Respiratory and ENT Flashcards
1
Q
How common is asthma in children?
A
Common - affects 10-15% of school age children
More common in developed countries
2
Q
How can asthma be broadly split into two types?
A
1) Extrinsic - definitive external cause identified, most common in atopic individuals
2) Intrinsic / cryptogenic - no causative agent identified, usually late onset (middle aged)
3
Q
What is the pathophysiology of asthma?
A
Increase in circulating IgE causes increased allergic atopic reactions
Reversible inflammatory disease of airways that responds to bronchodilators
4
Q
List three features of asthma
A
1) Bronchial muscle contraction
2) Mast cell and basophil degranulation = inflammation
3) Increased mucus sectetion
5
Q
List some triggers for asthma (5)
A
1) Drugs
- Beta-blockers = vasodilation and bronchoconstriction
- NSAIDS
2) Pollution / environmental allergens / dust
3) Cold air
4) Viruses
5) Physical exertion
6
Q
List some risk factors for asthma (7)
A
1) Personal hx atopy
2) FH atopy
3) Triggers (eg pollen), dust, exercise, viruses, chemicals etc
4) Prematurity and low birth weight
5) Viral infections in early childhood
6) Maternal smoking
7) Early exposure to broad-spectrum abx
Breast feeding = protective
7
Q
List some important features for an asthma hx
A
PC
- Cough
- Wheeze
- Difficulty breathing
- Chest tightness
HPC
- Recurrent and frequent
- Worse at night and early in the morning
- Worse after triggers
- Improvement after correct treatment
- Occur apart from colds
DH
- Improvement of symptoms after therapy
FH
- Atopy
Birth hx
- Preterm
SH
- Pets
- Smoking
- Housing
- Schools = missed days
8
Q
What is the triad of asthma symptoms?
A
Recurrent episodes of
1) Cough = worse at night
2) SOB
3) Wheeze
9
Q
Ddx for wheeze:
Present from birth (3)
Shortly after birth (5)
Sudden onset in a previously well child (4)
A
Wheeze present from birth:
1) Presents immediately, constant wheeze without variation = structural abnormality eg bronchogenic cyst, vascular ring
2) Weak cry, stridor = laryngeal abnormality
3) Signs of HF = congenital heart disease
Wheeze shortly after birth:
4) Hx of prematurity or ventilation = bronchopulmonary dysplasia
5) Recurrent bacterial infections and FTT = immunodeficiency
6) Persistent cough and FTT, FH of chest disease = CF
7) Persistent nasal discharge and OM = ciliary dyskinesia
8) Vomitting and aspiration = GORD
Sudden onset in a previously well child:
9) Hx choking, unilateral reduced breath sounds = foreign body
10) Persistent wet cough = CF, bronchiectasis, recurrent aspiration, immunodeficiency, GORD
11) Finger clubbing, purulent sputum = CF, bronchiectasis
12) Focal signs in chest = developmental anomaly, post-infection, bornchiectasis, TB, foreign body
10
Q
List the features of moderate acute asthma in a child <2yr
A
Moderate acute asthma:
- Able to talk in sentences
- SpO2 =/> 92%
- Peak flow =/> 50% best or predicted
- HR =/> 140bpm aged 2-5yr or 125 over 5yr
- RR =/> 40 or 30 over 5yr
11
Q
List the features of severe acute asthma in a child <2yr
A
Severe acute asthma:
- Can’t complete sentences in one breath or too breathless to talk / feed
- SpO2 <92%
- Peak flow 33-55% best or predicted
- HR >140bpm aged 2-5yr or >125bpm over 5yr
- RR >40 aged 2-5yr or >30 over 5yr
12
Q
List the features of life-threatening asthma in a child <2yr
A
Any one of the following in a child with severe asthma:
- SpO2 <92%
- Peak flow <33% best or predicted
- Silent chest
- Cyanosis
- Poor response effort
- Hypotension
- Exhaustion
- Confusion
13
Q
What are the normal vital signs for:
1) Infant
2) 1-2yrs
3) 2-5yrs
4) 5-12yrs
5) >12yrs
A
1) Infant
HR: 110-160bpm
Systolic BP: 80-90mmHg
RR: 30-40
2) 1-2yrs
HR: 100-150bpm
Systolic BP: 85-95mmHg
RR: 25-35
3) 2-5yrs
HR: 95-140
Systolic BP: 85-100mmHg
RR: 25-30
4) 5-12yrs
HR: 80-120bpm
Systolic BP: 90-100mmHg
RR: 20-25
5) >12yrs
HR: 60-90bpm
Systolic BP: 100-140mmHg
RR: 14-18
14
Q
List some ddx for acute asthma (4)
A
Foreign body
PE
Anaphylaxis
Pneumothorax
15
Q
List some ddx for chronic asthma
A
COPD Heart failure ACEi cough CF GORD + aspiration SVC obstruction Bronchitis TV Primary ciliary dyskinesia
16
Q
What investigations should be performed for asthma? (6)
A
1) PEFR
2) Spirometry
3) Allergy testing
4) Exercise test
5) Bloods - Raised IgE and eosinophils
6) CXR
17
Q
What is the stepwise management of asthma in children <12yrs?
A
1) Inhaled SABA
2) Inhaled corticosteroid (ICS): 200mcg / day
3) Add inhaled LABA (5yrs or over)
Add leukotreine receptor antagonist (LRTA) (<5yrs)
4) If only partial benefit: inc ICS to 400mcg / day
If no response: stop LABA and increase ICS to 400mcg / day
If control still inadequate: try LTRA
5) Increase ICS to 800mcg / day
OR consider addition of 4th drug eg theophylline
- Refer to respiratory paediatrician
6) Daily oral steroids = refer to respiratory paediatrician
Consider moving up ladder if using 3 or more doses of SABA per week
18
Q
What is the stepwise management of asthma in children 12 yrs or over?
A
1) Inhaled SABA Inhaled corticosteroid (ICS) - 400mcg / day
2) Add inhaled LABA (usually combined inhaler with ICS and LABA)
If only partial benefit: increase ICS to 800mcg / day
If no response: stop LABA and increase ICS to 800mcg / day
If control still inadequate, try LRTA
3) Consider increasing ICS to 2000mcg
Or addition of 5th drug: LRTA, theophylline
4) Daily oral steroid and refer to specialist
19
Q
What is the management of acute asthma in children?
A
OH SHIT ME
Oxygen 15L/min Salbutamol NEB b2b Hydrocortison IV 4mg / kg Ipatropium NEB b2b Theophylline PO (or aminophylline IV) Magnesium sulphate IV Escalate
20
Q
List types of bronchodilators and examples
A
1) Beta 2 agonist
- Salbutamol = Short acting
- Salmeterol = long acting
2) Anticholinergics
- Ipatropium
- Triotropium
3) Leukotriene receptor agonist
- Montelukast
4) Xanthines
- Theophylline = PO
[aminopyhlline is theophylline + ethylenediamine, given IV]
21
Q
What is bronchiolitis? How common is it?
A
Acute viral infection of LRTI
= most common cause of severe respiratory infection in infancy (1/3rd babies develop bronchiolitis before age 1)
22
Q
Who does bronchiolitis affect?
A
Mostly ages 2-12 months
Peaks ages 3-6 months
M>F
23
Q
What is the cause of bronchiolitis?
A
80% due to respiratory syncytial virus (RSV)
[90% of children are immune to RSV by age 2yrs]
Other causes: human metapneumovirus (hMPV), adenovirus, rhinovirus, and parainfluenza and influenza viruses
(possible to be infected with multiple viruses)
24
Q
What proportion of bronchiolitis need admitting?
A
1-2%, the others manage at home
25
What is the pathophysiology of bronchiolitis?
Initial URTI spreads to LRT = infection of bronchiolar respiratory cells and ciliated epithelial cells causes increased mucus secretion, cell death, lymphocytic infiltration and submucosal oedema
Mucus and oedema causes narrowed small airways and V/Q mismatch - leading to hypoxia
26
What are some risk factors for bronchiolitis? (9)
Un/under developed lungs + immune system most common:
1) Premature birth
2) Chronic lung disease eg CF or bronchopulmonary dysplasia
3) Congenital heart disease
4) Neurological disease with hypotonia and pharyngeal disco ordination
5) Immunocompromise
6) Tobacco smoke exposure
7) Non-breast fed
8) Contact with multiple children eg nursery, older siblings
9) Down's syndrome
27
How may bronchiolitis present?
NICE guidelines - consider bronchiolitis in children <2yr with 1-3 day hx of coryzal symptoms followed by:
1) Persistent cough AND
2) Either tachypnoea or chest recession (or both); AND
3) Either wheeze or crackles (or both)
Other features include low grade fever (under 39 degrees Celsius) and poor feeding
28
What are some other signs of hypoxia / increased work of breathing?
1) Tracheal tug
2) Cyanosis
3) Tachypnoea
4) Sub-costal recession
5) Nasal flare
29
List some ddx for bronchiolitis
1) Viral induced wheeze
2) Pneumonia
3) Asthma
4) Bronchitis
5) Pulmonary oedema
6) Foreign body inhalation
7) Oesophageal reflux
8) Aspiration
9) CF
10) Kartagener's syndrome
11) Tracheomalacia / broncomalacia
12) Pneumothorax
30
What features may make you consider a pneumonia over bronchiolitis?
Higher temperature and focal crackles
31
Describe features of bronchiolitis vs viral induced wheeze
Bronchiolitis:
- Younger = 2-12mnths
- Hx of a viral illness followed by a wheeze
- Salbutamol ineffective (too young)
- Treated symptomatically
- Chest sounds of wheeze and crackles
Viral wheeze:
- Older = 1-3yrs
- Shorter hx, wheeze develops in hours-day
- Salbutamol effective
- Chest sounds clear cut wheeze
- Treated same as asthma
NB said that asthma cannot be accurately diagnosed before age 5 (but viral wheeze treated as asthma)
32
What features may make you consider a viral induced wheeze over bronchiolitis?
Wheeze with no crackles, episodic symptoms and/or a FH of atopy
33
How may young infants present with bronchiolitis?
May be lone apnoea
34
What investigations are performed for bronchiolitis? (4)
1) Full examination inc CRT, RR, HR, chest signs
2) Pulse oximetry - hypoxia
3) Nasopharyngeal swab fluorescent antibody test - RSV +ve
4) CXR - non specific: maybe hyperinflation, atelectasis, consolidation
35
When should referral to secondary care be considered in bronchiolitis? (6)
Consider referral to secondary care if:
1) RR >60
2) Inadequate fluid intake
3) Signs of dehydration
4) Child <3months of age
5) Born prematurely
6) Comorbidity eg respiratory or heart disease, or immunodeficiency
36
What features require immediate referral to secondary care for bronchiolitis? (5)
1) Apnoea (observed or reported)
2) Marked chest recession or grunting
3) RR >70
4) Central cyanosis
5) O2 sats <92%
Or clinical judgement that the child looks severely unwell
37
What treatment is given for acute bronchiolitis?
Usually mild, self-limiting illness that can be managed at home
Supportive treatment focussing on fluid input, nutrition and temperature control
Advise parents that symptoms peak between 2-5 days of onset
If awaiting referral to hospital, give supplementary oxygen if sats are persistently <92%
38
What treatment is given for severe bronchiolitis?
Admission in severe cases, symptomatic treatment:
1) Oxygen - achieve >92%
2) CPAP / intubation and ventilation
3) Fluids - limit oral needs, use NG tube or IV depending on severity (may need intermittent feeding as may vomit)
NB salbutamol ineffective = too young to have adrengergic receptors
39
What prophylactic treatment can be given for bronchiolitis?
Palivizumab = monoclonal antibody to RSV can be given to high risk groups my monthly IM injection
ie those with bronchopulmonary dysplasia due to premature birth, congenital heart disease or immunodeficiencies
40
What is croup also known as?
Acute laryngotracheitis
Acute laryngotracheobronchitis
41
How common is croup?
The most common cause of acute airway obstruction
42
Which ages are mostly affected by croup?
Typically 6 months - 6 years
Peak incidence at 2 years
43
What is the aetiology of croup?
Viral cause
```
Most commonly: Parainfluenza viruses types I, II, III and IV (approx 80%)
RSV
Adenoviruses
Rhinoviruses
Enteroviruses
Measles
Metapneumovirus
```
44
What is the pathophysiology of croup?
Viral URTI causes nasopharyngeal inflammation that may spread to the larynx and trachea, causing sub glottal inflammation, oedema and compromise of the airway at is narrowest portion
Movement of vocal chord impaired = characteristic cough
45
How may croup present?
Initially nonspecific symptoms of viral URTI eg coryzal symptoms, fever, cough (12-72hrs)
This progresses to characteristic seal barking cough and hoarse cry (worse at night)
+/- stridor
Decreased chest sounds if severe airflow limitation
+/- respiratory distress - tachypnoea, intercostal recession etc
46
What is the natural course of croup?
3-7 days but can persist for up to 2 weeks
Usually mild and self limiting, but the distressing symptoms can mean parents present to GP/A&E
Severe cases can compromise upper airway
47
List some ddx for croup (8)
1) Epiglottitis
2) Inhaled foreign body
3) Acute anaphylaxis
4) Bacterial tracheitis
5) Diphtheria
6) Laryngeomalacia / other cause of upper airway stenosis
7) Quinsy
8) Retropharyngeal abscess
48
What is the scoring system for the severity of croup?
Westley scoring system
49
What features to the Westley scoring system involve?
1) Inspiratory stridor
Not present = 0
When agitated/active = 1
At rest = 2
```
2) Intercostal recession
Not present = 0
Mild = 1
Miderate = 2
Severe = 3
```
3) Air entry
Normal = 0
Mildly decreased = 1
Severely decreased = 2
4) Cyanosis
None = 0
When agitated/active = 4
At rest = 5
5) Level of consciousness
Normal = 0
Altered = 5
Scoring / 17
0-3 = mild
4-6 = moderate
>6 = severe
50
Most children will have mild croup which can be managed at home. When is immediate assessment in hospital required?
Moderate or severe croup, or impending respiratory failure
Any suspicion of epiglottis, bacterial tracheitis, quinsy, retrophayngeal abscess or laryngeal diptheria eg suspicion of severe infection
Also if <6months, immunocompromised etc
51
What investigations may be performed for croup? (5)
1) Pulse oximetry (<95% = significant respiratory impairment)
Risk vs benefit of distressing child for:
2) CXR
3) Bloods
4) Rapid influenza A throat swab
NB DO NOT EXAMINE THROAT
52
What is the management of croup?
```
Supportive:
Antipyrexials
Fluid
Keep child calm and comfortable
Inpatient care includes oxygen to maintain sats above 93%
```
Steroids (more severe cases):
Dexamethasone 150 micrograms/kg PO
OR prednisolone 1-2mg/kg PO
OR budesonide 2mg NEB
Adrenaline NEB:
For patients in moderate to severe distress
53
What are some complications of croup?
Complications rare
Bacterial superinfection may result in pneumonia or bacterial tracheitis. Most freq:
- S aureus (most common)
- Group A strep
- Moraxella catarrhalis
- Strep pneuomaniae
- Haemophilus influenzae
- Anaerobes
Pulmonary oedema
Pneumothorax
Lymphadenitis
OM
54
MOVE THIS CARD
What is "spasmodic croup?"
Barking cough with no apparent respiratory symptoms
55
How common is epiglottitis?
Rare since introduction of Haemophilus Influenza type B (Hib) vaccine
in 1992
56
What it the usual age of presentation of acute epiglottitis?
2-5yrs
57
What is the aetiology of epiglotttis?
Mostly strep
Also staph aureus, Haemophilus influenzae type b, pseudomonas, moraxella catarrhalis and mycobacterium tuberculosis
HSV
Candida and aspergillus in immunocompromised
Non-infectious causes include thermal eg steam / crack cocaine smoking, foreign bodies etc
Reactive epiglottitis can occur as a result of head and neck chemo
58
How may epiglottitis present? (10)
1) Sore throat
2) Odynophagia
3) Inability to swallow secretions = drooling in children
4) Muffled 'hot potato' voice
5) Fever
NB many signs and symptoms are common and occur with less serious disorders, pt with epiglottis may present with these and they may evolve very quickly over a few hrs
6) Tachycardia
7) Anterior neck tenderness over hyoid bone
8) Ear pain
9) Cervical lymphadenopathy
10) Tripod sign = moves inflamed structures forward, thus easing upper airway obstruction
59
What is odynophagia?
Painful swallowing
60
How may more sever epiglottitis present? (5)
4 X D'S
1) Dyspnoea
2) Dysphagia
3) Dysphonia
4) Drooling
5) Respiratory distress
6) Stridor
Epiglottis appears swollen and cherry red
61
How can epiglottitis be distinguished from croup in children?
Rare for a child to have a cough with epiglottitis
Croup has seal barking cough
62
List some ddx for epiglottis
```
Pharyngitis
Laryngitis
Inhaled foreign body
Croup
Retropharyngeal abscess
```
63
What investigations should be done for epilglottitis?
DO NOT EXAMINE THROAT WITH TONGUE DEPRESSOR = risk of laryngeal obstruction, they should be urgently referred for laryngoscopy
Fibre-optic laryngoscopy = gold standard
- Should only be done in theatre as may need to intubate or perform tracheostomy in the event of an upper airway obstruction
- If not possible, lateral neck x-ray can show 'thumbprint sign'
Throat swabs when airway secure
Blood cultures if systemically unwell
CT or MRI if abscess suspected
64
How is epilglottitis managed?
Initial presentation may resemble viral sore throat, so high index of suspicion needed
Emergency referral if stridor present
Management usually conservative with IV abx but intubation may be needed
- IV cefotaxime 25-50mg/kg/8hr
Surgical tracheostomy may ne required in pt with severe airway obstruction where intubation is not possible
Drainage of abscess if present
65
What are some complications of epiglottitis? (5)
1) Abscess formation (25%)
2) Meningitis
3) Sepsis
4) Pneumothorax
5) Pneumo-mediastinitis (v rare)
66
What is the pathophysiology of epiglottitis?
Severe swelling and oedema of lingual surface of epiglottis seriously threatens the child's airway
= MEDICAL EMERGENCY
67
Differentiate between croup and epiglottitis
```
Croup:
Time course - days
Prodrome - coryza
Cough - barking
Feeding - can drink
Mouth - closed
Toxic - no
Fever - <38.5
Stridor - rasping
Voice - hoarse
```
```
Epiglottitis:
Time course - hours
Prodrome - none
Cough - none (or slight)
Feeding - none
Mouth - drooling saliva
Toxic - yes
Fever - >38.5
Stridor - soft
Voice - weak / silent
```
68
Failure for croup to improve on steroids/nebulisers indicates what?
Bacterial tracheitis
69
What is bacterial tracheitis?
Defined by presence of thick mucopurulent exudate and tracheal mucosal sloughing that is not cleared by coughing, risks include occluding the airway
Often hx of viral infection (like croup) with an acute deterioration
Pronounced tracheal tenderness may be present
70
What is the pathophysiology of pneumonia?
LRTI - acute inflammatory exudate causing air loss and consolidation below the level of the larynx
71
What is the aetiology of childhood pneumonia?
Viral more common in <2yrs:
- RSV
- Adenovirus
- Influenza
- Parainfluenza
- Hib in immunocompromised
Bacterial (most are strep peumoniae):
- Neonates = GBS, E coli, kiebsiella, s aureus
- Infants = s pneumoniae (lobar pneumonia), chlamydia
- School age = s pneumoniae, s aureus, GAS, Bordetella peruses, mycoplasma pneumoniae
72
What are some risk factors for child pneumonia?
Immunodeficiency
CF
Sickle cell disease
Tracheostomy in situ
73
What are some signs for childhood pneumonia?
1) Fever
2) Cough
3) Malaise
4) Respiratory distress eg tachypnoea, cyanosis, intercostal recession, use of accessory muscles
Hx of preceding URTI very common
74
What is defined as tachypnoea in:
1) 0-5 months
2) 6-12 months
3) Over 12 months
1) 0-5 months = >60/min
2) 6-12 months = 50/min
3) Over 12 months >40/min
75
What are some complications of childhood pneumonia?
Persistent pneumonia after 48hr treatment could be effusion or empyema
A fluid sample should be taken by chest drain:
- Empyema diagnosed if acidic pH and protein +++
- Fluid should be allowed to drain from chest drain and not removed until dry for 48hr
76
What may childhood pneumonia show on examination?
1) Dullness to percussion
2) Inspiratory crackles
3) Diminished breath sounds`
77
When should a bacterial pneumonia be suspected?
In children where there is a persistent or repetitive fever >38.5 degrees with chest recession and a raised respiratory rate
78
Audible wheezing is not seen very often in LRTI, however when is it more common?
Diffuse infections such as mycoplasma pneumoniae and bronchiolitis
79
What pneumonia may be accompanied by conjunctivitis in the newborn? When?
Chlamydial pneumonia may be accompanied by chlamydial conjunctivitis
Second or third week
80
Which pneumonia is particularly concerning in first 24 hours of life?
Streptococcal sepsis and pneumonia
81
What type of pneumonia may present with abdominal pain in toddlers?
Lower lobe pneumonia
82
List some ddx for pneumonia
1) Asthma
2) Inhaled foreign body
3) Pneumothorax
4) Cardiac dyspnoea
5) Pneumonitis from other causes eg extrinsic allergic alveolitis, smoke inhalation, gastro-oesophageal reflux
84
What should be tested for in recurrent childhood pneumonia? How?
CF - Sweat test
85
When should admission be considered for a childhood pneumonia? (9)
1) O2 sats <92%
2) RR >70 (or 50 in older child)
3) Significant tachycardia
4) Prolonged CRT
5) Difficulty breathing shown by intermittent apnoea, grunting, not feeding
6) Presence of comorbidity eg congenital heart disease / CF
7) Age <6 months
8) Those in which abx have failed - should see improvement within 48hrs PO abx
9) Pleuritic pain
86
What is the treatment of childhood pneumonia?
Mostly managed at home with fluids, antipyretics and abx if bacterial
```
<5yrs = amoxicillin
>5yrs = amoxicillin / macrolide eg clarithromycin
Severe = Coamoxiclav / IV cephaloposporin (cefotaxime, cefuroxime)
```
87
List common URTIs
```
Rhinitis
Sinusitis
Nasopharyngitis (common cold)
Tonsillitis
Epiglottitis
Laryngitis
Pharyngitis (inflammation of pharynx, uvula and tonsils)
Laryngotracheotitis
Tracheitis
```
88
What proportion of respiratory infections in ENT are URTI?
80%
Acute tonsillitis very common esp <2yr
89
What are some common causative organisms of URTI?
Usually viral:
- Adenovirus
- Enterovirus
- Rhinovirus
- Influenza A & B
- Parainfluenza
- EBV (more older children)
Bacterial = rarer but seen more in older children:
- Group A beta-haemolytic streptococcus eg strep pyogenes
90
What is the Centor Criteria?
Give up to 50% predictive power for bacterial cause of URTI
91
What features are included in the Centor Criteria?
One point for each criterion met:
1) Fever >38 degrees celcius
2) Tonsillar exudate
3) Absent cough
4) Anterior cervical lymphadenopathy
5) Age 3-14
- Bacterial cause <3 is rare
- >45 = -1
92
What symptoms are seen in an URTI?
```
Painful throat
Fever
Blocked nose
Nasa; discharge
Ear ache
Abdo pain = mesenteric adeniitis
```
93
What is mesenteric adenitis?
Intermittent abdo pain caused by inflamed lymph glands usually due to viral infection
Adenitis = inflamed lymph glands
94
What should be included on examination of URTI?
ENT examination:
- Ear discharge
- Bulging ear drum
Cervical lymphadeopathy
Purulent tonsillar exudate
- Seen in GABH or EBV
Check there is no difficulty breathing in infants / neonates
95
What is the treatment of an URTI?
Mostly symptomatic
Sale drops/gentle suction can remove obstructing mucus
Abx only if swab +ve for GABH:
- Penicillin V
or erythromycin for 10 days
96
Why should you avoid amoxicillin when treating an URTI?
Can cause an itchy maculopapular rash if the cause is EBV
97
Why should you avoid amoxicillin when treating an URTI?
Can cause an itchy maculopapular rash if the cause is EBV
98
What may an URTI precipitate in a child?
Febrile convulsions
99
What is a wheeze?
A high-pitched, whistling sound that occurs when smaller airways are narrowed by presence of any of the following:
1) Bronchospasm
2) Swelling of the mucosal lining
3) Excessive amounts of secretions
4) Inhaled foreign body
Head on inspiration or expiration or both
100
How common is wheezing?
Very common throughout childhood - approx 1/3 children have at least one episode before their third birthday
Rare in neonatal period
101
List some common causes of wheeze (5)
1) RTI
2) Asthma
3) Bronchiolitis
4) Viral induced wheeze
5) Cigarette smoke / air pollution
4) Gastro-oesophageal reflux
5) Foreign body inhalation
102
List some rarer causes of wheeze (9)
1) Trachea-Oesophageal fistula following bronchopulmonary dysplasia
2) Bronchiectasis
3) HF
4) Congenital heart disease
5) CF
6) Immmunodeficiency
7) Extrinsic compression of airways eg tumours, vascular rings
8) Tacheobronchomalacia
9) Ciliary dyskinesia
103
What is a viral induced wheeze?
Quick key points from handout
Wheeze related to viral UTI
Reversible obstruction = responds to bronchodilators
Admit if needing inhalers more
than 4hrly or needing oxygen
Often "grow out of it" but may develop into asthma
No role for steroids
Occasionally use montelukast or ICS if recurrent
Beware of foreign body !
104
What investigations are done for possible causes of wheeze?
1) CXR
- Foreign body, structural anomalies, cardiomegaly, masses and pulmonary infiltrates
2) Sweat chloride test
- CF
3) Allergy testing
4) Barium swallow for tracheo-oesophageal fistula and other anomalies
5) Spirometry in those >6yrs
Rarer causes can do echocardiogram, MRI / CT chest
105
What is CF?
Autosomal recessive disease caused by mutation of CFTR on Chr7
106
List the features of CF
1) High sodium sweat
2) Pancreatic insufficiency
3) Biliary disease
4) GI disease
5) Respiratory disease
107
When does CF present? What is the most common presentation?
Normal digestive function possible with <5% pancreatic function = CF can present at any age
Most common presentation its recurrent LRTI with chronic sputum production
108
What screening is performed for CF?
Immunoreactive trypsinogen (IRT) measured on a dried spot of blood obtained on the Guthrie card at day 6 of life
Samples with raised IRT will undergo CFTR mutation screening
109
How is CF diagnosed antenatally?
Amniocentesis / CVS
US demonstration of bowel perforation / hyperchogenic bowel (bright bowel) = 4% due to CF
110
How is CF diagnosed perinatally?
How may it present?
Screening
Bowel obstruction with meconium ileus (bowel atresia)
Haemorrhagic disease of the newborn
- Vit K deficiency due to malabsorption of fat soluble vitamins due to pancreatic insufficiency (but rare as vit K given after birth)
Prolonged jaundice
- Extrahepatic biliary obstruction from bile of increased density with secondary intrahepatic bile stasis
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What is meconium ileus?
Obstruction of the terminal ileum by abnormally thick / adherent meconium
Symptoms include bilious vomiting, abdo distention and failure to pass meconium in the first few days of life
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How may CF present in infancy and childhood?
1) Recurrent RTI
2) Diarrhoea
3) FTT
4) Rectal prolapse
- Frequent coughing or hard-to-pass stools
5) Nasal polyps = in children this is nearly always due to CF
6) Acute pancreatitis
7) Portal HTN and variceal haemorrhage
8) Electrolyte abnormality
- Can develop Pseudo-Bartter's syndrome
9) Hypoproteinaemia and oedema
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How may CF present in adolescence?
1) Recurrent RTI
2) Atypical asthma
3) Bronchiectasis
4) Heat exhaustion / electrolyte disturbance
5) Portal HTN and variceal haemorrhage
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What signs may be observed with CF?
1) Finger clubbing
2) Cough with purulent sputum
3) Crackles
4) Wheeze - mainly upper lobes
5) FEV1 shows obstruction
NB babies with CF usually have no signs / symptoms
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What investigations are done for CF?
Sweat test = 98% sensitive
- Chloride >60mmol/L with sodium concentration lower than that of chloride on 2 separate occasions
Molecular genetic testing for CFTR gene
Sinus X-ray / CT scan = opacification of sunless
CXR / CT thorax
Lung funcitoning test - spirometry unreliable <6yr
Sputum microbiology, common pathogens include:
- Haemophilus influenzae
- S aureus
- Pseudomonas aeruginosa
- Burkholderia cepacia
- E coli
- Klebsiella pneumoniae
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What is most of the morbidity and mortality with CF in respiratory disease related to?
Chronic infection and inflammation lead to bronchiectasis, progressive airflow obstruction, cor pulmonate and death
+ Inc bacterial resistance esp p aeruginosa
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What interventions are used to prevent and treat respiratory problems related to CF?
Mucus clearance, preventing infection nd maintaining good lung function are main aims
Chest physiotherapy twice daily (inc with infective exacerbations)
Regular sputum samples sent for culture
Prophylactic abx to reduce s aureus in children and to prevent secondary bacterial infections when pt has presumed acute viral RTI
Patient with P. aeruginosa have 2/3 inc risk of death >8yr:
- Given combination of PO, NEB and IV abx
Bronchodilators:
- LABA / SABA
Dornase alfa NEB
- Reduces sputum viscosity
High-dose ibuprofen may slow progression of lung diseases
Azithromycin may reduce inflammation
Hypertonic saline by NEB given for osmotic action
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How are nasal polyps treated in CF? What is recurrence rate?
Steroids initially
Polypectomy if fails
50% require repeat within 2 yrs
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How may pancreatic insufficiency present in CF?
Meconium ileus
FTT
Steatorrhoea
Malnutrition
Which can lead to:
Anaemia
Vit deficiency
Sometimes obstruction
```
Which can cause:
Rectal prolapse
Intussusception
Volvulus
Obstruction
```
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How is pancreatic insufficiency diagnosed in CF?
Confirm with stool elastase = presence of unsplit fat globule in stool or 2-3 days stool collection of fatal fat
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How is adequate weight maintained in CF?
Weigh regularly
High energy needs - esp those with recurrent chest infections and large fat lose in stools
Protein intake at least 2 x normal amounts
Take enteric-coated enzyme preparation before meals with dose adjusted to achieve normal stools
Drugs may be needed to reduce acid secretion, as well as vitamin supplements for fat-soluble vitamins A, D and E
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What proportion of children and adolescents with CF develop diabetes?
2% children
19% adolescents
(40-50% adults)
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What is the treatment of CF-related DM?
Insulin replacement usually required with dose adjusted to match high dietary intake
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What other systems are affected by CF?
Liver disease - 30% of adults will develop
- Check LFTs
- Usually seen hepatosplenomegaly
- USS
- Liver transplant in liver failure if good lung function
Osteoporosis - 33% adults
- Take calcium, vit D, bisphosphonates as required
- Regular DEXA scans
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What follow up is required for CF pts?
Recommended to be seen twice yearly by MDT working in specialist centre
Investigations performed at regular intervals:
1) Pulmonary function tests
2) DEXA scan
3) Blood glucose
4) Respiratory cultures
5) Liver USS
6) Vit D levels
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What vaccines are offered in CF populations?
1) Annual influenza
2) Pneumococcal
+ varicella antibodies checked for and varicella vaccine offered to children who need it
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What is the estimated survival for a child born with CF?
40-50yrs
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What investigations may be done in pneumonia?
Not required in a CAP with child going home
1) FBC - raised WCC
1) Blood cultures - rarely positive in pneumonia (fewer than 10% are bacteraemic in pneumococcal disease)
2) Sputum culture / nasopharyngeal aspirate
3) CXR
Often hard to distinguish between viral / bacterial
