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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
When might a child be admitted for bronchiolitis?
``` <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 ```
26
What is the management of bronchiolitis?
``` Supportive: Ensure adequate intake (oral/ NG tube/ IV fluids) Saline nasal drops/ suctioning Supplementary oxygen Ventilatory support ```
27
What are the options for ventilatory support in order of progression?
1. High flow humidified oxygen 2. CPAP 3. Intubation & ventilation
28
How is high flow oxygen supplied and what may it be called?
Tight nasal cannula | Airvo/ Optiflow
29
How does high flow oxygen work to oxygenate the lungs?
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)
30
What is CPAP?
Continuous positive airway pressure (sealed nasal cannula at much higher and controlled pressure)
31
What does intubation involve?
Inserting endotracheal tube into trachea to fully control ventilation
32
What is done to monitor children on ventilatory support?
Capillary blood gases
33
What are the most helpful sign of poor ventilation on a capillary blood gas?
Rising pCO2 | Falling pH
34
What is given to high risk babies as a prevention against bronchiolitis caused by RSV?
Monthly injection of Palivizumab= monoclonal antibody that targets RSV.
35
What does having bronchiolitis as an infant make you more likely to have during childhood?
Viral induced wheeze
36
What is viral-induced wheeze?
Acute wheezy illness caused by viral infection
37
What causes viral induced wheeze?
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
38
What can viral induced wheeze lead to?
The restricted ventilation can lead to respiratory distress
39
What are the typical features of viral-induced wheeze that differentiate it from asthma?
Presenting before 3 years No atopic history Only occurs during viral infections
40
How does viral induced wheeze usually present?
Evidence of viral illness for 1-2 followed by SOB, signs of respiratory distress, expiratory wheeze throughout chest
41
How is viral-induced wheeze managed?
The same as acute asthma
42
What age is most affected by bronchiolitis?
<12 months (3-6 months)
43
What age range is viral-induced wheeze most commonly seen?
1-6
44
What is asthma?
Chronic inflammatory airway disease leading to variable airway obstruction
45
What happens to the smooth airways in asthma?
They are hypersensitive and respond to stimuli by constricting
46
What are the atopic conditions?
Asthma Eczema Hay fever Food allergies
47
What is the typical presentation of asthma?
``` 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 ```
48
What is usually heard on auscultation of asthma?
Bilateral widespread polyphonic wheeze
49
What presenting features may indicate it is not asthma?
``` Wheeze only related to viral infection Isolated/ productive cough Investigations are normal No response to treatment Unilateral wheeze ```
50
What are the typical triggers for asthma?
``` Dust Animals Cold air Exercise Smoke Food allergens ```
51
How is asthma diagnosed?
Clinically based on typical history and examination
52
Below what age are children typically not diagnosed with asthma?
Not diagnosed until at least 2-3 years olf
53
If there is a high probability of asthma, what is done to confirm the diagnosis?
Trial of treatment implemented and if this improves symptoms, diagnosis is made
54
What investigations can be done if there is intermediate probability of asthma or diagnostic doubt?
Spirometry with reversibility Direct bronchila challenge test Fractional exhaled nitric oxide Peak flow variability diary
55
What is spirometry with reversibility?
Spirometry performed before and after taking medication
56
What is the direct bronchial challenge test?
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
57
What is the fractional exhaled nitric oxide test?
Test to see how much nitric oxide is in your breath. Higher level indicates airway inflammation
58
What are the principles of asthma treatment?
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
59
What is the stepwise medical treatment of asthma in under 5's?
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
60
What is the stepwise medical treatment of asthma in 5-12 year olds?
1. SABA as required 2. Add regular corticosteroid inhaler 3. Add LABA 4. Titrate up corticosteroid inhaler 5. Consider adding montelukast or theophylline 5. Increase corticosteroid to high dose
61
What is the stepwise medical treatment of asthma in over 12's?
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
62
What are SABA's and what is their mechanism of action?
Short acting beta 2 adrenergic receptor agonists - act on beta-2 receptors in smooth muscles to cause relaxation and dilation
63
What is the most commonly used SABA?
Salbutamol
64
How long do SABA's take to work and how long do they last?
Works straight away and lasts 1-2 hours
65
What is the most commonly used inhaled corticosteroid for asthma?
Beclometasone
66
How do ICS's work to treat asthma?
Reduce inflammation and reactivity og the arways
67
What is the difference between a salbutamol and ICS inhaler?
Salbutamol used as a reliever when needed | ICS used as a preventer and taken regularly
68
What is an example of a LABA?
Salmeterol
69
What is a LAMA and what is their mechanism of action?
Long acting muscarinic antagonists- block acetylcholine receptors which are stimulated by the parasympatheric nervous system and usually cause smooth muscle contraction, to cause bronchodilation
70
What is an example of a LAMA?
Tiotropium
71
What is an example of a leukotriene receptor antagonist?
Montelukast
72
What are leukotrienes?
Chemicals produced by the immune system that cause inflammation, bronchocronstriction and mucus secretion in the airways
73
What is the action of leukotriene receptor antagonists?
Block the effects of leukotrienes
74
What is the action of Theophylline?
Relaxes smooth muscle and reduces inflammation
75
What is the issue with using Theophylline?
Narrow therapeutic window and can be toxic in excess
76
What is MART?
Maintenance and reliever therapy (combination inhaler with low dose steroid and fast acting LABA)
77
What is a commonly queried side effect of inhaled corticosteroids in children?
Can slightly reduce growth velocity and cause small reduction in final height when used long term (dose dependent)
78
What should be used with inhalers in children and why?
Spacer device to maximise effectiveness- increase amount reaching lungs instead of mouth and then being swallowed
79
What are the different types of spacers?
Metered dose inhaler Dry powder inhaler Breath actuated inhaler
80
What is the inhaler technique when used without a spacer?
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
81
What should be done after using a steroid inhaler and why?
Rinse mouth to reduce the risk of oral thrush
82
What is inhaler technique when using a spacer?
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
83
How should spacers be cleaned?
Once a month (avoid scrubbing) and allow to air dry
84
How does an acute exacerbation of asthma present?
``` Worsening SOB Signs of respiratory distress Tachypnoea Expiratory wheeze Reduced air entry on auscultation ```
85
What is the most ominous sign on chest auscultation and what does this indicate?
Silent chest- indicated airways are so tight it is not possible for enough air to move through airways to create a wheeze
86
What are the signs of a moderate asthma exacerbation?
Peak flow >50% predicted | Normal speech
87
What are the signs of a severe asthma exacerbation?
``` 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) ```
88
What are the signs of a life-threatening asthma exacerbation?
``` Peak flow <33% predicted Sats <92% Poor respiratory effort/ exhaustion Hypotension Silent chest Cyanosis Altered consciousness/ confusion ```
89
What are the main steps of management in an acute asthma exacerbation/ viral induced wheeze?
Supplementary oxygen as required (<94%) Bronchodilators Steroids Antibiotics
90
What are the stepwise bronchodilator options in acute asthma?
1. Salbutamol 2. Ipratropium bromide 3. IV magnesium sulphate 4. IV aminophylline
91
How are salbutamol/ ipratropium bromide administered in acute asthma?
Inhaled or nebulised
92
What kind of medication is ipratropium bromide?
Anti-muscarinic
93
How can a mild asthma exacerbation be managed?
As an outpatient with regular salbutamol inhalers via a spacer
94
How many puffs of salbutamol can be given during a mild asthma exacerbation and how often?
4-6 puffs every 4 hours
95
How are moderate/ severe asthma exacerbations treated?
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
How many puffs of salbutamol can be given during a severe asthma exacerbation and how often?
10 puffs every 2 hours
97
What may need to be done if they don't respond to any of the medical treatment?
Intubation and ventilation before the airway becomes too constricted
98
What is done once control is established?
Work your way back down the ladder as they get better
99
What is a typical step down regime of inhaled salbutamol?
10 puffs 2 hourly--> 10 puffs 4 hoursly --> 6 puffs 4 hourly --> 4 puffs 6 hourly
100
What are the side effects of high doses of salbutamol?
Potassium absorption into cells Tachycardia Tremor
101
At what point after a severe asthma exacerbation can discharge be considered?
When the child is well on 6 puffs 4 hourly of salbutamol.
102
What is a typical reducing regimine of salbutamol when discharged home?
6 puffs 4 hourly for 48 hours--> 4 puffs 6 hourly for 48 hours--> 2-4 puffs as required
103
What is pneumonia?
Infection of the lung tissue that causes inflammation and sputum filling the alveoli/ airways
104
How does pneumonia present on CXR?
Consolidation
105
What causes pneumonia?
Bacteria, viruses or atypical bacteria (e.g. mycoplasma)
106
How does pneumonia present?
``` Cough (wet, productive) High fever Tachypnoea Tachycardia Increased work of breathing Lethargy Delirium ```
107
What are the signs of pneumonia?
``` Tachypnoea Tachycardia Hypoxia Hypotension Fever Confusion ```
108
What are the characteristic chest signs of pneumonia?
Bronchial breath sounds Focal coarse crackles Dullness to percussion
109
What are the two normal breath sounds and where are they heard?
``` Bronchial= over the tracheobronchial tree Vesicular= Over lung tissue ```
110
What do bronchial breath sounds sound like?
Harsh breath sounds equally loud on inspiration and expiration
111
Where might bronchial breathing be heard if there is infection and what causes this?
Heard in lung fields and caused by consolidation of the lung tissue
112
What causes focal coarse crackles?
Air passing through sputum
113
Why do you get dullness to percussion in pneumonia?
Due to lung tissue collapse/ consolidation
114
What are the main bacterial causes of pneumonia in children?
``` Strep pneumonia Group A/ B strep Staph aureus H. Influenza Mycoplasma pneumonia ```
115
What are the most common viral causes of pneumonia?
RSV Parainfluenza Influenza
116
What is the investigation of choice for diagnosing pneumonia?
CXR
117
What other investigations can be done into suspected pneumonia?
``` Sputum cultures Throat swabs VIral PCR Blood cultures (if suspected sepsis) Capillary blood gas analysis ```
118
How is pneumonia managed?
Amoxicillin Macrolide can be added to cover atypical pneumonia Oxygen as required
119
What tests should be done if a child is having recurrent admissions for LRTI's and requiring antibiotics?
``` Thorough history FBC CXR Serum immunoglobulins Immunoglobulin G to previous vaccines Sweat test (CF) HIV test ```
120
What conditions may cause recurrent lower respiratory tract infections in children?
``` Reflux Aspiration Neurological disease Heart disease Asthma Cystic fibrosis Primary ciliary dyskinesia Immune deficiency ```
121
How is pneumonia diagnosed clinically?
History of cough and/ or difficulty breathing <14 days with increased resp rate
122
What resp rates would be used to diagnose pneumonia in children: >2 months 2-11 months >11 months
``` >2= >60 2-11= >50 >11= >40 ```
123
What is croup?
An acute infective respiratory disease affecting young children
124
What age children are usually affected by croup?
6 months- 2 years
125
What kind of infection is croup?
Upper respiratory tract infection
126
What does croup cause?
Oedema in the larynx
127
What is the most common causes of croup?
Parainfluenza virus Influenza Adenovirus RSCV
128
What did croup use to commonly be caused by before vaccines and did this have a high mortality?
Diptheria which led to epiglottitis
129
What is the presentation of croup?
``` Increased work of breathing Barking cough Hoarse voice Stridor Low grade fever ```
130
How is croup usually managed and how quickly does it clear up?
Supportive treatment- usually resolves within 48 hours
131
What can be given to treat croup?
Oral dexamethasone (single dose of 150mcg/ kg)
132
What are the stepwise treatment options in severe croup?
``` Oral dexamethasone Oxygen Nebulised budesonide Nebulised adrenalin Intubation and ventilation ```
133
What is the most common cause of epiglottitis?
Haemophilus influenza type B
134
Why is epiglottitis a life-threatening emergency?
It can swell to the point of completely obscuring the airway within hours of symptoms developing
135
Why is epiglottitis now rare?
Due to routine vaccination programme
136
How might epiglottitis present?
``` Sore throat Stridor Drooling Tripod position High fever Difficulty/ painful swallowing Muffled voice Scared/ quiet child ```
137
What investigations should be done for suspected epiglottitis?
NONE- Do not examine as can make it worse. | Lateral Xray of neck
138
What is the characteristic sign of epiglottitis on neck xray?
Thumb sign
139
What is the key point in the management of epiglottitis?
Not distressing the patient as this can cause closure of the airway- leave them alone and comfortable
140
What is the management of epiglottitis?
Ensure airway is secure- prepare for intubation and tracheostomy with anaestetist IV antibiotics Steroids
141
What is the prognosis for epiglottitis?
Most recover without intubation | Patients that are intubated can be exubated after a few days
142
What is a common complication of epiglottitis?
Epiglottic abscess (collection of pus around the epiglottis)
143
What is laryngomalacia?
Condition in infants where the supraglottic larynx is structured in a way that allows it to cause partial airway obstruction (floppy larynx)
144
What does laryngomalacia lead to and why?
Chronic stridor on inhalation as the larynx flops across the airway as the infant breathes in
145
What is the structures are at the entrance of the larynx?
two aryepiglottic folds that run between the epiglottis and arytenoid cartilages
146
What is the role of the aryepiglottic folds?
Constrict the opening of the airway to prevent food or fluids entering the larynx and trachea
147
What happens to the aryepiglottic folds in laryngomalacia?
They are shortened, pulling on the epiglottis and changing its shape to a classic omega shape
148
What happens to the tissue surrounding the supraglottic larynx in laryngomalacia?
It becomes softer and has less tone, meaning it can flop across the airway
149
How does laryngomalacia present?
Inspiratory stridor- usually intermittent and more prominent when feeding, upset, lying on back or during URTI's
150
What age does laryngomalacia typically present?
Peaks at 6 months
151
How does laryngomalacia resolve?
Resolves as the larynx matures and grows so is better able to support itself
152
What kind of infection is whooping cough?
An upper respiratory tract infection
153
What causes whooping cough?
Bordetella pertussis (gram negative)
154
Why is it called whooping cough?
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
Who is vaccinated against pertussis?
Children and pregnant women
156
How does whooping cough present initially?
Mild coryzal symptoms Low grade fever Mild dry cough
157
How do symptoms progress in pertussis?
More severe coughing fits begin after a week and get progressively worse
158
What is a paroxysmal cough?
Frequent and violent coughing with cough free periods in between
159
What may coughing fits cause?
Fainting Vomiting Pneumothorax
160
How is pertussis diagnosed?
Nasopharyngeal or nasal swab with PCR testing or bacterial culture Oral fluid can be tested for anti-pertussis toxin immunoglobulin G
161
How is pertussis managed?
Notifiable disease Supportive management Macrolide antibiotics can be beneficial in early stages Phrophylactic antibiotics to vunerable close contacts
162
How long does it usually take for pertussis symptoms to resolve?
8 weeks- several months
163
What is a key complication of whooping cough?
Bronchiectasis
164
What is CLDP?
Chronic lung disease of prematurity
165
What is CLDP also known as?
Bronchopulmonary dysplasia
166
In who does CLDP usually present?
Babies born before 28 weeks gestation
167
What do babies with CLDP suffer with at birth?
Respiratory distress syndrome, requiring oxygen therapy or intubation and ventilation
168
How is CLDP diagnosed?
CXR | If the infant requires oxygen therapy after reaching 36 weeks gestational age
169
What are the features or CLDP?
``` Low oxygen sats Increased work of breathing Poor feeding/ weight gain Crackles/ wheezes on auscultation Increased susceptibility to infection ```
170
How can CLDP be prevented before birth?
Corticosteroids given to mothers showing signs of premature labour to speed up development of fetal lungs
171
How can the risk of CLDP be reduced after birth?
Using CPAP rather than intubation and ventilation Using caffeine to stimulate respiratory effort Not over oxygenating
172
How is CLDP managed?
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
What kind of condition is CF?
Autosomal recessive genetic condition
174
What causes CF?
Genetic mutation of cystic fibrosis transmembrane conductance regulatory gene on chromosome 7
175
What is the most common variation of the CF gene?
delta-F508
176
What does the most common mutation code for/
Cellular channels- particularly a type of chloride channel
177
How many people are carriers of the mutation?
1 in 25
178
What are the 3 key consequences of the cystic fibrosis mutation?
Thick pancreatic and biliary secretions Thick airway secretions Absence of vas deferens in males
179
What do the thick pancreatic and biliary secretions lead to?
Blockage of ducts, resulting in a lack of digestive enzymes in the digestive tract
180
What do thick airway secretions lead to?
Reduced airway clearance Bacterial colonisation Suscptibility to airway infections
181
When is cystic fibrosis screened for?
At birth with the newborn bloodspot test
182
What is usually the first sign of CF?
Meconium ileus
183
What is meconium ileus?
When the meconium (first stool that should occur within 24 hours) is thick and sticky and gets stuck, obstructing the bowel
184
In how many CF babies does meconium ileus occur?
20%
185
How does CF usually present later in childhood if not diagnosed at birth?
Recurrent LRTI's Failure to thrive Pancreatitis
186
What are the main symptoms of CF?
``` Chronic cough Thick sputum production Recurrent RTI's Steatorrhoea Abdominal pain/ bloating Salty skin Failure to thrive ```
187
What is steatorrhoea and what causes it?
Loose, greasy stools cause by lack of fat digesting lipase enzymess
188
Why do CF children taste salty?
There is more concentrated salt in the sweat
189
What are the clinical signs of CF?
``` Low height/ weight on growth charts Nasal polyps Finger clubbing Crackles/ wheezes on auscultation Abdominal distension ```
190
What are the causes of clubbing in children?
``` Hereditary Cyanotic heart disease Infective endocarditis CF TB IBD Liver cirrhosis ```
191
How is CF diagnosed?
Newborn blood spot testing Sweat test Genetic testing for CFTR gene during pregnancy or shortly after birth
192
What is the gold standard test for diagnosing CF?
Sweat test
193
How is the sweat test performed?
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
What is the diagnostic chloride concentration for CF?
>60mmol/ L
195
What are some common respiratory colonisers in CF?
``` Staph aureus H. influenza Klebsiella pneumonia E. Coli Pseudomonas ```
196
What do patients with CF take to prevent Staph aureus infection?
Long term prophylactic flucloxacillin
197
What is the most problematic CF coloniser and why?
Pseudomonas aeruginosa as it is very hard to treat and worsens the prognosis
198
Why should people with CF avoid each other?
To avoid spreading pseudomonas
199
How can pseudomonas colonisation be treated?
Long term nebulised antibiotics (e.g. Tobramycin)
200
How is CF managed?
``` 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
What are CREON tablets and why are they given to CF patients?
Replace missing lipase enzymes to digest fats in patients with pancreatic infsufficiency
202
What is DNase and why can it be given to CF patients?
Enzyme that can break down DNA material in respiratory secretions making them less viscous and easier to clear
203
What are more extreme treatment options for CF?
Lung transplant Liver transplant Fertility treatment Genetic counselling
204
How are CF patients monitored?
Specialist clinic appt. every 6 months/ Regular monitoring of sputum for colonisation Screening for diabetes, osteoporosis, vit D deficiency, liver failure
205
What does the prognosis of CF depend on?
``` Severity of symptoms Type of mutution Adherence to treatment Frequency of infection Lifestyle ```
206
What is the median life expectancy for CF?
47 years
207
What is the prognosis for CF?
90% develop pancreatic insufficiency 50% develop diabetes 30% develop liver disease Most males are infertile
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What is Primary ciliary dyskinesia?
Autosomal recessive condition affecting the cilia of various cells in the body
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What is PCD also known as?
Kartagner's syndrome
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In which populations is PCD more common?
Those where there is consanguinity (parents are related to each other)
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What does PCD cause?
Dysfunction of the motile cilia around the body, particularly the respiratory tract leading to a buildup of mucus in the lungs
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How does PCD present (respiratory)?
Frequent and chronic chest infections Poor growth Bronchiectasis
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What areas are primarily affected by PCD?
Lungs Fallopian tubes Sperm
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How does PCD affect fertility?
Affects cilia in fallopian tubes and flagella of sperm, leading to reduced or absent fertility
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What is Karagner's triad?
3 key features of PCD: 1. Paranasal sinusitis 2. Bronchiectasis 3. Situs inversus
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What is bronchiectasis?
Where airways become widened, leading to build up of mucus
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What is situs inversus?
Condition where all the visceral organs are mirrored inside the body (e.g. everything is on the wrong side)
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What is dextrocardia?
When the heart is reversed
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What percentage of patients with situs inversus will have PCD?
25%
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What percentage of patients with PCD have situs inversus?
50%
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How does PCD usually present/
Recurrent respiratory tract infections
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How is PCD diagnosed?
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
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How is PCD managed?
Daily physiotherapy High calorie diet Antibiotics
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What is the key downside to using monteleukasts?
Can cause night terrors

Paeds (18 decks)

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