Paediatric respiratory

Question 1

What are the classic clinical features of cystic fibrosis?

Answer 1

Suppurative lung disease, pancreatic exocrine insufficiency manifesting as steatorrhea and failure to thrive, male infertility, elevated sweat electrolytes

Question 2

What are the management priorities for cystic fibrosis

Answer 2

Nutrition: high-fat high calorie diet, pancreatic enzyme replacement, vitamin supplementation

Salt supplementation

Pulmonary care, chest physiotherapy, mx of infections with aggressive antibiotics

Question 3

what are the main features of mycoplasma pneumonia?

Answer 3

subacute course
prominent cough
+/- headache
+/- sore throat

Question 4

why do children with cerebral palsy frequently present to hospital with respiratory issues like pneumonia?

Answer 4

higher risk of aspiration
Poor cough
immobility and scoliosis
increased secretions (due to benzo use)

Question 5

which cough is typically heard in the morning?

Answer 5

bronchiectasis cough because secretions pool over night

Question 6

what is important to clarify with parents about the relationship between cough and vomit?

Answer 6

ask the parents- does vomiting occur AFTER episodes of coughing or does it occur WITHOUT coughing?

remember that children don’t expectorate well and often will swallow their mucous

Question 7

at what age group do we normally expect inhaled foreign bodies?

Answer 7

between 12 months (when neat pincer grip develops) and to 2-3 yrs old (toddler phase)

Question 8

what is the most common cause of chronic wet cough in children?

Answer 8

bronchitis- persistent infection in the large conducting airways

Question 9

how long do normal viral respiratory infections usually last in children?

Answer 9

1-2 weeks but in some children it can last up to a month

Question 10

what the radiological features of bronchiectasis on a CXR?

Answer 10

tram tracking
hyperinflation
patchy opacities

Question 11

what does a sweat test for CF involve for a child?

Answer 11

Put pilocarpine in the forearm and then put an electric current across it to induce sweat glands.
The sweat will be analysed for Chloride concentration.

> 60 mmol/L of Cl- is diagnostic of CF

Question 12

what are some common causes of chronic suppurative lung disease in children?

Answer 12

Cystic fibrosis
Primary ciliary dyskinesia
primary or secondary immunodeficiency
chronic severe pneumonia
TB
missed foreign body

Question 13

what are some causes of acquired/secondary immunodeficiency in children?

Answer 13

HIV/AIDs
malignancy + treatment
malnutrition
steroids use

Question 14

what are the clinical features of primary ciliary dyskinesia?

Answer 14

chronic rhinitis and sinusitis
chronic suppurative lung disease
OM
males are infertility
50% of patients have dextrocardia (kartagener's syndrome)

Question 15

what is a good screening test for primary ciliary dyskinesia

Answer 15

nasal nitric oxide- low measurement of NO can indicate PCD

Question 16

what does DIOS refer to in cystic fibrosis?

Answer 16

DIOS= distal ileum obstruction syndrome, a complication of CF.

essentially equivalent to meconium ileus
faecal matter becomes impacted in ileum causing obstruction

Question 17

how many classes of mutations relating to CFTR gene are there?

Answer 17

5 classes

Question 18

what are the complications/disease manifestations of CF?

Answer 18

pancreatic exocrine insufficiency–> malabsorption–> poor growth + poor bone health (decreased vitamin D) + other nutritional deficiencies

pancreatic destruction can lead to insulin requiring diabetes

prone to bowel obstruction (DIOS)

concurrent respiratory infections–> bronchiectasis–> failure to thrive –> chronic lung disease

biliary obstruction–> liver damage–> cirrhosis

infertility in males

chronic sinusitis–> nasal polyps–> obstructed nasal passage

Question 19

what are some general management considerations for patients with CF?

Answer 19

• Key aim is to improve longevity and QOL
• Daily chest physiotherapy to clear secretions
• Postural drainage (ending up sitting upright)
• Aggressive antibiotics for respiratory infections
• Good infection control
• Replacing pancreatic enzymes
Replacing nutrient deficiency such as vitamin D
• High calorie diet and optimising nutrition for growth
• Salt replacement
• –> lung transplantation if end-stage
• Encourage active play/exercise and PEP games
Psychosocial support

Question 20

what are the types of medications often used for CF patients?

Answer 20

• Bronchodilators- ventolin
  
  • Mucolytics e.g. dornase alfa or hypertonic saline
  • Corticosteroids post physiotherapy
  • Antibiotics (prophylactic)
  • Creon
  • Salt supplements

Question 21

CF patients are managed by a multidisciplinary team. Can you list the members of this team?

Answer 21

• CF physician/general paediatrician, GP
• Dietician 
• Physiotherapist
• Community nurse
• Genetic counsellor
• Pharmacist
• CF educator
Psychosocial supports--> CF Victoria

Question 22

what are some key routine ix required for children with CF?

Answer 22

• Bronchoalveolar lavage to culture sputum if the child cannot expectorate- dictates antibiotic regimen
• Or sputum culture
• HRCT- looking at disease progression
• Spirometry in children > 5 yrs old

FBE/UEC/LFTs/vitamin D- ongoing monitoring

Question 23

what is the ratio of H2Co3 to NaHCo3 to maintain normal physiological pH in the body?

Answer 23

1 H2Co3 (Co2) : 20 NaHCo3 (bicarb)

so if co2 drops, bicarb must also drop to maintain the 1:20 ratio

if co2 increases, bicarb must also increase

Question 24

when do we start to see compensation for respiratory/metabolic electrolyte derangement on ABGs?

Answer 24

If the lungs compensate for a metabolic abnormality, compensation occurs within hours (e.g. increase/decrease respiratory rate), but the kidneys take 2-4 days to compensate for respiratory abnormality.

Any acute respiratory problem will always be uncompensated, as the kidneys would not have had time to compensate.

Question 25

on ABGs, if you see a change in Co2 (high or low), but the bicarb is normal, what do you think? what will bicarb eventually do?

Answer 25

uncompensated respiratory acidosis/alkalosis

eventually the kidneys will try and compensate to normalise pH by increasing bicarb if Co2 is high or excreting bicarb if CO2 is low

Question 26

what changes on ABG may indicate an uncompensated metabolic acidosis/alkalosis?

Answer 26

normal Co2 levels

but high/low bicarb levels

Question 27

on the ABG you notice that both the CO2 and the Hco3 level is abnormal. what is happening? how might you further delineate your answer?

Answer 27

some sort of compensation for respiratory/metabolic acidosis/alkalosis is happening.

to work this out, you need to look at the pH. Is is full or partial compensation? Is the pH on the acid (low) or alkali (high) side? Whichever parameter would produce the pH is the primary disorder.

E.g.if pH is acidic (low), HCO3 is acidic (low) and pCO2 is alkalotic (low), the result would be partially compensated metabolic acidosis.

Question 28

what is the antibiotic of choice for mycoplasma pneumoniae pneumonia for a previously well child older than 1 yrs?

Answer 28

oral roxithromycin

Question 29

when should you think about admitting a child with pneumonia to hospital?

Answer 29

age less than 3 months
very unwell + working hard (resp distress), altered conscious state
CXR shows extensive consolidation
Evidence of pleural effusion/empyema

Question 30

what do we mean by ‘air trapping’ in the lungs on HRCT?

Answer 30

hyperinflation

Question 31

a child is admitted with pneumonia. despite IV antis, they are still febrile and you worry about an empyema. CXR shows blunting of costophrenic angle. how might we distinguish an empyema from pleural effusion in a child (think investigations)?

Answer 31

do a chest ultrasound

Question 32

why is it important to know which day it is since bronchiolitic symptoms start in a child you are assessing for bronchiolitis?

Answer 32

we know that bronchiolitis tends to get worse on day 2-3 before it gets better, so if an infant presents with day 1 bronch with mild WOB and still feeding, we would still admit them to SSU in case they deteriorate the next day and require respiratory/enteral support

Question 33

describe the asthma predictive index?

Answer 33

children are more likely to have asthma if:

they have 1 of- parental history of asthma, eczema, sensitivity to allergens

or if they have 2 of-allergic rhinitis, > 4% eosinophilia, food allergies, wheezing in non-URTI induced situations

Question 34

in a child who is wheezing and clinically appears to have asthma, but is unable to perform spirometry due to age, what can you do to confirm the diagnosis of asthma?

Answer 34

is it responsive to salbutamol????????

Question 35

what are some things on history which may indicate that a child’s asthma management is uncontrolled and needs to be reviewed?

Answer 35

Daytime symptoms > 2 times per week
Limitation to activities
Any nocturnal symptoms
Frequent need for reliever
multiple hospital presentations/exacerbations
PFTs less than 80% predicted (children over 5yrs)

Question 36

according to the RCH guidelines, how frequent should we give a puff of salbutamol to a 5 yr old child with a moderate asthma attack? what about a 7 yr old child?

Answer 36

for children under 6 yrs, 6 puffs every 20 mins for 1 hour

Review 10 mins post third dose

If >6yrs 12 puffs= 1 dose; so 12 puffs every 20mins for 1 hour then review

If the child is not responding after the first 6 or 12 puffs after 20 mins, call an ambulance

Question 37

what is atrovent?

Answer 37

ipratropium

Question 38

when might we consider discharge of an inpatient child who has had an asthma attack?

Answer 38

Adequate O2 sats
No WOB; clinically well
Tolerating oral feeds and fluids
Parents have been counselled and educated about asthma and medications etc

Question 39

when might we consider commencing a preventer for an asthmatic child?

Answer 39

if they are requiring salbutamol more than twice per week, have difficulty participating in activities/sport, have persistent day time or nocturnal symptoms

Question 40

first line treatment options for preventers in asthmatic kids?

Answer 40

low dose inhaled steroids
or
montelukast- LT receptor antagonist

Question 41

how long does it take for oral prednisolone to work in an acute asthma attack, and what is the dose that we give to children?

Answer 41

1-2mg/kg daily for a child of oral prednisolone

approx 6-8hrs for it to work!

Question 42

what is singulair?

Answer 42

montelukast

Question 43

what is the difference between a preventer and a controller in asthma medication?

Answer 43

preventer= single drug usually inhaled corticosteroid or montelukast.

controller= corticosteroid + LABA

Question 44

what is a good indicator of a severe asthma attack, which should prompt parents to seek urgent medical attention for their child?

Answer 44

if their child is unable to talk at all they should come into ED

Question 45

what are the medications/drugs we use in a severe/deteriorating asthma attack?

Answer 45

Aminophylline IV
Magnesium sulfate IV
Ipratroprium (adrovent) nebulised
Salbutamol IV/nebulised
oral prednisolone/IV methylprednisolone 
02

Question 46

what does ‘an salbutamol burst’ and ‘spacing out’ refer to in paediatric asthma management?

Answer 46

a ‘burst’ is when we use 6 or 12 puffs every 20 mins (depending on child age) for 1 hour and then reassess.

So 3 x 6 or 12 puffs = a ‘burst’

‘Spacing out’ refers to increasing the time period between each salbutamol administration. Once the child can receive the ventolin every 4 hrly, then they can be discharged from hospital.

Question 47

a child comes in for their 2nd admission of viral induced asthma. They do not get interval/nocturnal or exercise symptoms. do they need a preventer?

Answer 47

NO

very limited role of preventer medication in viral induced asthma

Question 48

at what age should we use a mask with a spacer for asthma?

Answer 48

generally under 4 yrs we use a mask attached to the spacer

or for any child of any age who is unable to maintain a tight seal around the mouthpiece

Question 49

what volume spacer is recommended by the RCH for administering salbutamol?

Answer 49

small volume spacers can be used for children of all ages.

large volume spacers can be used in children aged 6yrs and above

Question 50

you start a child on a preventer medication for asthma. what are some things you need to consider and how would you educate the parent/patient?

Answer 50

Preventer medications need to be taken EVERY DAY even when the patient is asymptomatic.
- you can put reminder alarms on the phone or place the medication near your toothbrush to remind you to take it every day.

If using montelukast- there is a chance it may not be effective in any child.

The child will need regular follow up review if put on a preventer.

If symptoms of asthma cannot be adequately controlled with preventer despite good compliance, then may consider steroid+ LABA (controller) medication such as Seretide

Question 51

what might you advise a child who gets exercise induced asthma?

Answer 51

take 2 puffs of salbutamol prior to starting exercise. May need to repeat the dose if symptoms occur during exercise

Question 52

what is flixotide?

Answer 52

fluticasone

Question 53

what is the pharmacological management of a mild asthma attack (e.g. child has some wheeze but is able to talk normally with subtle WOB).

Answer 53

salbutamol PRN

review in 20 mins

oral prednisolone 1-2mg/kg daily for 3 days if required (e.g. symptoms refractive to salbutamol administration)

Question 54

what is the pharmacological management of a MODERATE asthma attack? e.g. child has some increased work of breathing, some limitation of ability to talk, and lowish sats?

Answer 54

Salbutamol 6 or 12 puffs for 1 hr every 20 mins

oxygen if sats are less than 92%

oral prednisolone 2mg/kg then continuing on 1mg/kg for further 1-2 days post asthma attack

Question 55

a child has been an inpatient for bacterial pneumonia. they were treated with IV fluclox and gent while an inpatient. what antibiotic should they go home on?

Answer 55

oral amoxycillin

Question 56

what does bronchopulmonary dysplasia refer to?

Answer 56

children who are born prematurely and require supplemental oxygen beyond 28 days of life may have long term chronic lung changes.

–> fibrosis and cystic change

–> more susceptible for respiratory tract infections

–> reduced exercise tolerance

Question 57

what is the difference between stertor and stridor?

Answer 57

stridor is the abnormal high pitched sound produced by turbulent airflow through a partially obstructed airway at the level of the larynx or below.

stertor- low pitched snoring sound generated above the larynx.
usually presents as noisy breathing during inspiration and expiration

Question 58

how do we medically manage mild- moderate croup?

Answer 58

Prednisolone1mg/kg, AND prescribe a second dose for the next evening.
OR

a single dose ofOral Dexamethasone0.15mg/kg.

Question 59

how do we medically mange severe croup?

Answer 59

Nebulised adrenaline (1 mL of 1% adrenaline solutionplus 3mlNormal Saline, or 4ml of adrenaline 1:1000.)

Give 0.6mg/kg (max 12mg) IM/IV dexamethasone

Question 60

describe an asthma management plan

Answer 60

Asthma Management Plan
(Also recommend annual influenza vaccine.)

• Daily treatment:
o If you have been prescribed a preventer ‐ you should use it every day.
o If you have a spacer ‐ you should use it for your preventer.
o If you use an inhaled steroid (eg. Flixotide, Becotide, Pulmicort) ‐ you should rinse you mouth out after using it.

• Treatment of symptoms:
o For mild symptoms: take 2 puffs of Ventolin
o Use your spacer if you have one. Repeat doses as often as you need to. Don’t stop taking your preventer. If you need Ventolin more often than every 3 hours, then see your doctor or go to hospital.

• Treatment of acute exacerbation:
o For more severe symptoms: take up to 12 puffs of Ventolin (if > 6 years old).
 Sit down and remain calm.
 MDI + Spacer: 4 separate puffs, 4 breaths per puff.
 Wait 4 minutes. If there is no improvement, repeat previous step.
 If there is still no improvement, call the ambulance and repeat puffs of Ventolin while waiting.
o If from previous experience you suspect this is a more severe attack, or if the symptoms are not getting better in about 6 to 8 hours with regular use of Ventolin take prednisolone (1 mg/kg) immediately then once each morning for up to 3 days.

• Seek help from the doctor/hospital if:
o If you have a bad attack or are worried.
o If you need Ventolin more than every 3 hours.
o If you get little or no relief from Ventolin.
o Wheezing lasts more than 24 hours and is not getting better.
o If you have a very severe attack: call an ambulance & take up to 12 puffs of Ventolin every 15 to 30 minutes

Question 61

what are the signs that asthma control is deteriorating?

Answer 61

• you have night-time wheezing, coughing or chest tightness
• you have morning asthma symptoms when you wake up
• you need to take your reliever more than usual eg. more than 3 times per week
• your asthma is interfering with your usual activities