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Resp > Bronchiectasis and Cystic Fibrosis > Flashcards

Bronchiectasis and Cystic Fibrosis Flashcards

1
Q

What is bronchiectasis?

A

Chronic and irreversible dilation of one or more bronchi

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2
Q

What are the consequences of bronchi dilation?

A
  • exhbit poor mucus clearance

- predisposition to recurrent or chronic bacterial infection

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3
Q

What is the aetiology of bronchiectasis?

A

-chronic inflammation
leads to destruction of elastic fibres and muscle fibres that give elasticity and movement to bronchi but once destroyed they permanently dilate the bronchi

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4
Q

How do you diagnose bronchiectasis?

A

High resolution CT scan

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5
Q

What would you find?

A

dilated bronchi and thickened walls

-bronchus is larger than its pulmonary arteriole

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6
Q

What is the key defining feature of bronchiectasis ?

A

signet ring - due to the bronchus being larger than its accompanying pulmonary arteriole

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7
Q

What are the clinical symptoms of bronchiectasis?

A
  • daily sputum production
  • breathless on exertion
  • intermittent haemoptysis
  • chronic cough
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8
Q

What are the clinical signs?

A
  • hypoxaemia (seen with pulse oximetry)
  • history of intermittent fevers
  • fine crackles on exam
  • sometimes have wheezing
  • high pitched inspiratory squeaks
  • history of weight loss (hyper metabolic state due to chronic inflammation)
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9
Q

What are the causes of bronchiectasis?

A
  • TB
  • immune deficiency
  • CF
  • alpha 1 anti trypsin deficiency
  • obstruction
  • toxic insult (aspiration of gastric acid)
  • HIV
  • malignancy
  • RA
  • IBD
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10
Q

what are the common organisms causing bronchiectasis?

A
  • haemophillus influenzae
  • pseudomona aeruginosa
  • mycobacterium tuberculosis
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11
Q

Why does bronchiectasis need to be controlled?

A

bronchial dilation leads to mucous accumulation –>impaired ciliary function and increased risk of infection –> leads to inflammation if infection is caught –>loss of more bronchial elastic fibres and smooth muscle –> more dilation

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12
Q

What would you find if you did lung functions tests on someone with bronchiectasis?

A
  • obstructive patten
  • reduced FEV 1
  • normal diffusing capacity
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13
Q

Why is the diffusing capacity the same even though there is an accumulation of mucus which increases the diffusion distance?

A

-the mucus accumulation occurs in the bronchi which is not in the respiratory conducting portion as gaseous exchange takes place in the alveoli

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14
Q

What are the differences between bronchitis and bronchiectasis?

A
  • bronctis is due to mucous gland hyperplasia due to tobacco smoke
  • bronchiectasis is due to chronic infections that cause airway dilation and scarring
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15
Q

What is a purulent sputum?

A

green/yellow sputum

-maybe even smell

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16
Q

How does bronchiectasis management?

A
  • physio with airways clearance 2/3 times a day
  • exclude immunodeficiency
  • long-term therapies
  • immunisations up to date
  • sputum sampling
17
Q

What is an exacerbation in bronchiectasis?

A

a person with bronchiectasis with a deterioration in 3 or more key symptoms for at least 48 hrs

  • cough
  • sputum volume
  • sputum purulence
  • breathlessness
  • fatigue
  • haemoptysis
18
Q

What is the most common identifiable cause of bronchiectasis? (in europe / UK)

A

CF

19
Q

What is cystic fibrosis?

A
  • autosomal recessive disorder
  • mutantation of gene located on chromosome 7
  • leads to abnormal function of CFTR (key role in maintaining lung epithelium)
  • characterised by thickened secretions of mucus
20
Q

What does CFTR stand for and what is it?

A

Cystic fibrosis transmembrane conductance regulator - epithelial chloride channel

  • transmembrane protein that transport cl- and bicarbonate and regulates ENAC
  • it enables Cl- to be transported out of cells into airways controlling the moment of water
21
Q

What co-morbidites are caused because of epithelial cell destruction?

A
  • malabsorption (pancreas)
  • billiary cirrhosis (liver)
  • heat shock (sweat glands release Cl- and NA+)
  • infertility (vas deferens)
22
Q

So if the CFTR gene is mutated, what happens?

A

reduced cl- transport across membrane so water doesn’t leave epithelium to hydrate the mucus so you get thick mucus production which blocks ducts

23
Q

How is CF diagnosed?

A
  • history of CF in a sibling
  • positive new born screening result
  • increases sweat chloride conc
  • genotyping

Resp (8 decks)

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