - pack year history eczema hayfever atopic disease in family pets phycosocial aspects - stress
Specific features to be included in the clinical history of COPD
childhood asthma inhalers taken (+ doses) occupation (past and present) smoking - pack year history past respiratory diseases Ischaemic heart diease
assessing the severity of acute COPD
Uses FEV1/FVC
> 80% = mild
50 - 79% = moderate
30 - 49% = severe
<30% = very severe
assessing the severity of acute severe asthma
can be: moderate, severe, life threatening or near fatal. objectively measured using:- - ability to speak - heart rate - respiratory rate - peak expiratory flow - O2 saturation and PaO2
near fatal = raised PaCO2
nebulisers
used if a patient cannot use an inhaler
proven aetiological factors of asthma
Atopy - tendency for IgE response to allergens
Smoking
maternal smoking during pregnancy
occupational factors - spray paint, rodent urinary protein, grains, crustacean
putative aetiological factors for asthma
obesity diet reduced exposure to microbes pollution cleaning sprays
The British Thoracic Society’s “Stepwise” approach to management of asthma
There are different steps for different intensities of treatment
Patients should try to be on the lowest step on which they can maintain control of their asthma
This reduces cost and adverse effects
Epidemiology
The incidence, distribution and determinants of disease in a population
Atopy
a (genetic) predisposition toward developing type I hypersensitivity reactions (excessive IgE production)
Hyperplasia
increased proliferation
Emphysema pathophysiology
increase in size of air spaces (hyperinflation) due to destruction of elastin causing dilatation and destruction of alveolar cell walls
Airways collapse due to pressure in chest cavity on expiration, no elastin to hold them open
Air becomes trapped
Centriacinar emphysema
smoke particles get trapped in the airspaces of the bronchioles causing inflammation.
neutrophils make elastase which destroys elastin
Panacinar emphysema
a-1 antitrypsin (a protein which destroys elastase) deficiency.
leads to elastin destruction in bronchioles and alveoli
Periacinar (bullous) emphysema
alveoli expand to form large (>1cm) air spaces called bulla.
rupture can leak air into the pleural cavity causing pneumothorax
Clinical definition of chronic bronchitis
a cough lasting for at least 3 consecutive months of the year for at least 2 consecutive years
Morphological changes of large airways in chronic bronchitis
- mucous gland hyperplasia
- goblet cell hyperplasia
- some inflammation and fibrosis
morphological changes of small airways in chronic bronchitis
- goblet cells appear
- inflammation and fibrosis (in long standing disease)
cause of obstruction in chronic bronchitis
airways obstructed by thick mucous and fibrosis
Causes of hypoxaemia in COPD
- Airway obstruction
- loss of alveolar surface area for gas exchange
- reduced respiratory drive (central chemoreceptors get used to high [H+] in CSF
These all cause low ventilation
Hypoxic cor pulmonale
enlargement of the right ventricle of the heart.
because higher blood pressure is needed in the lungs due to vasoconstriction due to shunt
Asthma mechanism
Type I hypersensitivity in the airways.
IgE is produced in response to stress, chemicals cold, etc…
IgE triggers mast cell degranulation which causes:-
1. Bronchial smooth muscle contraction (immediate response)
2. Chronic inflammation of airways
This narrows the airways
Clinical definition of asthma
Chronic airflow obstruction causing WHEEZE which is VARIABLE and REVERSIBLE with asthma treatment
Symptoms of asthma
Wheeze
Dry cough
Dyspnoea (at rest)
Chest tightness
Investigations used for diagnosis of asthma
Spirometry: Low FEV1/FVC
Peak-flow monitoring: Variable obstruction
reversibility with bronchodilator/ oral corticosteroids
Skin prick test: proves atopy
Bronchial provocation: response to lower loses of bronchial constrictors (methacholine etc.)
Fractional exhaled NO: ↑ due to inflammation in airways
CXR: Hyperinflation
FBC: Eosinophillia = atopy
Definition of COPD
airway obstruction (chronic bronchitis) and hyperinflation (emphysema). Constant and not fully reversible
Symptoms of COPD
Breathlessness on exertion chronic cough with sputum recurrent chest infections wheeze/ chest tightness weight loss
Investigations used for diagnosis of COPD
Spirometry: low FEV1/FVC = fixed airflow obstruction
Reversibility: Minimal w/ bronchodilators and oral corticosteroids
CXR: Hyperinflated lungs, compressed diaphragm and heart
Full pulmonary function testing: ↑RV, ↑TLC and RV/TLC> 30% = emphysema
Blood gases: ↓PaO2, ↑PaCO2
Management strategies for COPD
smoking cessation!
vaccinations (increased risk of respiratory infection)
long term oxygen therapy
pulmonary rehabilitation (exercise, support, etc.)
Inhaled therapy
characteristics of obstructive lung diseases
airflow limitation
reduced peak expiratory flow rate
reduced FEV1
pathogenesis
the development of a disease from the causes and origin to the end stages
Pulmonary embolism deffinition
a thrombus (blood clot) formed in the venous system that embolises to the pulmonary arteries
pulmonary embolism symptoms
pleuritic chest pain
cough + haemoptysis
dyspnoea
MASSIVE PE:
syncope
cardiac arrest
pulmonary embolism investigations
Pre-test probability: Wells/ Geneva score
V/Q scan: Low perfusion
CT pulmonary angiogram: Shows occlusion of pulmonary artery
D-dimer: High
ECG: Tachycardia
Blood Gases: ↓PCO2 + Hypoxia
Pulmonary embolism management
Immediate oxygen for resuscitation
Anticoagulation:
Heparin, Warfarin, DOACs
Massive PE: Thrombolysis Pulmonary embolectomy
pulmonary infarction definition
local necrosis of lung tissue caused by obstruction of arterial blood (usually due to PE)
most common in people with underlying lung disease (e.g. COPD) as well tissue is well oxygenated from multiple sources
pulmonary infarction management
100% oxygen (for initial resuscitation)
mechanical ventilation
ultimately depends on cause
pulmonary hypertension definition
elevated blood pressure (>25mmHg) in pulmonary arteries
causes of pulmonary hypertension
Idiopathic Secondary to chronic respiratory disease Secondary to left heart disease Artery Occlusion (Chronic thromboembolic PH /CTEPH) Collagen vascular disease Portal hypertension Congenital HD (L -> R shunt) HIV
symptoms of pulmonary hypertension
exertional dyspnoea
exertional presyncope/ syncope
chest tightness
pulmonary hypertension investigations
Doppler echo: Estimates pulmonary artery systolic pressure
V/Q scan: CTEPH
CT(PA)/ CXR: Enlarged pulmonary arteries
Right heart catheterisation: Direct measurement of pulmonary artery pressure
management of pulmonary hypertension
Treatment of underlying condition Oxygen Anticoagulation Diuretics Lung/ Heart transplant Thromboendarterectomy (CTEPH)
PULMONARY VASODILATORS: CCBs
Endothelin receptor antagonists
Phosphodiesterase inhibitors
Prostacyclin
theophyllines
IV/ oral bronchodilator used in asthma
venography
x-ray of the veins using an IV contrast. used to identify DVT and PE
Pleural effusion definition
an abnormal collection of fluid in the pleural space
Transudates
caused by an imbalance of hydrostatic forces influencing formation and absorption of pleural fluid
usually bilateral
pleural fluid protein <30g/l
Exudates
caused by an increase in the permeability of the pleural surface and/or local capillaries.
usually unilateral
pleural fluid protein >30g/l
Causes of transudates
left ventricular failure
liver cirrhosis
hypoalbuminaemia
peritoneal dialysis
hypothyroidism
nephrotic syndrome
mitral stenosis
pulmonary embolism
constrictive pericarditis
ovarian hyperstimulation syndrome
meig’s syndrome
Causes of exudates
malignancy
parapneumonic
pulmonary embolism/ infarction rheumatoid arthritis autoimmune diseases benign asbestos effusion pancreatitis post myocardial infarction
yellow nail syndrome
Drugs: amiodarome, nitrofuratoin, phenytoin, methotrexate, carbamazapine……
Symptoms of pleural effusion
- asyptomatic (if small and accumulates slowly)
- increasing breathlessness
- pleuritic chest pain ( improves if inflammatory, worsens w/ malignancy)
- dull ache
- dry couch
- weight loss, malaise, fevers, night sweats…
Signs of pleural effusion
On affected side of chest:
- decreased expansion
- stony dullness to percussion
- bronchial breathing sounds
- decreased vocal resonance
clubbing increased JVP trachea moved away from large effusion cervical lymphadenopathy peripheral oedema
when to investigate pleural effusion
clinical picture is usually characteristic for bilateral transudate. investigate if…
-is unilateral
unusual features
fails to respond to appropriate treatment
Investigations for pleural effusion
- CXR (confirms presence)
- CT (differentiates malignant from benign)
- Pleural aspiration
- Pleural biopsy
- Thoracoscopy
Management of pleural effusion
Treatment of cause
Pleurodhesis (drain) Chemical pleurodhesis (e.g. talc slurry) Surgical pleurodhesis (performed at time of thoracoscopy)
Palliative (e.g. if due to malignancy) = repeated pleural aspiration
Malignant mesothelioma defnition
pleural malignanct caused by exposure to aasbestos (occurs 20-40yrs after exposure)
Malignant mesothelioma clinical presentations
breathlessness
chest wall pain
radiology = usually unilateral pleural thickening
Malignant mesothelioma treatment
pleuropneumonectomy (remova of entire lung and parietal pleura) if very fit
otherwise, palliative treatment
pleural fibrosis
caused by inflammation, surgical trauma, pleural effusion, asbestos etc.
pleura develops fibrous tissue and loses elastic properties which impairs lung function
pleural plaques
Benign plaques of the pleura caused by asbestos exposure. Can become calcified but rarely inhibit lung function.
almost always asymptomatic
Empyema
collection of pus in the pleural cavity which can eventually form fibrous scar tissue and inhibit lung function.
caused by pneumonia, trauma, surgery…
Symptoms: chest pain, fever, NO cough.
Diagnosed by pleural aspiration
appearance of tuberculous granuloma in TB
Rounded outline,
Epithelioid and langhan’s giant cells (transformed macrophages),
Lymphocytes, plasma cell and fibroblasts,
(Central caseous necrosis)
symptoms of primary TB
Usually asymptomatic,
Sometimes fever and malaise
Symptoms of post-primary TB
May be asymptomatic for months, Cough w/ sputum and haemoptysis, Pleuritic pain, Breathlessness, (Malaise, fever, weight loss, night sweats)
CXR of post-primary TB
Patchy, often bilateral shadowing in upper zones/ upper lower lobe,
Cavitation if advanced,
Calcification if chronic or healed
Anti-tuberculous drug therapy
For 2 months: Rifampicin, Isoniazid, Ethambutol, Pyrazinamide
Then For 4 months: Rifampicin, Isoniazid
DOT
Directly Observed Therapy
needed to make sure TB patients take medication as multi-drug resistant organisms pose a world issue
Public health duties of doctors managing TB
Legal requirement to report all cases to public health,
Should ensure patients take drugs,
TB contact tracing: must screen close household contacts and, if they are infected, casual contacts.
HIV screening in patients from areas with high HIV prevalence.
Pathogenesis of primary TB
Inhaled mycobacteria implant in distal airspaces,
No preceding exposure or immunity (usually in children),
Usually no symptoms
Can be cleared, become latent, or progress
Pathogenesis of post-primary TB
Caused by reactivation of latent mycobacteria or new re-infection from an outside source.
May progress to:
- TB caseous pneumonia (when lesions coalesce)
- Miliary TB
- Fibrocaseous TB
Fibrocaseous Tuberculosis
Large areas of fibrosis and caseous necrosis (cell death in which the dead tissue maintains a “cheese-like” appearance)
Miliary TB
TB that has disseminated throughout the body, characterised by minute tubercles
Caseating granuloma
A granuloma that shows caseous necrosis
Pharmacology of anti-tuberculous drugs
Rifampicin - inhibits mycobacterial RNA synthesis.
Isonazid - inhibits mycobacterial cell wall synthesis.
Ethambutol - inhibits mycobacterial cell wall synthesis
Pyrazinamide - inhibits mycobacterial growth.
CFTCR
Cystic Fibrosis Transmembrane Conductance Regulator
An active transport chloride channel found in many organs of the body
Mutations causing CF
There are ~30 mutations in the gene for CFTCR that cause cyctic fibrosis. Different mutations result in different phenotypes of the disease
Most common CFTCR gene mutation
Delta F508 - responsible for 2/3 cases worldwide.
A deletion of 3 nucleotides resulting in the loss of phenylalanine, producing a CFTCR protein with abnormal folding
Respiratory effects of CF
very thick secretions cause cillia collapse and excessive inflammation