Bronchiectasis and CF Flashcards
(32 cards)
What is youngs syndrome?
Thickened mucosal secretions
Leads to azoospermia and recurrent sinobronchial infection
What is allergic bronchopulmonary aspergillosis?
Exaggerated response of the immune system to the fungi aspergillus
Most commonly occurs in asthma and CF patients
Causes airway inflammation and bronchiectasis
What is bronchiectasis?
Chronic infection of bronchi and bronchioles causes airways to become thickened, inflamed and irreversibly damaged
This leads to dilatation of airways and impaired mucociliary transport
This leads to frequent infections
Vicious circle
What are the main organisms causative of exacerbations of bronchiectasis?
H.influenzae
Strep.pneu
Staph.a
Pseudomonas.aer
Causes of bronchiectasis
Idiopathic in 50% of cases
Mechanical obstruction
Post-infective bronchial damage
Granulomatous disease (TB, sarcoidosis)
Diffuse disease of lung parenchyma (idiopathic pulmonary fibrosis)
Immune overresponse (abpa, post-transplant)
Immune deficiency
Mucociliary defect (CF, youngs, Kartageners)
Symptoms of bronchiectasis
Persistent cough Occasionally haemoptysis Originally yellow green sputum More severe - persistent copious purulent dark green sputum and persistent halitosis Breathlessness Chest pain, malaise, fever, weight loss
Signs of bronchiectasis
Clubbing
Coarse creps (usually at bases) which shift with coughing
Wheeze if asthma, COPD or ABPA
Signs on chest X-ray of bronchiectasis
Tram lines shadows (dilated Bronchi seen as parallel lines radiating from hilum to diaphragm)
and ring shadows - thickening of bronchial walls
Fibrosis, atelectasis, pneumonic consolidations
Other investigations in bronchiectasis
HRCT - extent of disease (best diagnostic test - dilated bronchi with thickened walls)
Sputum culture - specificity
Spirometry - obstructive problem
Bronchoscopy - to locate haemoptysis and exclude obstruction
CF sweat test
Aspergillus precipitans
Management of bronchiectasis
Postural drainage
Antibiotics
Bronchodilators in COPD, asthma, CF and ABPA
Inhaled corticosteroids (fluticasone) has been shown to reduce inflammation and volume of sputum but does not affect frequency of exacerbations or lung function
What is Kartageners syndrome?
Triad of primary ciliary dyskinesia, situs inversus (dextrocardia) and abnormal frontal sinuses
And infertility
What is cystic fibrosis?
Alteration in viscosity and tenacity of mucus produced at epithelial surfaces
Where does cf affect?
Lungs Pancreas Gastrointestinal tract Hepatobiliary Reproductive tracts
Prevalence of CF
1 in 2500 live births
1 in 25 carrier (autosomal dominant inheritance)
Pathology of CF
Mutation on chromosome 7 in CFTR (CF transmembrane conductance regulator)
3 base pairs at position 508 - loss of phenylalanine
No CFTR - chloride channel therefore decreased chloride conductance and increased resorption of sodium
Significance of pseudomonas aeruginosa in CF
Frequent infecting agent
Some evidence CFTR has role in normal uptake and processing of it
Respiratory effects of CF
Lungs born normal Frequent respiratory infections Increased mucosal viscosity Disrupted clearance Salt content inactivates defensins - natural antimicrobial peptides
Vicious cycle of infection and inflammation
Bronchiectasis, resp failure and death
Lung signs in CF
Coarse crackles
Clubbing
Cyanosis
Often chronic sinusitis
SOB and haemoptysis with bronchiectasis
What is a worrying sign in CF
Chest pain - could be a mucus plug - lead to pneumothorax
What happens eventually in respiratory with CF?
Respiratory failure and cor pulmonale (right heart failure due to pulmonary hypertension)
GI effects of CF
85% - pancreatic dysfunction - decreased enzymes - malabsorption - steatorrhea and failure to gain weight
Meconium ileus (MI equivalent syndrome in adult) - 10% of children born with it - constipation and mass, colicky pain
What else can CF patients get following pancreatic dysfunction
11% get diabetes
Other GI CF symptom
Gall stones and biliary cirrhosis due to abnormalities of biliary tract
Reproductive effects of CF
Nearly all male are infertile due to absent vas deferens
Female okay - near normal fertility - but some have cervical mucus abnormalities
