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Bronchiectasis (Examination (Chest (Coarse, wet crackles (Localised /…

- - - - Clubbing
      - Sputum pot
      - Small and young: CF
      - Cachexia
      - LNs
      - Tachypnoea
    - - RA: rheumatoid hands
      - Yellow nails
      - CF: young pt., nasal polyps
      - Hypogammaglobulinaemia: splenomegaly
      - IBD: abdominal scars
  - - - Localised / patchy: secondary to infection
      - Widespread: secondary to systemic disease
      - May change with cough
  - - - Cor pulmonale
      - Specific cause: e.g. RA hands
    - - Idiopathic pulmonary fibrosis
      - Chronic lung abscess
- - - - MDT: GP, pulmonologist, physio, dietician, immunologist
      - Physio: postural drainage, active cycle breathing, rehab
    - - Abx
        
        Exacerbations: e.g. cipro for 7-10d
        
        May use prophylactic azithromycin
      - Bronchodilators: nebulised β agonists
      - Treat underlying cause
        
        CF: DNAase, pancreatin (Creon), ADEK vitamins
        
        ABPA: Steroids
        
        Immune deficiency: IVIg
      - Vaccination: flu, pneumococcus
    - - May be indicated in severe localised disease or obstruction
  - - - PEFR
      - Dipstick: proteinuria – amyloidosis
    - - FBC: ACD
      - Serum Ig: may do provocative testing
      - Aspergillus: RAST, precipitins, ↑IgE, eosinophilia
      - RA: anti-CCP, RF, ANA
    - - CXR: tramlines and ring shadows (bunch of grapes)
      - HRCT
        
        Signet ring sign: thickened dilated bronchi +
        smaller adjacent vascular bundle
        
        Pools of mucus in saccular dilatations
    - - Bronchoscopy + mucosal biopsy
        
        Focal obstruction
        
        PCD
      - CF sweat test
      - Aspergillus skin prick testing
- - - - Physio: postural drainage, active cycle breathing
        
        Abx: acute infections and prophylaxis
        
        Mucloytics: DNAse
      - Segregate from other CF pts.: risk of transmission
      - Pseudomonas
      - Burkholderia
      - Vaccination: flu, pneumococcus
      - Advanced: heart-lung transplant
    - - Pancreatic enzyme replacement: pancreatin (Creon)
      - ADEK supplements
      - Insulin
    - - Rx of complications: e.g. DM
      - Fertility and genetic counselling
      - DEXA osteoporosis screen
- - - - X-linked recessive mutation of Bruton’s tyrosine kinase
        Failure to generate mature B cells → ↓ Ig
        Rx: pooled Ig → passive immunity
    - - Commonest primary immune deficiency: 1/5000
        Splenomegaly: 25-50%
        Normal IgM, ↓IgG, ↓IgA
    - - Normal Ig levels but inability to make specific antibodies