Bronchiectasis and Cystic fibrosis Flashcards Preview

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Flashcards in Bronchiectasis and Cystic fibrosis Deck (18)
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Define bronchiectasis

Persistent or progressive dilatation of thick-walled bronchi

Resulting in poor mucus clearance that predisposes to recurrent/chronic infection


Describe the pathogenesis of bronchiectasis

Vicious cycle of:

  • Neutrophilic inflammation: impairs immunity
  • Recurrent infection
  • Airway damage: further impairs mucociliary clearance


List three causes of bronchiectasis

  • Cystic fibrosis
  • Previous severe LRTI
    • eg. pneumonia; pertussis; TB; mycoplasma; influenza
  • Gastric or foreign body aspiration
  • Mucociliary clearance disorders; immunodeficiency
  • Endobronchial tumours
  • Allergic bronchopulmonary aspergillosis
  • RA; UC


What is primary ciliary dyskinesia

A rare autosomal recessive condition featuring chronic infections of the sinuses, middle ear, and lungs.

Associated syndromes:

  • Young: Bronchiectasis + sinusitis + azoospermia
  • Kartagener: Bronchiectasis + sinusitis + situs inversus


Name two common organisms in bronchiectasis

  • Haemophilus influenzae
  • Pseudomonas aeruginosa
  • Moraxella catarrhalis


What causative agent is particularly associated with bronchiectasis?

Pseudomonas aeruginosa

Worse prognosis due to limited oral abx available for gram -ve bacteria, and biofilm formation

Ciprofloxacin is first-line for P. aeruginosa, but commonly causes GI upsets and photo-sensitivity.


Give three presenting features of bronchiectasis

  • Persistent productive cough
  • Daily expectoration of large volumes of purulent sputum
  • Dyspnoea
  • Haemoptysis: usually sign of infection
  • Chest pain between exacerbations: typically non-pleuritic
  • Associated nasal symptoms
  • Absence of smoking history


List three signs of bronchiectasis

  • Coarse crackles: esp in lower zones
  • Large airway rhonchi: low-pitched wheeze
    • On inspiration and expiration, due to secretions
  • Wheeze: high-pitched wheeze heard
    • On expiration, due to bronchospasm or obstruction
  • Clubbing (infrequent): cystic fibrosis


Name three complications of bronchiectasis

  • Infective exacerbations; chronic bacterial colonisation
  • Haemoptysis: may be lift-threatening
  • Respiratory failure; congestive heart failure
  • Coughing:
    • Pneumothorax; rib fractures
    • Urinary incontinence; sexual problems
  • Reduced QoL; anxiety; depression
  • Chronic fatigue
  • Nutritional deficiency


Request four investigations for suspected bronchiectasis

  • High-resolution CT: Gold-standard
  • Spirometry: assess airway obstruction
  • CXR: exclude other causes
  • Sputum culture: focused antibiotic treatment
  • Establish cause:
    • Sweat test: cystic fibrosis
    • Aspergillus skin test
    • Nasal NO: primary ciliary dyskinesia
    • Gross antibody deficiency screening
    • Bronchoscopy


What radiological findings are indicative of bronchiectasis?

  • Signet ring sign:
    • bronchus >1.5x size of accompanying pulmonary artery
  • Lack of tapering
  • Bronchi visible within 1cm of pleural surface
  • CXR: Tram-lines (parallel line shadows)


Outline the management of bronchiectasis

  • Treat underlying cause if known
  • Influenzae vaccine
  • Chest physiotherapy: mucus clearance techniques
    • Pulmonary rehab if MRC dyspnoea score 3 or more
  • Salbutamol for wheeze/dyspnoea
    • Consider hypertonic saline
  • Abx for 2/52
    • Unknown organism: use previous cultures as guide
    • Empirical: Amoxicillin
      • Ciprofloxacin: first-line if P. aeruginosa


Describe the pathogenesis of cystic fibrosis

  • Autosomal recessive
  • Mutations of CFTR gene: abnormal CFTR chloride channel
  • Impaired chloride movement alters fluid movement
  • Dehydration and thickening of secretions


Outline the diagnostic criteria for cystic fibrosis

At least one of:

  • Typical clinical features
  • History of CF in a sibling
  • Positive newborn heel prick test

Plus, one of:

  • Sweat test: sweat NaCl >60mmol/L
  • Genotyping: two CFTR mutations


List five presenting features of cystic fibrosis in adults

 Pancreatic dysfunction:

  • Intestinal malabsorption (>90%); low BMI
  • CF-related diabetes
  • Steatorrhoea

Lung disease:

  • Recurrent respiratory infections
  • Chronic daily cough and sputum production
  • Bronchiectasis

Gastrointestinal (non-pancreatic):

  • Distal intestinal obstruction syndrome
  • CF-related liver disease (20%) and cirrhosis (2%)
  • Gallstones
  • Increased risk of GI malignancy

Other: Male infertility, osteoporosis, arthropathy


List two neonatal-specific features of cystic fibrosis

  • Meconium ileus (15-20%):
    • Intestinal obstruction
    • Bilious vomiting
    • Delayed (48hr) passing of meconium
  • Failure to thrive
  • Rectal prolapse

Neonates often have no signs or symptoms


List three signs seen with cystic fibrosis

  • Clubbing
  • Bilateral coarse crackles
  • Wheeze: mainly in upper lobes
  • Cyanosis

Neonates often have no signs or symptoms


List four pieces of lifestyle advice for cystic fibrosis

  • Smoking cessation; regular exercise
  • Avoid:
    • Other CF patients and infectious individuals
    • Jacuzzis: Pseudomonas aeruginosa
    • Stables, compost, rotting vegetation: Aspergillus
  • Clean and dry nebulisers thoroughly
  • Annual influenza vaccine
  • NaCl tablets in hot weather/vigorous exercise
  • Chest physiotherapy
  • High calorie diet
  • Vitamin ADEK and Creon supplements