Bronchiectasis & CF Flashcards

1
Q

What is Bronchietasis?

A

Chronic irreversible dilation of one or more bronchi

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2
Q

Which diagnostic investigation is used to identify Bronchiectasis?

A

High resolution CT

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3
Q

What is the Signet Ring Sign?

A

Where the bronchus is markedly dilated compared to its adjacent pulmonary artery (looks like a ring)

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4
Q

Name 3 symptoms of Bronchiectasis

A

Chronic cough, daily sputum production, haemoptosis, breathlessness on exertion

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5
Q

Name 3 causes of Bronchiectasis

A

Post infective, immune deficiency, idiopathic, alpha-1-antitrypsin deficiency, obstruction

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6
Q

Name 2 common causative organsims of Bronchiectasis

A

Haemophilus influenzae and Moraxella catarrhalis

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7
Q

What is chronic bronchitis?

A

Excess mucus secretion caused by smoking/air pollutants

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8
Q

What is the management for Bronchiectasis?

A

Physio airway clearance and regular sputum sampling

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9
Q

Define an exacerbation

A

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

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10
Q

What is the predominant mutation in Cystic Fibrosis?

A

Phe508del

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11
Q

What clinical presentations in CF?

A

Meconium ileus, intestinal malabsorption and recurrent chest infections

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12
Q

What is Orkambi?

A

Lumacaftor = CTFR chaperone and Ivacaftor = CTFR potentiator

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