Respiratory Flashcards

1
Q

How many generations of airways from trachea to alveoli?

How many have cartilage support?

A

23 generations total

16 have cartilage

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2
Q

What is the gene involved in AAT deficiency?

Which alleles are highest risk for cirrhosis?

Which are highest risk for emphysema?

A

Gene is called SERPINA1 on chromosome 14

Usually occurs in those homozygous for Z or M(malton) alleles

ZZ homozygotes ot ‘null’ alleles

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3
Q

What is the mechanism of COPD development in AAT deficiency?

A

Results from imbalance between neutrophil elastase in lung, and AAT

  • Increased elastase burden
  • Increased destruction of elastin
  • Lung proteolytic degradation
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4
Q

What is the function of alpha-1 trypsin?

A

AAT is a protease inhibitor, which inhibits elastase.

Thus deficiency leads to excessive elastin destruction

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5
Q

What is “Samter’s Triad” in AERD?

A

Nasal polyps, asthma, and sensitivity to aspirin

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6
Q

What is the main substance implicated in AERD?

A

Leukotrienes

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7
Q

What does high FENO indicate?

A

Predicts response to ICS

Measure of eosinophilic inflammation

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8
Q

What is the most effective strategy to halt progress in COPD?

A

Smoking cessation

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9
Q

What are the 3 interventions in COPD with mortality benefit?

A

Smoking cessation
Long term O2 therapy if meeting criteria
Transplant / lung reduction surgery

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10
Q

PaO2 cut offs for home O2?

A
PaO2 <55
PaO2 <60 in
- RHF
- Pulmonary HTN
- Secondary polycythaemia
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11
Q

Most common side effect of ICS?

A

Hoarse voice

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12
Q

Most common bacterial cause of COPD exacerbation?

A

Haemophilus influenzae

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13
Q

What is the BODE Index?

A

Predictor of mortality in COPD

B - BMI low
O - Obstruction (FEV1)
D - Dyspnoea score
E - exercise tolerance

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14
Q

MOst common genetic mutation in Type 1 pulm HTN?

A

Bone morphogenetic protein receptor type 2 (BMPR2) mutations

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15
Q

Most common genetic mutation in Type 1 pulm HTN?

A

Bone morphogenetic protein receptor type 2 (BMPR2) mutations

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16
Q

What is the most common genetic mutation in CF?

A

Delta F508

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17
Q

What does the CFTR protein do when functioning normally?

A

CFTR protein functions mainly as a chloride channel

  • also regulates activity of other chloride and sodium channels on cell surface
  • Active at the apical membrane of epithelial cells
  • Regulates movement of sodium and water across cell membrane
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18
Q

What are the mechanisms of hypoxia which will give a raised A-a gradient?

A

V/Q mismatch
Shunt
Diffusion abnormality

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19
Q

What is the equation for A-a gradient calculation?

A

[150 - (PaCO2 x 1.25)] - PaO2

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20
Q

What is a normal A-a gradient?

A

Should be around Age/4 + 4

21
Q

What are the mechanisms of hypoxia which will give a normal A-a gradient?

A

Hypoventilation

Low FiO2

22
Q

What are the two main mechanisms for hypercapnia?

A

Decreased minute ventilation

Increased dead space

23
Q

What are the 2 main mechanisms for decreased minute ventilation?

A

“can’t breathe” - decreased respiratory drive

“won’t breathe” - decreased nerve / muscle function, or thoracic cage limitation

24
Q

What is the criteria for obstruction on PFTs?

A

FER <0.7

25
Q

What is the criteria for reversibility of obstruction on PFTs?

A

Improvement of >12% and >200ml in FEV1 or FVC

26
Q

What causes reduced DLCO in absence of significant restriction or obstruction?

A

Pulmonary vascular disease (e.g. thromboembolic disease, pulmonary HTN)

27
Q

When are MIP / MEP useful to measure in PFTs?

A

To evaluate for respiratory muscle weakness

28
Q

What is the 12 month mortality after an exacerbation of COPD requiring admission?

A

25%

29
Q

What is the PBS criteria for triple therapy in COPD?

A

FEV1< 50 and history of repeated exacerbations with significant symptoms despite dual therapy

30
Q

What is the biological therapy option for severe allergic asthma?

A

Omalizumab

- IgE mab

31
Q

What is the biological therapy option for severe eosinophilic asthma?

A

Mepolizumab

- anti-IL 5 ab

32
Q

What are the classic pathological features of Usual Interstitial Pneumonia (IPF)?

A

Honeycombing
Traction bronchiectasis
Reticular opacities

33
Q

What is nintedanib?

Common SE?

A

Anti-fibrotic for IPF
Multi-kinase inhibitor

SE: diarrhoea

34
Q

What is pirfenidone?

A

Anti-fibrotic for IPF

Inhibits TGF-beta and fibroblast proliferation

35
Q

What are the classic pathological features of Non-specific Interstitial Pneumonia?

A

Ground glass opacity
Traction bronchiectasis
Reticular opacities

36
Q

Which immune cells are key to sarcoidosis pathogenesis?

A

Th1 cells

37
Q

What is the leading cause of pulmonary HTN worldwide?

A

Schistosomiasis

38
Q

What class of drugs are ambrisentan, macitentan, bosentan?

How do they work?

A

Endothelin receptor antagonists

Work to block endothelin receptor, which inhibits vasoconstriction

39
Q

Which 2 medication classes should NOT be used in combination in pulmonary HTN?

A
sGC stimulators (Riociguat) + PDE5 inhibitors
- increased risk of hypotension and death
40
Q

What is the role of azithromycin in bronchiectasis?

A

For patients with 3 or more exacerbations per year

Dosing 500mg 3x / week

Anti-inflammatory, reduces exacerbation frequency

41
Q

Where is the brain centre for inspiration?

A

Dorsal medullary centre

42
Q

Where is pulmonary surfactant produced?

A

Type 2 alveolar epithelial cells

43
Q

3 ILD conditions linked to smokng?

A

Respiratory bronchiolitis - associated
Desquamative
Langerhans histiocytosis

44
Q

Main site of airway resistance in lungs?

A

Medium-sized bronchioles

45
Q

What causes NSIP pattern (~4)

A

CTD
HIV
Drugs
Hypersensitivity

46
Q

What is Light’s criteria?

A

If at least one of the following three criteria is fulfilled, the fluid is defined as an exudate:

  • Pleural fluid protein/serum protein ratio >0.5
  • Pleural fluid LDH/serum LDH ratio >0.6
  • Pleural fluid LDH greater than 2/3 the upper limits of the laboratory’s normal serum LDH
47
Q

What are pleural fluid tumour markers for mesothelioma?

A

Mesothelin

Fibulin 3

48
Q

What are 3 common presentations of adult-diagnosed CF?

A

GI symptoms
Diabetes
Infertility