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RACP Paediatrics Exam > Respiratory > Flashcards

Respiratory Flashcards

1
Q

Describe the diameter of the airways during inspiration and expiration.

A

Upper airways - Narrow during inspiration and dilated on expiration. More likely to have obstruction during inspiration.
Lower airways - Narrow during expiration and dilated during inspiration. More likely to have obstruction during expiration.

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2
Q

What is the most sensitive measure for small airway obstruction?

A

FEF 25-75%

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3
Q

When doing spirometry in 6-12 year old children, the flow volume curves should appear similar in configuration on repeat testing.
What is the maximum allowable variation on repeat testing?

A

5%

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4
Q

Which respiratory measure is the best parameter to evaluate the degree of respiratory muscular weakness in a child with neuromuscular disease?

A

FVC

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5
Q

Required change in FEV1/FVC after bronchodilator?

A

12% and 200mL

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6
Q

What level of FEV1 do you consider referral for lung transplant in CF?

A

<30%

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7
Q

What is the best prognostic respiratory parameter for CF?

A

FEV1

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8
Q

What does KCO correct for?

A

Alveolar loss - if DLCO is reduced but then normal on KCO this indicates there is loss of alveolar such as lobectomy that has been corrected for which normalises then result.

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9
Q

What modulator would you put a child with a G551D mutation on?

A

Ivacaftor (Kalydeco)

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10
Q

What modulator would you put a child with CF who is homozygous or heterozygous for F508del on?

A

Trikafta - Elexecaftor and tezacaftor and ivacaftor

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11
Q

At what age can you use tikafta?

A

Aged 6 and up

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12
Q

At what age can you be prescribed Ivacaftor?

A

Aged 2 and up

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13
Q

What are the side effects of trikafta?

A

Abnormal LFT
Rash
Anxiety/depression
Cataracts
Weight gain

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14
Q

What is indicative of a positive chloride sweat test in CF?

A

Sweat chloride of >60mmol/L
30-59mmol/L is indicative of CF or CF SPID

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15
Q

What is required for a diagnosis of CF?

A

Two cystic fibrosis genetic mutations and/or a positive sweat chloride test

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15
Q

What CFTR mutation class does F508del belong to?

A

Class two - trafficking defect

16
Q

What CFTR mutation class does G551D belong to?

A

Class three - gating defect

17
Q

What CFTR mutation class does G542X belong to?

A

Class one - No protein

18
Q

What CFTR mutation class does R117H belong to?

A

Class four - Less function

19
Q

The newborn screening algorithm for cystic fibrosis tests for?

A

IRT - genetic testing (blood trypsinogen)

20
Q

Diagnostic criteria for Allergic bronchopulmonary aspergillosis.

A
21
Q

What part of the brain does OSA affect?

A

Prefrontal cortex - Executive functioning

22
Q

What gene is related to congenital central hypoventilation syndrome? What tumour are they predisposed to?

A

PHOX2B - Polyalanine repeat gene
Neural crest tumours such as neuroblastoma

23
Q

Why do children with Prada Willi require sleep study prior to commencing GH therapy?

A

GH therapy can cause hypertrophy of the tonsils leading to OSA

24
Q

Treatment for narcolepsy?

A

Methylphenidate or Dexamphetamine
2nd line modafinil

25
Q

Cataplexy treatment

A

Best treatment sodium oxybate but difficult to get so usually use fluoxetine or venlafaxine first

26
Q

What is the gene mutation in cleidocranial dysplasia?

A

RUNX2

27
Q

In a child with congenital central hypoventilation syndrome and Hirschsprung disease what condition would you be thinking of?

A

Haddad Syndrome - suboptimal response to hypoxia and hypercarbia

28
Q

What would the exhaled nitric oxide test show in a child with asthma?

A

Raised exhaled nitric oxide >20ppb

29
Q

Step wise approach to asthma in children under 6 years.

A
  1. Salbutamol only
  2. Low dose ICS or montelukast
  3. High dose ICS or Low dose ICS + montelukast
30
Q

Step wise approach to asthma in children over 6 years.

A
  1. Salbutamol only
  2. Low dose ICS or montelukast
  3. High dose ICS or Low dose ICS + montelukast or Low dose ICS + LABA
31
Q

What is the target of omalizumab?

A

IgE

32
Q

What is the target of mepolizumab?

A

Eosinophils -Anti- IL-5

33
Q

What is the target of dupilumab?

A

IL-4Ra

34
Q

What are the clinical findings in pulmonary fibrosis?

A

Fine crackles
Clubbing of fingers

35
Q

A child with Duchenne muscular dystrophy, latest FVC has fallen to 55% what would this mean for him clinically regarding sleep disordered breathing and/or daytime breathing difficulties?

A

Sleep disordered breathing in REM sleep only.
FVC <60% REM disordered breathing
FVC <40% REM and non-REM disordered breathing
FVC <20-25% Diurnal respiratory failure

RACP Paediatrics Exam (25 decks)

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