Respiratory Flashcards

1
Q

What is the most common cause of bronchiolitis obliterans?

A

Adenovirus

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2
Q

What genetic defect is Ivacaftor approved for in the use of cf?

A

G551D heterozygotes

Class III mutation

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3
Q

What will the flow volume loop look like for fixed upper airway obstruction and what is an example?

A

Flat inspiratory curve
Reduced expiratory loop
Inspiration affected more than expiration

Suglottic stenosis

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4
Q

What is the key feature of pulmonary sequestration?

A

Has its own systemic blood supply

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5
Q

What are the causes of extra-thoracic obstruction (flow volume loop shows flattened inspiratory curve)

A

Vocal cord paralysis
Tracheomalacia
Airway burns

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6
Q

What should you suspect in a child presenting with chronic productive cough, constant nasal discharge, frequent ear infections

A

Primary Ciliary Dyskinesia

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7
Q

What should you suspect in a child presenting with dry cough, multiple episodes of croup - cough noted to be very loud

A

Tracheobronchomalacia

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8
Q

What are the risks of a child developing asthma if

  • neither parent has asthma
  • one parent has asthma
  • both parents have asthma
A
  • 6%
  • 20%
  • 60%
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9
Q
What is the equivalent paO2 for 
Hb sats of 
- 90% 
- 80% 
- 70 %
A
  • 60
  • 50
  • 40
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10
Q

What is the diagnosis and the best diagnostic investigation ?
CF patient with wedge shaped consolidation on CXR

A

ABPA

IgE

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11
Q

Persistent nasal discharge
Recurrent chest infections
Bronchiectasis on CT

A

Primary ciliary dyskinesia

Nasal brushings

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12
Q
What is the diagnosis and the best diagnostic tool? 
European girl with erythema nodosum 
Crackles on lungs 
Dry eyes 
Hepatomegaly
A

Sarcoidosis
Biopsy of gastrocnemius muscle
Serum amyloid A, ACE, IL2

Biopsy will show non caseating epithelial granuloma = highly specific and sensitive

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13
Q

Which cells form the gas exchange surface with capillary endothelium ?

A

Type 1 pneumocytes

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14
Q

What is the diagnosis ?

ABCA 3 mutation

A

Inherited Surfactant deficiency

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15
Q

Pathogenesis of idiopathic pulmonary hypertension is associated with which mediator?

A

Endothelin 1

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16
Q

How do recorded sats correlate with arterial oxygen partial pressures?

A

Sats 90 = paO2 60mmgHg
80 - 50
70 - 40

17
Q

What does the DF508 mutation actually mean?

A

Deletion of phenylalanine at position 508 in CFTR gene

18
Q

What electrolyte disturbance would you expect with CF?

A

Hyponatraemic hypochloraemic metabolic alkalosis

19
Q

What are the 6 different types of CFTR mutations ?

A

1 - protein not synthesised
2 - folding defect (DF508)
3 - channel opening defect - block in regulation
4 - reduced conductance- ion transport defect
5 - decreased protein synthesis
6 - decreased half-life of protein