CF, bronchiectasis and pneumonia Flashcards
(26 cards)
How common is CF?
Autosomal recessive, carrier frequency of 1 in 25, CF incidence 1 in 2000-2500, more than 2000 mutations
CF can affect many systems in the body- what are they?
Repeated chest infections, pancreatic insufficiency, male infertility etc.
What is the cause of cystic fibrosis?
Genes code for the CFTR protein- moves to cell surface- acts as a chloride channel
Mucociliary clearance being affected- bacteria stuck in the lungs as mucous isn’t removed
Cystic fibrosis- mutations divided into 7 classes- class 1 and 7 results in most severe, class 6 least severe disease
CF can causes bronchiectasis and pulmonary exacerbation what are they?
Bronchiectasis- dilated airways and signet ring sign
Pulmonary exacerbation- increased cough, increased sputum production, change in sputum colour, fever/ malaise (feeling of discomfort)
What is used to treat CF?
Therapy- nebulised antibiotic, oral/ IV antibiotics, DNase, Vitamin A, D, E, and K (fat soluble vitamins- as pancreas broken down in CF patients) , pancreatic enzymes and insulin
What are the symptoms of CF?
Symptoms- weight loss, increase cough- 60 ml of thick green sputum, haemoptysis, fatty stools and reduced vision
What clinical signs may be present for CF?
Clubbing, coarse crackles, wheeze, none
Why doe someone with CF lose weight?
Infection and pancreatic malabsorption- not taking enzymes
Why has Helen got diabetes?
Over 30% of adult have CF related diabetes (CFRD)- exocrine and endocrine part of pancreas becomes more damaged
What is causing Helen’s loss of vision?
Vitamin A deficiency- fat soluble vitamin
What is bronchiectasis?
- Chronic dilation of one or more bronchi
- Poor mucous clearance
- Predisposition to bacterial infection
What are the causes of bronchiectasis?
Detected in about 40% of cases
Post infection- measles, whooping cough, childhood pneumonia, TB
Immune deficiency- common variable hypogammaglobinaemia, late onset idiopathic Ig deficiency, selective immunoglobin deficiency, HIV, lung transplant
Allergic bronchopulmonary aspergillosis
Defective clearance- primary ciliary dyskinesia (Kartafener’s), cystic fibrosis
Toxic- aspiration
Mechanical- tumour, lymph node (middle lobe syndrome), foreign body
What are the symptoms of bronchiectasis?
Symptoms- persistent. Purulent sputum (non smokers), haemoptysis, breathless, wheeze, sinusitis and nasal symptoms, weight loss, pleurisy, nasal polyps (growth within your nose)
What are the clinical signs?
Clinical signs- clubbing, coarse crackles, wheeze, none
Clinical features- clubbing- increased curvature, loss of angle
What sort of pathogens may cause an exacerbation?
Staphylococcus aureus, streptococcus pmeumoniae, haemophilus influenzae, pseudomonas aeruginosa, aspergillus
Could the history of asthma and mucus plugs be relevant?
Allergic bronchopulmonary aspergillosis (ABPA)- affects people with asthma and CF
What investigation should be undertaken for bronchiectasis?
History, genetics/ sweat test/ faecal elastase (exocrine part of pancreas and is secreted shows how good the exocrine function is), ciliary beat frequency/ nasal NO, sperm analysis, autoantibody screen, sputum- MC&S, AFB, fungal, aspergillus antibodies, lung function, immunology review
What is the treatment for bronchiectasis?
Treatment- physiotherapy, mucolytics/ hypertonic saline, prompt antibiotics (10+ days), IV antibiotics as required, nebulised antibiotic, annual influenza vaccination
What is pneumonia?
An infection that inflames the air sacs in one or both lungs. The air sacs may fill with fluid or pus (purulent material), causing cough with phlegm or pus, fever, chills, and difficulty breathing.
What are the symptoms of pnuemonia?
Progressive breathlessness, fever with shivering attacks, left sided sharp chest pain, general malaise, lethargy
How is chest pain described?
Pleurisy- sharp and worse on inspiration
What are the signs of pneumonia?
Hot, flushed with peripheral dilation, dull to percussion/ increased breath sound on auscultation, crackles, pleural rub
What is hypotension?
Inflammatory stimulus (peripheral dialation), decreasing peripheral arterial resistance, cardiac output increases.
What is the treatment for bronchiectasis?
Antibiotics, oxygen, analgesia, fluids, physiotherapy, nutrition (infection- need a higher calorie intake to stop muscles wasting away) and stop smoking
