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FRCPC > Respirology > Flashcards

Respirology Flashcards

1
Q

Name 3 side effects of salbutamol

A

Tachycardia
Hypokalemia
Hyperglycemia
Tremors

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2
Q

During what time period is atrovent helpful in acute asthma exacerbation?

A

Within first hour

↑ FEV1 by 10% if added to β2 agonist

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3
Q

How long to LABAs last?

A

12 hours

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4
Q

When do you add LABAs?

A

Add to ICS if inadequate control with optimal dose

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5
Q

Can LABAs be used in isolation?

A

NO. Must be used with inhaled steroid (combo inhaler)

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6
Q

Name 3 pathophysiologic mechanisms by which steroids work in asthma?

A

↓Cytokine production and inhibits various factors in inflammatory cascade.

↓Mediator release for macrophages and eosinophils

Inhibits eosinophil and lymphocyte production

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7
Q

Name 3 side effects of ICS

A

Oral thrush
Hoarseness
↓linear growth with high dose

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8
Q

What is an indication for adding LTRAs to asthma therapy?

A

Mild/moderate asthmatics

Allergic asthma

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9
Q

Name 9 signs of good asthma control

A 
Daytime symptoms <4 days/week
Night time symptoms <1 night/week
Normal physical activity
Mild, infrequent asthma exacerbations
No absence from school due to asthma
Need for fast acting beta 2 agonists <4 doses/week
FEV1/PEF >=90% personal best
PEF diurnal variation <10-15%
Sputum eosinophils (adults) <2-3%
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10
Q

What are the principles of asthma management continuum?

A

1) Confirm diagnosis
2) Environmental control, education and written action plan
3) B2 agonist on demand
4) Add ICS
5) If >=12 years, add LABA to low dose ICS, if 6-11 years, increase to medium dose ICS
6) If >=12 years, add LTRAl; if 6-11 years, add LABA or LTRA
7) Prednisone or anti-IgA

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11
Q

What dose of fluticasone is considered low, medium and high dose ICS?

A

Low dose
>=12 years <=250 mcg/day
6-11 years <=200 mcg/day

Medium dose
>=12 years 251-500 mcg/day
6-11 years 201-500 mcg/day

High dose
>=12 years >500 mcg/day
6-11 years >500 mcg/day

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12
Q

Can you develop tachyphylaxis to LABA?

A

YES

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13
Q

A patient’s asthma is not well controlled. Name 5 next steps in your management.

A 
Assess inhaler technique
Ask about compliance
Ask about environmental triggers
Assess for comorbidities
Consider adjuncts
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14
Q

Causes of false negative sweat chloride

A
  • Hypoalbuminemia
  • Hyponatremia
  • CFTR mutations with preserved sweat duct function
  • Insufficient sweat quantity
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15
Q

Name 5 causes of poorly controlled asthma in a patient on ICS and B2 agonists.

A

Poor medication compliance
Poor technique when administering inhaled medications
Diagnosis other than asthma
Chronic exposure to asthma triggers -Smoke exposure.
Comorbidities such as obstructive sleep apnea
Significant psychosocial impact of asthma on family

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16
Q

Name 3 complications associated with central hypoventilation syndrome

A

Arrythmias-including sinus pauses
Increased risk of Hirschsprungs
Increased risk of neural crest tumours

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17
Q

Name 4 causes of false positive sweat chloride

A
  • Adrenal insufficiency
  • Hypothyroidism
  • Anorexia nervosa
  • Eczema
  • GSD Type I
  • Nephrogenic DI
  • MPS
  • Malnutrition
  • Ectodermal dysplasia
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18
Q

Name 3 criteria for the diagnosis of asthma on spirometry

A

3 ways to diagnose asthma:

  1. Spirometry

Postbronchodilator increase in FEV1 of >=12%
Reduced FEV1/FVC (<80-90%)
Reduced FEV1 compared to normative values for age
Reduced FEF 25-75

OR

  1. Peak expiratory flow variability

Increase in peak expiratory flow >=20% post bronchodilator or after course of controller therapy
OR
Diurnal variation

  1. Positive challenge test

Metacholine challenge
OR
Exercise challenge
challenge

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19
Q

Name 5 causes of declining PFTs in CF

A
  • Allergic bronchopulmonary aspergillosis
  • Pulmonary exacerbation
  • Non compliance
  • CF diabetes
  • Viral illness
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20
Q

What is the treatment for ABPA

A

Oral steroids

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21
Q

Name 2 tests to confirm the diagnosis of ABPA

A

-Aspergillus skin test
-Elevated total serum IgE concentration (typically >1000 IU/mL)
Others:
-Serum specific Aspergillus IgE
-Eosinophil count
-Precipitating serum antibodies to A. fumigatus

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22
Q

Name 3 organisms that colonize the airways unique to CF

A
  1. Staph aureus
  2. Pseudomonas aeruginosa
  3. Burkholderia cepacia complex (BCC)
  4. Aspergillus
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23
Q

Name 2 tests to confirm the diagnosis of ABPA

A
  • Aspergillus skin test
  • Elevated total serum IgE concentration (typically >1000 IU/mL)

Others:

  • Serum specific Aspergillus IgE
  • Eosinophil count
  • Precipitating serum antibodies to A. fumigatus
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24
Q

Name 3 bacteria that can cause:

i) epiglottitis
ii) croup
iii) bacterial tracheitis

A

i) H. flu type B, beta hemolytic strep
ii) parainfluenza, RSV, influenza
iii) staph aureus, strep pneumoniae, h. flu

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25
Q

Name 3 organisms that cuse chronic pulmonary infection in CF

A 
S Aureus	(most common, esp in young)
H Influenza
Psuedomonas Aeruginosa (older patients)
Burkholderia Cepacia
Aspergillus fumigatus(ABPA)
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26
Q

What is a positive sweat chloride test?

A

Sweat Test Chloride >60mmol/l is positive

30-60 mmol/L Gray Zone – further testing

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27
Q

What does newborn screening for CF typically entail?

A

2 serial assays

1) Serum immunoreactive trypsinogen (IRT)
2) If positive, repeat IRT OR DNA analysis for mutations in CFTR.
3) If positive screen, sweat chloride

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28
Q

For optimal accuracy when should sweat chloride be performed?

A

> 2 kg and >2 weeks of age

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29
Q

What kind of prenatal testing can be done for CF?

A

CVS or amnio with DNA analysis

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30
Q

What kind of PFT pattern is seen in CF in a) early disease b) late disease?

A

Early disease-obstructive, small airways disease (decreased FEF 25-75)
Late disease-restrictive

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31
Q

Name 4 treatment goals in CF

A

Maintain normal lung function

  • Regular chest physiotherapy
  • Treat chronic infections with oral or inhaled Abx
  • Treat acute infections with oral or IV Abx
  • Use mucolytics

Maintain normal nutrition & growth

  • High fat/high energy diet
  • Pancreatic enzymes, vitamin supplements
  • Supplements/g-tube feeds

Regular follow-up with multi-D team

Educate patient and family about CF

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32
Q

What is the most common CF causing mutation?

A

Delta F508

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33
Q

What kind of PFT pattern is seen in CF in a) early disease b) late disease?

A

Early disease-obstructive, small airways disease (decreased FEF 25-75), decreased FEV1, increased RV/TLC (increased gas trapping)
Late disease-restrictive, gas trapping

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34
Q

What are the clinical features of ABPA?

A

Present with increasing cough, wheeze, exercise intolerance
Decrease FEV1
Rust colour sputum***
CXR often has upper lobe involvement
Lab work with eosinophilia and serum IgE>1000

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35
Q

Name 4 treatment goals in CF

A

Maintain normal lung function

  • Regular chest physiotherapy
  • Treat chronic infections with oral or inhaled Abx
  • Treat acute infections with oral or IV Abx
  • Use mucolytics (hypertonic saline, DNAse)

Maintain normal nutrition & growth

  • High fat/high energy diet
  • Pancreatic enzymes, vitamin supplements
  • Supplements/g-tube feeds

Regular follow-up with multi-D team

Educate patient and family about CF

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36
Q

If you see asthmatic with nasal polyp, what test should you order?

A

Sweat chloride

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37
Q

What are the clinical features of ABPA?

A

Present with increasing cough, wheezing
Rust colour sputum
Decrease FEV1
CXR often has upper lobe involvement, proximal
bronchiectasis
Lab work with eosinophilia and increased IgE.

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38
Q

What is the pathophysiology of PCD?

A

Dysfunction of cilia

-Ineffective mucociliary clearance

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39
Q

How do you diagnose PCD?

A

Screen: Low nasal Nitric Oxide
Diagnosis: Nasal/bronchial biopsy with EM of cilia
High speed videomicroscopy of cilia
Genetics

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40
Q

Name 5 clinical manifestations of PCD

A 
Persistent rhinitis Cobblestoning of posterior pharynx***
Sinusitis
Recurrent otitis media***
Bronchiectasis
Chronic cough
Male infertility
50% have situs inversus totalis***

***differentiate from CF

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41
Q

How do you diagnose PCD?

A

Nasal scraping or bronchial biopsy (EM of cilia)***

Low nasal Nitric Oxide
High speed videomicroscopy of cilia
Genetics

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42
Q

Name 5 causes of pneumothorax

A 
Idiopathic/spontaneous (most common)
Thoracic trauma
RDS/ meconium aspiration 
CF with pleural blebs 
Asthma
Marfans
Aggressive mechanical ventilation
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43
Q

How do you treat large pneumothorax, not under tension?

A

100% O2 (nitrogen washout)

If not effective, chest tube

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44
Q

Name 3 signs of tension pneumothorax

A

Hemodynamically unstable
Decreased venous return
Mediastinal shift
Pleurodesis for recurrent PTX

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45
Q

Treatment of tension pneumothorax

A

Needle decompression (2nd ICS, midclavicular line)
AND
Chest tube

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46
Q

How do you distinguish between transudate and exudate on pleural fluid analysis?

A

Transudate

  • Protein < 3g/dL
  • Pleural fluid protein/serum protein ratio< 0.5
  • Pleural fluid LDH/serum LDH < 0.6
  • Pleural fluid LDH less than two-thirds the upper limits of the laboratory’s normal serum LDH
  • pH >7.45

Exudate

  • Protein > 3g/dL
  • Pleural fluid protein/serum protein ratio > 0.5
  • Pleural fluid LDH/serum LDH > 0.6
  • Pleural fluid LDH greater than two-thirds the upper limits of the laboratory’s normal serum LDH
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47
Q

What is the differential diagnosis of bronchiectasis

A

Lobar

  • Foreign body
  • TB
  • Aspiration

Generalized

  • CF
  • PCD
  • Immunodeficiency (e.g. CVID, HIV)
  • ABPA-proximal airway!
  • Post-infectious (measles, pertussis, adeno, recurrent severe pneumonia)
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48
Q

Name 5 conditions that can cause digital clubbing

A 
CF
TB
Pulmonary fibrosis
IBD
Liver cirrhosis
Cyanotic CHD
Infective endocarditis
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49
Q

What is bronchiectasis?

A

Irreversible dilated airways

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50
Q

What is the work up for bronchiectasis?

A

Sputum culture
CXR-
CT chest-“train track” appearance of airway
Bronchoscopy

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51
Q

How is alpha 1 antitrypsin deficiency diagnosed?

A

Decreased serum levels of AAT

52
Q

What is the clinical presentation of A1AT?

A

Most peds pateints with AAT deficiency present with liver disease (neonatal hepatitis, cirrhosis (in children or adults), and hepatocellular Ca)

For lung disease (emphysema) present in 30s-40s
In 20s if smoker!***

53
Q

What lung findings are consistent with A1AT?

A

Emphysematous changes

54
Q

Name 10 symptoms of OSA

A 
Night: 
Snoring
Restless sleep
Frequent awakenings
Choking or gasping arousals 
Enuresis
Apneas
Paradoxical chest and abdo motion
Day: 
Morning headaches
Mouth breathing
Daytime somnolence
Poor academic performance
Dry mouth
Chronic nasal congestion/rhinorrhea
Hyponasal speech
Difficulty swallowing
Poor appetite
Altered Mood/behaviour
Difficulty in morning wakening
55
Q

Name 5 complications of OSA

A 
FTT
PHTN/cor pulmonale
Depression
Metabolic syndrome
Systemic hypertension
Cognitive problems
56
Q

What is the treatment for OSA?

A
  1. First line-T+A

2. CPAP-for minimal adenotonsillar hypertrophy, facial dysmorphisms, no surgical candidates

57
Q

What investigations should you order for OSA?

A

Lateral neck x-rays
Overnight oximetry
Early morning cap gas
Sleep study

58
Q

Name 5 causes of central apneas

A

Primary:
Idiopathic
CCHS
Arnold-Chiari malformation

Secondary:
HIE
Tumour
Brainstem malformation
Raised ICP/herniation
Medications (opioids, anaesthetics)
Neuromscular (e.g SMA, GBS, phrenic nerve injury) 
Infection (e.g. RSV)
59
Q

What is the treatment of central hypoventilation?

A

BiPap

Trach and vent

60
Q

What is spirometry?

A

Looking at lung volumes (TV, VC)

61
Q

What does RV/TLC represent?

A

Gas trapping

62
Q

What is normal FEV/FVC?

A

0.8-0.85

63
Q

What cannot be measured with spirometry?

A

RV

TLC

64
Q

Spot diagnosis: http://www.thoracicmedicine.org/articles/2012/7/4/images/AnnThoracMed_2012_7_4_250_102187_f3.jpg

A

Congenital lobar emphysema

65
Q

Spot diagnosis: https://images.radiopaedia.org/images/219099/fc64bd9dea93b87955ecca9e28d6f0_gallery.jpg

A

CCAM or congenital pulmonary airway malformation

66
Q

What is the risk of a CPAM?

A

Infection

Small risk of malignancy (pleuropulmonary blastoma (PPB) and bronchoalveolar carcinoma)

67
Q

Indications for surgical removal of CPAM?

A

Symptomatic
Infection (after treated)
Type 4 CPAMs-cystic forms of pleuropulmonary blastoma

Consider resection with high risk features:

  • Large lesions
  • Bilateral or multifocal cysts
  • Family history of pleuropulmonary blastoma-associated conditions
68
Q

How do you make diagnosis of CDH clinically?

A

Bowel sounds in chest

NG tube in chest

69
Q

What is the most common cause of pleuritic chest pain in CF?

A

Pulmonary exacerbation

vs PTX

70
Q

What happens in DMD when FVC <40% predicted?

A

Nocturnal hypoventilation

71
Q

What investigation should you order in an older child with recurrent stridor?

A

Laryngoscopy

72
Q

Describe the appropriate technique for using an MDI without a spacer technique (adolescents)

A
  1. Shake MDI for 5 seconds.
  2. Hold the MDI upright
  3. Breathe out normally.
  4. Put the mouthpiece between your teeth and close your lips around mouthpiece
  5. Keep tongue away from the opening of the mouthpiece.
  6. Press down the top of the canister
  7. Breathe in slow over 4-6 seconds
  8. Hold breath 5 seconds
  9. Wait 15 to 30 seconds between puffs
  10. Recap mouthpiece.
  11. Rinse mouth (if steroid)
73
Q

What is the best way to assess initial adequacy of ventilation?

A

Auscultation
O2
Venous gas

74
Q

In distinguishing between diaphragmatic eventration and CDH what is the best test?

A

Abdominal ultrasound

75
Q

What is the best way to decrease the risk of development of asthma in a child?

A

Elimination of environmental smoke exposure

76
Q

List 3 factors that predict the persistence of asthma into adulthood

A
  • Atopy (eczema, allergic rhinitis, food allergy, inhalant allergen sensitization)
  • Reduced lung function
  • Higher airway hyperresponsiveness
  • Parental asthma
  • Severe LRTI
  • Wheezing apart from colds
  • LBW
  • Environmental tobacco smoke exposure
  • Male gender
77
Q

Above what age can PFTs normally be done?

A

Feasible in kids ≥ 6 who can preform full, forceful, prolonged expiration effort

78
Q

List 3 conditions that can cause obstructive pattern on PFTs

A

Asthma
CF
Bronchilits obliterans

79
Q

List 3 conditions that can cause restrictive pattern on PFTs

A 
Obesity
Late CF
Neuromuscular
Scoliosis
Pulmonary fibrosis
Interstitial lung disease
Previous thoracic surgery
80
Q 
In obstructive lung disease, what would be expected for the following parameters:
TLC
VC
RV
RV/TLC ratio
FEV1
FEV1: FVC
FEF 25-75
PEF
DLCO
A 
TLC-Normal or ↓
VC-Normal or ↓ 
RV-Normal or ↑
RV/TLC ratio-
FEV1-↓
FEV1: FVC-↓
FEF 25-75-↓
PEF-↓
DLCO-Normal or ↑
81
Q 
In restructive lung disease, what would be expected for the following parameters:
TLC
VC
RV
RV/TLC ratio
FEV1
FEV1: FVC
FEF 25-75
PEF
DLCO
A 
TLC-↓
VC-↓
RV-Normal or ↓
RV/TLC ratio-Normal or ↑
FEV1-↓
FEV1: FVC-Normal 
FEF 25-75-↓
PEF-↓
DLCO-Normal or ↓
82
Q

What do PFTs in a patient with deconditioning look like with respect to:

i) FEV1/FVC
ii) RV/TLC
iii) FEF 25-75

A

i) FEV1/FVC-Normal or ↑ (decreased FVC)
ii) RV/TLC-Normal or ↑
iii) FEF 25-75-Normal or ↓

83
Q

What test should you do in a patient with persistent asthma symptoms despite normal spirometry?

A

Metacholine challenge

84
Q

List 5 important aspects of asthma education

A
  • Involve all family members, consider literacy, social, financial factors etc.
  • Review asthma pathophysiology
  • Expectations for good control, goals of therapy
  • Address barriers to medications compliance, misconceptions, side effects of meds
  • Teach proper technique for inhalers, peak flow measures
  • 2 part ‘Written management plan’ –routine care, exacerbation flags and management
85
Q

How do you diagnose CF?

A

Clinical symptoms consistent with CF in at least one organ system

  • Chronic sinopulmonary disease
  • Characteristic gastrointestinal and nutritional abnormalities
  • Salt loss syndromes
  • Obstructive azoospermia

OR

A history of cystic fibrosis in a sibling

OR

A positive newborn screen

AND 1 of the following:

1) Elevated sweat chloride ≥60 mmol/L (on two occasions)
2) Presence of two disease-causing mutations in CFTR, one from each parental allele
3) Abnormal nasal potential difference

86
Q

How does heliox work?

A

Reduces pulmonary airway resistance

Helium is much less dense than nitrogen. When substituted for nitrogen, helium helps maintain laminar flow across an obstructed airway, decreases airway resistance, and improves ventilation

87
Q

Describe the appropriate technique for using an MDI with a spacer

A
  1. Uncap mouthpiece
  2. Insert MDI into spacer
  3. Shake canister x 5 seconds.
  4. Hold the MDI upright
  5. Breathe out normally through your mouth.
  6. Put the mouthpiece between your teeth and close your lips tightly around mouthpiece of spacer, or, if using a mask, place the mask completely over your nose and mouth.
  7. Keep your tongue away from opening of spacer.
  8. Press down the top of the canister
  9. Breathe in deeply and slowly through your mouth x 3-5 seconds three to five seconds
  10. Hold breath x five seconds.
  11. Wait approximately 15 to 30 seconds between puffs
  12. Recap mouthpiece
  13. Rinse mouth
88
Q

What is the lifetime prevalence of asthma in Canada (CPS)?

A

1

89
Q

List 4 risk factors for ICU admission in an asthmatic (CPS)

A
  • Previous life-threatening events,
  • Admissions to ICU
  • Intubation
  • Deterioration while already on systemic steroids.
90
Q

What is a reasonable SO2 goal in asthma exacerbation (CPS)?

A

> =94%

91
Q

Ipatropium bromide should be used with caution in patients with what allergy (CPS)?

A

Soy allergy

92
Q

When should MgSO4 be used in acute asthma exacerbation (CPS)?

A

In moderate and severe asthma with incomplete response to conventional therapy during the first 1 to 2 h

93
Q

List 2 side effects of MgSO4

A

Hypotension

Bradycardia

94
Q

If you start an IV salbutamol infusion, what 3 things should you monitor? (CPS)

A
  1. Cardiac monitoring-risk of arrhythmia
  2. Glucose-hyperglycemia
  3. Lytes-hypokalemia
95
Q

List 2 serious complications of intubated asthmatics (CPS)

A

Pneumothorax

Impaired venous return–> Cardiovascular collapse

96
Q

What is the purpose of diagnosing asthma in preschoolers (age 1-5) (CPS)? Give 3 reasons.

A
  1. Wheezing in early life is associated with reduced lung function that can persist into adulthood
  2. Airway remodelling has been documented in toddlers
  3. Evidence indicates that recurrent preschool wheezing responds to inhaled corticosteroid (ICS) therapy
97
Q

Diagnostic criteria for asthma in preschoolers (age 1-5) according to CPS statement

A

Children with frequent (≥8 days/month) asthma-like symptoms or recurrent (≥2) exacerbations

  1. Documentation of airflow obstruction

Preferred: Documented wheezing and other signs of airflow obstruction by physician or trained health care practitioner

Alternative: Convincing parental report of wheezing or other symptoms
of airflow obstruction

  1. Documentation of reversibility of airflow obstruction

Preferred: Documented improvement in signs of airflow obstruction to SABA ± oral corticosteroids* by physician or trained health care practitioner

Alternative: Convincing parental report of symptomatic response to a 3-month trial of a medium dose of ICS (with as-needed SABA)

Alternative: Convincing parental report of symptomatic response to SABA

  1. No clinical evidence of an alternative diagnosis
98
Q

List 5 conditions in the differential for chronic wheezing

A 
Tracheomalacia
TEF
Vascular rings/slings
Cardiovascular disease with PA dilatation
FB aspiration
GER with microaspiration
Immunodeficiency
CF
PCD
BPD
Bronchiolitis obliterans
99
Q

When after administering ventolin and oral steroids should you reassess patient for response?

A

Ventolin-30 mins after 1st dose or 60 mins after vent BtoB x 3

Oral steroids-3-4 hours after dose

100
Q

In children 1-5 years of age with recurrent (≥2) episodes of asthma-like symptoms, no wheezing on presentation, what treatment is recommended and for how long (CPS)?

A

A therapeutic trial with a medium (200 µg to 250 µg) dose ICS and ventolin prn x 3 months to confirm reversibility of airflow obstruction

Clear consistent improvement in the frequency and severity of symptoms and/or exacerbations confirms the diagnosis

101
Q

In children1-5 years of age with recurrent (≥2) episodes of asthma-like symptoms and wheezing on presentation, what should you do to confirm diagnosis of asthma (CPS)?

A

Direct observation of improvement with inhaled bronchodilator (with or without oral corticosteroids) by HCP

102
Q

In suspected preschool asthma, when should you refer to specialist (CPS)?

A

Diagnostic uncertainty

Repeated (≥2) exacerbations requiring rescue oral corticosteroids or hospitalization or frequent symptoms (≥8 days/month) despite moderate (200 μg to 250 μg) daily doses of inhaled corticosteroids

Life-threatening event such as ICU admission

Need for allergy testing

103
Q

What is the most common cause of localized bronchiectasis in previously healthy children?

A

Foreign body

104
Q

What is Kartageners?

A

PCD + situs inversus (occurs in 50% of PCD)

105
Q

List 5 causes of chronic cough (>4 weeks)

A 
Psychogenic cough-"honking" cough
Post-viral
Asthma
Pertussis
Protracted bacterial bronchitis
Bronchiectasis
Postnasal drip
GERD
ILD
FB
Cardiac
106
Q

List 5 conditions that cause hemoptysis

A 
Bronchiectasis
FB
Airway trauma
Pnemonia/lung abscess
TB
Aspergilloma
Coagulopathy
Pulmonary capillaritis (Wegeners, Goodpastures, SLE, HSP)
Idiopathic pulmonary hemosiderosis
PE
Pulmonary AVM
CHD
107
Q

List 3 clinical, laboratory or radiographic features consistent with atypical pneumonia

A
  1. Subacute onset
  2. Prominent cough
  3. Minimal leukocytosis
  4. Nonlobar infiltrate
  5. Usually school-age
108
Q

List 10 complications of CF

A

Respiratory tract involvement

  • Chronic productive cough
  • Asthma
  • Airway colonization by pseudomonas, s. aureus, B. cepacia
  • Bronchiectasis
  • Atypical mycobacterial infection
  • ABPA
  • PTX 2ndary to subpleural blebs
  • Hemoptysis

Sinus disease

  • Sinusitis
  • Nasal polyps

GI

  • Pancreatic insufficiency
  • Recurrent pancreatitis (in pancreatic sufficiency patients)
  • Distal intestinal obstruction syndrome (DIOS)
  • Meconium ileus
  • Cystic fibrosis-related diabetes
  • Vitamin deficiencies
  • Prolonged neonatal jaundice
  • Biliary cirrhosis with portal hypertension
  • Cholelithiasis
  • Dermatitis resembling acrodermatitis enteropathica, with fatty acid and zinc deficiency
  • Rectal prolapse
  • Volvulus in fetal life

Genitourinary

  • Bilateral absence of vas deferens
  • Male infertility
  • Reduced female fertility

Other

  • Hypochloremic, hyponatremic alkalosis
  • Pseudotumor cerebri
  • Osteoporosis
  • Clubbing
  • Hypertrophic osteoarthropathy
  • Recurrent venous thrombosis
  • Nephrolithiasis and nephrocalcinosis
  • Delayed puberty
109
Q

What two complications increase the risk of liver disease in CF?

A

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

110
Q

Treatment of pulmonary exacerbation in CF (past SAQ)

A

-If mild →Tx with 2-3 weeks of PO antibiotics based on most recent sputum cultures; close f/u with repeated PFTs

-If moderate to severe
→admission to hospital, IV abx x 14 days, inhalational therapy, chest PT, supplemental O2, nutritional support

If concerned re: pseudomonas, need to double cover (e.g. Ceftazidime and gentamicin)

111
Q

Difference between bronchopulmonary sequestration and CPAM

A

Sequestration lacks normal communication with the tracheobronchial tree and that receives its arterial blood supply from the systemic circulation

CPAMs are connected to the tracheobronchial tree and are supplied from the pulmonary circulation

112
Q

List 2 conditions associated with extralobar pulmonary sequestration

A 
CDH
Vertebral anomalies
CHD
Pulmonary hypoplasia
Colonic duplication
113
Q

List 10 conditions with increased risk of OSA

A
  1. Adenotonsillar hypertrophy-most common
  2. Obesity
  3. Cerebral palsy
  4. T21
  5. Craniofacial anomalies (eg, retrognathia, micrognathia, midface hypoplasia)
  6. History of low birth weight
  7. Muscular dystrophy or other neuromuscular disorders
  8. Myelomeningocele
  9. Achondroplasia
  10. Mucopolysaccharidoses (eg, Hunter syndrome and Hurler syndrome)
  11. Prader-Willi syndrome
  12. Orthodontic problems (eg, high narrow hard palate, overlapping incisors, crossbite) 13. Family history of OSA
  13. History of prematurity and multiple gestation
114
Q

Formula for calculating BMI

A

Weight (in kg)/(height in m)^2

115
Q

List 2 diagnostic criteria for ARDS (past SAQ)

A
  1. Respiratory symptoms within one week
  2. Bilateral opacities consistent with pulmonary edema on CXR or CT
  3. Rule out cardiac failure or fluid overload
  4. A moderate to severe impairment of oxygenation must be present: Pao2/Fio2 <200
116
Q

List the 4 most common causes of BRUE

A
  1. No identified cause (50%)
  2. GERD
  3. Seizure
  4. LRTI (RSV, pertussis)
  5. NAI

Less common:
Cardiac disease, upper airway obstruction, metabolic disorders, anaphylaxis, bacterial infections (including UTI), intussuseption, toxic ingestion

117
Q

List 5 low risk characteristics of patients with BRUE

A
  1. Age >60 days
  2. Gestational age ≥32 weeks and postconceptional age ≥45 weeks
  3. Occurrence of only one BRUE
  4. Duration of BRUE <1 minute
  5. No CPR by a trained medical provider was required
  6. No concerning historical features:
    - Social risk factors for child abuse
    - Respiratory illness or exposure
    - Recent injury
    - Symptoms in days preceding the event (fever, fussiness, diarrhea or decreased intake)
    - Administration or access to medications
    - History of episodic vomiting or lethargy
    - Developmental delay or congenital anomalies
    - Family history of BRUE or sudden unexplained death in a sibling
  7. No concerning physical examination findings
    - Signs of injury, including bleeding or bruising
    - Bulging fontanelle
    - Altered sensorium
    - Fever or toxic appearance
    - Respiratory distress
    - Abdominal distension or vomiting
118
Q

Management for low risk BRUE

A

Education about BRUEs
Recommend parent CPR class
Consider brief in hospital observation (1-4 hours)
Follow up with HCP in 24 hours

119
Q

Investigations for BRUE in patients that do not meet low risk criteria

A 
CBC 
Lytes+extended lytes
Cr, BUN
Glucose
UA
CXR
ECG
Urine toxicology
Consider pertussis and RSV swabs
Consider metabolic w/u
120
Q

Empiric antibiotics for pulmonary abscess

A

Clindamycin+ Gentamicin (need anaerobic coverage

121
Q

What organisms are commobly involved in pulmonary abscess?

A

S. aureus
Oral anaerobes
Gram negatives

**Do pleural culture as often positive in pulmonary abscess

122
Q

How do you differentiate transudate and exudate using Light’s criteria?

A

Pleural fluid is exudate if one of the following is met:

  1. Pleural fluid protein/serum protein ratio > 0.5
  2. Pleural fluid LDH/serum LDH ratio > 0.6
  3. Pleural fluid LDH > 2/3 upper limit of the laboratory’s normal serum LDH
123
Q

List 3 characteristics of pleural fluid consistent with empyema

A
  1. Low pleural fluid glucose <3.33 mmol/L or a pleural fluid/serum glucose ratio <0.5
  2. Pleural fluid acidosis (pH < 7.3)
  3. Cell count >50,000/microL
  4. LDH>1000
  5. Pleural fluid protein/serum protein ratio > 0.5
  6. Pleural fluid LDH/serum LDH ratio > 0.6
124
Q

List 3 complications of chest tube

A

Malposition
Organ injury (heart, lungs, diaphragm, liver)
Infection

125
Q

Describe the clinical features of pleurodynia

A 
Lasts 3-6 days, up to 2 weeks
Fever
SEVERE spasmodic chest/abdo pain 
Pleuritic
Malaise, myalgias
Pleural friction rub
Normal CXR
126
Q

What 2 viruses most commonly cause pleurodynia?

A

Coxsackie

Echovirus

127
Q

List 4 groups at higher risk for severe disease from bronchiolitis (CPS)

A

Premature (<35 weeks GA)
<3 months of age at presentation
Hemodynamically significant cardiopulmonary disease
Immunodeficiency

FRCPC (27 decks)

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