Respiratory Flashcards
Describe the diameter of the airways during inspiration and expiration.
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.
What is the most sensitive measure for small airway obstruction?
FEF 25-75%
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?
5%
Which respiratory measure is the best parameter to evaluate the degree of respiratory muscular weakness in a child with neuromuscular disease?
FVC
Required change in FEV1/FVC after bronchodilator?
12% and 200mL
What level of FEV1 do you consider referral for lung transplant in CF?
<30%
What is the best prognostic respiratory parameter for CF?
FEV1
What does KCO correct for?
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.
What modulator would you put a child with a G551D mutation on?
Ivacaftor (Kalydeco)
What modulator would you put a child with CF who is homozygous or heterozygous for F508del on?
Trikafta - Elexecaftor and tezacaftor and ivacaftor
At what age can you use tikafta?
Aged 6 and up
At what age can you be prescribed Ivacaftor?
Aged 2 and up
What are the side effects of trikafta?
Abnormal LFT
Rash
Anxiety/depression
Cataracts
Weight gain
What is indicative of a positive chloride sweat test in CF?
Sweat chloride of >60mmol/L
30-59mmol/L is indicative of CF or CF SPID
What is required for a diagnosis of CF?
Two cystic fibrosis genetic mutations and/or a positive sweat chloride test
What CFTR mutation class does F508del belong to?
Class two - trafficking defect
What CFTR mutation class does G551D belong to?
Class three - gating defect
What CFTR mutation class does G542X belong to?
Class one - No protein
What CFTR mutation class does R117H belong to?
Class four - Less function
The newborn screening algorithm for cystic fibrosis tests for?
IRT - genetic testing (blood trypsinogen)
Diagnostic criteria for Allergic bronchopulmonary aspergillosis.
What part of the brain does OSA affect?
Prefrontal cortex - Executive functioning
What gene is related to congenital central hypoventilation syndrome? What tumour are they predisposed to?
PHOX2B - Polyalanine repeat gene
Neural crest tumours such as neuroblastoma
Why do children with Prada Willi require sleep study prior to commencing GH therapy?
GH therapy can cause hypertrophy of the tonsils leading to OSA
Treatment for narcolepsy?
Methylphenidate or Dexamphetamine
2nd line modafinil
Cataplexy treatment
Best treatment sodium oxybate but difficult to get so usually use fluoxetine or venlafaxine first
What is the gene mutation in cleidocranial dysplasia?
RUNX2
In a child with congenital central hypoventilation syndrome and Hirschsprung disease what condition would you be thinking of?
Haddad Syndrome - suboptimal response to hypoxia and hypercarbia
What would the exhaled nitric oxide test show in a child with asthma?
Raised exhaled nitric oxide >20ppb
Step wise approach to asthma in children under 6 years.
- Salbutamol only
- Low dose ICS or montelukast
- High dose ICS or Low dose ICS + montelukast
Step wise approach to asthma in children over 6 years.
- Salbutamol only
- Low dose ICS or montelukast
- High dose ICS or Low dose ICS + montelukast or Low dose ICS + LABA
What is the target of omalizumab?
IgE
What is the target of mepolizumab?
Eosinophils -Anti- IL-5
What is the target of dupilumab?
IL-4Ra
What are the clinical findings in pulmonary fibrosis?
Fine crackles
Clubbing of fingers
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?
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
