Paediatrics Respiratory Flashcards
(139 cards)
1
Q
What is bronchiolitis?
A
Inflammation and infection in the bronchioles (small airways of the lungs)
2
Q
What is bronchiolitis usually caused by?
A
Virus - respiratory syncytial virus is the most common cause
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
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
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
6
Q
What are coryzal symptoms?
A
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
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
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
10
Q
What can result from bronchiolitis in infancy?
A
Viral induced wheeze during childhood
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
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
13
Q
What is there limited evidence for treating bronchiolitis with?
A
Nebulised saline
Bronchodilators
Steroids
Antibiotics
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.
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)
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
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)
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)
19
Q
What typically causes a viral induced wheeze in children?
A
RSV or rhinovirus
20
Q
What law states that flow rate is proportional to the radius of the tube to the power 4?
A
Poiseuille’s law
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
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)
23
Q
What is the management of viral induced wheeze?
A
Same as acute asthma in children
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)
25
How does an acute exacerbation of asthma present?
**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
26
What are the signs of moderate, severe and life-theatening asthma?
27
What are the components of management of acute asthma / viral induced wheeze?
**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)
**Antibiotic**s only if a bacterial cause is suspected (e.g. amoxicillin or erythromycin)
28
How can bronchodilators be stepped up in acute asthma?
Inhaled or nebulised **salbutamol** (a beta-2 agonist)
Inhaled or nebulised **ipratropium bromide** (an anti-muscarinic)
**IV magnesium sulphate**
**IV aminophylline**
29
How can mild cases of acute asthma be managed?
As an outpatient with **regular salbutamol inhalers** via a **spacer** (e.g. 4-6 puffs every 4 hours)
30
What is the stepwise approach for moderate to severe cases?
**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**
31
If all the steps of acute asthma management have been covered, what is the next step?
Anaesthetist and ICU need calling (intubation and ventilation)
32
How to review a patient with acute asthma?
- 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
33
What else may need monitoring when on high doses of salbutamol?
Serum potassium (causes potassium to be absorbed from the blood into the cells)
Salbutamol can cause **tachycardia** and a **tremor**
34
What to advise on discharge of a patient with **acute asthma**?
- Finish **course of steriods** (typically 3 days in total)
- Provide **safety-net information** about when to seek help
- Provide individualised written **asthma action plan**
35
What is asthma?
**Chronic inflammatory airway disease**
Smooth muscle airways is **hypersensitive** and responds to stimuli by **constricting** and causing **airway obstruction**
36
Name some other **atopic** conditions?
Asthma
Eczema
Hay fever
Food allergies
(run in families so ask FH)
37
What presentation typically suggests asthma?
- **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**
38
What respiratory symptoms suggest a diagnosis other than asthma?
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
39
Name some typical triggers for asthma?
- **Dust**
- **Animals**
- **Cold** air
- **Exercise**
- **Smoke**
- **Food allergens** (e.g. peanuts, shellfish or eggs)
40
How is asthma diagnosed?
**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
41
What tests can help with **diagnosis of asthma**?
**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)
42
What are the principles for the **stepwise ladder** for asthma?
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
43
What is the **medical therapy** for **asthma** in **under 5**?
- 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
44
What is the **medical treatment** for asthma in patients 5-12?
- **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)
45
What is the medical treatment for asthma in patients over 12?
- **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
46
Can inhaled steroids slow growth? What is the argument for them?
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
47
What is a complication of poor inhaler technique?
Medication in the mouth - **reduces effectiveness** and causes complications such as **oral thrush**
48
How to used a metered dose inhaler?
- 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
49
How to use a metered dosed inhaler with a spacer?
- 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)
50
What is pneumonia caused by?
**Bacteria**
**Virus**
**Atypical bacteria** e.g. mycoplasma
51
How does pneumonia present in children?
**Cough** (wet and productive)
**High fever** (\>38.5)
Tachypnoea
Tachycardia
Increased work of breathing
Lethargy
Delerium (acute confusion associated with infection)
52
What additional **signs** for pneumonia in children?
Hypoxia (low oxygen)
Hypotension (shock)
Fever
Confusion
53
What are the chest signs of pneumonia in children?
**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
54
What are the bacterial causes of pneumonia in children?
**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).
55
What are the viral causes of pneumonia in children?
**Respiratory syncytial virus** is the most common
Parainfluenza virus
Influenza virus
56
What are the investigations for pneumonia in children?
**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
What is the management of pneumonia in children?
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
What should be looked for in a child with recurrent LRTIs?
Reflux
Aspiration
Neurological disease
Heart disease
Asthma
CF
Primary ciliary dyskinesia
Immune deficiency
59
What tests can be done for children with recurrent LRTI?
**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
What is **croup**?
Acute infective respiratory disease of children aged **6 months to 2 years**
URTI causing **oedema** in the **larynx**
61
What are the causes of croup?
**Parainfluenza** (most common)
Influenza
Adenovirus
**Respiratory syncytial virus**
62
What caused croup previously
**Diphtheria** leading to **epiglottitis** with a high mortality - vaccination means that this is rare in developed countries
63
How does croup present?
Increased **work of breathing**
**Barking** cough in episodes
Hoarse voice
Stridor
Low grade **fever**
64
What is the management of croup?
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
What is **epiglottitis**?
Inflammation and swelling of the epiglottis caused by infection, typically with **haemophilus influenza type B**
66
Is epiglottitis an emergency?
Yes but its rare now due to routine **vaccination** against **haemophilus influenza type B**
67
How does epiglottitis present?
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
What are the investigation of epiglottitis?
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
What is the management of epiglottitis?
**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
What is a complication of epiglottitis?
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
What is **laryngomalacia**?
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
What is **stridor**?
**Harsh whistling sound** caused by **air being forced through an obstruction of the upper airway**
73
What are the structural changes in **laryngomalacia**?
**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
How does the epiglottis change in laryngomalacia?
Ayrepiglottic folds are shortened which **pulls on the epiglottis** and changes its shape to a characteristic "**omega**" shape
75
How is the tissue around the **supraglottic larynx** different in **laryngomalacia**? When is it worse?
Softer and less tone meaning it can **flop across the airway**
Worse on inspiration - causing **whistling sound**
76
When does **laryngomalacia** present?
Infants, peaking at 6 months with **inspiratory stridor** - intermittent and worse when feeding, upset on back, during URTIs.
77
What is the management of laryngomalacia?
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
What is **whooping cough**?
**URTI** caused by **bordetella pertussis** (a gram negative bacteria) (called whooping due to severe sucking in of air in between coughs)
79
Who is **vaccinated** against **pertussis**?
Children and pregnant women (less effective after a fews years after each dose)
80
How does pertussis present?
Mild **coryzal** symptoms
Low grade **fever**
**Coughing fits** a week later (**paroxysmal coughing**)
81
What can be a result of harsh coughing?
Vomit
Faint
**Pneumothorax**
(not all patients will "whoop" and infants with pertussis may present with **apnoeas** rather than a cough)
82
How is whooping cough diagnosed?
**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
What can be tested for if whooping cough has been present for more than 2 weeks?
**Anti-pertussis toxin immunoglobulin G** - tested for in oral fluid of children aged 5 to 16 and blood of those aged over 17
84
Is **whooping cough** a notifiable disease?
**Yes** (so inform public health)
85
What is the management of whooping cough?
**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
How to avoid spread in whooping cough?
Avoid contact with vulnerable people
Disposing of tissues
**Careful hand hygiene**
87
What is the **antibiotic choice** in **whooping cough**?
**Macrolide** antibiotics such as **azithromycin**, **erythromycin** and **clarithromycin** can be beneficial in the early stages (within 21 days)
**Co-trimazole** is an alternative
88
What are vulnerable people (pregnant, unvaccinated infants or HCW) given who have contact with patients with whooping cough?
**Prophylactic abx**
89
How long do symptoms of **whooping cough last?**
Resolve in 8 weeks
Can be several months - hence "100 day cough"
90
What is a complication of whooping cough?
**Bronchiectasis**
91
What is chronic lung disease of prematurity?
Occurs in **premature babies** (before 28 weeks gestation) in those that suffer with **respiratory distress syndrome** requiring oxygen or intubation and ventilation
92
What is chronic lung disease of prematurity also known as?
**Bronchopulmonary dysplasia**
93
How is CLDP diagnosed?
**Chest X-ray** and when the infant requires oxygen therapy after they reach **36 weeks gestational age**
94
What are the features of CLDP?
- Low oxygen sats
- Poor feeding
- Crackles and wheezes on chest auscultation
- Increased susceptibility to infection
95
How can CLDP be prevented?
Giving **corticosteroids** e.g. **betamethasone** to mothers that show signs of premature labour at less than 36 weeks gestation
96
How can CLDP be prevented once neonate is born?
Using **CPAP** rather than **intubation** and **ventilation** where possible
Using **caffeine** to stimulate respiratory effort
Not over-oxygenating with supplementary oxygen
97
Whats the management of chronic lung disease of prematurity?
**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
What do babies with CLDP require protection from?
**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
What is **cystic fibrosis**?
**Autosomal recessive** condition of **mucus glands** caused by a mutation in **cystic fibrosis transmembrane conductance regulatory gene** on **chromosome 7**
100
What is the most common variant of the mutation on chromosome 7?
**Delta-F508**
101
What does the gene involved in CF code for ?
Chloride channel
102
How many carriers are there of CF?
**1 in 25** (1 in 2500 have CF)
103
What are the **3 consequences of CF**?
**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
When is CF screened for?
At birth with **newborn bloodspot test**
105
What is the first sign of CF?
**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
How does CF present later in life?
**Recurrent LRTIs**
Failure to thrive
Pancreatitis
107
What are the symptoms of CF?
- 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
What are the signs of CF?
- **Low weight** / height on growth charts
- **Nasal polyps**
- **Finger clubbing**
- **Crackles and wheezes** on auscultation
- Abdo **distention**
109
What are the causes of **clubbing** in **children**?
- Hereditary **clubbing**
- **Cyanotic heart disease**
- **Infective endocarditis**
- CF
- TB
- **Inflammatory bowel disease**
- Liver cirrhosis
110
What are the three ways to diagnose CF?
**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
How is the sweat test for CF performed?
- 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
What is the **diagnostic** **chloride concentration** for cystic fibrosis ?
More than 60 mmol / L
113
What are some common colonisers in CF?
**Staphylococcus aureus**
**Haemophilus influenza**
**Klebsiella** pneumoniae
**Escherichia coli**
Burkhodheria cepacia
**Pseudomonas aeruginosa**
114
What prophylaxis do patients with CF take?
**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
Why are there no more social events for children with CF?
Due to risk of spreading pseudomonas
116
How is **pseudomonas colonisation** treated?
Long term **nebulised antibiotics** such as **tobramycin**, oral **ciprofloxacin** is also used
117
What is the **management** of **cystic fibrosis**?
**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
What are the **further** treatment options in **cystic fibrosis**?
**Liver transplantation** for end stage respiratory failure
**Liver transplant** in liver failure
**Fertility treatment** involving testicular sperm extraction for **infertile** males
**Genetic counselling**
119
How often are patients with CF followed up?
Every 6 months
120
What are patients with **cystic fibrosis** monitored for?
Colonisation of **pseudomonas**
## Footnote
**Diabetes**
**Osteoporosis**
**Vitamin D deficiency**
**Liver failure**
121
What are the complications of cystic fibrosis? What is the life expectancy?
**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
What is **primary ciliary dyskinesia** (PCD) also known as?
**Kartagner's syndrome**
123
What is the mode of inheritance for PCD?
**Autosomal recessive condition**
124
Where is PCD more common?
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
What is the result of PCD?
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**
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What is the link to **primary ciliary dyskinesia**?
To **situs inversus**
127
How does **PCD** present?
Frequent and recurrent **chronic chest infections**
**Poor growth**
**Bronchiectasis**
(like CF)
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What is **Kartagner's triad?**
Three key features of PCD:
- Paranasal sinusitis
- Bronchiectasis
- Situs Inversus
(not all patients will have these)
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What is **situs inversus**?
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)
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What **heart problem** may be associated with **situs inversus**?
**Transposition** of the **great arteries**
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How is PCD diagnosed?
**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**
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What is the management of PCD?
Similar to **cystic fibrosis** and **bronchiectasis** with **daily physio**, **high calorie diet** and **abx**
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What **conditions** put children in **extremely vulnerable** category?
- Solid **organ transplant recipients**
- Certain **cancers**
- Respiratory conditions e.g. CF / asthma
- **Immunodeficiency disorders** e.g. SCID
- Long term **immunosuppressive** therapies
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What is the **history** of a **covid** patient?
Fever
Dry cough
Headache
Sore throat
Myalgia
SoB
Diarrhoea
N+V
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What is found on **examination** of a **covid patient**?
**Low O2 sats**
**Increased RR**
**Intercostal regression**
Tracheal tug
Tachycardia
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What are the **differentials** for **COVID-19**?
**URTI** (tonsillitis, otitis media)
**LRTI** (pneumonia)
**Exacerbation of asthma**
**Viral induced wheeze**
Allergy
UTI
Gastroenteritis
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What are the **investigations** for COVID-19?
**CXR** (bilateral infiltrates)
**CRP** and **ESR** elevated
**FBC** (lymphopenia, neutrophilia)
**Liver enzymes** (elevated)
**Lactate dehydrogenase** (elevated)
**D-dimer** (raised)
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What is the **treatment** of **COVID-19**?
- **Oxygen** supplementation (low flow - high flow - CPAP - BiPAP - Mechanical ventilation)
- **Fluid management**
- Abx (if secondary bacterial infection / sepsis)
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What is the **complication** of **COVID-19**?
**ARDS**
