RESP: CF, Brochiectasis and OSA Flashcards
Causes of bronchiectasis?
Post infective- Whooping cough/TB
Immune deficiency- Hypogammaglobulinaemia
Genetic- CF, primary cilary kinesia, young’s syndrome, kartagener syndrome
Obstruction- foreign body, tumour
Toxic insult- gastric aspiration,
Secondary immune deficiency- HIV
Appearance of bronchiectasis on CT?
Signet rings
What is Young’s syndrome?
Triad of bronchiectasis, sinusitis, and reduced fertility
Bronchiectasis common organisms?
Haemophilius influenzae Non- tuberculous mycobacteria Fungi- aspergillus, candida Pseudomonas aeruginosa Moraxella catarrhalis Stenotrophomonas maltophilia
Management of bronchiectasis?
Treat underlying cause
Antibiotics/ IV for severe infection
Flu and Covid vaccines
Bronchodilators
Antibiotic prophylaxis for those with recurrent infections
Physiotherapy for mucus/airway clearance
Pulmonary Rehab – MRC Dyspnoea Score >3
What is the MRC dyspnoea score?
Used to assess the degree of baseline functional disability due to dyspnoea.
Breatheless with strenuous exercise-0
Breathless when walking up slight hill 1
Walk slower than people their age due to breathlessness/ has to stop walking due to breathlessness 2
Stop walking after 100m as am breathless 3
Too breathless to leave the house/ breathless when dressing/ undressing- 4
How to identify pt with acute exacerbation of bronchiectasis
A person with bronchiectasis with a deterioration in 3 or more key symptoms for at least 48 hours:
Cough
Sputum volume and / or consistency
Sputum purulence
Breathlessness and / or exercise tolerance
Fatigue
Haemoptysis
What can CF be diagnosed based on?
One or more characteristic phenotypic features OR history of CF in a sibling OR positive newborn screening test result
PLUS:
Increased sweat chloride concentration (>60mmol/L Na+, Cl-) in sweat test
OR
Identify of two CF mutations - genotyping
OR
Demonstration of abnormal nasal epithelial ion transport
Note: Quesbook says the following:
- Neonatal heel prick day between day 5 and day 9
- Sweat test: sweat sodium and chloride >60mmol/L
- Faecal elastase: this can provide evidence for abnormal pancreatic exocrine function.
- Genetic screening: This can identify CF mutations
Pathophysiology of CF?
Autosomal receive condition.
Mutation in CFTR gene (which usually regulates sodium channel called ENAC)
Mutation in CFTR gene
—> increases Na+ absorption
—> get abnormal Chloride secretion (as CFTR usually codes cAMP regulated chloride channel) in epithelial cells lining the airways,
—>less water secreted
—> OVERALL: get thicker mucus= impair cilia function
Presenting features of CF?
Presenting features:
- Meconium ileus:
is in newborns, bowel is blocked by sticky secretions. Have signs of intestinal obstruction soon after birth - billows vomiting, abdominal distension, delay in passing meconium.
- Intestinal malabsorption:
main cause is severe deficiency of pancreatic enzymes - Recurrent chest infections
- Newborn screening
Respiratory features of CF?
Chronic sinusitis Nasal polyps Cough Wheeze Haemoptysis Recurrent LRTI Bronchiectasis Pneumothorax Cor pulmonale Respiratory failure
Apart from respiratory system, CF causes multi-organ damage. What are features of damage in:
1. GI system?
- Pancreatic insufficiency, so have DM and/or steatorrhea
Cirrhosis
Portal HTN
Gallstones
Apart from respiratory system, CF causes multi-organ damage. What are features of damage in:
2.. Reproductive system?
- Male infertility
Apart from respiratory system, CF causes multi-organ damage. What are features of damage in:
3. MSK?
- Clubbing
Arthritis
Osteoporosis
Investigations in known CF patients (to monitor disease aseverity, assess symptoms etc)?
Sputum culture - chest infection Glucose tolerance test - assess DM Bloods - FBC, U+Es, coat screen, vitamins, blood sugar, bone profile Spirometry: obstructive defect Aspergillus skin prick test or serology
Imaging: CXR - bronchiectasis, hyperinflation
Abdominal ultrasound - Distal Intestinal Obstruction, liver cirrhosis, chronic pancreatitis
Lifestyle advice for pts with CF?
No smoking
Avoid other CF pts
Avoid friends/relatives with colds and infections
Avoid jaccuzis (pseudomonas)
Clean and dry nebulisers thoroughly
Avoid stables, compost or rotting veg - Aspergillus inhalation
Annual flu vaccine
Sodium chloride tablets in hot weather/exercise
Medical management for infective exacerbation of CF?
- Antibiotics, although for patients with recurrent chest infections prophylactic long-term antibiotics may be prescribed.
- Nebulised mucolytics (Dornase Alfa)
- Bronchodilators (Inhaled corticosteroids or B2-agonists)
Medical management for pancreatic insufficiency in CF?
Insulin replacement
Creon - exocrine enzymatic replacement
Vitamins - A, D, E, K
Medical management for worsening progressive lung disease in CF?
Oxygen therapy
Ventilation
Diuretics (especially if have cor pulmonale)
Management of CF?
Physio airway clearance Exercise Mucolytic treatment Pancreatic enzyme replacement therapy Fat soluble vitamin replacement Long term abx Optimise CF related DM - insulin therapy Novel CFTR modulators = Kaftrio Long term monitoring for CF related DM, CF related liver disease, osteoporosis.
What are you differentials for Haemoptysis : infection related?
Pneumonia
Tuberculosis
Bronchiectasis / CF
Cavitating lung lesion (often fungal
What are you differentials for Haemoptysis : Malignancy related?
Lung cancer
metastases
What are you differentials for Haemoptysis : Haemorrhage related?
Bronchial artery erosion
Vasculitis
Coagulopathy
