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Resp Flashcards

1
Q

What makes up the conducting portion of the respiratory tract

A 
Trachea
Main bronchus
Segmental bronchus
Bronchioles
Terminal bronchioles
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2
Q

What makes up the respiratory portion of the respiratory tract?

A

Respiratory bronchioles
Alveolar ducts
Alveoli

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3
Q

What is bronchiolitis?

A

Inflammation and infection in the bronchioles

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4
Q

What are the bronchioles?

A

The small airways of the lungs

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5
Q

What is the most common cause of bronchiolitis?

A

RSV

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6
Q

What is RSV?

A

Respiratory syncytial virus

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7
Q

What age does bronchiolitis occur?

A

Under 1 year

most common < 6 months

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8
Q

After what age is bronchiolitis rarely diagnosed?

A

2

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9
Q

Why does bronchiolitis only effect young infants?

A

Their arways are so small that even a small amount og mucus and inflammation has a significant effect on their ability to circulate air to and from the alveoli

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10
Q

Why does bronchiolitis not affect adults?

A

The swelling and mucus are proportionally so small compared to the size of their airway that is has little noticeable effect on breathing

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11
Q

What is heard when auscultating a child with bronchiolitis?

A

Harsh breath sounds
Wheeze
Crackles

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12
Q

How does bronchiolitis present?

A 
Coryzal symptoms
Signs respiratory distress
Dyspnoea
Tachypnoea
Poor feeding
Mild fever
Apnoeas
Wheeze/ crackles
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13
Q

What are coryzal symptoms?

A

Typical upper resp tract symptoms: running/ snotty nose, sneezing, mucus in throat, watery eyes

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14
Q

What is dyspnoea?

A

Heavy, laboured breathing

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15
Q

What are the key signs of respiratory distress?

A 
Tachypnoea
Use of accessory muscles 
Intercostal and subcostal recessions
Nasal flaring
Head bobbing
Tracheal tugging
Cyanosis
Abnormal airway noises
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16
Q

What accessory muscles muscles may be used in respiratory distress?

A 
Sternocleidomastoid 
Pectoralis major
Trapezius
Abdominal muscles
Intercostals
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17
Q

What is wheezing and when is it heard?

A

Whistling sound caused by narrowed airways heard during expiration

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18
Q

What causes a wheeze?

A

Any obstruction in the bronchioles

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19
Q

What causes grunting?

A

Exhaling with the glottis partially closed to increase positive end-expiratory pressure

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20
Q

What is stridor and when is it heard?

A

High pitched inspiratory noise cause by obstruction of the upper airway (e.g. croup)

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21
Q

Does stridor happen on inspiration or expiration?

A

Can be either or both

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22
Q

How does bronchiolitis usually begin?

A

As an URTI with coryzal symptoms

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23
Q

What is the course of bronchiolitis?

A

Half get better, half develop chest symptoms following coryzal symptoms, which last 7-10 days

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24
Q

How long does does it take to fully recover from bronchiolitis?

A

2-3 weeks

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25
Q

When might a child be admitted for bronchiolitis?

A 
<3 months with any pre-existing condition
>50% reduction in normal milk intake
Clinical dehydration
Resp rate >70
Oxygen sats <92%
Moderate-severe respiratory distress
Apnoeas
Parents can't manage at home
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26
Q

What is the management of bronchiolitis?

A 
Supportive: 
Ensure adequate intake (oral/ NG tube/ IV fluids) 
Saline nasal drops/ suctioning
Supplementary oxygen
Ventilatory support
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27
Q

What are the options for ventilatory support in order of progression?

A
  1. High flow humidified oxygen
  2. CPAP
  3. Intubation & ventilation
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28
Q

How is high flow oxygen supplied and what may it be called?

A

Tight nasal cannula

Airvo/ Optiflow

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29
Q

How does high flow oxygen work to oxygenate the lungs?

A

Delivers oxygen continuously with added pressure to oxygenate the lungs and prevent the airways collapsing. (Adds positive end-expiratory pressure to maintain airway at end of expiration)

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30
Q

What is CPAP?

A

Continuous positive airway pressure (sealed nasal cannula at much higher and controlled pressure)

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31
Q

What does intubation involve?

A

Inserting endotracheal tube into trachea to fully control ventilation

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32
Q

What is done to monitor children on ventilatory support?

A

Capillary blood gases

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33
Q

What are the most helpful sign of poor ventilation on a capillary blood gas?

A

Rising pCO2

Falling pH

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34
Q

What is given to high risk babies as a prevention against bronchiolitis caused by RSV?

A

Monthly injection of Palivizumab= monoclonal antibody that targets RSV.

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35
Q

What does having bronchiolitis as an infant make you more likely to have during childhood?

A

Viral induced wheeze

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36
Q

What is viral-induced wheeze?

A

Acute wheezy illness caused by viral infection

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37
Q

What causes viral induced wheeze?

A

When the small airways of a child encounter a virus, they develop inflammation and oedema, which causes the walls to swell and constrict, restricts the space for air to flow and causing a wheeze

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38
Q

What can viral induced wheeze lead to?

A

The restricted ventilation can lead to respiratory distress

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39
Q

What are the typical features of viral-induced wheeze that differentiate it from asthma?

A

Presenting before 3 years
No atopic history
Only occurs during viral infections

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40
Q

How does viral induced wheeze usually present?

A

Evidence of viral illness for 1-2 followed by SOB, signs of respiratory distress, expiratory wheeze throughout chest

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41
Q

How is viral-induced wheeze managed?

A

The same as acute asthma

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42
Q

What age is most affected by bronchiolitis?

A

<12 months (3-6 months)

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43
Q

What age range is viral-induced wheeze most commonly seen?

A

1-6

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44
Q

What is asthma?

A

Chronic inflammatory airway disease leading to variable airway obstruction

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45
Q

What happens to the smooth airways in asthma?

A

They are hypersensitive and respond to stimuli by constricting

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46
Q

What are the atopic conditions?

A

Asthma
Eczema
Hay fever
Food allergies

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47
Q

What is the typical presentation of asthma?

A 
Episodic symptoms with intermittent exarcerbations
Diurnal variability 
Dry cough
Wheeze
SOB
Typical triggers
History of other atopic conditions
Family history of atopy
Symptoms improve with bronchodilators
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48
Q

What is usually heard on auscultation of asthma?

A

Bilateral widespread polyphonic wheeze

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49
Q

What presenting features may indicate it is not asthma?

A 
Wheeze only related to viral infection
Isolated/ productive cough
Investigations are normal
No response to treatment
Unilateral wheeze
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50
Q

What are the typical triggers for asthma?

A 
Dust
Animals
Cold air
Exercise
Smoke
Food allergens
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51
Q

How is asthma diagnosed?

A

Clinically based on typical history and examination

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52
Q

Below what age are children typically not diagnosed with asthma?

A

Not diagnosed until at least 2-3 years olf

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53
Q

If there is a high probability of asthma, what is done to confirm the diagnosis?

A

Trial of treatment implemented and if this improves symptoms, diagnosis is made

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54
Q

What investigations can be done if there is intermediate probability of asthma or diagnostic doubt?

A

Spirometry with reversibility
Direct bronchila challenge test
Fractional exhaled nitric oxide
Peak flow variability diary

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55
Q

What is spirometry with reversibility?

A

Spirometry performed before and after taking medication

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56
Q

What is the direct bronchial challenge test?

A

Breathing in gradually increasing doses of medication (eg. histamine) to irritate the airways and cause them to get narrower. People with asthma will be affected at a much lower dose

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57
Q

What is the fractional exhaled nitric oxide test?

A

Test to see how much nitric oxide is in your breath. Higher level indicates airway inflammation

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58
Q

What are the principles of asthma treatment?

A

Start at most appropriate step for severity of symptoms
Review at regular intervals
Step up and down based on symptoms
Aim to achieve no symptoms or exacerbations on lowest dose/ number of treatments
Check inhaler technique and adherence at each review

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59
Q

What is the stepwise medical treatment of asthma in under 5’s?

A
  1. Short acting beta-2 agonist inhaler as required
  2. Add low dose corticosteroid inhaler OR leukotriene antagonist (e.g. montelukast)
  3. Add other option from step 2
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60
Q

What is the stepwise medical treatment of asthma in 5-12 year olds?

A
  1. SABA as required
  2. Add regular corticosteroid inhaler
  3. Add LABA
  4. Titrate up corticosteroid inhaler
  5. Consider adding montelukast or theophylline
  6. Increase corticosteroid to high dose
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61
Q

What is the stepwise medical treatment of asthma in over 12’s?

A

SAME AS ADULTS:

  1. SABA
  2. Regular corticosteroid inhaler
  3. LABA
  4. Steroid increased to medium dose. Consider montelukast, theophylline or LAMA
  5. Steroid increased to high dose. Combine additional treatments. Consider oral SABA
  6. Add oral steroids
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62
Q

What are SABA’s and what is their mechanism of action?

A

Short acting beta 2 adrenergic receptor agonists - act on beta-2 receptors in smooth muscles to cause relaxation and dilation

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63
Q

What is the most commonly used SABA?

A

Salbutamol

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64
Q

How long do SABA’s take to work and how long do they last?

A

Works straight away and lasts 1-2 hours

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65
Q

What is the most commonly used inhaled corticosteroid for asthma?

A

Beclometasone

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66
Q

How do ICS’s work to treat asthma?

A

Reduce inflammation and reactivity og the arways

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67
Q

What is the difference between a salbutamol and ICS inhaler?

A

Salbutamol used as a reliever when needed

ICS used as a preventer and taken regularly

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68
Q

What is an example of a LABA?

A

Salmeterol

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69
Q

What is a LAMA and what is their mechanism of action?

A

Long acting muscarinic antagonists- block acetylcholine receptors which are stimulated by the parasympatheric nervous system and usually cause smooth muscle contraction, to cause bronchodilation

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70
Q

What is an example of a LAMA?

A

Tiotropium

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71
Q

What is an example of a leukotriene receptor antagonist?

A

Montelukast

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72
Q

What are leukotrienes?

A

Chemicals produced by the immune system that cause inflammation, bronchocronstriction and mucus secretion in the airways

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73
Q

What is the action of leukotriene receptor antagonists?

A

Block the effects of leukotrienes

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74
Q

What is the action of Theophylline?

A

Relaxes smooth muscle and reduces inflammation

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75
Q

What is the issue with using Theophylline?

A

Narrow therapeutic window and can be toxic in excess

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76
Q

What is MART?

A

Maintenance and reliever therapy (combination inhaler with low dose steroid and fast acting LABA)

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77
Q

What is a commonly queried side effect of inhaled corticosteroids in children?

A

Can slightly reduce growth velocity and cause small reduction in final height when used long term (dose dependent)

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78
Q

What should be used with inhalers in children and why?

A

Spacer device to maximise effectiveness- increase amount reaching lungs instead of mouth and then being swallowed

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79
Q

What are the different types of spacers?

A

Metered dose inhaler
Dry powder inhaler
Breath actuated inhaler

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80
Q

What is the inhaler technique when used without a spacer?

A

Shake
Sit/ stand up straight
Lift chin and fully exhale
Make tight seal around inhaler with lips
Take steady breath in whilst pressing canister
Continue breathing for 3-4 seconds after pressing
Hold breath for 10 seconds
Wait 30 seconds before giving a further dose

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81
Q

What should be done after using a steroid inhaler and why?

A

Rinse mouth to reduce the risk of oral thrush

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82
Q

What is inhaler technique when using a spacer?

A

Assemble spacer
Shake inhaler and attach to correct end
Sit/ stand up straight and lift chin
Make seal around spacer mouthpiece/ place mask over face
Spray dose into spacer
Take steady breaths in and out 5 times until mist fully inhaled

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83
Q

How should spacers be cleaned?

A

Once a month (avoid scrubbing) and allow to air dry

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84
Q

How does an acute exacerbation of asthma present?

A 
Worsening SOB
Signs of respiratory distress
Tachypnoea
Expiratory wheeze 
Reduced air entry on auscultation
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85
Q

What is the most ominous sign on chest auscultation and what does this indicate?

A

Silent chest- indicated airways are so tight it is not possible for enough air to move through airways to create a wheeze

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86
Q

What are the signs of a moderate asthma exacerbation?

A

Peak flow >50% predicted

Normal speech

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87
Q

What are the signs of a severe asthma exacerbation?

A 
Peak flow <50% predicted
Sats <92%
Unable to complete sentence
Signs of respiratory distress
Resp rate > 40 (1-5), >30 (>5's) 
Heart rate >140 (1-5), >125 (>5)
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88
Q

What are the signs of a life-threatening asthma exacerbation?

A 
Peak flow <33% predicted
Sats <92%
Poor respiratory effort/ exhaustion
Hypotension
Silent chest
Cyanosis
Altered consciousness/ confusion
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89
Q

What are the main steps of management in an acute asthma exacerbation/ viral induced wheeze?

A

Supplementary oxygen as required (<94%)
Bronchodilators
Steroids
Antibiotics

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90
Q

What are the stepwise bronchodilator options in acute asthma?

A
  1. Salbutamol
  2. Ipratropium bromide
  3. IV magnesium sulphate
  4. IV aminophylline
91
Q

How are salbutamol/ ipratropium bromide administered in acute asthma?

A

Inhaled or nebulised

92
Q

What kind of medication is ipratropium bromide?

A

Anti-muscarinic

93
Q

How can a mild asthma exacerbation be managed?

A

As an outpatient with regular salbutamol inhalers via a spacer

94
Q

How many puffs of salbutamol can be given during a mild asthma exacerbation and how often?

A

4-6 puffs every 4 hours

95
Q

How are moderate/ severe asthma exacerbations treated?

A
  1. Salbutamol via spacer
  2. Nebulised salbutamol/ ipratropium bromide
  3. Oral prednisone
  4. IV hydrocortisone
  5. IV magnesium sulphate
  6. IV salbutamol
  7. IV aminophylline
96
Q

How many puffs of salbutamol can be given during a severe asthma exacerbation and how often?

A

10 puffs every 2 hours

97
Q

What may need to be done if they don’t respond to any of the medical treatment?

A

Intubation and ventilation before the airway becomes too constricted

98
Q

What is done once control is established?

A

Work your way back down the ladder as they get better

99
Q

What is a typical step down regime of inhaled salbutamol?

A

10 puffs 2 hourly–>
10 puffs 4 hoursly –>
6 puffs 4 hourly –>
4 puffs 6 hourly

100
Q

What are the side effects of high doses of salbutamol?

A

Potassium absorption into cells
Tachycardia
Tremor

101
Q

At what point after a severe asthma exacerbation can discharge be considered?

A

When the child is well on 6 puffs 4 hourly of salbutamol.

102
Q

What is a typical reducing regimine of salbutamol when discharged home?

A

6 puffs 4 hourly for 48 hours–> 4 puffs 6 hourly for 48 hours–> 2-4 puffs as required

103
Q

What is pneumonia?

A

Infection of the lung tissue that causes inflammation and sputum filling the alveoli/ airways

104
Q

How does pneumonia present on CXR?

A

Consolidation

105
Q

What causes pneumonia?

A

Bacteria, viruses or atypical bacteria (e.g. mycoplasma)

106
Q

How does pneumonia present?

A 
Cough (wet, productive)
High fever
Tachypnoea
Tachycardia
Increased work of breathing
Lethargy
Delirium
107
Q

What are the signs of pneumonia?

A 
Tachypnoea
Tachycardia
Hypoxia
Hypotension
Fever 
Confusion
108
Q

What are the characteristic chest signs of pneumonia?

A

Bronchial breath sounds
Focal coarse crackles
Dullness to percussion

109
Q

What are the two normal breath sounds and where are they heard?

A 
Bronchial= over the tracheobronchial tree 
Vesicular= Over lung tissue
110
Q

What do bronchial breath sounds sound like?

A

Harsh breath sounds equally loud on inspiration and expiration

111
Q

Where might bronchial breathing be heard if there is infection and what causes this?

A

Heard in lung fields and caused by consolidation of the lung tissue

112
Q

What causes focal coarse crackles?

A

Air passing through sputum

113
Q

Why do you get dullness to percussion in pneumonia?

A

Due to lung tissue collapse/ consolidation

114
Q

What are the main bacterial causes of pneumonia in children?

A 
Strep pneumonia
Group A/ B strep
Staph aureus
H. Influenza
Mycoplasma pneumonia
115
Q

What are the most common viral causes of pneumonia?

A

RSV
Parainfluenza
Influenza

116
Q

What is the investigation of choice for diagnosing pneumonia?

A

CXR

117
Q

What other investigations can be done into suspected pneumonia?

A 
Sputum cultures
Throat swabs 
VIral PCR
Blood cultures (if suspected sepsis) 
Capillary blood gas analysis
118
Q

How is pneumonia managed?

A

Amoxicillin
Macrolide can be added to cover atypical pneumonia
Oxygen as required

119
Q

What tests should be done if a child is having recurrent admissions for LRTI’s and requiring antibiotics?

A 
Thorough history
FBC
CXR
Serum immunoglobulins
Immunoglobulin G to previous vaccines
Sweat test (CF) 
HIV test
120
Q

What conditions may cause recurrent lower respiratory tract infections in children?

A 
Reflux
Aspiration
Neurological disease
Heart disease
Asthma
Cystic fibrosis
Primary ciliary dyskinesia
Immune deficiency
121
Q

How is pneumonia diagnosed clinically?

A

History of cough and/ or difficulty breathing <14 days with increased resp rate

122
Q

What resp rates would be used to diagnose pneumonia in children:
>2 months
2-11 months
>11 months

A 
>2= >60
2-11= >50
>11= >40
123
Q

What is croup?

A

An acute infective respiratory disease affecting young children

124
Q

What age children are usually affected by croup?

A

6 months- 2 years

125
Q

What kind of infection is croup?

A

Upper respiratory tract infection

126
Q

What does croup cause?

A

Oedema in the larynx

127
Q

What is the most common causes of croup?

A

Parainfluenza virus
Influenza
Adenovirus
RSCV

128
Q

What did croup use to commonly be caused by before vaccines and did this have a high mortality?

A

Diptheria which led to epiglottitis

129
Q

What is the presentation of croup?

A 
Increased work of breathing
Barking cough
Hoarse voice
Stridor
Low grade fever
130
Q

How is croup usually managed and how quickly does it clear up?

A

Supportive treatment- usually resolves within 48 hours

131
Q

What can be given to treat croup?

A

Oral dexamethasone (single dose of 150mcg/ kg)

132
Q

What are the stepwise treatment options in severe croup?

A 
Oral dexamethasone
Oxygen
Nebulised budesonide
Nebulised adrenalin
Intubation and ventilation
133
Q

What is the most common cause of epiglottitis?

A

Haemophilus influenza type B

134
Q

Why is epiglottitis a life-threatening emergency?

A

It can swell to the point of completely obscuring the airway within hours of symptoms developing

135
Q

Why is epiglottitis now rare?

A

Due to routine vaccination programme

136
Q

How might epiglottitis present?

A 
Sore throat
Stridor
Drooling
Tripod position
High fever
Difficulty/ painful swallowing
Muffled voice
Scared/ quiet child
137
Q

What investigations should be done for suspected epiglottitis?

A

NONE- Do not examine as can make it worse.

Lateral Xray of neck

138
Q

What is the characteristic sign of epiglottitis on neck xray?

A

Thumb sign

139
Q

What is the key point in the management of epiglottitis?

A

Not distressing the patient as this can cause closure of the airway- leave them alone and comfortable

140
Q

What is the management of epiglottitis?

A

Ensure airway is secure- prepare for intubation and tracheostomy with anaestetist
IV antibiotics
Steroids

141
Q

What is the prognosis for epiglottitis?

A

Most recover without intubation

Patients that are intubated can be exubated after a few days

142
Q

What is a common complication of epiglottitis?

A

Epiglottic abscess (collection of pus around the epiglottis)

143
Q

What is laryngomalacia?

A

Condition in infants where the supraglottic larynx is structured in a way that allows it to cause partial airway obstruction (floppy larynx)

144
Q

What does laryngomalacia lead to and why?

A

Chronic stridor on inhalation as the larynx flops across the airway as the infant breathes in

145
Q

What is the structures are at the entrance of the larynx?

A

two aryepiglottic folds that run between the epiglottis and arytenoid cartilages

146
Q

What is the role of the aryepiglottic folds?

A

Constrict the opening of the airway to prevent food or fluids entering the larynx and trachea

147
Q

What happens to the aryepiglottic folds in laryngomalacia?

A

They are shortened, pulling on the epiglottis and changing its shape to a classic omega shape

148
Q

What happens to the tissue surrounding the supraglottic larynx in laryngomalacia?

A

It becomes softer and has less tone, meaning it can flop across the airway

149
Q

How does laryngomalacia present?

A

Inspiratory stridor- usually intermittent and more prominent when feeding, upset, lying on back or during URTI’s

150
Q

What age does laryngomalacia typically present?

A

Peaks at 6 months

151
Q

How does laryngomalacia resolve?

A

Resolves as the larynx matures and grows so is better able to support itself

152
Q

What kind of infection is whooping cough?

A

An upper respiratory tract infection

153
Q

What causes whooping cough?

A

Bordetella pertussis (gram negative)

154
Q

Why is it called whooping cough?

A

The coughing fits are so severe that the child is unable to take in any air between coughs and subsequently makes a loud whooping sound to suck in air when the coughing finishes

155
Q

Who is vaccinated against pertussis?

A

Children and pregnant women

156
Q

How does whooping cough present initially?

A

Mild coryzal symptoms
Low grade fever
Mild dry cough

157
Q

How do symptoms progress in pertussis?

A

More severe coughing fits begin after a week and get progressively worse

158
Q

What is a paroxysmal cough?

A

Frequent and violent coughing with cough free periods in between

159
Q

What may coughing fits cause?

A

Fainting
Vomiting
Pneumothorax

160
Q

How is pertussis diagnosed?

A

Nasopharyngeal or nasal swab with PCR testing or bacterial culture
Oral fluid can be tested for anti-pertussis toxin immunoglobulin G

161
Q

How is pertussis managed?

A

Notifiable disease
Supportive management
Macrolide antibiotics can be beneficial in early stages
Phrophylactic antibiotics to vunerable close contacts

162
Q

How long does it usually take for pertussis symptoms to resolve?

A

8 weeks- several months

163
Q

What is a key complication of whooping cough?

A

Bronchiectasis

164
Q

What is CLDP?

A

Chronic lung disease of prematurity

165
Q

What is CLDP also known as?

A

Bronchopulmonary dysplasia

166
Q

In who does CLDP usually present?

A

Babies born before 28 weeks gestation

167
Q

What do babies with CLDP suffer with at birth?

A

Respiratory distress syndrome, requiring oxygen therapy or intubation and ventilation

168
Q

How is CLDP diagnosed?

A

CXR

If the infant requires oxygen therapy after reaching 36 weeks gestational age

169
Q

What are the features or CLDP?

A 
Low oxygen sats
Increased work of breathing
Poor feeding/ weight gain
Crackles/ wheezes on auscultation
Increased susceptibility to infection
170
Q

How can CLDP be prevented before birth?

A

Corticosteroids given to mothers showing signs of premature labour to speed up development of fetal lungs

171
Q

How can the risk of CLDP be reduced after birth?

A

Using CPAP rather than intubation and ventilation
Using caffeine to stimulate respiratory effort
Not over oxygenating

172
Q

How is CLDP managed?

A

Formal sleep study to assess oxygen sats
May be discharged with low dose oxygen which can be weaned over the first year of life
Protection against RSV to prevent risk of bronchiolitis (monthly injections of Palivizumab)

173
Q

What kind of condition is CF?

A

Autosomal recessive genetic condition

174
Q

What causes CF?

A

Genetic mutation of cystic fibrosis transmembrane conductance regulatory gene on chromosome 7

175
Q

What is the most common variation of the CF gene?

A

delta-F508

176
Q

What does the most common mutation code for/

A

Cellular channels- particularly a type of chloride channel

177
Q

How many people are carriers of the mutation?

A

1 in 25

178
Q

What are the 3 key consequences of the cystic fibrosis mutation?

A

Thick pancreatic and biliary secretions
Thick airway secretions
Absence of vas deferens in males

179
Q

What do the thick pancreatic and biliary secretions lead to?

A

Blockage of ducts, resulting in a lack of digestive enzymes in the digestive tract

180
Q

What do thick airway secretions lead to?

A

Reduced airway clearance
Bacterial colonisation
Suscptibility to airway infections

181
Q

When is cystic fibrosis screened for?

A

At birth with the newborn bloodspot test

182
Q

What is usually the first sign of CF?

A

Meconium ileus

183
Q

What is meconium ileus?

A

When the meconium (first stool that should occur within 24 hours) is thick and sticky and gets stuck, obstructing the bowel

184
Q

In how many CF babies does meconium ileus occur?

A

20%

185
Q

How does CF usually present later in childhood if not diagnosed at birth?

A

Recurrent LRTI’s
Failure to thrive
Pancreatitis

186
Q

What are the main symptoms of CF?

A 
Chronic cough
Thick sputum production
Recurrent RTI's
Steatorrhoea
Abdominal pain/ bloating
Salty skin 
Failure to thrive
187
Q

What is steatorrhoea and what causes it?

A

Loose, greasy stools cause by lack of fat digesting lipase enzymess

188
Q

Why do CF children taste salty?

A

There is more concentrated salt in the sweat

189
Q

What are the clinical signs of CF?

A 
Low height/ weight on growth charts
Nasal polyps
Finger clubbing
Crackles/ wheezes on auscultation
Abdominal distension
190
Q

What are the causes of clubbing in children?

A 
Hereditary
Cyanotic heart disease
Infective endocarditis
CF
TB
IBD
Liver cirrhosis
191
Q

How is CF diagnosed?

A

Newborn blood spot testing
Sweat test
Genetic testing for CFTR gene during pregnancy or shortly after birth

192
Q

What is the gold standard test for diagnosing CF?

A

Sweat test

193
Q

How is the sweat test performed?

A

Pilocarpine applied to a patch of skin, electrodes are placed either side of the patch and a small current is passed between, causing the skin to sweat. The sweat is absorbed with guaze and tested for chloride concentration

194
Q

What is the diagnostic chloride concentration for CF?

A

> 60mmol/ L

195
Q

What are some common respiratory colonisers in CF?

A 
Staph aureus
H. influenza
Klebsiella pneumonia
E. Coli
Pseudomonas
196
Q

What do patients with CF take to prevent Staph aureus infection?

A

Long term prophylactic flucloxacillin

197
Q

What is the most problematic CF coloniser and why?

A

Pseudomonas aeruginosa as it is very hard to treat and worsens the prognosis

198
Q

Why should people with CF avoid each other?

A

To avoid spreading pseudomonas

199
Q

How can pseudomonas colonisation be treated?

A

Long term nebulised antibiotics (e.g. Tobramycin)

200
Q

How is CF managed?

A 
MDT
Chest physiotherapy several times a day
Exercise
High calorie diet
CREON tablets
Prophylactic flucloxacillin
Treat chest infections
Bronchodilators
Nebulised DNase
Nebulised hypertonic saline
Vaccinations
201
Q

What are CREON tablets and why are they given to CF patients?

A

Replace missing lipase enzymes to digest fats in patients with pancreatic infsufficiency

202
Q

What is DNase and why can it be given to CF patients?

A

Enzyme that can break down DNA material in respiratory secretions making them less viscous and easier to clear

203
Q

What are more extreme treatment options for CF?

A

Lung transplant
Liver transplant
Fertility treatment
Genetic counselling

204
Q

How are CF patients monitored?

A

Specialist clinic appt. every 6 months/
Regular monitoring of sputum for colonisation
Screening for diabetes, osteoporosis, vit D deficiency, liver failure

205
Q

What does the prognosis of CF depend on?

A 
Severity of symptoms
Type of mutution
Adherence to treatment
Frequency of infection
Lifestyle
206
Q

What is the median life expectancy for CF?

A

47 years

207
Q

What is the prognosis for CF?

A

90% develop pancreatic insufficiency
50% develop diabetes
30% develop liver disease
Most males are infertile

208
Q

What is Primary ciliary dyskinesia?

A

Autosomal recessive condition affecting the cilia of various cells in the body

209
Q

What is PCD also known as?

A

Kartagner’s syndrome

210
Q

In which populations is PCD more common?

A

Those where there is consanguinity (parents are related to each other)

211
Q

What does PCD cause?

A

Dysfunction of the motile cilia around the body, particularly the respiratory tract leading to a buildup of mucus in the lungs

212
Q

How does PCD present (respiratory)?

A

Frequent and chronic chest infections
Poor growth
Bronchiectasis

213
Q

What areas are primarily affected by PCD?

A

Lungs
Fallopian tubes
Sperm

214
Q

How does PCD affect fertility?

A

Affects cilia in fallopian tubes and flagella of sperm, leading to reduced or absent fertility

215
Q

What is Karagner’s triad?

A

3 key features of PCD:

  1. Paranasal sinusitis
  2. Bronchiectasis
  3. Situs inversus
216
Q

What is bronchiectasis?

A

Where airways become widened, leading to build up of mucus

217
Q

What is situs inversus?

A

Condition where all the visceral organs are mirrored inside the body (e.g. everything is on the wrong side)

218
Q

What is dextrocardia?

A

When the heart is reversed

219
Q

What percentage of patients with situs inversus will have PCD?

A

25%

220
Q

What percentage of patients with PCD have situs inversus?

A

50%

221
Q

How does PCD usually present/

A

Recurrent respiratory tract infections

222
Q

How is PCD diagnosed?

A

Careful family history
Nasal brushing or bronchoscopy to take sample of ciliated epithelium for analysis of cilia action
Examination and imagine for situs inversus
Semen analysis

223
Q

How is PCD managed?

A

Daily physiotherapy
High calorie diet
Antibiotics

224
Q

What is the key downside to using monteleukasts?

A

Can cause night terrors

Paeds (18 decks)

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