Respirology

Question 1

CHARGE syndrome

Answer 1

Coloboma
Heart disease
Atresia choanae
Retarded growth+ development or CNS anomalies or both
Genital anomalies or hypogonadism or both
Ear anomalies or deafness or both

Question 2

2012 Asthma guidelines for good asthma control

Answer 2

Daytime sx: < 4 days /week
Activity: normal
Nighttime sx: < 1 night /week
Exacerbations: mild, infrequent
Absence from work or school: none
FEV1 or PEF >= 90% of personal best
other:
PEF diurnal variation < 10-15% 
sputum eosinophils < 2-3%

Question 3

False negative sweat chloride test

Answer 3

1. Peripheral edema
2. Insufficient quantity
3. Hyponatremia
4. Dilution
5. CF transmembrane conductance regulator mutations with preserved sweat duct function

Question 4

False positive sweat chloride test

Answer 4

1. Atopic dermatitis
2. Ectodermal dysplasia
3. Malnutrition
4. Congenital adrenal hyperplasia
5. Adrenal insufficiency
6. Hypothyroidism
7. Glucose-6-phosphatase deficiency
8. Nephrogenic diabetes insipidus
9. Munchausen syndrome by proxy
   and more

Question 5

Common organisms pulmonary exacerbation CF

Answer 5

Younger children = MSSA
Young adulthood = Pseudomonas
Colonizing pathogens: MSSA, MRSA, Pseudomonas, haemophilus, burkhorderia, stenotrophomonas
Burkholderia cepacia complex = deteriorate very quickly

Question 6

Asthma escalation 12 yrs old (CTS guidelines)

Answer 6

• once 12 yrs old can add LABA to low dose ICS

- if 11 yrs increase ICS to medium dose from low dose

Question 7

Bronchiolitis O2 saturation threshold

Answer 7

90%

Question 8

RSV prophylaxis qualification

must get, reasonable to offer, consider for

Answer 8

SHOULD get:
1. significant CHD or CLD who require ongoing treatment if <12mo
2. preterm infants <36wks in remote communities who would require air transport for hospitalization and <6mo
MAY get:
1. still on O2 with CLD and 2nd RSV season
2. prems <30 weeks without CLD and <6mo
CONSIDER for:
1. term Inuit infants <6mo if community with high rates of hospitalization (e.g. Baffin)
2. <24mo if on home O2, had prolonged hospitalization for severe pulm disease, or severe immunocompromise

Question 9

Truncated inspiratory loop on spirometry

Answer 9

vocal cord dysfunction

Question 10

Diagnosis of asthma

Answer 10

1. obstruction (decreased FEV1/FVC) + increase in FEV1 by >= 12% with bronchodilator
2. peak flow: increase by 20+ % with bronchodilator
3. methacholine +Ve = <4mg/mL

Exercise: worsening in FEV1 by >= 15%

Question 11

Congenital central hypoventilation syndrome - At risk for….

Answer 11

Holter monitor annually - at risk of asystole (may need pacemaker)
Also at risk for neuroblastoma, neural crest tumors, Hirschsprung disease
Dont let them swim!

Question 12

Camphor ingestion (hydrocarbon) pulmonary findings

Answer 12

Pneumatoceles and pleural effusions may occur within 48-72 hrs

Question 13

ICS and height

Answer 13

• slight decrease in height

-1.1cm for budesonide 400mcg daily dose

Question 14

chronic cough duration

Answer 14

4+ weeks

order CXR and spirometry!

Question 15

Consequences of missed foreign body

Answer 15

• recurrent pneumonia
• bronchiectasis
• cardiac arrest and death

Question 16

CF presentations - infancy

Answer 16

• FTT
• meconium ileus
• recurrent resp sx
• hyponatremic, hypochloremic, metabolic alkalosis
• prolonged jaundice
• severe pneumonia

Question 17

CF presentations - childhood

Answer 17

• recurrent resp sx
• FTT
• recurrent rectal prolapse
• clubbing
• bronchiectasis
• nasal polyps
• chronic pseudomonas colonization

Question 18

CF diagnosis

Answer 18

• sweat chloride >= 60
• CFTR analysis
• NBS misses 1-5% (immunoreactive trypsinogen)

Question 19

Poor prognostic factors in CF

Answer 19

• female
• lung disease (FEV1, burkhorderia, PTX, massive hemoptysis)
• nutritional status +DM

Question 20

PCD diagnostic options

Answer 20

• nasal nitric oxide measurements (95%)
• genetics
• electron microscopy of cilia

Question 21

consequences of OSA

Answer 21

• FTT
• inflammatory
• QoL
• cardiovascular: HTN, cor pulmonale
• neurocognitive: behavioral, attention, school performance, development

Question 22

OSA diagnosis

Answer 22

PSG = gold standard

Question 23

Treatment adenoidal hypertrophy

Answer 23

Non-pharm: nasal hygiene
- sleep hygiene
Pharm: intranasal corticosteroids, montelukast
Surgical: adenoidectomy +/- tonsillectomy

Question 24

CPAM presentation neonatal (if symptomatic)

Answer 24

• resp distress

CXR: cystic mass, +/-mediastinal shift away from lesion

Question 25

Trach emergency response - initial steps

Answer 25

call for help

1. manually ventilate with 100% oxygen and attempt suctioning
2. remove trach
3. insert new trach

Question 26

Sensitivity

Answer 26

true positive/ (true positive + false negative)

number positive by test/total number positive

Question 27

Specificity

Answer 27

true negative /(true negative + false positive)

number negative by test/ total number without disease

Question 28

Cough DDX in first few months of life

Answer 28

CRADLE

• CF
• RTIs
• Aspiration e.g. FBA, GERD
• Dyskinetic cilia
• Lung and airway malformations e.g. laryngeal web
• Edema e.g. CHF

Question 29

Positive predictive value

Answer 29

TP/(TP+FP)

= true positive / total positive

Question 30

Negative predictive value

Answer 30

TN/ (FN+TN)

= true negative/total negative