Bronchiectasis

 

Bronchiectasis itself:

Cause

·     Cystic fibrosis

·     Tuberculosis

·     Pneumonia

·     Other congenital e.g. Kartagener’s

Pathophysiology

·     Small airway inflammation -> destruction, dilation, thickening

·     Impaired mucociliary clearance

·     Susceptible to infection, which is permanent once established

Presentation

History:

·     Chronic productive cough

·     Dyspnoea

·     Wheezing

·     Haemoptysis

·     Pleuritic chest pain

Investigations:

·     Spirometry: mixed obstructive and restrictive

·     Imaging: dilated, thick walled airways (multiple types)

·     Sputum: Ps.aeruginosa and friends

Severity

Summary:

·     Multiple scoring systems

·     Clinical + radiological + microbiological

·     Predictive of morbidity and mortality

FACED:

·     FEV1

·     Age

·     Colonisation (Ps.aeruginosa)

·     Extent (number of lobes affected)

·     Dyspnoea (mMRC)

Complications

·     Airway hyperreactivity

·     Mixed obstructive/restrictive disease

·     VQ mismatch -> hypoxaemia

·     Pulmonary hypertension, cor pulmonale

 

Cystic fibrosis:

Cause

·     Mutation in CFTR (many described)

Extrapulmonary disease

·     Anaemia

·     GORD

·     Chronic liver disease, portal hypertension, liver failure

·     Pancreatic insufficiency, diabetes

·     Infertility

Natural history

·     Median life expectancy ~40

·     Death due to cor pulmonale

Treatment

Bronchiectasis:

·     Chest physio (coughing, huffing, percussion)

·     Drugs (mucus thinners, CFTR modulators, bronchodilators)

·     Surgery (lung transplant)

Pancreatic insufficiency:

·     Exocrine: creon

·     Endocrine: insulin

 

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