Respiratory

Question 1

Expected findings in obstructive spirometry?

Answer 1

FEV1:FVC ratio <70% (below the 1.64 Z score)
FEV1 <80% predicted
FVC normal
Concave scooping of flow-volume loop

Question 2

Expected findings in restrictive spirometry

Answer 2

Normal FEV1: FVC ratio
Low FVC

Question 3

Expected findings in mixed restrictive and obstructive spirometry

Answer 3

Reduced FEV1:FVC
Low FEV1 and
Low FVC

Question 4

Expected flow-volume loop in fixed central or upper airway obstruction

Answer 4

Box shaped

(eg subglottic stenosis or tracheal stenosis)

Question 5

Expected flow-volume loop in variable extrathoracic airway obstruction?

Answer 5

Flattened inspiratory loop
(eg laryngeal paralysis or vocal cord dysfunction)

Question 6

Expected flow-volume loop with variable intrathoracic airway obstruction (Eg tracheomalacia)

Answer 6

Flattended expiratory loop with relatively normal inspiratory loop

Question 7

Causes of oxygen dissociation curve shift to the right (reduced affinity)?

Answer 7

Increased temp
Increased 2,3 DPG
Increased H+ (acidosis)

Question 8

Causes of oxygen dissociation curve shift to left (higher affinity of Hb for O2)

Answer 8

Carbon monoxide
Lower temp
Lower 2,3 DPG
Lower H+

Question 9

Most common organism resulting in bronchiolitis obliterans?

Answer 9

Adenovirus (serotypes 3, 7, 21)

Question 10

CT findings in bronchiolitis obliterans

Answer 10

Mosaic attenuation
Gas trapping
Hyperinflation
Central bronchiectasis (tram tracks)

Question 11

What is Idiopathic Pulmonary Haemosiderosis?

Answer 11

Accumulation of haemosiderin inside pulmonary macrophages that occurs after recurrent haemoptysis without a clear cause
- Managed with steroids or immunosuppression

Question 12

Most common class of CFTR mutation?

Answer 12

Class II (delta F508)
- “trafficking” mutation due to missense (CFTR protein is misfolded, keeping it from moving to cell surface)

Question 13

What class of mutation are G551D and S549N?

Answer 13

Class III mutation - “gating mutation”
- CFTR moves to the cell surface but the channel gate does not open properly

Question 14

Which classes of CFTR mutation are usually associated with pancreatic insufficiency?

Answer 14

Class 1-3

Question 15

What defines CFPID?

Answer 15

1. Sweat chloride <30 and 2 variants and at least 1 of unclear phenotype
2. Sweat chloride 30-59 and 1 CF causing variant

Question 16

What defines CF?

Answer 16

1. Sweat chloride >60 OR
2. Sweat chloride 30-59 with 2 CF causing variants

Question 17

Causes of false positive sweat test

Answer 17

Adrenal insufficiency
Hypothyroidism and hypoparathyroidism
GSD
MPS
G6PD deficiency
Diabetes Insipidus

Question 18

IV treatment of pseudomonas in CF?

Answer 18

IV tobramycin + ceftazidime or
IV meropenem

Question 19

How does Ivacaftor work?

Answer 19

partially corrects the Cl- channel defect, allowing Cl- transport - helpful for class III “gating” mutations

Question 20

Which classes of mutation can Trikafta be used for

Answer 20

Class 2-6 (incl class 2 - delta F508)

Question 21

How does trikafta work?

Answer 21

Contains corrector and potentiators”

Helps more protein reach the cell surface and to stay open for longer at the cell surface

Question 22

Side effects of Trikafta

Answer 22

*Rash incl SJS
*Liver dysfunction
*Headache
*Cataracts
* Mood disturbance

Question 23

Benefits of Trikafta

Answer 23

Improved lung function
Reduced exacerbations
Improved BMI
Reduced sweat chloride
Reduced symptom scores

Question 24

How much creon per g of fat?

Answer 24

Creon 10 000 units for each 6-8g fat

Question 25

Poor prognostic factors in CF

Answer 25

Burkholderia cepacia Pseudomonas CFRD Malnutrition Frequent exacerbations Female gender

Question 26

A mass of abnormal, non-functioning lung tissue isolated from normal functioning lung tissue and fed by systemic arteries, often found in left lower lobe

Answer 26

Pulmonary sequestration

Question 27

Stages of sleep

Answer 27

N1= transition to light sleep N2= light sleep (k complexes and spindles) N3= deep sleep - very hard to rouse REM= decreased tone, rapid eye movement, vivid dreams

Question 28

Definition of apnoea

Answer 28

>/= 90% decrease in baseline flow for 2 or more respiratory cycles Obstructive = continued effort Central= absence of effort (no chest movement) + desaturated >3% or arousal

Question 29

Definition of hypopnoea

Answer 29

>/= 30% decrease in baseline flow for 2 or more respiratory cycles with desaturated or arousal

Question 30

Genetic mutation in congenital central hypoventilation syndrome?

Answer 30

PHOX2B (autosomal dominant)

Question 31

Conditions associated with congenital central hypoventilation syndrome?

Answer 31

Hirschsprung disease Failure to develop fever when unwell Neural crest tumours eg neuroblastoma Iris problems Cardiac arrhythmia

Question 32

Management of narcolepsy

Answer 32

Dexamphetamine or methylphenidate 2nd line = modafinil (with authority)

Question 33

Management of cataplexy

Answer 33

Fluoxetine or venlafaxine BEST treatment is sodium oxybate (GHB) but safety concerns

Question 34

Tests for narcolepsy

Answer 34

1. Multiple sleep latency test (MSLT): need abnormal latency (<8mins) and 2+ REM onsets 2. HLA DR2, DRB1*0602 3. Low Hypocretin1 levels in CSF (rarely done)

Question 35

What stage of sleep do night terrors occur in?

Answer 35

N3 More common in first part of night

Question 36

What is sleep onset association disorder?

Answer 36

A type of behavioural insomnia where child needs certain conditions to fall asleep eg being rocked or fed and can't self-soothe. Unable to get back to sleep without same routine

Question 37

What is limit-setting sleep disorder?

Answer 37

A type of behavioural insomnia seen in young children where they protest and actively resist going to bed -> delayed sleep onset and inadequate sleep

Question 38

What is 'delayed sleep phase'

Answer 38

Delay in going to sleep seen in adolescent due to decreased and delayed melatonin peak and exacerbated by after school sport/homework/texting, TV etc

Question 39

Difference between night terror and nightmare?

Answer 39

Night terror - child unable to be comforted - goes straight back to sleep - cannot recall event - occurs in Non-REM sleep (N3)

Question 40

Mild, moderate and severe OSA by OAHI

Answer 40

Mild: 1-5/hr Moderate: 5-10/hr Severe: >10/hr

Question 41

Which stage of sleep do sleep talking, bruxism and periodic leg movements occur?

Answer 41

Both REM and NREM sleep

Question 42

MOA of omalizumab?

Answer 42

Anti-IgE

Question 43

MOA of mepolizumab?

Answer 43

Anti- IL5

Question 44

MOA of dupilumab?

Answer 44

Anti-IL4 and IL13

Question 45

What would you expect the FeNO to be in asthma?

Answer 45

FeNO usually elevated (>25ppb) in asthma

Question 46

What is considered as positive bronchodilator response on spirometry?

Answer 46

Increase in FEV1 of >/= 12% and/or 200ml

Question 47

Low dose flixotide?

Answer 47

100microg /day

Question 48

Standard dose flixotide?

Answer 48

200-250microg/day

Question 49

Step up approach in a 5-11yr old with asthma?

Answer 49

1. Low dose ICS 2. Standard dose ICS/LABA eg Symbicort or breo 3. Add montelukast if poor control 4. High dose ICS/LABA and refer to respiratory (may need to consider biologic)

Question 50

Tests for primary ciliary dyskinesia

Answer 50

- Nasal NO (low) - screening test - Electron microscopy of nasal or bronchial biopsy - Videomicroscopy analysis of nasal brush biopsy - Genetics (only identifies 50-60%)

Question 51

Features of Kartagener syndrome?

Answer 51

1. PCD 2. Situs inversus 3. Chronic sinusitis

Question 52

4 features of chronic rinosinusitis?

Answer 52

1. facial pain 2. nasal obstruction 3. nasal mucopurulent drainage 4. cough

Question 53

Findings on pleural fluid analysis in empyema?

Answer 53

High protein High LDH Low glucose

Question 54

Difference between transudative and exudative (eg empyema) pleural effusions?

Answer 54

Exudative effusions will have high protein and LDH