Paediatric Respiratory 1 Flashcards

1
Q

What is estimated tidal volume at rest?

A

6-7 mL/kg body weight

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What happens to FEV1/FVC in restrictive and obstructive lung disease?

A
  • Normal in restrictive, may be increased (normal 75-80%)

- Decreased if obstructive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Describe what happens to the flow-volume loop in restrictive and obstructive lung disease:

A
  • Restrictive - flow less affected, narrow oblong. May be smaller overall
  • Obstructive - volume less affected, concave expiratory loop
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What diseases lead to decreased FRC?

A
  • Alveolar interstitial diseases
  • Thoracic deformities
  • Restrictive lung diseases

examples: scoliosis, neuromuscular diseases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What diseases lead to increased FRC?

A
  • Intrathoracic airway obstruction
  • Obstructive lung diseases
  • examples: bronchiolitis, asthma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Poiseuille’s law:

A

Resistance to laminar flow = ( 8 x length x viscosity ) / (pi x radius^4)

R=8lη÷Πr4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Describe the different types of sleep and what you might see on a PSG:

A

N1 - sleep transition
N2 - light sleep with spindles (look like fuzzy caterpillars) and K complexes (up then down waves)
N3 - slow wave and deep sleep, small EMG
REM - lots of eye movement, very small EMG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What medication(s) are used to treat periodic limb movement disorder?

A
  • Often see less iron in basal ganglia
  • Check ferritin, Fe supplement aim top 1/2 of normal range/above 50
  • In adults use antiparkinsonian meds
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What paO2 corresponds with sats of 50%, 70%, 80%, 90% and 97% on pulse oximetry?

A
50%  =  PaO2  27mHg  
70%  =  PaO2  40mmHg
80% = PaO2 45mmHg
90%  =  PaO2  60mmHg  
97%  =  PaO2  96mmHg
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Which factors shift the oxygen dissociation curve to the right?

A

To the right = more free oxygen available e.g. for exercising muscles! Breaks Hb and O2 apart/decrease affinity.

  • increased temperature
  • increased PCO2
  • decreased pH
  • increased 2,3-diphosphoglycerate.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Which factors shift the oxygen dissociation curve to the left?

A

To the left = you want the Hb and O2 to stick together/increase affinity e.g. in lungs

  • decreased temperature
  • decreased PCO2
  • increased pH
  • decreased 2, 3 diphosophoglycerate
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What should the PVR be in childhood (as % of systemic resistance)?

A

3 days after birth - 50%

2-3/12 after birth - 15%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Definition of pulmonary arterial hypertension:

A
  • Mean PA pressure ≥25 mm Hg
    WITH
  • Normal pulmonary capillary wedge pressure ≤15 mmHg (i.e. normal venous pressure)
  • Increased PVR index ≥3 Wood units/m^2
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the cells that react to lung inflammation and what chemicals do they produce?

A
  • Alveolar macrophages

- Tumor necrosis factor-α and interleukin-1β

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Where are the mutations causing cystic fibrosis and what are the most common ones?

A
  • CFTR protein on Chromosome 7
- Commonest is deletion F508 (class 2, protein transport problems)
In Australia/NZ
- Gly551Asp/G551D (defective regulation of CFTR, class 3)
- Gly542X (no CFTR, class 1)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

How common is cystic fibrosis?

A

1/3500 live births in white populations

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

How common is meconium ileus in CF?

A

10-15%

Some genetics seem to make it more likely

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What are the diagnostic criteria for CF?

A

Typical clinical features (respiratory, GI, GU) / sibling with CF / positive newborn screening test

plus

Lab evidence:
2X elevated sweat chloride (>60 mEq/L) obtained on separate days (NB: intermediate is 30-59, can still have CF) or 2 CF mutations or an abnormal nasal potential difference measurement

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What is the screening test for CF in NZ?

A

Tier 1: Measurement of immunoreactive trypsin (IRT).
Tier 2: If the IRT level is in the highest 1%, this is followed by genetic analysis using a limited panel
of the most common CF genetic mutations in NZ.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is Kartagener Triad?

A
  • Situs inversus totalis (50% of patients with primary ciliary dyskinesia have it; 25% of patients with situs inversus have PCD)
  • Chronic sinusitis
  • Bronchiectasis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What is FVC 25-75% useful for?

A
  • Indicator of mild airway obstruction

- Small airways disease

22
Q

What genetic/syndromic anomalies are associated with choanal atresia?

A
  • CHARGE syndrome
  • Treacher-Collins
  • Kallmann syndrome
  • VA(C)TER(L) association
  • Pfeiffer
23
Q

What is ABPA associated with?

A
  • Asthma or CF
24
Q

How does monteleukast work?

A
  • Block effects of cysLT1 receptor (leukotriene receptor antagonists)
25
Q

Vocal cord dysfunction/paralysis on flow-volume loop…

A

Flattening of inspiratory curve

26
Q

Tracheal stenosis on flow-volume loop…

A

Flattening of inspiration and expiration curve

27
Q

What is omalizumab and what do you use it for?

A
  • Humanized, monoclonal anti‐IgE antibody that binds specifically to circulating IgE molecules, thus interrupting the allergic cascade.
  • > 12yo severe allergic asthma when all other avenues exhausted. Given by a subcut injection – may get site reactions.
  • Well tolerated, but occasional anaphylaxis
28
Q

How does carbon monoxide cause toxicity?

A
  • Reversible binding of carbon monoxide to cytochrome A3.

- Binds to the usual OXYGEN site of Hb.

29
Q

What is surfactant and which cells make it?

A
  • Mixture of phospholipids + proteins

- Synthesized, packaged, secreted by alveolar type II pneumocytes (AEC2s) in distal air spaces

30
Q

What does surfactant do?

A
  • Decrease surface tension at end-expiration

- Prevent atelectasis and VQ mismatch

31
Q

What is ivacaftor approved for?

A
  • CF treatment - class 3 mutations

- Gly551Asp/G551D

32
Q

What is surfactant mostly made of?

A
  • Phosphatidycholine
33
Q

When do you get false negatives on a sweat test?

A
  • <75g sweat
  • Malnutrition
  • Mineralocorticoid use
  • Adrenal insufficiency
  • Fucosidosis
  • G6PD deficiency
34
Q

What are the wheezy viruses?

A
  • Rhinovirus
  • RSV
  • Increased risk of wheeze persisting at age 6
35
Q

What is Orkambi and what is it for?

A
  • CF with homozygous F508del mutation
  • ivacaftor and lumacaftor
  • corrector - protein folds properly then stays open
36
Q

What is Pulmozyme?

A
  • Pulmozyme - recombinant human deoxyribonuclease I - enzyme which selectively cleaves DNA
  • dornase alfa
  • Inhaled, anti-neutrophil, reduces gas trapping, improves airway clearance & CT findings by making the mucous less viscous.
37
Q

What factors increase 2, 3 DPG?

A
  • Hormones: thyroxine, HGH, adrenaline, testosterone

- High altitude (paO2 lower, need more to get oxygen off Hb to tissues)

38
Q

Flattened expiratory curve in flow-volume loop:

A

Intrathoracic upper airway obstruction e.g. tracheomalacia

39
Q

Flattened inspiratory curve in flow-volume loop:

A

Extrathoracic upper airway obstruction e.g. vocal cord paralysis or dysfunction

40
Q

Flattened rectangular shape to all of flow-volume loop:

A

Fixed upper airway obstruction e.g. neck lump, tracheal stenosis or goitre

41
Q

What is compliance and how does it vary in younger vs older children?

A
  • Lung compliance is the change in lung volume per unit change in pressure
  • Neonates have LOW lung compliance i.e. need high pressures to ventilate
  • but HIGH chest wall compliance
42
Q

What is the response to bronchodilator in asthma?

A

FEV1 increases by 15%

43
Q

Decreased FEV1:FVC is…

A
  • More likely obstructive
  • Should also have decreased FEV1
  • Decreased FEF 25-75% if small airways affected
44
Q

Poor technique clues on flow-volume loop…

A
  • Abnormal dips in expiratory curve

- early starts of inspiratory curve

45
Q

Why are changes in FRC important?

A
  • FRC maintains PAO2/PACO2 at more constant levels during inspiration and expiration
  • decreased FRC -> decreased PAO2 in expiration as decreased volume, therefore hypoxemia
  • compensate with increased PEEP and increased I-time in ventilation
  • increased FRC - lungs are less compliant
46
Q

What are signs/factors suggesting poor prognosis for CF?

A
  • Poor nutritional status
  • Pseudomonas aeruginosa
  • Burkholderia cepacia
  • Diabetes
  • Frequent exacerbations
  • Female gender
47
Q

What are the indications for lung transplant in CF?

A
  • FEV1 <30% predicted
  • Rapid decline in lung function
  • Declining exercise tolerance
  • Nutritional problems
  • Life threatening complications
48
Q

What are the features of diaphragmatic hernia?

A
  • 1/2-3000
  • Affects contralateral lung
  • Long term nutritional problems
  • 30-60% malrotation
  • R side 12%/L side 88%
49
Q

How does mepolizumab work?

A

Anti IL-5

50
Q

What happens physiologically with sleep?

A

Decreased muscle tone -> 2X airway resistance, 1/2 VT

Sleep worsens all ventilatory issues (except laryngomalacia)