Paediatric Respiratory 1 Flashcards

(50 cards)

1
Q

What is estimated tidal volume at rest?

A

6-7 mL/kg body weight

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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

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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
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4
Q

What diseases lead to decreased FRC?

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

examples: scoliosis, neuromuscular diseases

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5
Q

What diseases lead to increased FRC?

A
  • Intrathoracic airway obstruction
  • Obstructive lung diseases
  • examples: bronchiolitis, asthma
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6
Q

Poiseuille’s law:

A

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

R=8lη÷Πr4

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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

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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
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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
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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.
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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
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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%

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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
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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

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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)
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16
Q

How common is cystic fibrosis?

A

1/3500 live births in white populations

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17
Q

How common is meconium ileus in CF?

A

10-15%

Some genetics seem to make it more likely

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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

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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.

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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
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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?

24
Q

How does monteleukast work?

A
  • Block effects of cysLT1 receptor (leukotriene receptor antagonists)
25
Vocal cord dysfunction/paralysis on flow-volume loop...
Flattening of inspiratory curve
26
Tracheal stenosis on flow-volume loop...
Flattening of inspiration and expiration curve
27
What is omalizumab and what do you use it for?
- 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
How does carbon monoxide cause toxicity?
- Reversible binding of carbon monoxide to cytochrome A3. | - Binds to the usual OXYGEN site of Hb.
29
What is surfactant and which cells make it?
- Mixture of phospholipids + proteins | - Synthesized, packaged, secreted by alveolar type II pneumocytes (AEC2s) in distal air spaces
30
What does surfactant do?
- Decrease surface tension at end-expiration | - Prevent atelectasis and VQ mismatch
31
What is ivacaftor approved for?
- CF treatment - class 3 mutations | - Gly551Asp/G551D
32
What is surfactant mostly made of?
- Phosphatidycholine
33
When do you get false negatives on a sweat test?
- <75g sweat - Malnutrition - Mineralocorticoid use - Adrenal insufficiency - Fucosidosis - G6PD deficiency
34
What are the wheezy viruses?
- Rhinovirus - RSV - Increased risk of wheeze persisting at age 6
35
What is Orkambi and what is it for?
- CF with homozygous F508del mutation - ivacaftor and lumacaftor - corrector - protein folds properly then stays open
36
What is Pulmozyme?
- 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
What factors increase 2, 3 DPG?
- Hormones: thyroxine, HGH, adrenaline, testosterone | - High altitude (paO2 lower, need more to get oxygen off Hb to tissues)
38
Flattened expiratory curve in flow-volume loop:
Intrathoracic upper airway obstruction e.g. tracheomalacia
39
Flattened inspiratory curve in flow-volume loop:
Extrathoracic upper airway obstruction e.g. vocal cord paralysis or dysfunction
40
Flattened rectangular shape to all of flow-volume loop:
Fixed upper airway obstruction e.g. neck lump, tracheal stenosis or goitre
41
What is compliance and how does it vary in younger vs older children?
- 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
What is the response to bronchodilator in asthma?
FEV1 increases by 15%
43
Decreased FEV1:FVC is...
- More likely obstructive - Should also have decreased FEV1 - Decreased FEF 25-75% if small airways affected
44
Poor technique clues on flow-volume loop...
- Abnormal dips in expiratory curve | - early starts of inspiratory curve
45
Why are changes in FRC important?
- 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
What are signs/factors suggesting poor prognosis for CF?
- Poor nutritional status - Pseudomonas aeruginosa - Burkholderia cepacia - Diabetes - Frequent exacerbations - Female gender
47
What are the indications for lung transplant in CF?
- FEV1 <30% predicted - Rapid decline in lung function - Declining exercise tolerance - Nutritional problems - Life threatening complications
48
What are the features of diaphragmatic hernia?
- 1/2-3000 - Affects contralateral lung - Long term nutritional problems - 30-60% malrotation - R side 12%/L side 88%
49
How does mepolizumab work?
Anti IL-5
50
What happens physiologically with sleep?
Decreased muscle tone -> 2X airway resistance, 1/2 VT | Sleep worsens all ventilatory issues (except laryngomalacia)