Exam Review Flashcards

Question

In a CF kid, do you expect the LCI to be normal/abnormal? Is abnormal a higher or lower value than normal?

Answer 1

Abnormal = Higher

Answer 2

FRC measured with MBW is lower, as it only measures ventilated lung CEV would be lower Therefore ? no change to LCI

Answer 3

Expiratory muscle strength Chest wall compliance and shape Lung elasticity It is determined by the force generated by the muscles of expiration and the inward elastic recoil of the lungs as they oppose the outward elastic recoil of the chest wall. Dynamic compression of the airways during the forced expiratory effort may also be an important determinant of the RV as airway collapse occurs, thus trapping gas in the alveoli

Answer 4

Baseline SpO2 <95% or end tidal CO2 >45 when awake | AHI >10 on PSG OR 4 + episodes of SpO2 <92% OR drops in SpO2 of at least 4% /hr of sleep

Answer 5

Respiratory infection present and peak cough flow <270lpm Baseline peak cough flow <160 or max expiratory pressure <40cm H20 Baseline FVC <40% predicted OR <1.25L in older teen/adult

Answer 6

No, we are not worried because B multivorans only rarely, if ever, causes cepacia syndrome **worry about Cenocepacia - genomovar III

Answer 7

B cenocepacia

Answer 8

Severe decline in CF lung function possibly developing into a life-threatening systemic infection known as cepacia syndrome. Extremely difficult to treat because of virulence and resistance Cepacia syndrome is characterized by a rapidly progressive fever, uncontrolled bronchopneumonia, weight loss, and in some cases, death.

Answer 9

``` Sweat chloride (>=60mmol/L) Cystic Fibrosis Transmembrane Conduction Regulator (CFTR) mutation analysis ```

Answer 10

- Nasal potential difference - Intestinal current measurement (from rectal biopsy) - Fecal elastase to assess for pancreatic insufficiency - Ultrasound to assess for pancreatic echogenicity and - Congenital bilateral absence of the vas deferens - Chest CT for assessment of bronchiectasis

Answer 11

Neutrophils | IL-8, (Il-1, IL-17)

Answer 12

Eosinophil | IL-5

Answer 13

Bronchogenic (foregut) cyst are closed epithelium lined sacs developing abnormally in the thorax from the primitive developing upper gut and respiratory tract. Typically single and unilocular. 50% are situated in the mediastinum close to the carina (less frequently adjacent to the esophagus and alongside tracheobronchial tree), more common on the right. Cyst lined by respiratory-type epithelium. Wall is often fibrous and inflamed, may contain seromucous glands and cartilage plates.

Answer 14

Peripheral chemoreceptors are located in (1) the carotid bodies (at the division of the common carotid artery into its external and internal branches) and (2) the aortic bodies (between the ascending aorta and pulmonary artery). They respond primarily to changes in PO2. They are also responsive to changes in arterial pH (carotid but not aortic bodies) and CO2.

Answer 15

Central chemoreceptors are located in the ventral lateral medulla. Increases in PCO2 or H+ concentration produce an increase in ventilation.

Answer 16

PTLD is a broad term describing B cell proliferation post transplant, often related to EBV Can occur in patients with solid organ (eg. Lung transplant) and HSCT. For our purposes, more common amongst lung than HSCT Pathophysiology: EBV positive B cell proliferation because of T cell suppression A late complication >6 months in lung transplant and within the first year (more so in first 1-5 months) post HSCT

Answer 17

Lung transplant, as compared to other solid organ transplants, since higher level of immunosuppression More common in CF patients with lung transplant partially due to age of patients, but also CF specific risk factors Children, since more likely to be EBV negative HSCT: allogenic, T cell depleted graft, EBV negative recipient with EBV positive donor

Answer 18

- Mediastinal lymphadenopathy - Pleural effusion - Pulmonary nodule - Pulmonary mass - Consolidation

Answer 19

Decrease immunosuppression, though increased risk of rejection Rituximab if CD20+ Chemotherapy (CHOP protocol – cyclophosphamide, doxorubicin, vincristine, prednisone) or radiation therapy

Answer 20

- Bilateral lymphadenopathy - Normal CXR - Reticular opacity - Ground glass opacity - Volume loss (in stage 4)

Answer 21

- Thickening of interlobular septa - Beaded or irregular thickening of the bronchovascular bundles - Nodules along bronchi, vessels (peribronchovascular), and subpleural regions - Bronchial wall thickening - Ground glass opacification - Parenchymal masses or nodular consolidation, occasionally with cavitation - Parenchymal bands - Cysts - Fibrosis with distortion of the lung architecture and traction bronchiectasis - CT findings show upper to mid lung zone predominance

Answer 22

Stage 0: normal CXR Stage 1: bilateral lymphadenopathy Stage 2: bilateral lymphadenopathy + interstitial infiltrate Stage 3: parenchymal infiltrate with no bilateral lymphadenopathy Stage 4: pulmonary fibrosis

Answer 23

Increased ESR, CRP Hypercalcemic, hypercalciuria—in 10-30% of cases   Increased angiotensin converting enzymes (ACE), in case 60-80% of cases —>so ACE is more sensitive than high calcium levels   Anemia  Hypergammaglobulinemia  

Answer 24

``` Upper airway: Sinusitis Nasal septal ulceration Otitis media Mastoiditis Oral ulcer Saddle nose deformity Subglottic stenosis Epistaxis ``` ``` Lower airway: Diffuse alveolar haemorrhage Nodules, which may or may not be cavitating Mediastinal lymphadenopathy Pleural effusion Tracheal stenosis ```

Answer 25

Abnormal breathing while awake - Hyperventilation and hypocapnea alternating with hypoventilation/apnea during which they may have oxygen desaturation - Breathing is normal in between episodes - Hypoventilation/apnea can last 20-120 seconds - During hyperventilatoin: child is excited or agitated - During hypoventilation/apnea: child doesn’t appear distressed, they may even be calm and smiling - No associated bradycardia - There can be severe cyanosis and EEG seizures

Answer 26

Difference = differential cyanosis difference in oxygen saturation (O2 sat) of at least 5% or a difference in partial pressure of oxygen (Pao2) of at least 20 mm Hg between the arms and legs associated with congenital heart disease or persistent pulmonary hypertension of the newborn

Answer 27

PPHN - with shunting primarily or exclusively across PDA. (If shunting was primarily from right atrium to left atrium, then were would be cyanosis as opposed to differential cyanosis) Critically obstructive left sided cardiac disease, where there is enough antegrade flow to provide right subclavian flow, but distal flow is supplied across ductus arteriosus, critical aortic stenosis, critical coarctation, interrupted aortic arch

Answer 28

Inhaled nitric oxide selectively dilates pulmonary vasculature in ventilated areas of the lung. Mechanism of action: NO activates soluble guanylyl cyclase (sGC) to produce cyclic guanosine monophosphate (cGMP) leading to decrease vascular smooth muscle tone (ie, vasodilation)

Answer 29

- saccular stage - thinning of interstitium - increased airspaces (sacs) - vascular expansion - fibroproliferative changes

Answer 30

- canalicular stage - undifferentiated epithelial cells - paucity of capillaries - vascular expansion - reduced SA

Answer 31

Alveolar simplification | Subpleural cysts

Answer 32

Oxygen concentrator: - Filter room air and removes nitrogen so that purified O2 can be delivered - Concentration of up to 95% - Large , needs electrical source (immobile) or small battery powered( mobile). - Need back up cylinder. - Noisy - Higher up-front cost, but very little long-term maintenance cost - Safer, create oxygen as needed, limiting the concern for oxygen leaks Oxygen Cylinder: - Metal tanks containing pressurized oxygen or liquid oxygen released at specific flow rates to provide oxygen to the patient ( - Concentration of up to 100% - Lighter, more portable - Quiet - Lower initial cost. However, greater cost over time as they need to be refilled or replaced frequently - Small possibility of a leak causing safety concerns due to an increased chance of fire ignition

Answer 33

TB meningitis | TB pericarditis

Answer 34

FB, lung malformation, TB, obstructive (external compression), aspiration, tumour, infection (pneumonia)

Answer 35

The gene is typically responsible for expression regulation of genes involved in the development of the autonomic nervous system

Answer 36

``` Hirschsprung Abnormal pupillary response Cardiac asystole, sinus pauses Development of neural crest tumours ANS dysregulation ```

Answer 37

Class 1: Protein synthesis defect (BLUEPRINT for making a door, for example) - premature termination codons (PTC) Class 2: Protein maturation defect (MANUFACTURER) misfolding, premature degradation and impaired protein biogenesis Classic example: delF508 Class 3: Gating defect (DOOR NO OPEN) impair the regulation of the CFTR channel, resulting in abnormal gating characterized by a reduced open probability Example: G551D Class 4: Conductance defect (DOOR TOO SMALL) conductance defect-alter the channel conductance by impeding the ion conduction pore, leading to a reduced unitary conductance eg R117H with 5T Class 5:Reduced quantity (TOO LITTLE PRODUCTION) Alter its abundance by introducing promoter or splicing abnormalities Class 6: Reduced stability (POOR QUALITY MATERIAL) destabilize the channel in post-ER compartments and/or at the plasma membrane by reducing its conformational stability

Answer 38

Non-caseating granulomas

Answer 39

Granulomatous lesions can occur in any organ but most commonly in lung, lymph nodes, eyes, skin, liver, heart Non-specific symptoms are common (fatigue, weight loss, fever) Lofgren syndrome is less common in children (acute arthritis, BHL, erythema nodosum) Can have cardiac, ocular, neurological, renal involvement and hypercalcemia/hypercalciuria Common skin lesions include papules, plaques, nodules, changes in old scars, erythema nodosum, hyperpigmented lesions, and hypopigmented lesions. Central nervous system involvement may present with headache, seizures, cranial nerve palsies, motor signs, hypothalamic dysfunction, and hydrocephalus. Early onset sarcoidosis (<4yo) → skin rash, uveitis, arthritis, absence of lung disease

Answer 40

often normal, advanced = restriction with low DLCO

Answer 41

Specific changes of waxy yellow mucosal nodules and nonspecific changes → erythema, edema, granularity and cobblestoning of the airway mucosa and bronchial stenosis (typically in the lobar and segmental bronchi).

Answer 42

lymphocytosis in >85%, normal or low neutrophils (except in late disease) and CD4:CD8 is increased

Answer 43

Diagnosis is established when typical clinical features are supported by a tissue biopsy showing noncaseating granulomas

Answer 44

Generally adults do quite well - 2/3 spontaneous remission in 2 years consider steroids for severe cases

Answer 45

Pulmonary: worsening symptoms, decreasing lung function, progressive imaging findings Any of: cardiac, ocular, renal, CNS, hypercalciuria Severe clinical symptoms

Answer 46

A horizontal groove along the lower border of the thorax corresponding to the costal insertion of the diaphragm. It reflects increased work of breath against airflow resistance eg Asthma

Answer 47

Asthma Bronchiolitis Obliterans Rickets - soft ribs drawn in by diaphragm under tension Tends to be more common in infancy when the chest wall is softer

Answer 48

Hemoglobin converted from ferrous(Fe2+) to ferric state (Fe 3+) by oxidation by MetHb. The presence of ferric heme molecules causes a structural change in the hemoglobin molecule, resulting in reduced oxygen-carrying capacity and impaired unloading of oxygen at the tissue. This left shift in the oxygen saturation curve results in functional anemia. The low saturation is due to the waveband of the saturation monitor. Pulse oximetry can only measure two frequencies, oxyhemoglobin and reduced Hb. As MEtHb rises the saturation falls and plateaus at 85%. Despite normal Arterial PO2. So called saturation gap

Answer 49

Arterial Blood is chocolate brown colour Measure blood level of MetHb (blood gas) Evelyn Malloy method Co-oximeter-->gives a print out of various hemoglobin

Answer 50

Micrognathia Big tongue posterior, glossoptosis Airway obstruction +/-Cleft palate

Answer 51

``` Airway obstruction: ONO, Cap Gas, PSG Feeding difficulties: OT assessment, formal feeding study Airway Obstn Non-Surgical: - Lateral or prone positioning. - Nasopharyngeal airway - CPAP/BIPAP Surgical: Tongue-Lip adhesion to increase oropharyngeal gap Floor of mouth release Mandibular distraction Tracheostomy* Feeding: NG or G tube ```

Answer 52

Asthma is seen in TEF Patients with a history of EA/TEF have an abnormally high prevalence of bronchial hyperreactivity, suggesting that airway reactivity in these individuals may be due to events in early childhood such as chronic aspiration, rather than atopy Airway malacia Allergy/Atopy more common Aspiration/Reflux Protracted bronchitis - (more prominent in younger children with TEF) Recurrent TEF

Answer 53

Th-1 =IL-12 TH1 --- IFNg, IL-2, TNF-a = Cell mediated immunity Th-2 = IL-4 TH2---IL-4, IL-5, IL-13 = Allergy, IgE, eosinophilia, AHR

Answer 54

``` More than or equal to 93% for 95% of the recording time if continuous oxymetry (BTS 2009 ) If BPD + PHT- more than 94% In CF more than or equal to 90% Sickle more than or equal to 94 % In ATS 2018- BPD MORE THAN 93 % AHA- BPD with PH - 92-95% ```

Answer 55

QS/QT=CcO2-CaO2/CcO2-CvO2 x 100 %

Answer 56

Up to 5% is accepted

Answer 57

Surfactant protein B deficiency, ABCA3 deficiency , NKX2 deficiency , Lysinuric protein intolerance , Meth Transfer RNA synthetase deficiency (MARS) etc

Answer 58

This term is usually used when they present early in newborn or infancy . Most of the findings and pathology is due to interstitial changes though alveolar proteinosis is also seen

Answer 59

autoimmune PAP (GMCSF receptor antibody)

Answer 60

``` Primary= Autoimmune PAP (GMSCF receptor antibody) Hereditary PAP (receptor deficiency) ```

Answer 61

Whole lung lavage for primary PAP GMCSF -inhaled or s/c Immunosuppression : steroids/Hydroxychloroquine Lung transplant in congenital PAP and acquired PAP

Answer 62

Eventration left diaphragm Volume loss left lung due to hypoplasia or collapse Unilateral Diaphragmatic palsy (many causes are idiopathic if no trauma/surgery ) - eg. due to left mediastinal mass causing phrenic nerve compression Subpulmonic pleural effusion (with diaphragmatic hernia, you shouldn’t be able to actually see the diaphragm) Abdominal mass - eg. splenic

Answer 63

VC is decreased since abdominal organs push on the lung affecting compliance of the lung

Answer 64

More common than bilateral Birth trauma, cardiac surgery ( PDA ligation, coarctation repair ), neck surgery , idiopathic (all causes of unilateral can theoretically cause bilateral)

Answer 65

Birth trauma, Chiari malformation, idiopathic , hereditary neuropathies

Answer 66

Recurrent laryngeal nerve supplied all muscles of larynx except cricothyroid which is supplied by superior laryngeal nerve Originates from Vagus nerve

Answer 67

Class I: protein synthesis defect frameshift, splicing, or nonsense mutations that introduce premature termination codons (PTC), resulting in severely reduced or absent CFTR expression. ``` Class II: maturation defect misfolding, premature degradation by the endoplasmic reticulum (ER) quality-control system, and impaired protein biogenesis, severely reducing the number of CFTR molecules that reach the cell surface. Prototype is Phe508del(although this mutation also can have additional class 3 and class 6 function if escapes ER degradation) ``` Class III: gating defect impair the regulation of the CFTR channel, resulting in abnormal gating characterized by a reduced open probability ie. G551D Class IV: conductance defect-alter the channel conductance by impeding the ion conduction pore, leading to a reduced unitary conductance eg R117H with 5T (also has some class 3) → with 5T makes it more significant for CF (without 5T is unknown significance) Class V: reduced quantity- do not change the conformation of the protein but alter its abundance by introducing promoter or splicing abnormalities Class V1: Reduced stability

Answer 68

Kalydeco -Gating potentiator ) approved for class 3 and one class 4 mutation(R1117) approved more than 6 months of age

Answer 69

(Corrector) partially partially reverts the ΔF508-CFTR functional expression defect by stabilizing the NBD1-MSD1/2 interface, by alone no action but modest effect in combination (Orkambi) with Ivacaftor in homozygous df508 - approved for more than 2 years

Answer 70

Tezacaftor + ivacaftor -for homozygous df 508 and one residual function - approved for more than 6 years , symdeko has less side effects than orkambi and less drug interactions. (Not that much more efficacious than orkambi)

Answer 71

Teza+Iva+elazacaftor(next generation corrector) -for homo and hetero df 508

Answer 72

Ivacaftor is a CFTR gating potentiator which is fda approved for use in class 3 and one class 4 mutation(R1117) ,more than 6 months of age Lumicaftor is a CFTR corrector which partially reverts the ΔF508-CFTR functional expression defect by stabilizing the NBD1-MSD1/2 interface, by alone no action in homozygous df 508 . When ivacaftor used in combination with lumicaftor (Orkambi) it has modest effects in homozygous df 508. Df508 is mainly class 2 but even with lumacaftor, only about 1/3rd of the structurally normal protein reaches the cell surface; addition of ivacaftor keeps the channel open for a longer time and also works on the class 3 action of df508 .

Answer 73

Symbicort TURBUHALER comes in three strengths - 100 BUDESONIDE / 6 FORMOTEROL and 200 BUDESONIDE /6 FORMOTEROL, Forte 400 Budesonide /12 Formoterol Advair MDI comes in two strengths - Fluticasone 125/ Salmeterol 25 Fluticasone 250 /Salmeterol 25 Equivalent dose of Budesonide DPI 200 = 125 Fluticasone MDI and discus Equivalent dose of Formoterol 12 = 50 Salmeterol (multiple head to head comparison studies have used these dose ) While converting from symbicort 200/6 1 inhalation = Advair MDI 125/25 1 puff

Answer 74

``` Wrong inhaler technique Presence of environmental triggers Smoking - active and passive ABPA complicating asthma Neutrophilic asthma Presence of un addressed comorbidities like obesity, OSA, allergic rhinitis, GERD, VCD ,anxiety ```

Answer 75

>500mcg per day

Answer 76

1) Recurrent (>/=2) episodes of asthma like symptoms or exacerbations even when triggered by virus 2) Objective documentation of airway obstruction ( or convincing report of symptoms ) 3) Objective documentation of reversibility (or convincing and repeated response report by parent ) of airway obstruction with treatment with SABA/steroid when documented/reported obstruction or convincing parental report of improvement in symptoms to 3 month of medium dose ICS) 4) No alternative diagnosis

Answer 77

``` Low = 100-125 mcg/day Med = 200-250 mcg/day ```

Answer 78

- Shares visceral pleura - More common with infections - Doesn't usually have associated anomalies - Blood supply = Aorta, Venous usually to left atrium but can also be to right side

Answer 79

- Own visceral pleura - Usually detected incidentally on imaging - More common to have associated anomalies - Blood supply = Aberrant vessel from Aorta, Venous drainage to right atrium

Answer 80

Expiratory breaking by diaphragm keeps the end expiratory volume above FRC- this is because of very compliant chest wall . FRC is determined when lung recoil is balanced by chest wall recoil. Hence FRC is more closer to residual volume. Airway closure is above FRC so need to have a higher end expiratory volume, above FRC

Answer 81

Because the operating volume of the lung is small (low FRC ) , airways close early In normal health, closing capacity is less than FRC (closing capacity = closing volume +RV) In obesity , because FRC is reduced and the residual volume (RV) is not, ERV declines. ERV reduction is greatest in the supine position when the diaphragm ascends in the chest, and the weight of the lower thorax and the abdomen is applied to the lungs. At this point, the ERV may approach or be exceeded by the closing volume and gas may be trapped in the chest.

Answer 82

Compliance - lung chest wall and resp system compliance is reduced TLC - very little effect FRC- low ERV- decreased RV - very little effect RV/TLC - normal to slightly increased DLCO increased due to increased pulmonary capillary blood volume Spirometry - Usually normal in adults ; airway dysynapsis in children ( FVC disproportionately increased to FEV1 , decreased FEV1/FVC ratio in children )

Answer 83

Tracheomalacia with brassy TEF cough TEF recurrence Recurrent aspiration leading to bronchitis and bronchopneumonia Gastroesophageal reflux ( -->Barrett’s esophagus) Esophageal stricture Dysphagia Obstructive and restrictive ventilatory defects Airway hyperreactivity

Answer 84

Pneumothorax TB Acute/Chronic Eosinophilic pneumonia Hydatid disease

Answer 85

Qf = Kf[(Pc − Pis) − σ(πpl − πis)] where Qf = net flow of fluid Kf = capillary filtration coefficient; this describes the permeability characteristics of the membrane to fluids and the surface area of the alveolar-capillary barrier Pc = capillary hydrostatic pressure Pis = hydrostatic pressure of the interstitial fluid σ = reflection coefficient; this describes the ability of the membrane to prevent extravasation of solute particles such as plasma proteins πpl = colloid osmotic (oncotic) pressure of the plasma πis = colloid osmotic pressure of the interstitial fluid Note that the surface area of the alveolar-capillary barrier is included in the Kf. The Starling equation is very useful in understanding the potential causes of pulmonary edema, even though only the plasma colloid osmotic pressure (πpl) can be measured clinically.

Answer 86

PPIs Bronchodilators (e.g, albuterol, formoterol, salmeterol) Anti-inflammatory (e.g., Azithromycin) Fluoroquinolone (e.g, Ciprofloxacin) Anti-fungal (e.g., voriconazole) Anti-mycobacterial medications (e.g,. Bedaquiline, Clofazamine) Motility agents (Domperidone, Cisapride)

Answer 87

Macrolide Antibiotics (clarithromycin, erythromycin)--generally, need to dose adjust Antifungals (voriconazole, ketoconazole, itraconazole, posoconazole)--generally, need to dose adjust PPIs, H2 blockers, antacids (ranitidine, omeprazole, esomeprazole, lansoprazole) Hormonal contraceptives Immunosuppressants (cyclosporine, tacrolimus) Steroids (Prednisone/Methylprednisolone)--practically, don’t need to dose adjust ``` Anti-inflammatories (ibuprofen) Montelukast Benzodiazopines (midazolam) Anti-depressants (Citalopram, Escitalopram, Sertraline) Oral hypoglycemics Warfarin ``` Ivacaftor is metabolized by cytochrome CYP3A. (don’t use a strong inducer with Ivacaftor. If it’s a strong inhibitor, you can still use the drug, but you need to do a dose adjustment). Since ivacaftor is part of all the CF drugs, this is very relevant. strong CYP3A inducers(not recommended) and inhibitors(need dose adjustment except rifabutin which is not recommended)

Answer 88

Hypoventilation: + obstructed Sleep disordered breathing Recurrent infections: poor cough clearance, Aspiration: swallowing dysfunction Scoliosis Cor pulmonale: Tracheostomy, small number of patient

Answer 89

Advantage of PTCCO2 monitoring compared to PETCO2 is that the accuracy of transcutaneous measurements is not degraded by mouth breathing, supplemental oxygen, or mask ventilation The signal has a longer response time than the PETCO2 to acute changes in ventilation. PTCCO2 will not provide data for breath-by-breath changes, e.g., changes in the first few breaths after an apnea. Poor seal on skin pick up V/Q mismatch of lung disease

Answer 90

``` Nasal prongs , side stream measurement Advantage Reflects alveolar gas concentration, ventilation during sleep Breath by Breath changes Real time ``` D: Mouth breathing and occlusion of the nasal cannula can impair the ability of end-tidal PCO2 monitoring to detect apnea Low tidal volume and fast respiratory rates will impact ability to measure accuracy Leak in circuit, or mouth Poor signal may be obtained with poor positioning of the cannula, rapid respiratory rates without stable exhaled CO2 levels, mouth breathing, simultaneous delivery of oxygen, and blockage of the cannula with secretions or humidity

Answer 91

``` Location: periphery, centre or hilum Pulmonary cyst Regular shaped cyst Thin walled cyst, Water lily sign Meniscus, crescent sign Bronchial displacement Pericentric emphysema Air fluid level ```

Answer 92

No - Risk of rupture and seeding, anaphylaxis Percutaneous drainage Medical treatment with albendazole (alone has limited success)

Answer 93

Lack of flow distal to pulmonary artery Filling defect in Pulmonary artery Non-Specific = Parenchymal infiltrates, Atelectasis

Answer 94

``` Atrovent 3 doses IV Magnesium IV Aminophyline IV Methly prednisone IV salbutamol BIPAP Heliox ``` Support O2 Steroids

Answer 95

“classical sign” due to bronchial compression

Answer 96

HPV 6 and 11

Answer 97

Airway obstruction Distal involvement including the tracheal and tracheobronchial tree Requirement for tracheostomy Pulmonary cavitation Malignant transformation: esp with HPV 11

Answer 98

PAO2 = PiO2 - (PaCO2/R)

Answer 99

``` Recurrent respiratory infections Upper airway obstruction Obstructive sleep apnea Alveolar hypoventilation Atelectasis ```

Answer 100

An enlarged tongue and bone Craniofacial abnormalities, namely micrognathia, result in predominant nasal breathing and oral snoring. Upper airway obstruction and OSAS, combined with thoracic cage deformity and tracheal distortion due to shortened spinal height = frequent total airway collapse.

Answer 101

``` Pulmonary artery sling (most common) Double aortic arch (and many other CV) Tracheal bronchus Pulmonary hypoplasia Down syndrome Pfeiffer syndrome VACTERL ``` Very rare - there is a segment of the trachea, often distal, where the tracheal rings are truly complete. Most common cause of congenital tracheal stenosis. Complete tracheal rings result from a defect in embryogenesis after the eighth week of gestation causing a complete cartilaginous ring with absence of the usual posterior membranous portion of the trachea.

Answer 102

``` Lymphoid interstitial pneumonitis Pulmonary tumours Bronchiolitis obliterans Bronchiectasis Immune reconstitution inflammatory syndrome (IRIS) Airway hyperreactivity/asthma Aspiration pneumonitis Pulmonary HTN (limited data in children) Upper airway disease ``` Respiratory disease is the most common complication occurring in human immunodeficiency virus (HIV)-infected children. Acute disease = most likely to be infectious

Answer 103

1) Alveolar type 2 epithelial cell hyperplasia 2) Interstitial thickening (same as mesenchymal thickening) 3) Finely granular lipoproteinaceous material and macrophages in alveoli distal air spaces 4) Abnormal lamellar body with large whirls and vacuolar inclusions

Answer 104

Inflammatory, such as granulomatous with polyangitis, rheumatoid arthritis, sarcoid Fat emboli Malignancy, such as squamous cell carincoma Langerhans cell histiocytosis

Answer 105

CAVITY is the acronym to remember C = cancer, such as squamous cell carcinoma A = autoimmune, such as GPA, rheumatoid arthritis (rheumatoid nodules) V = vascular, such as septic pulmonary emboli or I = infection, such as bacterial or fungal, such as pulmonary abscess, pulmonary TB, NTAM, aspergillus T = trauma, such as pneumatoceles

Answer 106

?impaired lung function - increased FVC Hyperinflation Chronic cough, SOB, wheeze Peripheral bullae and case reports of pneumomediastinum Increased risk of pulmonary aspergillosis

Answer 107

``` Acute irritation of the airway Cough Sputum production Wheeze, chest tightness Can also produce bronchodilation ```

Answer 108

E-cigarette or vaping produce use associated lung injury (EVALI)—which has a very broad definition and varies pathologies including diffuse alveolar damage, acute eosinophilic pneumonia, hypersensitivity pneumonitis. Suspected compounds: THC containing products, vitamin E. Other mechanisms of injury: pyrolysis, thermal injury, release of metals Chronic: - Chronic bronchitis - Chronic decline in FEV1 - Chronic airflow obstruction and possible bronchiolitis obliterans –at least 1 case report, which was in a Canadian youth

Answer 109

1) Use of an e-cigarette in 90 days before symptoms onset AND 2) Pulmonary infiltrate on CXR or CT AND 3) Absence of pulmonary infection including (at minimum) negative viral and influenza 4) No evidence of alternate diagnosis

Answer 110

``` Bronchiectasis Bronchiolitis obliterans Aspiration Interstitial lung disease: Lymphoid interstitial pneumonia Malignancy—eg. Kaposi sarcoma Asthma Pulmonary hypertension ```

Answer 111

Tracheal stenosis Bronchiectasis Pulmonary nodules—including necrobiotic nodules Colobronchial fistula Hypersensitivity pneumonitis from medications such as mesalazine Interstitial lung disease

Answer 112

Neutrophilia | IL17

Answer 113

Eosinophils IL5 IL4 IL13

Answer 114

History of severe allergic reaction to any vaccine component or after previous dose of any influenza vaccine Concomitant aspirin or salicylate-containing therapy in children and adolescents; Children aged 2 through 4 years who have received a diagnosis of asthma or whose parents or caregivers report that a health care provider has told them during the preceding 12 months that their child had wheezing or asthma or whose medical record indicates a wheezing episode has occurred during the preceding 12 months; Children and adults who are immunocompromised due to any cause (including immunosuppression caused by medications or by HIV infection); Close contacts and caregivers of severely immunosuppressed persons who require a protected environment; Pregnancy; Receipt of influenza antiviral medication within previous 48 hours.

Answer 115

1) Aerodynamic filtering done by the nose, including nasal turbinates, adenoids and tonsils – based on particulate size 2) Respiratory mucous, airway surface liquid layer and mucociliary clearance 3) Airway reflexes: eg. Sneezing, coughing and bronchoconstriction 4) Macrophages

Answer 116

Ig A. Secretory IgA is the most predominant Ig isotype present in airway secretions and important for the mucosal response. Functions include: neutralising viruses & exotoxin, enhancing lactoferrin & lactoperoxidase activities and inhibits microbial growth.

Answer 117

1) Alveolar type II epithelial cell hyperplasia 2) Interstitial fibrosis 3) Poorly organised lamellar bodies (loosely packed lamellae and vacuolar inclusions, or fused lamellar bodies and multivesicular bodies) 4) Absence of tubular myelin 5) Lobular remodeling 6) Alveolar proteinosis

Answer 118

CVS: BP changes - Hypotension/Hypertension, arrhythmias, circulatory collapse, cardiac arrest, sudden death, flushing CNS: Headaches, seizures, altered behaviour (mood alteration, hyperactivity, psychosis, disorientation), sleep disturbances Metabolic: Hyperglycemia, Hypokalemia Immune: increased risk of infections Anaphylactic shock (ass’d with the succinate ester of methylprednisolone)

Answer 119

First seen to be positive in the first 2 – 3 days after acute bleeding.

Answer 120

Trimethoprim-sulfamethoxazole (TMP-SMZ) is the preferred agent for both prophylaxis and treatment in children as well as adults. 1. Pentamidine Nebulised form - usually well tolerated. Main side-effects are coughing & wheezing, which can be prevented by the use of inhaled beta-agonists. Avoid in patients < 5 yrs old or if there is a history of asthma. 2. Dapsone CI in patients with G6PD deficiency or those who experienced severe side-effects with TMP-SMX. Adverse reactions: agranulocytosis, aplastic anaemia, rash, nausea and sulfone syndrome (rash, fever, hepatitis, lymphadenopathy and methemoglobinaemia) 3. Atovaquone Most common side-effects include rash, nausea, diarrhoea, elevated transaminases and headache, which are usually mild. used in malaria as well!

Answer 121

1. Bochdalek - BACK (posterolateral) – 80% occur through the left pleuroperitoneal canal. Defect is in the diaphragm itself and usually there’s no associated membranous sac. 2. MorgAgni (Anterior diaphragmatic hernia) - rare anterior defect of the diaphragm, also referred to as a retrosternal, or parasternal hernia. Accounting for ~ 23% of all CDH cases. Characterized by herniation through the foramina of Morgagni which are located immediately adjacent and posterior to the xiphoid process of the sternum

Answer 122

Canalicular Phase (17 – 26 wks): Respiratory bronchioles developing - By the end of this stage each ends in a terminal sac (also termed a saccule). - The glandular appearance is lost as the interstitium has less connective tissue and the lung develops a rich vascular supply that is closely associated with the respiratory bronchioles. - The canalicular stage comprises the differentiation of the epithelia that allows the morphological distinction between conducting and respiratory airways. This distinction permits the recognition of the acinus/ventilatory unit for the first time.

Answer 123

That portion of lung distal to a terminal bronchiole and supplied by a ﬁrst-order respiratory bronchiole or bronchioles.

Answer 124

"Every Pulmonologist Can See Alveoli" 1) Embryonic 4-7 wks 2) Pseudoglandular 5-17 wks 3) Canalicular 16-26 wks 4) Saccular 24 wks- term 5) Alveolarization 36 wks to 21 years

Answer 125

Lung buds; trachea, main stem, lobar, and segmental bronchi; trachea and esophagus separate

Answer 126

Subsegmental bronchi, terminal bronchioles, and acinar tubules; mucous glands, cartilage, and smooth muscle

Answer 127

Respiratory bronchioles, acinus formation and vascularization; type I and II AEC differentiation

Answer 128

Dilation and subdivision of alveolar saccules, increase of gas-exchange surface area, and surfactant synthesis

Answer 129

Further growth and alveolarization of lung; increase of gas-exchange area and maturation of alveolar capillary network; increased surfactant synthesis

Answer 130

``` Drugs Idiopathic Infection (bacteria, fungi, mycobacteria, parasites, virus) Inflammation (acute/chronic eosinophilic pneumonia, Churg-strauss, Rheumatoid effusion) Malignancy (lymphoma) PE Toxicity Trauma ```

Answer 131

``` Infection Inflammatory/connective tissue disease Malignancy Lymphatic malformation/Chylothorax Post op surgical complications PE Abdominal Pathology Endocrine ```

Answer 132

``` Cardiac disease Hepatic Cirrhosis Renal disease Hypoalbuminemia SVC obstruction ```

Answer 133

10% decline

Answer 134

The severity of EIB can be graded as mild, moderate, or severe if the percent fall in FEV1 from the pre-exercise level is >10% but < 25%, >25% but <50%, and >50%, respectively.

Answer 135

Qf = Kf[(Pc − Pis) − σ(πpl − πis)] where Qf = net flow of fluid Kf = capillary filtration coefficient; this describes the permeability characteristics of the membrane to fluids and the surface area of the alveolar-capillary barrier Pc = capillary hydrostatic pressure Pis = hydrostatic pressure of the interstitial fluid σ = reflection coefficient; this describes the ability of the membrane to prevent extravasation of solute particles such as plasma proteins πpl = colloid osmotic (oncotic) pressure of the plasma πis = colloid osmotic pressure of the interstitial fluid

Answer 136

``` Direct influences of HIV Immune dysregulation with increased inflammation Recurrent/severe infections ART Environmental exposures ``` ART may halt progression of lung damage but will not reverse established chronic disease No ART = LIP, chronic/recurrent infections with bronchiectasis, BO, decreased lung function Use of early ART prevents lung damage Chronic lung disease = persistent bronchovascular reticular markings x 6 mos or more or consolidation/nodules x 3 mos or more

Answer 137

Diffuse infiltration of lymphocytes and scattered nodules of mononuclear cells Unclear etiology → ?lymphoproliferaton with response to the HIV alone or co-infection with another virus EBV = common co-infection Insidious course and slowly progressive Median age = 2.5-3yrs Dx = lung biopsy Tx = non-specific → bronchodilators, oxygen, steroids Some may progress to lymphoproliferative disease with polyclonal, polymorphic B-cell content with extranodal systemic and prominent pulmonary involvement or to malignant lymphoma

Answer 138

Non-Hodgkin Lymphoma

Answer 139

Esophagitis related to Candida, HSV, CMV etc. can be associated with swallowing difficulties and increased risk of pulmonary aspiration Neurological impairment secondary to HIV encephalopathy can further increase this risk

Answer 140

May be focal or bilateral May be associated with LIP or LRTI Associated with severity of immunosuppression Tx = optimizing ART, physio (airway clearance), treat intercurrent infections

Answer 141

Described with myobacterial species: TB, M.bovis, MAC TB IRIS may occur weeks to mos after initiation of ART and can happen either from unrecognized mycobacterial infection or an immune response directed against antigen in those on therapy Characterized by paradoxical worsening in signs with increased lymphadenopathy and new clinical/radiologic respiratory signs Must be distinguished from other infections To minimize risk, those with probable or confirmed TB should start tx 2 weeks prior to ART if possible Don’t stop ART or TB meds May use steroids if severe

Answer 142

Recurrent infections Lymphoid proliferation with tonsillar and adenoidal hypertrophy and pharyngeal infiltration → upper airway obstruction Can get Candida epiglottitis

Answer 143

One of the surfactant proteins, which include A-D Promotes adsorption and organization of lipids in surfactant

Answer 144

Full term neonate with severe bilateral lung disease, similar to infant with RDS: Respiratory distress is rapid, within minutes to hours Hypoxemic respiratory failure and may even need HFOV or ECMO May initially respond to exogenous surfactant, but this is not a sustained response and they will respond less to subsequent doses of surfactant

Answer 145

``` 1 = protein synthesis defect 2 = maturation defect 3 = gating defect 4 = conductance defect 5 = reduced quantity 6 = reduced stability ```

Answer 146

Class 1-3 Minimal function mutations = produced either no protein or a defective protein that is unresponsive to CFTR modulators Includes: nonsense mutation, insertion and deletion mutations, canonical/canonical splicing mutations and certain severe protein misfiling mutations

Answer 147

Class 4-5 Symdeko is Tezacaftor + Ivacaftor = for homozygous delF 508 and one residual function - approved for more than 6 years

Answer 148

Ivacaftor is a CFTR gating potentiator which is fda approved for use in class 3 and one class 4 mutation(R1117) ,more than 6 months of age Lumicaftor is a CFTR corrector which partially reverts the ΔF508-CFTR functional expression defect by stabilizing the NBD1-MSD1/2 interface, by alone no action in homozygous df 508 . When ivacaftor used in combination with lumicaftor (Orkambi) it has modest effects in homozygous df 508. Df508 is mainly class 2 but even with lumacaftor, only about 1/3rd of the structurally normal protein reaches the cell surface; addition of ivacaftor keeps the channel open for a longer time and also works on the class 3 action of dF508 .

Answer 149

Ivacaftor is metabolized by cytochrome CYP3A. (don’t use a strong inducer with Ivacaftor. If it’s a strong inhibitor, you can still use the drug, but you need to do a dose adjustment). strong cyp3a inducers = Rifampin, Rifabutin, Phenytoin Phenobarb, herbal supplements (St Johns wort) is not recommended for use with Ivacaftor. strong cyp3a inhibitors -azoles,erythro clarithro telithromycin- needs dose adjustment seville orange, grapefruit-to avoid caution when co administering other cyp3a substrates -tacrolimus, cyclosporine ,digoxin

Answer 150

Diurnal variation = maximum PM pre-bronchodilator PEF - minimum AM pre-bronchodilator PEF/recent maximum x 100% Diurnal Variation = day’s highest PEF - day’s lowest PEF/ mean of day’s highest and lowest PEF >10 (>13 in children) is considered to be significant

Answer 151

PC20 <4mg/ml 4-16 = borderline, >16 = negative

Answer 152

``` Daytime sx <4 days/week Nighttime sx < 1 day /week Normal physica activity Ventolin use <4 times per week No school absences Mild, infrequent exacerbations FEV1 or PEF ≥ 90% personal best Diurnal variation <10-15% Sputum eosinophils <2-3% ```

Answer 153

Intrathoracic abnormality, e.g, Congenital diaphragmatic hernia, CPAM Pulmonary hypoplasia associated with oligohydramnios Genetic syndrome, e.g., Trisomy 21, Trisomy 13, Trisomy 18, Cerebrocostomandibular syndrome Chest wall development, e.g., Asphyxiating thoracic dystrophy/Jeune syndrome Giant omphalocele

Answer 154

``` Flail chest → pain control, no splinting Traumatic pneumothorax Hemothorax Tracheal or bronchial rupture Pulmonary compression injury (e.g., explosive blast causing pulmonary contusion, pathologically see edema, hemorrhage, atelectasis). Post-obstructive pulmonary edema Post-traumatic atelectasis Diaphragmatic rupture ```

Answer 155

Foreign born individuals and Aboriginal people in particular are disproportionately affected by TB Incarcerated Homeless Higher among males 1:0.8 Highest age-specific rate 75+ age group Higher incidence in Northern Territories (3 most populous provinces, with 75% of population, make up 69% TB cases - BC, ON, QC)

Answer 156

Drug-induced HP (ie. MTX, cyclosporine) Avian antigen exposure (ie. pigeon) - most common! Bagassosis ( mold exposure) Aspergillus fumigatus (organic compost) Ventilation pneumonitis (air conditioner) Farmer's lung (moldy hay)

Answer 157

Corticosteroids (eg. Prednisone) Calcineurin inhibitors (eg. Tacrolimus, Cyclosporine) Cell cycle inhibitors (eg. Mycophenolate {MMF}, Azathioprine)

Answer 158

Mean Pulmonary arterial pressure at rest ≥ 20 mmHg Pulmonary artery wedge pressure (<15mmHg) Elevated Pulmonary Vascular Resistance > 3 woods units x m2

Answer 159

Cardiac cath Necessary: (1) to confirm the diagnosis and assess the severity of PH (2) to exclude other potentially treatable conditions, such as pulmonary thromboembolic disease (3) to rule out left heart disease (4) to assess response to vasodilators before starting therapy (and to reassess on therapy to determine need for escalation or change in treatment) (5) to determine operability in patients with APAH-CHD (6) to determine suitability for lung transplantation.

Answer 160

Nitric Oxide: Activates guanylate cyclase → production of cyclic GMP and subsequent smooth muscle relaxation Selectively relaxes pulmonary vasculature without affecting arterial pressure Also has antiproliferative effects on smooth muscle and inhibits platelet adhesion Phosphodiesterase inhibitors (eg. Sildenafil): Prevent degradation of cyclic GMP → potentiates the effect of NO activity by inhibiting PDE 5 Endothelin Receptor Antagonists (eg. Bosentan-dual receptor): Block ET receptors (found in smooth muscle and endothelial cells) thus promoting vasodilation and prevents proliferation Prostacyclin Analogs/receptor agonists (eg. Epoprostenol, Iloprost, Trepostinil): Acts as a pulmonary vasodilator, inhibits vascular smooth muscle proliferation, inhibits platelet aggregation, improves endothelial dysfunction and can also act as a possible cardiac inotrope Ca channel blockers (eg. Nifedipine): Pulmonary vasodilator - by inhibiting Ca influx through slow channel in cardiac and vascular smooth muscle cells

Answer 161

PEP may have an effect on the peripheral airways and collateral channels of ventilation (Opens collaterals) Increase in lung volume may allow air to get behind the secretions and assist in mobilizing. (Air behind secretions is pushed) The increase in pressure is transmitted to airways creating back pressure stenting them during exhalation , therefore preventing premature airway closure thus reducing gas trapping . Prolonged Exhalation with huff breathing helps mobilize secretions

Answer 162

1) Ultrastructural | 2) Functional (30%)

Answer 163

Diaphragm paralysis generally results from injury to the phrenic nerve during thoracic or neck surgery. Tumors of the mediastinum, peripheral neuropathy, and agenesis of the phrenic nerve are less likely causes. In the newborn, stretching of the root C3–C5 during breech delivery is a frequent cause, most often in association with brachial plexus injuries (Erb’s paralysis). Most diaphragm paralyses are on the right side, with bilateral paralysis occurring in only 10% of cases. Bilateral diaphragm paralysis can be responsible for a severe restrictive syndrome, with total lung capacity often below 50% of predicted value

Answer 164

In unilateral diaphragm paralysis (UDP), it is believed that the non-paralyzed hemidiaphragm increases in strength to compensate for the dysfunction of the paralyzed hemidiaphragm; therefore, most of these patients are able to maintain appropriate ventilatory conditions at rest and during mild exercise. However, this is not true for all patients, because many of them have unexplained dyspnea, exercise limitations, and a reduction in inspiratory muscle capacity Elevation of the paralyzed diaphragm is greater in the supine position, due to pressure from the abdominal content, and can be responsible for orthopnea. On chest x-ray, unilateral paralysis of the right hemidiaphragm should be suspected if it is more than two rib spaces higher than the left hemidiaphragm; on the left side, it results in an elevation of the hemidiaphragm of at least one rib space above the right hemidiaphragm. Contralateral mediastinal shift also can be observed in severe cases.

Answer 165

Fluoroscopy with a sniff test and ultrasonography in a spontaneously breathing patient show a paradoxical inspiratory upward motion of the paralyzed hemidiaphragm compared to the contralateral side. Electromyography in association with percutaneous stimulation of the phrenic nerve can confirm the paralysis. Repeated evaluations by fluoroscopy, ultrasonography, or electromyography can aid in assessing diaphragmatic function.

Answer 166

Leak, inappropriate trigger sensitivity, inappropriate settings, anxiety Patient-ventilator asynchrony (PVA) describes the poor interaction between the patient and the ventilator and is the consequence of the respiratory muscle activity of the patient being opposed to the action of the ventilator. A mismatch between the breaths demanded by the patient and those delivered by the ventilator.

Answer 167

Cheap Easy to use/maintain Directly measures all aspects of inflammation Rapid results Evidence of benefit of clinical outcome (Can be used to titrate steroid dosage, less side effects, better control Identifies adherence)

Answer 168

``` Non invasive way of respiratory support Safe Easy to apply Quick to start Minimal morbidity, Reduce PICU admission On ward support Sedation not needed May support oral enteral feeding ```

Answer 169

Eliminate most of the anatomic dead space Create a reservoir with high FiO2 in the nasal cavity Improve gas exchange Significantly reduce the work of breathing

Answer 170

Using humidified (warmed) gas keeps mucus more fluid and aids airway recovery (eg, after surgery) Reduced respiratory rate Less dyspnoea (laboured breathing or shortness of breath / breathlessness) and mouth dryness, and greater overall comfort Ease of set-up – easier to fit a nasal cannula than an oxygen mask Low level of patient compliance needed (sedation possible but not required) Patients say it is very comfortable and allows them to communicate

Answer 171

Strep Pneumo, Staph aureus, Pseudomonas aeruginosa Strep Pyogenes Defn: Necrosis and liquefaction of consolidated lung Usually single lobe can lead to: BP fistula, Abscess, Air fluid level, Pneumatocele Necrotizing pneumonia usually follows pneumonia caused by particularly virulent bacteria

Answer 172

Clinical manifestations of necrotizing pneumonia are similar to those of uncomplicated pneumonia, but they are more severe. Necrotizing pneumonia should be considered in a child with prolonged fever or septic appearance. The diagnosis can be confirmed by chest radiograph (which demonstrates a radiolucent lesion) or contrast-enhanced computed tomography (CT); the findings on chest radiograph may lag behind those of CT.

Answer 173

With hyperinflation, compliance decreases and work of breathing increases (this effect is more predominant and bad for these hyperinflated asthma patients).

Answer 174

Hyperinflation can decrease airway resistance by the tethering effect of parenchyma on airway Barrel chest is due to air trapping and hyperinflation in an asthma exacerbation.

Answer 175

Rapid rise to peak Smooth curve with no glottic closure within measured time for FEV Don’t need back extrapolation volume in preschoolers At least 2 acceptable curves with FEVt and FVC are within 0.1L or 10% of highest value No maximum number of maneuvers, while being attuned to their fatigue level (min 3)

Answer 176

1) Smaller absolute lung volumes and large airway size relative to lung volume 2) Forced expiration is therefore completed in a shorter time 3) VBE in children is typically lower than in adults, whereas VBE/FVC is higher. Both findings can be simply explained by the much smaller absolute lung volumes of very young subjects

Answer 177

``` Bronchiectasis (most common) Tracheal stenosis Ileobronchial colobronchial fistula COP (Cryptogenic organizing pneumonia) granulomatous and necrobiotic nodules ILD Pulmonary Vasculitis Drug induced disease (Sulfasalazine and Mesalamine can cause HP) Opportunistic infection Malignancy Pulmonary thromboembolism ```

Answer 178

1) Decrease frequency 2) Increase amplitude 3) Increase I:E ratio HFOV→ you avoid the cyclical application of high distending airway pressures (barotrauma) and the associated cyclical delivery of large tidal volumes (volutrauma) so you keep the alveoli open and recruited more consistently with minimal lung damage.

Answer 179

``` Increase PIP (increase delta p) → primary way to regulate PaCO2 Increase frequency ``` HFJV is a form of time-cycled, pressure-limited ventilation such that if ventilator parameters are held constant, a decrease in chest wall or lung compliance will result in a reduction in minute ventilation. The driving pressure, rather than the respiratory frequency, is most influential for CO2 elimination.

Answer 180

In patients with airway obstruction, SVC > FVC. Using the larger volume would enable the FEV1/VC ratio to most sensitive to detection of airflow obstruction In normal subjects, SVC = IVC = FVC are about the same

Answer 181

Pulmonary arteries originate from 6th branchial arch arteries at 7 weeks gestation (end of embryonic, early pseudoglandular stage) Pulmonary trunk arises from truncus arteriosus, which divides at 8 weeks

Answer 182

Medullary respiratory centre: o Pre-botzinger complex: essential for generation of respiratory rhythm o Dorsal respiratory group – associated with inspiration o Ventral respiratory group – associated with expiration. This group is usually quiet during normal breathing, but is active during active exhalation, such as exercise. Pons: o Apneustic centre: has an excitatory effect on inspiratory area of medulla. Unclear if this area plays a role in normal human respiration. Activity of this area is seen in brain injury o Pneumotaxic centre: switches off/inhibits inspiration and thereby, affects inspiratory volume and respratory rate. It’s not needing for basic rhythm, but it “fine tunes” the respiratory rhythm

Answer 183

``` pH = pKA + log (HCO3-/CO2) pH = 6.1 + log([HCO3-]/0.03 x pCO2) ```

Answer 184

Low volume lung , bilateral diffuse air space opacification

Answer 185

Triglycerides >1.1 mmol/dl Total count >1000 cells;>80% lymphocytes Chylomicrons + --not usually tested for though Sudan 3 + staining for fat globules Exudate- Pleural fluid LDH > 2/3rd of upper limit of normal or > 0.6 of serum LDH, Pleural fluid Protein > 0.5 of serum protein (2-6 g/dl) (Lyte’s) Electrolytes and glucose same as plasma

Answer 186

Upper respiratory tract - chronic sinusitis , nasal septal perforation/ulcer , oral ulcers, subglottic stenosis Lower respiratory tract- tracheal or bronchial stenosis, diffuse alveolar hemorrhage , granulomas (nodules) Antibodies - PR3 and MPO ANCA, mostly PR3 which is more specific.

Answer 187

1) It is effort dependent 2) It has high variability 3) PEF is less sensitive than standard spirometry in detecting reversibility of airflow obstruction after bronchodilator administration

Answer 188

Loud P2 Pan systolic murmur in right sternal border ( TR murmur) and ejection murmur in pulmonic area HEPATOMEGALY/raised JVP left parasternal heave

Answer 189

CFSPID 1 - CFSPID if NBS shows high IRT and one mutation (inconclusive screen) with intermediate sweat x 2 2 - CFSPID if screen positive with two mutations (only one CF causing) and negative sweat Follow in CF clinic with repeat CF testing at 2 months and 6 months , a small % may turn out to be CF (becomes sweat pos with symptoms of CF ) 2 genetic tests may be: Sanger sequencing or next generation sequencing of the whole CFTR gene. Sanger sequencing: looking at a small sequence of genome, cheaper. Next generation: faster, looks at a wider section of genome, amplifies multiple areas at the same time. Next generation is mostly used currently

Answer 190

Both types of nonfatal drowning result in decreased lung compliance, ventilation-perfusion mismatching, and intrapulmonary shunting, leading to hypoxemia that causes diffuse organ dysfunction. Key mechanism: surfactant dysfunction and inflammation

Answer 191

Bronchial casts are characterized by the formation of obstructive airway plugs that may be large enough to fill the branching pattern of an entire lung. Type I – Inflammatory casts – contain fibrin and eosinophilic infiltrate , e.g.,CF, asthma Type II – noninflammatory casts – acellular containing mucin and fibrin without inflammatory infiltrated e.g., plastic bronchitis in post-Fontan patient

Answer 192

1) Central sleep apnea – can provide rate so ventilation will occur despite central apnea 2) Neuromuscular disorder – inspiratory pressure required to provide ventilation

Answer 193

P50 is the PO2 at which 50% of the hemoglobin present in the blood is in the deoxyhemoglobin state and 50% is in the oxyhemoglobin state. (At a temperature of 37C, a pH of 7.4 and PCO2 of 40mmHg, normal human blood has a P50 or 26 or 27mmHg. If the oxyhemoglobin dissociation curve is shifted to the right, the P50 increases. If it is shifted to the left, the P50 decreases).

Answer 194

``` Increased temperature Reduced pH Increased 2,3 DPG Increased PCO2 Memory tool: think of exercising muscles--hot, acidosis ```

Answer 195

``` Recurrent Aspiration Recurrent pneumonia Wheeze secondary to asthma Pulmonary hypertension chILD appears to be more common in children with Down Syndrome ```

Answer 196

Pulmonary Alveolar Proteinosis ``` Fungal infection (PAS positive) ILD (Pulmonary fibrosis, sarcoidosis can be PAS positive)Surfactant protein deficiency (proteinaceous secretions) Surfactant protein deficiency (proteinaceous secretions) ```

Answer 197

``` reduced FVC reduced TLC reduced ERV, FRC normal FEV1 and FEV1/FVC (although increased risk of asthma) reduced MIP/MEP increased DLCO ```

Answer 198

< 5% Equation: CcO2-CaO2/CcO2-CvO2

Answer 199

No specific diagnostic features of disease, with an absence of extensive inflammation, reactive injury, architectural distortion, and fibrosis seen on lung biopsy Minor and nonspecific changes involving the distal airways including: - Mildly increased airway smooth muscle - Mildly increased numbers of alveolar macrophages - Increased number of ‘ clear cells’ within bronchioles - Patchy mild periairway lymphocytic inflammation and fibrosis commonly seen Stain = Bombesin stain One or more bronchioles with > 10% of airway epithelial cells immunopositive

Answer 200

Cilia Ultrastructure – Classic 9+2 Arrangement Commonest defect - Outer dynein arm defect Diseases due to sensory ciliopathy: PCKD, Bardet-Biedl Syndrome, Alstrom Syndrome, Joubert, Retinitis pigmentosa

Answer 201

Affected dependant lung will be hyperlucent. Normal lung will compress and partially collapse and appear denser (Normal). This debucitus technique is helpful when kids can’t do inspiratory/expiratory

Answer 202

Enlarged internal bronchial diameter Signet ring sign (bronchi : vessel ratio >1.0) Failure of airway to taper in lung periphery Bronchial wall thickening ( Tram track) Mucus plugging / impaction ( Tree in bud pattern) Mosaic perfusion Air trapping on expiration Air-fluid levels in distended bronchi

Answer 203

*Think of 3s! 3 episodes of apnea >3 seconds each separated by continued breathing for ≤ 20 seconds

Answer 204

IL-4, IL-5, IL-13

Answer 205

``` Prolonged steroids Pancreatic insufficiency (both exocrine and endocrine) Low weight bearing exercises Delayed puberty Chronic pulmonary infections Malnutrition ```

Answer 206

Palivizumab = a humanized murine monoclonal immunoglobulin G-1 directed against an epitope on the F glycoprotein of RSV It is produced by recombinant DNA technology and directed against an epitope of the F glycoprotein of RSV. Palivizumab binds to this glycoprotein and prevents viral invasion of the host cells in the airway. This reduces viral activity and cell-to-cell transmission, and blocks the fusion of infected cells

Answer 207

Hypertension Nephropathy Also: Hirsutism, gingival hyperplasia, neurologic toxicity, seizures, headache, and sleep disturbance, diabetes

Answer 208

Classic = "halo sign" (distinct nodular lesion with surrounding areas of decreased attenuation) "air crescent sign" (late finding of nodular cavitation, occurring after recovery of neutrophil counts) Galactomannan = double sandwich EIA to detect galactomannan (polysaccharide cell wall antigen of Aspergillus) Best validated for neutropenic patients with hematologic malignancy or HSCT Blood less sensitive than pulmonary Galactomannan from BAL - if shows up in blood - more likely to be a severe infection.

Answer 209

IgE >500 (minimum), >1000 = classic positive skin prick test with Aspergillus worsening lung function increased Aspergillus serum specific IgE and IgG Radiographic changes on CXR or CT not cleared with antibiotics or physio Additional: Increase in blood eosinophilia when not on steroids (>40 ug/L) Aspergillus species specific precipitating antibodies central bronchiectasis Aspergillus species specific containing mucus plugs 1st = steroids -> Prednisone 0.5-2mg/kg.day 1-2 weeks then taper within 2-3 mos 2nd = antifungal: oral Itraconazole 5mg/kg/day x 3-6 mos (therapeutic drug monitoring and LFTs) add -> BD, inhaled steroids etc only if indicated for asthma

Answer 210

Tracheomalacia (Overall, tracheomalacia improves with age) Recurrent bronchitis/ pneumonia due to poor secretion clearance Aspiration due to esophageal stricture GERD due to poor esophageal peristalsis Recurrence of fistula Chest wall deformities due to surgery predominantly (but Could also be related to vertebral anomalies with VACTERL association)

Answer 211

Live virus vaccination: measles, mumps, polio (recent) Immunosuppressive drugs: corticosteroids, tumour necrosis factor (TNF) inhibitors, and others Metabolic disease: chronic renal failure, severe malnutrition, stress (surgery, burns) Diseases of lymphoid organs: lymphoma, chronic lymphocytic leukemia, sarcoidosis Age: infants <6 months, the elderly ``` Infections: Active TB (especially if advanced) HIV infection (especially if CD4 count <200) Other viral infection (measles, mumps, varicella) ```

Answer 212

Non tuberculosis mycobacterium infection Recent BCG vaccine ( within 12 months ) Allergic reaction at the injection site

Answer 213

Intensive + continuation phase Intensive = 3-12 weeks of IV Amikacin PLUS 1 of: Tigecycline, Imipenum, Cefoxitin (consider adding macrolide) - "For ABS you need to do CIT-ups" Continuation = Inhaled Amikacin with 2-3 of: PO Minocycline, Moxifloxacin, Linezolid, Clofazimine

Answer 214

severe decline in CF lung function possibly developing into a life-threatening systemic infection known as cepacia syndrome. extremely difficult to treat because of virulence and resistance cepacia syndrome is characterized by a rapidly progressive fever, uncontrolled bronchopneumonia, weight loss, and in some cases, death.

Answer 215

``` Foreign body Congenital: CLO, CPAM Swyer James PTX Compensatory: Lung collapse – Pulmonary Hypoplasia Ventilator associated (deep ETT) ```

Answer 216

With the PEP device, there is normal inhalation, but there is resistance during exhalation, which results in the creation of back pressure (positive expiratory pressure) This results in build up of gas pressure behind mucous through collateral ventilation. Through forced expiratory maneuvers, the mucous can be moved from peripheral to central airways. Coughing maneuvers enables expectoration of mucus Side effects: - Hemoptysis - Nausea

Answer 217

Hypertonic saline acts directly as an osmotic agent and may increase airway surface liquid volume Side effects: Bronchoconstriction (give with ventolin) Cough Dislodgement of GT tube, rupture of the tube Less likely worsening Pulmonary functions

Answer 218

Inhaled recombinant DNAse treats CF lung disease by reducing sputum viscosity and increasing mucus clearance, attacks neutrophil DNA Side effects: - voice alteration - sore throat - laryngitis - rash - chest pain

Answer 219

``` Enoxaparin adv: less doses more effective longer half life 4 hrs No need for close monitoring ``` ``` dis: expensive bruising at injection site only partially reversed with vitamin k higher half life increase risk of bleeding ``` ``` Warfarin Adv: Oral reverse with vitamin k once daily dosing very low cost ``` ``` dis: Slow onset of action requires monitoring for INR narrow therapeutic window difficult control preoperative might interact with food and other drugs ```

Answer 220

Unilateral more common on the left (due to longer course of the recurrent laryngeal nerve, and generally due to a nonfunctioning peripheral nerve) → cardiac surgery Peripheral nerve pathology Mediastinal lesions (tumours, vascular malformations or thoracic surgery) Birth trauma often cause of transient palsies (for both unilateral and bilateral) Acquired VC paralysis → usually a unilateral condition coming from iatrogenic injury to the recurrent laryngeal nerve (L > R) Risk factors = PDA repair, Norwood cardiac repair + esophageal surgery (TEF), older children with thyroid surgery May have spontaneous recovery months after injury (only if nerve is intact)

Answer 221

``` Bilateral palsies → lesions of the CNS Arnold-Chiari malformation Hydrocephalus Meningoceles Myasthenia gravis Can also be idiopathic ``` Most common cause is congenital (as opposed to acquired which is seen with unilateral) Most with bilateral paralysis present with significant airway compromise (with good voice)

Answer 222

Recurrent laryngeal nerve → which comes from the vagus nerve (CN 10). (It supplies both adductor and abductor muscles of larynx

Answer 223

Local: thrush, dysphonia - Adrenal insufficiency: hypoglycemia, altered mental status, fatigue, weakness, anorexia, Cushingoid features, growth failure, or weight loss (Adrenal crisis--pediatric deaths related to fluticasone) - Height: decreased growth velocity in prepubertal children in first 1-2 years of treatment, but this is not progressive or cumulative. Final result of 0.7% decrease in adult height (GINA 2019, page 140)

Answer 224

As per Kendig’s chapter 16, page 260, with use of MDI: take a slow breath over 4-5 seconds, then 10 second breath hold. - Infants use the MDI with tidal breathing and they require 5-10 breaths. (chapter 16, page 266 upper left hand corner) - Ensure the inhaler isn’t empty, based on counted doses

Answer 225

Unless there is an external dose counter, there is no way of knowing if it’s empty without counting dose. Take note of date that inhaler was first used and count number of daily doses to get a sense of when inhaler needs to be replaced, or count individual doses.

Answer 226

``` Pulmonary edema (1-2 mos) VOD (1-2 mos) DAH (1-2 mos) Idiopathic pneumonia syndrome (1-6 mos) GVHD (acute and chronic) - 2mos on ILD (3 mos on) PTLD (3 mos on) BO (3 mos on) ```

Answer 227

Early (<30 days) - Pseudomonas, other G+ and G- species, Candida Late (>30 days) - Staph aureus, PJP, Aspergillus, CMV, Toxo, VZV, EBV, Adenovirus Late (>100 days) - Encapulated gram positive (H. flu, S pneumo)

Answer 228

Gram negative: - PJP - Legionella - Capnocytophagia Gram positive: - Listeria - Corynebacterium

Answer 229

Septra for both treatment and prophylaxis Treatment dose: Septra 15-20 mg/kg/day IV or oral in 3-4 divided doses x 21 days Prophylaxis: One DS tablet daily or 3x/week or as one single strength tablet daily Second line: - Combo of Clindamycin + Primaquine - Dapsone - Atovaquone If PJP + mild-moderate hypoxemia = Septra + steroids

Answer 230

1) Must have BEV <5% of FVC or <0.100 L (whichever is greater) 2) Must have no evidence of faulty zero flow setting 3) Must have no cough in first second of expiration 4) Must have no glottic closure in first second of expiration 5) Must have no glottic closure after first second of expiration 6) Must achieve one of 3 EOFE indicators: - Expiratory plateau - Expiratory flow >15 s - FVC is within repeatability tolerance or is greater than the largest prior FVC 7) No evidence of obstructed mouthpiece or spirometer 8) Must have no evidence of a leak 9) If max inspiration = more than FVC, then FIVC - FVC must be ≤ 0.100L or 5% of FVC, whichever is greater

Answer 231

≥6yo: difference between largest FVC or FEV1 ≤ 0.150L | ≤6yo difference between largest FVC or FEV1 ≤ 0.100L

Answer 232

1) The pediatric chest is typically more rounded with less developed musculature 2) More flexible and elastic rib cage (combo of 2 = very compliant chest) 3) The ribs and sternum of a child can thus support a significant amount of blunt force without fracture BUT the visceral structures may still have sustained serious injury. 4) Increased mediastinal mobility, together with the absence of preexisting vascular disease in children, make injuries to the mediastinum and great vessels less frequent 5) Conditions such as tension pneumothorax or hemothorax are very poorly tolerated and must be recognized and addressed emergently.

Answer 233

- Local tenderness - Ecchymosis - Sometimes a concavity or paradoxical respiratory movement. - The sternal segments are typically well aligned without much displacement. - Dyspnea, cyanosis, arrhythmias (most commonly sinus tachycardia), and hypotension may be evidence of an underlying cardiac contusion. - Radiographic demonstration of fractures is most commonly by chest x-ray or CT scan Children with traumatic injury of the sternum should be admitted to the intensive care unit given the increased risk for arrhythmias. Cardiac tamponade and blunt myocardial damage must be ruled out

Answer 234

The creation of a tension pneumothorax requires a valvular mechanism through which the amount of air entering the pleural space exceeds the amount escaping it. The positive intrapleural pressure is initially dissipated by a mediastinal shift, which compresses the opposite lung and can result in ipsilateral pulmonary collapse and angulation of the great vessels entering and leaving the heart.

Answer 235

``` Chest wall and lung trauma Rupture of the esophagus Pulmonary cyst Emphysematous lobe Postoperative bronchial fistula. ```

Answer 236

If a considerable segment of chest wall is open, more air is exchanged at this site than through the trachea, because the pressures are similar. Inspiration collapses the ipsilateral lung and drives its alveolar air into the opposite side. During expiration, the air returns across the carina. The mediastinum becomes a widely swinging pendulum compressing the uninjured lung on inspiration and the lung on the injured side during expiration. Under these circumstances, little effective ventilation is taking place because of the tremendous increase in the pulmonary dead space and the decrease in tidal volumes.

Answer 237

Hemothorax

Answer 238

Esophageal injury is more commonly associated with penetrating trauma The most common cause of esophageal injury is iatrogenic during instrumentation of the esophagus Can lead to abscess

Answer 239

Adequate pleural space drainage IV antibiotics Maintenance of adequate nutrition

Answer 240

Hypotension Flushing CNS depression Hypermagnesemia

Answer 241

Tachycardia Arrhythmia Myocardial injury Hypokalemia

Answer 242

Steroids- either oral (eg. Dexamethasone) or IV (methylpred) MgSO4 Continuous ventolin/IV Ventolin (Amiopylline) --ICU, with severe exacerbation failed to improve despite maximal therapy.

Answer 243

Nausea Vomiting Tachycardia

Answer 244

Hypotension (since many patients have relative hypovolemia combined with decrease in preload due to positive pressure ventilation and reduced vascular tone caused by anaesthetic agents) Hypoxia Bronchospasm, Cardiac Arrest Difficulty with ventilation

Answer 245

``` Minimize dynamic hyperinflation and air trapping. Adequate oxygenation Permissive hypercarbia Goal pH >7.2 Strategy: - Slow ventilator rate with prolonged expiratory phase - Minimal end expiratory pressure - Short inspiratory time ```

Answer 246

In terms of mechanism for hypoxemia: - V/Q mismatch causes intra-pulmonary shunt (atelectasis) and dead space (due to airway over distension) - Patients have hypoxemia and hypercarbia - Atelectasis due to small airway obstruction = decreased ventilation, but adequate perfusion - Dynamic hyperinflation will stretch pulmonary vasculature = increased pulmonary vascular resistance, increased RV afterload and compromising RV function - Large negative intrathoracic pressure after inspiration = increased LV afterload = systolic BP decreases during inspiration = pulsus paradoxus (decreased BP on inspiration)

Answer 247

Alveolar hemorrhage is confirmed when lavage aliquots are progressively more hemorrhagic, a finding characteristic of DAH from all causes Hemosiderin-laden macrophages -> Prussian blue staining When greater than 20 percent of 200 macrophages stain positive for hemosiderin, a diagnosis of DAH is usually made. It’s normal to have up to 3%. It’s normal to have up to 3% of macrophages stain positive for iron. it takes 50 hours after a bleed for the macrophages to become positive for iron staining. If there is no further bleeding, the iron will clear in 12-14 days In general: hemosiderin laden macrophages at day 3 post hemorrhage, peak at day 7-10 post hemorrhage

Answer 248

First choice: Application of ice cold saline → gentle instillation of 10-15 mL aliquots from a fully wedge bronchoscopy Second choice: epinephrine 1:100,000 dilution with small aliquots of 2 mL (maximum adult dose of 0.6 mg) Could think about otrivin drops down the scope CF kid - can use foley catheter and blow up the balloon to tamponade it while waiting for other management

Answer 249

``` Infection Lung Abscess Pneumonia Trauma Vascular Disorders Coagulopathy Congenital Lung Malformations Miscellaneous (catamenial, factitious, neoplasm) DAH ```

Answer 250

``` Idiopathic Pulmonary Capillaritis GPA Microscopic Polyangiitis Anti-GBM disease SLE HSP Behcets Cryoglobuminemic Vasculitis JIA COPA syndrome ```

Answer 251

``` Idiopathic pulmonary hemosiderosis Acute idiopathic pulmonary hemorrhage of infancy Heiner’s syndrome Asphyxiation/abuse Cardiovascular causes Pulmonary vein atresia/stenosis Total anomalous pulmonary venous return Pulmonary veno-occlusive disease Mitral stenosis Left-sided heart failure Pulmonary capillary hemangiomatosis Pulmonary telangiectasia ```

Answer 252

CT chest with contrast to look for PE, AVM Test for coagulation defects Bronchoscopy to look for foreign body, airway hemangioma, tumor, presence of hemosiderin laden macrophages Infection: blood and sputum cultures, TB testing with purified protein derivative If diffuse alveolar opacities—>echo to look for cardiac disease, CT with contrast, cardiac cath If systemic involvement (eg. renal disease, rash, joint disease)—>ANA, ANCA, CBC, ESR, CRP, urinalysis, metabolic panel, d-dimer, von willebrand factor, anti-phospholipid antibody, lupus anticoagulant If DAH and no cardiac, renal or systemic disease with negative antibodies (ANA, ANCA, anti-GBM)—>transthoracic biopsy either through open approach (mini-thoracotomy) or VATS

Answer 253

Drop in oxygen saturation by 2% of more when going from supine to upright. Another definition: decrease in the arterial oxygen tension (by more than 4 mmHg [0.5 kPa]) or arterial oxyhemoglobin desaturation (by more than 5 percent) when the patient moves from a supine to an upright position. This is caused by conditions where being in the upright position causes more blood flow through an intrapulmonary shunt such as AVM (AVM has predilection for lower lobes in 50-70% of cases), or increased blood flow through intrapulmonary vascular dilatations (which are preferentially in dependent position) in hepatopulmonary syndrome, or increased flow through an inter-atrial shunt such as PFO Pulmonary AVM: first line is echo (bubble) to screen,can also look for desat on CPET

Answer 254

Platypnea = dyspnea in the upright position, which is better in the supine position

Answer 255

Pulmonary varix (dilated vessel) may be apparent as a non-specific soft tissue mass, often with a relatively unusual orientation compared to adjacent vessels. More than one raises the possibility of hereditary hemorrhagic telangiectasia

Answer 256

The characteristic presentation of a PAVM on non-contrast CT is a homogeneous, well-circumscribed, non-calcified nodule up to several centimeters in diameter or the presence of a serpiginous mass connected with blood vessels CT is often the diagnostic imaging modality of choice.

Answer 257

1) Lifelong antibiotic prophylaxis prior to dental and other potentially non sterile procedures. 2) When receiving intravenous fluids or medications, meticulous care should be taken to avoid the introduction of air bubbles (eg, use of intravenous in-line filters). 3) Avoid scuba diving 4) If PAVM is >=3 -> embolization 5) For patients with asymptomatic PAVMs in whom the feeding artery is <2 mm = yearly clinical observation, and a non-contrast CT every three to five years. 6) PAVMs that progressively enlarge or become symptomatic during follow-up should undergo evaluation for embolotherapy with pulmonary angiography 7) Consider genetic testing for HHT

Answer 258

Brain MRI at infancy and at puberty | Bubble echo at diagnosis, every 3-5 years, at puberty. If AVM, then CT chest.

Answer 259

You should consider checking the sibling for HHT by doing genetics HHT physical exam: - Vitals: saturation, orthodeoxia in supine and upright position - Epistaxis-->endonasal telangiectasia - Retinal telangiectasia and hemorrhage - Telangiectasia on lips, oral mucosa, finger tips. Can use a hand held illuminator to look for vascular anomalies on digits - Resp: thoracic bruit is heard in ½ of HHT patients with cyanosis, clubbing (association between cyanosis and clubbing with cerebral abscess and stroke) - CNS: Bruit if cerebrovascular malformation and open fontanelle - GI: · High-output heart failure · Hepatomegaly · Portal hypertension · Encephalopathy · Right-upper-quadrant pain and jaundice

Answer 260

Helium has the lowest specific gravity of any gas = Low specific gravity is associated with low density Combining helium and oxygen gas (Heliox) results in a gas with a similar viscosity to air but with a substantially lower density. Laminar flow is the most efficient way in which oxygen is delivered to the more distal parts of the bronchial tree.

Answer 261

1) Breathing Heliox leads to a reduction in the Reynolds number, converting turbulent flow into more efficient laminar flow. 2) Because of its low density, Heliox decreases the pressure gradient needed to achieve a given level of turbulent flow and this in theory reduces the work of breathing. The use of Heliox in asthma and upper airways obstruction is not for the treatment of the underlying disease, but is used to reduce airways resistance and respiratory muscle work until definitive treatments act

Answer 262

Upper airway obstruction Croup Asthma COPD

Answer 263

To be effective at reducing airways resistance by significant amounts the concentration of helium must be high, ideally greater than 70% of the inhaled gas mixture. This will limit the amount of oxygen that can be delivered simultaneously. More commonly (practically): croup and post-extubation stridor), need to be on <30% fiO2 to use heliox It’s also expensive!

Answer 264

Relatively increased levels of neutrophils in viral preschool wheeze Airway neutrophilia has been associated with the release of the potent neutrophil attractants IL‐8 and leukotriene (LT)B4

Answer 265

Relatively higher eosinophils Even during relatively asymptomatic periods, atopic wheezers in the study below still had higher eosinophil counts IL13, IL5, IL4, GM-CSF. Eosinophils release leukotrienes. (In general, IL5 is the most important)

Answer 266

1. Initiation of events that lead to Th2 inflammation 2. Suppression of Th1 mediated immunity 3. Recruitment of Th2 cells to the lung 4. Release of growth factors that contribute to the development of airway remodeling

Answer 267

IL-5 IL-5 also promotes eosinophil differentiation, growth, and survival

Answer 268

Glucocorticoids increase eosinophil apoptosis and block the survival effect of interleukin-5, resulting in a reduction in airway eosinophilia

Answer 269

1. Oral mucositis (peak 1–2 weeks after) 2. Pulmonary edema ( rapid onset, usually within the 2–3 weeks following HSCT) 3. Peri-Engraftment Respiratory Distress Syndrome (first 14 days following HSCT) 4. Idiopathic Pneumonia syndrome )initial peak approximately 2 weeks and a later peak 6–7 weeks post-HSCT.) 5. Diffuse Alveolar Hemorrhage (within 30 days) The preengraftment or early phase, from day 0 to day 30 or sooner, encompasses the time of marrow recovery leading to normalization of the peripheral neutrophil count

Answer 270

``` Anthrax Varicella Measles TB Smallpox Cryptococcus ```

Answer 271

Poorly organized, with loosely packed lamellae and vacuolar inclusions, having the appearance more of multivesicular or composite bodies.

Answer 272

Absent or small, abnormally formed lamellar bodies | Eccentrically placed electron-dense inclusions within the abnormal lamellar bodies give them a “fried-egg” appearance.

Answer 273

``` TB non-TB mycobacterial (more rare) post-TB scarring Sarcoidosis Chronic hypersensitivity pneumonitis, Post radiation fibrosis ABPA ```

Answer 274

Most common zonal predilection in bronchiectasis Mostly idiopathic Post infective bronchiectasis (recurrent childhood infections) Aspiration - Pulmonary aspiration diseases In association with immunodeficiency (Hypogammaglobulinemia) Primary ciliary dyskinesia As a sequelae of bronchiolitis obliterans in the setting of posttransplantation rejection

Answer 275

1. Infection or aspiration 2. Congenital conditions 3. Fibrosing Diffise Lung Disease 4. Endobronchial/peribronchial lesions 5. Other (BO)

Answer 276

Oxygen saturation is the fraction of oxygen-saturated hemoglobin relative to total hemoglobin (unsaturated + saturated) in the blood

Answer 277

Amount of oxygen in blood- includes oxygen bound to Hb and dissolved in blood O2 content = sats/100 x Hb (g/dl )x 1.39 + PaO2 X 0.003 in ml/dl of blood If Hb is in g/L divide it by 10 (generally the case with Canadian units) Normal is 20 ml O2 per 100 ml of blood

Answer 278

Amount of oxygen delivered to tissues per minute O2 DELIVERY = Cardiac output (Stroke volume x HR) x (arterial oxygen content) in ml/min Normal = 1000 ml O2/min for 5 litres/min CO

Answer 279

Campylobacter jejuni ,Mycoplasma Viruses- CMV, EBV, HIV, Influenza, Enterovirus D68

Answer 280

CHARGE and VACTERL (more common)

Answer 281

``` Tracheomalacia Aspiration Recurrent respiratory infections Wheeze Chronic cough Recurrence of fistula ```

Answer 282

5th week (during the embryonic stage: 3-8 weeks) Respiratory system starts as a median outgrowth, the laryngotracheal groove, which appears in the floor of the caudal end of the anterior foregut in the 4th week - this protrudes as lung bud which develops into the tracheobronchial tree Tracheoesophageal septum forms a partition between the trachea and esophagus at the end of 5th week.

Answer 283

``` Embryonic phase (3-7 weeks) Pseudoglandular (6-16 weeks) Canalicular (16- 26 weeks) Saccular (26- 36 weeks) Alveolar (from 36 weeks) ```

Answer 284

3-7 weeks Lung buds; trachea, main stem, lobar, and segmental bronchi; trachea and esophagus separate LUNG BUD

Answer 285

6-17 weeks Subsegmental bronchi, terminal bronchioles, and acinar tubules; mucous glands, cartilage, and smooth muscle BRONCHIAL TUBULES

Answer 286

16-26 weeks Respiratory bronchioles, acinus formation and vascularization; type I and II AEC differentiation ACINAR TUBULES

Answer 287

26-36 weeks Dilation and subdivision of alveolar saccules, increase of gas-exchange surface area, and surfactant synthesis TERMINAL SACCULES

Answer 288

36 - maturity Further growth and alveolarization of lung; increase of gas-exchange area and maturation of alveolar capillary network; increased surfactant synthesis ALVEOLI

Answer 289

Laryngeal, tracheal, esophageal, and bronchial atresia Tracheoesophageal and bronchoesophageal fistulas Tracheal and bronchial stenosis Bronchogenic cysts Ectopic lobes Extrapulmonary sequestration Pulmonary agenesis

Answer 290

Tracheomalacia and bronchomalacia Intralobar bronchopulmonary sequestration CPAM Acinar aplasia or dysplasia ACD-MPV Congenital pulmonary lymphangiectasia, and other pulmonary vascular malformations Hypoplasia with CDH

Answer 291

Congenital alveolar dysplasia Alveolar capillary dysplasia Pulmonary hypoplasia

Answer 292

Chi square | Fisher's exact test

Answer 293

T-test for two groups | ANOVA for more than 2 groups

Answer 294

``` Mann Whitney (Wilcoxon-rank sum) for 2 groups Kruskal Wallis for more than 2 groups ```

Answer 295

Alternating atelectasis with hyperinflation Severe airway epithelial lesions (e.g., hyperplasia, squamous metaplasia) Marked airway smooth muscle hyperplasia Extensive, diffuse fibroproliferation Hypertensive remodeling of pulmonary arteries Decreased alveolarization and surface area

Answer 296

``` Less regional heterogeneity Rare airway epithelial lesions Mild airway smooth muscle thickening Rare fibroproliferative changes Fewer arteries but “dysmorphic” Fewer, larger and simplified alveoli ```

Answer 297

Most common = ASPERGILLUS ,staph | Also: Burkholderia C ,Serratia, Nocardia

Answer 298

Cysic fibrosis = nNO is reduced in some patients with CF Viral illness = transiently decreased nasal nitric oxide Sinusitis = transiently decreased nasal nitric oxide

Answer 299

Cooperative child > 5 years of age (diagnostic accuracy in children <5 years has not been established) - Clinical phenotype of PCD - Exclude CF - Ideally more than one nNO test (on two separate occasions, at least two weeks apart) since viral illness and sinusitis can transiently lower nasal NO Low = <77nl/min

Answer 300

Endocrine: adrenal insufficiency, pseudohypoaldosteronism, hypothyroidism, hypoparathyroidism Skin: eczema, ectodermal dysplasia Malnutrition Renal: Nephrogenic DI Metabolic: G6PD, glycogen storage disease type 1

Answer 301

``` Insufficient sweat quantity due to incorrect technique or low weight (>2 kg and >2 weeks are the recommendations for sweat chloride in Kendig’s) Malnutrition Hyponatremia Edema Dilution ```

Answer 302

Hunter’s syndrome is type II mucopolysaccharidosis, which is a type of lysosomal storage disorder Deposition of glycosaminoglycans in throat and trachea = airway obstruction, OSA (they frequently need CPAP) Tracheobronchomalacia from deposition of GAG in tracheal/bronchial cartilage - this can affect airway clearance and cause recurrent infection infection, Restrictive lung disease due abnormal shape of ribs, scoliosis, pectus carinatum, enlargement of abdominal organs which affects diaphragm excursion Recurrent pneumonia due to above mechanisms

Answer 303

1. Airway Obstruction 2. Restrictive Lung Disease 3. Other (Disproportional length of trachea and spine, spinal cord compression, cardiac and CNS disease)

Answer 304

1. Start of test criteria: - If maximum inspiration after end of test is greater than FVC, then FIVC – FVC must be ≤0.100 L or 5% of FVC, whichever is greater - Back extrapolated volume ≤ 5% of FVC or ≤0.100 L, whichever is greater 2. During test: - No cough in first second - No glottic closure in the first second or after the first second - No obstruction of mouthpiece - No leak 3. One of the following end of test criteria should be achieved: - Plateau = ≤ 25 mL/s in the last second of expiration - Expiratory time ≥ 15 seconds - FVC is within the repeatability tolerance or greater than the largest prior observed FVC

Answer 305

Repeatability criteria depend on age a) If above 6 years of age: the difference between the two largest FEV1 and the two largest FVC must each be within 150 mL b) If 6 year or younger: the difference between the two largest FEV1 and FVC must within 100 mL or 10% of highest value, whichever is greate

Answer 306

The largest FEV1 and the largest FVC show up on the report. Other indices come from the maneuver with the largest sum of FVC and FEV1.

Answer 307

1) Glossopharyngeal breathing 2) Delivery of stacked breaths using a resuscitation bag with a one-way valve and mouthpiece 3) Mechanical inspiration through a mechanical insufflator or volume cycled ventilator

Answer 308

Carina (anywhere from pharynx to terminal bronchiole) Mechanical receptors in airway Chemical receptors in GI tract

Answer 309

Vagal and glossopharyngeal nerve

Answer 310

Upper brainstem and Pons

Answer 311

Vagus, Phrenic, and Spinal motor nerves to diaphragm, abdominal wall and muscles.

Answer 312

1. Afferent receptors: anywhere from pharynx to terminal bronchioles 2. Afferent receptor is stimulated 3. Afferent neuron (glossopharyngeal and vagus to cough centre in brainstem and pons 4. Signal from brainstem sent via efferent signal to larynx, diaphragm, chest wall muscles, abdominal muscles, pelvic floor 5. Cortical influence can voluntarily initiate or suppress cough

Answer 313

CT with pulmonary angiography

Answer 314

``` Factor V Leidein Prothrombin gene mutation Recent surgery, trauma, immobilization Active malignancy Heavy cigarette smoking Obesity Drugs ```

Answer 315

Ototoxicity Nephrotoxicity Neurotoxicity (neuromuscular blockade)

Answer 316

1. PAH - idiopathic, heritable, PPHN, drugs and toxins 2. PH due to left heart disease 3. PH due to chronic lung disease (ILD, COPD, BPD) 4. Chronic Thromboembolic PH (CTEPH) 5. Unknown/Multifactorial: heme, systemic inflammatory, metabolic, CHD

Answer 317

1) Anticholinergics: Transdermal scopolamine, glycopyrrolate, atropine S/E: Irritability, restlessness, sedation, and delirium from central effects of the drugs. Also, the inhibition of sweat glands has caused significant inhibition of temperature regulation. Inhibition of GI motility has resulted in worsening constipation in a population already plagued with such difficulties. Photophobia, urinary retention, and facial flushing also result from these medications. 2) Botox injections Botulinum toxin A inhibits the release of acetylcholine at the nerve terminal by inactivation of the 25-kDa synaptosome-associated protein SNAP-25, a protein essential for the fusion and release of acetylcholine-containing vesicles at the cell membrane. Adverse effects: Pain, dry mouth, difficulties swallowing, speech impairment.

Answer 318

Vasculature in front of trachea which is in front of esophagus Aortic arch encircles left mainstem

Answer 319

Bilateral indentation of Esophagus

Answer 320

AP: oblique defect upward to right Lat: small defect on right posterior wall

Answer 321

± Anterior indentation above carina between trachea and esophagus (course behind trachea and in front of esophagus)

Answer 322

The PHOX2B gene mutation, consisting primarily of increased polyalanine expansions, manifests an autosomal-dominant mode of inheritance and de novo mutations at the first generation. This gene is critical for embryologic development of the autonomic nervous system

Answer 323

Tumors of autonomic neural crest derivatives such as Neuroblastoma and Ganglioneuroblastoma. Pulmonary hypertension Cardiac ( Heart Block, Prolonged Sinus Pauses ) Risks related to prolonged use of non invasive ventilation SIDS

Answer 324

1- Suspected or confirmed active pulmonary, laryngeal or miliary Mycobacterium tuberculosis 2- Extrapulmonary Mycobacterium tuberculosis until pulmonary disease ruled out 3- VZV 4- Measles 5- Small pox

Answer 325

Neutrophils | Major cytokine = IL8, IL1B, IL6, TNF alpha

Answer 326

Resistance decreases as lung volume rises because th airways are pulled open Airway resistance = highest in the medium-sized bronchi, low in very small airways (large cross-sectional area)

Answer 327

The lower lobes In an upright position, due to a greater intrapleural pressure gradient in the upper lung, the alveoli in the upper zone are already more distended. When gas is administered, the lower lobes receive a greater portion of gas. Any change in the transpulmonary pressure during a normal respiratory cycle will cause a greater change in volume in the alveolus in the lower, gravity-dependent region of the lung than it will in the alveolus in the nondependent region of the lung, as shown by the arrows in the figure. Because the alveoli in the lower parts of the lung have a greater change in volume per inspiration and per expiration, they are better ventilated than those alveoli in nondependent regions (during eupneic breathing from the FRC).

Answer 328

1. Foreign body removal | 2. Assessment of posterior trachea (e.g., tracheoesophageal fistula) and larynx (e.g,. laryngeal cleft)

Answer 329

Limited ability to access more distal areas of the lung, and those requiring a flexible curve to reach, e.g., right upper lobe Limited ability to obtain bronchoalveolar lavage specimens

Answer 330

Fick’s Law: Diffusion= [surface area x diffusion coefficient x (P1-P2)]/thickness 1. Surface area 2. Thickness of the alveolar-capillary diffusion barrier 3. Solubility of the gas 4. Molecular weight of the gas 5. Partial pressure difference The volume of gas per unit of time moving across the alveolar-capillary barrier is directly proportional to the surface area of the barrier, the diffusivity, and the difference in concentration between the two sides, but is inversely proportional to the barrier thickness.

Answer 331

Directly proportional to the solubility of the gas and inversely proportional to the sq root of the molecular weight

Answer 332

There is a difference in pre and post-ductal saturation. There is right to left shunt of de-oxygenated blood from the pulmonary artery to the aortic arch via the patent ductus arteriosus, distal to the take off of the right brachiocephalic artery, and prior to the take off of the left subclavian artery. 2 causes: - Persistent pulmonary hypertension of the newborn - Interrupted aortic arch, aortic coarctation

Answer 333

1. Airway obstruction (laryngeal diphtheria – extension of pharyngeal diphtheria, or dislodgement of pharyngeal membrane) 2. Secondary pneumonia? Deaths are usually as a result of airway obstruction, myocarditis/cardiomyopathy, or sepsis

Answer 334

Corynebacterium diphtheriae Primarily involves tonsils, pharynx, larynx, nose, skin, occasionally other mucous membranes Mild catarrhal form or severe membranous form Incubation: 1-6d Clinical presentation: 3-4d URTI. Fever, membranous pharyngitis, fetor oris, cervical adenopathy, may have bull neck appearance secondary to edema of surrounding soft tissue. Adherent gray-white membrane, surrounded by inflammation, toxin causes local tissue destruction which promotes multiplication and transmission of bacteria. Can bleed with removal. Pharyngeal membrane, can have laryngeal extension. Disruption of membrane can cause airway obstruction. "membranous pharyngitis"

Answer 335

``` Secondary pneumonia Toxin-mediated disease Myocarditis or cardiomyopathy Neuritis or paralysis Adrenal failure with hypotension ```

Answer 336

Diphtheria antitoxin to neutralize circulating toxin (administer before lab confirmation). Antibiotics (penicillin, erythromycin x 14d) to eradicate the organism, stop toxin production and reduce transmission, but do not replace antitoxin. Prevention: vaccination, prophylaxis for close contacts

Answer 337

1. Rectus abdominus 2. Transversus abdominis 3. External and internal oblique 4. Internal intercostal muscles (depresses rib cage downwards, opposite of external intercostals)

Answer 338

1. PEP may have an effect on the peripheral airways and collateral channels of ventilation (Opens collaterals) 2. Increase in lung volume may allow air to get behind the secretions and assist in mobilizing. (Air behind secretions is pushed) 3. The increase in pressure is transmitted to airways creating back pressure stenting them during exhalation , therefore preventing premature airway closure thus reducing gas trapping . 4. Prolonged Exhalation with huff breathing helps mobilize secretions

Answer 339

Anterior: Thymoma, teratoma Middle: lymphoma, metastatic disease Posterior: neuroblastoma, pheochromocytoma, rhabdomyosarcoma

Answer 340

Anterior: thymus (cyst, enlargement), thyroid goiter Middle: lymphadenopathy, bronchogenic cyst Posterior: hemangioma, ganglioneuroma, neurofibroma

Answer 341

Pleural protein to serum protein > 0.5 Pleural LDH to serum LDH > 0.6 Pleural LDH >2/3 upper limit normal serum LDH

Answer 342

Hypersensitivity Pneumonitis (Bird Fancier's lung)

Answer 343

Hypersensitivity Pneumonitis known as extrinsic allergic alveolitis = an immune mediated lung disease occurring in response to repeated inhalation of an antigen. The initial event involves sensitization to an inhaled antigen in the distal airway, but the level and duration of antigen exposure that is required for sensitization is unknown. After antigen exposure in a sensitized individual, an acute alveolitis develops with an increase in neutrophils. This typically peaks at 48 hours and is followed by an increase in the number of macrophages and lymphocytes. Typically CD8+ T cells predominate, but CD4+ T cells can also be found distinguishing HP from sarcoidosis.

Answer 344

5-10 μm - More likely to deposit in the upper airways. However this can be avoided to achieve lower airway penetration by a slower respiratory flow. < 1 μm exhaled ( they have low inertia and settling velocity) <3 µm will deposit in the lower and smaller airways Particles greater than 5 µm generally deposit in the oropharynx or upper airways. 1–7 µm considered as a “ respirable range” Therefore minimizing the particle size of aerosolized drugs is essential for targeted deposition in the smaller airways

Answer 345

``` Particle size Particle velocity Hygroscopic properties Drug viscosity and surface tension Suspension vs solution ```

Answer 346

``` Age Inspiratory flow rate Breathing pattern (inspiratory volume, rate) Nasal vs mouth breathing Anatomy (upper and lower airways) Disease severity Physical and cognitive ability Adherence, contrivance ```

Answer 347

1. Pleuritis with pleural effusion 2. Acute lupus pneumonitis 3. Chronic lupus ILD 4. Pulmonary hemorrhage 5. Pulmonary HTN 6. Shrinking lung syndrome

Answer 348

Derived from the multiple breath washout (MBW) test which involves following the washout of an inert tracer gas from lungs during relaxed tidal breathing. With each successive breath of the washout, there’s a fall in the peak concentration of the exhaled tracer gas and the LCI is the number of ‘lung turnovers’ required for the end-tidal tracer gas concentration to fall to 1/40th (2.5%) of it’s initial concentration. ``` LCI = Cumulative Expired Volume/FRC Normal = <7 ```

Answer 349

LCI reflects abnormalities of the smaller airways and is increased in the presence of airway narrowing caused by either inflammation, mucus plugging/retention and airway wall structural damage. Thus in the presence of obstructive disease washout of the tracer gas will take longer to complete requiring a greater number of breaths resulting in an elevated LCI. Because of inhomogeneity of ventilation / time constants.

Answer 350

Residual injury to the peripheral airways | Small airway obstruction and gas trapping- increased FEV1 and increased RV/TLC

Answer 351

1. Asthma diagnosis confirmed by history and objective measures 2. Treatment needed: high dose ICS and second controller for previous year / oral steroids >50% last year -asthma either uncontrolled despite these or needing the above for prevention of loss of control 3. Environmental factors, comorbidities, adherence and inhaler technique addressed before labelling as severe asthma

Answer 352

1. CTS asthma control criteria ACQ >1.5 or ACT or cACT >20 2. Frequent(>2) exacerbation needing oral steroids 3. 1 or more Severe exacerbation needing ICU or mechanical ventilation 4. FEV1<80% with low ratio

Answer 353

``` Daytime symptoms <4 days/week, Night-time symptoms <1 night/week, Physical activity Normal, Exacerbations Mild, infrequent Absence from work or school due to asthma None Need for a fast-acting b2-agonist <4 doses/week FEV1 or PEF >90% personal best PEF diurnal variation <10–15% Sputum eosinophils* <2–3% ```

Answer 354

IgE levels >1000 IU/ml needed in classic ; only>500 needed for minimal criteria If 200-500 repeat test Criteria: 1. Acute or subacute clinical deterioration that is not attributable to another etiology 2. A serum total IgE level >1000 unless receiving systemic steroids 3. Presence of IgE antibodies to A. fumigatus or immediate cutaneous hypersensitivity to Aspergillus 4. Precipitating antibodies to Aspergillus or serum IgG antibody to Aspergillus by an in vitro test 5. New or recent infiltrates on CXR or CR That do not respond to antibiotics or physio

Answer 355

Central varicose bronchiectasis AND high attenuation mucus plugs in CT suggests ABPA

Answer 356

1. Adequately treated with steroids - no symptoms 2. Technical faults - constant expiratory flow is not maintained - will have symptoms 3. Smoking can cause lower FENO levels - can have symptoms 4. Non eosinophilic asthma(steroid resistant) - will have symptoms

Answer 357

Delta of 10 for values <50 and delta of 20% for values more than 50 are considered significant changes in between visits Lower cutoffs are 20 and 25 (>12 yrs) Upper cutoffs are 35 (<12 yrs) and 50 In between cutoffs the values are interpreted according to clinical context (20-35 in children and 25 to 50 in adults)

Answer 358

FENO can be used to a) diagnose eosinophilic airway inflammation b) predict steroid responsiveness and c) to monitor treatment and d) to indicate nonadherence to steroid therapy Three isoforms of NOS (nitric oxidate synthetase)- nNOS (neuronal), INOS (inducible) (both in airway epithelIum) and eNOS (endothelia)l iNOS has the capacity to generate large quantities of NO when transcriptionally upregulated by inflammatory cytokines, such as tumor necrosis factor (TNF)-alpha, interleukin 1 (IL-1)-beta, interferon (IFN)-gamma, IL-4, and IL-13 . In vitro evidence suggests that this upregulation can be abolished by glucocorticoids in vitro and in vivo . The majority of exhaled NO originates from the lower airways. A FENO less than 25 ppb in adults and less than 20 ppb in children younger than 12 years of age implies the absence of eosinophilic airway inflammation and non responsiveness to glucocorticoids

Answer 359

Echinococcus genus

Answer 360

``` Transmission - eating water and food contaminated by echinococcus eggs in the faeces of the definite host or close contact with canine,sheep No human to human transmission - Humans are accidental hosts Definite host (adult sexually mature worms are seen in the intestine) - canines For E multilocularis which causes alveolar form - fox is the definitive host ``` ``` Intermediate host (maintain larval forms) = sheep and livestock Eggs are present in the faeces of definitive host- these eggs once swallowed by humans, hatch and embryos penetrate intestine and are hematogenously spread to mainly liver and lungs where they grow into 3 layered cysts ```

Answer 361

``` Cough (coughs up grape cysts) Chest pain Hemoptysis Fever Bronchospasm ```

Answer 362

1. Meniscus sign /crescent sign 2. Cumbo sign (double air layer appearance or onion peeL or double arch) 3. Water Lilly or camellote sign 4. Ultrasound signs- same as xray plus - honeycomb appearance , ball of wool sign, cyst wall calcification

Answer 363

1. Cystic (predominant form affecting lungs) 2. Alveolar 3. Polycystic echinococcosis (least common)

Answer 364

Surgery ,USS guided aspiration and chemotherapy USS guided aspiration if single cyst (Puncture, aspiration, injection and reaspiration) has widely replaced surgery and chemo Pretreat with albendazole before percutaneous drainage /surgery Lung resection is not advised - cystectomy is advised if going for surgery Chemo for upto 1 month after removal is controversial In asymptomatic patients, multiple cysts in >1 organs, recurrence - chemo alone can be tried Chemo - albenadazole or mebendazole , can add praziquantel after surgery for synergy

Answer 365

``` Blowing into the eyes Poor mask and headgear fit High leak Inappropriate settings Not densitised with mask Poor sensing - dyssynchony Lack of humidification ```

Answer 366

``` AF = clinical syndrome characterized by rapid onset of weakness, including weakness of muscles of respiration and swallowing, progressing to max severity within days to weeks. Flaccid = absence of spasticity or other signs of disordered CNS tracts ``` Causes: Enterovirus D68, West nile, Adenovirus, Guillan barre, Botulism, Tetanus, Spinal cord compression Mechanisms: - Atelectasis - Aspiration - Hypoventilation/respiratory muscle weakness - Secondary infection - GB -> autonomic neuropathy -> cardiac failure -> pulmonary edema

Answer 367

- Age, in particular pediatric age group and highest incidence at 2-4 years of age - Pulmonary infection such Mycoplasma pneumonia - Fat embolism - Post operative atelectasis - Lower concentration of fetal haemoglobin - Bronchospasm due to asthma - Higher WBC count - Previous ACS (most important)

Answer 368

Dry: o Allergic rhinitis and post nasal drip o Asthma o GERD o Tracheobronchomalacia +/ - an associated abnormality like vascular ring o Habit cough o Interstitial lung disease – eg. Eosinophilic lung disease o Post infectious cough: Pertussis and Mycoplasma pneumonia (Kendig’s) o Drugs like ACEi Wet: o Cystic fibrosis o Primary ciliary dyskinesia o Retained foreign body – consider in child<5 years, typically wet cough, but could be dry. Associated: wheeze, halitosis. o Aspiration o Immunodeficiency o Chronic endobronchial suppurative disease: § Non-CF bronchiectasis- eg. Immunodeficiency § Persistent bacterial bronchitis Other: - Chronic infection: TB (cough, monophasic wheeze from hilar lymphadenopathy or tuberculoma), fungal (histoplasmosis, cocciodoides), Chlamydia pneumonia, Mycoplasma pneumonia, non-tuberculous mycobacteria - Chalmydia trachomatis, CMV can cause “afebrile pneumonia of infancy” - Non-infective bronchitis: from exposure to environmental pollutants - environmental tobacco smoke, biologic pollutants (eg, dust mite, mold, pets), nitrogen dioxide from unvented gas heating, and particulate matter from wood stoves and cooking - Mediastinal mass

Answer 369

Mueller maneuver = inhalation against closed glottis decreases intrathoracic pressure and increases blood return to the lungs = Increased DLCO

Answer 370

Decreased DLCO

Answer 371

decreased V = decreased DLCO (normal if DLCO is corrected for volume)

Answer 372

low Hb-->decreased DLCO (normal if DLCO if corrected for Hb) | Pulmonary hemorrhage -->increased DLCO

Answer 373

High carboxyhemoglobin-->decreased DLCO

Answer 374

DLCO = [VA x Pulm cap blood volume x Hgb] / [A-C membrane thickness x COHb]

Answer 375

1. Exclude patients with perinatal lung disease 2. Within 7 days of known clinical insult 3. Respiratory failure not fully explained by cardiac failure or fluid overload 4. Chest imaging findings of new infiltrate(s) consistent with acute pulmonary parenchymal disease 5. Oxygenation CPAP ≥ 5, P/F ≤ 300, SpO2/FiO2 ≤ 264 Mild = 4 ≤ OI ≤8 Moderate = 8 ≤ OI ≤ 16 Severe = OI ≥ 16

Answer 376

mean airway pressure MAP × FiO2 × 100÷PaO2

Answer 377

TLC: reduced RV: may increase due to air trapping FRC: decreased ERV: decreased

Answer 378

The BTS air travel guidelines recommend that complete resolution of the pneumothorax and that then a minimum 7 days should elapse prior to flying. In patients with underlying conditions longer periods are required = CF, the consensus is recommending at least 2 weeks from resolution.

Answer 379

1. Spirometry (wait 2 weeks post pneumo) if FEV1 <50% predicted - do HCT 2. Hypoxic challenge testing: If SpO2 falls below 90%, in-flight oxygen is advised

Answer 380

1. Immune Reconstitution Inflammatory Syndrome 2. ILD 3. Pulmonary HTN 4. Recurrent infections 5. Bronchiectasis/BO 6. Malignancy

Answer 381

A syndrome that develops in the context of recovery of CD4 cell counts following the initiation of Antiretroviral therapy (ART). IRIS is believed to represent an excessive inflammatory response to an antigen, most frequently a subclinical or recently treated opportunistic infection. It is most commonly seen in conjunction with such infections as TB, PCP, MAC, and Cryptococcus Most cases occur in the first few weeks after starting ART, though cases of IRIS have been reported up to 12 months following the initiation of therapy. Risk factors include low CD4 count prior to initiation of ART and opportunistic infection treatment proximal in time to ART initiation.

Answer 382

ABCA3 Tx = Oxygen/supportive, steroids, hydroxycholorquine

Answer 383

1. Giant cells, granulomas, or Schaumann bodies 2. Interstitial pneumonia (UIP)–like Pattern. 3. A nonspecific interstitial pneumonia (NSIP)–like pattern or a bronchiolitis obliterans organizing pneumonia like disease can also be seen in chronic HP

Answer 384

The classic triad of 1. Interstitial lymphocytic-histiocytic cell infiltrate. 2. Bronchiolitis obliterans. 3. Scattered poorly formed non-necrotizing granulomas.

Answer 385

1. Interstitial mononuclear cell infiltrate. | 2. Granulomas and macrophages with foamy cytoplasm have also been reported.

Answer 386

A peak flow reading will only be as good as your effort. Numbers can be artificially high with tongue thrusts or spitting. Or they may be artificially low due to not enough effort or poor technique

Answer 387

1. High elastic recoil results in exhalation of FVC prior to 6 seconds 2. Require coaching specific to their developmental level, with possible incentive display. 3. Restrictive lung disease

Answer 388

pH unchanged pO2 increased pCO2 decreased HCO3 unchanged

Answer 389

Qf = Kf[(Pc − Pis) − σ(πpl − πis)] where Qf = net flow of fluid Kf = capillary filtration coefficient; this describes the permeability characteristics of the membrane to fluids and the surface area of the alveolar-capillary barrier Pc = capillary hydrostatic pressure Pis = hydrostatic pressure of the interstitial fluid σ = reflection coefficient; this describes the ability of the membrane to prevent extravasation of solute particles such as plasma proteins πpl = colloid osmotic (oncotic) pressure of the plasma πis = colloid osmotic pressure of the interstitial fluid The Starling equation describes the movement of liquid across the capillary endothelium

Answer 390

Decreased colloid osmotic pressure

Answer 391

Increased capillary hydrostatic pressure

Answer 392

1. Double aortic arch (most common) 2. Aberrant left subclavian and right aortic arch (second most common) 3. Circumflex aorta (rare) 4. Persistent 5th aortic arch (rare) 5. Cervical aortic arch 6. Pulmonary artery sling (not a true vascular ring) Symptoms: Respiratory: noisy breathing, barking cough, wheeze, frequent URTI, apneic episodes, intermittent cyanosis (from tracheal compression) GI: difficulty swallowing (solids) – sensation of food being stuck, slow eating, hyperextension during feeds (from esophageal compression)

Answer 393

1. Thoracic surgery with iatrogenic injury to recurrent laryngeal nerve (e.g., PDA repair, Norwood cardiac repair, esophageal surgery especially TEF repair, thyroid surgery) 2. Peripheral nerve pathology 3. Mediastinal lesion (e.g., tumor)

Answer 394

1. Idiopathic (usually) 2. Arnold-Chiari malformation 3. Hydrocephalus 4. Meningocele 5. Myasthenia gravis 6. Birth trauma (may be transient)

Answer 395

Left due to the path of the recurrent laryngeal nerve

Answer 396

Usually left side Breathy voice Acceptable airway Risk of aspiration

Answer 397

Normal voice Biphasic stridor Airway compromise

Answer 398

Autosomal co-dominant Yes - Yes if symptomatic. Discuss predispositional testing if asymptomatic. Avoid smoke exposure to reduce risk of emphysematous changes.

Answer 399

AAT inhibits the activity of a number of proteolytic enzymes, the most important of which is neutrophil elastase Lung disease in AAT deficiency occurs primarily secondary to loss of function, resulting in an imbalance between levels of AAT relative to the amount of neutrophil elastase. Neutrophil elastase degrades elastin and extracellular matrix elements located within the lower respiratory tract that normally function to maintain the structural integrity of the lung. Major role = inhibit neutrophil elastase -> degrades elastin (also basement membrane + matrix) Synthesized by hepatocytes

Answer 400

1. Normal (>200umol/L) 2. Deficiency (<20), Z-variant = most common Z variant = normal mRNA and synthesis rate, ~15% released into circulation (85% blocked in the terminal secretory pathway of hepatocytes) 3. Null - no detectable circulating AAT 4. Dysfunction - AAT Pittsburgh (thrombin inhibitor), PI*F (decreased association with elastin)

Answer 401

Most common abnormal alleles = S and Z S = associated with a mild decrease in circulating levels and no hepatocyte accumulation of AAT Z = substantial decrease of circulating levels and hepatocyte accumulation of AAT Highest to lowest levels = PI*MS, PI*SS, PI*MZ, PI*SZ, PI*ZZ PI*ZZ = autosomal co-dominant gene PI*MS and PI*SS have levels that are above what is needed to protect the lungs -> no increased risk of lung disease Null allele -> no detectable levels and pts are at the highest risk of lung disease (but no liver disease as AAT is not produced and therefore not accumulated in hepatocytes)

Answer 402

Deficiency/null + homozygous or heterozygous

Answer 403

Permanent dilation of the air spaces distal to the terminal bronchioles secondary to destruction of alveolar wall and without fibrosis ?secondary to protease-antiprotease imbalance Imbalance between antielastase defenses and excess leukotriene elastase -> elastase degradation *major pathogenic factor = cigarette smoke (capable of inactivating AAT and recruits inflammatory cells)

Answer 404

Confirm absence of AAT peak Early onset pulmonary emphysema Family history of AAT Dyspnea and cough in multiple family members Liver disease NYD COPD Bronchiectasis with no evident etiology Asthma that doesn't reverse with treatment Unexplained panniculitis and anti-protease 3 vasculitis

Answer 405

Panacinar emphysema with basal predominance PFT - Decreased FEV1, normal (or decreased) FVC, decreased FEV1/FVC (loss of elastic recoil from parenchymal disease with dynamic collapse of normal airways) - Increased compliance = hyperinflation with increased RV and TLC (pleth>dilution), air trapping - Decreased DLCO (impairment of gas exchange) ○ More advanced disease -> MIPS/MEPS CXR -> usually normal initially Advanced: hyperinflation and increased radiolucency (especially in the lower segments

Answer 406

1. Bronchodilator: if helpful, add ICS if evidence of bronchial hyperreactivity 2. Antibiotics with bronchitis or URTI -> beta lactams or quinolones 3. Oxygen if desats with exercise 4. Nutrition 5. Augmentation Treatment

Answer 407

1. IV human plasma-derived augmentation therapy 2. Inhalation 3. Recombinant AAT 4. Synthetic elastase inhibition Indications: Based on the presence and level of obstructive lung disease = FEV1 30-65% or normal lung function with rapid decline (change >120mls/yr)

Answer 408

1. Liver disease - neonatal cholestasis -> resolves by adolescence (30-40% -> cirrhosis, carcinoma >50yo) 2. Necrotizing panniculitis 3. Multisystem vasculitis

Answer 409

Characterized by the triad of: 1. Abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVDs) in the setting of liver disease 2. Portal hypertension 3. Congenital portosystemic shunts

Answer 410

``` Pneumonia Atelectasis Chest trauma Alveolar hemorrhage Malignancy. ```

Answer 411

1. Spontaneous recurrent epistaxis 2. Multiple telangiectasias (in multiple sites) 3. Visceral lesions (AVMs -> GI bleeding, or pulmonary, cerebral or spinal hemorrhage) 4. First degree relative with diagnosed HHT Need 3/4

Answer 412

1. CBC + diff 2. Ferritin, stool for occult blood 3. Brain Angiography, endoscopy, chest CT, bubble echo 4. Genetics

Answer 413

TST - IGRA has not been validates in <2yrs | ** TST can be false negative <2

Answer 414

1. BCG given in infancy and the person tested in now ≥ 10yo 2. High probability of TB infection: close contacts, aboriginal Canadians from a high-risk community or immigrants/visitors from a country with high TB incidence 3. There is a high risk of progression from TB infection to disease

Answer 415

Treat as latent TB until repeat TST 8 weeks later is done (The rifampin may be discontinued if, after a period of 8 weeks after the last contact, the repeat TST is negative, and the child remains asymptomatic and is immunocompetent and more than 6 months of age) NEJM paper on rifampin x 4 months equivalent to INH x 9 months

Answer 416

No further treatment needed

Answer 417

Conversion = a TST of 10 mm or greater when an earlier test resulted in a reaction of less than 5 mm. Original between 5-9: An increase of 6 mm or more - this is a more sensitive criterion, which is suggested for those who are immune compromised with increased risk of disease or for an outbreak. An increase of 10 mm or more - this is a less sensitive but more specific criterion. In general, the larger the increase, the more likely that it is due to true conversion TST conversion occurs within 8 weeks of exposure. Certain medical conditions will increase risk of conversion as well as drugs (steroids, anti-TNF) Systemic corticosteroids at daily doses of ≥15 mg prednisone equivalent for 1 month or longer should first be tested for LTBI.

Answer 418

``` AIDS HIV Transplantation SIlicosis Chronic renal failure requiring hemodialysis Recent TB infection (within last 2 years) Abnormal CXR (fibronodular disease) Carcinoma of the head and neck ```

Answer 419

Significant contact with a person with suspected of confirmed contagious pulmonary TB Asymptomatic, normal CXR, normal TST

Answer 420

Asymptomatic, normal CXR, but positive TST

Answer 421

Disease in person wth TB infection in whom signs and symptoms or radiographic manifestations are apparent

Answer 422

Child under 5 years AND high risk of TB infection

Answer 423

HIV (well) Close contact with active contagious case (past 2 years) Presence of fibronodular disease on CXR (healed TB) Organ transplant TNF-alpha inhibitors Other immunosuppressive medications End stage renal disease

Answer 424

All others including: - TST conversion (within 2 years) - Diabetes, malnutrition - Hematologic malignancies

Answer 425

Mycobacterium TB infection NTM Infection BCG in the past Incorrect technique

Answer 426

``` Incorrect technique Active TB (esp if disseminated) Steroids Young age Malnutrition Viral infections (measles, varicella, influenza) Live vaccines ```

Answer 427

Interferon Gamma Release Assays Measures T-cell release of IFN-gamma in response to M. TB A: More specific for M. Tuberculosis (does cross react with BCG and most NTM) Does not require follow-up visit in 48-72 hours Most useful for latent TB diagnosis in BCG recipients

Answer 428

Cross reacts with some NTM species Limited S&S for active TB diagnosis (cannot tell LTBI from active, sensitivity decreased by temporary anergy of acute illness, reduced sensitivity in immune compromised individuals)

Answer 429

1. TST and IGRA done BUT do not distinguish LTBI from active disease and do not "rule out" Pulmonary TB 2. CXR 3. Gastric aspirates (3 consecutive mornings, taken before ambulation or feeding) 4. BAL in select cases 5. Microbiology (acid fast staining, DNA probes, PCR)

Answer 430

``` Hepatotoxicity Peripheral Neuropathy (interferes with pyridoxine metabolism) ```

Answer 431

``` Hepatotoxicity Hypersensitivity reactions Memory Impairment Drug interactions Body fluid turns orange ```

Answer 432

Hepatotoxicity | Increased uric acid levels

Answer 433

Optic neuropathy (decreased acuity, decreased visual fields, colour blindness)

Answer 434

Intensive = 3-12 weeks of IV Amikacin PLUS 1 of: Tigecycline, Imipenum, Cefoxitin (IV) (consider adding macrolide) "For ABS you need to do CIT-ups" Amikacin: diarrhea, hearing loss vertigo Tigecycline: nausea/vomiting, diarrhea Cefoxitin: nausea/vomiting, diarrhea

Answer 435

Inhaled Amikacin with 2-3 of: PO Minocycline, Moxifloacin, Linezolid, Clofazimine

Answer 436

Tac: peripheral neuropathy, HTN, nephropathy, diarrhea MMF: leukopenia, thrombocytopenia

Answer 437

1. PLeuritis with pleural effusion 2. ILD 3. Pulmonary hemorrhage 4. Pulmonary HTN 5. Shrinking lung syndrome 6. Acute pneumonitis

Answer 438

Restriction + low DLCO

Answer 439

Brain MRI ?genetics (CCHS)--but might be more convinced if hypoventilation Other: metabolic work up, infectious work-up

Answer 440

``` 2 trach tubes, one size and one size below Obturator from current trach tube Portable Suction and catheter Syringes for sterile water If vented - ventilator and bagger ``` Others: Two clean trach tubes with holders attached and obturators in place: One tube that’s the same size as the tube your child has in place One tube that’s a size smaller than the tube your child has in place Trach ties and noses Spare inner cannula Small blanket or towel roll Sterile water and water soluble lube Portable suction Luer-Lock syringe (if trach tube is cuffed) Instructions for CPR and how to troubleshoot an obstructed tube Oxygen Emergency phone numbers

Answer 441

NIV should not be used in patients: - After recent facial or upper airway surgery - In the presence of facial abnormalities such as burns or trauma - If there is fixed obstruction of the upper airway - If the patient is vomiting. Contraindications to NIV include: - Recent upper gastrointestinal surgery - Inability to protect the airway - Copious respiratory secretions - Life threatening hypoxaemia - Severe co-morbidity - Confusion/agitation - Bowel obstruction - Poor glottic function - Inability to achieve adequate ventilation noninvasively - Patient preference - Lack of caregiver expertise.

Answer 442

Anticholinergic - a long-acting muscarinic receptor antagonist

Answer 443

There are five subtypes of muscarinic receptors, three of which are found in human airways (M 1 , M 2 , M 3 ). All three types of muscarinic receptors mediate neurally induced bronchoconstriction. Tiotropium has a high affinity for M receptors but dissociates very slowly from type M1 and M3 receptors, resulting in prolonged prevention of bronchoconstriction and a 24-hour bronchodilatation effect.

Answer 444

The effect on lung function is comparable to that of salmeterol, and there may also be acute bronchodilatation in patients with poorly controlled symptoms.

Answer 445

Tiotropium has a high affinity for M receptors but dissociates very slowly from type M1 and M3 receptors, resulting in prolonged prevention of bronchoconstriction and a 24-hour bronchodilatation effect.

Answer 446

Longer acting than ventolin so more convenient to use

Answer 447

1. Tolerance and reduced sensitivity to the bronchodilator effects of salbutamol 2. Increased risk of exacerbation, serious adverse effects and death

Answer 448

Methacholine mimics the neurotransmitter acetylcholine to directly interact with muscarinic receptors on airway smooth muscle, resulting in contraction and airway narrowing. Similar direct airway responses can be demonstrated with histamine. When the muscarinic receptors are directly stimulated, the response is felt to reflect a property of the airway smooth muscle, but airway structure and geometry also influence the response. Methods: - Methacholine challenge - Histamine

Answer 449

Bronchoconstriction can be elicited through exercise or other stimuli to the airways that act indirectly to cause airway narrowing. Both exercise and eucapnic hyperventilation cause airway drying and cooling, and the stimulus to bronchoconstriction is now recognised to involve the osmolar change in the airway epithelium resulting from water loss. An osmolar stimulus can also be induced by inhalation of hypertonic saline or, in a more recently developed test, by inhalation of mannitol powder. Other indirect challenge tests causing bronchoconstriction by stimulation of inflammatory cells and mediators and/or sensory nerves include adenosine monophosphate and allergen challenge. Methods: - Exercise - Mannitol - Hypertonic saline - osmotic stimulus - Allergen challenge

Answer 450

Limit to 2500 lipase units per kg per meal or 10,000 lipase units per kg per day Side effects: fibrosing colonopathy

Answer 451

Small bowel bacterial overgrowth C-diff Celiac

Answer 452

Cs = VT/Pplat-PEEP

Answer 453

Cd = VT/PIP-PEEP Decreases with increasing RR or airway resistance An increase in respiratory rate will result in an increase in venous return. (and increased airflow)

Answer 454

PFTs frequently show decreased forced expiratory volume in one second (FEV1) and decreased ratios of FEV1 to forced vital capacity (FEV1/FVC), consistent with airflow limitation and small airway obstruction

Answer 455

1. Less regional hetergeneity 2. Mild airway smooth muscle hypertrophy 3. Rare fibroproliferation 4. Alveolar simplification 5. Fewer arteries, but "dysmorphic' 6. Rare airway epithelial lesions

Answer 456

1-If there is evidence of immunity, nothing. Observe clinically , if developed disease treat with antiviral 2-If no evidence of immunity , then depend on steroid route/dose and duration . Definition of high dose steroid in regard to immunosuppression: Receiving daily corticosteroid therapy at a dose ≥15 mg (or >2 mg/kg/day for patients weighing <10 kg) of prednisone or equivalent for ≥14 days; 3- The timing to vaccine if patient is on high dose steroid is 4 weeks after stopping

Answer 457

``` Neonatal cholestasis Focal biliary cirrhosis Multilobular cirrhosis Non-cirrhotic portal hypertension Hepatic steatosis Gallstones ```

Answer 458

``` HIFLOW Heat and humidification of secretions Decrease inspiratory effort FRC increases (CPAP effect with PEEP) Lighter - more tolerable Oxygen dilution (can minimal oxygen dilution by meeting flow demands) Washout of dead space ```

Answer 459

1. Cardiac ( Heart Block – Prolonged Sinus Pauses ) ECG – Seventy-two-hour continuous electrocardiography (Holter monitoring) 2. Ophthalmic (meiosis, strabismus, and convergence insufficiency) – Ophthalmologic assessment , Pupillometry (measurement of pupil size and reactivity) 3. Malignancy (CCHS is associated with tumors of neural crest origin, primarily neuroblastomas) risk is strongly associated with PHOX2B genotype 4. GI (Hirschsprung) – Clincaly monitoring [Delayed passage of meconium (failure to pass meconium within the first 48 hours of life), Constipation, Abdominal distension, Tight anal sphincter, or symptoms of enterocolitis (fever, vomiting, abdominal distension, and explosive diarrhea). Contrast enema and rectal suction biopsy. 5. Neurobehavioral - Cognitive deficits in children with CCHS, including learning disabilities, limitations in perceptual skills, vocabulary and abstract reasoning, Neurocognitive testing – Annual neurodevelopmental screening is recommended, to monitor for progressive dysfunction (which might be caused by insufficient ventilation, recurrent hypoxemia, or by the underlying disorder), and to optimize support services

Answer 460

* For patients with PHOX2B NPARMs, close surveillance for neuroblastoma is recommended, consisting of chest and abdominal imaging and urine catecholamines (every three months until age two years, then every six months until age seven years). * For patients with longer PARMs (20/28 and longer), surveillance for ganglioneuromas and ganglioneuroblastomas is recommended, consisting of annual chest and abdominal imaging. * For patients with short PARMs (24 through 27 repeats), surveillance is not required.

Answer 461

Tuberculous meningitis | Constrictive pericarditis, and patients at high risk of constrictive tuberculous pericarditis.

Answer 462

Inhaled nitric oxide selectively dilates pulmonary vasculature in ventilated areas of the lung. Mechanism of action: NO activates soluble guanylyl cyclase (sGC) to produce cyclic guanosine monophosphate (cGMP) leading to decrease vascular smooth muscle tone (ie, vasodilation) Additional effects of NO include suppression of both smooth muscle proliferation and platelet aggregation

Answer 463

1. Hemodynamic deterioration 2. Methemoglobulinemia 3. Rebound pulmonary HTN Weaning approach: Can wean rapidly to 5 ppm After 5 ppm, need to wean slower to b/c of rebound pulmonary hypertension

Answer 464

Chronic Hyperinflation - chronic pulmonary disease | Air trapping in obstructive disease results in increased residual volume

Answer 465

Compliance decreases in obstructive disease (if there is hyperinflation) Hypoxemia : V/Q mismatch ( stretched alveoli and narrow extra alveolar vessels)

Answer 466

``` Ipratropium bromide IV steroids IV MgSO4 Start BIPAP IM epinephrine IV Ventolin ```

Answer 467

Avoid triggers Compliance with LTRA (Preferred) or ICS Consider use of ipratropium bromide as reliever Consideration should be given to use of ipratropium bromide as a reliever medication. If this is ineffective, beta-agonist reliever should be added. If symptoms are severe enough to require a preventer medication then consider starting a leukotriene antagonist as first line. The aim here would be to avoid the potential corticosteroid related increase in cardiac event risk. Failure to achieve control on a leukotriene anatagonist could lead to addition of low-dose inhaled corticosteroids.

Answer 468

LABA with rapid onset Formoterol has a rapid onset of action comparable with albuterol (within three to five minutes), in addition to being long-acting (up to 12 hours of bronchodilation).

Answer 469

More consistent use (for reliever therapy) results in improved ICS use ( increased compliance). Decreases in asthma exacerbation and emergency visits.

Answer 470

Not concerned The SMART trial initially showed worse outcomes in african americans. The results have been controversial because there were differences in baseline asthma characteristics between white and african american patients (lower baseline peak exp flow, more nocturnal symptoms, more emergency department visits and hospitalisations).

Answer 471

Papillomas: Macroscopically visible “cauliflower-like” exophytic lesions Location: Larynx Less often, affects the oral cavity, trachea and bronchi.

Answer 472

``` FB Lung malformation TB Obstructive (external compression) Aspiration Tumour Infection ```

Answer 473

The gene is typically responsible for expression regulation of genes involved in the development of the autonomic nervous system

Answer 474

``` Hirschsprung Abnormal pupillary response Cardiac asystole, sinus pauses Development of neural crest tumours ANS dysregulation ```

Answer 475

Class 1: Protein synthesis defect (blueprint for making a door, for example) Class 2: Protein maturation defect (manufacturer) Class 3: Gating defect (door doesn't open) Class 4: Conductance defect (door too small) Class 5: Reduced quantity (production vs demand) Class 6: Reduced stability (poor quality material)

Answer 476

Non-caseating granulomas

Answer 477

Stage 0: Normal imaging Stage 1: Bilateral hilar infiltrates Stage 2: Parenchymal infiltrates + hilar infiltrates Stage 3: Parenchymal infiltrates without hilar infiltrates Stage 4: Fibrosis

Answer 478

Granulomatous lesions can occur in any organ but most commonly in lung, lymph nodes, eyes, skin, liver, heart Non-specific symptoms are common (fatigue, weight loss, fever) Lofgren syndrome is less common in children (acute arthritis, BHL, erythema nodosum) Can have cardiac, ocular, neurological, renal involvement and hypercalcemia/hypercalciuria Common skin lesions = papules, plaques, nodules, changes in old scars, erythema nodosum, hyperpigmented lesions, and hypopigmented lesions. Central nervous system involvement may present with headache, seizures, cranial nerve palsies, motor signs, hypothalamic dysfunction, and hydrocephalus. Early onset sarcoidosis (<4yo) → skin rash, uveitis, arthritis, absence of lung disease

Answer 479

Often normal | Advanced = restriction with low DLCO

Answer 480

Specific changes of waxy yellow mucosal nodules and nonspecific changes → erythema, edema, granularity and cobblestoning of the airway mucosa and bronchial stenosis (typically in the lobar and segmental bronchi). BAL: lymphocytosis in >85%, normal or low neutrophils (except in late disease) and CD4:CD8 is increased

Answer 481

Typical clinical features are supported by a tissue biopsy showing noncaseating granulomas

Answer 482

The clinical course is chronic or progressive in 10%–30%, and fatalities occur in 1%–5% of patients, typically due to progressive pulmonary fibrosis or central nervous system or cardiac involvement Pulmonary involvement may lead to progressive pulmonary fibrosis and end stage chronic lung disease; this seems to be more likely with stage III pulmonary disease at the onset in both adults and children.

Answer 483

A horizontal groove along the lower border of the thorax corresponding to the costal insertion of the diaphragm. It reflects increased work of breath against airflow resistance Ex: Asthma, BO Also seen in rickets as soft ribs drawn in by diaphragm under tension (Tends to be more common infancy when the chest wall is softer)

Answer 484

Hemoglobin converted from ferrous(Fe2+) to ferric state (Fe 3+) by oxidation by MetHb. The presence of ferric heme molecules causes a structural change in the hemoglobin molecule, resulting in reduced oxygen-carrying capacity and impaired unloading of oxygen at the tissue. This left shift in the oxygen saturation curve results in functional anemia. The low saturation is due to the waveband of the saturation monitor. Pulse oximetry can only measure two frequencies, oxyhemoglobin and reduced Hb. As MEtHb rises the saturation falls and plateaus at 85%. Despite normal Arterial PO2 - saturation gap

Answer 485

Arterial Blood is chocolate brown colour Measure blood level of MetHb (blood gas) Evelyn Malloy method Co-oximeter-->gives a print out of various hemoglobin

Answer 486

Micrognathia Big tongue posterior, glossoptosis Airway compromise +/-Cleft palate

Answer 487

Airway obstruction: ONO, Cap Gas, PSG Feeding difficulties: OT assessment, formal feeding study Airway Obstruction Non-Surgical: Lateral or prone positioning. Nasopharyngeal airway CPAP/BIPAP Surgical: Tongue-Lip adhesion to increase oropharyngeal gap Floor of mouth release Mandibular distraction Tracheostomy* Feeding: NG or G tube

Answer 488

Asthma is seen in TEF Patients with a history of EA/TEF have an abnormally high prevalence of bronchial hyperreactivity, suggesting that airway reactivity in these individuals may be due to events in early childhood such as chronic aspiration, rather than atopy Airway malacia Allergy/Atopy more common Aspiration/Reflux Protracted bronchitis - (more prominent in younger children with TEF)

Answer 489

TH1: IL-12 TH1->IFNg, IL-2, TNF-a: Cell mediated immunity TH2: IL 4 TH2->IL-4, IL-5, IL-13: allergy, IgE, eosinophilia, AHR

Answer 490

≥ 93% for 95% of the recording time if continuous oximetry

Answer 491

QS/QT=CcO2-CaO2/CcO2-CvO2 x 100 % Normal = 2-5%

Answer 492

Surfactant protein B deficiency This term is usually used when they present early in newborn or infancy .

Answer 493

Autoimmune PAP (GMCSF receptor antibody)

Answer 494

PAP can also be classified as primary and secondary primary (Alveolar proteinosis is the main abnormality) or secondary to other disorders Primary is Autoimmune PAP (GMSCF receptor antibody) or hereditary PAP (receptor deficiency) Secondary could be due to connective tissue disease , infection, immunodeficiency, malignancy, chemical inhalation

Answer 495

Whole lung lavage for primary PAP GMCSF -inhaled or s/c Immunosupression : steroids/Hydroxychloroquine lung transplant in congenital PAP and acquired PAP

Answer 496

Eventration left diaphragm Volume loss left lung due to hypoplasia or collapse Unilateral Diaphragmatic palsy (many causes are idiopathic if no trauma/surgery ) - eg. due to left mediastinal mass causing phrenic nerve compression Subpulmonic pleural effusion (with diaphragmatic hernia, you shouldn’t be able to actually see the diaphragm) Abdominal mass - eg. splenic

Answer 497

Would reduce vital capacity due to the effects of posture/gravity on static lung volumes plus a paralyzed diaphragm that can lead to ineffective ventilation and paradoxical breathing exaggerating the normal physiologic response.

Answer 498

Birth trauma Cardiac surgery ( PDA ligation, coarctation repair ) Neck surgery Idiopathic (all causes of unilateral can theoretically cause bilateral)

Answer 499

Birth trauma Chiari malformation Idiopathic Hereditary neuropathies

Answer 500

Recurrent laryngeal nerve supplied all muscles of larynx except cricothyroid which is supplied by superior laryngeal nerve Originates from Vagus nerve

Answer 501

Surgical complications: 1- Dehiscence of the airway anastomosis 2- Dehiscence of the vascular anastomosis 3- recurrent laryngeal nerve injury Non surgical 1- Hyperacute rejection 2- Bacterial infection 3- Fungal infection

Answer 502

``` 1-Acute humoral rejection 2- Posttransplant Lymphoproliferative Disease 3- Chronic Lung Allograft Dysfunction 4- Renal dysfunction 5- infection( viral/Bacterial/Fungal) ```

Answer 503

Multiple rib fractures resulting in the destruction of the integrity of the thoracic skeleton can cause paradoxical movement of the “flail chest” The unsupported area of the chest moves inward with inspiration and outward with expiration which leads to dyspnea. The explosive expiration of coughing is dissipated and made ineffective by the paradoxical movement and intercostal pain. This can lead to airway obstruction, atelectasis and pneumonia.

Answer 504

Mechanical positive pressure ventilation. Surgical stabilization in severe flail chest (has shown to lower the incidence of pneumonia, shorten ICU stay, reduce ventilatory requirement, reduce overall hospital cost) Pain control to allow unrestricted respiration. With severe fractures, the alleviation of pain and the restoration of cough are important and can be provided by analgesics

Answer 505

CCHS Genes encoding cardiac ion channels, such as SCN5A, KCNQ1, KCNH2, or RYR2], or proteins involved in myocardial conduction – These account for less than 5 percent of SIDS cases Serotonin transporter gene, or monoamine oxidase A (MAOA) gene, which affects serotonergic and noradrenergic transmission. Fatty acid oxidation defect

Answer 506

Risks to unborn babies: - Stillbirth, and premature birth. - Low birth weight - Increase risk of developing later: leukemia, lymphomas, or brain tumours. Risks to children and infants: - Sudden infant death syndrome (SIDS), - Asthma - Pneumonia - Bronchitis - Ear infection. - Breathlessness and coughing.

Answer 507

HFOV achieves effective gas exchange while avoiding high peak airway pressures and the inflation deflation cycles characteristic of conventional ventilation. Lung volume (and hence oxygenation) is maintained by the application of a high continuous mean airway pressure. CO2 removal is achieved despite small tidal volumes (2–4 mL/ kg) by imposing a breath frequency of 300–900 (5–15 Hz) per minute, resulting in large minute volumes. Therefore HFOV involves the application of the open lung strategy accompanied by small swings in pressure and volume in the distal airways and alveoli.

Answer 508

1. Decrease Frequency of Oscillations; can help with minute ventilation and decrease CO2 2. Increase amplitude of oscillations- DELTA P or power ; can help with minute ventilation and decrease CO2 3. Increase I:E ratio

Answer 509

Haller index CT chest - AP: transverse diameter (measured at deepest point of deformity) The Haller index is calculated by dividing the transverse diameter of the chest by the anterior-posterior distance on CT of the chest on the axial slice that demonstrates the smallest distance between the anterior surface of the vertebral body and the posterior surface of the sternum.

Answer 510

``` Aspergillus S. aureus Burkholderia cepacia Nocardia Serratia ```

Answer 511

1. Patient and clinician preference 2. Patient cannot successfully use NIV 3. Inability of local infrastructure to support NIV 4. 3 extubation failures during critical illness despite optimal use of NIV and cough assist 5. Failure of cough assist to prevent aspiration and drops in SpO2<95% requiring frequent suctioning

Answer 512

Step 1: Volume recruitment/Deep lung inflation technique Step 2: Manual and Mechanically Assisted Cough techniques Step 3: Nocturnal ventilation Step 4: Daytime ventilation Step 5: Tracheostomy

Answer 513

1. Reversibility of acute deterioration and/or lung transplant eligibility (Options for bridge to transplant) 2. Patient/family wishes, goals of care

Answer 514

Recommend caution if considering adopting a dog (hypoallergenic or otherwise), as dog protein is still present which could worsen asthma symptoms (principle of identifying and eliminating exacerbating or aggravating factors). (Be cautious)

Answer 515

Positive (histamine) and negative (saline) control tests should be included to detect inherent skin factors that may affect the reaction to allergen, such as dermatographism and extensive dryness or eczema.

Answer 516

Ensure appropriate mask and headgear fit to avoid pain, skin breakdown, mid-face hypoplasia and mask leak, which can lead to asynchrony Come up with a desensitization plan Adjust trigger sensitivity if unable to trigger (increase trigger sensitivity) or if there is autocycling (decrease trigger sensitivity) Adjust rate Consider adjusting the pressure by 1-2 units, as he becomes desensitized Adjust humidity setting-->maybe too much or too little humidity Consider a ramp

Answer 517

Anaerobic threshold = when you switch from predominantly aerobic to anaerobic metabolism-->lactate production exceeds removal-->high CO2 production as your try and buffer presence of acid-->big increase in ventilation-->VCO2 production increases relative to oxygen consumption (VO2) ATS CPET statement: use a combination of V slope and ventilatory equivalence (PETCO2, PETO2): since abnormal ventilation pattern and acute hyperventilation can invalidate the noninvasive measurements of anaerobic threshold. Method 1: V-slope method - VCO2 increases relative to VO2 -> there's a huge amount of CO2 production since you've switched to anaerobic metabolism, low oxygen consumption size you aren't using as much aerobic metabolism Method 2: PETO2 and PETCO2 versus workload - loos for where the lines separate

Answer 518

PCV 13 (prevnar) versus pneumococcal polysaccharide 23 vaccine (pneumovax): - They are both polysaccharide vaccines, but prevnar has a conjugated protein to stimulate response in infant (<2 years of age). Infants can’t mount an immune response to pure polysaccharide vaccine - Prevnar is part of routine immunization schedule - Pneumovax is additional pneumococcal coverage, indicated for individuals with chronic respiratory disease, above 2 years of age Three differences for PCV 13 versus Pneumovax 23: <2 years Conjugated Less strains

Answer 519

Both have polysaccharide antigens of the bacteria, but in the conjugate vaccine, the polysaccharide antigen is attached to a protein Young infants cannot mount an immune response to just the polysaccharide antigen, so the protein is required

Answer 520

Cases are most frequently described within reasonably well-defined populations, such as: - Children - Military recruits - Incarcerated people - Men who have sex with men - Sports teams - Native populations Common risk factors that have been identified in CA-MRSA outbreaks include: - Overcrowding - Frequent skin-to-skin contact between people - Participation in activities that result in abraded or compromised skin surfaces - Sharing of potentially contaminated personal items - Challenges in maintaining personal cleanliness - Hygiene - Limited access to health care

Answer 521

Calcineurin inhibitors (Tacrolimus or Cyclosporine) Cell cycle inhibitors (Azathioprine or MMF) Steroids Induction immunotherapy → polyclonal agent (antilymphocyte or antithrombocyte) IL-2 receptor antagonist (daclizumab or basiliximab)

Answer 522

Most common malignancy post transplant EBV-driven lymphoma Most common site in the first post transplant year = allograft Typical symptoms = cough, fever, dyspnea (all B symptoms as well) CXR: single or multiple round or ovoid pulmonary nodules After the first year, incidence of extra pulmonary PTLD increases Other sites = GI, skin, lymphatic tissue (lymph nodes, nasopharynx) EBV PCR: S/S marker for PTLD Tx (early): reduction in immunosuppression Also: rituximab (anti-CD20 monoclonal antibody), low dose cyclophosphamide and prednisone

Answer 523

New EBV PCR positive -->very important to include EBV in an answer since this would be the most specific feature Persistently low lung function Radiological findings

Answer 524

Radiation pneumonitis Pulmonary fibrosis Interstitial pneumonitis

Answer 525

``` Dosimetric factors: Measurement, calculation and assessment of the ionizing radiation dose absorbed by the human body Treatment factors (radiation dose, chemo) → Radiation >10 Gy Patient factors (female, smoking, age) → Younger age at irradiation Medical history: including atopy ```

Answer 526

``` Aspergillus species S. aureus Burkholderia (Pseudomonas) cepacia complex (Serratia marcescens) (Nocardia species) ``` The organisms that infect patients with CGD are catalase producing. Catalase is an enzyme that inactivates the hydrogen peroxide normally produced by some bacteria and fungi during growth. Although most microorganisms produce hydrogen peroxide, some do not. It was thought that CGD phagocytes could use the hydrogen peroxide produced by catalase-negative microbes to generate reactive oxidants, thereby bypassing the intrinsic CGD defect.

Answer 527

Nephrotoxicity: acute increase in plasma creatinine Hypertension, which is related to nephrotoxicity Neurotoxicity: mild tremor. Severe headache, visual abnormality and seizure with posterior leukoencephalopathy on brain imaging, pain syndrome Glucose intolerance and diabetes Hyperlipidemia Risk of infection such as bacterial, viral (eg. CMV), fungal Risk of malignancy — Both cyclosporine and tacrolimus are associated with an increased risk of squamous cell skin cancer and benign or malignant lymphoproliferative disorders GI: anorexia, nausea, vomiting, diarrhea Cyclosporine: hirsutism and gingival hyperplasia Additional: TTP/HUS

Answer 528

Diarrhea – most common S/E | Bone marrow suppression with cytopenia (eg. Leukopenia). Regular CBC (at least every 6-8 weeks)

Answer 529

Atelectasis-->low ventilation, but ongoing perfusion-->shunt, so venous admixture, but effect on saturation would depend on shunt fraction. As well, hypoxemia in acute respiratory disease is due to VQ mismatch, but there are compensatory mechanisms like hypoxic pulmonary vasoconstriction

Answer 530

Oximeter has 2 different wavelengths of light (660 nm and 940 nm) Oxygenated Hb absorbed light at 660 nm Normal Hb (without oxygen) absorbs light at 940 nm Oximeter calculates HbO2/Hb + HbO2 The oximeter measurements are timed with arterial pulse so you get an arterial oxygen saturation Limitations of pulse oximetry: Does NOT detect COHb or methemologlobin or dyshemoglobin Perfusion Technical issues: skin pigmentation, nail polish, motion artifact

Answer 531

PaO2 normal SaO2 - normal (since on the ABG this is calculated from PaO2) SpO2 - abnormal (often 85%) - this is the one measured from saturation Methemoglobin on blood gas is the key way of detecting

Answer 532

Shunt In TAPVR, the heart has no direct pulmonary venous connection into the left atrium. The pulmonary veins may drain above the diaphragm into the right atrium directly, into the coronary sinus, or into the superior vena cava via a “vertical vein,” or they may drain below the diaphragm and join into a “descending vein” that enters into the inferior vena cava or one of its major tributaries, often via the ductus venosus. All forms of TAPVR involve mixing of oxygenated and deoxygenated blood before or at the level of the right atrium (total mixing lesion). This mixed right atrial blood either passes into the right ventricle and pulmonary artery or passes through an atrial septal defect (ASD) or patent foramen ovale into the left atrium, which will be the only source of systemic blood flow.

Answer 533

``` Posterior cricoarytenoid (abduction) Lateral cricoarytenoid (add) Transverse arytenoid (add) ```

Answer 534

Rectus abdominis External and internal oblique muscles Transversus abdominis Internal intercostal muscles.

Answer 535

- Inappropriate treatment - Drug resistance - New bugs - Non-infectious: - ABPA - CF related diabetes - Poor nutrition

Answer 536

- Sputum sample with antibiotic susceptibility testing - CF bacteria, AFB smear and culture (at least 2 samples to actually diagnose NTM), fungal. - Fasting glucose and 2 hour post prandial glucose x 48 hours in hospital - IgE, IgE to aspergillus - Bronchoscopy - CXR, CT scan

Answer 537

Clinical (both are required): - Pulmonary symptoms, nodular or cavitary opacities on CXR or CT showing multifocal bronchiectasis with multiple small nodules - Appropriate exclusion of other diagnoses Micro criteria (need one) - Positive culture results from at least 2 separate expectorated sputum samples - Postive culture results from at least one bronchial wash or lavage - Transbronchial or other lung bx with mycobacterial histopath features and postive cx for NTM

Answer 538

If MAC that is macrolide susceptible with NO cavitation (non-cavitary): macrolide (ideally azithromycin), rifampin, ethambutol (MAC ARE) Azithro is the preferred macrolide because: Less interactions with CYP3A enzyme system, which is also affected by rifampin Immunomodulatory benefits of azithro in patients with CF Treatment in CF patient with macrolide resistant MAC, systemically ill, AFB smear positive, or cavitary lesion on imaging: 1-3 months of amikacin at the start of the treatment course, along with standard 3 oral antibiotics Duration of therapy — Treatment should be continued until sputum cultures are consecutively negative for at least 12 months.

Answer 539

``` Nodules Tree-in-bud opacities Cavities Bronchiectasis Consolidation ```

Answer 540

Annual sputum culture or BAL for NTM, not a swab sample

Answer 541

Acute infection – TB, viral, bacterial, atypical, fungal, parasites Protracted bacterial bronchitis ``` Bronchiectasis: o Immunodeficiency o Primary ciliary dyskinesia o Cystic fibrosis o Aspiration o ABPA ```

Answer 542

``` Immunoglobulins – IgG, A, M, E. Vaccine titres – IgG for diptheria, tetanus, HiB; HIV CBC Sweat chloride Sputum culture PFTs (complete + bronchodilator) CXR Mantoux ```

Answer 543

Low nasal nitric (<77 nL/min) is quite consistent in PCD, though other things can also result in low nasal nitric oxide (viral illness, sinusitis, cystic fibrosis) Low nasal NO is relatively sensitive (90% range) and specific (90% range) for PCD Recommendation: in a child >=5 years of age with a clinical phenotype of PCD and CF excluded, low NO on 2 separate occasions can make a presumptive diagnosis of PCD

Answer 544

Ultrastructural (most common = dynein arm defect) Other U/S = radial spoke defects, microtubular transposition defects Functional

Answer 545

Pulmonary: - Regular airway clearance - Consider antibiotics during pulmonary exacerbations, regular sputum cultures - Ensure appropriate nutrition and growth - Immunizations-->influenza, routine, pneumovax - Avoid smoking - ENT: audiology assessment, ?hearing aids, referral to ENT physician - Genetic testing and counselling - Sibling screening

Answer 546

History: Daytime symptoms <4 days per week (daytime symptoms >3 days per week) No night time symptoms SABA doses <4 doses per week (>3 doses per week) No physical activity limitation Not missing any work or school Mild, infrequent exacerbations Objective testing: FEV1>=90% of personal best Diurnal variation of PEF <10-15% (but CTS didn’t recommend use of diurnal variation for diagnosis in children, so this would be more so for >=12years) Sputum eosinophils <2-3% (adults)

Answer 547

- Retrospective assessment over the last 4 weeks - Daytime symptoms > 2 days per week (3 days or greater is too many days) - Any night time symptoms - Reliever use >2 times per week (not including pre-exercise) - any activity limitation due to asthma (since this will decrease the probability of having symptoms) - Based on the above, patients can be classified as well controlled, partly controlled or not controlled

Answer 548

1. Explain pathophysiology at a level appropriate to developmental status of child 2. Demonstrate technique for use devices like MDI or turbohaler 3. Provide a written action plan, with additional instructions for yellow zone or red zone 4. Discussion about environmental triggers like pets and smoking 5. Ideally education provided in patient’s native language 6. Ability to monitor adherence such as with pharmacy data

Answer 549

Non-adherence Poor technique Having a severe exacerbation, defined as need for oral steroid, in the last year Prior ICU admission Significant BD response while on treatment Low lung function, FEV1<60% Ongoing exposure to triggers like smoking

Answer 550

- Poor asthma control - Low lung function (FEV1<60%) - High BD reversibility - >= 1 severe exacerbation in the last 12 months (as per CTS, severe exacerbation is where oral steroids are needed) - Ever being intubated or in ICU for asthma - high blood eosinophils - elevated FeNO - comorbiities: GERD, food allergy, pregnancy

Answer 551

1. Seek confirmation for the diagnosis of asthma-->review of prior spirometries, methacholine challenge 2. Differentiate between severe asthma versus uncontrolled asthma and explore adherence, medication technique and triggers ``` 3. Ensure no red flags suggesting an alternate primary diagnosis or significant co-morbidities: o Bronchiectasis—CF, PCD o Aspiration o Immunodeficiency o Foreign body o ABPA o Tracheobronchomalacia o Obesity o Co-morbidities: GERD, rhinitis, VCD (inducible laryngeal obstruction) ``` 4. If severe asthma, then consider: o Treat co-morbidities like GERD, rhinitis o Tiotropium o Symbicort maintenance and reliever therapy o Biologic – eg. Xolair

Answer 552

o IgE is in target range (30-1300 for 6-11 years, 30-700 for >=12 years) o Evidence of sensitization either via in vitro or skin testing to an aeroallergen Omalizumab is dosed every 2-4 weeks, depending on IgE and patient weight

Answer 553

o. Injection site reaction o No association with lymphoma has been found o Risk of anaphylaxis – 0.09%, more so after first 3 doses and within first 2 hours. o All doses are given subcutaneously in a monitored environment Patients need to be monitored for 2 hours after first 2 doses and for ½ hour after subsequent doses

Answer 554

- Surfactant dysfunction mutation: surfactant protein B, ABCA 3, NKX2.1, pulmonary alveolar proteinosis - Diffuse developmental disorder such as ACD-MPV - Growth abnormalities such as pulmonary hypoplasia +/- T21, associated with congenital heart disease, filamin A mutation - Condition of undefined etiology: PIG, NEHI - Immunodeficiency – PJP - Infection – eg. GBS sepsis, RSV, TB

Answer 555

- Genetics: surfactant protein B, C, ABCA 3, NKX2.1, FOXF1 - Tracheal aspirate for infectious pathogens such as Chlamydia trachomatis, pertussis, viral, bacterial - CT chest with contrast

Answer 556

``` Microbiology Histopathology Immunohistochemistry Immunofluorescence EM ```

Answer 557

AEC2 hyperplasia Interstitial fibrosis Alveolar proteinosis Large and loosely packed lamellar bodies with vacuolar inclusions

Answer 558

``` Oxygenation Antimicrobials: broad spectrum abx (including macrolide) Blood transfusion/exchange transfusion Fluids Bronchodilators Incentive spirometry Analgesia ```

Answer 559

Regular evaluation for cardiorespiratory symptoms PH: screening echo at age 3 years, repeated every 5 years and more often if symptomatic Asthma and OSA: only investigate if there are symptoms Consider hydroxyurea Vaccines: Amoxicillin PFT and lung volumes annually starting at age 6 (Spiro with every visit if they have asthma) Lung volumes q5 years in children with no asthma or ACS episodes Bronchodilator challenge should be done in those with obstruction on PFT

Answer 560

Interstitial lung diseases Neuromuscular weakness Chest wall disease

Answer 561

Low Forced vital capacity with normal FEV1:FVC ratio, Low TLC , normal to increased RV/TLC ratio ,since TLC is more reduced than RV in a generalized neuromuscular disease Also low MIP and MEP and normal DLCO/VA

Answer 562

Deflazacort (steroid)- there is no consensus on age of starting according to AAN practice guidelines 2016; Most of the studies started it between 5-15 years average 8 years ref: ATS DMD guidelines At the time of motor skills plateau ~5-8 years

Answer 563

Steroids help by anti inflammatory effect and delayed depletion of muscle progenitor cells Improved muscle strength and lung function delays cardiomyopathy and scoliosis Side effects- cataract, bone demineralisation and fractures , excess weight gain, behavioural changes

Answer 564

When there is loss of ambulation

Answer 565

Around 10-12 years after which it declines

Answer 566

Ambulatory yearly, early non ambulatory 6 monthly FVC, MIP, MEP , Peak cough flow , sats PCO2 in clinic visit , Either PSG or overnight oxymetry with end tidal or tCO2 annually when FVC <60%

Answer 567

Respiratory muscle weakness | Scoliosis

Answer 568

Pneumococcal and influenza vaccine to prevent respiratory infections Start Lung volume recruitment when FVC < 40%- <60% in the 2018 update Start manually assisted cough or MIE device when FVC <40 (<50% in 2018 update) or Peak Cough Flow <270 with respiratory infections (<160 when well) or MEP<40 (<60 in 2018 update) Start Nocturnal BIPAP Symptoms or signs of hypoventilation when awake sats <95 , PCO2 >45 An apnea-hypnea index >10/h on polysomnography OR four or more episodes of SpO2< 92% OR drops in SpO2 of at least 4%/ h of sleep 2018 update - PSG- petCO2 or ptcCO2 of more than 50 mm Hg for at least 2% of sleep time, a sleep-related increase in petCO2 or ptcCO2 of 10 mm Hg above the awake baseline for at least 2% of sleep time, an SpO2 of 88% or less for at least 2% of sleep time or for at least 5 min continuously, or AHI >5 Also FVC <50% and MIP <60% IN THE 2018 UPDATE Start day time mouthpiece ventilation when patient self extends into daytime NIV or is dyspneic or Nocturnal NIV not enough to keep day time sats and CO2 normal or cannot speak a sentence without breathlessness

Answer 569

Failed NIV Cannot successfully use NIV No infrastructure for NIV Patient and clinician preference 3 failed extubation after needing acute ventilation Failed non invasive methods of cough assist

Answer 570

Restriction - cobb >50 Nocturnal hypovent- cobb>60 Cardioresp failure - cobb >90 In DMD, >20 cobb angle and FVC 40% is indication for surgical management since bracing is not effective in these patients

Answer 571

Surgery doesn't improve FVC , in fact there is evidence to indicate it might decrease FVC temporarily The decrease in vital capacity usually lasts for 6 weeks to 3 months after fusion but can persist for up to 1 year

Answer 572

Individuals with DMD should receive yearly immunization with the inactivated influenza vaccine (ie, the injectable vaccine, not the live, attenuated nasal vaccine) and pneumococcal vaccines (including PCV13 and PPSV23), according to guidelines available from the US Centers for Disease Control and Prevention

Answer 573

Nocturnal BIPAP - Initial settings- S/T mode , IPAP 8/4 (min delta 4) to get chest rise and adequate tidal volume, EPAP minimum 4 BUR just below baseline (2-4 below)

Answer 574

24/7 non invasive ventilation - Start day time Mouthpiece ventilation in addition to night time BIPAP Trach and 24/7 vent are option based on patient preference and effectiveness of MIE in clearing secretions

Answer 575

Decreased respiratory muscle strength with progression | Scoliosis causing chest wall restriction

Answer 576

``` FVC, MIP, MEP, PCF Overnight oximetry, PTCO2/TCO2 Echo PSG Optimize respiratory care by making sure you have LVR, cough assist or NIV as indicated by stage of the disease before the surgery CXR ```

Answer 577

Try to insert OG tube and Get Chest Xray with OG tube in situ

Answer 578

Type A Esophageal atresia alone Type C- Esophageal atresia with proximal eso pouch and distal fistula (most common 84%) Type B- Proximal TE fistula with distal esophageal atresia Type D - TEF with distal connection Type E: H-type fistula (no EA)

Answer 579

Embryonic stage

Answer 580

2/3rd have associated anomalies VACTERL (Vertebral anomaly, anorectal malformation, TE fistula, cardiac , renal and limb anomaly CHARGE (coloboma, heart defects, atresia choanae, retarded growth, genital and ear abnormalities ) Potter syndrome ,Pierre Robin, Di George

Answer 581

Immediately after birth with Increased secretions, choking and respiratory distress and inability to feed if there is esophageal atresia (95% of the time) Those without esophageal atresia present later with recurrent pneumonia and failure to thrive

Answer 582

Surgical ligation of the fistula with anastomosis of the esophageal segments Reanastomosis might not be possible if the esophageal remnants are far apart in which case a staged approach might be needed.

Answer 583

``` Recurrent aspiration Tracheomalacia Recurrence of fistula Esophageal stenosis GERD Asthma and wheeze ```

Answer 584

``` Obstructed TAPVC Congenital Diaphragmatic Hernia Alveolocapillary dysplasia with MPV Sepsis and PPHN Surfactant protein B deficiency with pulmonary hypertension MAS (meconium aspiration) ```

Answer 585

Chest Xray Echo to look at cardiac anatomy and assess pulmonary hypertension Ultrasound thorax and abdomen might be needed if there is doubt CT abdomen might be needed in some cases preoperatively

Answer 586

Bochdalek (posterior)-95% and Morgagni (anterior) Bochdalek- posterolateral herniation, Most common, presents earlier and is more severe Morgagni- Anterior, presents later and milder Eighty-six percent of Bochdalek defects are left-sided, 13% right-sided, and up to 2% bilateral

Answer 587

Surgical repair(closing defect and reducing abdominal viscera back to abdomen) - after medically stabilizing and controlling pulmonary hypertension In patients without pulmonary hypertension and who are medically stable, repair is done at 48-72 hours of life

Answer 588

``` Lung hypoplasia Pulmonary hypertension Neurodevelopmental delay Recurrence of diaphragmatic hernia Chronic lung disease GERD Chest wall deformities Hearing loss Long term ventilator dependence ```

Answer 589

Observed/expected LHR -ratio of the lung area divided by the head circumference, compared to a reference value for that gestational age Best survival predictor = Lung Head Ratio value >1.0

Answer 590

Good- Isolated left-sided hernias, an intra abdominal stomach, and diagnosis after 24 weeks are favorable prognostic factors Bad- Syndromic CDH, Prematurity, right CDH, bilateral hernia, associated major cardiac anomalies, presence of liver in thorax

Answer 591

Pulmonary hypertension

Answer 592

Obstruction (Low FEV1 FVC FEV1/FVC ratio and increased RV/TLC)

Answer 593

Intrauterine occlusion of trachea with a balloon , so that lung fluid stays and promotes lung growth Tracheal occlusion at 24-28 weeks gestation for severe and 30 weeks for moderate CDH, leads to accumulation of lung fluid which in turn causes lung stretch. This activates a pathway that leads to proliferation and increased growth of the airways and pulmonary vessels, nicely summarized in the acronym PLUG: “Plug the Lung Until it Grows” Now a plug-unplug sequence,” and reversal of occlusion around 34 weeks is an important component in the fetal treatment strategy.

Answer 594

Congenital , Idiopathic - (infantile , juvenile and adolescent) Scoliosis associated with neuromuscular disease Most common is the idiopathic scoliosis (85%)

Answer 595

2-3% (Cobb angle more than 10 = scoliosis) | Lower prevalent with higher Cobb angles ( 0.5% cobb more than 20 and 0.2% cobb more than 30)

Answer 596

Cobb angle Identify the vertebra whose endplates are most tilted towards each other- Lines are then drawn along the endplates, and the angle between the two lines, where they intersect, measured.

Answer 597

Restrictive lung disease and pulmonary hypertension | Cardiopulmonary insufficiency

Answer 598

Restriction - cobb >50 Nocturnal hypovent- cobb>60 Cardioresp failure - cobb >90

Answer 599

Cobb angle Presence of associated neuromuscular problems Age of onset (lung hypoplasia)

Answer 600

Decreased FVC,normal ratio , TLC and normal or increased RV/TLC since TLC is more decreased than RV You can also see obstruction if there is obstruction of the bronchus due to distortion Most sensitive marker of reduced thoracic cage mobility is FVC

Answer 601

Predicted values based on arm span instead of standing height , can also use height predicted based on ulnar length if arm span can't be used

Answer 602

Bracing is mainly used in idiopathic scoliosis Bracing - only if Cobb angle is more than 25 - 50 and non progressive Surgical when cobb angle is more than 50 or progress despite bracing in idiopathic scoliosis - growth friendly distraction or spinal fusion The surgical threshold will also depend on the category of scoliosis whether it is idiopathic or neuromuscular and type of surgery on whether the spine growth is complete or not If the 14 year old has DMD - surgery is considered if cobb >20 and FVC <40%

Answer 603

Cobb Less than 20 and non progressive , wait and watch Surgery- Growth friendly distraction technique before bone growth is complete and spinal fusion after spine growth is complete In congenital scoliosis - serial casting until 5 years followed by growth friendly distraction surgery In idiopathic scoliosis - bracing between 20-50 - surgery choice based on spine growth In neuromuscular disease - bracing avoided, surgery when Cobb >20 and FVC <40%

Answer 604

Assuming that it is juvenile idiopathic scoliosis answer is bracing Bracing is advised if >20 or progressive Indication for growth friendly distraction is more than 40 at time of diagnosis or progressive despite bracing in juvenile scoliosis ; If cobb less than 20; wait and watch In adolescent idiopathic scoliosis the threshold is 25 for bracing ; more than 50 for spinal fusion In congenital scoliosis - serial casting up to 5 years of age followed by distraction devices are used as they do not respond to bracing If underlying neuromuscular disease- treatment is growth friendly distraction

Answer 605

Chest Xray PFT Echo

Answer 606

``` Bleeding Fluid shifts causing pulmonary edema Pneumonia Atelectasis Multifactorial respiratory failure related to pain, sedation, analgesia, restriction, fat embolism etc ```

Answer 607

Moderate BPD = At 36 weeks needing FiO2 <30%

Answer 608

Endogenous risk factors for BPD: - gestational immaturity - lower birth weight - male sex - white/non-black race - family history of asthma - SGA Prenatal risk factors for BPD: - maternal smoking - Pre-eclampsia - placental abnormalities - Chorioamnionitis - IUGR - no maternal steroids - perinatal asphyxia Post natal risk factors for BPD: - lower Apgars - RDS - PDA - higher weight -adjusted fluid intake - earlier use of IV lipid - light exposed TPN - duration of O2 therapy

Answer 609

``` Duration and approach of mechanical ventilation (increased O2, PIP, rate decreased PEEP) Hypocarbia Colonization with ureaplasma urealyticum Post-natal CMV Post-natal sepsis ```

Answer 610

Features of "old" BPD (6) 1) alternating atelectasis with hyperinflation 2) severe airway epithelial lesions 3) marked airway smooth muscle hyperplasia 4) extensive diffuse fibroproliferation 5) hypertensive remodeling of pulmonary arteries 6) decreased alveolarization and overall surface area Features of "new" BPD (6) 1) less regional heterogeneity 2) rare airway epithelial lesions 3) mild airway smooth muscle thickening 4) rare fibroproliferative changes 5) fewer arteries but "dysmorphic" 6) fewer, larger, and simplified alveoli

Answer 611

``` High PIP, high volumes Hypocarbia High oxygen or duration of oxygen Co-morbidities: PPHN, PDA, IVH, prolonged ventilation, sepsis Invasive Ventilation ```

Answer 612

Obstruction | Airway hyperreactivity

Answer 613

Compliance decreases in BPD due to less surfactant thus increased surface tension Dynamic lung compliance is markedly reduced in infants with established BPD, even in those who no longer require oxygen therapy. The reduction in compliance is due to small airway narrowing, interstitial fibrosis, edema, and atelectasis. Established BPD is primarily characterized by reduced surface area and heterogeneous lung units in which regional variations in airway resistance and tissue compliance lead to highly variable time constants throughout the lung.

Answer 614

Increased muscularization of smooth muscle leads to increased pulmonary vascular resistance Decreased compliance

Answer 615

``` Once there is BPD established: Longer I time Large tidal volume High PEEP Lower rate ``` ``` For RDS (want to prevent BPD): Lower PIP Higher PEEP Increased RR with lower tidal volumes Tolerating higher CO2 ```

Answer 616

``` Oxygen Non-invasive ventilation RSV immunization Trach and vent (sajith will do this) Nutrition ```

Answer 617

BTS = ≥ 93%

Answer 618

Majority are off by 2 years. | PFT could show some obstruction.

Answer 619

It refers to the abnormal location of gas within the pulmonary interstitium and lymphatics usually due to positive pressure ventilation. It typically results from rupture of overdistended alveoli following barotrauma in infants with respiratory distress syndrome.

Answer 620

Antenatal steroids are used to help increase lung maturation in premature babies. It has been shown to decrease the morbidity/mortality of BPD but not necessarily the incidence. The use of antenatal steroids in mothers at high risk of delivering a premature infant has reduced the incidence of neonatal death and RDS by 50% but failed to decrease the incidence of BPD, even in combination with postnatal surfactant

Answer 621

Exogenous surfactant is used in premature babies to decrease the surface tension and increase compliance. It has been shown to decrease mortality from BPD but not the incidence. Surfactant reduces the combined outcome of death and BPD but not BPD alone

Answer 622

No evidence for inhaled steroids iNO is a pulmonary vasodilator and has shown some effects in BPD. In clinical studies, inhaled nitric oxide (iNO) therapy acutely lowered PVR and improved oxygenation in patients with PH in various settings, including premature infants with severe RDS and established BPD. Experimental data have demonstrated that iNO therapy may be lung-protective in several animal models of experimental BPD and, importantly, may be associated with increased alveolar and vascular growth Caffeine has been shown to help with decreasing airway resistance and postulated to have an additive effect to diuretics but does have undesirable side effects. More evidence for helping with the prevention of BPD.

Answer 623

Benefit: Has shown to help those with BPD successfully extubate (DART protocol) but does not have any effect with reducing the overall incidence or morbidity of BPD. Some evidence as well for short pulses for use with acute lung deterioration. Acute treatment with steroids improves lung mechanics and gas exchange and reduces inflammatory cells and mediators in tracheal samples of patients with BPD Harm is substantial and would include: HTN, osteoporosis, increased risk of IVH, poor head growth, neurodevelopmental concerns Other side effects include systemic hypertension, hyperglycemia, cardiac hypertrophy, intestinal perforation, poor somatic growth, sepsis, intestinal bleeding, and myocardial hypertrophy. Currently the routine use of early high-dose steroids in premature newborns is strongly discouraged CPS: Avoid early steroids, DART after day 7

Answer 624

``` Infectious (bacterial most likely, viral, fungal) pneumonia Underlying congenital lung lesion Foreign body Abscess - lower on differential Aspiration pneumonitis/pneumonia TB/NTM Atelectasis - lower on differential ```

Answer 625

Underdeveloped pores of Kohn and Lambert's channels that may cause the centrifugal spread of fluid or bacteria Mostly children <8

Answer 626

``` S. pneumoniae S. Aureus H. Influenzae Mycoplasma (M. pneumoniae) Chlamydia pneumoniae Moraxella Viral ```

Answer 627

1-3 mos: GBS, E.coli, Chlamydia trachomatis | Up to 1 yr: S. pneumoniae, S. aureus

Answer 628

``` Adenovirus Influenza RSV Human metapneumovirus Enterorhinovirus ```

Answer 629

S. pneumoniae - PO Amoxicillin or IV Ampicillin if ++ unwell

Answer 630

Air fluid level = Abscess | Staph aureus, S. pneumonia, Pseudomonas

Answer 631

CT chest with contrast

Answer 632

IV antibiotics: Ampicillin-sulbactam, Cephalosporin with Clinda x 4-6 weeks

Answer 633

BP fistula Empyema Pyothorax Pneumothorax

Answer 634

U/S Chest tube with thoracentesis Consider TPA if clinically indicated and no BP fistula

Answer 635

3 criteria: Pleural LDH/Serum LDH >0.6 Pleural protein/Serum Protein >0.5 Pleural LDH > ⅔ upper limit of normal for serum LDH 1 criteria = exudate ``` Exudates = Infectious, Chylothorax Transudates = CHF, cirrhosis, renal ```

Answer 636

``` LDH Protein Glucose pH Cell count Culture/gram stain TB ```

Answer 637

90% by 6 mos, nearly all by 12 mos (empyema as per Kendig)

Answer 638

``` Hepatotoxicity Neuropathy Retinopathy Bodily fluids may turn orange Supplementation with pyridoxine (with isoniazid) certain populations ```

Answer 639

1) BCG vaccination was given in infancy, and the person tested is now aged 10 years or older; 2) There is a high probability of TB infection: close contacts of an infectious TB case, Aboriginal Canadians from a high-risk community or immigrants/visitors from a country with high TB incidence; 3) There is high risk of progression from TB infection to disease.

Answer 640

``` Aggammaglobulinemia CVID CGD Hyper IGE syndrome IgA deficiency ```

Answer 641

Top list: viral, PJP, LIP ``` Viral; RSV, influenza, parainfluenza, human metapneumovirus, and adenovirus. Influenza, Measles and varicella Bacterial(25%)infection; strep, Staph, H Infl, E coli, Salmonella Opportunistic infection PJP (4%) Fungal infection TB(10%) CMV Co-infection NTM: MAC uncommon Chronic lung disease LIP as persistent. Kaposi sarcoma ```

Answer 642

Oxygen High dose Septra for 21 days step down if improvement to oral As Hypoxic Prednisone 5 day then taper over 10-14 days

Answer 643

Confirm diagnosis, Immunoglobulins are usually elevated Lung biopsy Bronchodilators O2 Steroids oral

Answer 644

Malignant schwannoma Tumors of sympathetic origin - Neuroblastoma - Ganglioneuroma - Ganglioneuroblastoma - Pheochromocytoma Chemodectoma Possibly: Partial right sided Eventration Right sided diaphragmatic hernia (possibly to posterior) CPAM Sequestration Esophageal Duplication cyst (foregut cyst)

Answer 645

``` Malignant lymphoma (Hodgkin and non-Hodgkin) - most common Teratoma - common Thymus (benign enlargement) - common Thyroid (retro- or sub-sternal extension) Thymic cyst Angioma Langerhans cell histiocytosis Mesenchymal tumors Lymphangioma (cystic hygroma) ```

Answer 646

Foregut duplication cyst (bronchogenic, enteric, and neuroenteric) - most common; bronchogenic cyst is more common Lymphadenopathy Vascular malformations

Answer 647

Radiation pneumonitis: Within 30-90 days, steroids, low DLCO, normal lung function, fever chest pain ,dyspnea, hypoxia. Crackles. CXR diffuse hazy opacities and ground glass changes Radiation Fibrosis: 6 months to 24 months. Progressive dyspnea, Irreversible, restrictive defect, low DLCO , chronic. CXR: there may be streaky densities, decreased lung volume or atelectasis, or pleural thickening

Answer 648

Early: - Pulmonary oedema - VOD - Diffuse alveolar hemorrhage - Idiopathic pneumonia syndrome Medium to long term - Idiopathic pneumonia syndrome - Infection - ILD - GVHD - PTLD - Bronchiolitis obliterans Severe BO: Lung transplant

Answer 649

``` Post op atelectasis Pneumothorax Lung infarct ABC, Examination/history/CXR Vitals Airway clearance ```

Answer 650

Analgesia Can get pleuritic chest pain post embolisation (not uncommon) ?lung infarct ?PE CT with contrast, D-dimer, thrombotic screen

Answer 651

Spinal artery thrombosis --paraplegia | Severe chest pain-->analgesia

Answer 652

DLCO is a measure of transfer of Carbon Monoxide (CO) from the gaseous phase into the red blood cells - ease of uptake of oxygen across the alveolar/capillary membrane. Dependent on ventilation and perfusion of the lung. It provides information on the rate at which the oxygen is transferred via passive diffusion from the lungs to the circulation The diffusion across the alveolar-capillary interface can be impaired by both structural and functional changes of the alveoli or the capillaries Can be measured by single breath or steady state. Measured by breathing a known concentration of CO and calculating the absorption. CO is negligible concentration in blood and so allows a measure of true diffusion down its partial pressure gradient between alveoli and capillaries.

Answer 653

V/Q mismatch Lung fibrosis Poor diffusion less effective gas exchange due to abnormal alveolar/capillary membrane. Less time for oxygen to diffuse, transfer of oxygen is diffusion limited

Answer 654

Serum preciptating antigen to bird Inhalational challenge Kill the cockatiel, still may have environmental exposure Steroids

Answer 655

1. Symptoms compatible with HP 2. Evidence of exposure to antigen 3. Radiographic characteristics consistent with HP 4. BAL fluid lymphocytosis (CD8 > CD4) 5. Lung biopsy demonstrates histology consistent with HP 6. Positive natural challenge that produces symptoms and objective abnormalities after reexposure to the offending antigen

Answer 656

Alveolitis, noncaseating granulomas, giant cells, foamy alveolar macrophages or fibrosis

Answer 657

Congenital lobar overinflation (emphysema) Other congenital pulmonary abnormalities (e.g., CPAM) Congenital diaphragmatic hernia (no clear bowel seen in left hemidiaphragm, although gastric bubble not clear) Foreign body (age dependent) Bronchial obstruction (e.g., endobronchial tumor, infection) Filamin A deficiency Contralateral Agenesis

Answer 658

LUL (42%), RML (35%), RUL (21%), lower lobes (2%) Caused by deficiency of bronchial cartilage, occasionally partial obstruction (e.g., mucosal flap or twisting of lobe on pedicle)

Answer 659

Options for Investigations: Bronchoscopy – may reveal causes of intrinsic obstruction and permit removal of foreign body or inspissated secretions Echo – to assess heart and great vessels V/Q – useful if unclear if pathologically distended lobe on one side or congenitally small contralateral lung with secondary physiology expansion Management - dependent on symptoms - Conservative management – if no respiratory compromise - Surgical resection (lobectomy) – if respiratory distress (rarely develops beyond neonatal period) - Ventilation – low pressure, high frequency oscillation to prevent barotrauma to affected lobe and further respiratory compromise to adjacent lung

Answer 660

1) Idiopathic 50% 2) Bronchial cartilage absence, hypoplasia or dysplasia 25% 3) Parenchymal diseases – Polyalveolar lobe – Pulmonary alveolar glycogenosis ``` 4) Internal bronchial obstruction – Bronchial stenosis – Bronchomalacia – Meconium aspiration – Hypertrophic mucosa membranes – Mold mucous plaques – Foreign body aspiration – Bronchial polyp ``` ``` 5) External bronchial obstruction – Pulmonary artery sling anomaly – Pulmonary rotation anomaly – Bronchogenic cyst – Lymphadenopathy – Mediastinal mass – Duplication of esophagus ```

Answer 661

1) Surgical excision (open vs thorascopic) - Preferred if infected or suspected malignancy 2) Embolization - Reduces shunt and risk of high output cardiac failure, - Leaves residual tissue - consider “unquantifiable” risk of malignancy

Answer 662

84% – usually posterior LLL, encircled by visceral pleura, no pleural separation from rest of the lobe

Answer 663

beneath LLL (15% abdominal), male 4x, increased association with malformations

Answer 664

CT appearances variable – solid 49%, cystic 29%, cavitating 12%, pneumonia 8% Most common LLL (66%), posterior basal (20%) Arterial supply – thoracic (77%) or abdominal (19%) aorta, may have at least two arterial supplies Drainage by pulmonary venous system in 90% Nonspecific presentation Most common organisms Aspergillus fumigatus and pseudomonas aeruginosa

Answer 665

Avoidance of IPPV to limit gastric and bowel distention, intubation at birth if respiratory distress present Sedation all mechanically ventilated infants with CDH, deep sedation and NMB selective for greater ventilation needs or O2 requirement. Lung protective ventilation of particular importance given pulmonary hypoplasia associated with CDH. - T piece with ventilator to avoid peak inspiratory pressure >25cm H2O - target pH 7.25-7.40, pCO2 45-60mmHg - supplemental O2 for SpO2>85, but not >95% - initial ventilation mode: gentle, intermittent mandatory ventilation (HFOV if PIP required is >25cmH2O)

Answer 666

Hemodynamic support: -treatment of poor perfusion (judicious crystalloid not >20cc/kg, ionotropic agents -dopamine or epinephrine, hydrocortisone), if persistent assess cardiac function Echo (2 standard) - w/i 48h birth - one at 2-3wk life Management of pulmonary hypertension - iNO for suprasystemic PAH without LV dysfunction. Stop if no clinical/echo response. - Sildenafil if refractory, or when weaning iNO - milrinone for cardiac dysfunction, especially if association PHTN - PGE1 for PDA patency and R afterload reduction if RV failure/closing ductus ECMO - can be considered, should be discussed during prenatal counselling - no survival benefit in currently available evidence - avoid OR until decannulated, if unable to wean off consider surgery or palliation

Answer 667

- Surgical consultation - criteria before surgery: U/O>1ml/kg/hr, FiO2 <0.5., SpO2 85-95%, normal MAP for age, lactate <3mmol/L, PA pressure < systemic. - failure to meet criteria by 2 weeks; consider attempted repair or palliation - patch repair if not amenable to primary repair - open repair (high recurrence with minimally invasive technique)

Answer 668

The severity of congenital diaphragmatic hernia (CDH) can be estimated prenatally using observed-to-expected lung–head ratios (by ultrasound) and total fetal lung volumes (by magnetic resonance imaging), as well as fetal liver position. L CDH LHR <25% poor outcome, R CDH LHR <40% predicts poor outcome

Answer 669

``` BPD PIE chILD (disorders more prevalent in infancy) o ACDMPV (FOXF1, increased risk associated malformations) o Lung Growth abnormalities § Brain, thyroid, lung (NKX2.1) § Filamin A (FLNA) o PIG o Surfactant deficiency § ABCA3 § TTF-1 (NKX2.1) § Surfactant protein B § Surfactant protein C Congenital cardiac disease Chronic aspiration – reflux, congenital abnormality, e.g,. TEF) Congenital cystic lung abnormality (although very diffuse) CDH Post-infectious Right pulmonary hypoplasia ```

Answer 670

HFJV Minimize barotrauma and volutrauma There is no definitive treatment for PIE. Management is supportive and directed at providing adequate gas exchange and minimizing the risk of further air leak. This is best accomplished by decreasing the mean airway pressure as much as possible, which is achieved by reducing the peak inspiratory pressure, positive end expiratory pressure, and inspiratory duration. The inspired oxygen concentration should be increased to compensate for the decreased mean airway pressure. We often use high frequency ventilation in infants with PIE to avoid large cyclic swings in tidal volume, although trials of this intervention are not available Positioning the infant with the affected side down, minimal chest physiotherapy and endotracheal suctioning, and if possible, decreasing ventilator pressure and inspiratory times

Answer 671

Pulmonary interstitial emphysema (PIE) consists of air trapped in the perivascular tissues of the lung. This results in decreased compliance and overdistention of the lung. The interstitial air also compresses airways, resulting in increased airway resistance. PIE typically affects mechanically ventilated extremely low birth weight infants and may involve one or both lungs. It usually presents within 96 hours of birth with gradually worsening hypoxemia and hypercarbia. Ventilator settings are often increased in response to the poor gas exchange, which may exacerbate air trapping and lead to further worsening of oxygenation and ventilation. Overdistention of the lung may cause vascular compression, resulting in decreased venous return and impaired cardiac output. PIE may precede the development of pneumothorax or other air leak.

Answer 672

Minimize volutrauma, barotrauma, atelectrauma Avoid oxygen toxicity (e.g., SpO2 goal 92-95% as in BPD) Consideration of HFOV iNO for pulmonary hypertension potential of need for ECMO if deterioration

Answer 673

1- Exhaustion , with development of metabolic acidosis , decreasing pH in the presence of a normal or rising PCO2 2- Breath sounds start as audible , bilateral wheezes and become distant as air trapping increases 3- Severe hypoxemia while receiving oxygen 5- Altered mental status 6- Life threatening dysrhythmia 7- PCO2 rises while pH declines 8- Cardiac or respiratory arrest

Answer 674

1- Keep peak and Pplat minimized ( alveolar plateau pressure must still be kept low ( <30cmH2O) despise high PIP 2- Maintain FiO2 as needed to keep PaO2 60-100mmHg 3- Permissive hypercapnia ( PaCO2 45 -80mmHg) is acceptable as long as pH range is acceptable ( 7.10 - 7.20) 4- Ventilator cannot be matched to patient needs ( use of sedatives and paralytics) particularly in the 1st 24 hours 5- Avoid increasing air trapping (auto-PEEP - put their PEEP below auto PEEP) by providing longer expiratory time: Vt 4-8ml/Kg ; I:E 1:3 Low RR- Inspiratory flow 80-100L/min Auto PEEP - expiratory hold in patient not spont breathing on vent

Answer 675

>240 mL in 24 hours

Answer 676

Infection (in those without underlying conditions)

Answer 677

History - no nausea or vomiting - Lung disease - Asphyxia possible with hemoptysis Sputum - frothy - liquid or clotted appearance - bright red or pink Lab - alkaline pH - macrophages and neutrophils

Answer 678

``` Trauma Exposure to inhalation injury Smoking Pneumothorax History of syncope, cyanosis, heart disease? Previous pneumothorax? ID: History of fever? Cough? infection? PE risk factors: OCP use, pregnancy menses - catamenial hemoptysis, pneumothorax IV drugs, cocaine Vaping - EVALI Cannabis Rib fracture (Anxiety) Costochondritis Cardiac--pericarditis, myocarditis GERD - hiatus hernia Boerhaave syndrome - esophageal rupture ```

Answer 679

1) Spontaneous - primary or secondary (underlying lung disease - COPD, asthma, CF, infection, ILD, CTD, scleroderma, Marfan's, malignancy) 2) Traumatic 3) Iatrogenic

Answer 680

Patients who fail aspiration treatment because of a persistent air leak, or those who present with a recurrent spontaneous pneumothorax, and many patients with SSP should be managed with a thoracostomy tube or pigtail catheter

Answer 681

1) A first PSP and an air leak that fails to resolve after approximately five days of thoracostomy drainage. This is the time period typically suggested for adults but is based on panels of experts rather than empiric evidence 2) Recurrence of PSP (either ipsilateral or contralateral).

Answer 682

1- Blood gas, oxygen saturation 2- CXR 3- PFT including : 6-minute walk distance (6MWD) test and DLCO 4- ECG Other investigations to be arranged urgently: 5- ECHO to assess RV function and PH 6- Cardiac Cath with acute vasoreactivity testing (AVT) 7- BNP and PRO BNP 8- MRI can be useful as part of the diagnostic evaluation and during follow-up to assess changes in ventricular function and chamber dimensions Cardio consult

Answer 683

Refer for lung transplant evaluation | Consider atrial septostomy

Answer 684

``` 1- CF with end stage lung disease 2- Severe pulmonary hypertension with failure of all medical therapies 3- Congenital interstitial lung diseases 4- Bronchiolitis obliterans 5- alveolar capillary dysplasia 6- Retransplant 7- Severe Pulmonary fibrosis ```

Answer 685

``` Active malignancy within 2 years Sepsis Active TB Severe NM disease Documented refractory nonadherence Multiple organ dysfunction Acquired immunodeficiency Syndrome Hepatitis C with histiologic liver disease ```

Answer 686

``` Pleurodesis Renal insufficiency Markedly abnormal body mass infex Mechanical ventilation Scoliosis Poorly controlled DM Osteoporosis Chronic airway infection with multiple resistant organisms Hepatitis B surface antigen positive ```

Answer 687

Donor downsizing using linear stapling devices or lobectomy and lobar transplant. Ex-vivo lung perfusion Living donor lobar lung transplantation is still happening in Japan

Answer 688

• FEV1 is <50% predicted and rapidly declining (>20% relative decline in FEV1 within 12 months) OR • FEV1 is <50% predicted with markers of shortened survival OR • FEV1 is <40% predicted

Answer 689

low FEV1. 6-min walk test (6MWT) distance < 400 m Room air hypoxemia (SpO2 b 88% or PaO2 b 55 mmHg, at rest or with exertion; at sea level) Pulmonary hypertension (PA systolic pressure N 50mmHg on echocardiogram or evidence of right ventricular dysfunction in the absence of a tricuspid regurgitant jet), Hypercarbia (PaCO2 N 50 mmHg, confirmed on arterial blood gas) BMI <18 kg/m2 for adults (or BMI less than 5th percentile for children) Increased frequency of pulmonary exacerbations (N2 exacerbations per year requiring IV antibiotics or one exacerbation requiring positive pressure ventilation) Massive hemoptysis Pneumothorax

Answer 690

Infection – bacterial, viral and less likely fungal Rejection – likely ACR (acute cellular rejection) at this point in time Pulmonary edema Other: Anastomotic leak Aspiration

Answer 691

Transbronchial biopsy | Then IV pulse steroid for 3 days

Answer 692

Chronic lung allograft dysfunction – BO/BOS, RAS Antibody mediated rejection Infection – viral/bacterial/fungal Posttransplant lymphoproliferative disease ``` Tests: PFT’s (spiro with BDR, lung volumes) CXR Sputum sample CT chest BAL Transbronchial biopsy DSA (Donor Specific HLA antibodies) Open lung biopsy CMV/EBV ```

Answer 693

Consider switching immunosuppression meds Azithromycin Treat any concurrent infection ( bacterial/Viral or fungal) Re-transplant Ensure adherence to medication Post lung transplant BO (combination of rejection + CMV + other stuff) : azithro, re-transplant Post infectious BO: pulse steroids, (likely extrapolate FAM) Post HSCT BO: pulse steroids, FAM

Answer 694

1.5-5 mild 5-10 moderate >10 severe

Answer 695

loud single P2 pan-systolic murmur from TR diastolic murmur from pulmonary insufficiency RV gallop Evidence of RV failure – elevated JVP, hepatomegaly, peripheral edema Cyanosis Left parasternal heave

Answer 696

``` Phosphodiesterase inhibitors (eg. Sildenafil) Prevent degradation of cyclic GMP → potentiates the effect of NO activity by inhibiting PDE 5 ``` Endothelin Receptor Antagonists (eg. Bosentan-dual receptor) Block ET receptors (found in smooth muscle and endothelial cells) thus promoting vasodilation and prevents proliferation Prostacyclin Analogs/receptor agonists (eg. Epoprostenol, Iloprost, Trepostinil) Acts as a pulmonary vasodilator, inhibits vascular smooth muscle proliferation, inhibits platelet aggregation, improves endothelial dysfunction and can also act as a possible cardiac inotrope Ca channel blockers (eg. Nifedipine) Pulmonary vasodilator - by inhibiting Ca influx through slow channel in cardiac and vascular smooth muscle cells

Answer 697

Methemoglobinemia platelet dysfunction Rebound PH

Answer 698

1. PAH (includes PPHN and PVOD) 2. PH secondary to left heart disease 3. PH secondary to chronic lung disease 4. CTEPH 5. Multifactorial

Answer 699

Porto-pulmonary hypertension Hepato-pulmonary syndrome Fluid overload – pulmonary edema/pleural effusions Portal HTN → consumptive thrombocytopenia → pulmonary hemorrhage Infection, including pneumonia, due to immunoglobulin loss/decreased production ``` Lymphocytic pneumonitis (primary biliary cirrhosis) Aspiration pneumonitis ( with patient in hepatic encephalopathy ) Restrictive lung function from mass effect of ascites ``` Tests: CXR – assess for pulmonary edema/pleural effusions/small lung volumes (from ascities) Echo + bubble study – assess for pulmonary hypertension and for intra-pulmonary shunt ABG – assess PaO2 CT chest angio – assess for AVM Macro-Aggregated Albumin Lung Perfusion Scan = radioactive lung perfusion scan

Answer 700

Bubbles from right side of heart would enter left heart within 3 – 6 cardiac cycles (< 3 cycles c/w intracardiac shunt) < 3 cycles suggest intracardiac and 3 – 6 cycles suggest intra-pulmonary) R-L shunt: Extra-cardiac (intra-pulmonary) – AVM (isolated pulmonary AVM or in context of HHT) Of note, the non-pulmonary shunts (cerebral, liver) are actually left to right shunts

Answer 701

Oxygenation defect – PaO2 < 80 mmHg or A-a gradient > 15 mm Hg in room air Pulmonary vascular dilatation – positive contrast echocardiography (bubble echo) Portal hypertension +/- cirrhosis

Answer 702

Widened barrier to diffusion - bc of pulmonary edema and vessel dilation R-L shunt V/Q mismatch - bc of atelectasis

Answer 703

Liver Transplant

Answer 704

Hypoventilation – global neurological disease with hypotonia (ie. T21, PWS), SCI, anterior horn injury (SMA), peripheral neuropathy (GBS), NMJ (MG), muscular (DMD/BMD etc), hypoventilation syndromes (CCHS, ROHAAD etc), Meds - opioid for pain relief V/Q mismatch – ie. airways obstruction (asthma, CF, PCD, bronchiectasis), alveolar hemorrhage, pneumonia, alveolar edema etc à unlikely all result in respiratory symptoms (cough, SOB, wheeze etc). Widened barrier to diffusion – ILD, interstitial edema, ACD/MPV etc R-L shunt: Intra-cardiac – CHD Extra-cardiac (intra-pulmonary) – AVM (HHT), HPS reduced Patm – not issue

Answer 705

Blood gas – assess for hypoventilation, hypoxemia (if arterial) CXR - assess for causes of V/Q mismatch CT chest contrast - vascular anomalies, shunting, PE PFT’s – for V/Q mismatch/widened barrier to diffusion Bubble echocardiography –assess for shunting

Answer 706

``` FHx of HHT Telangiectasias? History of epistaxis Sx/Sx consistent with visceral AVMs Cyanosis, SOB, SOBOE, exercise intolerance, hemoptysis; embolic phenomenon – TIA, stroke, cerebral abscess GI bleed History of portal HTN H/A, seizure, focal neurological deficits, intra-cranial hemorrhage ```

Answer 707

Clinical diagnosis: - Epistaxis - Telangiectasias - AVMs (visceral lesions) - Family history 2 positive = suspected HHT >3 = Confirmed HHT Or Genetic testing If +ve confirms the diagnosis If -ve doesn’t rule out the disease

Answer 708

ENG gene – HHT type 1 ACVRL1 (also called ALK1) gene – HHT type 2 SMAD4 gene – HHT in association with juvenile polyposis (JPHT) GDF2

Answer 709

Progressive enlargement of the lesions Symptomatic hypoxemia Presence of paradoxical embolization Feeding vessels of 3 mm or larger

Answer 710

Extrinsic compression: Anterior mediastinal mass, lympadenopathy, Vascular ring Intraluminal: hemangioma, web, cyst, papilloma, malignancy Airway: tracheomalacia, tracheal stenosis

Answer 711

PA sling | Vascular rings tend to cause posterior esophagus compression

Answer 712

Complete tracheal rings and associated tracheal stenosis

Answer 713

Surgical management if symptomatic | Translocate the anomalous left pulmonary artery onto main pulmonary artery

Answer 714

Slide tracheoplasty, to be discussed with ENT

Answer 715

Do no harm Patient autonomy Best interest of the patient Justice (equal distribution)