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Respiratory System > Bronchiectasis & Cystic Fibrosis > Flashcards

Bronchiectasis & Cystic Fibrosis Flashcards

1
Q

Define Bronchiectasis

A

The chronic, irreversible dilation of one or more bronchi

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2
Q

What is the problem with bronchi dilation in bronchiectasis?

A

Deformed bronchi exhibit poor mucus clearance, predisposing to chronic bacterial infection

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3
Q

What causes bronchiectasis?

A

A range of chronic inflammatory conditions that cause destruction of elastic and muscular components of the bronchial wall

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4
Q

What finding will you see on a CXR if someone has bronchiectasis? What is this called?

A

The bronchial dilation is bigger than the adjacent pulmonary blood vessel and the bronchial wall is thickened

Signet Ring Sign

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5
Q

What are some of symptoms of bronchiectasis?

A

Very common:

  • Chronic cough
  • Daily sputum production

Common:

  • Breathlessness on exertion
  • Nasal symptoms
  • Coughing up blood occasionally
  • Chest Pain
  • Fatigue
  • Weight Loss
  • Intermittent fever

Less Common:

  • wheeze
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6
Q

What are some of the clinical signs of bronchiectasis?

A
  • Hypoxaemia on pulse oximetry
  • Fever (common)
  • Bloody cough
  • Fine crackles
  • Ronchi (sounds like underwater)
  • High pitched inspiratory squeaks
  • Crackles and wheeze (sometimes)
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7
Q

Identify some causes of bronchiectasis

A
  • Post infective
  • Immune deficiency
  • Mucocilliary clearance defects
  • Idiopathic
  • alpha-1-antitrypsin deficiency
  • Obstruction
  • Toxic insult
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8
Q

Which common organisms can cause bronchiectasis?

A
  • Haemophilus influenzae
  • Pseudomonas aeruginosa (if in hospital alot)
  • Moraxella catarrhalis
  • Streptococcus penumoniae
  • Aspergillus and Candida funghi
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9
Q

What are the main differentiating factors between chronic bronchitis and bronchiectasis?

A

Chronic Bronchitis: usually tobacco, smoke, air pollutants with a cough productive of sputum

Bronchiectasis: exposure to persistant, sever infections with a cough purlent of sputum with fever!

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10
Q

How would you manage bronchiectasis?

A
  • Physio / airway clearance essential
  • sputum sampling routinely
  • exclude immunodeficient causes
  • annual flu vaccines
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11
Q

How do you define a bronchiectasis exacerbation?

A

A person with bronchiectasis with a deterioration in 3 or more key symptoms for the last 48 hours

  • Cough
  • Sputum volume and/ or consistency
  • Sputum purulence
  • Breathlessness and or exercise tolerance
  • Fatigue
  • Haemoptysis
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12
Q

What is the most common, identifiable cause of bronchiectasis?

A

Cystic Fibrosis

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13
Q

What is the predominant mutation in cystic fibrosis?

A

Phe508 del

Deletion of phenylalanine at postion 508 of the CFTR channel

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14
Q

What is the genetic pattern of inheritence of cystic fibrosis?

A

Autosomal recessive disorder

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15
Q

What is the effect of a defective CFTR channel?

A
  • The normal CFTR channels transports Cl- ions out of cells and water follows
  • In CF, the dysfunctional CFTR channel cannot pump Cl- out of cells and water cannot follow causing a thick, sticky mucus in the airways
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16
Q

There are over 2,000 mutations related to the CFTR gene, what are the 6 classes according to protein function?

A
  • No protein production of CFTR
  • Protein made but never gets to cell membrane
  • Protein made bu doesn’t work
  • Protein made but only partially active
  • Protein expressed at gene level but a substantial reduction in mRNA or protein synthesis
  • Protein made but unstable at membrane
17
Q

What are some of the classical clinical presentations of CF?

A
  • Meconium ileus - as bowel is blocked by sticky secretions
  • Intestinal malabsorption- from a severe deficiency in pacreatic enzymes
  • Recurrent Chest Infections
  • Newborn screening
18
Q

If someone is diagnosed with cystic fibrosis after 20 years of age, what type of mutation is likely?

A

Mutation associated with residual CFTR function

19
Q

Which organism frequently colonises and infects patients with cystic fibrosis?

A

Pseudomonas aeruginosa

20
Q

What lifestyle advice should be given to patients with cystic fibrosis?

A
  • No smoking
  • Avoid other CF patients
  • Avoid friends/ family with colds and infections
  • Avoid jacuzzis
  • Annual flu vaccine
  • Take NaCl tablets in hot weather/ vigorous exercise
21
Q

What is the MoA for Orkambi in treating CF?

A
  • Mix of 2 drugs: Ivacaftor and Lumacaftor
  • Ivacaftor: CFTR potentiator → improves transport of Cl- through the channel
  • Lumacaftor: CFTR chaperone during protein folding to increase the number of proteins trafficked to the cell surface

Respiratory System (20 decks)

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