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Resp > Lecture 11 - Bronchiectasis and CF > Flashcards

Lecture 11 - Bronchiectasis and CF Flashcards

1
Q

Bronchiectasis

A

Chronic irreversible dilation of one or more bronchi

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2
Q

Features of bronchiectasis

A
  • Dilated bronchi
  • Poor mucus clearance
  • Predisposition to recurrent bacterial infection
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3
Q

Causes of bronchiectasis

A

Chronic inflammation :

Post infection:

  • TB
  • whooping cough

Mucocilliary clearance defect:

  • CF
  • Primary ciliary dyskinesia
  • Young’s syndrome
  • Kartagener syndrome

Idiopathic
Alpha 1 antitrypsin deficiency
Rheumatoid arthritis
HIV

Obstruction:

  • Foreign body
  • Tumour
  • lymph node

Toxic insult:
- Gastric aspiration

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4
Q

Aetiology of bronchiectasis

A
  1. Chronic inflammation
  2. Destruction of elastic and muscular components of bronchus walls
  3. Peribronchial fibrosis
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5
Q

CT scan

A

Signet ring sign - bronchus more dilated than pulmonary artery branch

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6
Q

Symptoms of bronchiectasis

A

Chronic cough
Daily sputum production
Fever

SOB on exertion
Intermittent haemoptysis
Chest pain
Fatigue

Less common: wheeze

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7
Q

Signs of bronchiectasis

A 
Hypoxaemia - advanced
Recurrent fever 
Haemoptysis 
Weight loss
Fine crackles 
Rhonchi

Clubbing - less common

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8
Q

Breath sounds

A

Fine crackles
High pitched inspiratory squeaks
Rhonchi - turbulent flow- low pitched gurgling

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9
Q

Young’s syndrome

A

Bronchiectasis
Sinusistis
Reduced fertility

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10
Q

Kartagener syndrome

A

Bronchiectasis
Sinusitis
Sinus invertus

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11
Q

Common organisms that cause bronchiectasis

A 
Haemophilus influenzae 
Psuedomonas aeruginosa
Streptococcus pnuemoniae
Mycobacteria tuberculosis 
Nontuberculosis mycobacteria

Fungus - aspergillus and candida

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12
Q

Vicious cycle

A
  1. Bronchial dilation
  2. Leads to mucous accumulation and impaired ciliary function
  3. Increased risk of infection due to impaired ciliary function and mucous stasis
  4. Infection leads to inflammation and loss of elastic fibres and smooth muscle in the the bronchus
  5. Leads to more dilation
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13
Q

Alpha 1 antitrypsin deficiency

A
  1. Deficiency in alpha 1 antitrpsin
  2. Less inhibition of neutrophil elastase
  3. Increase in neutrophil elastase breaks down elastase in alveoli
  4. Less recoil and dilated
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14
Q

Sputum test

A

May see:
psuedomonas aeruginosa
Haemophilus influenzae
Atypical mycobacteria

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15
Q

Spirometry

A

Obstructive:

  • Reduced FEV1
  • FEV1/FVC less than 70%
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16
Q

Summary of bronchiectasis

A

Location: Bronchus

Pathology: Bronchus dilation and mucus accumulation with scarring

Aetiology: Persistant or severe infection

Symptoms: Chronic cough, sputum and fever

17
Q

Bronchiectasis management

A

Physio and airway clearance

Sputum sampling

Vaccinations against haemophilus influezae and streptococcus pnuemoniae

18
Q

Exacerbation

A

Deterioration of 3 or more symptoms for at least 48 hours

19
Q

Cystic fibrosis

A

Autosomal recessive

Mutation of the gene for the cystic fibrosis transmembrance conductance regulator CFTR

  • Cl- transported into lumen
20
Q

Cystic fibrosis causes

A

Thickened, sticky mucous accumulation

Malabsorption in pancreas
Biliary cirrhosis - affects liver
Infertility due to vas deferens defect

21
Q

CF diagnosis

A
  • History of CF in a sibling
  • +ve screening test in newborn

Sweat test - increased sweat chloride concentration

Identification of 2 CF mutations - extended genotyping

22
Q

Presentation of CF in newborns

A

Meconium ileus

Intestinal malabsorption - lack of pancreatic enzymes

Newborn screening

Recurrent chest infection

Type 3 diabetes - destruction of pancreatic beta islets of Langerhan cells - reduction of insulin

23
Q

Late diagnosis

A

Atypical CF
Some variants of CF may present later and misdiagnosed e.g.
idiopathic pancreatitis
recurrent lung infections

24
Q

CF lifestyle advice

A 
Avoid other CF patients
No smoking
Avoid people with chest infections
Avoid jacuzzis - psuedomonas
Vaccinations 
NaCl tablets in hot weather and vigorous exercise
25
Q

Avoid other CF patients

A

As immunocompromised and have more bacteria in lungs that can infect CF patients

26
Q

Management of CF

A

Physio and airway clearance
CF specialist centre
Vaccinations
Optimal nutritional state

27
Q

Classes of CF

A

I - no protein production
II - Protein made but doesn’t get to cell membrane
III - Protein made but doesn’t work
IV - Protein made but partially active (common in adults)
V - Protein expressed but reduced quantity
VI - Protein gets to membrne but unstable

28
Q

Lumacaftor

A

CFTR chaperone

29
Q

Ivacaftor

A

CFTR potentiator - improves Cl- transport through the channel as increases probability channel is open

30
Q

Mutation of CF

A

Phe508del
Deletion of phenylalanine at position 508of the polypeptide chain
Chromosome 7

Resp (22 decks)

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