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Paediatrics > NZ Respiratory > Flashcards

NZ Respiratory Flashcards

1
Q

Respiratory abnormalities associated with T21

A
  1. Pulmonary hypertension
  2. Bronchial stenosis
  3. Subpleural cysts
  4. Alveolar simplification
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2
Q

Diaphragmatic Hernia

A
  1. 1: 2-3000
  2. Contralateral lung is usually affected
  3. Long term nutritional problems are common
  4. Malrotation occurs in 30-60%
  5. R sided in 12% of cases
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3
Q

Poor prognostic factors for CF

A
  • Malnutrition
  • Pseudomonas
  • Burkholderia cepacia
  • Diabetes
  • Frequent exacerbations
  • Female gender
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4
Q

Indications for lung transplant in CF

A
  • FEV1 <30% predicted
  • Poor nutritional status
  • Poor exercise tolerance
  • Rapid decline in lung function
  • Major life threatening complications
  • QOL issues
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5
Q

Stages of sleep in childhood

A

N1: transition to light sleep, easily roused
N2: light sleep, k complexes and spindles
N3: deep sleep or “slow wave sleep”, still, very hard to rouse, very regular breathing
REM: “dream sleep”, decreased tone, rapid eye movements, partial paralysis, vivid dreams, irregular breathing, increased upper airway resistance, decr. tidal volume
–> Occurs during the latter half of sleep

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6
Q

Stages of sleep in newborns

A

Different due to decreased myelination

  1. Active sleep: equivalent to REM sleep
  2. Quiet sleep: equivalent to N3
  3. Indeterminate sleep
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7
Q

Respiratory events: apnoeas

A

Apnoea >90% decrease in baseline flow for 2 or more respiratory cycles
- Obstructive: continued effort
- Central: absence of effort + desat >3% or arousal
- Mixed: starts central ends obstructive
Hypopnoea >30% decreased baseline flow for 2 or more resp cycles with desat or arousal
- More commonly seen in children with OSA, usually don’t have apnoeas, but partial obstruction or hypopnoea

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8
Q

Normal number of obstructive events per hour of sleep

A

<1/hr (<5/hr in adults)

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9
Q

Respiratory events on polysomnograph

A
  1. Drop in nasal flow
  2. Look at effort bands (thorax and abdo) to see if there is increased effort of breathing - obstructive vs central
  3. Confirm arousal or desaturation
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10
Q

Parasomnias

A
  • Occur in N3 stage of sleep –> disturbance occurs, brain half awake
  • -> To fully wake someone up and stop the event, need to go back go sleep. Waking someone up will prolong the event
  • Includes confusional arousals, night terrors, sleep- walking
  • Usually occurs 60-90min into sleep, usually 1 or 2 per night, positive FHx
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11
Q

DDx for parasomnias

A

Nightmares
Frontal lobe seizures - very stereotyped events, features of pointing, pelvic thrusting, more likely to stand, sudden offset

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12
Q

Night terror vs nightmare

A
  1. Night terror: N3 phase, occurs 60-90min into sleep (predictable), not awake!, unable to be settled/comforted, unable to recall the event (like a seizure!)
    - If extreme, can trial clonazepam or zopiclone
  2. Nightmare: REM phase, no specific timeframe, able to be comforted as they can wake up from event, takes ~20min to settle, can recall events
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13
Q

Anticipatory waking

A

Since parasomnias are predictable in their timing, wake up the child 30min before event to reset the sleep cycle
- Events may still occur

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14
Q

Periodic limb movement disorder

A
  • Non-sleep stage specific disorder
  • Part of restless legs syndrome
  • Increased frequency of periodic limb movement during sleep –> disturbs pt from sleep
  • Due to partial iron deficiency in basal ganglia
  • Tx with Fe supp and aim for ferritin >50
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15
Q

Narcolepsy

A
  • Hypersomnia disorder
  • Genetics: HLA-DR2, DRB11501, DQA0102, DQB1*0602
  • Hypocretin-1 level in CSF (low hypocretin/orexin)
  • Features:
  • -> Short latency (<8min) with REM sleep during the day
  • -> Cataplexy: sudden loss of muscle tone
  • -> Sleep paralysis and hypnagogic hallucinations
  • Mx: good routine, scheduled naps, stimulants
  • -> Stim: ritalin, modafinil
  • -> Cataplexy: sodium oxybate, tricyclics, SSRIs, venlafaxine
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16
Q

Associations between sleep and obesity

A

Sleep deprivation associated w/ inc obesity

  • Sleep dep –> for next 48hrs, increased hunger and caloric intake
  • Other factors: altered thermoregulation and increased fatigue –> reduced energy expenditure
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17
Q

Behavioural insomnia

A

Mostly mixed phenotype

  1. Sleep association type i.e. children need to learn to fall asleep, need to have positive associations with bed time
  2. Limit setting disorder type i.e. naughty children with parents who can’t set limits
    - Mx:
    - -> Exclude physiologic causes for night waking e.g. OSA, GORD, asthma, eczema etc
    - -> Sleep hygiene and better associations
    - -> Sudden or graduated extinction: let them cry, parents don’t interact with children
    - -> Fading with positive bedtime routines: 20min of positive, quiet activity before bed, move bed time backwards by 15min each night
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18
Q

ADHD and insomnia

A

Decreased sleeping, increased movement in sleep
Increased sleep latency, more restless sleep
More night-time wakings
Difficulty waking/irritability with daytime sleepiness
- Is stimulant the problem? Trial children on atomoxetine instead of ritalin, consider clonidine or melatonin
- Behavioural therapy

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19
Q

Autism and insomnia

A
  • 44-83% have sleeping problems (significant)
  • Issues: difficulty settling, waking during the night for hours, early morning waking
  • Mx:
  • -> Behavioural therapy: 50% find it helpful
  • -> Melatonin: helps with sleep onset, but does not help decrease night time or early morning waking due to short half life
  • -> Melatonin SE: binds receptors in gonads, in animals - affected puberty and fertility
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20
Q

Delayed sleep phase treatment for a petulant adolescent

A
  1. Sleep hygiene
    - Dim light before bed, no texting, no computer/TV in room
  2. Bright light when awake
  3. Advance bed time by 15min every 3 nights
  4. Melatonin can be used as adjuvant
    N.B. Sunlight can move body clock by 2hrs, melatonin can move body clock by 20min!
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21
Q

Adverse effects of OSA

A
  1. Causes defects of executive function (MC): impulsiveness, inattention/poor concentration, memory decrements
  2. Disrupts sleep - tiredness/irritability
  3. Hard to wake or daytime sleepiness
  4. HTN, elevated lipids, insulin resistance
  5. Severe: FTT, pulmonary HTN, cor pulmonale
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22
Q

Major features of OSA in Hx

A
  1. Most sensitive: Snoring - 50%
  2. Increased odds ratio: (strength of association)
    - Frequent mouth breathing asleep + awake
    - Witnessed pauses or apnoea
    - Struggling to breath
    - Parents feel they have to poke child
  3. Ex-prem, FHx
  4. Minor features: restless sleep, sweating, cough or vomit in sleep, morning headache
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23
Q

Examination findings on OSA

A

Tonsil size and mouth breathing (increased OR)
Nasal patency: hyponasal speech
High arched or narrow palate

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24
Q

Investigations for OSA

A

Diagnosis = polysomnography
Risk stratification for adenotonsillectomy = oximetry
- Motion-resistant oximeter with 2 second averaging time
- 5 or more clusters of desats to <80%, associated elevated early AM CO2 on blood gas

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25
Q

Treatment options for OSA

A
  1. Adenotonsillectomy
  2. Nasal corticosteroids
    - Halve PSG score and cure mild OSA (shrinks adenoids)
  3. CPAP
    - For moderate post-surgical disease, severe but on waitlist, surgical contraindication
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26
Q

Outcomes from T&As in OSA pts

A
  • 80-85% of near or total cure
  • 50% cure in obese or severe OSAs
  • Benefits: improves behaviour and QoL, but not necessarily IQ and executive function
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27
Q

Risk factors for respiratory compromise (e.g. apnoeas, atelectasis) post-T&As

A
  • Syndromes: T21, DMD
  • Morbid obesity
  • Severe OSA
  • <3yo
  • Complications of OSA present prior to T and As –> FTT or cor pulmonale
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28
Q

Which pt groups are at risk of developing OSA?

A
  • Large tonsils/adenoids
  • Obese children
  • Floppy children: T21, NMD
  • Narrowing/crowding of upper airway: craniosynostosis, Pierre Robin sequence, Crouzon syndrome
    I.e. more at risk of obstructing upper airway
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29
Q

Obesity and pathophysiology of OSA

A
  • Decreased lung volumes due to increased pressure on chest and diaphragm squashes from below
  • Fatty deposit around pharynx narrowing airway further
  • Combined effect –> small airway volume –> increased pharyngeal collapsibility and airway resistance –> obstruction
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30
Q

OSA in obese pts increases risk of…

A
  • Metabolic syndrome and insulin resistance
  • HTN (sympathetic surge)
  • Stroke > MI (vibration of snoring of carotid arteries)
  • Poor sleep –> increases appetite
  • Pro-inflammatory state
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31
Q

ABG Rules: AG - HCO3

A

(Change in AG) - (Change in HCO3) in high anion gap metabolic acidosis to identify co-existing NAGMA and met alkalosis
= (Calc. AG - (N) AG) - (Measured HCO3 - (N) HCO3) = >6 is abnormal
- Remember: the ratio ofchange in acidic anion and HCO3 should be 1:1 i.e. addition of 1 acid –> decrease in 1 base (neutralisation)
- If change in AG > change in HCO3 = concurrent met alkalosis, there must have been more base to start with to have a smaller decr in HCO3 c.f. bigger increase in AG
- If change in HCO3 > AG = concurrent NAGMA, there must be another acidotic process going on, as change in AG alone can’t explain sig decrease in HCO3

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32
Q

ABG Rules: Compensation

A
  1. When PCO2 and HCO3 move in the same direction, compensation is present
    - -> Metabolic compensation implies chronicity as renal comp takes hours/days to complete
  2. Resp acid/alkalosis: every 10mmHg change in CO2 (40) –> HCO3 (24) to change by 1 (acute) or 4 (chronic)
  3. Metabolic acidosis:
    - pCO2 = (1.5xHCO3) + 8 (+/-2) OR
    - Last 2 digits of pH = CO2 (e.g. pH 7.19 = CO2 19)
  4. Metabolic alkalosis: pCO2 ~50 is the max that can be expected for compensation, otherwise expect a second process
    - (0.7 x HCO3) + 20
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33
Q

Exhaled ntric oxide (FeNO)

A
  • NO usually exhaled in breath. In asthma, high levels of NO + high levels of inducible NO synthase on epithelial cells of airways
  • High FeNO suggests up-regulation of airway inflammation and presence of eosinophilic inflammation
  • FeNO can decrease with corticosteroid therapy –> non-invasive method to MONITOR response to anti-inflammatory Tx
  • NOT conclusive for asthma diagnosis
  • Asthma = >35ppb in school aged children
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34
Q

Ix for primary ciliary dyskinesia

A
  1. Nasal nitric oxide
    - Abnormal = <250ppb or <75nL/min (incorporates flow)
    - True PCD <100ppb
  2. Ciliary biopsy/brushings - look at ultrastructure of cilia
  3. Genetic testing
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35
Q

DDx for nodular miliary pattern

= innumerable, small 1-4 mm pulmonary nodules scattered throughout the lungs

A
  1. TB
  2. PJP
  3. CMV
  4. Lymphocytic interstitial pneumonia
  5. Metastatic lesions (e.g. osteosarcoma)
  6. Sarcoidosis
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36
Q

When does the risk of pulmonary hypoplasia increase?

A

Oligohydramnios prior to 26 weeks gestation

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37
Q

Lung development: pseudoglandular stage

A

Weeks 6-16: development of lower conducting airways

  • By this stage all major elements of the lung have formed, EXCEPT for those involved in gas exchange
  • Aberrant development: bronchogenic cysts, congenital lobar emphysema, CDH
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38
Q

Lung development: canalicular stage

A

Weeks 16-26: formation of acini

  • Bronchial lumen and terminal bronchials become larger
  • Lung tissue becomes highly vascular, pulm capillaries occur
  • Terminal bronchioles form respiratory bronchioles –> divide into primordial alveolar ducts
  • -> The alveolar sacs allow beginning of gas transport
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39
Q

Lung development: terminal sac stage

A

Weeks 26-36: refinement of blood-air barrier & surfactant

  • Many more alveoli develop and epithelium become very thin (type I pneumocytes)
  • Capillaries begin to bulge into sacs - increases alveolar-blood barrier surface area
  • Development and maturation of surfactant system
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40
Q

Lung development: alveolar phase

A

Weeks 36 to 3yrs: alveolar proliferation + development

  • Saccules become alveoli and alveoli attain polyhedral shape
  • Thinning of acinar walls, dissipation of interstitium and invagination of alveoli by pulmonary capillary
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41
Q

Role of betamethasone

A

Increases surfactant production

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42
Q

Lung development: embryonic phase

A

Weeks 3-6: development of proximal airways

  • Laryngotracheal groove develops caudal to fourth pair of pharyngeal pouch
  • Lung bud (laryngotracheal diverticulum) arises from foregut day 21 to 26
  • Aberrant development:
  • -> Laryngeal web or atresia: failure to recanalise the larynx after 10th week
  • -> Tracheal agenesis, tracheal stenosis: usually assoc. w/ variants of tracheo-oesophageal fistula
  • -> TOF: incomplete division of cranial foregut into resp and oesophageal parts during 4th wk
  • -> Pulmonary sequestration (accessory lung bud)
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43
Q

Gurgling upper airway noises

A

DDx:

  • Lingual cyst
  • Pharyngeal airway abnormality
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44
Q

Pulmonary hypoplasia

A
  1. Usually associated with congenital abnormality or pregnancy Cx that prevents adequate development of lung, airways and alveoli
  2. Causes:
    - Physial constraint: CDH (can cause hypoplasia on contralateral side if squashed), CCAM, pleural effusions due to hydrops, thoracic dystrophy
    - Oligohydramnios: foetal renal insuff, PROM
    - Isolated
  3. Presentation:
    - Resp insufficiency
    - PPHN
    - Tachypnoea/distress with viral infections in infancy - mild presentations
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45
Q

Management of pulmonary sequestration

A

Risk of complications from unresected sequestration outweighs risks of surgery

  • With extralobar seq, if infected, may require lobar resection
  • Very small risk of cancer in remaining abnormal lung tissue
  • Thorascopic resection
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46
Q

Where are pulmonary sequestrations located?

A

Most commonly, LLL - 90% extralobar, >60% intralobar

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47
Q

Complications associated with pulmonary sequestrations

A

Receives blood supply from systemic circulation (thoracic or abdo aorta)

  • Recurrent infections and pneumonia
  • Respiratory distress
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48
Q

CPAM

A
  • Benign hamartomas or dysplastic tumours (mixed w/ N lung tissue) –> overgrowth of terminal bronchioles in glandular pattern
  • Communicates with lung, normal pulmonary arterial and venous supply
  • Lower lobes > upper lobes, R=L, rarely multilobar
  • Antenatal USS: polyhydramnios, mediastinal shift, pleural effusion, hydrops (IVC comp)
  • Macrocystic dz assoc w/ better prognosis (type I)
  • Presentation: severity depends on degree of mediastinal compression and secondary pulmonary hypoplasia
  • Main risks: PTX, infection and bronchiectasis, small risk of malignant transformation –> all determined by size + degree of compression
  • Tx: plan with HRCT, surgical resection <12mth, TRUE spont resolution rare (can get smaller/disappear on XR)
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49
Q

Congenital lobar emphysema

A
  • Secondary to bronchial obstruction (other pathology) –> distension –> irreversible destruction of alveolar septae
  • Expiratory air trapping within affected lobe –> overdistension of affected lobe + compression of adjacent structures
  • -> Atelectasis of ipsilateral normal lung, mediastinal shift
  • Normal lung parenchyma, assoc. bronchial collapse
  • Main clue: lungs are NOT inflated on antenatal scans as air trapping occurs postnatally
  • Presentation: MC incidental finding on CXR for neonates with RDS
  • LUL (40-50%), RML (30-40%), RUL (20%)
  • Screen with echo as 15% associated with CHDs
  • Mx depends on Sx and lung function: observe vs surgical excision
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50
Q

Bronchgenic cysts

A
  • Abnormal budding from tracheobronchoeal diverticulum before 16 wks –> can be found anywhere along conducting airways
  • Single, unilocular, R is MC
  • Presentation: incidental finding vs tachypnoea, wheezing +/- FTT if compression of adjacent structures + lobar collapse
  • -> Older children: infections
  • Cx: infection, if ruptures –> PTX or haemoptysis, malignant transformation
  • Mx: Surgical resection
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51
Q

Airway abnormalities and syndromes

Choanal atresia

A

CHARGE syndrome

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52
Q

Airway abnormalities and syndromes

Down Syndrome

A

Subglottic stenosis

Tracheal stenosis

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53
Q

Airway abnormalities and syndromes

Velocardiofacial syndrome

A

Submucosal clefts

Laryngeal webs

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54
Q

Airway abnormalities and associations

Tracheal stenosis

A

Tracheo-oesophageal fistula - pre and post-operatively

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55
Q

CFSPID

A

CF sweat test positive, inconclusive diagnosis
- 2 genes positive, normal sweat test
- 1 gene positive (hetero) or no genes, indeterminate/borderline sweat test
At risk of “delayed” CF

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56
Q

CF Genetics: Class 1

- Chromosome 7q

A
  • Lack of CFTR synthesis
  • Premature stop codon due to nonsense mutation –> truncated protein synthesised in nucleus
  • W1282X, G542X, R553X
57
Q

CF Genetics: Class 2

  • Chromosome 7q
  • 90% in Australia
A
  • Defect in protein processing
  • Deletion of phenylalnine at position 508 - CFTR protein degraded in ER and does not reach the membrane
  • Del508, N1303K
58
Q

CF Genetics: Class 3

- Chromosome 7q

A
  • Gating mutation
  • CFTR transported to apical membrane, but defect in intracellular arms of the protein, does not respond to stimulus –> lack of channel opening
  • G551D
59
Q

CF Genetics: Class 4

- Chromosome 7q

A
  • Protein defect - missense mutation changes structure of protein pore
  • CFTR transported to membrane, but restricts the movement of Cl- across the pore –> conductance defect
  • R117H, R347P
60
Q

CF Genetics: Class 5

- Chromosome 7q

A
  • Reduced protein synthesis
  • Very small number, most do not cause significant lung disease
  • 278+5G –> A, A455E
61
Q

CF Genetics: Class 6

- Chromosome 7q

A
  • Increased protein turnover
  • Functional protein, but unstable structure –> quickly degraded
  • 120del 23, N287Y
62
Q

PFTs in CF

A
  • Decreased FEF25-75: indicates early obstructive disease
  • FEV1: predictor of survival, monitor of disease progression
  • -> FEV1 <30% - lung transplant, expected 50% 2yr mortality
  • RV and FRC increased early in course of lung disease
63
Q

Bacteria most commonly responsible for exacerbations in very young pts

A
  • S. aureus
  • P. aeruginosa (non-mucoid)
  • Hib
  • S. pneumoniae
  • E. coli
64
Q

Organisms associated with rapid decline in PFTs, increased morbidity and mortality

A
  • Chronic pseudomonas infection (mucoid type)
  • MRSA
  • Burkholderia cepacia
  • -> Cepacia syndrome: significant hypoxia + rapid resp failure, haemorrhagic pneumonia with rapid progression to death, overwhelmin sepsis
65
Q

What sort of reaction is ABPA due to?

A

Hypersensitivity Type 1 and 3

- Aspergillus cell wall Ag causes allergic response

66
Q

Risk factors for developing ABPA

A
  • Inhaled antibiotic use
  • Atopy
  • Male
  • Chronic infection
  • Previous pseudomonas infection
  • Poor lung function
  • HLA-DR2 and DR5
67
Q

5 Criteria for ABPA diagnosis

A
  1. Acute or subacute deterioration of lung function with chronic cough, wheeze, reduced ET, decline in PFTs, increased sputum not attributable to other cause
  2. IgE >1000 (unless receiving steroids)
  3. Immediate cutaneous reactivity to aspergillus (>3mm with surrounding erythema)
  4. Precipitating Abs to aspergillus or serum IgG to asper
  5. New findings on CXR or CT not improving with physio and ABx
68
Q

Management of ABPA

A
  1. Oral prednisolone 1-2mg/kg or methylpred pulses
  2. Antifungal agents: itraconazole, vori or posi for 6/12 with steroids
  3. If resistant to steroids, omalizumab - Anti-IgE
  4. Treat asthma-like Sx with ICS
  5. Monitor progress with serial IgE levels
69
Q

Importance of Scedosporium apiospermum infection in CF

A

= Fungus with poor sensitivity to antifungal drugs

- Its presence can PREVENT transplant

70
Q

Genetic defects in CF can confer protection against which bug?

A

Salmonella Typhi

71
Q

Prophylactic macrolide therapy in CF (Azithromycin)

A
  • Very effective anti-inflammatory agent esp in pts with chronic P. aeruginosa
  • Increase in FVC and FEV1 in pts with chronic pseudo
  • Reduce rate of pulmonary exac requiring IV therapy
  • Reduced hospital admissions
  • Resulted in small increases in weight
  • Azithromycin has long intracellular half life –> thrice weekly dosing
72
Q

Pulmozyme

A
  • Breaks down sulphide bond in mucus molecules
  • Improves lung function and reduce exacerbation rates, maintains effectiveness for >2yrs
  • <5yo: strict criteria
  • -> Severe course with >3 admissions
  • -> Sig. bronchiectasis, severe bronchiolitis with wheeze, severe impairment on spirometry
  • > 5yo: 1mth trial showing 10% increase in FEV1 for continued therapy
73
Q

Neb Hypertonic Saline 3-7%

A
  • Cough stimulant, can induce bronchospasm
  • Salbutamol 15min before therapy
  • Discontinue if tachypnoea, wheeze etc
74
Q

Side effect of high dose pancreatic enzyme replacement therapy or “overdosing”

A

High doses > 6000u lipase/kg: Fibrosing colonopathy, predispose to DIOS

75
Q

Most common microangiopathic Cx of CFRD

A
  1. Neuropathy 55%
  2. Gastropathy 50%
  3. Retinopathay 16%
  4. Microalbuminuria 14%
76
Q

Do CF pts go into DKA?

A

No - persistance of endogenous insluin production and impaired glucagon response prevents DKA

77
Q

CFRD is more severe with concomitant…

A

CF-related liver disease

78
Q

Clues to CFRD

A
  • Delayed puberty, decreased growth velocity/failure to gain weight
  • Worsening PFTs
  • Polyuria and polydipsia
  • Presence of significant hyperglycemia may indicate P. aeruginosa or Burkholderia infection/colonisation
79
Q

RIsk factors for CF-related liver disease

A
  1. Hx of meconium ileus
  2. Pancreatic insufficiency
  3. Male gender
  4. Mutations class I-III
    Cholestasis –> focal biliary cirrhosis –> multifocal biliary cirrhosis
80
Q

Neonatal cholestasis in CF pts

A

Presents with conjugated hyperbili and hepatomegaly

  • Usually resolves and is not a predictor of later CFLD
  • However, this may change if prolonged TPN or surgery related to meconium ileus
81
Q

CF related liver disease

A
  1. Focal biliary cirrhosis (20-40%): asymptomatic, persistently elevated LFTs, hepatomegaly, develops within first 12yrs of life
  2. Multilobar biliary cirrhosis (5-10%): cirrhosis, Cx by portal HTN, GI bleed, varices and nutritional deficiencies
  3. Hepatic steatosis (10-60%): relationship btwn steatosis and FBC is unclear; usually thought to be benign - inflammatory features of steatohepatitis don’t develop
82
Q

RF for hepatic steatosis

A
  1. Malnutrition
  2. Essential fatty acid deficiency
  3. Alcohol ingestion
83
Q

Pancreatic insufficiency and CF

A
  • 60% are pancreatic insufficient at time of diagnosis

- At 1yr post-Dx, 95% are insufficient

84
Q

Symptoms more likely to suggest asthma

A
  1. Wheeze (most sensitive and specific Sx of asthma)
  2. Chest tightness
  3. Nocturnal cough
  4. Breathlessness

Particularly if:

  • Worse at night or early morning
  • Obvious triggers e.g viral infection (MC 85%), exercise, cold air, allergen, stress, aspirin
  • Seasonal or recurrent

Other features: personal Hx of atopy or FHx of asthma

85
Q

Objective testing that is more likely suggestive of asthma

A
  • Obvious response to bronchodilators
  • Raised blood eosinophilia and FeNO
  • Bronchial hyper-responsiveness on challenge testing
  • Obstructive pattern on spirometry (low FEV1, low FEV1/FVC, low FEF25-75%)
86
Q

Features NOT suggestive of asthma

A
  • Isolated cough without wheeze
  • Wet, productive cough
  • No wheeze or repeatedly normal exam during an “exacerbation”
  • No response to trial of asthma treatment
  • Normal spirometry or peak expiratory flow when SYMPTOMATIC
87
Q

LABAs

A

A B2-receptor genotype (Arg16 polymorphism in the B2-receptor gene) pre-disposes children with asthma to down-regulation/internalisation of the B2-receptor –> tolerance and paradoxical bronchospasm –> SABA will not provide desired response
- Increased risk of mortality with LABA monotherapy

88
Q

What determines disease severity in adolescence and adulthood?

A

Disease severity in childhood

89
Q

Natural history of asthma

A
  • Children who lose overt Sx still have persistent signs of airway obstruction on PFTs and history
  • Recurrence of asthma after years of freedom from overt Sx can occur
  • Airway inflammation can persist in the absence of Sx
  • -> Airways still show significant abnormalities and evidence of active inflammation
90
Q

Low dose vs high dose ICS

A
  • Daily dose that achieved 80-90% of maximum efficacy is low doses (Fluticasone 100microg/day/Budesonide 200microg/day/Beclomethasone 200microg/day)
  • More evidence to suggest that adding an extra agent provides increased benefit in Tx rather than increasing ICS dose
91
Q

Low dose ICS and growth

A
  • Can have small, temporary effect on growth that is NOT progressive and or cumulative
  • Esp seen in pre-pubertal children in first 1-2yrs of Tx
  • Long-term outcomes showed a difference of 0.7% in adult height (~1cm)
  • Severe, poorly controlled asthma can also affect growth!
92
Q

Montelukast

A

Leukotriene receptor antagonist

- Psychiatric side effects

93
Q

Cromones

A

Mast cell stabilisers

- Sodium cromoglicate, nedocromil sodium

94
Q

Omalizumab

A

Anti-IgE - prevents IgE binding to mast cells

- Subcut 2-5 weekly depending on response

95
Q

Mepolizumab

A

Anti-IL 5

96
Q

Tiotropium bromide

A

Long-acting muscarinic antagonist

97
Q

Best predictor for severe asthma exacerbation

A

Hospitalisation or ED visit for severe exacerbation of asthma in last year

98
Q

Features associated with increased risk of severe asthma exacerbation and/or death from asthma

A

Symptoms:
- Poorly controlled asthma
- Hospitalisation/ED for exac of asthma in last yr
- Extreme SABA use (>1 cannister/mth)
- ICU admission or intubation ever
- Requirement for long-term oral steroids
- Personal Hx of severe food allergy and anaphylaxis
Medication adherence:
- Poor technique or poor adherence to preventer use
Social:
- Alcohol/drug abuse in family, tobacco use, poor health literacy, low SES/financial hardship, poor follow up, unhealthy housing

99
Q

Protracted bacterial bronchitis

A
  • May be precipitated by URTI, prolonged to chronic cough (>2-8wk) that is wet with sputum production
  • On examination, WELL, no wheeze or atopy, no added sounds
  • Sputum/BAL: bacterial counts of >10^4 cfu/mL, Haemophilus influenzae (non-typable)
  • Normal CXR
  • Responds to prolonged course of PO augmentin 4-6/52, with resolution seen in 2/52
100
Q

Most significant predictor of lung function (FEV1) decline in bronchiectasis

A

Frequency of hospital exacerbations

- Predicted FEV1 decline of 1.95% per admission

101
Q

Characteristic findings on HRCT for bronchiectasis

A
  1. Enlarged internal bronchial wall diameter relative to adjacent pulmonary = signet ring sign
  2. Lack of bronchial tapering as it reaches periphery
  3. Bronchi seen in lung periphery
  4. Bronchial wall thicking, mucous plugging or impaction
  5. Mosaic perfusion defects
  6. Air trapping on expiration
102
Q

Most common bacteria isolated on sputum/BAL for bronchiectasis

A
  1. Non-typeable Haemophilus influenzae
  2. Streptoccocus pneumoniae
  3. Moraxella catarrhalis
103
Q

Definition of chronic suppurative lung disease

A
  1. Disease spectrum: PBB –> CSLD –> Bronchiectasis
  2. Respiratory Sx and signs that are seen in bronchiectasis without bronchial abnormalities seen on HRCT
    - Recurrent (3 or more eps) chronic wet, productive cough (>4wks)
    - Variable responses to prolonged courses of POABx
    - Exertional dyspnoea, wheeze and other Sx of airway hyperresponsiveness
    - Recurrent chest infection
    - Growth failure
    - Clubbing
    - Hyperinflation or chest wall deformity
104
Q

Central apnoeas in infancy

A
  • Occur frequently, especially during active (REM) sleep, upon body movement and during transition between wakefulness to sleep
  • Frequency of central apnoeas decrease after 1st yr of life
105
Q

Complications of OSA

A
  1. Cardiovascular abnormalities secondary to increased sympathetic drive (as a result of repetitive partial/complete upper airway obstruction)
    - RVH
    - Systemic HTN
    - Pulmonary HTN and cor pulmonale
  2. Defects in executive function, behavioural and learning disabilities
  3. Poor growth or obesity
  4. T2 respiratory failure
106
Q

Definition of respiratory failure

A
  1. Type 1: arterial PaO2 <60mmHg
    - Causes of hypoxaemia: hypoventilation, diffusion impairment, shunt, V/Q mismatch
  2. Type 2: arterial PCO2 >55mmHg
    - Causes of hypercarbia: hypoventilation, V/Q mismatch
107
Q

Gas exchange during sleep

A
  • Decreased hypoxic and hypercapneic drive during sleep
  • Normal child experience increase in PaCO2 (4-6mmHg) and small decrease in arterial oxyhaemoglobin saturation PaO2 (2mmHg) in sleep
  • Changes in gas exchange are exaggerated in children with underlying lung disorders, upper airway abN and NMDs
108
Q

NMDs associated with progressive respiratory muscle weakness and development of chronic respiratory failure. What are the causes?

A
  1. Airway obstruction
    - Bulbar obstruction –> hypoventilation
    - Atelectasis due to hypoventilation
  2. Increased lung damage
    - Increased secretions due to ineffective cough
    - Aspiration due to bulbar weakness
    - Recurrent chest infection
  3. Restrictive disease
    - Scoliosis –> smaller lung volume
    - Stiff chest wall –> reduced compliance
    - Resp muscle fatigue/weakness
109
Q

Manifestations of respiratory insufficiency and failure in NMDs

A
  1. Sleep disordered breathing –> hypoventilation in REM
    - Desaturations, but normal CO2
  2. Hypoventilation throughout sleep
    - CO2 >50
    - Need to start NIV
  3. Respiratory failure with high CO2 while awake
    - CO2 >55
    - Headache, exhaustion
110
Q 
Which of the following has NIV NOT been shown to improve in NMD?
A. Episodes of chest infection
B. QoL
C. Blood gas abnormalities
D. Survival
E. Spirometry
A

E. Spirometry

  • Increasing ventilation decreases CO2
  • Increases survival - gains 5yrs
111
Q

What’s the indicator to start investigating NMDs for consideration of NIV?

A

Abnormal pulmonary function tests –> start assessment by doing PSG

112
Q

What are the indications for starting NIV in NMD

A
  • Sx of chronic nocturnal hypoventilation or daytime respiratory failure/hypercapnoea
  • -> I.e. hypoventilation during entire sleep, not just REM
    1. Sleepiness
    2. Fatigue/lethargy
    3. Headaches
    4. Increased night time waking
  • Recurrent chest infections –> preventing recurrent admissions
113
Q

Approximate FVC when NMD pt starts hypoventilating throughout entire sleep

A

40%

114
Q

Approximate FVC when NMD pt is in chronic respiratory failure

A

25%

115
Q 
Which is the best indicator of the need for NIV in NMDs?
A. FVC <60%
B. FEV1 <40% 
C. Nocturnal CO2 higher than awake CO2 
D. Nocturnal CO2 >50mmHg
E. Max mouth pressure <30cmH2O
A

C. Nocturnal CO2 higher than awake

D. Nocturnal CO2 >50mmHg

116
Q

FVC predictors: <60%, 40% and 25%

A
  1. FVC <60%: predicts sleep disturbed breathing/REM hypoventilation
  2. FVC <40%: predicts nocturnal hypoventilation
  3. FVC <25%: predicts daytime hypercapnoea and chronic respiratory failure
  4. FVC <1L: 8% 5yr survival
117
Q

What prolongs survival in NMD?

A
  • Scoliosis repair

- Commencement of NIV

118
Q

Other predictors of disease progression in NMD

A
  1. Cough
    - Ineffective cough with cough peak flow <270L/min
    = Vulnerable to resp failure with minor LRTI
    - <160L/min: unable to clear own secretions
  2. Mouth and Sniff Inspiratory Pressures (SNIP):
    - MIP <30mmHg predicts SDB
    - MIP <19mmHg predicts nocturnal hypoventilation
119
Q

What is SNIP testing for?

A
  • Inspiratory muscle strength (i.e. respiratory muscle function) in pts with neuromuscular disease, ineffective cough, dyspnoea, reduced vital capacity
  • Monitors whether known respiratory muscle weakness has improved/stabilised/worsened
120
Q

Ondine’s Curse

A
  • PHOX2B gene repeat expansion - polyalanine repeats proportional to severity
  • -> Normal = 20 alanines on both alleles
  • -> Mild = 20/24 and 20/25
  • -> Severe = 20/27 and 20/33
  • Autonomic dysregulation and arrhythmias >27
  • Neural crest tumours (e.g. neuroblastoma) >29
  • Presents in newborns as:
    1. Hypoventilation, monotonous RR and shallow resps in sleep and awake, apnoeas + cyanosis
    2. Autonomic dysregulation
    3. Altered development of neural crest cell structures (e.g. Hirschprung’s) or neural crest tumours
121
Q

When is self ventilation better in Ondine’s Curse?

A
  1. Awake
  2. REM sleep due to cortical inputs
    - Worst in N3 sleep (deep)
122
Q

Respiratory findings in CCHS

A
  1. Absent or near absent CNS response to hypercarbia or hypoxia awake or asleep
    - -> Absent arousal from sleep
    - -> Absent perception of asphyxia
  2. Markedly reduced tidal volume during sleep –> hypercarbia
123
Q

Effects of hypoxic injury in CCHS

A
  1. Developmental delay from repeated hypoxic injury
  2. Low IQ
    - -> Well-ventilated child can have normal IQ
124
Q

Autonomic manifestations in CCHS

A
  1. Failure to mount fever during infection

2. Cardiac arrhythmias and sudden death

125
Q

Light’s Criteria: Pleural effusion (transduate) vs Empyema

1. Pleural effusion

A

Appearance: clear

  1. Cell count: <1000
  2. Cell type: lymphocytes, monocytes
  3. LDH: <200
  4. Pleural:serum LDH: <0.6
  5. Protein >3g: uncommon
  6. Pleural:serum protein: <0.5
  7. Glucose: normal
  8. pH: normal
  9. Gram stain: negative
  10. Cholesterol: -
  11. Pleural:serum chol: <0.3
126
Q

Light’s Criteria: Pleural effusion (transduate) vs Empyema

2. Empyma

A

Appearance: cloudy/purulent

  1. Cell count: >50,000
  2. Cell type: polymorphonuclear leukocytes
  3. LDH: >2/3 ULN for serum LDH
  4. Pleural:serum LDH: >0.6
  5. Protein >3g: common
  6. Pleural:serum protein: >0.5
  7. Glucose: low
  8. pH: low <7.10
  9. Gram stain: occasionally positive (less than 1/3)
  10. Cholesterol: >55
  11. Pleural:serum chol: >0.3
127
Q

Management of empyema

A
  1. IV antibiotics (MC S. aureus and S. pneumo)
  2. Nutritional status: consider NG, monitor albumin - low due to protein loss into pleural space
  3. Chest tube drainage, if pleural fluid septae with loculations detected on USS - fibrinolytics
    - Chest tube: large amt of fluid, compromised lung function, failure to respond to IVABs 48-72hrs, purulent
  4. VATS - debridgement of fibrinous pyogenic material , break down loculations and drain pus
128
Q

Fibrinolytics

A
  • Only useful for loculations
  • Streptokinase (higher risk of anaphylaxis) or urokinase
  • -> SE: haemorrhage
  • Does not: improve mortality, hospital stay, need for surgery, length of stay, reduce pus viscosity
  • Does: breakdown loculations
129
Q

VATS

A

Use of early VATS (<48hrs after admission) vs. late VATS (>48hrs) signficantly decreased length of hospitalisation

130
Q

Reliable marker of pleural inflammation

A

LDH

131
Q

Complications from empyema

A
  • Staph: bronchopleural fistulas, pyopneumothorax
  • Local: purulent pericarditis, pulmonary abscesses, peritonitis from extension, OM of ribs
  • Effusion may organise into thick peel: restrict lung expansion, persistent fever, temporary scoliosis
132
Q

Conditions predisposing to lung abscess

A
  1. Chronic aspiration e.g bulbar weakness, tracheo-oesophageal fistula, GORD, seizures
  2. FB aspiration
  3. Haematogenous seeding
  4. Pneumonia
  5. Immunodeficiencies
  6. Conditions with impaired mucociliary clearance
133
Q

Anaerobic organisms causing lung abscess

A

Bacteroides spp.
Fusobacterium spp.
Peptostreptococcus spp.

134
Q

Negative prognostic indicator for lung abscess

A

Presence of aerobic organisms esp secondary lung abscess

  • Streptococcus spp., S. aureus, E. coli, K. pneumoniae, P. aeruginosa
  • -> Have predilection on L side of lung
135
Q

Pneumatocoele vs lung abscess

A
  1. Pneumatocoele:
    - Complication of bacterial pneumonia
    - Thin, smooth wall, localised air collection
    - Cystic appearance with or without air-fluid level
    - Resolves spontaneously
  2. Lung abscess:
    - Thick walled
    - Low density centre progressing to air-fluid level
136
Q

What can hasten the recovery and shorten course of IVABx in lung abscesses?

A

Early CT-guided percutaneous aspiration or drainage of abscess

137
Q

Granulomatosis with polyangiitis

A
  • Systemic vasculitis with necrotising granulomas
  • Sinus, lungs, kidneys (necrotising glomerulonephritis)
  • Epistaxis, purulent nasal discharge, sinusitis, haemoptysis with pulmonary vasculitis, non-productive cough, haematuria
  • c-ANCA is 90% sens and spec; PR3-ANCA is absent in other granulomatous diseases
  • Mx: cyclophosphamide +/- steroids
138
Q

Goodpasture syndrome

A
  • Antiglomerular basement membrane antibody
  • Adolescent/young adult males
  • Lung, kidneys
  • Pulmonary haemosiderosis - abrupt haemoptysis or chronic, progressive dyspnoea, Fe deficiency anaemia, fatigue and recurrent cough. Restrictive pattern with pulmonary fibrosis
  • Lung Sx precedes renal Cx - glomerulonephritis with linear deposition of IgG and C3 on basement membranes
  • ANCA negative
  • Mx: immunosuppressives, plasmapheresis

Paediatrics (16 decks)

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