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Tom Respiratory & sleep > Respiratory and Sleep > Flashcards

Respiratory and Sleep Flashcards

1
Q

Which phase of sleep is associated with the lowest muscle tone and therefore the highest rates of OSA?

A

REM

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2
Q

What phase of sleep is shown in this PSG?

A
  1. 30s per page = sleep staging (not breathing question)
    1. Eye movements not present (L oc and R oc) = not REM or awake
    2. EMG looks ‘fat’ = muscle tone is present, not REM
    3. K complexes and sleep spindles found on EEG

This is N2 sleep (light sleep)

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3
Q

Which sleep phase is shown in this PSG?

A

• EEG shows large triangular ‘delta’ waves (but also some K complexes)
• No eye movements

This is N3 sleep (deep sleep)

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4
Q

What sleep phase is shown in this PSG?

A

• Eye movements present - one electrode on the left and one on the right. So if you look to the left the LOC electrode goes up and the ROC electrode goes down (review above)
• In the other examples above, all of the waves are in the same direction - this indicates they are picking up brain waves (given they are located at the temples)
• EMG looks flat - hypotonia

This is REM - dream sleep

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5
Q

What are the potential risks of long term melatonin use in children with chronic sleep issues?

A

There are melatonin receptors on testis and ovaries
There are concerns that long term melatonin use could potentially delay sexual maturation

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6
Q

An adolescent presents with chronic fatigue.
Their actigraphy is shown below.
What is the diagnosis and treatment?

A

Diagnosis: Delayed sleep phase
• Sleep hygiene
○ Dim light before bed
○ No computer/TV in room
○ No stereo or texting
• Bright light when should be awake
• Advance bedtime by 15 minutes each 3 nights
Melatonin as adjuvant

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7
Q

In what phase of sleep do night terrors occur?

A

N3 - deep sleep

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8
Q

You see a 4 year old patient who’s parents report that they regularly wake from sleep and scream.
Their eyes are open, but they do not respond to their parents. They have noticed that they look sweaty and have dilated pupils.

Is the diagnosis

a. ADNFLE
b. night terror
c. nightmare

A

Answer is B, night terror

• 39% of children 
• Frequent in 3% of children 
• Trying to wake child will prolonged event 
• Clonazepam or zopiclone if extreme 
• Timing: 60-90 minutes into sleep  Child unable to be comforted, goes straight back to sleep, cannot recall event
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9
Q

A child lets out a scream at 4 am.
When their parents go to comfort them they respond to questions by stating that they were being chased.

Is the diagnosis

a. ADNFLE
b. night terror
c. nightmare

A

C = nightmare

Occurs during REM sleep
Child can be woken/comforted
Will recall the event unlike in night terror

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10
Q

A child shouts in sleep and makes abnormal pelvic thrusting and ‘bicycling’ movements
One of their parents reports that they used to do a similar thing

Is the diagnosis

a. ADNFLE
b. night terror
c. nightmare

A

A = autosomal dominant nocturnal frontal lobe epilepsy

Stereotyped movements - pelvic thrusts, bicycling
May vocalise
May walk
May have aura
rare

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11
Q

What are the features of Klein-Levin syndrome?

A

Every 6 weeks, patient will get 2 weeks of hyperphagia, hypersomnolence and increased libido

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12
Q

A patient undergoes MSLT (multiple sleep latency testing)
Their time is consistently <8 minutes
They have multiple episodes of SOREM (= sleep onset REM)
What is the diagnosis?

A

• Constant sleepiness
○ Narcolepsy (with and without cataplexy)
§ MSLT: short latency plus >= 2 REM onsets
○ Primary idiopathic hypersomnia with long or normal sleep time
§ MSLT: short latency but <2 REM onsets

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13
Q

What is the first line treatment for narcolepsy?

A

Methylphenidate or dexamphetamine
If fails this can trial modafanil (not funded for 1st line)

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14
Q

What is the frequency of CF carrier status amongst Caucasian populations?

A

1/25
Generally overall incidence of homozygosity this group is 1/2500 - 3000

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15
Q

What class of mutation is F508 and what does this mean/

A

Class 2
Misfolded protein is arrested by endoplasmic reticulum quality control and is not trafficked to the cell membrane

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16
Q

What is the second most common CF causing mutation in Australia and what class is it?

A

G551D
Class 3
Some protein reaches surface, but no function

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17
Q

What class of mutation is G542X and how does this mutation cause disease?

A

Class 1
No functional protein produced

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18
Q

Describe the pathophysiology of a class 4 cystic fibrosis causing mutation

A

protein reaches plasma membrane, has some function, which is impaired (decreased conductance)
Example would be R117H(5T)

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19
Q

Describe type 5 CF causing mutations

A

Type 5: reduced total CFTR protein at cell surface
A455E, 2789+5G-A

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20
Q

Describe type 6 CF causing mutations

A

Type 6: CFTR less stable (increased turnover)
120del23, N287Y, 432delTC

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21
Q

Which electrolytes pass outwards through the CFTR?

A

Chloride and bicarbonate

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22
Q

Which of the following is not a potential cause of a false positive sweat chloride test?

a. Klinefelters
b. Primary ciliary dyskinesia
c. congenital adrenal hyperplasia
d. eczema
e. hypothyroidism

A

Answer: B, PCD not a cause

Conditions causing false positives: eczema, malnutrition, anorexia, CAH, adrenal insufficiency, glucose-6-phosphatase deficiency, hypothyroidism, hypoparathyroidism, glycogen storage disorders, MPS, diabetes insipidus, klinefelters
False negative: malnutrition, skin oedema, mineralcorticoid use

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23
Q

Which of the following 2 answers represent a sweat test result that is suggestive of cystic fibrosis?

A

A and B
>60mmol/L considered diagnostic, some centres use higher cut-offs. A borderline test result is more likely in a patient with retained pancreatic function.
30 - 60 may be considered positive in children <6 months of age
Sweat weight needs to be >75-100 mg

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24
Q

What is the organism most commonly isolated in young patients with cystic fibrosis?

A

Staphylococcus aureus
Haemophilus influenzae also common, but not as much

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25
Q

Which of the following is not an appropriate agent for use in the treatment of pseudomonas aeruginosa disease in cystic fibrosis?

a. colistin
b. ceftazadime
c. tobramycin
d. doxycycline
e. aztreonam

A

Correct answer is D, p.aeruginosa would be resistant

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26
Q

In which CF causing mutation would monoagent ivacaftor be appropriate?

A

G551D
Class 3 - some protein on membrane surface, but does not function
Ivacaftor is a ‘potentiator’
‘Iva opened up’

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27
Q

What type of CFTR modulator is lumacaftor?

A

This is a ‘corrector’ which counteracts protein misfolding and trafficking (ie: active in class II mutations)
Lumacaftor - ‘loom’ (protein sowing/folding)
Tezacaftor - ‘tears a lumacaftor a new one’ - 2nd generation corrector

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28
Q

Which 2 CFTR modulators make up Orkambi?

A

Lumacaftor/ivacaftor
3-4% improvement in FEV1
Symdeko is similar but is tezacaftor/ivacaftor
Approved in F508
· Respiratory adverse effects: chest tightness, dyspnoea, bronchospasm, increased cough, pneumothorax
Non-respiratory adverse effects: GI upset, myalgia with increase in CPK, fatigue, headache

29
Q

What 3 drugs make up trikafta?

A

Correctors: tezacaftor, elaxacaftor
Potentiator: ivacaftor

· In the first trial also showed improvements in sweat chloride, pulmonary exacerbations and BMI
· Adverse effects
o Common: headaches, URTI, abdominal pain, diarrhoea, rashes, increased liver enzymes (ALT, AST), nasal congestion, increased creatine kinase
Associated with cataracts (hence opthal screening prior)
FDA approved in 12 year old patients with at least 1 F508del

30
Q

Using a pulse oximeter, an oxygen saturation of 90%, under normal metabolic conditions, corresponds to a PaO2 of approximately

A

Pulse oximetry is accurate above 90% oxyhaemoglobin saturation, but much less so below 70%, and inaccurate below 50%.

Pulse oximeters estimate arterial haemoglobin oxygen saturation (SaO2) and not arterial oxygen tension (PaO2). Because of the shape of the oxygen-dissociation curve, large changes in PaO2 may occur at the extremes of the curve with minimal change in SaO2.

They have a response time of 10–30 seconds, depending on the signal averaging time, circulation time and the site of the probe.

A simple way to remember the sats and the corresponding PaO2 is:

40-50-60 70-80-90… which basically means that…

PaO2 is 40mmHg at SpO2 70%

PaO2 is 50mmHg at SpO2 80%

PaO2 is 60mmHg at SpO2 90%

31
Q

What is the current definition of chronic neonatal lung disease?

A

Currently defined as dependence on supplemental oxygen at 36 weeks corrected gestational age
Previously described as oxygen dependency at 28 days of age (in the TSANZ position statement this definition is used for the term ‘bronchopulmonary dysplasia’)

32
Q

What target saturations are currently recommended for ex premature infants, and what is the evidence base underlying this?

A

93-95%
BOOST 1 trial - no difference between 91 - 94 and 95-98%
BOOST 2 - higher mortality with 85-89% target

33
Q

What is the fraction of inspired oxygen in most commercial airline cabins?

A

These cabins are pressurised to an equivalent of 1500 - 2400 m
This corresponds to an oxygen tension of 15-17%
Need fitness to fly test - show not going to have SpO2 <85%

34
Q

What is the significance of exhaled nitric oxide?

A

Eosinophilic inflammation
Suggests asthma as diagnosis

35
Q

You see an asthmatic child in clinic who is using a mask with a spacer
At what age should they transition to using a spacer without a mask?

A

2-4 possible to transition
5 - should transition

36
Q

Long acting B-agonists are potentially associated with increased risk of severe exacerbations in children. What mechanism underlies this?

A

Reduction in the density of B-2 receptors

https://www.nps.org.au/australian-prescriber/articles/long-acting-beta2-agonists-for-childhood-asthma#:~:text=Safety%20of%20long%2Dacting%20beta2%20agonists%20in%20children&text=These%20observations%20are%20consistent%20with,used%20with%20concomitant%20inhaled%20corticosteroids.

37
Q

What is the mechanism of action of omalizumab?

A

Binds free IgE and prevents it from binding to eosinophils
Reduces mediator release and allergic inflammation

38
Q

What is the mechanisms of action of mepoluzimab in asthma?

A

Anti IL-5

39
Q

You are reviewing a 13 year old patient with mild intermittent asthma and note they are currently only prescribed reliever therapy with salbutamol (ventolin)
You would like to commence SMART therapy

Which combination therapy would be appropriate?

a. fluticasone/salmeterol
b. budesonide/formoterol
c. fluticasone and vilanterol

A

a. fluticasone/salmeterol - Seretide - INCORRECT
b. budesonide/formoterol - Symbicort - CORRECT
c. fluticasone and vilanterol - Breo Ellipta

Only budesonide/formoterol products are approved because formoterol has a quick enough onset to be used as a reliever

40
Q

In the context of SMART therapy, what are the advantages of using a turbuhaler?

A

Symbicort tubuhaler is a DPI. Children can attempt a DPI at around age 5-6, most should be able to use by 8
It is more portable and has a lower carbon footprint
The disadvantage is the slightly more difficult technique
Note that the turbuhaler is 200/6 and rapihaler (ie MDI0 100/3) so starting preventer dose is 1 puff BD for former, 2 puffs BD for latter

41
Q

Describe the sequence of the tracheobronchial tree in the correct order
IE: trachea…

A

Trachea –> right and left main bronchus –> secondary or lobar bronchus –> segmental or tertiary bronchi –> bronchioles (conducting –> terminal –> respiratory)

42
Q

At what point in the tracheobronchial tree does the conducting zone cease and the gas exchange zone begin?

A

Transition between the terminal bronchioles and the respiratory bronchioles
Terminal - this is why they are called the terminal bronchioles
Respiratory - have alveoli in their walls

42
Q

At what point in the tracheobronchial tree does the conducting zone cease and the gas exchange zone begin?

A

Transition between the terminal bronchioles and the respiratory bronchioles
Terminal - this is why they are called the terminal bronchioles
Respiratory - have alveoli in their walls

43
Q

Which bronchopulmonary segments tends to have fluid entrapment in a chronically bed-ridden individual?

A

Superior segment of the inferior lobe
The tertiary bronchus that supplies this tends to be oriented quite posteriorly and is thus a dependant area if the patient is recumbent

44
Q

Describe the layers of the alveolar-capillary membrane

A

Squamous cell of the type 1 pneumocyte
Shared basement membrane
Squamous cell of the capillary
0.5 microns thick

45
Q

Name the stages of lung development?

A

Embryological
Pseudo glandular
Cannicular
Saccular
Alveolar

46
Q

What is the A-a gradient

A

The alveolar to arterial (A-a) oxygen gradient is a common measure of oxygenation (“A” denotes alveolar and “a” denotes arterial oxygenation)
It is the difference between the amount of the oxygen in the alveoli (ie, the alveolar oxygen tension [PAO2]) and the amount of oxygen dissolved in the plasma (PaO2)

47
Q

In a healthy adult lung, what is the typical gross ration of ventilation to perfusion

A

0.8
4L/minute of ventilation
5L/minute of perfusion

48
Q

In what lobe of the lung is VQ mismatch highest?

A

Upper lobes

49
Q

True or false: carbon monoxide shifts the oxygen-haemoglobin saturation curve to the LEFT

A

This is true
Carbon monoxide binds Hb and allosterically causes change in shape resulting in impaired oxygen offloading at tissues

50
Q

Which part of the airways does FEF25-75% represent?

A

Smaller conducting airways

51
Q

You review a patient in clinic with severe cystic fibrosis. Their FEV1 is 28%, FEV1/FVC ratio is reduced
You note a low FVC

Does this reflect?

a. restrictive lung disease
b. mixed obstructive and restrictive lung disease
c. severe obstructive lung disease

A

Answer is C
This is most likely severe obstructive lung disease with pseudo-restriction

52
Q

What disease process is shown in this flow volume loop?

A

Restrictive
Can be shown as this also (see attached)

53
Q

What type of pathology is shown in this image?

A

Fixed upper airway obstruction

54
Q

What type of pathology is shown in this image?

A

Variable extra-thoracic airway obstruction

55
Q

What type of pathology is shown in this image?

A

Variable intrathoracic airway obstruction

56
Q

what type of pathology is shown in this image?

A

Thoracic inlet obstruction

57
Q

which congenital surfactant deficiency is known for causing fatal lung disease during infancy?

A

Surfactant protein B deficiency
B = bad

58
Q

which congenital surfactant deficiency is known for causing interstitial lung disease during childhood?

A

Surfactant protein C deficiency
C = childhood

59
Q

what electrolyte abnormalities would you expect in a CF patient on a hot day?

A

People with cystic fibrosis are more vulnerable to electrolyte disturbance from dehydration due to pseudo-Bartter’s syndrome (hyponatremic, hypokalemic, hypochloremic metabolic alkalosis). This occurs because sweat production is different in children with cystic fibrosis.

Sweat is usually isotonic. However, in cystic fibrosis, sweat is hypertonic due to increased sodium and chloride loss in the sweat due to CFTR dysfunction. Under usual conditions, this increased tonicity is easily corrected. However, on hot days, there is an increased volume of sweat production which leads to hyponatremia. Hyponatremia triggers secondary hyperaldosteronism causing excessive renal potassium losses. Metabolic alkalosis occurs because there is intravascular volume depletion causing an increased extracellular concentration of bicarbonate.

60
Q

Management of new pseudomonas infection in a child with cystic fibrosis

A

See card
2 x anti-pseudomonal agents from different classes

61
Q

a 4 year old patient present with hypocalcaemic seizure and is found to have raised phosphate levels and normal vitmain D and parathyroid hormone

They also have short stature, short 4th and 5th metacarpals and a round face

what genetic locus is likely to be involved?

a. GNAS
b. AIRE
c. SHOX
d. NEMO

A

GNAS - codes the alpha subunit of the G protein coupled receptor signal

If somatic mutations in some tissues causes McCune Alright syndrome
Albright’s hereditary osteodystrophy - brachydactyly, round face, intellectual disability, subcutaneous ossification
Some aspects of GNAS are bi-allelically expressed, however, some of the hormone signalling pathways are normally paternally silenced and maternally expressed
So if you inherit the mutation from your Mother you would have PTH (+/- TSH and GH) resistance, if inherited from Father would not
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4417430/

62
Q

baby is being worked up for congenital hypothyroidism
high TSH and low T4
99-pertechnetate scan shows good uptake, slightly larger gland
what disorder is suggested?

A

Dyshormonogenesis/enzymatic defect

63
Q

baby is being worked up for congenital hypothyroidism
high TSH and low T4
iodine-123 scan shows decreased uptake, normally located gland
what are the differentials?

A

TSH receptor blocking antibody
Inactivating TSH receptor mutation
Sodium/iodine symporter mutation

64
Q

what is pendred syndrome?

A

Pendred syndrome – congenital hypothyroidism associated with sensorineural hearing defects; would expect low T4 and elevated TSH

65
Q

Boy with short stature, jittery, difficulties at school
T$ and T3 elevated
TSH 4 (normal <4)

A

Key points
Mutation in TSH receptor gene – usually THRB gene
Incidence 1/40,000 M=F
Autosomal dominant (Note: Nelson’s autosomal recessive)
Pathophysiology
Resistance of pituitary thyrotrophs to thyroid hormone 🡪 ↑ TSH 🡪 ↑ T4 and T3 production from the thyroid gland
The elevated levels of T3 and T4 do not downregulate TSH production as the pituitary gland is resistant
Outcome = ↑ T3 and T4 and normal or ↑ TSH
TSH secreted by these patients is rich in sialic acid and has increased bioactivity cf. normal TSH – results in high T4 and T3 and goitre even though TSH concentrations are normal or slightly high
Effect on metabolism = Increased thyroid hormone secretion compensates for resistance – most patients are clinically euthyroid
Clinical manifestations
HIGHLY HETEROGENOUS
Hallmark = paucity of symptoms and signs of thyroid dysfunction despite high T4 and T3
Most common clinical findings
Goitre
Hyperactivity
Tachycardia
Hearing loss – may be due to recurrent ear infections (more common in RTH)
Sensorineural deafness is common among individuals with TR-beta gene deletion
May have some signs of hypo or hyperthyroidism – variable and when present often inconstant
If hypothyroidism present
Growth retardation

66
Q

what is the primary mediator of short stature in chronic kidney disease?

A

low IGF-1
Growth hormone will be relatively high/normal

67
Q

what findings on chest drain fluid suggest chylothorax

A

High trigs
>80% lymphocytes

Tom Respiratory & sleep (1 decks)

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