13 - Bronchiectasis and Cystic Fibrosis Flashcards Preview

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Flashcards in 13 - Bronchiectasis and Cystic Fibrosis Deck (19)
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1
Q

What is bronchiectasis?

A

Chronic irreversible dilatation of one or more bronchi. Bronchi has poor mucus clearance and there is a predisposition to recurrent or chronic bacterial infection

(Aetiology of chronic inflammation damaging elastic and muscular walls)

2
Q

What is the gold standard diagnostic test for bronchiectasis and what would the result of this test be?

A

- High resolution CT

  • Bronchi bigger than adjacent vessels (pulmonary artery so signet ring sign)
  • Bronchial wall thickening (tram tracks)
  • Take a good history, e.g TB or infections as a kid?
3
Q

What are some of the clinical symptoms of bronchiectasis?

A
  • Chronic cough and daily sputum production
  • Breathlessness on exertion
  • Intermittent haemophysis
  • Nasal symptoms
  • Chest pain
  • Fatigue

Key is history as symptoms are vague

4
Q

What are some clinicial signs of bronchiectasis?

A
  • Hypoxaemia
  • Fever
  • Haemoptysis
  • Fine crackles (rales - popping alveoli)
  • Rhonchi (low pitched like snoring)
  • High pitch inspiratory wheeze
  • Crackles and wheezes
  • Weight loss
  • Clubbing fingers
5
Q

What are some causes of bronchiectasis?

A

- Post infective: whooping cough and TB

- Immune deficiency: hypergammaglobulin

- Genetic/Mucociliary clearance defects: CF, primary ciliary dyskinesia, Young’s syndrome, Kartagener syndrome

- Obstruction: foreign body, tumour, extrinsic lymph node

- Idiopathic

6
Q

What is Young and Kartagener syndrome?

A

Youngs: triad of sinusistis, bronchiectasis and reduced fertility (obstructive azospermia)

Kartagener: triad of sinusitis, bronchiectasis and situs invertus

7
Q

What are the common organisms in bronchiectasis?

A
  • H.Influenzae
  • Pseudomonas aeruginosa
  • Moraxella atarrhalis
  • Strenotrophomonas maltophilia
  • Fungi: aspergillus and candida
  • Non tuberculosis mycobacteria
  • Less common = Staph Aureus
8
Q

What causes a viscious cycle of bronchiectasis and why do we need to catch it early?

A
  • Bronchial dilation leads to mucus accumulation so impaired mucus clearance and increased risk of infection
  • Infection leads to inflammation and loss of elastic fibres and bronchial smooth muscle so more dilatation
  • Need to catch early to stop spread over whole lung as irreversible
9
Q

Is bronchiectasis restrictive or obstructive?

A
  • Obstructive so will see when doing lung function tests so can monitor progression
10
Q

How can you tell the difference between chronic bronchitis and bronchiectasis?

A
11
Q

How is bronchiectasis managed?

A
  • Treat underlying cause
  • Physiotherapy to clear mucus
  • Antibiotics based on sputum cultures for exacerbations
  • Chronic suppressive therapy
  • Supportive: flu vaccine, bronchodilators
  • Pulmonary rehab if MRC dysponea >3
12
Q

What is cystic fibrosis?

A

Autosomal recessive disorder leading to mutations in the CFTR. Can lead to multisystem disese (usually resp and GI tract) characterised by thick secretions

Main mutation is Phe508del

13
Q

How do we diagnose cystic fibrosis?

A
  • Phenotypic
  • Sweat test
  • Genetic testing
14
Q

What are the common CF presentations?

A

- Meconium Ileus: bowel obstructive so bilious vomiting, abdominal extension and delay in passing meconium

- Intestinal malabsorption: lack of pancreatic enzymes

- Recurrent chest infections

  • Doesn’t normally present as screened for in newborns
15
Q

Why may a patient not be diagnosed with CF until adulthood and what conditions may make you suspect CF in an adult?

A

May have mutation 5 where they just have a reduced synthesis of CFTR

Suspect in idiopathic pancreatitis, recurrent sinusitis, lung infections, infertility, allergic bronchopulmonary aspergilliosis

16
Q

What are some common complications with CF?

A
  • Infertility
  • Gall stones
  • Cardiac failure
  • Chronic liver disease
  • Osteoporosis
  • Arthritis
  • Respiratory failure (P.aeruginosa)
17
Q

What is some lifestyle advice a GP may give to a CF patient?

A
18
Q

How is CF managed?

A
  • MDT
  • Holistic care
  • Pneumococcal and Influenza vaccinations
  • Optimal nutritional state
  • Airway clearance
19
Q

What is a newly approved drug that is used for patients with CF?

A

Orkambi - lumacaftor and ivacaftor