Resp/ENT/Sleep Flashcards

1
Q

Definition of chronic cough

Ddx

A

persistence of cough >4 weeks

Most common 3:

  1. Protracted bacterial bronchitis
  2. Bronchiectasis
  3. Asthma

Aspiration
Atypical infx
Inhaled foreign body
Post viral
ILD
Cardiac disease
Ear disease
Oesophageal disease
Medications
Somatic/habit cough

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2
Q
  1. What is protracted bacterial bronchitis
  2. What are the most common bacteria found in these cases?
  3. What should you be suspicious of if the cough doesn’t respond to 4 weeks abx?
A
  1. Cough lasting >4 weeks that is WET in nature with response to 2 weeks abx tx (ADF) with no other features to indicate another cause
  2. H. influenzae, Moraxella catarralis, Strep pneumonia
  3. Bronchiectasis
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3
Q

Asthma management step wise approach

A
  1. Reliever (salbutamol) PRN
  2. low dose ICS preventer (eg fluticasone, budesonide, ciclesonide) or montelukast or cromeo
  3. higher dose ICS OR low dose ICS plus montelukast OR LABA plus low dose ICS
  4. Refer resp physician

Wait 4 weeks after starting ICS or cromones to assess for sx resolution and 2 weeks after montelukast before stepping up

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4
Q

Bronchiectasis

Definition
Adults vs children

A

ADULT DEFINITION
Irreversible dilatation of one or more bronchi (bronchi larger than accompanying blood vessel) w failure of bronchi to taper in periphery of lung
Bronchoarterial ratio >1
Radiological diagnosis - seen on CT.

CHILDREN

  • Reversible with early treatment
  • Bronchoarterial ratio >0.8
  • Bronchiole normal tubular -> cyclindrical/follicular (dilated) = ‘tree in bud appearance’ on CT -> varicose -> cystic
  • Up to cylindrical point it is reversible in children but beyond this it is not.

FEATURES

  • CT changes + chronic wet/productive cough
  • Frequent resp exacerbations
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5
Q

Spectrum of chronic suppurative lung disease

Underlying pathogenesis

A

Mildest end of spectrum
Protracted bacterial bronchitis (can occur in healthy well children)
-> repeated infections ->
Chronic suppurative lung disease (CT scans normal)
->
Radiological bronchiectasis

Pathophys:
- Insult to lung (infection) -> inflammatory response with cytokines, nests, elastase and bacterial byproducts -> impairs lungs normal mucociliary clearance -> incr mucus production/decr clearance -> blocks small airways -> incr propensity for microbial colonisation -> bacteria cause more inflammation -> cycle continues

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6
Q

Treatment bronchiectasis

A

Antibiotics
Maximise airway clearance (chest PT)
Diet optimisation/nutrition
Minimise environmental pollutants (smoking)
Exercise

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7
Q

Classic bugs that cause infection in children with CF bronchiectasis

A

PSA
Staph aureus

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8
Q

Non typable haemophilis influenza

  • what is its mode of pathogenicity?
  • what condition has increased susceptibility to this bug?
A

Secretes biofilm (ECM protecting against host immune response and abx, helping to prolong existence in airways)
Incr susceptibility in PBB and CF
Worsens inflammation cycle

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9
Q

Abx therapy (abx + duration) in exacerbation of bronchiectasis

  • mild-mod
  • vs mod-severe
A

Initial empiric tx
Mild-mod: PO Augmentin DF (amoxicillin) or Azithromycin. Cipro if PSA in recent culture.
Mod-Severe: IV ampicillin, cefotazime, ceftriaxone. Tazocin or ceftaz and tobra if PSA in recent cultures.

Minimum 10-14 days or cough free for 2 days
Start with oral abx, if not effective then will need IV abx

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10
Q

Indication for long term abx in bronchectasis

A

3+ exacerbations in previous 12 months

Aims for suppression rather than eradication

Decr exac and hospitalisation rate BUT risk of resistance

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11
Q

Benefits of macrolides in CF

Risks

Example of a macrolide

A

ex: Azithromycin

BENEFITS
Antimicrobial and anti-inflammatory
Routinely used in CF
Inhibits biofilms (PSA, NTHI)
Decr exac rate

RISKS
1. BUT macrolide resistance (staph aureus specifically) and risk of GI side effects
Exclude NTM prior to starting tx to avoid induction of resistance to macrolides
2. QTc prolongation (need to perform baseline ECG prior to starting tx)

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12
Q

What conditions have evidence for inhaled abx

A

CF
Non-CF BE with PSA only

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13
Q

Features of salbutamol toxicity
what do u see on blood gas

A
Tremor, tachycardia, tachypnoea
Metabolic acidosis (lactate high)
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14
Q

Pathophys of asthma

A

Two major components to airway obstruction

i. Chronic Airway inflammation
ii. Reactive airways = bronchoconstriction and airway hyperresponsivness

Pathology

i. Smooth muscle hyperplasia of bronchial and bronchiolar walls
ii. Thick tenacious mucous plugs
iii. Thickened BM
iv. Mucosal edema
v. Eosinophilia of the submucosa and secretions
vi. Increased mast cells in smooth muscle

Results in
Mucosal edema, bronchospasm and mucus plugging -> airflow obstruction -> increased resistance to airflow -> decreased ability to expel air -> hyperinflation

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15
Q

Explain VQ mismatch in asthma

Explain how ventolin can affect this

A

Airway inflammation and resulting airflow obstruction is NOT uniform throughout the tracheobronchial tree – distribution of inspired air is uneven, uneven circulation to the alveoli, uneven ventilation (VQ mismatch)

Ventolin
Can cause a paradoxical worsening in VQ mismatch (and thus spO2) early in treatment (first 30min). May need supplemental O2 for this short period of time.

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16
Q

Innervation of bronchial smooth muscle

A

Parasympathetic nervous system -> bronchoconstriction (M3 receptors) = cholinergic

Sympathetic nervous system -> bronchodilation (B2 receptors) = beta adrenergic

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17
Q

Spirometry findings in asthma

Define the values of abnormality for each

A

Airway obstruction defined as

  1. FEV1 <80% (predicted)
  2. FEV1/FVC <75% (varies with age)
  3. MMEF 25-75 <67% (predicted)

Bronchodilator reversibility = improvement of FEV1 of 12% in absolute values

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18
Q

How many actuations in a ventolin inhaler?

A

200

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19
Q

Asthma severity levels

A

Infrequent intermittent asthma (Symptom-free for at least 6 weeks at a time (flare-ups up to once every 6 weeks on average but no symptoms between flare-ups)

Frequent intermittent asthma (Flare-ups more than once every 6 weeks on average but no symptoms between flare-ups, normal exam and PFT between flares)

Persistent asthma
• Daytime symptoms >2 days/week
• Nocturnal symptoms >1 night/week
• Attacks <6 weeks apart
• May have abnormal lung function
• Multiple presentations to ED

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20
Q

Montelukast

Mechanism of action

Age cut off

Indication

A

Mechanism of action = LT receptor antagonist

Can be used in children >= 2 years of age

Main indication = alternative to ICS in children with Frequent intermittent asthma or Mild persistent asthma

Trialed first IF:

  1. The child is unable to use inhaled therapy
  2. The child also has significant allergic rhinitis
  3. The parents have strong concerns about adverse effects of ICS
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21
Q

Risk of LABA use and limitations of use in asthma

A

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

No evidence for children <5 years

Should not be started when child is clinically unwell and should not be used as monotherpay (always with ICS)

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22
Q

Mepolizumab

Omalizumab

A

Mepo

i. Humanised anti-IgE Monoclonal Ab
ii. Prevents binding of free IgE to high affinity receptors on basophils and mast cells
iii. Approved for moderate to severe allergic asthma in children 12 years or older
iv. Delivered by SC every 2-4 weeks

Omal

i. An add-on maintenance therapy for severe asthma with an eosinophilic phenotype in patients age 12 years and older
ii. Anti-IL-5 Mab injected SC every 4 weeks
iii. Decreases the production an survival of eosinophils, a major inflammatory cell involved in asthma pathogenesis

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23
Q

Diagnosis of CF

A

New born screening test looks for elevated IRT (‘immunoreactive trypsinogen’ produced by stressed pancreas)

  • screens for the most common 12 genotypes in the state although over 900 exist

Gold standard is the ‘Sweat Test’ (elevated Cl, Na, sweat weight)

However there is a risk of false negatives
Any child w evidence of pancreatic insufficiency (Steattorrhoea, fat globules or crystals in stool) +/- FTT should be considered for CF

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24
Q

CF gene (most common)

A

Delta 508 most common (90% of patients in australia)

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25
Pathophys CF
Mutation in the CF transmembrane regulator gene (CFTR transports Cl across the cell wall) Results in abnormal CFTR protein processing 1. Impaired Cl secretion from airway epithelial cell - \> incr Na and H20 into cell resulting -\> causes dehydration of airway surface liquid of cell resulting in THICK mucous and impaired clearance of mucous 2. Impaired HCO3 secretion from cell -\> airway surface liquid pH becomes more acidic -\> inhibits antimicrobial fxn -\> impaired killing of pathogenic bacteria entering lung Cycle of CFTR dysfunction -\> viscous secretions -\> infection and inflammation -\> lung damage and abnormal lung function Ultimately results in death due to resp failure
26
Classic bugs causing infection in CF
Most common early on: 1. S aureus 2. Non typable haemophilus influenza Most common later on (adolescence): 3. Pseudomonas is a bad prognostic indicator (becomes more predominant older you get) Uncommon 4. MRSA (if present in resp tract, almost pathopneumonic of CF) 5. Steno maltophilia 6. B cepacia least common but can cause very aggressive lung disease and death in some children Need abx prophylaxis for first 2 years of life to prevent colonisation especially w Staph
27
ABPA in CF Prevalence What is the pathophys Diagnostic features Treatment
Risk to CF patients (1-15% prevalence) Type 1 Hypersensitivity reaction - Exaggerated TH2 CD4+ immune response to aspergillus Diagnosis requires 5 features - Acute or subacute clinical deterioration (BROWN sputum characteristic) often w wheeze - Serum IgE \>1000 IU/ml - Immediate cutaneous reactivity to Aspergillus on RAST skin prick - Pos Aspergillus précipitans IgG - New or recent abnormalities on chest XR or CT (pulmonary infiltrates) - Aspergillus in sputum culture Treatment - Oral steroids (or pulse IV methyl predictive if noncompliant) - Oral antifungals (itraconazole)
28
Abx against pseudomonas in CF
Tobramycin (IV or nebulised) Ciprofloxacin Cayston (nebulised Aztreonam) 2 month initial eradication course (2 weeks IV Tobra then 2 months nebulised Tobra) If unsuccessful, for intermittent cycles of abx to try to lessen bacterial load
29
Indication, effect and MOA of azithromycin in CF patients
_Indication:_ patients with ***chronic PSA infection*** -\> used as a three times a week dosing regime to stabilise chronic lung disease in CF pts w PSA _Effects:_ 1. Improvement in lung function 2. reduced pulmonary exacerbations (PEx) 3. improvement in nutritional status _MOA:_ Azithromycin **suppresses alginate production (**PSA produces a gene that codes for alginate when it progresses from acute to chronic infection) Anti neutrophilic and anti inflammatory cytokine production
30
What is the purpose of cross infection prevention practises in CF patients?
Prevent cross infection of NTM between carriers No 2 CF patients should ever come into contact w each other
31
Mucolytic therapies in CF Examples Effect on disease
``` Pulmozyme (dornase alpha), neb Hypertonic saline (6%), neb Inhaled mannitol (dry powder delivery device) ``` Pulmozyme reduces number of resp exacerbations Shown to improve lung function with patients with severe lung disease but in patients with normal lung function their lung function continued to decline ALL: Get a transient increase in FEV1 then stability in lung function Not disease modifying agents
32
Ivacaftor - What is it used for - What does it do
- For class 3 (G551D) and 4 mutations in CF pts over age of 2 - \*Potentiator\* - Disease modifying agent: partially corrects the channel defect allowing Cl transport - Initial FEV1 increase then stability - Decr in sweat chloride to almost non CF levels - Improvement in nutrition
33
What disease modifying agents can you use in delta 508 homozygous (class 2) CF patients What effects do these have on CF
**Orkambi** in \>2yo (ivacaftor and lumicaftor) - _\*corrector\*_ - For Class 2 mutations (delta 508 homozygous) in CF in pts \>12yo - -\> improves protein folding and incr trafficking to cell surface - 3-4% incr in LFT and 34% decr in pulmonary exacerbations - No incr in nutrition or improvement in QOL **Symdeko** (Tezacaftor + ivacaftor) in \>5yo - _\*corrector\*_ - Incr FEV1, reduce pulm exacerbations **Trikafta** (elexacaftor, texacantor and ivacaftor) - _\*amplifier\*_ - improved/partially corrected protein folding and Cl passage through channel - reduces exacerbations, sweat Cl and QOL - improves FEV1 and BMI (incr)
34
CF complications
Pancreatic insufficiency fat soluble vitamin deficiency Malabsorption Growth failure/delay/pubertal delay CF-related diabetes (yearly OGTT and HBA1C \> 10yo to detect this). Signs incl reduction in weight and RFT in 2 yrs leading up to diagnosis. Assoc w reduction in life expectancy. Cancer of small bowel, colon, biliary tract (NOT stomach or rectum) C diff Crohns disease Osteoporosis (Dexa scans yearly over age of 10 to detect this) - sec to malabsorption of vit D, Ca Infertility (95% men infertile) CF related liver disease (starts w mild transaminitis -\> progress to cirrhosis and portal HTN). can start URSO to improve bile drainage. CF arthropathy Pseudo-bartters syndrome (metabolic alkalosis w low K and Na and dehydration) Anxiety and depression
35
ChILD Definition Presentation
Remodelling of lung interstitium and distal airways leading to abnormal gas exchange Presents w 3/4 of the following 1. Resp sx (cough, SOB, exercise tolerance) 2. signs (tachypnoea, creps, clubbing, FTT, resp failure, incr WOB) 3. hypoxia 4. diffuse abnormalities on cxr/ct
36
Tachypnoea and hypoxia soon after birth CT and biopsy below Diagnosis? Tx?
Pulmonary interstitial glycogenosis Type of congenital ILD Histological ddx from lung biopsy - round **glycogen laiden mesenchymal cell** Tx w corticosteroids - good long term outcome
37
Otherwise healthy infants during the first months to year of life with persistent tachypnea, crackles, and hypoxemia Diagnosis? Tx?
**Neuroendocrine cell hyperplasia of infancy** Form of ILD - histological or radiological diagnosis bx- neuroendocrine cell bodies in interstitium or bronchioles HRCT - ground glass opacities in RML and lingual and air trapping in lower lobes Tx is supportive Pulmonary symptoms and hypoxemia tend to improve with time but may persist for years.
38
Surfactant dysfunction Causes/genes presentation treatment
Genetic disorder - 4 genetic defects --\> SPB: _**lethal** neonatal RDS_ in homozygous individuals (AR) --\> SPC: AD, causes _interstitial lung disease_ of varying severity and age of onset --\>ABCA3: lethal neonatal respiratory distress **OR** ILD in those surviving and presenting at older ages P/w persistant tachypnoea, hypoxia time of presentation depends on specific genetic defect Treatment - Chronic ventilation - Lung transplant - Corticosteroids, hydroxyquinolone, azithromycin - nutritional supplementation due to incr WOB
39
Hypersensitivity pneumonitis pathophys presentation ix tx
IE 'Extrinsic Akllergic Alveolitis' ``` Type 4 (and 3) HS reaction to inhaled organic particles -\> granuloma formation in the lungs - Commonly to bird Ag ('Bird fancier or breeder's lung') and mould (Aspergillus; 'ABPA') ``` - also spray paints, glues, insecticides, dusts Diagnosis - mean age 10yo Presentation - Acute (fever, cough, body aches) - Subacute: chronic cough w weight loss, dyspnoea, fatigue - Chronic - as above but with _fibrosis (ILD)_ on CT Ix - CXR, CT (micronodules, ground glass opacities) - BAL (lymphocyte infiltration, granulomas, multinucleated giant cell) - Lung bx - Ag challenge Tx - remove Ag - corticosteroids
40
Connective tissue diseases with lung involvement - pathophys - causes
AutoAb to pulmonary parenchyma and vasculature Nonspecific interstitial pneumonia most common histological finding Lead to chILD Causes SLE Systemic sclerosis Dermatomyositis/polymyositis Sjogrens syndrome
41
Ix for chILD
Pulse oximetry Lung function (restrictive pattern, decr lung compliance and lung volumes) - spirometry - plethysmography - DLCO - Exercise testing HRCT (findings depending on underlying cause of ChILD) Genetic testing Bronchoalveolar lavage (findings depending on underlying cause of ChILD) Lung biopsy (gold standard)
42
Management of chILD
Supportive tx Nutritional support Immunisations Lung PT, exercise programs Avoid smoke exposure Family support, education +/- O2 support +/- If pulm HTN -\> sildenafil +/- steroids for certain conditions +/- steroid sparing agents /immunomodulators +/- other specific tx +/- lung transplant
43
What cells produce surfactant, when does this occur in-utero? What does surfactant do?
Type II alveolar epithelial cells around 20-24 weeks, produce surfactant around 30 weeks gestation Decr surface tension of the alveolar, facilitating expansion/keeping them patent.
44
Tracheomalacia DEfinition Sx
Absence/deficiency/malformation or softness or tracheal cartilage Congenital/acquired, primary or secondary types Sx - intrathoracic compression = collapse on exp -\> retained lower airway secretions, wheeze - extrathoracic -\> collapse on inspiration -\> chronic brassy barking or seal-like cough, stridor - Resp distress
45
Tracheo-oesohageal fisutla what is it how common is vacterl assoc what are the diff types and which is most common? complications
Defective separation of developing trachea from developing oesophagus 50% have VACTERL assoc type C is most common ~ 85% Complications - tracheomalacia at level of fistula - TOF-cough - GORD, dysphagia - Recurrent aspiration - associated anomalies (laryngeal cleft, other fistulas\_
46
**Vascular rings** What is most common type? Sx Which syndrome is associated?
Most commonly **double aortic arch** completely _encircles trachea and oesophagus_, causing **compression** Sx - stridor, resp distress, cough/wheeze, frequent infection, feeding difficulties/dysophagia/vomiting Associated anomalies (often cardiac such as ASD, syndromes such as De George)
47
Congenital pulmonary airway malformations (CPAM) What is it What is it caused by Where is the blood supply from Clinical features Mx Cx
Multicystic masses of segmental lung tissue with abnormal bronchial proliferation. Non-functioning. Due to abnormalities of branching morphogenesis of lung. Blood supply is from pulmonary circulation Clinical features - Can be detected antenatally (ultrasound) with pressure effects with hydrops foetalis, mediastinal shift, pleural effusions, polyhydramnios - present with neonatal distress - can be asymptomatic (carries small risk of malignany) Cx - Cancer risk (Pleuropulmonary blastoma (PPB))- particularly common in type 4 CPAM - Infection - Pneumothorax - Compression of mediastinal structures/mass effect -\> resp distress Mx - depends on sx or malignancy risk but often would be surgical excision
48
Pulmonary sequestration What is it Where is the blood supply from? How is it classified Mx
**='accessory lung'** Non functioning mass of lung tissue that _doesn't contribute to gas exchange_ (not communicating with normal tracheobronchial tree) Predominantly affecting lower lobes (LLL 60% \> RLL 40%) Blood supply from **systemic circulation** Classified based on pleural covering * Intralobar (75%) - does not have its own pleura * Extra lobar - has own pleura separating it from rest of lung; more commonly assoc w other congenital malformations (CDH most common, CPAM 15-20%, bronchogenic cysts, CHD) ​Presentation * in newborns as _respiratory distress, cyanosis, or infection_ * later in life with r_ecurrent pulmonary infections_ , persistant cough, exercise intolerance * If large can present as _CCF_ due to R -\> L shunting * Occasionally p/w _pulm haemmhorage_ Mx - surgical excision
49
Congenital lobar overinflation What is it Which areas of lung does it most commonly affect Clinical presentation Associations (1x)
Hyperinflation of 1 or more lobes, can cause compression of adj lung and mediastinal structures Most often affects LUL followed by RUL Presents postnatally with resp distress in neonatal period to first 6mo Possible wheeze, decr breath sounds, mediastinal shift, hyper resonant percussion note Assoc w cardiac disease (VSD, PDA, TOF) -\> screening echo
50
Congenital diaphragmatic hernia What is it Presentation Mx
Defect within the diaphragm where it doesn't have an associated membranous sac so the abdominal contents can herniate into thorax, inhibiting lung development Affects left side \> right side (liver protective) Scaphoid abdomen, funnel shaped chest Contralateral deviation of trachea and mediastinum +/- collapse of contralateral lung Resp distress Stabilise baby Surgical mx
51
Diaphragmatic eventration What is it Causes Diagnosis Mx
Abnormal elevation of all or part of diaphragmatic dome (congenital - incomplete development of the muscular portion of the diaphragm or innervation) Acquired (more common) - phrenic nerve injury from instrumental delivery, insertion of intercostal catheters or from cardiac surgery Diagnosed by USS -\> paradoxical movement of diaphragm Mx - conservative. Only surgical mx if severe resp distress
52
Pectus Excavatum What is it How does it present Mx
Depression of sternum and anterior chest - due to defect in sternal cartilage OR overgrowth of costal cartilage of ribs 60% cases assoc with shallow chest Presentation - generally asymptomatic. ?reduced exercise tolerance. Cosmetic concerns. Mx - Conservative. Surgical for cosmetic concerns but is a big surgery!
53
Effects of scoliosis on respiration Management options
Respiratory effects = restrictive lung disease - Rigid thoracic cage not allowing full expansion - Decr insp muscle strength - Reduced lung growth Mx - Bracing - Spinal implants/distraction devices (rods allowing for growth) - Spinal fusion (for severe scoliosis in adolescents)
54
Pre op assessment prior to scoliosis surgery
Lung function (FVC) Sleep study Multidisciplinary clinic to optimise other issues (aspiration, constipation, medication rationalisation) Establishment of pre-operative resp support (Bipap)
55
REM sleep
rapid eye movement slow muscle tone dulled airway reflex irregular brathing and decr tidal volume dreaming More REM sleep in 2nd half of the night (more NREM, or lighter sleep, in the first 1/2 of the night)
56
What is sleep disordered breathing
Continuum from primary snoring (sleep study) to severe obstructive apnoea (OSA can be clinical diagnosis but severity stratification requires sleep study)
57
OSA - Definition - Causes - Diagnosis - Mx
Recurrent oropharyngeal upper airway collapse during sleep leading to multiple cycles of hypoxic episodes followed by blood reoxygenation. Often worse in REM sleep (reduced muscle tone) **Causes** * *Adenotonsillar hypertrophy* * *Chronic rhinitis/hayfever* * Laryngomalacia (\<1) * *Obesity* * Anatomical * Syndromes (T21, PWS, BWS, Pierre-Robin) * Abnormal muscular tone (CP, hypotonia, muscular dystrophy) * Mucopolysaccharoidosis, Achondroplasia * *Diagnosis via** - Overnight oximetry (good PPV, but poor NPV) -\> look for clusters of desaturations assoc w rise in HR - Polysomnography (sleep study) * *Mx in children** * *Mild** - IN steroids (nasonex) - Weight/allergy mx - Positional - Watchful waiting (CHAT study) - Adenotonsillectomy if medical tx not successful * *Severe** - Adenotonsillectomy - Turbinectomy, septoplasty, other jaw distracting surgeries - CPAP - Rarely tracheostomy
58
Indications for PSG in neonates
Periodic desats during sleep ALTE w history of SDB Sydromes w compromised airway Quantification of impact of upper airway lesions (laryngomalacia) Assess for residual OSA post-op if clinical concern ?Congenital hypoventilation syndrome
59
CPAP vs BIPAP
Continuous/constant raised pressure applied to airway Pneumatic splint to upper airway - maintains airway potency, prevents collapse and reduces insp work of breathing BiPap - application of 2 different pressures for inspiration (assists ventilation) and expiration (maintains airway patency) - re-expands collapsed/poorly ventilated alveoli, decr WOB - improves O2, decr CO2 - resets chemoreceptors
60
Nocturnal hypoventilation Presentation Risk factors
Children with **NM disease are at risk (muscular dystrophies most at risk** - peripheral \>central neurological abnormalities) IDENTIFIED VIA * daytime hypercapnia * nocturnal desaturation Cx of Restrictive lung dsiease in patients w NM disease - reduced FVC Starts with REM disordered breathing, then progresses to NREM and REM disordered breathing Mx w BiPAP -\> reduces morbidly, mortality
61
Congenital central hypoventilation syndrome What is it Genetic mutaiton involved Presentation Associated conditions Mx
_Cannot produce ventilatory response to hypercapnia_. response to hypoxia is variable. Rare **autosomal dominant** condition of primary alveolar hypoventilation (mutation **PHOX2B gene** at 4p12) - all subjects heterozygous for a mutation Typical presentation is * in infancy with **hypoventilation during sleep** but normal when awake. * **NREM worse** than REM. * _P/W Protracted RTIs and unable to be weaned from ventilator._ * Severe forms hypoventilate awake and asleep. * Also incr risk during exercise as well. Assoc - hirshsprungs, **tumours of neural crest cell origin**, arrhythmias etc Mx - lifelong - tracheostomy/IPV - NIV - Phrenic nerve/diaphragmatic pacing for severe forms Monitoring - 72 hr holter monitor - echo - formal neurocog assessment - barium enema or rectal bx for those w hx constipation - imaging/annual review for monitoring of neural crest tumours
62
What is minute volume?
Same as minute ventilation TV x RR Volume of gas inhaled (inhaled minute volume) or exhaled (exhaled minute volume) from a person’s lungs in one minute
63
Dead space Anatomical vs pathological vs physiological
Anatomical dead space – air required to fill conducting airways not involved in gas exchange i. Usually about 150ml Pathological dead space – air not involved in gas exchange due to pathology Physiological dead space = Anatomical + Pathological i. Volume of gas that does NOT eliminate CO2
64
Alveolar ventilation - definition - formula - how does this relate to CO2 concentration
= (TV – physiological dead space) x RR Volume of atmospheric air entering alveoli Concentration of CO2 in alveolar gas and arterial blood is INVERSELY related to the alveolar ventilation
65
Round pneumonia - most common age group - most common pathogen - pathogenesis
Strep pneumonia Age 5 most common (\<8 majority) Pathogenesis - 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 - In adults, these allow lateral dissemination of infection throughout a lobe, leading to lobar pneumonia - In children, where these have not developed, the limited spread of infection results in round pneumonia
66
Definition of pneumonia What is complicated pneumonia? Aetiology
CLINICAL diagnosis when there a signs of a lower respiratory tract infection + wheeze excluded i. Presence of persistent or repetitive fever, cough and tachypnoea at rest ‘Complicated’ pneumonia = parapneumonic effusion, empyema, lung abscess or necrotising
67
What pathogens are associated with the following types of pneumonia? 1. Lobar 2. Bronchopneumonia 3. Necrotising 4. Caseating 5. Interstitial and peribronchiolar 6. HAP 7. Immunocompromised 8. Aspiration
1. Lobar pneumonia = **S. pneumoniae** pneumonia. 2. Bronchopneumonia = primary involvement of airways and surrounding interstitium -\> **Streptococcus pyogenes and Staphylococcus aureus** pneumonia. 3. Necrotizing pneumonia = associated with _aspiration pneumonia_ and pneumonia resulting from **S. pneumoniae, S. pyogenes, and S. aureus**) 4. Caseating granuloma = **tuberculosis** 5. Interstitial and peribronchiolar with secondary parenchymal infiltration – typically occurs when a severe **viral pneumonia is complicated by bacterial pneumonia** 6. Hospital acquired: GNR (​**Klebsiella pneumoniae, E.coli, Pseudomonas aeruginosa**), S. aureus * *(MRSA)** 7. **I**mmunocompromised pneumonia: * *Klebsiella, Pneumoncystis jiroveci, M. tuberculosis, aspergillosis** 8. Aspiration pneumonia: **Klebsiella, E.coli, S. aureus, gastric bacteria**
68
Tx of pneumonia Mild-mod Severe Progression desipite empirical abx
Admission is required for oxygenation, fluid therapy or moderate to severe work of breathing Administer oxygen to maintain O2 saturations \>92% If giving NG or IV fluids limit to half or 2/3 maintenance Antibiotics 1. **Non-severe pneumonia** - Amoxicillin 30 mg/kg TDS - IV benpen 60 mg/g Q6H – if unable to tolerate oral intake/vomiting 2. **Severe pneumonia** - IV ceftriaxone + flucloxacillin - Consider IV vancomycin if MRSA suspected 3. Consider IV azithromycin if pneumonia progressing despite antimicrobial therapy (?atypical)
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Indications for IV vanc in pneumonia
Concurrent skin infection due to MRSA Indigenous or Torres Strait Islander Necrotising pneumonia Previous MRSA colonization)
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How does Osteltamivir work? When is this indicated?
- Neuraminidase inhibitor - Blocks the function of viral neuraminidases of the influenza virus, by preventing its reproduction by budding from the host cell - Consider for patients w complicated disease thought to be related to influenza or patients at high risk of complications (CHD, resp disease, immunosuppression) - Use within 48 hrs of sx - shortens duration of sx
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Features of severe pneuonia
\>/= 2 of - severe resp distress - severe hypoxaemia or cyanosis - marked tachycardia - altered mental state OR empyema
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Pleural fluid analysis, findings of empyema - pH - glucose - LDH - Protein - serum protein ratio
1. pH \<7.0 2. Glucose \<40 mg/DL 3. LDH \>1000 IU 4. Protein level \> 3.0 g/dL 5. Pleural fluid: serum protein ratio \>0.5
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Features of adenovirus infection
Can be asymptomatic **Acute respiratory distress** = bronchiolitis, pneumonia o May have features typical of bacterial disease (lobular infiltrates, high fever, _parapneumonic effusions_) o Pharyngitis, coryza, sore throat, fever **Follicular conjunctivitis** o Pharyngoconjunctival fever = high temperature, pharyngitis, **non-purulent** conjunctivitis, lymphadenopathy Gastrointestinal infection = **diarrhoea**, usually self-limiting **Haemorrhagic cystitis**
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Penicillin susceptibility iV vs oral MIC (intermidiate)
**IV** Sens – MIC \<2; intermidiate 2-4 mcg/mL NOTE use ceftriaxone if intermediate sensitivities **Oral** Sensitive if MIC \<0.05; intermediate 0.05-1
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Natural history of Tb in children vs adults (risk of progression to Tb disease) - why do we treat latent disease in children?
Tb exposure -\> 70-90% no infection vs 10-30% become infected * Of those infected, in 90% Tb will remain dormant (not infectious, never develop Tb) vs 10% will develop active tb at some stage NOTE the **rate of Tb disease in children infected is INCREASED in younger children compared w adults** - This is why we treat latent infection in children (also because young children are at **higher risk of extra-pull disease** and have more years to potentially develop TB disease)
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TB definitions: Latent Tb infection Tb disease Uninfected
Latent - Asymptomatic but positive TST and normal CXR Tb disease - Positive TST, symptomatic and/or abnormal CXR or with ACB or MTB cultured Uninfected, TST negative and clinically well
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hypersensitivity in children w Tb - what type of HS? - when does it occur - how does it present
Type 4 HS Occurs 3–8 wk after primary infection and may be heralded by (or may be asymptomatic): * prolonged ('Wallgren's) fever * formation of a visible primary complex on chest radiograph (CXR) * hypersensitivity reactions such as erythema nodosum and tuberculin skin test (TST) conversion.
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Inferferon I Release Assays in Tb How do these work Examples of such assays (2) Advantages and disadvantages Indications for IRA
Test for 2 or 3 Ag only: ESAT-6, CFP10, TB7.7 1. Quantiferon gold assay - T cells from individuals infected w Tb become sensitised to ESAT-6 or CFP-10 Antigens - When T cells encounter these Ag in vitro, they release cytokine IFN-gamma - Assay measures the amount of IFN-gamma released 2. T-spot Tb Positives - Unaffected by BCG vaccine - Unaffected by non-Tb mycobacteria (highly specific) - Only one patient visit - Simple yes/no - less subjectivity in terms of measuring - not affected by 'booster' Disadvantages - Expensive - Requires blood test - Technically more difficult than skin prick Indications - If family cannot return for TST reading - If TST boosting likely to occur - Taking blood for other reasons - TST is borderline in lower risk ppl - TST positive but past BCG vaccination - If increased sensitivity required
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Diagnosis of M Tb
Sx + CXR evidence + TST or IRA Specimen - sputum (limited in children as unable to expectorate, swallow sputum instead) - gastric washings (NG) - BAL - Other tissue (LN, CSF, urine etc) Stain - Zeil Neilson for AFB Culture takes 6-8 weeks PCR
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Tb treatment regime
1. Isoniazid 2. Rifampicin 3. Ryrazinmide 4. Ethambutol (SE = optic neuritis -\> eye check prior to starting this) Generally well tolerated in kids w minimal SE Use just Isoniazid for 6mo for latent Tb w post TST OR 6-12 weeks if recent exposure but Neg TST Pulmonary Tb disease - just 3 of the drugs for 6 months + contact tracing +/ pred if any bronchial obstruction Military and extra-pulmonary Tb (spread via lymphatics/blood) - 3 drugs for 2 mo then Isoniazid/rifampicin for 10 mo (total 12 mo) - contact tracing - Pred if Tb meningitis Isolation until smear negative if disease present
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Bronchogenic cyst What is it Where is it found PResentation Cx CXR features Tx
* Congenital malformations of the bronchial tree * Arise from anomalous budding of the foregut during development (cannalicular stage, usually days 30-40) * Most common = middle mediastinal Presentation o Typically present during the _second decade of life_ with **recurrent coughing, wheezing (which may simulate asthma), and pneumonia** o Newborns with rapidly enlarging central cysts can develop respiratory distress, cyanosis, and feeding difficulty (_mass effect_/compression) CXR features • Usually fluid filled (lined with ciliated epithelium, produces mucous) -\> may have air fluid levels • Usually appear as paratracheal soft-tissue density rounded structures Cx * Superinfection * Haemmhorage * Rapid grow in size * Rare malignant transformation Tx * Some authors advocate surgical excision of all cysts given their tendency to become infected or rarely, to undergo malignant transformatio
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Pneumocystis pneumonia (PJP) RFs Presentation CXR features Tx
RFs - HIV infection (low CD4 cell count) - post-transplant - cancer (especially haematologic) - Immunosuppressive medications (incl chemo, steroids) Presentation - fever + dry cough (can be asymptomatic in pts w HIV) CXR - bilateral diffuse interstitial infiltrates Tx - Cotrimoxazole (trimethoprim-sulfamethoxazole)
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What is the normal pulmonary circulation MAP? what is pulm HTN defined as?
Pulmonary circulation MAP10mmHg Pulm HTN \> 25mmHg
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What do your central and peripheral chemoreceptors respond to and where are they located?
Central chemoreceptors i. Situated on ventral surface of medulla, surrounded by CSF ii. Respond to CSF [H+] iii. CSF [H+] is a reflection of CO2 in cerebral capillaries iv. ↑ PaCO2 → ↑ CSF [H+] → ↑ ventilation v. Do NOT respond to PaO2 Peripheral chemoreceptors i. Situated in carotid bodies at bifurcation of common carotid arteries in neck, and aortic bodies around arch of aorta ii. Rapid response iii. Respond to ↓ PaO2, ↓ pH, ↑ PaCO2 → ↑ ventilation
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VQ ratio What is the normal value? VQmismatch
The ratio of ventilation (V) and perfusion (Q) in any lung unit Normal = 0.8 (higher in upper portions of lung and lower at base) - This is due to gravity reducing blood flow (perfusion) to lung areas above the heart ii. In pathological conditions, blood flow or ventilation can be impaired resulting in mismatch - Pneumonia, asthma, pulmonary oedema -\> blocked airways/alveoli -\> reduce ventilation -\> reduce V/Q ratio (0=shunt) - PE -\> blocks blood vessels -\> reduced perfusion -\> incr V/Q (infinity = deadspace) iii. In hypoxic conditions, pulmonary arteries constrict (opposite to systemic) and redirect to other alveoli to reduce perfusion iv. In hypoxia, airways bronchodilate to increase ventilation
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A-a gradient How does fiO2 affect Aa gradient?
- The alveolar to arterial (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) A-a oxygen gradient = PAO2 - PaO2 Higher fiO2 -\> incr A-a gradient
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Bacterial tracheitis
invasive soft tissue infection of bronchi bacterial - staph aureus preceding viral illness common common \<6yo TOXIC presentation essentially a 'bacterial croup' Complex airway - may need airway support. IV vancomycin + cef neb adrenaline for stridor
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mechanism of ventolin why do you get a paradoxical worsening
beta2 agonist - bronchodilator initial hypoxaemia due to bronchospasm
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signs of ventolin toxicity
tachycardia, tachypnoea and metabolic acidosis
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Diagnosis of narcolepsy Features
multiple sleep latency test (following normal sleep study) Ft - Sleepiness - Cataplexy (sudden involuntary muscle weakness/'drop attacks') - Hallucinations before and for sleep - Sleep paralysis
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How might CF present/be picked up?
Majority picked up on NST GI - Mec ileus in ~15% - DIOS - adhesive small bowel obstruction - GORD (often need fundoplication) Respiratory: chronic productive cough, bronchiectasis, bronchitis, nasal polyps and sinusitis Pancreatic insufficiency w fat soluble vitamin malabsorption and FTT Pancreatitis Dehydration w hyponatraemia Infertility
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where is the CFTR gene located what is the mutation?
Large arm (q arm) of chromosome 7 Point mutation - Is a deletion (D) of Phenylalanine ('F')
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CF gene mutation classes
Class (1-4 most common in caucasian populations; 1-3 are severe, 4-6 are milder forms) 1. No protein/defective protein synthesis (G542X, 2nd most common) 2. No traffic: misfolded protein -\> not trafficked to cell surface (delta508del, most common) 3. No function: channel doesn't open/gating defect (G551D, 3rd most common) 4-6. Some protein reaches the surface but 4 - less function (less Cl can move through channel due to structural problem) 5 - less protein produced 6 - less stable -\> quick turnover of protein
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G551D
Class 3 CF mutation
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DF 508 del
Class 2 CFTR mutation
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G542X
Class I CFTR mutation
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NST/screening for CF
Immune reactive trypsin test (IRT HIGH) - if result in highest 1% of population, child goes on to have genetic testing for 3 CF genes (also can be done on the guthrie card) If both positive, referral to local paed for - repeat genetics - sweat test (CF is Na \>60) - stool sample (elastase low, chymotrypsin absent, and high fat content) - genetics for parents
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what is a positive/diagnostic sweat test result for CF what is the detection rate for CF?
Na and Cl both high! Cl \> 60mmol/L Na \>60mmol/L (\<40 NOT CF) sweat weight \>75mg 94% detection rate
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Conditions which give you false positive result on sweat Cl
Adrenal insufficiency Hypothyroid or parathyroidism Glycogen storage disorders MPS G6PD deficiency DI Klinefelters
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Conditions which give you false neg result on sweat Cl
Malnutrition Skin oedema Mileralocorticoid use
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NST picks up what % of CF cases?
95% of cases (5% missed)
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Can CF NST pick up carriers?
Yes
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Management of CF
``` Staph prophylaxis for at least first 2 years to prevent staph colonisation Treat infx (Abx according to bugs isolated on sputum in past) Airway clearance (PT, mannitol, hypertonic saline nebs, pulmozyme, DNaze) ```
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Infectious bacteria and Abx for infections/exacrebations in bronchiectasis
Staph aureus \> PO fluclox or augmentin or IV PSA -\> first isolation of PSA: IV tobra + ceftaz/mero or PO cipro and tobramycin neb as eradication trial -\> chronic PSA: Tobramycin alt months Reduce inflammation - azithromycin ``` Burkholderia cepacia (also poor prognostic indicator) - first isolation try for IV eradication: meropenem, temocillin and bactrim ``` Stenotrophomonas (unclear clinical significant but treat if symptomatic) - bactrim, doxy - IV tazocin, mero, ceftaz Non-tb mycobacteria (NTM) - M avid complex - M abscessus complex - other species - treat if smear positive and symptomatic (x2 positive samples) - need to be confident NTM disease is present (not as non-clinically significant commensal) as tx course if long(12-15 mo after first neg culture) and toxic
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Fungal aetiology of exacerbations and treatment of these in CF
- candida albicans 75-90%: nil stat in mouth or fluconazole if in BAL - aspergiillus fumigates 40-60% itcraconazole - scedosporium apiosperum: can be commensal. treat w voriconazole or posaconazole if symptomatic as can cause allergic pulmonary mycosis
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CF carrier rate and incidncece rate in developed world
carrier 1 in 25 in developed world incidence 1 in 2500
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Presentation and diagnosis of CF related diabetes
More common in girls \>10yo Often assoc w drop in FEV1 and LOW in absence of resp exacerbation Diagnosis on OGTT
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At what FEV1 cutoff would you refer a CF patient for lung transplant
FEV1 \<30%
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adjustments made to creon based on the ____ content of meals
fat
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What fat soluble vitamin deficiency causes neurological sx (ataxia, weakness, poor coordination, decr vibratory sense and bilateral hyporeflexia)?
Vitamin E deficiency
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Clinical utiltily of LFTs
Asthma CF NM disorders (DMD) Severe ChILD Chronic neonatal lung disease
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Lung volume definitions Vital capacity Residual volume tidal volume FRC
Vital capacity : vol of air expired following maximal inspiratory breath/effort residual volume: vol of air left in lungs following max insp/exp effort \*never reduced in any paediatric conditions\* Functional residual capacity: amt of air left in lungs following normal in/out breath (vital volume) tidal volume: normal insp and exp volumes
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Formula of total lung capacity can spirometry measure this and why?
Vital capacity + residual volume Cannot be measured by spirometry because cannot measure residual volume
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Patient factors that affect lung volumes
Height Gender Ethnicity Age
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Spirometry measures what is considered abnormal in kids?
1. FVC = vital capacity (max amt of expired air after maximum inspiraotyr capacity) - \> abnormal if \<80% predicted 2. FEV1 (or FEV 0.75) - \> vol of air expired in 1st (or first 0.75) seconds of expiration - \> abnormal if \<80% predicted 2b. ratio of FEV1/VC values - \> abnormal if \<78 ~80 (actual value, not % predicted) 3. FEF 25-75% - average RATE of flow during middle half of an FVC manoeuvre - \> marker of disease affecting MEDIUM sized airways (CF, asthma, bronchiolitis) - \> anything below 67% is abnormal Anything BELOW 1.64 z- scores of reference (we don't care about values that are 'too high')
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What type of defect is this?
Obstructive - PEF reduced - FVC may be reduced - Inspiratory curve normal - Exp curve CONCAVE
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what is the earliest PFT sign of obstructive lung disease?
Reduction in FEF 25-75% is earliest sign high sensitivity, low specificity - sensitive (normal value rules OUT restrictive lung disease) but NOT specific (low result does not mean it is necessarily an obstructive lung disease (hard to interpret if the VC or FVC is abnormal))
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Restrictive spirometry
TLC \< 1.64 z score (LOW) FVC reduced with Normal to increased FEV1/VC \*note most common cause of low TLC is low effort/poor technique
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obstructive spirometry values
Low FEV1 and FEV1/FVC \< 1.64 z score FVC normal
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what is this lung defect ?
suggestive of Restrictive lung disease - FVC reduced and flow is higher than expected at a given lung volume HOWEVER spirometry cannot strictly diagnose restrictive lung disease as cannot measure TLC (RV) - normal VC rules out restrictive lung disease - need body plesmysthography to measure TLC
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FVC low FEV1 low FEV1/FVC normal
Bad technique Restrictive
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Low FVC Normal or incr FEV1 Normal or incr FEV/FVC
Restrictive or poor technique/inadequate effort
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Low FVC, FEV1, FEV1/FVC
Mixed defect SEVERE obstruction or obstruction w inadequate effort (if FEV1/FVC ratio is low, obstruction has to be there)
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What findings rule out obstruction vs restriction on spirometry?
Normal FEF 25-75% rules out obstruction Normal FVC rules out restriction
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FVC nromal FEV 1 low FEV1/FVC low
Obstructive
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Normal FVC Normal FEV1 Low FEV1/FVC
Obstruction OR Dysanaptic growth = unequal growth of lung parenchyma relative to lung airways (seen in preterm and obese children)
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DLCO what is this and why do we care?
Measures diffusion capacity to CO Reduction in DLCO is seen in ILD Helps to distinguish between causes of restrictive lung disease - DLCO _low_ -\> ILD, pneumonitis, scleroderma, miliary tb etc - DLCO _normal_ -\> scoliosis, obesity, chest wall abnormalities or NM disorders
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Positive bronchodilator response - what is a positive result?
If you give 4 puffs of 100mcg salbutamol and repeat spirometry after 15 minutes, POSITIVE= FEV 1 and/or FVC INCR of \>12% and 200ml from baseline = airway hyperreactivity/asthma
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what does this spirometry loop show? ddx?
Shows relative flattening of inspiratory loop = variable (not fixed) EXTRA- thoracic (central or upper) airway obstruction (ex: laryngeal paralysis, vocal cord dysfuncton )
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what does this show? ddx?
relative flattening of exp flow loop compared w insp flow loop Variable (not fixed) INTRA-thoracic (central or upper) airway obstruction ddx: tracheomalacia
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what does this loop show? ddx?
flattening of insp and exp flow curves FIXED central or upper airway obstruction ddx tracheal stenosis, subglottic stenosis
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what is the answer?
D) obstruction w positive bronchilator response
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how do you diagnose restrictive lung disease?
Body plethysmography - need TLC which can't be diagnosed on spirometry as can't calculate RV
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what is the defect?
MIXED: definite obstruction and restriction cannot be ruled out without body plethysmography
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what is the defect?
Could either be EARLY obstruction (normal FEV1/FVC but low FEF25-75%) or NORMAL Negative BD response
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Presentation of FB
\<4yo Often unwitnessed choking/inhalation and may have occurred days or weeks prior to presentation NORMAL resp exam or CXR doesn't exclude inhaled FB ``` Persistent wheeze (may be focal and partially respond to bronchodilators) and/or cough WOB, stridor, Focal wheeze or decreased breath sounds +/- drooling ```
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Mx foreign body
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Mx of croup
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Diagnosis? **Sore throat** Fever **Neck pain and stiffness or torticollis** Fullness and redness of posterior pharyngeal wall; may be midline but can be laterally behind tonsil **Dysphagia and drooling**
Retropharngeal abscess lateral neck xray - paravertebral soft tissue swelling
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Diagnosis? Severe sore throat (often unilateral) Hot potato/muffled voice Trismus Swollen posterior palate and tonsil, with medial displacement of tonsil and deviation of the uvula
Peritonsillar abscess (quinsy) Causes - Strep pyogenes, staph aureus, HIB, neiseria - fusobacterium (anaerobic) tx - clindamycin or third gen cephalosporin (due to incr presence of beta lactamase producing oranisms resistant to penicilins) - or amox + metronidazole
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Diagnosis? Inadequate Hib immunisation or immunocompromised High fever and systemically unwell Muffled voice Hyperextension of neck Dysphagia Pooling of secretions, drooling Absent cough Low pitched expiratory stridor or stertor
Epiglottitis
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Diagnosis? Systemically unwell More severe and rapidly progressive symptoms (stridor etc) Recent URTI Markedly tender trachea Cough may be productive with thick secretions
Bacterial tracheitis
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Most common cause of croup
parainfluenza
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most common causes of bronchiolitis
RSV in 80% of cases Human metapneumovirus Adenovirus Influenza Parainfluenza Rhinovirus
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Diagnosis of primary ciliary dyskinesia
Screening - nasal NO levels (_low,_ need to be done on 2 separate occasions as can be transiently low w URTIs) Electron microscopy for cilia cross-sectional structure can be normal in some patients w PCD Gene panel
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Inheritance pattern, Sx and Cx of PCD
**Autosomal-recessive** Disorder of motile cilia characterised by **chronic lung disease (bronchiectasis)**, **chronic nasal congestion and sinusitis** from first few months of life, **recurrent OM** -\> **hearing impairment** and **subfertility** (all men infertile; women variable, incr risk ectopic preg). Nasal symptoms and respiratory distress usually start soon after birth, and by adulthood bronchiectasis is invariable.
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Stages of lung development inutero
Embyronic Pseudoglandular (from 4-6wks) Cannalicular (from 16wk) **S**accular = **s**urfactant (from 24 weeks) Then alveolar from 36wk
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In the performance of spirometry in 6 to 12-year-old children, the flow volume curves should appear similar in configuration on repeat testing. What is the maximum allowable variation on repeat testing?
150ml
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What condition is most likely to have low lung volumes and a low DLCO?
ILD
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Inhaled NO - what are its uses - MOA - method of inactivation - half life
Indications: neonatal pulmonary HTN, airspace disease MOA: Vasodilation of aerated airspaces via cGMP pathway, NO can redirect blood flow from poorly ventilated areas, atelectatic or diseased lung regions, to better aerated air spaces and improves oxygenation and ventilation perfusion mismatch Method of inactivation: When it reaches the vascular lumen, NO avidly binds to haemoglobin and is thereby inactivated Half-life of 2-6 seconds.
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Components of pulmonary surfactant
70-80% Phosphatylcholines (type of phospholipid) 10% Surfactant protein A 10% Neutral lipids
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Ddx biphasic stridor
FIXED obstruction/lesion needs to be at level of vocal cords or glottis * Laryngeal web or laryngeal mass * Subglottic haemangioma or subglottic stenosis * B/L vocal cord paralysis (assoc w FTT, feeding difficulties) * Other vocal cord lesion
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Stertor vs stridor
stertor - UPPER airway noise - noises produced nasal cavities, pharynx, tonsil stridor - middle airway noise (supra glottis, glottis, sub glottis)
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Ddx inspiratory stridor
Obstruction at level of glottis/supra glottic/sub glottis - Laryngomalacia - Subglottic stenosis - Other subglottic pathology (FB etc)
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Ddx expiratory stridor/wheeze
Obstruction at level BELOW subglottis ie trachea or below - tracheomalacia - lower tracheal or bronchial foreign body - vascular ring
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Commonest cause of stridor in neonatal age group (birth - 3mo)
1. Laryngomalacia 2. Bilateral vocal cord palsy 3. Sub glottic stenosis then can have choanal atresia, subglottic haemangioma etc
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Common causes of stridor in 18mo + group
Foreign body Tracheomalacia Acquired subglottic stenosis (intubation related etc)
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What airway pathology is associated with 22q11/di george syndrome?
Laryngeal webs - 65% of pts
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weak or absent cry biphasic stridor may be present at birth What's the diagnosis? What syndrome is this assoc with?
Laryngeal web Could also be assoc w di george syndrome
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Pink on crying, blue on BF in neonate - dx? cause? What is the bedside test to investigate for this condition? ix? mx? what is associated?
B/L Choanal atresia - bone or membrane at back of nasal passage behind turbinates blocking nasal passage Caused by failure of recanulisaiton of nasal fossa during development. Passage of size 6 french feeding tube through both nostrils CT sinuses is next 60% Assoc w other congenital anomalies (CHARGE syndrome/association) Mx - if B/L require surgical mx (hole in membrane or stent in bone)
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laryngomalacia sx pathophys tx
most common cause of stridor in neonates Inspiratory stridor esp when upset Feeding difficulty, choking +/- apnoeas, blue spells, O2 requirement 50% have associated severe reflux (Mx w losec) Pathophys: immature cartilage or neural maturation Intervene only w aponeas/O2 requirement/FTT (excision of floppy supraglottic areas)
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Bilateral vocal cord paralysis sx causes mx
Sx - inspiratory stridor, feeding difficulty, weak cough, hoarse voice, aspiration risk, dyspnoea Causes - Idiopathic 80% (neonatal age group) - Birth trauma - Tumour - Prolonged intubation Mx - some neonates improve on their own; 80% need temporary tracheostomy 12-24%
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Subglottic haemangioma or infantile/capillary
NOT present at birth Onset ~4-16 weeks after birth as lesion grows My be other associated haemangiomas visible on skin Sx - recurrent, persistent and/or progressive, **inspiratory or biphasic stridor**, **respiratory distress** and **feeding difficulties** Mx - propanolol 3mg/kg/day BD minimum 12 months. NOT excision.
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Congenital haemangioma - percentage of newborns affected, RF Natural hx Tx
1-3% newborns RF: LBW, preterm birth weight Start to proliferate around 4 weeks Involution from 12 months Tx - most don't require treatment. propranolol only if very large or in airway causing obstruction or in eyes or brain
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Rapidly involuting congenital haemangioma (RICH)
rapid onset and rapidly regress within 6 weeks of onset; but can ulcerate and cause transient thrombocytopaniea/coagulopathy.
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Non-involuting congenital haemangioma (NICH)
Involutes in late childhood (ie haemangioma in a 4 year old). DOES NOT respond to propranolol.
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Inspiratory stridor with FTT/feeding difficulties in child that improves when nursed prone
Vallecular or epiglottic cyst (between tongue and epiglottis) - presents first 2 weeks of life - Thyroglossal duct origin - complete excision is required to obtain long lasting cure.
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Tracheo and bronchomalacia - presentation ix tx
Excessive tracheal or bronchial collapsivity Clinical presentation includes early-onset EXP stridor or fixed wheeze, recurrent infections, brassy cough and even near-death attacks, depending on the site and severity of the lesion. ix - definitive/gold standard diagnosis w flexi bronchoscopy. radiological diagnosis w cxr or CT
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Most common cause of parotid mass in children 6mo and under
Parotid congenital haemangioma First line ix with doppler USS
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Laryngeal web what is it? why/how did it form presentaiton mx
A rare malformation consisting of a membrane-like structure that extends across the laryngeal lumen close to the level of the vocal cords Due to incomplete recanulisation of larynx Mostly seen between the vocal cords and has a concave posterior margin presentation - airway obstruction (biphasic stridor) weak cry or aphonia from birth mx - excision
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Types of stridor and sites of origin
Inspiratory - above glottis Expiratory - below sub glottis (thoracic trachea, bronchi) Biphasic - glottis, subglottis, cervical trachea
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Mx of feeding difficulties associated with cleft lip/palate
1. Special cleft palate bottles - If this fails then proceed to: 2. Nasopharyngeal airway (NOT NG tube) - if baby can breath, they can feed
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Fluctuating sensorineural hearing loss - ddx?
FLUCTUATING SNHL = congenital hearing loss assoc w vestibular aqueduct in inner ear (Mondini malformation) Minimal hearing loss at birth, progressing over first 10 years of life Assoc w balance disturbance, dizziness Associations - recurrent meningitis (connection to CSF) - thalidomide nad rubella embryopathies - pendred syndrome (BL SNHL and goitre) - CHARGE - Digeorge - Wildervanck
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what sort of hearing loss does a cholestatoma give?
Usually unilateral conductive hearing loss mixed only if it goes into inner ear
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what type of hearing loss is assoc w goldenhar syndrome?
Conductive
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what type of hearing loss is assoc w otitis media w effusion
SMALL amt of conductive hearing loss (\<50 dB)
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what type of hearing loss does otosclerosis give you?
abnormal fixation of stapes bone = conductive hearing loss
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Causes of hearing loss at birth
21% connexin 26 deficiency (AR GJB2 mutation ) 21% CMV infection 14% Syndromic 14$ 30% Other genetic non-syndromic 3% Pendred's syndrome Other environmental causes 14%
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Most common presentation of cholesteatoma
Otorrhea If bad enough, can cause hearing loss, otalgia, hearing loss, facial palsy, dizziness
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Mx of Otitis media what about if cx by mastoiditis?
1. Pain assessment and management 2. Antimicrobials Under 2yo: treat w abx for severe infections (risk of abx) Over 2yo: hold abx for 48 hrs Abx: amoxicillin 3. Mastoiditis: IV flucloxacillin plus 3rd generation cephalosporin (risk of intracranial cx)
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Causative Pathogens for otitis media
1. Streptococcus pneumoniae 2. nontypeable Haemophilus influenzae 3. Moraxella catarrhalis 4. Group A streptococcus
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Environmental RF for otitis media in children
1. daycare outside of the home/exposure to other children (biggest RF!) 2. low SES 3. Race 4. Formula feeding 5. Exposure to tobacco
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Peritonsillar abscess/Quinsy Causes Age group affected PResentation Ix Mx
Causes - GAS - Staph aureus - Haemophilis influenza Deep-seated infection that spreads secondary to tonsillar infection Occurs more commonly in young adults Presentation: 'hot potato voice' (muffled), unilateral throat pain, deviation of uvula, truismus (decr ROM jaw), tortocollis (decr ROM neck), red throat and exudative tonsil, drooling, fever ix - ultrasound mx - IV fluids, I&D, analgesia, IV augmentin
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REtropharyngeal abscess Cause Presentation/age affected Ix Mx
Cause (Secondary to infx elsewhere in head/neck region) - group A Streptococcus - S. aureus - H. influenzae Sx: Affects age \<= 5years Neck pain and MASS, limitation of neck movement/ tortocollis, + fever, sore throat, rarely respiratory distress (WOB, wheeze/stridor) ix - xray may show soft tissue swelling posterior to pharynx however CT is much better (look at airway narrowing and distinguish abscess from cellulitis) mx - IV augmentin
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Sleep disorder sx in children
Anxious Irritable Poor concentration Inattentive Impulsive
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physiological changes during sleep
Drop in BP and body temp Decr in muscle tone Incr in upper airway resistance (2x in REM) Decr in tidal volume (1/2 in REM) THUS sleep is like a stress test for your respiratory system -\> any impairment of ventilation when awake will be worse in sleep (including laryngomalacia)
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Functions of sleep
1. Consolidated learning + pre-req for ongoing learning 2. Restorative - immune and brain 3. Hormonal (GH secretion) -\> growth
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Effects of sleep deprivation
Blunted hormone response (GH) -\> poor sleep Pro-inflammatory cytokine secretion TNFalpha section -\> CV disease and DM Behavioural changes (similar to ADHD) Assoc w obesity in MALES ONLY (tired and hungry due to decr leptin and incr ghrelin and decr exertion) Assoc w increase mortality
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Stages of sleep
NREM - N1: transition to light sleep, easily roused - N2: light sleep (K complexes and spindles) - N3: deep sleep (slow wave sleep = delta waves), very hard to rouse, very regular breathing REM: dream sleep. more common in 2nd half of night. - decr tone - rapid eye movements - partial paralysis - irregular breathing - incr upper airway resistance - decr tidal volume - easily rousible
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what stage of sleep?
N2 (NRem)
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What stage of sleep?
REM Can see no chin movements (paralysed) and eye movements in opposite directions
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what percentage of children don't have any daytime naps by 5 years of age?
95%
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what stage of sleep decreases relative to the others as you age?
REM sleep decreases proportionate to NREM sleep
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Types of behavioural insomnia
\*problem falling asleep and staying asleep\* Type 1 - sleep association type Type 2 - limit setting disorder (naughty children) Type 3 - Mixed Mx - Exclude physiological cause (red flag - if child falls asleep ok but wakes up during night) - daytime behaviour/limit problems - Sleep hygiene If behavioural - sudden or graduated extinction (controlled crying) - fading (move bedtime 15min every 3 nights) with positive bedtime routines (reading etc)
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Physiologic causes for night waking
GORD Eczema Asthma OSA PLMD \*ADHD\*
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Mx of sleep disturbance in ADHD
Behavioural therapy Melatonin Atomoxetine instead of stimulants (norepinephrine reuptake inhibitor and is believed to work by increasing norepinephrine and dopamine levels in the brain)
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What is delayed sleep phase
Common in adolescents 'Permanent jet lag' - going to bed LATE (1, 2am) and getting up late Promoted by Late homework, TV, texting, internet Mx - sleep hygiene, advance bedtime by 15mins each 3 night, melatonin as adjuvant
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Night terror vs nightmare
Night terror is a form of Parasomnia - Abnormal arousal at end of N3 ie tend to occur same time every night - Runs in Families - 39% of children - Mx: timing wakenings, clonazepam Nightmare - awakening during REM. - can recall event in morning.
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What is rhythmic movement disorder
Age group \<4years Occurs just before sleep onset and slightly into stage 1 Head banging 3-6.5% Is a normal variant in most cases!! RF: autism, developmental delay
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Periodic limb movement disorder - what is it caused by - treatment
Part of restless legs spectrum but this occurs only in sleep Partial iron deficiency in CNS Treat with iron to keep systemic ferritin \> 50 (forcing iron into brain) -\> sometimes need to use injection of IV tx if PO oral doesn't work
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Hypersomnia what is it? what is the most common cause?
Difficulty staying awake in a situation where person would normally be expected to be awake most common cause is LACK OF SLEEP 2. OSA 3. Circadian rhythm 4. CNS tumours 5. Psychiatric disorders (depression etc) 6. Medications (beta blockers, alcohol) 7. Substance abuse
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What causes narcolepsy
Deficiency of hypocretin 1/orexin (involved in staying awake)
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Periodic sleepiness ddx for primary hypersomnias
Klein levin syndrome (**obese** patient w **periodic sleepiness and hypersexuality**) Menstrual related hypersomnia
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Constant sleepiness ddx for primary hypersomnias how to investigate?
1. Narcolepsy 2. Primary idiopathic hypersomnia with low or normal sleep time (= familial sleepiness) 1. Symptoms often begin between adolescence and young adulthood and develop over weeks to months. 2. People with IH have a hard time staying awake and alert during the day (chronic excessive daytime sleepiness, or EDS). 3. They may fall asleep unintentionally or at inappropriate times, interfering with daily functioning. They may also have difficulty waking up from nighttime sleep or daytime naps. 4. Sleeping longer at night does not appear to improve daytime sleepiness ix with **multiple sleep latency test** - measure short time for falling asleep (\< 8 min) WITH REM during day (abnormal)
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What type of CNS tumour causes excessive sleepiness
hypothalamic tumour
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Mx narcolepsy
Routine - regular good quality sleep Scheduled naps Stimulant medication - methylphenidate or dexamphetamine - Modafinil if the above fails +/- Cataplexy ('drop attacks' with sleep or laughter) - SSRI (fluoxetine) first line - venlafaxine
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Cytokines and inflammatory cells involved in pathophys of asthma (vs nonatopic response)
Atopic response: Th2 -\> IL4,5 13 -\> IgE mediated atopy and eosinophilic inflammation Non atopic: Th1 -\> IL2, IFN gamma -\> IgG protection and suppression of atopic Th2 response
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what does - multi breath nitrogen washout - DLCO measure?
- Multi breath nitrogen washout measures lung volumes (incl residual volume) - DLCO measures gas transfer from alveoli into blood (alveolar-capillary unit function) -\> reduced in CF, ILD, pulmonary vascular disease (PE, PPHN)
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Presentation of bronchogenic cyst
Asymptomatic at birth, often incidental finding on CXR (mediastinal mass +/- Air fluid level on CXR) Present when secondarily infected or they enlarge in size and compromise an adjacent airway Tx: surgical resection
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What is this cxr of? what are the features?
Bronchiologitis Features - Hyperinflation (horizontal ribs, flattened diaphragm) - patchy atelectasis (often RUL) - peribronchial thickening
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Pulmonary sequestration - what is it and what are the two types, presentations and relative prevalences?
Pulmonary sequestration, also called accessory lung, refers to the aberrant formation of segmental lung tissue that has no connection with the bronchial tree or pulmonary arteries. It is a bronchopulmonary foregut malformation (BPFM). 2 types: - Intralobar sequestration (ILS) - \> accounts for the majority (75-85% of all sequestrations 4,5,7) - \> present later in childhood with recurrent infections - \> venous drainage via pulm veins to LA - Extralobar sequestration (ELS) - \> Less common (15-25% of all sequestrations 4,5,7) - \> Usually present in the neonatal period with respiratory distress, cyanosis, or infection - \> almost always affect LLL however 10% of extralobar sequestrations can be subdiaphragmatic - \> venous drainage via systemic veins to RA
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prevalence of asthma
10% or 1 in 10
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Asthma - good control - vs partial control - vs poor control
- good control: daytime sx \<=2 days per week, no limitation in activity, no nocturnal sx and need for reliever \<= 2 days per week - vs partial control: daytime sx \> 2 d/week, any limitation in activity, nocturnal sx, need for reliever \>2 d/week - vs poor control either daytime sx \>2 days per week, min-hrs and recurring OR \>= 3 ft of partial control within same week
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Treatment of acute asthma (severe)
O2 if spO2 \<=90% persistently Salbumatol via MDI-spacer 1 dose every 20 minutes for an hour then reassess need for ongoing Ipratropium by MDI/spacer - 1 dose every 20 minutes for 1 hour only Oral prednisolone (see below) IV MgSO4 IV aminophilline Consider ICU admission If critical - needs neb salbutamol, atrovent and IV methylpred in addition to the things above - Consider IV salbutamol - ICU admission
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Sodium cromoglycate - what is its MOA - indication for use
Mast cell stabiliser First line tx for frequent intermittent asthma in the 1-2yo age group (before trialling ICS as second line)
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What is pseudo-bartter syndrome?
Hypokalaemic alkalosis secondary to Na and K loss in SWEAT Occurs in very unwell children with CF
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What is the spirometry defect(s) seen in CF?
Early on get obstructive picture Picture becomes more restrictive w disease progression (FVC reduced \> FEV1)
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Causes of bronchiectasis
Post-infectious (Severe pneumonia, whooping cough, measles) Inhaled FB CF IgA deficiency IgG subclass deficiency Primary ciliary dyskinesia
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what is the defect that causes primary ciliary dyskinesia
AR (incomplete penetrance) Absence of dyne arms on the cilia resulting in absent or defective ciliary action Affects lungs, nose, ears and sperm ducts Sx - Neonatal respiratory distress, poor feeding, FTT - Chronic sinusitis - Otitis media (conductive hearing loss) - Chronic productive cough +/- wheeze - Infertility - poor sperm fertility, ciliary dysfunction in fallopian tubes - Can also present with neonatal hydrocephalus (ventricles lined by cilia, important for CSF flow) and retinitis pigmentosa 50% assoc w situs inversus
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What is the respiratory presentation of alpha 1 antitrypsin deficiency?
Emphysema - But presents at 30-50 years of age, rarely younger - Hyperinflation on CXR
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What is this condition based on the path shown? What is the classic triad of features Causes?
Pulmonary haemosiderosis: chronic diffuse alveolar haemorrhage - brown lung tissue with haemosiderin laden macrophages Triad 1. Iron def anaemia (low Hb and haematocrit, low iron; incr relics and bilirubin) 2. Haemoptysis 3. CXR: multiple alveolar infiltrates Causes - primary; goodpastures - secondary: vasculitis (SLE, Wegeners, HSP), HUS, PPHN
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Diagnostic pathway of asthma
1. history and exam findings 2. spirometry (if \>= 5yo) 3a. If spirometry abnormal, do bronchodilator reversibility (BDR) testing - \> if positive, asthma. - \> If negative BDR, do FeNO (exhaled nitric oxide) -\> if elevated, suggests asthma. 3b. If spirometry normal, does not exclude asthma. Can go on to do FeNO.
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Exhaled nitric oxide (FeNO) test - what is it used to diagnose/what does it indicate?
Use in children \>= 8yrs Positive test suggests eosinophilic inflammation ``` Provides supportive (not conclusive) evidence for asthma diagnosis -\> high level in asthma, atopy, allergic rhinitis, eczema, eosinophilic bronchitis ``` -\> Normal test does NOT exclude asthma Can be used to distinguish between poorly controlled (high) vs well controlled asthma (low)
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Asthma treatment step up approach \< 5years
Step 1: SABA PRN Step 2: Regular preventer (Low dose ICS or montelukast) + reliever PRN Step 3: Low dose ICS + Montelukast + reliever PRN Step 4: Refer to paed/resp specialise
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Asthma treatment step up approach \> 5years
Step 1: SABA PRN Step 2: Regular preventer (Low dose ICS OR montelukast) + reliever PRN Step 3: High dose ICS OR Low dose ICS + Montelukast OR ICS/LABA combination (low dose) + reliever PRN Step 4: Refer to paed/resp specialise
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What asthma inhaler to use in different age groups
\<4: mask w MDI and small vol spacer 5+: MDI and spacer (no mask!)
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SE of ICS
Small effect on growth 0.7% reduction in adult height
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LABA use in children Examples Age group to use in principles of use Why aren't they commonly used in children?
ex: formoterol, salmeterol Should not be used \<=4 years old Should ALWAYS be used in combination with an ICS (mono therapy is unsafe) Causes downregulation/reduciton in Beta2 adrenoceptors
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SMART therapy
Symbicort (low dose budesonide-formoterol) combination can be used for both regular BD maintenance use AND as a PRN reliever (instead of salbutamol) \*Note - not suitable for Seretide (fluticasone/salmeterol) as not as quick onset - not suitable for young children
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MOA Omalizumab in asthma
Binds to free IgE so it can't bind to IgE receptor Reduces cell bound IgE and thus get downregulation of number of receptors Have to be \>6yrs, have severe asthma, have very high levels IgE etc Injection administered q4weekly
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MOA Mepolizumab and Reslizumab in asthma
Bind to IL-5 (monoclonal Ab) Inhibit proliferation/activation of eosinophils and B cells
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MOA Benralizumab in asthma
monoclonal Ab that Binds IL-5 receptor prevents activation/proliferation of eosinophils and B cells
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Bronchoprovocation testing in asthma - indication - diagnostics
Bronchoprovocation testing with either methacholine or histamine is useful when spirometry findings are normal or near normal, especially in patients with intermittent or exercise-induced asthma symptoms. Bronchoprovocation testing helps determine if airway hyperreactivity is present, and a negative test result usually excludes the diagnosis of asthma. Methacholine is administered in incremental doses up to a maximum dose of 16 mg/mL, and a 20% decrease in FEV1, up to the 4 mg/mL level, is considered a positive test result for the presence of bronchial hyperresponsiveness.
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What electrolyte/blood gas abnormalities might you see in undiagnosed CF?
Hyponatraemia from excessive sweating Hypokalaemic hypochloraemic metabolic alkalosis
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What tests is most likely to detect early respiratory failure in neuromuscular disease?
Polysomnography
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is bronchiectasis obstructive or restrictive?
obstructive - causes lower airway DILATATION
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MOA - montelukast
**leukotriene receptor antagonist** * blocks the action of leukotriene D4 in the lungs resulting in decreased inflammation and relaxation of smooth muscle.
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Genetic surfactant deficiency conditions (x3) and inheritance patterns and presentation
1. **_Surfactant Protein B_** (SP-B) Deficiency: **neonatal** onset, **AR**. resp distress despite surfactant replacement and ventialtion. early death in first few motnhs of life. 2. **_ABCA3 deficiency_**: **neonatal or childhood** onset, **AR**. may present at birth, similar to SP-B deficiency, or in older children in whom course of the disease may be milder and more chronic with cough and failure to thrive. 3. Surfactant **_Protein C._** can present at **any age**. **AD**. Variable presentation, from acute respiratory distress to a more slow-onset and chronic lung disease.
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_Parotid swelling ddx_ 1. Fever + intraoricular pus when pressure applied to parotid gland =? 2. Multiple episodes (onset age \<5yo) of parotid swelling and/or pain associated with fever or malaise over a period of years (nonobstructive, nonsuppurative) =? 3. Recurrent episodes swelling and pain worse before/after meals - may present as bacterial infection =? 4. Recurrent B/L parotidis in children and dry eyes \>5yo, fhx autoimmune diosease =? what labs would assist in diagnosis? 4. list Viral causes
1. Acute suppurative parotitis (staph aureus infx) 2. Idiopathic recurrent parotiditis 3. sialolithiasis 4. Sjogrens. positive antinuclear antibody, SS A, and SS B antibodies; presence of rheumatoid factor; and hypergammaglobulinemia 5. Viral: Mumps, HIV, CMV
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Congenital lobar emphysema/overinflation
* Characterized by respiratory distress due to _overexpansion of one or more pulmonary lobes_ of the _histologically normal lung_ without the destruction of alveolar walls with compression of surrounding lung parenchyma * The affected lobe is essentially non-functional because of overdistention and air trapping. * LUL \> RUL and RML \> lober lobes Aetiology * 50% idiopathic/unknown * Paucity/underdevelopment of cartilage in airways leading to airtrapping Presentation * Almost 50% of the patients are symptomatic at birth and another 50% present within the first six months of life * Resp distress, wheezing, retractions, and cyanosis * Difficulty in feeding * history of chronic cough and recurrent respiratory infections Ix * CXR - overinflation, hyperlucency of affected lobe; may push mediastinal structures over to contralateral side due to mass effect * CT is gold standard Tx * Conservative tx if mild-mod sx * Lobectomy if severly sx-atic