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FRACP 2023 Cram Homicrons Edition > Respiratory > Flashcards

Respiratory Flashcards

1
Q

Compare signs in upper airway infections

A

Croup- 3mo-5y, barking cough/stridor + coryza
EBV- teenager, malaise/fatigue, lymphadenopathy/HSM
Quinsy/paratonsillar abscess- teenager, trismus, hot potato voice, drooling
Epiglottitis- toxic, fever, drooling, stridor, unvaccinated
Bacterial tracheitis- toxic, fever, stridor
Retropharyngeal abscess- odynophagia/dysphagia, drooling, dysphonia, neck stiffness/torticollis

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2
Q

Indications for neuraminidase inhibitors in pneumonia

A

Inpatients with complicated disease thought to be related to influenza
Patients requiring ICU during influenza season
Child with suspected influenza at high risk of complications (congenital heart disease, respiratory disease, or immunosuppression)

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3
Q

Most common cause pneumonia <5yrs & >5yrs

A

<5yrs = viral most common (adeno, entero, RSV, parainfluenza etc), bacterial - ENT bugs- strep pneumo, moraxella, h.influenza, MSSA, s.pyogenes
>5yrs = strep. pneumo, mycoplasma/chlamydia (atypical)

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4
Q

Pathogenesis of round pneumonia

A

Inter-alveolar communication and collateral airways (pores of Kohn and canals of Lambert) develop as children age and allow air-drift between the parenchymal subsegments- infection walled between these spaces

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5
Q

Signs of empyema on pleural fluid tap

A

Empyema consistent with:
pH <7.0
Glucose <40 mg/DL
LDH >1000 IU
Protein level > 3.0 g/dL
Pleural fluid: serum protein ratio >0.5

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6
Q

Syndromes associated with laryngeal cleft

A

G syndrome: hypertelorism, hypospadias, stridor, and swallowing difficulties
Opitz-Frias syndrome: midline defects (as above)
Pallister-Hall syndrome
VACTER/VACTERL
CHARGE

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7
Q

Distinguish saccular laryngeal cyst from larygocoele

A

Saccular cyst distinguished from a laryngocele in that its lumen is isolated from the interior of the larynx and it contains mucous, not air

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8
Q

Stages of lung development

A

Embryonic (Wk 4 -5) lung buds from ventral foregut, lobar division
Pseudoglandular (5-17) Airway branching- vascular & conducting airways complete
Canalicular(16-25) - bronchioles/ ^ capillaries, alveolar Type 1&2 alveolar cells, air blood barrier
Saccular (24-40) Alveolar ducts, air sacs
True alveoli seen by 32 weeks gestation – recognizable by 36 weeks
Alveolar (32-36 week to 8 years)
Secondary septation occurs, marked increase of the number and size of capillaries and alveoli

Most development is postnatal - 85% of alveolarization occurs postnatally
15 million alveoli at birth (term) 🡪 300-600 million as an adult [exponentially increase until 2 years old

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9
Q

When do babies have true alveoli

A

True alveoli seen by 32 weeks gestation – recognizable by 36 weeks
Alveolar (32-36 week to 8 years)

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10
Q

Differences between CPAM/Sequestration

A

CPAM - dysplastic lung mixed w normal lung, blood supply from lungs, connected to bronchial tree
Sequestration- blood supply systemic, no connection to bronchial tree

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11
Q

Difference intrapulmonary vs extrapulmonary sequestration

A

Intrapulmonary 75-85%, lower lobe, no pleura
Extrapulmonary 15-25% boys, L) lung, 60% assoc w congenital defects- Diaphragmatic hernia
Colonic duplication
Vertebral anomalies
Pulmonary hypoplasia + CPAM
Scimitar syndrom

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12
Q

Compare Granulomatosis w Polyangiitis & Churg-Strauss Syndrome

A

Systemic disease leading to interstitial lung disease
GPA: 90% cANCA raised, pulm hemorrhage, rhinosinusitis- nasal discharge/septum degradation- skin, joint, conjunctivitis, peri/myocarditis can be rapidly progressive
Rx: long term immunosuppression, plasma exchange, rituximab
Churg-Strauss: pANCA raised, rhinitis/asthma, eosinophilia, multisystem vasculitis (kidneys, skin, heart)
Rx: steroids

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13
Q

Symptoms of sarcoidosis

A

> 8yrs
Idiopathic, multisystem inflammatory disorder
Skin rash, iridocyclitis, and arthritis- often without lung Dx
CXR shows changes despite no Sx 90%
Bloods: hypergamm, ACE elevated, high Ca, elevated CD4
Bx: non-caseating granulomas- predominant in upper lobes, ACE
Rx: 75% self resolve, if Stage 3 and above steroids +/- other immunosuppression

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13
Q

Symptoms of sarcoidosis

A

> 8yrs
Idiopathic, multisystem inflammatory disorder
Skin rash, iridocyclitis, and arthritis- often without lung Dx
CXR shows changes despite no Sx 90%
Bloods: hypergamm, ACE elevated, high Ca, elevated CD4
Bx: non-caseating granulomas- predominant in upper lobes, ACE
Rx: 75% self resolve, if Stage 3 and above steroids +/- other immunosuppression

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14
Q

Mx of chylothorax

A

50% resolve spontaneously
Enteral feeds
Medium chain triglycerides (MCTs) can be provided – absorbed directly into intestinal cells and transported directly to the liver via the portal vein – BYPASSING the thoracic duct
TPN if not improving 1-2wks
Consider pleuroperitoneal shunt

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15
Q

Composition chyle

A

TG in the form of chylomicrons
T lymphocytes
Electrolyte concentration similar to plasma
Immunoglobulins and fat soluble vitamins

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16
Q

Dx of this CXR?

A

CCAM
- Hamartomatous or dysplastic lung tissue mixed with normal lung
- Often unilateral
- Connected to pulmonary blood supply
Type 1 – macrocystic, single or several large cysts
Type 2 – microcystic, multiple small cysts
Type 3 – lesion is solid with bronchiole-like structures, poorest prognosis

Present with resp distress as neonate if large, recurrent infection in childhood if small

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17
Q

Dx of this CXR?

A

Eventration of diaphragm
- elevation, with paradoxical downwards motion in other diaphragm
-Assoc: congenital cardiac lesions, pulmonary sequestrations and chromosome trisomies

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18
Q

Dx of this CXR?

A

Congenital lobar emphysema
- Most commonly L) upper lung/upper lung fields
- Usually presents neonatal period with resp distress
- Airspace widening w mediastinal shift/contralateral atelectasis

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19
Q

Dx of this CXR?

A

CDH
- Bochaldek - most common, more L) sided, resp distress at birth/diagnosed antenatally
- Foramen of Morgani (2-6%)- anterolateral, R) sided, asymptomatic

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20
Q

Dx of this CXR?

A

Pneumomediastinum
- Spinnaker sail sign
- Elevated thymus
- Conservative Mx

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21
Q

Dx of this CXR?

A

Eosinophillic pneumonitis
- Bilateral infiltrates
- Acute: often in teenagers, idiopathic, BAL shows >25% Eosinophils, Rx: steroids
- Chronic: more common in adults

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22
Q

Dx of this CXR?

A

Churg-Strauss
- Rhinitis -> eosinophilia -> vasculitis
- Skin, heart & GI manifestations, elevated pANCA, eosinophils on BAL
- More insidious onset

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23
Q

Dx of this CXR?

A

Miliary TB
> 2 organs affected
Early complication of primary infection
Due to massive release of organisms
CXR- diffuse, bilateral, micronodular pattern

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24
Q

Dx of this CXR?

A

Primary TB
Adenopathy with small parenchymal foci
Signs/ symptoms infrequent, non-specific

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25
Q

TB screening principles

A

TST
- Preferred first line screening test in children <5 years (less reliable in those <6 months)
-Contraindicated if previous TB disease or previous large reaction to TST
Positive:
>=5 mm in children who have household contacts
>=10 mm in children with history of close contact or endemic area
>=15 mm in all other children
FN: active disease, immunocompromise, recent infection
FP: BCG vaccine 50% 9-12mo post
- All children with positive TST need CXR

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26
Q

TB diagnosis

A

CXR
AFB culture- Sputum x3 early morning samples (can take up to 6wks), GeneXpert: real time PCR, higher sensitive in smear +ve disease

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27
Q

Dx of this CXR?

A

Bronchiolitis obliterans/BOOP

Diagnosis on HRCT/Bx
Patchy consolidation with a predominantly subpleural and/or peribronchial distribution
Small, ill-defined peribronchial or peribronchiolar nodules
The reverse halo sign (atoll sign) is considered to be highly specific, although only seen in ~20% of patients with cop 5.

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28
Q

Dx of this CXR?

A

Follicular bronchiolitis
- Lymphoproliferative Dx
- Rx steroids

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29
Q

Dx of this CXR?

A

Sarcoidosis
>8yrs presentation
Skin, rash, eye involvement, resp Sx less common, however 90% have CXR findings
75% self resolve, steroid responsive

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30
Q

Dx of this CXR?

A

Hypersensitivity pneumonitis
- Range of triggers- interstitial/alveolar disease
- Recurrent pneumonia following exposure 4-6h (birds, farm, humidifiers, mould)
- Upper zone predominant

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31
Q

Dx of this CXR?

A

GPA
- 90% cANCA positive
- Rapid onset/progression
- Rhinosinusitis, dyspnoea, pulmonary hemorrhages
- Rx: steroids, plasma exchange, immunosuppressants, rituximab

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32
Q

Biggest risk for future asthma exacerbations?

A

One or more severe or life-threatening flare-ups in the past year

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33
Q

Increased risk of life-threatening asthma- what condition?

A

Confirmed food allergy

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34
Q

Diagnosis asthma/airway obstruction on spirometry

A

Airway obstruction defined as:
FEV1 <80% (predicted)
FEV1/FVC <75% (varies with age)
MMEF 25-75 <67% (predicted)
Bronchodilator reversibility = improvement of FEV1 of 12% in absolute values

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35
Q

Role of FENO in asthma

A

Fractional exhaled NO (FeNO)
Suggests eosinophilic inflammation and supportive diagnosis
Not done in routine practice
Higher NO may be suggestive

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36
Q

What asthma med associated with decreased duration of hospital stay?

A

Steroids 🡪

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37
Q

Cause of VQ mismatch in salbutamol therapy

A

Can open up areas of poorly perfused lung/diversion of BF to other areas- leads to transient worsening of VQ mismatch, occurs early in illness.
5-10 mins until recorrects, supplemental O2 for Rx

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38
Q

Concern w safety of LABA in children?

A

LABA also results in phosphorylation and internalisation of beta 2 receptor – results in increased mortality

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39
Q

When would you trial leukotriene receptor antagonist first (monteleukast)

A

Trialed first IF:
The child is unable to use inhaled therapy
The child also has significant allergic rhinitis
The parents have strong concerns about adverse effects of ICS

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40
Q

Role of omalizumab/mepolizumab in asthma & MOA

A

Omalizumab:
Humanised anti-IgE Mab
Prevents binding of free IgE to high affinity receptors on basophils and mast cells
Approved for moderate to severe allergic asthma in children 12 years or older

Mepolizumab:
An add-on maintenance therapy for severe asthma with an eosinophilic phenotype in patients age 12 years and older
Anti-IL-5 Mab injected SC every 4 weeks

41
Q

CF infections:
Which bug is the…
- most common overall
- second most common in early life
- risk factor for reduced survival
- contraindication to transplant

+ ABx used to treat

A

Staph aureus most common overall (MRSA = worse survival)
- Fluclox/cefalexin/IV Vanc, amoxicillin prophylaxis if colonised <12mo

Haemophilus Influenzae - second most common in early life
- Augmentin

Burkholderia Cenocepacia- contraindication to transplant
- Ciprofloxacin/bactrim

Pseudomonas Aeruginosa - risk factor for reduced survival/pulmonary decline
- Tobramycin (neb)/oral cipro

42
Q

Features of NMTB infection in CF

A

Form mycobacterium abscess complex, requires prolonged Rx- intensive IV tigecycline/cefoxitin/azithromycin, treat other potential infections

43
Q

Features of ABPA in CF

A
  • Usually >6yrs, 15% pts with CF, also seen in asthma
  • Exaggerated Th2 response to aspergillus in bronchi
  • Increasing SOB/cough/wheeze, orange/rust coloured mucous
  • CXR: evidence of infiltrates especially in UL + centrally/ ‘gloved finger’ appearence/tree-in-bud
  • Ix: high total IgE, eosinophilia, IgG +ve for aspergillus

Rx: high dose steroids, itraconazole, omalizumab if refractory

44
Q

Overview of CF treatments

A
  1. Mucolytics
    - Hypertonic saline, mannitol
    - Inhaled dornase alfa/pulmozyme
  2. ABx
    - Staph prophylaxis <2yrs
    - 2/52 augmentin if URTI when staph colonised
    - If pseudomonas or infection when colonised 2/52 ciprofloxacin
  3. Bronchodilators
  4. Gene modulators
    - Ivacaftor (>1yr): Class 3 (G551d)
    - Lumicaftor-Ivacaftor (>12yrs) : Class 2
    - Tezacaftor-Ivacaftor (>12yrs): Class 2
45
Q

CF pancreatic disease- endocrine

A

CFRDM
- Insulin deficiency from destruction of islet cells
Presents later >10yrs (20-50% by early adulyhood)
- Initially OGTT +ve then HbA1c elevated, NO ABx positive, less DKA & microvascular/macrovascular Cx

46
Q

CF biliary disease

A

NAFLD 70%
Liver disease- severe 10-15%, dysfunction up to 30% <15yrs

Liver disease independent of genotype but is associated with meconium ileus + pancreatic insufficiency

47
Q

CF pancreatic disease - exocrine

A

> 85% of children- common in F508del
- Most present in first year of life
- Supplement with pancreatic enzymes, fat soluble vitamins

48
Q

What constitutes a positive sweat test

A

(in infant >2weeks)
>60 mEq/L of chloride diagnostic – lower in infants 30-40 mEq/L

  • Will require DNA testing/repeat sweat test if unyeilding genetic Ix
48
Q

What constitutes a positive sweat test

A

(in infant >2weeks)
>60 mEq/L of chloride diagnostic – lower in infants 30-40 mEq/L

If positive:
- Will require DNA testing/repeat sweat test if unyeilding genetic Ix

49
Q

Findings of CF on CXR

A

Hyperinflation, bronchial thickening, lymphadenopathy, extensive bronchiectasis with lobar atelectasis

50
Q

Findings of CF on PFTs

A

Early- decrease in MMEF 25-75%

Reduced FEV1, FEV1/FVC with bronchodilator response

As disease progresses more restrictive pattern

50
Q

Findings of CF on PFTs

A

Early- decrease in MMEF 25-75%

Reduced FEV1, FEV1/FVC with bronchodilator response

As disease progresses more restrictive pattern

51
Q

Relationship mec ileus to CF

A

10-20% of newborn infants with CF have meconium ileus
Frequency greater (=30%) among siblings born subsequent to a child with meconium ileus
Conversely 80—90% of individuals with meconium ileus have CF

52
Q

Percentage of male infertility in CF

A

> 90% of males azoospermic due to failure of development of the Wolffian duct structures

53
Q

HRCT appearence of bronchiectasis

A

HRCT gold standard for diagnosis
- Key radiographic criteria = broncho-arterial ratio

Other: tram lines, signet ring appearance]Lower lobes most commonly affected

54
Q

Congenital causes of bronchiectasis

A

Williams-Campbell syndrome = absence of annular bronchial cartilage

Marnier-Kuhn syndrome = congenital tracheobronchomegaly- connective tissue disorder

55
Q

Diagnostic criteria for bronchiectasis

A

Clinical and imaging criteria
Recurrent (>=3 episodes) wet or productive cough, each lasting >4 weeks, with or without other features
Exertional dyspnoea
Recurrent chest infections
Growth failure
Clubbing
Hyperinflation or chest wall deformity
Presence of characteristic radiographic features on HRCT

56
Q

What triad of sx is seen in Kartagener Syndrome

A

Kartagner triad = 1) situs inversus totalis 2) chronic sinusitis 3) bronchiectasis

57
Q

Mx of acutely toxic child w bacterial tracheitis/epiglottitis- stable.

A
  1. Nebulised adrenaline & dexamethasone
  2. Minimise handling
  3. IV ceftriaxone
  4. Prepare for intubation/PICU support (would consider earlier if haemodynamic instability)
58
Q

Features of mycoplasma pneumonia- inc. CXR findings.

A

School aged child
Malaise, low grade fever, headache, +/- cough/dyspnoea
Not responding to normal ABx
CXR findings variable- peribronchial intestitial infiltrates/nodular opacification

59
Q

Dx of this CXR?

A

Pneumatocoele
- Thin/smooth walled collection with air-fluid level
- Complication of severe bacterial pneumonia
- Often no additional therapy needed/asymptomatic

60
Q

Dx of this CXR?

A

Lung Abscess
- Rare in children, unless underlying conditions
- Thick walled, purulent material- destruction of lung parenchyma
- Bugs: Staphylococcus, E.Coli, Klebsiella pneumoniae, Pseudomonas, fungal in immunocompromised
- Total 4-6wks ABx, 2-3wks IV

61
Q

Dx of this CXR?

A

Hyatid cyst
- Echinococcus (granulosuus most common, multilocaris- most likely in lung) tapeworm
- Human is intermediate in life cycle- hatch babies into us then are ingested by definitive host to form tapeworm
- Often asymptotic

62
Q

Dx of this CXR?

A

Cryptococcus pneumonia
- Encapsulated yeast
- Immunocompromised host
- C.Neoformans- asymptomatic then pneumonia/meningitis
- CXR: poorly defined mass lesions, pulmonary nodules, and, rarely, pleural effusion.

63
Q

CF class 1 mutation, genes & Rx

A

No protein production
- Premature stop codon
- G542X, W1282X, R553x

GWR- great work retard, not even made

64
Q

CF class 2 mutation, genes & Rx

A

Defect in processing
- Trapped, does not reach membrane

F508del
(N1303K, I507del)

FNI- fucking no idea- lost in the cell

65
Q

CF class 3 mutation, genes & Rx

A

Regulation defect
- Reaches membrane but does not work

G551D
(S549N,V520F)

GSV- can’t shoot a goal

66
Q

CF class 4 mutation, genes & Rx

A

Reduced Cl transport
- Ion channel opening faulty

R117H
D115H
R347P

RDR- registered day rest (tradies work less hard)

67
Q

CF class 5 mutation, genes & Rx

A

Splicing defect
- Insufficient production

A455E - Ass, I have no ass, theres not enough protein

68
Q

CF gene modulators & mutations effective on

A
69
Q

What does DLCO measure?

A

Measures the ability of the lungs to transfer gas from the inspired air into the bloodstream.
- Pulmonary interstitial disease/vasculitis (conditions that may affect gas transfer)

↓ DLCO
- Increased membrane thickness: APO/fibrosis/ILD
- Decreased perfusion: PE/vasculitis
- Increased perfusion: hemorrhage (GPA/Goodpastures)/exercise
- Decreased 02 carrying: anaemia
- Decreased lung volume: RLD

70
Q

Function/method of delivery of Dornase Alfa

A

Recombinant DNAse
- Mucolytic/breaks up respiratory secretions
- Inhaled/nebulised

71
Q

What is the Herring Breuer reflex?

A

Prevents lung over-inflation
Stretch receptors respond to excessive stretch
- Inhibit inspiration
-active within the tidal volume range throughout the first year of life

72
Q

Indications for PSG?

A
  • Night time snoring, or WOB
  • Daytime symptoms
  • Narcolepsy
  • Paroxysmal events during sleep – E.g. seizures, atypical parasomnias
  • Predisposing diagnosis – E.g. Trisomy 21, CLD
  • NM disease
  • Nocturnal oxygen, CPAP, ventilation
  • Tracheostomy decanulation
73
Q

Blood test findings in narcolepsy?

A

HLA-DQB1*06:02 haplotype and low cerebrospinal fluid orexin (hypocretin)

74
Q

Dx on this CXR?

A

Pulmonary fibrosis

75
Q

Dx of this spirometry pattern?

A

Neuromuscular disease

76
Q

When is a preventer indicated in kids >6yrs?

A
  • Freq intermittent (<6wks)
  • Persistent- day asthma once a week, night 2x per month
  • Severe flare ups
77
Q

Low, med and high dose ICS for teenagers

A

Fluticasone
Low 100–200
Med 250–500
High>500

Beclomethasone
Low 100–200
Med 250–400
High >400

78
Q

Risk factors for thunderstorm asthma (severe episode, death)

A
78
Q

Risk factors for thunderstorm asthma (severe episode, death)

A
79
Q

When to start a preventer adult/adolescent?

A
  • asthma symptoms twice or more during the past month
  • waking due to asthma symptoms once or more during the past month
  • an asthma flare-up in the previous 12 months.
80
Q

What age range is a spacer appropriate for?

A

2-7 yrs
(younger infants consider face mask)
Teenagers- inhaled via spacer may lead to greater increase in HR

81
Q

When is a preventer considered in 1-5yrs?

A

Mild flares
- Consider if <6-8wks
- If sx 1x per week 2x night/mo

Moderate flares
- If <6-8wks

Life threatening
- Even if >6mo

82
Q

Dx on this CXR?

A

Complex pneumatocoele
- Complication of pneumonia
- Tension physiology- midline shift

83
Q

Dx on this CXR?

A

CPAM

84
Q

Dx on this CXR?

A

Sequestration = systemic blood supply

85
Q

Dx on this CXR?

A

Congenital lobar emphysema

86
Q

Dx on this CXR?

A

Bronchogenic cyst

87
Q

Dx on this CXR?

A

Dx on this CXR?

88
Q

Mx of lymphatic effusion? (chylothorax)

A

Medical Mx
- MCT milk formula (directly absorbed into portal venous system)
- TPN
- Somatostatin/octerotide- reduces intestinal BF, pancreatic and biliary secretions/lymphatic circulation

Surgical (if persistent)
- Pleurodesis
- IP shunt
- Thoracic duct ligation

89
Q

Types of apnoea- findings on PSG

A

Central apnoea = loss of respiratory muscle movement and decreased nasal air flow followed by a desaturation event.

Obstructive apnoea = decreased nasal air flow with increased respiratory and abdominal muscle movement +/- desaturation event.

90
Q

Physiologic vs anatomic dead space

A

Physiologic dead space = the volume of gas that does not eliminate carbon dioxide.

Anatomic dead space = the volume of the
conducting airways.

91
Q

Risk factors for pseudomonas infection in CF?

A
  • Recent broad spectrum ABx
  • History of prolonged hospitalisation/LRTI
  • Invasive devices
  • Immunosuppression
  • Current/prev ICU admission

Comorbidities
- DM
- Renal disease
- Lung disease
- Tumors/transplant/dialysis
- Immunosuppression

92
Q

Causes of bronchiolitis obliterans?

A
  • Post infectious: Adenovirus, Mycoplasma pneumoniae
  • Drugs e.g. Penicilliamine
  • Autoimmune:
  • SLE, RA, Scleroderma
  • GVHD after BMT
  • Lung transplantation.
  • Noxious fume inhalation e.g.
    diacetyl (popcorn lung)
93
Q

Congenital causes of chylothorax

A

Lymphatic malformations
Downs/Noonan’s syndrome

94
Q

Common site of FB aspiration?

A

Right larger and more vertical – aspirated matter lodges in right bronchus

95
Q

Impact of COX1 on asthma?

A

Thought to cause a deficiency in the protective bronchodilator/ antiinflammatory prostaglandins, and an excess of proinflammatory/ bronchoconstrictive leukotrienes.

96
Q

Differences in sleep stages

A

NREM 20%
Stage 1 = light, easy to rouse
Stage 2 = sleep spindles/K complexes, difficult to rouse, important for memory
Stage 3 & 4 = higher voltage waves/ slow wave sleep- more difficult to rouse

REM 80%
- Desynchronized/low voltage
- Sawtooth patterns
- Muscle atonia with rapid eye movements

FRACP 2023 Cram Homicrons Edition (29 decks)

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