Respiratory

Question 1

Bronchiectasis CXR findings

Answer 1

Peribronchial thickening Retained secretions Compensatory emphysema Tubular / cystic

Question 2

Bronchiectasis causes

Answer 2

Childhood infections - Measles, pertussis Bronchial obstruction Chronic aspiration Kartagener’s syndrome - Bronchiectasis, dextrocardia, absent frontal sinuses Immune deficiency

Question 3

Causes of pulmonary oedema

Answer 3

Cardiac failure Fluid overload Cerebral CVA, head injury, raised ICP Drowning Aspiration Radiation Thoracocentesis Drugs Inhaled toxins (smoke, hydrocarbons_ Shock lung

Question 4

What is asthma?

Answer 4

• Heterogenous disease usually characterised by
  
  • Chronic airway inflammation
  • Wheeze, SOB, chest tightness. cough
  • Variable expiratory airflow limitation
• No SINGLE gold standard diagnostic test

Question 5

What are the factors that increase the risk of asthma?

Answer 5

Exposure to paracetamol + antibiotics in 1st year of life

Question 6

What is the pathophysiology of asthma?

Answer 6

• Smooth muscle hyperplasia
• Thick tenacious mucus plugs
• Thickened basement membrane
• Muscus oedema –> abnormal mucociliary clearance
• Eosinophili of the submucosa + secretions
• Increased mast cells in smooth muscle

Question 7

Answer 7

Raised exhaled nitric oxide

• Elevated in ANYONE with atopy + eczema
  
  • Therefore need a even high level if patient you are testing also has eczema

Question 8

Answer 8

B

Question 9

Asthma spirometry

Answer 9

Question 10

Other than spirometry can you use in asthma?

Answer 10

• Direct challenges are more likely to provoke a response

Question 11

At what age do we diagnose asthma?

Question 12

Does inhaled corticosteroids affect growth?

Answer 12

YES

Frequent use can reduce height by 1.5-2cm

Question 13

What questions do you ask for asthma control?

Answer 13

• Daytime asthma symptoms > 2 /week
• Any night waking?
• Reliever needed for symptoms >2/weel
• Any activity limitation due to asthma

Question 14

Spacer vs mask use in asthma

• When to use + effect on lung deposition

Answer 14

Question 15

Answer 15

Check inhaler technique + adherence

Montelukast side effect: ADHD like behaviour, suicidal ideation, sleep difficulties

Question 17

What is the treatment algorithm for preschool wheeze with frequent symptoms?

Answer 17

If this does not work requires further investigation for CF/ bronchiectasis / immune deficiency

Question 18

Why don’t we give LABA’s without ICS?

Answer 18

LABA increase the risk of sudden death + severe exacerbations therefore ALWAYS need to give them with ICS

Question 19

What is the treatment algorithm for asthma?

Answer 19

Question 20

Answer 20

D: ultrafine doubles the potency

Fluticasone 1/2 as potent as beclomethasone

Question 21

What are the concerns about SABA only treatment in asthma?

Answer 21

• SABA only treatment is associated with increased risk of exacerbations + lower lung function
• Regular use increases allergic responses + airway inflammation
• Beta receptor down regulation, decreased bronchoprotection, rebound hyperresponsiveness, decreased bronchodilatory response
• Over use of SABA (e.g. > 3 canisters / year) is associated with increased risk of severe exacerbations
• >12 canisters / year is associated with increased risk of asthma related death

Question 22

Answer 22

A wrong- would make it worse

B wrong- binds to IgE in the blood but doesn’t work if IgE levels are > 1500

• Would give this if IgE wasn’t so high

C Mepolizumab

Question 23

What is the mechanism of action of omalizumab (anti IgE)

Answer 23

10 % risk of anaphylaxis

1st time the dose is given need to be in hospital for 4hrs post

Subseqent doses requires to wait 25min post administration

Question 24

What is the mechanism of action of mepolizumab, reslizumab + benralizumab (anti IL5)?

Answer 24

Question 25

How do sleep disorders usually present in childhood?

Answer 25

* Anxious * Irritable * Impulsive * Inattentive * Poort concentration

Question 26

What are the screening questions for sleep disorders?

Answer 26

**BEARS** Bedtime problems Excess daytime sleepiness Awakening at night Regularity + duration of sleep Snoring + apnoea

Question 27

What happens during sleep?

Answer 27

* Reduction in BP * Reduction in temperature * Decreased tone; * Upper airway resistance increases (x 2 in REM) * Tidal volume decreases (1/2 in REM) * Any impairment of ventilation when awake will be worse when asleep

Question 28

What is the function of sleep

Answer 28

* Immune restoration * Hormonal/growth * Consolidate learning

Question 29

What are the different stages of sleep?

Answer 29

* **REM**"dream sleep" decreased tone (chin EMG), rapid eye movement, partial paralysis, vivid dreams, irregular breathing, increased upper airway resistance, decreased tiday volume * **N1** transition to light sleep, easily roused * **N2** light sleep (K complexes + spindles) * **N3** deep sleep or "slow wave sleep", still, very hard to rouse. Very regular breathing.

Question 30

How do you tell if it is a breathing or sleeping question on PSG?

Answer 30

* Breathing: 1 or 2 min / page * Sleeping: 30 sec / page * Are there eye movements (Yes- awake or REM) * What is the chin tone? (Big- awake, small- REM) * Are there K complexes or spindles?

Question 31

What stage of sleep is this?

Answer 31

**N2 sleep:** sleep spindles + K complexes present + medium chine EMG

Question 32

What stage of sleep is this?

Answer 32

**N3 sleep**

Question 33

What stage of sleep is this?

Answer 33

**REM sleep**

Question 34

Normal sleeping times

Answer 34

**Approximate = 16 - age (from birth to sleep time of 8-9hrs)** * Birth = 16 hours * 2 years = 14 hours * 6 years = 10 hrs

Question 35

Normal napping

Answer 35

1 year old = 2 naps / day 2 year old = 1 nap / day 5year old = no napping

Question 37

Type of waves in each phase of wakefullness/ sleep

Answer 37

**BATS** **D**rink **B**lood * Awake- **beta** waves * Relaxed wakefulness- **alpha** waves * NON REM * N1: **theta** waves * N2: **spindles** (memory consolidation) + K complexes * N3: **delta** waves * REM: **beta** waves * Dreams * Active sympathetic system: rise in HR + body temp * Eyes rapidly move but rest of body is paralysed

Question 38

What is the MOST restful sleep?

Answer 38

**N3** (slow wave sleep) Entered into relatively quickly after the onset of sleep Preserved in the face of reduced total sleep Increases (rebounds) after a night of restricted sleep

Question 39

During which stage of sleep do night terrors + bed wetting occur?

Answer 39

Slow wave sleep: N3 (during in SWS peaks during early childhood which explains why sleep waking / bedwetting / night terrors is MOST common in preschool age children)

Question 40

When during the night do you have more - Non- REM sleep - REM sleep

Question 41

Does REM sleep % increase or decrease as you age?

Question 42

What hormones are deficient in narcolepsy?

Answer 42

Hypocretin + orexin

Question 43

Characteristics of narcolepsy

Answer 43

Multiple sleep latency SHORT with \> 2 REM onsets * Abnormal latency \< 8 min _Features_ * Can have cataplexy = sudden + temporary loss of muscle tone * Sleep paralysis * Hypnagogic hallucinations * Sleepiness _Narcolepsy special tests_ * HLA for DR2, DRB1 1501, DQB1\*0602 * Hypcretin levels = low

Question 44

What is the definition of apnoea?

Answer 44

\>90% decrease baseline flow for \> 2 breaths * Obstructive: continued effort * Central: absence of effort * Mixed: starts central + ends obstructive

Question 45

What is the defition of hypopnoea?

Answer 45

* \>30% decreased baseline flow for 2 or more respiratory cycles with O2 desat of 3%

Question 46

What sort of apnoea is this?

Answer 46

**CENTRAL** You have see complete absence of effort + air flow (which lasts \> 2 breaths) just prior to the desat

Question 47

What sort of apnoea is this?

Answer 47

**OBSTRUCTIVE** Also note paradoxical movement of abdomen + thorax

Question 48

What event is this?

Answer 48

Hypopnoea \> 30% decrease in airflow signal that lasts \> 90% duration of \> 2 normal breaths

Question 49

What is the prevalence of OSA in children?

Answer 49

3%

Question 50

How is OSA best diagnosed?

Answer 50

Polysomnograpghy = gold standard

Question 51

What are the most common consequences of OSA in children? * Decrements of memory + attention * Excessive daytime tiredness * FTT * Pulmonary hypertension

Answer 51

**Decrements of memory + attention** - Causes defects of executive function

Question 53

What are the adverse effects of OSA?

Answer 53

* Defects in executive functioning * Disrupts sleep * Hard to wake * Daytime sleepiness * Inattention, impulsive, irritable, poor concentration, poor academic performance * Hypertension * Elevated lipids * Insulin resistance * Severe: FTT, pulmonary hypertension, cor pulmonale

Question 54

What is the role of oximetry in diagnosis OSA?

Answer 54

* Good positive predictive value * Poor negative predictive value * IF negative test is inconclusive * CANNT differentiate between central + obstructive sleep apnoea * MUST have a 2 second averaging time Cluster = \>5 desats within 30 minute period Positive = \> 3 clusters with \> 3 desaturations (\>3% drop in SpO2)

Question 55

A 3 year old is referred because his parents are concerned about his breathing during sleep. He snores loudly most nights, is restless and wakes frequently. During the day he is a mouth breather and said to be "on the go" all the time. Investigation? Treatment?

Answer 55

Polysomnography Adenotonsillectomy: 80-85% near or total cure (50% if obese) * Improves behaviour + QOL * Does NOT improve IQ Nasal corticosteroids HALVE the PSG score + may cure mild OSA CPAP: reserved for moderate pos surgical disease, severe on waitlist or contraindications to surgery

Question 56

What are the risk factors for respiratory compromise post adenotonsillectomy?

Answer 56

* Obesity * Age \< 3 years * Underlying syndrome * Severe OSA * Complications of OSA

Question 57

Obesity + OSA

Answer 57

* In childhood normally an issue of tonsils + adenoids not obesity but this is changing due to the obesity pandemic * Obesity causes; * Decreased lung volumes (fat pressing of the chest) * Decreased airway tension * Fatty deposits around pharynx narrowing the airway * Combined effect increased pharyngeal collapsibility

Question 58

Can adenoids + tonsils re-grow

Answer 58

Adenoids can Tonsils CANNOT

Question 59

What is the definition of respiratory failure?

Answer 59

* Type 1: hypoxic * O2 \<8 (60mmHg) * Type 2: hypercarbnoea * CO2 \> 7kPA (55mmHg)

Question 60

Which of the following has NIV NOT been shown to improve NMD? * Episodes of chest infection * Quality of life * Blood gas abnormalities * Survival * Spirometry

Answer 60

**Spirometry**

Question 61

Which of the following is NOT an indication for NIV in NMD? * Recurrent chest infection * Abnormal lung function * Symptoms of chronic alveolar hypoventilation/ respiratory failure * Elevated daytime CO2

Answer 61

Abnormal lung function

Question 62

What is the mean survival in NMD for; * Nocturnal hypoventilation * Daytime respiratory failure

Answer 62

* Nocturnal hypoventilation: 50 months * Daytime respiratory failure: 9-10 months

Question 63

Congenital central hypoventilation syndrome (Ondine's curse)

Answer 63

* Hypoventilation which is WORSE during sleep * Autosomal dominant * PHOX2B mutation * Polyalanine repeats proportional to severity * Normal 20/20 * Mild 20/24 20/25 * Severe 20/27 20/33 * Absent or near absent response to hypercarbia + hypoxia awake + asleep * Usually presents in infancy with apniea + cyanosis * Ventilation is BETTER awake + in REM due to cortical inputs * Ventilation is WORSE in non-REM * MOST infants require trachey-ventilation * Don't have drive to breathe themselves

Question 64

What is Ondine's curse associated with?

Answer 64

* Failure to develop fever when unwell * Cardiac arrhythmias * Sudden death * Hirschprung's * Neural crest tumours * Iris problems *Require lifelong ventilation + supervision*

Question 65

What is the association between Prader Willisyndrome + sudden death?

Answer 65

* ~60% deaths related to respiratory problems (mainly infection) * Hypoventilation due to * Hypotonia * Kyphoscoliosis * Decreased CO2 responsiveness * 75% of deaths occur within first 7-9 months of commencing growth hormone therapy * Due to IGF1 adenotonsillar hypertrophy * Therefore should be assessed for OSA prior to commencing GH + then early in treatment * Adenotonsillectomy not always curative in PWS * Children with normal PSG may still suffer sudden death. They may also die on CPAP

Question 66

Carrier frequency of CF

Answer 66

1/25

Question 67

What is the 1st + 2nd most common cause of bronchiectasis?

Answer 67

CF is the MOST common Primary immunodeficiency is the 2nd most common

Question 68

Which of the following drugs may be taken up to 8 hours prior to a histamine bronchial challenge? * Montelucast * Salbutamol * Salmeterol * Nedocromil sodium * Theophylline

Answer 68

Salbutamol

Question 70

An eight year old boy is admitted with an episode of acute asthma. His oxygen saturation is 92% in air. He is prescribed prednisolone 1mg/kg and hourly salbutamol 12 puffs. Three hours later his O2 saturation in air has dropped to 89%. He looks well and is less distressed than when admitted. He has widespread wheeze with good air entry. Based on these findings what is the most appropriate next step in his management? * Add ipratropium bromide * Blood gas analysis * Change to IV salbutamol * CXR to rule out a pneumothorax * Reduce frequency of salbutamol

Answer 70

Reduce frequency of salbutamol * Need to look at the whole picture it seems he is clinically improving * Don't just focus on O2 saturations * Reducing frequency of salbutamol may improve V/Q mismatch which will improve O2 saturations

Question 71

Omalizumab is a biological product (monoclonal antibody) used in the treatment of severe asthma. What is its specific molecular target? * Circulating IgE * Eosinophilic basic protein * Eotaxin 1 * IL5 * Leukocyte immunoglobulin like receptor 7

Answer 71

Circulating IgE

Question 72

An 8 year old boy with previous eczema is now having exercise induced wheeze. Which finding below is the most consistent with a diagnosis of asthma? * FVC 85%, FEV1 83% FEV1/FVC 95% * Exhaled NO 70 (normal 5-20) * Widespread bronchial wall thickening on CXR * Nasal NO 80 (normal \>250) * Total serum IgE 125

Answer 72

* FVC 85%, FEV1 83% FEV1/FVC 95%: more of a restrictive pattern * **Exhaled NO 70 (normal 5-20)** * Widespread bronchial wall thickening on CXR: more in keeping with bronchiectasis * Nasal NO 80 (normal \>250): low which is seen in PCD * Total serum IgE 125

Question 73

What are the 5 phases of lung development?

Answer 73

Embryonic (26 days to 6 weeks gestation) Pseudoglandular (6 to 16 weeks gestation) Canalicular (16-28 weeks gestation) Saccular (28-36 weeks gestation) Alveolar (36 weeks through to infancy)

Question 74

Key features of PJP infection

Answer 74

* O2 saturation strikingly lower than would be expected from symptoms * Unicellular fungi * PCP is identified by staining of cysts with methenamine silver nitrate, or toluidine blue, which stains dark bodies within the cysts * Pneumocystis pneumonia is most often observed when the CD4+ T helper cell count falls below 200 cells/mm3. CD4+ T cells are absolutely critical for resolution of Pneumocystis, having an essential role in the recruitment and activation of effector cells against the organism.

Question 75

Of the following, the strongest risk factor associated with SIDS is: Smoking by the mother in the prenatal period Smoking by the father in the prenatal period Exposure of the infant to environmental tobacco smoke after he or she is born Smoking by the mother prenatally only in association with alcohol use There is no association between smoking and SIDS

Answer 75

**Smoking by the mother in the prenatal period**

Question 76

A 4 wk old healthy-appearing term infant is evaluated in the office for stridor, which has persisted since birth. The noisy breathing is accompanied by moderate signs of inspiratory obstruction including suprasternal and subcostal retractions. He feeds adequately and is gaining weight but frequently spits up. The most likely cause of his symptoms is: Bronchomalacia Vascular ring Laryngomalacia Tonsil and adenoid hypertrophy Bronchiolitis

Answer 76

**Laryngomalacia**

Question 77

A 4 yr old boy presents with sore throat and fever of sudden onset. He has difficulty swallowing and his breathing is labored. He is drooling and sitting upright and leaning forward in a tripod position. What is the appropriate next step in patient management? Complete blood cell count and blood culture followed by immediate prophylactic intravenous antibiotics Lateral radiograph of the neck Dose of oral dexamethasone Direct laryngoscopy in the operating room Complete physical examination including inspection of the oral cavity

Answer 77

**Direct laryngoscopy in the operating room**

Question 78

A 2 yr old boy is presented to the emergency department at 3 AM with a chief complaint of fever and cough. His respiratory rate is 36 breaths/min, his temperature is 39°C, and his pulse oximetry reading is 96%. On physical examination he has a barky cough and stridor only with crying. He is well hydrated, able to drink, and consolable. What is the appropriate next step in patient management? Nasal washing for influenza virus and respiratory syncytial virus Lateral radiograph of the neck Nebulized racemic epinephrine Complete blood cell count and blood culture Dose of dexamethasone

Answer 78

**Dose of dexamethasone** The effectiveness of oral corticosteroids in viral croup is well established. Corticosteroids decrease the edema in the laryngeal mucosa through their anti-inflammatory action. Oral steroids are beneficial, even in mild croup, as measured by reduced hospitalization, shorter duration of hospitalization, and reduced need for subsequent interventions such as epinephrine administration. Most studies that demonstrated the efficacy of oral dexamethasone used a single dose of 0.6 mg/kg; a dose as low as 0.15 mg/ kg may be just as effective. Intramuscular dexamethasone and nebulized budesonide have an equivalent clinical effect; oral dosing of dexamethasone is as effective as intramuscular administration. A single dose of oral prednisolone is less effective. There are no controlled studies examining the effectiveness of multiple doses of corticosteroids.