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Paediatrics > Paediatrics Respiratory > Flashcards

Paediatrics Respiratory Flashcards

1
Q

What is bronchiolitis?

A

Inflammation and infection in the bronchioles (small airways of the lungs)

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2
Q

What is bronchiolitis usually caused by?

A

Virus - respiratory syncytial virus is the most common cause

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3
Q

When does bronchiolitis typically occur?

A

Children under 1 year (most common in children under 6 months)

Rarely its diagnosed in children up to 2 years of age, particularly in ex-premature babies with chronic lung disease

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4
Q

What happens when a virus affects the airways of adults?

A

Swelling and mucus are proportionally so small that it has little noticable effect on breathing

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5
Q

How does bronchiolitis present?

A

Coryzal symptoms. These are the typical symptoms of a viral upper respiratory tract infection: running or snotty nose, sneezing, mucus in throat and watery eyes.

Signs of respiratory distress

Dyspnoea (heavy laboured breathing)

Tachypnoea (fast breathing)

Poor feeding

Mild fever (under 39ºC)

Apnoeas (episodes where the child stops breathing)

Wheeze and crackles on auscultation

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6
Q

What are coryzal symptoms?

A
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7
Q

What are the signs of respiratory distress in paediatrics?

A

Raised respiratory rate

Use of accessory muscles of breathing, such as the sternocleidomastoid, abdominal and intercostal muscles

Intercostal and subcostal recessions

Nasal flaring

Head bobbing

Tracheal tugging

Cyanosis (due to low oxygen saturation)

Abnormal airway noises

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8
Q

What abnormal airway noises can be heard during bronchiolitis?

A

Wheezing = whistling sound caused by narrowed airways, typically heard during expiration

Grunting is caused by exhaling with the glottis partially closed to increase positive end-expiratory pressure

Stridor is a high pitched inspiratory noise caused by obstruction of the upper airway, for example in croup

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9
Q

What is the course of respiratory syncytial virus?

A
  • Starts as URTI with coryzal symptoms
  • 1-2 days after onset of coryzal symptoms chest symptoms develop
  • Symptoms are worst on day 3 or 4
  • Symptoms usually last 7 to 10 day
  • Full recover within 2-3 weeks
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10
Q

What can result from bronchiolitis in infancy?

A

Viral induced wheeze during childhood

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11
Q

Why may a child be admitted for bronchiolitis?

A
  • Aged under 3 months or any pre-existing condition such as prematurity, Downs syndrome or cystic fibrosis
  • 50 – 75% or less of their normal intake of milk
  • Clinical dehydration
  • Respiratory rate above 70
  • Oxygen saturations below 92%
  • Moderate to severe respiratory distress, such as deep recessions or head bobbing
  • Apnoeas
  • Parents not confident in their ability to manage at home or difficulty accessing medical help from home
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12
Q

What is the management of bronchiolitis?

A

Supportive management:

  • Ensuring adequate intake. This could be orally, via NG tube or IV fluids depending on the severity. It is important to avoid overfeeding as a full stomach will restrict breathing.
  • Saline nasal drops and nasal suctioning can help clear nasal secretions
  • Supplementary oxygen if the oxygen saturations remain below 92%
  • Ventilatory support if required
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13
Q

What is there limited evidence for treating bronchiolitis with?

A

Nebulised saline

Bronchodilators

Steroids

Antibiotics

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14
Q

What are the ventilatory support options for brochiolitis treatment?

A

High-flow humidified oxygen via tight nasal cannula (i.e. “Airvo” or “Optiflow”). It adds “positive end-expiratory pressure” (PEEP) to maintain the airway at the end of expiration.

Continuous positive airway pressure (CPAP). Similar way to Airvo or Optiflow, but can deliver much higher and more controlled pressures.

Intubation and ventilation. This involves inserting an endotracheal tube into the trachea to fully control ventilation.

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15
Q

How can ventilation be assessed in paediatric patients?

A

Capillary blood gases are useful in severe respiratory distress

Rising pCO2

Falling pH (respiratory acidosis = type 2 respiratory failure)

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16
Q

What can be given as prophylaxis against bronchiolitis caused by RSV? Who is it given to?

A

Palivizumab (monoclonal antibody) given as a monthly injection as prevention against bronchiolitis

Extremely-premature and those with congenital heart disease

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17
Q

Is palivizumab a true vaccine?

A

No as it does not stimulat the infant’s immune system - instead provides passive protection by circulating the body until the virus is encountered, at which point it works as an antibody against the virus (levels of circulating antibodies decrease over time which is why a monthly injection is required)

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18
Q

What is a viral induced wheeze?

A

Acute wheezy illness caused by a viral infection (due to inflammation, oedema, swelling of the walls of the airways, contraction of smooth muscles of the airways)

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19
Q

What typically causes a viral induced wheeze in children?

A

RSV or rhinovirus

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20
Q

What law states that flow rate is proportional to the radius of the tube to the power 4?

A

Poiseuille’s law

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21
Q

How can a viral induced wheeze be distinguished from asthma?

A

Not definitive but:

  • Presenting before 3 years of age
  • No atopic history
  • Only occurs during viral infections
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22
Q

How does a viral induced wheeze present?

A

Shortness of breath

Signs of respiratory distress

Expiratory wheeze throughout the chest

(neither viral induced wheeze or asthma cause a focal wheeze - if you hear this then be very cautious and investigate further for foreign body or tumour)

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23
Q

What is the management of viral induced wheeze?

A

Same as acute asthma in children

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24
Q

What is an acute exacerbation of asthma characterised by?

A

Rapid deterioration in the symptoms of asthma (could br triggered by any of the normal triggers of asthma e.g. infection, exercise or cold weather)

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25
Q

How does an acute exacerbation of asthma present?

A

Progressively worsening shortness of breath

Signs of respiratory distress

Fast respiratory rate (tachypnoea)

Expiratory wheeze on auscultation heard throughout the chest

The chest can sound “tight” on auscultation, with reduced air entry

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26
Q

What are the signs of moderate, severe and life-theatening asthma?

A
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27
Q

What are the components of management of acute asthma / viral induced wheeze?

A

Supplementary oxygen if required (i.e. oxygen saturations less than 94% or working hard)

Bronchodilators (e.g. salbutamol, ipratropium and magnesium sulphate)

Steroids to reduce airway inflammation: prednisone (orally) or hydrocortisone (intravenous)

Antibiotics only if a bacterial cause is suspected (e.g. amoxicillin or erythromycin)

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28
Q

How can bronchodilators be stepped up in acute asthma?

A

Inhaled or nebulised salbutamol (a beta-2 agonist)

Inhaled or nebulised ipratropium bromide (an anti-muscarinic)

IV magnesium sulphate

IV aminophylline

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29
Q

How can mild cases of acute asthma be managed?

A

As an outpatient with regular salbutamol inhalers via a spacer (e.g. 4-6 puffs every 4 hours)

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30
Q

What is the stepwise approach for moderate to severe cases?

A

Salbutamol inhalers via a spacer device: starting with 10 puffs every 2 hours

Nebulisers with salbutamol / ipratropium bromide

Oral prednisone (e.g. 1mg per kg of body weight once a day for 3 days)

IV hydrocortisone

IV magnesium sulphate

IV salbutamol

IV aminophylline

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31
Q

If all the steps of acute asthma management have been covered, what is the next step?

A

Anaesthetist and ICU need calling (intubation and ventilation)

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32
Q

How to review a patient with acute asthma?

A
  • Review prior to next dose of bronchodilator
  • Look for evidence of cyanosis (central or peripheral), tracheal tug, subcostal recessions, hypoxia, tachypnoea or wheeze on auscultation
  • If they look well then consider stepping down the number and frequency of intervention
  • Step down inhaled salbutamol: 10 puffs 2 hourly, 10 puffs 4 hourly, 6 puffs 4 hourly, then 4 puffs 6 hourly
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33
Q

What else may need monitoring when on high doses of salbutamol?

A

Serum potassium (causes potassium to be absorbed from the blood into the cells)

Salbutamol can cause tachycardia and a tremor

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34
Q

What to advise on discharge of a patient with acute asthma?

A
  • Finish course of steriods (typically 3 days in total)
  • Provide safety-net information about when to seek help
  • Provide individualised written asthma action plan
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35
Q

What is asthma?

A

Chronic inflammatory airway disease

Smooth muscle airways is hypersensitive and responds to stimuli by constricting and causing airway obstruction

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36
Q

Name some other atopic conditions?

A

Asthma

Eczema

Hay fever

Food allergies

(run in families so ask FH)

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37
Q

What presentation typically suggests asthma?

A
  • Episodic (intermittent exacerbations)
  • Diurnal variaion (worse at night)
  • Dry cough with wheeze and SoB
  • Typical triggers
  • FH of atopy
  • Widespread polyphonic wheeze
  • Improves with bronchodilators
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38
Q

What respiratory symptoms suggest a diagnosis other than asthma?

A

Wheeze only related to coughs and colds (viral induced wheeze)

Productive cough

No response to treatment

Unilateral wheeze suggesting focal lesion or inhaled foreign body

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39
Q

Name some typical triggers for asthma?

A
  • Dust
  • Animals
  • Cold air
  • Exercise
  • Smoke
  • Food allergens (e.g. peanuts, shellfish or eggs)
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40
Q

How is asthma diagnosed?

A

No gold standard

Clinical on history and examination

Usually after 2 to 3 years old

If low possibility then refer to specialist for diagnosis

If high possibility then trial of treatment with diagnosis if symptoms improve

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41
Q

What tests can help with diagnosis of asthma?

A

Spirometry with reversibility testing (in children aged over 5)

Direct bronchial challenge test with histamine or methacholine

Fractional exhaled nitric oxide (FeNO)

Peak flow variability (measured several times a day for 2 to 4 weeks)

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42
Q

What are the principles for the stepwise ladder for asthma?

A

Start at the most appropriate step for severity of symptoms

Review regularly

Step up and down ladder based on symptoms

Aim for no symptoms or exacerbations on the lowest dose

Always check inhaler technique and adherence at each review

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43
Q

What is the medical therapy for asthma in under 5?

A
  • Start SABA (e.g. salbutamol)
  • Add low dose corticosteroid inhaler or leukotriene antagonist (i.e. oral montelukast)
  • Try both of the above
  • Refer to specialist
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44
Q

What is the medical treatment for asthma in patients 5-12?

A
  • SABA
  • Regular low dose cortiosteroid inhaler
  • Add a LABA (e.g. salmeterol)
  • Titrate up the corticosteriod inhaler to a medium dose
  • Add oral leukotriene receptor antagonist e.g. montelukast or oral theophylline
  • Increase the dose of the inhaled corticosteroid to a high dose
  • Referral to a specialist (may require daily oral steroids)
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45
Q

What is the medical treatment for asthma in patients over 12?

A
  • SABA as required
  • Regular low dose corticosteroid inhaler
  • LABA (continue only if good response)
  • Titrate up corticosteroid dose to medium
  • Trial leukotriene receptor antagonist / oral theophylline / LAMA (i.e. tiotropium)
  • Titrate up inhaled corticosteroid to high
  • Combine options from step 5
  • Refer to specialist
  • Add oral steroids at the lowest dose possible to achieve good control
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46
Q

Can inhaled steroids slow growth? What is the argument for them?

A

Yes up to 1cm when used longer than 12 months

It prevents asthma attacks which could lead to high doses of oral steroids, poorly controlled asthma can lead to a more significant impact on growth and development.

Child has regular reviews to ensure they’re on minimum dose

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47
Q

What is a complication of poor inhaler technique?

A

Medication in the mouth - reduces effectiveness and causes complications such as oral thrush

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48
Q

How to used a metered dose inhaler?

A
  • Remove cap
  • Shake inhaler
  • Sit / stand up straight
  • Lift the chin
  • Fully exhale
  • Make a tight seal
  • Take a steady breath in whilst pressing
  • Hold breath for 10 seconds
  • Wait 30 seconds before further dose
  • Rinse mouth after using steroid inhaler
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49
Q

How to use a metered dosed inhaler with a spacer?

A
  • Assemble spacer
  • Shake
  • Attach inhaler to correct end
  • Sit / stand straight
  • Lift chin slightly
  • Make a seal around mouthpiece
  • Spray dose into spacer
  • Take steady breaths in and out until mist is inhaled

Clean once a month - avoid scrubbing inside and allow them to air dry to avoid static (can interact with mist and prevent inhalation)

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50
Q

What is pneumonia caused by?

A

Bacteria

Virus

Atypical bacteria e.g. mycoplasma

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51
Q

How does pneumonia present in children?

A

Cough (wet and productive)

High fever (>38.5)

Tachypnoea

Tachycardia

Increased work of breathing

Lethargy

Delerium (acute confusion associated with infection)

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52
Q

What additional signs for pneumonia in children?

A

Hypoxia (low oxygen)

Hypotension (shock)

Fever

Confusion

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53
Q

What are the chest signs of pneumonia in children?

A

Bronchial breath sounds: harsh breath sounds equally loud on inspiration and expiration

Focal coarse crackles: caused by air passing through sputum similar to using a straw to blow into a drink

Dullness to percussion due to tissue collapse / consolidation

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54
Q

What are the bacterial causes of pneumonia in children?

A

Streptococcus pneumonia

Group A strep (e.g. Streptococcus pyogenes)

Group B strep occurs in pre-vaccinated infants, often contracted during birth as it often colonises the vagina.

Staphylococcus aureus. This causes typical chest xray findings of pneumatocoeles (round air filled cavities) and consolidations in multiple lobes.

Haemophilus influenza particularly affects pre-vaccinated or unvaccinated children.

Mycoplasma pneumonia, an atypical bacteria with extra-pulmonary manifestations (e.g. erythema multiforme).

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55
Q

What are the viral causes of pneumonia in children?

A

Respiratory syncytial virus is the most common

Parainfluenza virus

Influenza virus

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56
Q

What are the investigations for pneumonia in children?

A

Chest X-ray (not routinely required)

Sputum cultures / throat swabs for bacterial culture / viral PCR to find causative organism and guide treatment

Blood cultures for sepsis

Capillary blood gas analysis to monitor respiratory / metabolic acidosis and the blood lactate level in unwell patients

57
Q

What is the management of pneumonia in children?

A

Antibiotics according to local guidelines

Amoxicillin is often first line

Macrolide (erythromycin, clarithromycin or azithromycin) will cover atypical pneumonia or as monotherapy in patients with a penicillin allergy

IV abx if sepsis or problem with intestinal absorption

Oxygen to keep sats above 92%

58
Q

What should be looked for in a child with recurrent LRTIs?

A

Reflux

Aspiration

Neurological disease

Heart disease

Asthma

CF

Primary ciliary dyskinesia

Immune deficiency

59
Q

What tests can be done for children with recurrent LRTI?

A

Full blood count for WBCs

Chest xray - any structural abnormality in the chest or scarring from the infections.

Serum immunoglobulins - selective antibody deficiency.

Test immunoglobulin G to previous vaccines (i.e. pneumococcus and haemophilus). Some patients are unable to convert IgM to IgG, and therefore cannot form long term immunity to that bug. This is called an immunoglobulin class-switch recombination deficiency.

Sweat test to check for cystic fibrosis.

HIV test, especially if mum’s status is unknown or positive.

60
Q

What is croup?

A

Acute infective respiratory disease of children aged 6 months to 2 years

URTI causing oedema in the larynx

61
Q

What are the causes of croup?

A

Parainfluenza (most common)

Influenza

Adenovirus

Respiratory syncytial virus

62
Q

What caused croup previously

A

Diphtheria leading to epiglottitis with a high mortality - vaccination means that this is rare in developed countries

63
Q

How does croup present?

A

Increased work of breathing

Barking cough in episodes

Hoarse voice

Stridor

Low grade fever

64
Q

What is the management of croup?

A

Most managed at home with fluids and rest

Sit upright during attacks

Measures to avoid spread e.g. hand washing and staying off school

Stepwise options in severe croup:

  • Oral dexamethasone (150mcg/kg - can be repeated after 12 hours, prednisolone is an alternative e.g. at GPs)
  • Oxygen
  • Nebulised budesonide
  • Nebulised adrenaline
  • Intubation and ventilation
65
Q

What is epiglottitis?

A

Inflammation and swelling of the epiglottis caused by infection, typically with haemophilus influenza type B

66
Q

Is epiglottitis an emergency?

A

Yes but its rare now due to routine vaccination against haemophilus influenza type B

67
Q

How does epiglottitis present?

A

Similar way to croup by with more rapid onset

  • Fever
  • Sore throat and stridor
  • Sitting forward and drooling
  • Tripod position
  • Muffled voice
  • Scared and quiet child
68
Q

What are the investigation of epiglottitis?

A

If acutely unwell then treat before

Lateral xray of the neck shows a characteristic thumbprint sign caused by the oedematous and swollen epiglottis - also useful to exclude a foreign body

69
Q

What is the management of epiglottitis?

A

Do not distress patient

Alert most senior paediatrician and anaesthetist available

Intubation should be prepared for incase of sudden upper airway closure

Tracheostomy may be required in ICU

Once airway is secured then: IV abx (ceftriaxone) or steroids (dexamethasone)

70
Q

What is a complication of epiglottitis?

A

Epiglottic abscess which is a collection of pus around the epiglottis also threatening the airwary making it a life threatening emergency - treatment similar to epiglottitis

71
Q

What is laryngomalacia?

A

Part of larynx above vocal cords (supraglottic larynx) is structured in a way that allows it to cause partial airway obstruction causing chronic stridor on inhalation when the larynx flops across the airway as the infant breathes in

72
Q

What is stridor?

A

Harsh whistling sound caused by air being forced through an obstruction of the upper airway

73
Q

What are the structural changes in laryngomalacia?

A

Shortened aryepiglottic folds (run between the epiglottis and the arytenoid cartilages - they constrict the opening of the airway to prevent food or fluids entering the largyn and traches)

74
Q

How does the epiglottis change in laryngomalacia?

A

Ayrepiglottic folds are shortened which pulls on the epiglottis and changes its shape to a characteristic “omega” shape

75
Q

How is the tissue around the supraglottic larynx different in laryngomalacia? When is it worse?

A

Softer and less tone meaning it can flop across the airway

Worse on inspiration - causing whistling sound

76
Q

When does laryngomalacia present?

A

Infants, peaking at 6 months with inspiratory stridor - intermittent and worse when feeding, upset on back, during URTIs.

77
Q

What is the management of laryngomalacia?

A

Problem resolves as larynx matures and grows - better able to support itself - usually no interventions and the child is left to grow out of the condition.

Rarely tracheostomy may be necessary, surgery is also an option.

78
Q

What is whooping cough?

A

URTI caused by bordetella pertussis (a gram negative bacteria) (called whooping due to severe sucking in of air in between coughs)

79
Q

Who is vaccinated against pertussis?

A

Children and pregnant women (less effective after a fews years after each dose)

80
Q

How does pertussis present?

A

Mild coryzal symptoms

Low grade fever

Coughing fits a week later (paroxysmal coughing)

81
Q

What can be a result of harsh coughing?

A

Vomit

Faint

Pneumothorax

(not all patients will “whoop” and infants with pertussis may present with apnoeas rather than a cough)

82
Q

How is whooping cough diagnosed?

A

Nasopharyngeal or nasal swab with PCR testing or bacterial culture can confirm the diagnosis within 2 to 3 weeks of the onset of symptoms

83
Q

What can be tested for if whooping cough has been present for more than 2 weeks?

A

Anti-pertussis toxin immunoglobulin G - tested for in oral fluid of children aged 5 to 16 and blood of those aged over 17

84
Q

Is whooping cough a notifiable disease?

A

Yes (so inform public health)

85
Q

What is the management of whooping cough?

A

Supportive care

Vulnerable / acutely unwell / those under 6 months and patients with apnoeas, cyanosis or patients with severe coughing fits may need to be admitted

86
Q

How to avoid spread in whooping cough?

A

Avoid contact with vulnerable people

Disposing of tissues

Careful hand hygiene

87
Q

What is the antibiotic choice in whooping cough?

A

Macrolide antibiotics such as azithromycin, erythromycin and clarithromycin can be beneficial in the early stages (within 21 days)

Co-trimazole is an alternative

88
Q

What are vulnerable people (pregnant, unvaccinated infants or HCW) given who have contact with patients with whooping cough?

A

Prophylactic abx

89
Q

How long do symptoms of whooping cough last?

A

Resolve in 8 weeks

Can be several months - hence “100 day cough”

90
Q

What is a complication of whooping cough?

A

Bronchiectasis

91
Q

What is chronic lung disease of prematurity?

A

Occurs in premature babies (before 28 weeks gestation) in those that suffer with respiratory distress syndrome requiring oxygen or intubation and ventilation

92
Q

What is chronic lung disease of prematurity also known as?

A

Bronchopulmonary dysplasia

93
Q

How is CLDP diagnosed?

A

Chest X-ray and when the infant requires oxygen therapy after they reach 36 weeks gestational age

94
Q

What are the features of CLDP?

A
  • Low oxygen sats
  • Poor feeding
  • Crackles and wheezes on chest auscultation
  • Increased susceptibility to infection
95
Q

How can CLDP be prevented?

A

Giving corticosteroids e.g. betamethasone to mothers that show signs of premature labour at less than 36 weeks gestation

96
Q

How can CLDP be prevented once neonate is born?

A

Using CPAP rather than intubation and ventilation where possible

Using caffeine to stimulate respiratory effort

Not over-oxygenating with supplementary oxygen

97
Q

Whats the management of chronic lung disease of prematurity?

A

Sleep study assesses oxygen sats during sleep - supporting diagnosis and guiding management

Babies discharged on a low dose of oxygen to continue at home e.g. 0.01 litres per minute via nasal cannula - weaned off over first year of life

98
Q

What do babies with CLDP require protection from?

A

Respiratory syncytial virus to reduce risk and severity of bronchiolitis involving monthly injections of a monoclonal antibody against the virus called palivizumab - expensive at around £500 per injection so reserved for babies meeting certain criteria

99
Q

What is cystic fibrosis?

A

Autosomal recessive condition of mucus glands caused by a mutation in cystic fibrosis transmembrane conductance regulatory gene on chromosome 7

100
Q

What is the most common variant of the mutation on chromosome 7?

A

Delta-F508

101
Q

What does the gene involved in CF code for ?

A

Chloride channel

102
Q

How many carriers are there of CF?

A

1 in 25 (1 in 2500 have CF)

103
Q

What are the 3 consequences of CF?

A

Thick pancreatic and biliary secretions causing the ducts to become blocked and a lack of digestive enzymes such as pancreatic lipase in the digestive tract

Low volume thick airway secretions which reduce airway clearance resulting in bacterial colonisation and susceptibility to airway infections

Congenital bilateral absence of the vas deferens in men - male infertility (generally have healthy sperm)

104
Q

When is CF screened for?

A

At birth with newborn bloodspot test

105
Q

What is the first sign of CF?

A

Meconium ileus - first stool baby passes is called meconium and should be within first 24 hours of birth - in CF the meconium is thick and sticky causing it to get stuck and obstruct bowel causing abdo distention and vomiting

106
Q

How does CF present later in life?

A

Recurrent LRTIs

Failure to thrive

Pancreatitis

107
Q

What are the symptoms of CF?

A
  • Chronic cough
  • Thick sputum production
  • Recurrent respiratory tract infections
  • Loose greasy stools (steatorrhoea) due to lack of lipase
  • Abdo pain and bloating
  • Parent may state that child tastes salty when they kiss them
  • Failure to thrive (poor weight and height)
108
Q

What are the signs of CF?

A
  • Low weight / height on growth charts
  • Nasal polyps
  • Finger clubbing
  • Crackles and wheezes on auscultation
  • Abdo distention
109
Q

What are the causes of clubbing in children?

A
  • Hereditary clubbing
  • Cyanotic heart disease
  • Infective endocarditis
  • CF
  • TB
  • Inflammatory bowel disease
  • Liver cirrhosis
110
Q

What are the three ways to diagnose CF?

A

Newborn blood spot testing (picks up most cases)

Sweat test is the gold standard for diagnosis

Genetic testing for CFTR gene can be performed during pregnancy by amniocentesis or chorionic villous sampling or as a blood test after birth

111
Q

How is the sweat test for CF performed?

A
  • Patch of skin is chosen for the test (typically on arm or leg)
  • Pilocarpine is applied to the skin on this patch
  • Electrodes placed either side and a current passed
  • Causes skin to sweat
  • Sweat is absorbed with lab issued gauze or filter paper and sent to the lab for testing for chloride concentration
112
Q

What is the diagnostic chloride concentration for cystic fibrosis ?

A

More than 60 mmol / L

113
Q

What are some common colonisers in CF?

A

Staphylococcus aureus

Haemophilus influenza

Klebsiella pneumoniae

Escherichia coli

Burkhodheria cepacia

Pseudomonas aeruginosa

114
Q

What prophylaxis do patients with CF take?

A

Flucloxacillin against staph aureus infection

Pseudomonas is a particularly troublesome coloniser which is hard to treat and worsens the prognosis of patients with CF

115
Q

Why are there no more social events for children with CF?

A

Due to risk of spreading pseudomonas

116
Q

How is pseudomonas colonisation treated?

A

Long term nebulised antibiotics such as tobramycin, oral ciprofloxacin is also used

117
Q

What is the management of cystic fibrosis?

A

Chest physio several times a day to clear mucus and reduce risk of infection

Exercise for respiratory function and clear sputum

High calorie diet for malabsorption

CREON tablets for pancreatic insufficiency (replaces missing lipase)

Prophylactic flucloxacillin to reduce risk of bacterial infections (especially staph aureas)

Treat chest infections when they occur

Bronchodilators e.g. salbutamol for bronchoconstriction

Nebulised DNase (dornase alfa) - an enzyme which can break down DNA material in respiratory secretions, making them less viscous and easier to clear

Nebulised hypertonic saline

Vaccinations including pneumococcal, influenza and varicella

118
Q

What are the further treatment options in cystic fibrosis?

A

Liver transplantation for end stage respiratory failure

Liver transplant in liver failure

Fertility treatment involving testicular sperm extraction for infertile males

Genetic counselling

119
Q

How often are patients with CF followed up?

A

Every 6 months

120
Q

What are patients with cystic fibrosis monitored for?

A

Colonisation of pseudomonas

Diabetes

Osteoporosis

Vitamin D deficiency

Liver failure

121
Q

What are the complications of cystic fibrosis? What is the life expectancy?

A

Pancreatic insufficiency (90% of patients)

Cystic fibrosis-related diabetes (50% of patients)

Liver disease (30% of patients)

Inferfility in males due to absent vas deferens

Life expectancy = 47 years

122
Q

What is primary ciliary dyskinesia (PCD) also known as?

A

Kartagner’s syndrome

123
Q

What is the mode of inheritance for PCD?

A

Autosomal recessive condition

124
Q

Where is PCD more common?

A

Populations where there is consanguinity meaning the parents are related to one another (increased risk of child having two copies of the same recessive genetic mutation)

125
Q

What is the result of PCD?

A

Dysfunction of the motile cilia around the body - especially respiratory tract

Causes a build up of mucus in the lungs providing a site for infection

Cilia in fallopian tube and flagella of sperm is affected causing reduced or absent fertility

126
Q

What is the link to primary ciliary dyskinesia?

A

To situs inversus

127
Q

How does PCD present?

A

Frequent and recurrent chronic chest infections

Poor growth

Bronchiectasis

(like CF)

128
Q

What is Kartagner’s triad?

A

Three key features of PCD:

  • Paranasal sinusitis
  • Bronchiectasis
  • Situs Inversus

(not all patients will have these)

129
Q

What is situs inversus?

A

The internal (visceral) organs are mirrored inside the body

Heart and stomach on the right with the liver on the left (dextrocardia is where only the heart is reversed)

130
Q

What heart problem may be associated with situs inversus?

A

Transposition of the great arteries

131
Q

How is PCD diagnosed?

A

Sample of ciliated epithelium from nasal brushing or bronchoscopy and examination of the cilia

Recurrent respiratory tract infections

Look for history of consanguinity in the patents

CXR for situs inversus

Semen analysis for male infertility

132
Q

What is the management of PCD?

A

Similar to cystic fibrosis and bronchiectasis with daily physio, high calorie diet and abx

133
Q

What conditions put children in extremely vulnerable category?

A
  • Solid organ transplant recipients
  • Certain cancers
  • Respiratory conditions e.g. CF / asthma
  • Immunodeficiency disorders e.g. SCID
  • Long term immunosuppressive therapies
134
Q

What is the history of a covid patient?

A

Fever

Dry cough

Headache

Sore throat

Myalgia

SoB

Diarrhoea

N+V

135
Q

What is found on examination of a covid patient?

A

Low O2 sats

Increased RR

Intercostal regression

Tracheal tug

Tachycardia

136
Q

What are the differentials for COVID-19?

A

URTI (tonsillitis, otitis media)

LRTI (pneumonia)

Exacerbation of asthma

Viral induced wheeze

Allergy

UTI

Gastroenteritis

137
Q

What are the investigations for COVID-19?

A

CXR (bilateral infiltrates)

CRP and ESR elevated

FBC (lymphopenia, neutrophilia)

Liver enzymes (elevated)

Lactate dehydrogenase (elevated)

D-dimer (raised)

138
Q

What is the treatment of COVID-19?

A
  • Oxygen supplementation (low flow - high flow - CPAP - BiPAP - Mechanical ventilation)
  • Fluid management
  • Abx (if secondary bacterial infection / sepsis)
139
Q

What is the complication of COVID-19?

A

ARDS

Paediatrics (18 decks)

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