Bronchiestasis, CF, Pneumonia Flashcards
What is bronchiectasis?
Disease with chronic and irreversible dilation of 1 or more bronchi so lungs cannot expel air properly. Inflammation permanently destroys elastic-like tissues and muscles surrounding bronchi.
Airflow obstruction and impaired clearance of secretions
What can bronchiectasis result in?
Poor mucus clearance so predisposition to bacteria
What can be seen on a chest scan for bronchiectasis?
- Dilated airways - Bronchus smaller than artery - Signet ring sign
Around 1/3 of cases of bronchiestasis in adults are associated with a severe lung infection in childhood. What are these childhood infections?
- Whooping cough 2. TB 3. Measles 4. Severe pneumonia
Some cases of bronchiestasis occur due to immunodeficiency. What are these immunodeficiencies?
- Common variable hypogammaglobulinaemia 2. Late onset idiopathic Ig deficiency 3. Selective immunoglobulin deficiency Immunodeficiency can be acquired after HIV
Some people with bronchiestasis develop ABPA. What is ABPA?
A complication of an allergic condition. People with ABPA have an allergy to a type of fungi known as aspergillus. If they breathe in fungal spores, it can trigger allergic reaction and persistent inflammation, which can progress to bronchiestasis
How can CF lead to bronchiestasis?
Mucus becomes clogged in lungs which is ideal environment for bacterial infection, leading to bronchiestasis
What is primary ciliary dyskinaesia (Kartagener’s)? How can it lead to bronchiestasis?
Problem with cilia due to inheriting faulty gene –> defective clearance of mucus
How can aspiration lead to bronchiestasis?
Stomach contents accidentally passes into lungs instead of down gastrointestinal tract –> inflammation and infection
What are the conditions that cause inflammation in other areas of the body that can be associated with bronchiestasis?
- Rheumatoid arthritis 2. Sjogren’s syndrome 3. Crohn’s disease 4. Ulcerative colitis 5. IBD 6. Coeliacs 7. Yellow nail syndrome 8. Alpha 1 antitrypsin deficiency
What are symptoms of bronchiestasis?
- Persistent purulent sputum (non smokers) - Haemoptysis - Breathless - Wheeze - Sinusitis and nasal symptoms - Weight loss - Pleurisy - Nasal polyps
What clinical signs are likely to be present on examination of bronchiestasis?
- Clubbing - Coarse crackles - Wheeze - None
What is clubbing?
Tissue at base of the nails is thickened and the normal angle between the base and adjacent ski is obliterated
What pathogens may cause an exacerbation of bronchiestasis?
- Staphylococcus aureus - Streptococcus pmeumoniae - Haemophilus influenzae - Pseudomonas aeruginosa - Aspergillus
What investigations should be taken for bronchiestasis?
- History - Genetics - Faecal elastase - Cilia beat frequency - Autoantibody screen - Sputum - Lung function - Aspergillus antibodies
Is bronchiestasis obstructive or restrictive?
Obstructive
What is cystic fibrosis?
Inherited (autosomal recessive) condition that causes sticky mucus to build up in lungs and digestive system. Lungs get increasingly damaged over time (cysts and fibrosis)
How common is CF?
Carrier frequency of 1 in 25 CF incidence 1 2000-2500 More than 2000 mutations
What is CF caused by?
Mutation in gene CFTR (found on chromosome 7) Most common mutation is Phe508del (deletion of 3 nucleotides that results in loss of phenylalanine at 508th position on protein). F508 deletion.
Ciliated airway epithelial cells in patient have mutated protein which leads to airway dehydration and abnormally viscous mucus production.
What are symptoms of CF?
- Repeated chest infections - Wheezing, coughing, shortness of breath, damage to airways (bronchiestasis) - Jaundice - Diarrhoea, constipation - Difficulty putting on weight and growing (don’t absorb nutrients properly as mucus clogs pancreas)
- Haemoptysis
- Reduced vision
What else can CF lead to?
- Diabetes (over 30% of adults have CF related diabetes) - Osteoporosis - Infertility in males - Liver disease
What is the product of the CFTR protein?
A chloride ion channel important in creating sweat, digestive juices and mucus
- Facilitates movement of chloride from swaet fuct into cytoplasm
What does mutation in CFTR result in?
Excess chlorine in sweat which prevents sodium reabsorption by epithelial sodium channels and the combination of Na and Cl creates the salt which is lost in high amounts in sweat –> lost salt forms basis of sweat test
Water follows Na so depth of airway lining fluid will be depleted (airway dehydration) and cilia will be left in the mucous layer
Gene is required to regulate the hydration of sweat, digestive juices, airways and mucus
How can CF be diagnosed?
- Pancreatic function –> faecal elastase
- CT chesst, US liver and pancreas
- Sperm (obtructive azoospermia)
Is CF obstructive or restrictive?
Obstructive
What is the average survival age of those with CF?
Around 35-40
How can CF be diagnosed?
- Newborn babies are screened
- Sweat test (measure salt which will be high in CF)
- Genetic test (check for faulty gene)
What are chances of child having CF if both parents are carriers?
1/4 their child will be normal
1/2 their child will inherit faulty gene from one parent and be a carrier
1/4 their child will inherit faulty gene from both parents and have CF
What are chances of child having CF if one parent is a carrier and one parent has CF?
1/2 their child will be carrier
1/2 their child will have CF
What clinical signs may be present in CF?
- Clubbing
- Coarse crackles
- Wheeze
- None
What is reduced vision associated with CF caused by?
Vitamin A deficiency (fat soluble vitamin)
What are Class II / Class III mutations responsible for?
Class II –> Protein Processing Mutations (e.g. F508del)
Class II –> Gating Mutations
How does the drug ivacaftor (Kalydeco) help CF?
Class III - CFTR potentiator (VX770)
Forcing the gate on the CFTR channel to stay open so chloride can move through channel
What are potentiators / correctors in regards to CF treatment?
Potentiators –> Increase channel activity of CFTR protein located at cell surface
Correctors –> Increase delivery and amount of functional CFTR protein to the cell surface
How are class II mutations treated?
The drug combination lumacaftor/ivacaftor (Orkambi®) works by enabling CFTR protein with an F508del mutation to fold in a more correct shape, and then activates the protein to allow more chloride to pass through. Although this drug combination is not a perfect fix, it helps the mutant CFTR protein to move some chloride. This movement of chloride reduces the symptoms of CF.
What is pneumonia?
An acute lower respiratory infection associated with recently developed radiological signs. Causes swelling of tissue in one of both lungs. Alevolar sacs become inflamed and fill up with liquid. Caused by bacterial infection
What are symptoms of pneumonia?
- Progressive breathlessness
- Fever with shivering attacks
- Left sided sharp chest pain
- General malaise / lethargy
- Cough and chest pain (sharp and worse on inspiration)
- Rapid heartbeat
- High temperature
How can pneumonia be diagnosed?
- Listen to back –> crackles, rattles
- Tapping on chest –> dull sound as lungs filled with fluid
How can pneumonia and hypotension be associated?
- Inflammatory stimulus
- Decreasing peripheral arterial resistance
- Cardiac output increases
What common organisms can cause pneumonia?
- Streptococcus pneumoniae
- Chlamydia pneumoniae
- Mycoplasma pneumoniae
- Staphylococcus aureus
- Influenza
How can pneumonia be treated?
- Antibiotics
- Drain
- Pleural aspiration
What type of lung disease is bronchiestasis?
Obstructive lung disease (along with emphysema, bronchitis and CF)
Physiological abnormalities of bronchi in bronchiectasis?
Bronchi are dilated, inflamed, and easily collapsible, resulting in airflow obstruction and impaired clearance of secretions
Causes of bronchiestasis?
- Prior infections (Bordetella pertussis, measles, bacterial pneumonia)
- Immunodeficiencies
- Other acquired causes: e.g. tuberculosis, connective tissue diseases, allergic bronchopulmonary aspergillosis; and foreign body aspiration
- Other congenital causes; e.g. ciliary dyskinesia including Kartagener syndrome, Alpha-1 antitrypsin deficiency
What are congential causes of bronchiectasis?
Ciliary dyskinesia including Kartagener syndrome, Alpha-1 antitrypsin deficiency
Symptoms of bronchiecstasis
Dyspnoea (breathlessness)
Chronic sputum production which is often green / yellow in colour
Repeated chest infections
Halitosis (bad breath)
Clinical signs of bronchiectasis?
- Coarse crepitations heard with stethoscope over affected area of lung
- Hypoxaemia (low blood oxygen)
- Hypercapnia (high blood CO2)
High resolution CT scan result for bronchiectasis?
Dilated airways with ring shadows ‘signet ring sing)
Obstructive pattern on spriometry
Blood gases test results for bronchiectasis?
Low O2 and high CO2
Medical intervention for bronchiectasis?
- Controlling infections and bronchial secretions
- Relieving airway obstructions
- Prompt antibiotic therapy for pulmonary exacerbations
- In a small number of cases where there is localised bronchiectasis surgery to affected lung can be undertaken Removal of airway secretions with chest physiotherapy such as postural drainage.
- Use of nebulised antibiotics such as colomycin, tobramycin to reduce pulmonary exacerbations in patients colonised with Pseudomonas Aeruginosa
- Maintain body weight with nutritional support is key.
1ary/2ary prevention for bronchiectasis?
- Children should be immunized against measles, pertussis and other acute respiratory infections of childhood
- Adult vaccination (especially against pneumonia and influenza)
- All patients should avoid smoking
What anatomical structures does CF affect?
Airway epithelial cells that line respiratory tract
Causes multi-system disorder e.g. lungs, sweat glands, pancreas and bowel
Why are males with CF usually infertile?
Congenital absence of vas deferens
How is CF related diabetes caused?
Loss of Islets of Langerhans cells in the pancreas which are responsible for making insulin (CFTR regulated insulin secretion)
Symptoms of CF?
Coughing / Sputum production / Pyrexia
Shortness of breath
Chronic lung infection
Repeated chest exacerbations
Abnormal bowel motions
Clinical signs of CF?
Salty skin, / Poor growth / Poor weight gain
Poor absorption of nutrients through the gastrointestinal tract including fat soluble
vitamins
Coughing up blood (haemoptysis)
Finger clubbing / Cyanosis
Abnormal test results for CF?
Before or at birth through genetic screening
Sweat test in early childhood
Obstructive lung function
Sputum cultures positive for Staphylococcus, Haemophilus and Pseudomonas
aeruginosa
Abnormal pancreatic function
Azoospermia (no sperm)
Difficulties getting enough oxygen to the body (hypoxia)
Medical treatment for CF?
Specialist multidisciplinary centres
Physiotherapy / Nutrition
Treat chronic and acute infections
Ultimately, lung transplantation is often necessary
What anatomical structures are affected by pneumonia?
- Parenchyma of lung (alveoli) –> alveolar filling with fluid (consolidation and exudation)
- Bronchial tree –> bronchial obstruction due to tumour or plugging by secretions
What is pneumonia?
Inflammatory condition of lung including inflammation of bronchi and alveoli. Pneumonia fills alveoli with fluid and stops O2 from reaching bloodstream
What is most common pneumonia?
Bacterial bronchopneumonia
What can cause aspiration pneumonia?
Vomiting when semi-conscious and with unprotected airway
Symptoms of pneumonia?
Productive cough
Fever / shaking (rigors) / chills
Unilateral chest pain aggravated by breathing (pleuritic) – “sharp
stabbing”
Difficulty breathing (dyspnoea)
Headache / confusion / unsteadiness
Clinical signs of pneumonia?
High temperature
Green or brown sputum
Tachypnoea (fast rate of breathing)
Tachycardia (high heart rate)
Hypotension (low blood pressure)
Hypoxia (low oxygen) and cyanosis (blue appearance) if severe.
Bronchial breathing (harsh sounding breath sounds) and crackles heard
with a stethoscope
Chest X-ray of pneumonia?
Consolidation (white wedge appearance outlining lobe of lung)
Blood/sputum culture present in pneumonia?
E.g. Streptococcus pneumoniae
What are some atypical pneumonias?
Mycoplasma pneumoniae, Chlamydia pneumoniae
Medical treatment for pneumonia?
Oxygen
Fluids (intravenous if low blood pressure)
Bacterial pneumonia is treated with antibiotics (e.g. penicillin)
If severe - assisted ventilation may be required
1ary/2ary prevention for pneumonia?
Leading cause of death among the young, the old, and the chronically ill.
Vaccination is available for pneumococcal pneumonia and flu
What are coarse crackles caused by?
Coarse crackles are louder, more low pitched and longer lasting. They indicate excessive fluid on the lungs which could be caused by aspiration, pulmonary oedema from chronic heart disease, chronic bronchitis, pneumonia.
What is the faecal elastase test?
Elastase 1 is a protease synthesised by pancreatic acinar cells and secreted into the duodenum. It is not degraded during transit and is concentrated in the faeces.
This test measures the amount of elastase in stool (faeces) to help evaluate whether a person’s pancreas is functioning properly.
