Respirology Flashcards

1
Q

What is the most sensitive PFT for small airways disease:

a) FEV1
b) FEV1/FVC
c) FEF 25-75
d) peak flow velocity

A

c) FEF 25-75
● FEF 25-75: mean expiratory flow in middle half of FVC maneuver
o Reflects flow through small airways (< 2mm diameter)

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2
Q

A 15 year old boy with recurrent pneumonia. RML x3, RLL, LLL in the past. Next test:

a) Quantitative immunoglobulins
b) Pulmonary function tests
c) Lung scan

A

a) Quantitative immunoglobulins
Recurrent Pneumonia= 2 or more episodes in single year or 3 or more episodes ever (with CXR clearing between occurrences) - think primary immune deficiency
*if recurrent in same lobe suspect structural abnormality

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3
Q

14 year old boy with Duchenne’s, who is in a wheelchair, has recently seen his FVC fall from 30% to 21% predicted. What symptom will he most likely complain of?

a) Headache early in morning
b) Headaches in the afternoon
c) Tingling of his fingers
d) Dyspnea with exertion

A

a) Headache early in morning

- Nocturnal hypoventilation (early sign of muscle weakness) - morning headache, daytime fatigue

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4
Q

A 12 year old presents to your office with a history of quickly fatiguing with exercise. Her physical exam is unremarkable in your office. You send her for PFTs. What would you expect the results to be if her trouble was related to deconditioning? What if it was related to asthma? What about restrictive lung disease?
a. Fill in a table with normal, increased or decreased for the following PFTs: FEV1/FVC, MMEF25-75 and RV/TLC (rows for asthma and deconditioning).

A
Asthma: FEV1/FVC decreased
- MMEF25-75 decreased
- RV/TLC increased
Deconditioning: FEV1/FVC normal
- MMEF25-75 normal
- RV/TLC normal
Restrictive: FEV1/FVC normal/increased
- MMEF25-75 normal
- RV/TLC normal/increased
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5
Q

Newborn has significant respiratory distress and CXR that is consistent with pneumonia. She is ventilated with PIP 36, PEEP 5 rate 60 bpm, FiO2 1.0, she is not saturating very
well. What is the likely diagnosis? What intervention should you start now?

A
  1. PPHN

2. iNO

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6
Q
You see a 6 year old girl with a history of a productive cough and persistent otitis media. On physical exam, you note cobblestoning of the oropharynx. What is the
most appropriate diagnostic test:
a. Immunoglobulins 
b. CT chest 
c. tracheal aspirate
d. bronchial biopsy
e. call a psychic hotline
A

a. Immunoglobulins
- primary immune deficiency (chronic cough and otitis media)
- cobblestoning more related to GERD/postnasal drip

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7
Q
8 year old girl with cough at night and with exertion for the past three months. PFTs are all normal. What would you do next:
A) CXR
B) Treat with b2 agonist
C) Methacoline challenge
D) PH probe
A

C) Methacoline challenge

- very specific for asthma

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8
Q

7 year old with sore throat in the mornings, bad breath, chronic cough with abdominal pain for 2 weeks. Her cough is worse with activity. What test will give you the diagnosis?

a) Throat swab
b) Pulmonary function tests with methacholine challenge
c) pH probe
d) Upper GI series

A

c) pH probe
pH monitoring- quantitative and sensitive documentation of acid reflux episodes (pathologic)- insufficient to prove/disprove diagnosis of GERD, can’t measure non-acid reflux- better for acid suppression during treatment, apneas and atypical presentations (cough, stridor, asthma)

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9
Q

6y F with chronic cough x 8 mos. Occurs unrelated to illness. It is a harsh cough during the day that decreases at night. Previous unsuccessful treatment for croup x 4 What is the likely diagnosis?

a. Athma
b. Post-viral cough
c. Habit cough
d. Vascular ring

A

c. Habit cough

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10
Q

A 1 year old child has a cough, and mom wants to know if she can use an over-the- counter cough preparation for him/her. What do you tell her?

A

No safe cough medications for kids under 6

- no studies show significant benefit, and there is risk of serious side effects with over the counter cold medications

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11
Q

A child is seen at 4 weeks of age in your office. They have had viral URTI symptoms for a week and now have cough and tachypnea. There is no increased work of breathing or wheezing. On CXR there is patchy atelectasis and interstitial infiltrates. What is the most likely etiology:

a. Ureaplasma urealyticum
b. Chlamydia pneumonia
c. RSV

A

b. Chlamydia pneumonia
- young infants, afebrile illness, insidious onset between 1-3m
- repetitive cough “staccato”, tachypnea, rales in afebrile 1 month old is characteristic
- absence of fever and wheezing helps distinguish from RSV

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12
Q

A 2 year old child is transferred to your center after 12 hours in a peripheral centre being treated for croup. They have had an acute onset of stridor and a barking cough after 1-2 days of viral URTI symptoms. There has been minimal response to 2 doses of neb racemic epinephrine and 2 doses of corticosteroid. On arrival the child is anxious, stidorous and has increased work of breathing. What is the next step in your management process:

a. Orotracheal intubation
b. Heliox
c. IV ventolin

A

a. Orotracheal intubation
- think bacterial tracheitis in kid with croup who is not responding to croup treatment
- tx: vanco or cloned and cefotax or ceftriaxone

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13
Q

3 mo with recurrent URTI. Has had on + off stridor since birth. On exam looks well, afebrile, VSS, intermittent stridor on inspiration. Most likely diagnosis?

a. Laryngomalacia
b. Viral croup
c. Laryngeal web
d. Vascular ring

A

a. Laryngomalacia

o Sx at 1-2 weeks and increase up to 6m (vs vascular ring where symptoms present at about 3 months)

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14
Q

A 4-year-old child has a chest x-ray done for a different reason, but it shows an asymptomatic solid circular lesion in the anterior mediastinum:

a) ganglioneuroma
b) neuroblastoma
c) lymphoma
d) teratoma
e) metastasis from a Wilms’ tumor

A

ANSWER: d) teratoma (more rare but anterior ONLY, in adults asymptomatic; missing kids info)

a) ganglioneuroma (posterior)
b) neuroblastoma (posterior)
c) lymphoma (can be anterior or other areas of mediastinum); non-Hodgkin or Hodgkin’s; but typically older age peak incidence
e) metastasis (not common site for mets)

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15
Q

Newborn with respiratory distress and cystic lesion in LUL with tracheal deviation. What is the most likely diagnosis:

  1. CCAM
  2. Pulmonary sequestration
  3. Pneumonia
A
  1. CCAM
    congenital pulmonary airway malformation (CPAM)
    o Sx- asymptomatic, resp distress, resp infections, pneumo, hydrops, hypoplasia of other lobes, chest pain, mediastinal shift away
    o Ix- CT scan (even if asymptomatic)
    o Rx- resection by 1 year (malignant potential)
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16
Q

In asthma, bronchiolar hyperresponsiveness:

a. Is present even if spirometry is normal
b. Decreases with a URI
c. Is not a sensitive test for the diagnosis of asthma
d. Is not inherited

A

a. Is present even if spirometry is normal

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17
Q

3-year-old with asthma exacerbation in moderate respiratory distress. Can speak in sentences.
Tachypneic and wheezing. Which test would you do:
a) chest x-ray
b) arterial blood gas
c) spirometry (FVC, FEV 1 )
d) O 2 saturation by pulse oximetry
e) flow, end tidal CO 2

A

d) O 2 saturation by pulse oximetry

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18
Q
5 year old with asthma. Treated with ventolin overnight and Q 30 this morning. Aminophyline added this morning to help improve oxygenation. Child complaining of nausea and weakness. You should check:
A) serum sodium
B) serum glucose
C) serum potassium
D) serum magnesium
A

C) serum potassium
hypokalemia
o Symptoms: nausea (GI decreased peristalsis), neuromuscular excitability (hyporeflexia, paralysis), arrhythmias

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19
Q

You are called about an asthmatic with a unilateral pneumothorax. In arranging medical air transport to your Intensive Care Unit, you suggest:

a) insert a chest tube on the affected side
b) insert a chest tube if the pneumothorax is greater than 10%
c) insert a chest tube only if the patient requires intubation
d) insert a needle into the 2nd intercostal space, midclavicular line
e) transfer without intervention

A

a) insert a chest tube on the affected side

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20
Q

Which is true regarding asthma management:

a) beta-2 agonists act primarily on small airways
b) systemic beta-2 agonists work better than inhaled
c) steroids increase the responsiveness to beta-2 agonists
d) Cromolyn is useful in the acute phase of asthma

A

c) steroids increase the responsiveness to beta-2 agonists

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21
Q

Patient on budesonide 200 mcg bid for five years for poorly controlled asthma. Best to monitor?

a. No investigations
b. Height velocity
c. Cortisol levels

A

b. Height velocity

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22
Q

A 6-year-old asthmatic has been receiving 400 mcg of budesonide 4 times daily for the past 2 months with no improvement. His cough is worse at night. Physical examination is normal. His inhalation technique is adequate. What next:

a) increase budesonide to 600 mcg 4 times daily
b) add oral prednisone for 5 days
c) add sodium cromolyn
d) add theophyline
e) add a long-acting beta-2 adrenergic medication

A

e) add a long-acting beta-2 adrenergic medication
● Add-On Therapy to ICS if symptomatic or experiencing significant AE despite optimal use of moderate dose inhaled steroids
o (1) consider increasing steroid dose
o (2) add long-acting beta two agonist (if min. 4 y.o.)
▪ combined meds= Advair (fluticasone + Salmeterol), symbicort (budesonide + formoterol), zenhale (mometasone + formoterol)

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23
Q

The following is true about MDI’s in adolescents with asthma

a. pinch nose when using MDI
b. dispense medication at beginning of exhalation
c. hold breath for 3 seconds after puff
d. take medication in slowly through the whole of inspiration
e. hold MDI in mouth

A

d. take medication in slowly through the whole of inspiration
- consider put in mouth depending on how question is phrased
- should hold breath for 10 seconds after inhalation
- with spacer: slow (5 sec) inhalation with 5-10sec breath-hold OR regular breathing for 30 seconds (5-10 breaths)

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24
Q

Teenager with an MDI for asthma without aerochamber.
Method of proper administration:
A) put in mouth
B) hold breath for 3 secs with inhalation
C) hold breath for duration of inhalation
D) hold nose during inhalation

A

A) put in mouth

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25
Q

Indication that asthma is in poor control:

a. 2 ventolins per week pre-exercise
b. 2 ventolins per week for symptomatic wheeze
c. 2 ventolins per week for night-time cough
d. 2 ventolins per month with colds

A

c. 2 ventolins per week for night-time cough
- well controlled: daytime symptoms max twice per week with no night time symptoms
- very poorly controlled: more than 1 night per week

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26
Q

What is the best indicator for mortality in asthma

a) previous intubation
b) previous oral steroids
c) family history
d) history of atopy

A

a) previous intubation

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27
Q

Which of the following is correct with respect to the use of a spacer with an MDI:

  1. Decreases spray effect
  2. Increases oral deposition
  3. Decreases bronchial deposition
  4. Usually requires a normal tidal volume
  5. Does not require coordination
A
  1. Does not require coordination
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28
Q

Asthma Question re: atopy. What is an indication of bad control? What should be monitored in chronic asthma for control?

A
  1. bad control: any night time symptoms, limitations of activity, 2+ exacerbations per 6 months, daytime symptoms more than twice weekly
  2. monitor PFTs, ventolin usage, ED visits and hospitalizations
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29
Q

A 6 year old boy has been on moderate dose inhaled corticosteroids continuously for his asthma. His mother is concerned about his final adult height. What do you tell her about the current literature in the area:

a. He will be slightly shorter than his expected adult height
b. There will be no effect on his adult height

A

a. He will be slightly shorter than his expected adult height

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30
Q

8 yo with BMI 25, SOB with exertion. Dad with allergic rhinitis. Patient has mild eczema. PFTs show. FVC 80%, FEV1 84% → 87% with bronchodilator and FVC to 85%. What is the treatment?

a. BID fluticasone
b. Salbutamol prior to exercise
c. Montelukast
d. Physical training

A

d. Physical training
- BMI 25 super obese for an 8 year old
- normal PFTs and no bronchodilator response (response is 12% increase)

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31
Q

Which of the following is the most helpful measure to decrease risk of asthma?

a. dust mite covers
b. elimination of environmental smoke exposure
c. removing pets from the home
d. breastfeeding

A

d. breastfeeding
- all of them except removing pets will decrease risk of asthma (exposure to pets is protective against asthma)
- choosing BF over elimination of smoke because the therapeutic effect comes from long standing avoidance of cigarette smoke even prenatally

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32
Q

Teen with asthma. Still symptomatic on inhaled fluticasone 125 mcg BID and monteleukast. Uses ventolin QID. Best treatment to address acute and chronic symptoms.

a. Salmeterol and fluticasone
b. Formoterol and budesonide
c. Salmeterol and ciclesonide

A

b. Formoterol and budesonide
“in adult patients who are poorly controlled the use of budesonide/formoterol in a single inhaler as a rescue medication instead of a SABA in addition to its regular use as a controller therapy has been shown to be effective”

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33
Q

Teenager with history of asthma. She is currently on Fluticasone 125 mcg bid. She has been needing to use her Ventolin puffers, two to three times a week in the day time over the past while. What four suggestions could you make for her management?

A
  • review proper puffer technique including use of aerochamber and ensure patient compliance with medication
  • eliminate environmental exposures
  • no tobacco smoke (first or second hand)
  • allergens (pets, dust mites, mold)
  • wood burning stoves, strong chemical odours, dust
  • could increase to medium dose ICS
  • could add LABA (formoterol)
  • could add LTRA (montelukast)
  • if significant allergic component could use omalizumab
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34
Q

4 year old with asthma on inhaled steroids. Name 2 mechanisms of delivery of asthma medication.

A
  • multiple dose inhaler
  • DPI (dry powder inhaler) device (diskus, flexhaler autohaler, twisthaler)
  • nebulized solution
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35
Q

Child in status asthmaticus who has been given inhaled beta agonists, ipratroprium bromide and iv steroids. Heʼs still in trouble. What are FOUR other medications that can be tried?

A
  1. IV magnesium sulphate
  2. IV ventolin
  3. IV aminophylline
  4. heliox
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36
Q

An 18-year-old male presents with left sided chest pain that radiates to his shoulder. There is a pneumothorax visible on chest x-ray. What is the most likely explanation:

a) idiopathic
b) cocaine abuse
c) status asthmaticus
d) emphysematous bleb
e) previously undiagnosed Marfan syndrome

A

a) idiopathic

Most common cause of spontaneous= Primary idiopathic: usually resulting from sub pleural blebs.

37
Q

17 yr old male found to have pneumothorax on CXR. He
had been at a party that night. What is the number one cause of pneumothorax?
a) Spontaneous
b) Cocaine
c) Status asthmaticus
d) Undiagnosed Marfan syndrome

A

a) Spontaneous

38
Q

Teen with tension pneumothorax, where do you put the needle?

a. needle over 3rd rib, in the second intercostal space at the midclavicular line
b. needle over 5th rib, in the fourth intercostal space midclavicular line
c. Needle in 2nd IC space, anterior axillary line

A

a. needle over 3rd rib, in the second intercostal space at the midclavicular line
- OR 4th ICS in anterior axillary line

39
Q

Teen with ARDS. What is the most likely:

  1. increased pulmonary airway resistance
  2. decreased compliance
  3. decreased elastic recoil
  4. hyperinflation
A
  1. decreased compliance
40
Q

15 y/o status post therapeutic abortion. Sudden onset of respiratory distress. On exam, bilateral crackles. She is coughing blood. What is the best test:

  1. ECG
  2. Pulmonary angiography
  3. CXR
  4. Pulmonary V/Q scan
  5. Leg dopplers
  6. CT chest
A
  1. Pulmonary angiography - gold standard
  • spiral CT with contrast could also be an option, but not normal CT chest
  • has a PE (pregnancy is risk factor)
41
Q

2-year-old with persistent wheezing localized to the RLL x 8 weeks. Unable to obtain inspiratory and expiratory films. Next test:

a) lateral decubitus chest x-ray
b) CT chest
c) MRI chest
d) nuclear lung scan
e) bronchoscopy

A

a) lateral decubitus chest x-ray
▪ Side with FB will not deflate when placed in dependent position (i.e. if right side has foreign body, put right side down and right side will not deflate as expected
o If high degree of suspicion, bronch should be performed despite (-) results.
▪ And if (+) Hx and convinced can go right to bronch.

42
Q

Infant has problem of vomiting with feeds and chronically wheezy. Upper GI shows indentation of upper esophagus. What are two diagnoses you consider?

A
  • vascular ring
  • pulmonary artery sling - these 2 specifically say in Nelson’s that they are associated with esophageal indentation, wheeze and vomiting
43
Q

Wheezing toddler with URTI symptoms. Which is a proven therapy?

a. O2
b. racemic epi
c. iv steroids
d. bronchodilators

A

a. O2

for bronchiolitis

44
Q

Proven therapy in a kid with recurrent viral wheezing?

a. Ventolin
b. Fluticasone X 3 weeks

A

a. Ventolin

45
Q

teenage elite female athlete is having episodes of shortness of breath, chest tightness and wheezing with exercise. She has had an oxygen sat during the episode of 100%, a negative bronchodilator challenge and a negative CXR. What is the most likely cause of her condition? What is the best management of this?

A

Paradoxical vocal cord dysfunction
- speech therapy and behaviour modification are therapeutic

  • symptoms include throat tightness, dyspnea, wheeze, chest tightness, cough
  • no response to bronchodilators
  • CXR is normal
  • PFTs are normal aside from the inspiratory flow loop
46
Q

What is the management of exercise induced asthma?

A

Note re exercise induced asthma:

  • diagnosis of EIA can be confirmed by 15% decrease in FEV1 5-10 minutes after an exercise test (non asthmatic people increase their FEV1 in response to exercise)
  • SABA 15 minutes prior to exercise
  • warm up and cool down
  • avoid exercising in cool, dry environments
47
Q

Kid with wheeze 5-6 yrs old. You have dx asthma. Unresponsive to therapy, steroid, laba, leukotriene inhibitor. Has dyspnea and wheeze. List 4 alternative diagnosis to asthma in your ddx.

A
  • allergic rhinitis/sinusitis
  • foreign body aspiration
  • laryngeal web, cyst or stenosis
  • vocal cord dysfunction/paralysis
  • TEF
  • vascular ring, sling or external mass compressing airway
  • viral bronchiolitis
  • GERD
  • bronchiectasis (CF)
48
Q

A 3 month old child had a TEF repaired in the first few weeks of life. He now presents in your office with stridor. List 3 causes of his stridor.

A
  • refistulization (recurrence of TEF)
  • stricture
  • GERD
49
Q

A child is noted to have nasal polyps. Next step:

a) referral for surgical excision
b) intranasal corticosteroids
c) oral antihistamines
d) oral decongestants
e) arrange a sweat chloride

A

e) arrange a sweat chloride

50
Q

14 year old with CF has sudden onset of severe left chest pain over for the past three hours. The pain is now involving the left shoulder. Some respiratory distress. Mother notes that he has been well, but did miss physio that week. Most likely diagnosis is:

a. RLL pneumonia
b. pneumothorax
c. pleural effusion

A

b. pneumothorax

51
Q

Teen boy with CF has had 2 days of gradually increasing pleuritic chest pain. What is the most likely cause?

a. Infective exacerbation
b. Pneumothorax
c. Pleurodynia

A

b. Pneumothorax

52
Q

In a child with cystic fibrosis, which of the following findings would have the worst prognostic implications:

a. liver disease
b. hemoptysis
c. malnutrition
d. pneumothorax
e. pancreatitis

A

ANSWER: c. malnutrition
*a. liver disease (third most common cause of death in CF after resp failure and transplant complications)
- other prognostic factors:
o Gender (F slightly worse)
o Type of infection (Burkholderia cepacia leads to early deterioration)
o FeV1
o CF related DM associated with poor prognosis

53
Q

What clinical situation predisposes to the worst outcome for cystic fibrosis?

a) malnutrition
b) liver disease

A

a) malnutrition

54
Q

What can cause a false negative sweat chloride test?

  1. Low albumin
  2. Low magnesium
  3. Low phosphate
  4. Low chloride
A
  1. Low albumin
    - other false negatives: meconium ileus, dilution, malnutrition, edema, insufficient sweat quantity,
    hyponatremia, hypoproteinemia
55
Q

Girl with rectal prolapse x 2 reduced easily in the ER. What do you do?

a. reassure mom
b. observe and follow in 3 months
c. sweat chloride
d. barium enema

A

c. sweat chloride

56
Q

3 month old boy comes in for routine newborn care. The mother describes symptoms of URTI and mentions that when he has coughed on two recent occasions, she has noticed a swelling protruding from his anus [picture of rectal prolapse]. List the most likely underlying etiology .

A
  • most cases are idiopathic

- rule out underlying conditions including CF and sacral nerve root lesions

57
Q

16 yo female with CF and complaints of chest pain, cough that produces a rust colored sputum. What is the most likely diagnosis (1 line). List 2 investigations to confirm diagnosis. What is your treatment?

A
  1. ABPA - allergic bronchopulmonary aspergillosis
  2. dx: sputum culture for aspergillus
    - assessment of sputum for elevated IgE antibodies
    - elevated serum IgE
  3. oral corticosteroids first line
    - may need antifungals, but goal is to control the allergic reaction to the fungus, not treat the fungus
58
Q

List 3 organisms that colonize airway of patients with CF

A
  • staph aureus
  • hemophilus influenza
  • pseudomonas
  • MRSA
  • stenotrophomonas maltophilia
  • burkholderia cepaciae
  • aspergillus
59
Q

You are unable to obtain a sweat sample in a neonate. List 3 other ways to confirm the diagnosis of CF.

A
  • genetic testing - identifying 2 CFTR mutations
  • fecal elastase testing (pancreatic insufficiency)
  • positive newborn screen
60
Q

Kid with CF. Reduced PFT by 30%. Maxed out on salbutamol. Give four interventions to improve his lung function

A
  • chest physiotherapy
  • human recombinant DNase (dornase)
  • nebulized hypertonic saline
  • routine aerobic exercise
  • ensure adequate nutrition
  • antibiotic therapy
  • inhaled corticosteroids
  • voluntary cough and forced expiratory maneuver (in-ex sufflator)
61
Q

A chest tube was inserted into a child with empyema and whiteout of the left lung field. 12 hours after the chest tube insertion, a repeat CXR shows persistent pleural air. The chest tube system is patent. List 3 causes of the persistent pleural air

A
  • bronchopleural fistula
  • necrotizing pneumonia
  • equipment failure
62
Q

A 16-month-old ex-prem with BPD presents with fever (39.4), cough, rhinorrhea, and dyspnea. On exam, febrile, RR 40, no wheeze, but decreased air entry over LLL. There have been several other infants in the community who have been recently admitted to hospital and found to have RSV. What would be your management of this infant:

a) outpatient Ventolin q4h
b) outpatient Pulmicort
c) outpatient antibiotics
d) admit for treatment with Ribavirin
e) admit for blood culture, IV antibiotics, and tests for RSV

A

e) admit for blood culture, IV antibiotics, and tests for RSV
- or maybe outpatient antibiotics depending on clinical picture, but err on side of caution given BPD

63
Q

4-month-old ex-prem with RSV. pH 7.31, pCO 2 60, pO 2 94. Best management:

a) humidified oxygen and careful monitoring
b) intubate and ventilate
c) RSV immune globulin
d) steroids
e) antibiotics

A

a) humidified oxygen and careful monitoring

64
Q

A 2 month old child is seen with a 3-4 day history of viral URTI symptoms, now has progressively increasing work of breathing. RR is 65, O2sat is 91% on room air. On auscultation there is diffuse wheezing. Of the following treatment modalities, which has been proven effective in this disorder:

a. Neb ventolin
b. Neb racemic epinephrine
c. Corticosteroids
d. O2

A

d. O2

65
Q

Baby with severe bronchiolitis, wheezing, severe retractions, lethargy. HR 160, RR 12, Sats 82% what is the next step.

a. Give racemic epi
b. Give salbutamol
c. Give steroids
d. Bag-mask ventilation

A

d. Bag-mask ventilation

66
Q

B) How does palivizumab minimize risk and by what mechanism does it work?

A

decreases hospitalization risk but does not decrease severity of infection in those who get RSV despite prophylaxis; works by conveying passive immunity

67
Q

List a couple of babies who should get palivizumab

A
  1. Clinically significant CHD or CLD (on bronchodilator, steroids, diuretics or supplemental O2) and <12 months of age at start of RSV season
  2. prems without CLD born before 30+0 weeks who are less than 6 months old at start of RSV season (can offer it but it’s not essential)
  3. infants born before 36+0 weeks, less than 6 months old at start of RSV season who would need medevac for hospitalization
68
Q

A patient with recurrent pneumonia, sinusitis, bronchiectasis in RML. One should investigate for all of the following except:

  1. CF
  2. Alpha-1-antitrypsin deficiency
  3. Ciliary dyskinesia
  4. Hypogammaglobulinemia
  5. CGD
A
  1. Alpha-1-antitrypsin deficiency
    - CF, PCD and immunodeficiencies can all cause bronchiectasis
    - alpha-1 antitrypsin causes liver disease in kids, lung disease not usually seen until 20s
69
Q

14 yo boy with recurrent wheezing. CXR shows bronchiectasis. Most likely diagnosis?

A

CF (most common cause of bronchiectasis in industrialized nations)

70
Q

Bronchiectasis and recurrent wheezing in a 14 year old boy. What are 3 causes

A
  1. CF
  2. primary ciliary dyskinesia
  3. humoral immune deficiency
  4. recurrent pulmonary infection/infection with pertussis, measles or TB
71
Q

A 3-year-old boy comes for a regular checkup. He attends day care and he always seems to get “colds”. He does not eat well and his weight gain has been poor. He snores, and usually wakes up several times during the night. You notice that he cannot breathe through his nose and that his tonsils are large. Most appropriate investigation:

a) chest x-ray
b) lateral view of the nasopharynx
c) overnight oxygen saturation recording
d) morning capillary blood gas analysis
e) electrocardiogram

A

c) overnight oxygen saturation recording

- poor man’s oximetry

72
Q

Parents are worried about their 9 year old daughter; she has been snoring a lot and having episodes overnight where she stops breathing. Polysomnography was done and shows episodes of significant, severe central apnea. What to do?

a) consult ENT
b) MRI brain
c) brainstem evoked auditory potentials
d) pH probe
e) CPAP overnight

A

b) MRI brain

73
Q

Which of the following is treatment for obstructive sleep apnea?

  1. CPAP
  2. T & A
  3. BiPAP
  4. Nasal O2
A
  1. T & A

TA hypertrophy the most common cause of obstructive sleep apnea

74
Q

You are seeing a 6 year old boy in your office. His mother is concerned that he snores, and occasionally seems to pause in his breathing while he is asleep.
a. What is the most common reason for obstructive sleep apnea in children?

A

a. adenotonsillar hypertrophy

75
Q

Name 2 severe complications of OSA

A
  • pulmonary hypertension, systemic hypertension, right sided heart failure
76
Q

List 4 daytime symptoms of obstructive sleep apnea.

A
  • mouth breathing
  • chronic nasal congestion
  • hyponasal speech
  • morning headache
  • poor appetite
  • secondary enuresis
  • decreased mood, behavioural difficulties (irritability, aggression, impulsivity), impaired learning and academic function
  • daytime sleepiness
77
Q

5 things you would tell a boy to improve his sleep hygiene

A
  • consistent bedtime
  • consistent waking time
  • age appropriate number of hours in bed per night
  • sleep in a quiet, dark place
  • avoid hunger and eating prior to bed
  • avoid caffeine, alcohol and nicotine
  • relaxation techniques before bed
  • no screens before bed
  • read a book before bed
  • exercise in the day but not within 2 hours of bedtime
78
Q

3 yo with CP with recurrent symptoms of aspiration. Admitted with pneumonia, abscess and pleural effusion. What would be your choice of antibiotics.

a) Vanco & Amp
b) Amp & gent
c) Clinda & Gent
d) Azithro
e) Cefuroxime

A

c) Clinda (staph, strep and anaerobic coverage) & Gent (if gram negative suspected)

OR pip/tazo or amox/clav

79
Q

3 year old with cerebral palsy chokes with feeds on past history. Currently presents with fever, increased respiratory rate and chest X-ray shows an air bubble on left chest, surrounded by consolidation with a pleural effusion. What is the diagnosis?

a) Lung Abscess
b) Pulmonary sequestration
c) Diaphragmatic hernia

A

a) Lung Abscess

80
Q

Child with CP has recurrent choking episodes. Presents with LLL pneumonia and air fluid level and significant pleural effusion. What is the investigation to help with management.

a. Sputum cultures
b. Blood culture
c. Pleural fluid culture
d. Bronchoscopy and culture

A

c. Pleural fluid culture

81
Q

A child presents to the emergency room with shortness of breath and wheezing. This occurred suddenly after playing with older sibling. What should be the next management step after a CXR?

a) Bronchoscopy
b) Ventolin via nebulizer
c) Racemic epinephrine

A

a) Bronchoscopy

- foreign body

82
Q

The reason that oxygen-helium mixtures are used in airway diseases:

a. reduces pulmonary airway resistance
b. reduces small airway inflammation
c. reduces peribronchial inflammation

A

a. reduces pulmonary airway resistance

- decreases turbulence of flow

83
Q

How does helium work in a ventilated patient?

A

● 60-80% helium + 20-40% oxygen
● reaches lower airway more easily because lower resistance/turbulent flow
o lowered gas density= laminar flow overcomes airway obstruction

84
Q

What statement is not true regarding Intal (sodium chromoglycate/cromolyn):

a. no bronchodilator effect
b. mast cell stabilizer
c. not used in children less than 5 years old
d. good for exercise induced asthma
e. prevents late onset allergic effect

A

c. not used in children less than 5 years old (false- must be min. 2 y.o.)
- can be used for prevention of exercise induced asthma or allergen induced bronchospasm (but is not a bronchodilator so do not use in acute exacerbation)
- prevents mast cell release

85
Q

12 month old M with pneumonia and toxic. How to treat?

a) IV Cefuroxime and po erythromycin
b) IV Ampicillin

A

b) IV Ampicillin

86
Q

5½-year-old child with a recent upper respiratory tract infection, now has respiratory distress and BP 150/110. Most likely:

a) anxiety
b) pneumonia
c) myocarditis
d) Henoch-Schonlein purpura
e) post-streptococcal glomerulonephritis

A

e) post-streptococcal glomerulonephritis

87
Q

Child with eczema and recurrent pneumonia. Hepatomegaly, petechiae, otitis media and low platelets. What do you expect?

a. elevated IgA and IgE
b. immune response to polysaccharide vaccine
c. oral Candida
d. abnormal mitogen proliferation

A

a. elevated IgA and IgE (low IgM and IgG)

Wiskott-Aldrich syndrome: atopic dermatitis,
thrombocytopenic purpura w/ normal-appearing megakaryocytes but small defective platelets, and undue susceptibility to infection
- have poor response to polysac vaccines

88
Q

Alpha-1 antitrypsin. Most likely presentation in children?

a) jaundice
b) emphysema
c) bronchiectasis
d) pneumonia

A
a) jaundice
Typical presentation in kids:
- neonatal cholestasis
- later-onset childhood cirrhosis
Dx: - serum immunoassay shows low level of alpha-1 antitrypsin
89
Q

Child with recurrent OM, sinusitis, and dextrocardia.

a. ) Diagnosis?
b. ) Test to confirm diagnosis.

A

a) Primary ciliary dyskinesia (with Kartagener triad):
- Triad:
- Situs inversus totalis
- Chronic sinusitis and otitis (Clinical feature that distinguishes it from CF)
- Bronchiectasis
b) - curettage from nasal epithelium or endobronchial brushing to obtain suitable specimen for transmission electron microscopy