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SA respiratory > S8) Cystic Fibrosis and Bronchiectasis > Flashcards

S8) Cystic Fibrosis and Bronchiectasis Flashcards

1
Q

What is bronchiectasis?

A

Bronchiectasis is the chronic dilatation of one or more bronchi wherein the bronchi exhibit poor mucus clearance and there is predisposition to recurrent/chronic bacterial infection

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2
Q

Identify 5 causes of bronchiectasis

A
  • Post infective e.g. whooping cough, TB
  • Immune deficiency e.g. hypogammaglobulinaemia, secondary - HIV
  • Obstruction e.g. foreign body, tumour, extrinsic lymph node, compression = bronchus can’t be cleared = inflammation etc
  • Inhalation of toxic chemicals/gases
  • Secondary immune deficiency e.g. HIV, malignancy
  • mucociliary clearance defects - CF
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3
Q

Identify 4 genetic causes of bronchiectasis

A
  • Cystic fibrosis
  • Primary ciliary dyskinesia
  • Young’s syndrome (bronchiectasis, sinusitis, reduced fertility)
  • Kartagener syndrome (bronchiectasis, sinusitits, situs inversus)
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4
Q

Identify 3 causative organisms of bronchiectasis

A
  • Haemophilus influenzae
  • Fungi e.g. aspergillus, candida
  • Non-tuberculous mycobacteria
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5
Q

Describe the management of bronchiectasis

A
  • Treat underlying cause - removed the mucus, underlying congenital problem, autoimmune?
  • Physiotherapy for mucus clearance (⅔ times a day)
  • Antibiotics according to sputum cultures
  • Flu vaccine
  • Bronchodilators // corticosteroids
  • correct nutrient deficits
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6
Q

What is cystic fibrosis?

A

Cystic fibrosis is a multisystem, autosomal recessive disease occurring due to a mutation in the CFTR molecule which is characterised by thickened secretions

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7
Q

How does cystic fibrosis present?

A
  • Meconium ileus – intestinal obstruction by sticky secretions (bilous vomiting & abdominal distension)
  • Intestinal malabsorption – severe deficiency of pancreatic enzymes
  • Recurrent chest infections
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8
Q

How does one confirm the diagnosis of Cystic fibrosis?

A
  • 1/more characteristic phenotypic features
  • History of CF in a sibling
  • Positive newborn screening test result
  • Increased sweat [Cl-]
  • Identification of two CF mutations (genotyping)
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9
Q

Identify 4 common cystic fibrosis complications and describe how they are treated

A
  • Respiratory Infections – aggressive therapy with physio and prophylactic antibiotics
  • Low body weight – pancreatic enzyme replacement therapy, high calorie intake and extra supplements
  • Distal Intestinal Obstruction Syndrome (DIOS)
  • CF Related Diabetes
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10
Q

Describe the management of cystic fibrosis

A
  • Avoid smoking
  • Avoid other CF patients
  • Avoid jacuzzis (pseudomonas)
  • Annual influenza immunisation
  • Sodium chloride tablets in hot weather / vigorous exercise
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11
Q

What is the aetiology of bronchiectasis?

A
  • chronic inflammation
  • can permanently damage elastic and muscular components of bronchial wall and causes fibrosis
  • more risk of infection
  • then more inflammation and cillary damage
  • cycle
  • primarily caused by pertussis and TB
  • remember this infection and pus can also spread to the alveoli
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12
Q

what investigation do you use to determine for bronchiectatsis

A

CXR

  • classic diagnosis is thickened walls and dilated bronchi - tram track signs
  • need high resolution CT (it will show the stage of the situation too)
  • lungs will be thickened and scarred
  • signet ring sign - dilated bronchus next to pulmonary artery and in this case its BIGGER than the artery (should be smaller)
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13
Q

what are clinical symptoms of bronchiectasis

A
  • chronic cough
  • breathless on exertion
  • chest pain
  • fatigue
  • nasal symptoms
  • haemoptysis
  • flem - see how much they are collecting over 24 hours

(mainly find out through history)

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14
Q

what are signs of bronchiectasis

A
  • hyperaemia
  • fever
  • haemoptysis (cough up blood)
  • fine crackles
  • high pitched inspiratory peaks
  • crackles and wheezing
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15
Q

what are some red flags in a history that can be a risk factor bronchiectasis

A
  • severe chest infection earlier in life
  • life long chest infections
  • recurrent chest infections
  • recurrent sinus infections
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16
Q

what are some pulmonary function tests

A
  • obstructive airways:
  • reduced forced expiratory volume
17
Q

differentiate between chronic bronchitis and bronchiectasis

A
  • chronic - white sputum, no history of intermitted fevers
  • bronchiectasis - intermitted fevers, sputum is foul smelling
18
Q

how do we define exacerbation in bronchiectasis

A
  • symptoms for at least 48 hours:
  • cough
  • sputum volume
  • breathlessness
  • fatigue
  • haemoptysis
19
Q

how does CFTR malignancy cause CF

A
  • transports chloride and bicarbonate and regulates ENAC channels
  • enables Cl to be transported into airways
  • it regulates sodium reabsorption into cells
  • plays a role in the hydration of mucous
  • if its defective it reduces hydration
  • mucus becomes thick and can cause problems within the ciliary hair

basically:

  • controls movement of chloride out cells, Na follows Cl for the electrochemical gradient, water follows Na, so if there is a problem with Cl transport then water wont move and mucus becomes thick
20
Q

what is the classic presentation of people with CF

A
  • meconium ileum - bowel is blocked due to mucus so intestinal obstruction = vomiting, abdominal distension
  • intestinal malabsorption - mainly in infancy, due to blockage of exocrine glands
  • recurrent chest infections
  • newborn screening
21
Q

CF lifestyle

A
  • no smoking
  • no other friends with CF - cross infection
  • avoid people who are ill
  • annual influenza immunisation
  • sodium chloride tablets
  • need nutritional support - better nutrition = better lungs
22
Q

tram track signs

A
23
Q

CT comparison of a normal lung and a lung with bronchiectasis

A
24
Q

signet ring sign on a CT

A
25
Q

CF can effect multiple systems in the body, name some problems it can cause

A
  • chronic sinusitis
  • oesophageal reflux
  • chronic liver disease
  • gall stones
  • cardiac failure
  • arthritis

SA respiratory (23 decks)

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