CHH Flashcards
(937 cards)
1
Q
Development - 6 weeks
A
Head control – 45⁰ when prone
Stabilises when sitting
Follows object to midline
Startles to loud noise
Social Smile
2
Q
Development - 3 months
A
Head steady when in sitting position
Follows past the midline
Vocalises, coos and laughs
Spontaneous smile
3
Q
Development - 6 months
A
Rolls front to back
Palmar Grasp/Transfers
Turns to loud sound, Babbles
Mouths objects, Holds a bottle
4
Q
Development - 9 months
A
Stands with support
Pincer Grip
Bangs cubes
Responds to own name
Play’s Peek-a-boo
Holds/Bites food
5
Q
Development - 12 months
A
Stands independently
Casts bricks
Mama/Dada
Waves/Claps
Drinks from a beaker with a lid
6
Q
Development - 18 months
A
Walks (9-18 months)
2 Cube tower, scribbles
Vocab: 3-6 words, Understands nouns
Imitative play
7
Q
Development - 24 months
A
Run, Kicks Ball
4 Cube Tower
Draws a vertical line
Vocab: 50 words
2 Words Together
Understands Verbs
Removes a garment
8
Q
Development - 2.5 years
A
Jumps
Throw Ball over head
6 Cube Tower
Draws a Horizontal Line
Vocab: 6 Body parts
3-4 word sentences
Understands prepositions
Eats well with a spoon
9
Q
Development - 3 years
A
Balance 1 foot, 1 second
8 cube tower, 3 brick bridge
Draws a circle
Uses Adjectives
Understands negatives
½ understandable speech
Eats with a fork/spoon
Puts on a t-shirt
Takes turns
10
Q
Development - 4 years
A
Balance 1 foot, 3 seconds
Hops
Builds Steps (6 bricks) Draws a Cross
Understands Comparatives
Knows 4 colours
Sympathy, imaginative play
Dresses alone
11
Q
Development - 5 years
A
Balance 1 foot 5 seconds
Heel-toe walk
Skips
Draws a Triangle & Person 6 parts
Understands Complex (3 part) instructions Counts to 5
Can play a board game
Brushes teeth
Uses a knife
12
Q
Use of anti-pyretics
A
Either paracetamol or ibuprofen, but not normally both simultaneously
Generally only used if the child is distressed
Anti-pyretics will not prevent febrile convulsions
13
Q
Febrile convulsions - when and why?
A
Ages 6 months to 5 years.
Usually due to infection or inflammation outside the CNS in an otherwise well child
14
Q
Simple febrile convulsion
A
isolated, generalised, tonic-clonic seizure
15
Q
Complex febrile convulsion
A
Complex if 1+ of:
- Focal onset/focal features
- Duration >15 minutes
- Recurs within 24h/same illness
- Incomplete recovery after 1 hour.
16
Q
Febrile convulsions - management
A
- If cease before presentation – do not give drug treatment.
- If >5 mins – rectal diazepam/buccal midazolam.
Always check blood glucose if child is unconscious/is convulsing
17
Q
How is suspected meningitis managed in the community?
A
Single dose of benzylpenicillin IV/IM and immediate transfer to hospital (call 999)
18
Q
Abx in meningitis - <3 months
A
IV cefotaxime and IV amoxicillin (to cover listeria meningitis) and IV gentamicin
19
Q
Abx in meningitis - >3 months
A
IV ceftriaxone
also IV gentamicin (only if probable sepsis)
20
Q
Management of meningitis
A
Ideally blood cultures and LP before Abx (unless significant delay)
Start Abx
If probable or confirmed bacterial meningitis – dexamethasone, ideally with first dose of antibiotics
Supportive therapy - high flow O2, fluids, antipyrexials
21
Q
Why is dexamethasone used in bacterial meningitis?
A
Decreased sequelae in pneumococcal meningitis and Haemophilus Influenzae meningitis.
No evidence of improved outcome or harm in meningococcus/viral meningitis.
22
Q
UTI in children
A
= commonest bacterial infection of children.
Most commonly caused by E. coli.
Urgently admit if <3 months and UTI is suspected.
Older children may also need admission if at risk of serious illness.
23
Q
When should a UTI be treated?
A
If leucocytes and nitrites (or just nitrites) are positive on urine dip
If good clinical suspicion of UTI
24
Q
What should be done if only leucocytes or neither leucocytes/nitrites are raised on a urine dip?
A
Potentially look for other focus of infection.
Only treat UTI if high clinical suspicion
25
Follow-up for UTI
When is it appropriate to refer to a specialist?
If not improved after 24-48 hours, review treatment and diagnosis
Follow up result of any urine sent for culture and review antibiotics
Refer urgently to specialist if:
- Poor response to appropriate treatment
- History/clinical features suggest urinary tract obstruction
26
What age group is affected by bronchiolitis?
Birth – 2 years
| BUT most common in 1st year
27
What causes bronchiolitis?
Viral cause - ~75% RSV
Can be bacterial superinfection in more severe cases
28
Management of bronchiolitis
1. Close fluid management (IV/NG/oral)
2. Oxygen to maintain sats
=> may need CPAP/high flow nasal cannula oxygen
~15% of patients will require admission to intensive care for intubation/ventilation
29
What groups of children are typically affected by viral-induced wheeze?
Age 6 months – 5 years
=> Most will “grow out of it” before school
Association between passive smoking and severe disease.
30
What can cause viral induced wheeze?
All respiratory viruses
31
Management of viral-induced wheeze?
Fluids
Oxygen to maintain sats
Salbutamol (10 puffs MDI or nebuliser):
=> Hourly or back-to-back initially
=> Stretch to 2-hourly as tolerated
=> Can discharge when at 3-4 hourly
32
What is more likely with an older child presenting with viral-induced wheeze?
The child is more likely to present with asthma later on
33
What age group is affected by croup?
6 months – 6 years
| most common age 2-3
34
what causes croup?
Parainfluenza most common cause, but possible with all respiratory viruses.
35
Management of croup
Fluids, antipyretics
Aim to keep child calm
Oxygen to maintain sats
Single dose of oral dexamethasone (or nebulised budesonide)
Nebulised adrenaline if inadequate response to steroids
36
What age group is affected by pneumonia?
All paediatric age range
37
What causes pneumonia?
Strep. pneumoniae,
Staph. aureus,
H. influenzae,
Some cases are likely to be viral, but cannot be distinguished clinically
38
Management of pneumonia
Fluids, oxygen to maintain sats.
Amoxicillin 1st line if uncomplicated CAP (5-7 days)
Benzylpenicillin/Cefuroxime if IV treatment needed
Consider macrolide if no response to 1st line treatment at 48h
39
What causes otitis media?
50% viral cause
Bacterial causes include:
- S. pneumoniae
- H. influenzae
- S. pyogenes
- M. catarrhalis
- S. aureus
40
Management of otitis media
80% of cases resolve without treatment in ~4 days (regardless of cause)
Delayed Abx may be considered as this is as effective as immediate treatment
=> Amoxicillin – 5 days (clarithromycin if pen allergy)
41
when might immediate antibiotic treatment be considered in otitis media (instead of delayed Abx)?
in a systemically unwell child with fever, vomiting, pain for >48h and otorrhoea, and those with other comorbidities
42
What is the most common chronic illness affecting children?
Asthma
43
What is asthma?
An inflammatory condition, leading to reversible airway obstruction causing intermittent wheeze.
The airways narrow due to smooth muscle contraction and mucous hypersecretion
44
Symptoms of asthma
Cough, Wheeze, Breathlessness, Chest tightness
AND evidence of variability in airway obstruction
45
What are signs of poorly controlled asthma?
Increase in cough, SoB, wheeze
Difficulty walking/talking/sleeping
Reduced relief from/frequent use of SABA
46
What clinical features would increase the probability of a diagnosis of asthma?
Symptoms - frequent, worse at night/early morning, occur in response to triggers
Personal or family Hx of atopy
Widespread wheeze heard on auscultation
History of improvement of lung function in response to adequate therapy
47
What clinical features would decrease the probability of a diagnosis of asthma?
Symptoms with colds only, no interval symptoms
Isolated cough in the absence of wheeze/SoB
Dizziness/light-headedness/peripheral tingling
Normal PEF when symptomatic
No response to trial of asthma therapy
Clinical features pointing to alternative diagnosis
48
What PMHx may be relevant for asthma?
Atopy - hay fever, eczema
| Recent URTI
49
What FHx may be relevant for asthma?
Atopy
| Asthma
50
What SHx may be relevant for asthma?
Impact on daily activities/hobbies
Pets
Smoking
Parental smoking
51
Diagnosis of asthma
Normally based on history and examination
- PEF - looking for reversibility/diurnal variation
- Spirometry (only really from age 6+ due to technique)
- ?IgE/skin prick tests for allergens
- ?CXR to r/o other conditions
52
Non pharmacological management of asthma
trigger avoidance, breathing exercises
53
Pharmacological management of asthma
Step-wise approach - start with most appropriate therapy based on presenting severity
SABA as required, plus:
1. very low dose ICS (or LTRA if <5 years)
2. very low dose ICS plus LABA/LTRA
3. consider increasing to low dose ICS
4. Refer to specialist care for further therapies
Step up if using SABA >3 times per week.
54
What is defined as complete control of asthma?
* No daytime symptoms
* No night-time waking due to asthma
* No need for rescue medication
* No asthma attacks
* No limitations on activity, including exercise.
* Normal lung function (FEV1 and/or PEF >80% predicted)
* Minimal side effects from medication
55
Actions of Salbutamol
Relieve acute breathlessness by relaxing bronchial smooth muscle
Duration of action = 3-6 hours (maximum effect at 30mins)
56
Side effects of salbutamol
Palpitations, tremor
Vasodilatation,
Hypokalaemia,
Muscle cramps
57
What would you expect to see on the U&Es of a patient on back-to-back salbutamol nebs?
hypokalaemia
58
Actions of inhaled corticosteroids
Provide control of the disease by reducing airway inflammation
Symptoms alleviated after ~3-7 days from initiation
59
When are ICS considered in asthma?
Any child who is:
- Using SABA ≥3 times/week
- Symptomatic ≥3 times/week
- Waking one night a week
- Aged 5-12 years with an exacerbation requiring oral steroids in the last 2 years
60
Adverse effects of ICS
Local - oral candidiasis, hoarseness, infections
Systemic - growth restrictions, osteoporosis, acute adrenal crisis
61
Use of LABAs in asthma
Limited evidence for use <5 years, and not licensed for <4 years
Added on to therapy with ICS (not used alone)
62
Why should a LABA only be used alongside inhaled steroids?
LABA alone causes increased risk of asthma-related death
63
Use of LTRAs (e.g. montelukast) in asthma
Reduce inflammation and hyper-responsiveness
Regular preventative therapy – alternative to/in addition to ICS
Less effective than ICS alone or ICS plus LABA in children >5
Rare adverse event: Churg-Strauss syndrome
64
Churg-Strauss syndrome
= a rare form of systemic vasculitis:
```
eosinophilia,
vasculitic rash,
worsening pulmonary symptoms,
cardiac complications
peripheral neuropathy
```
65
What are the issues around using aminophylline/theophylline in managing asthma?
Narrow therapeutic range – target plasma levels between 10-20mg/L.
Side effects within therapeutic range – nausea, headache, insomnia, palpitations, arrythmias
At toxic levels – arrythmias and seizures
66
Indications for use of oral steroids in asthma
1. Acute exacerbation – 3-5 days short course
| 2. Chronic severe asthma – when response to other drugs is inadequate (high dose ICS continued to reduce oral dose).
67
Stopping oral steroids used for asthma
can be stopped abruptly, UNLESS:
- Course >3 weeks
- Already on maintenance oral corticosteroids
- Repeated short courses.
68
Use of monoclonal antibody (e.g. Omalizumab) in asthma
s.c. injection every 2-4 weeks depending on the patient’s IgE level and weight
Reduces the steroid burden for the patient, without increasing the risk of adverse events
mAb forms complexes with free IgE and prevents its interaction with these receptors
69
What is important to remember when prescribing salbutamol
State the dose, frequency, and max number of doses in 24h explicitly to the child/parents
“as required drug” in a hospital drug chart
route - inhaled or via nebuliser
70
What is important to remember when prescribing ICS
Prescribe by BRAND!
Important to put brand name and dose
regular prescription on drug chart
71
What is important to remember when prescribing a LABA
regular prescription on drug chart
Often in combination inhalers (e.g. seretide)
Do not initiate in rapidly deteriorating asthma
Initiate at a low dose and check effect before increasing – review effect regularly
72
Benefits of using a spacer in asthma
* Remove the need for coordination – tidal breathing is effective.
* Reduce risk of oral infection from ICS
* Suitable for managing mild/moderate exacerbations.
73
Stepping down asthma treatment
Review regularly and titrate steroids down if possible.
Do not step-down treatment if ongoing symptoms and needing reliever, or if they have had a recent exacerbation
If there is symptom control - reduce ICS dose slowly
(e.g. by 25-30% every 3 months)
74
Severe asthma exacerbation
```
SpO2 <92%
PEF 33-50%
Tachypnoea
Tachycardia
Audible wheeze
Accessory muscle use
```
75
Signs/symptoms suggesting a life-threatening asthma attack
```
SpO2 <92%
PEF <33%
RR reduced (exhaustion)
Tachycardia
Silent chest
Cyanosis
Altered consciousness/confusion
```
76
Life-threatening asthma exacerbation - "CHEST"
```
Cyanosis
Hypotension
Exhaustion
Silent chest
Tachycardia
```
77
Mild-moderate Asthma exacerbation - management
Admission if poor response
SABA via spacer (up to 10 puffs every 2 mins)
Consider PO prednisolone
78
Moderate-severe Asthma exacerbation - management
Admission
O2 via mask (if sats <94%)
SABA via nebuliser (if on O2)
PO prednisolone 20mg
79
life-threatening Asthma exacerbation - management
Initially
- Urgent admission
- O2 (if sats <94%)
- SABA + ipratropium via nebuliser (every 20-30 minutes)
- PO prednisolone / IV hydrocortisone
If no improvement:
- Contact PICU for review
- IV treatments – magnesium, aminophylline
- Blood gases
80
When can a patient be discharged after admission for an asthma exacerbation?
May be discharged when off nebulisers and >4 hours between inhalers:
- Continue SABA PRN
- Continue prednisolone PO for 3-5 days.
GP follow-up in 48 hours
81
What is the difference between normal saline and Hartmann's solution?
Hartmann’s is considered to be more “physiological” than Normal Saline as it contains other electrolytes in concentrations similar to plasma.
Both are distributed in the intra-vascular and interstitial spaces, making them useful for both resuscitation and fluid maintenance.
82
How to calculate fluid requirements in children?
First 10kg weight => 100 mL/kg OR 4 mL/kg/hour
Second 10kg weight => 50 mL/kg OR 2 mL/kg/hour
All additional Kg weight => 20 mL/kg OR 1 mL/kg/hour
83
Which solutions should be used for maintenance fluids in children?
Isotonic crystalloids that contain sodium in the range 131-154 mmol/L must be used as first line maintenance fluids and for resuscitation, commonly used with 5% glucose
AVOID fluids with low/no saline
84
Why should fluids with no/low saline be avoided for maintenance fluids in children?
Children are more prone to dilutional hyponatraemia than adults
Increased ADH production during illness causes retention of water, and thereby increased risk of dilutional hyponatraemia
85
Monitoring for IV fluids
Check U&Es at least daily (every 4-6 hours if abnormal)
Assess fluid balance (input vs output) and hydration status
Check blood glucose at least every 12 hours
If hyponatraemia symptoms – check U&Es, glucose and serum osmolality immediately
86
Dilutional Hyponatraemia
the most common electrolyte abnormality seen in patients on IV fluids
Predominantly neurological symptoms – seizures, drowsiness, confusion.
Hyponatraemic seizures (usually generalized tonic-clonic) are a medical emergency
87
Recognising hyponatraemia
Na+ of 130-135 is often asymptomatic
Na+ <130 is mainly neurological symptoms:
- Headache
- N+V
- Lethargy, irritability
- Hyporeflexia
- Decreased conscious state
- Seizures
- Dry, inelastic Skin
- Apnoea
88
How much potassium should be added to IV fluids?
If K+ serum level is in range when starting fluids, aim to give about 1mmol/kg/day
=> split across fluid bags and then adjust according to U&Es
If K+ serum level is unknown when starting fluids – give with caution/avoid until serum level known and urine output established
89
How can you recognise dehydration in a child?
Gold standard = acute weight loss
Normally just estimated clinically:
3 groups to consider:
1. No clinically detectable dehydration
2. Clinical Dehydration
3. Clinical Shock – ~10% dehydrated.
90
What features might suggest clinical dehydration?
Tachycardia
CRT >2s
Decreased skin turgor
Tachypnoea
91
What features might suggest clinical shock in a dehydrated child?
```
Weak pulses
CRT >3s
Decreased skin turgor
Hypotension
Decreased consciousness
Very tachycardic
```
92
Management of a child who is clinically dehydrated
ORT 50ml/kg over 4 hours PLUS maintenance fluid (via NG tube if needed)
Use IV fluids if child deteriorates despite ORT/persistent vomiting of anything given PO or NG tube
93
Management of a child who is clinically shocked
Sodium chloride 0.9% 20mL/kg IV bolus
=> repeat if remains shocked
=> refer to PICU if shocked after 2nd bolus
One shock resolved, commence IV deficit replacement
Attempt to stop IV fluids early, with gradual introduction of ORT during IV fluids.
94
What is the size of an IV fluid bolus in a child?
20 mL/kg
95
Oral Rehydration Therapy
Dioralyte – 1 sachet in 200ml.
- Under 5 years – aim for 50mL/kg over 4 hours, plus maintenance volume.
- Over 5 years – 200mL after each loose stool (in addition to normal fluid intake)
96
How to calculate IV fluid replacement
if shocked, then add 100 ml/kg to maintenance and give over 24 hours
if not shocked, add 50 mL/kg to maintenance and give over 24 hours
97
Fluid choice for resuscitation vs replacement
Resuscitation – isotonic crystalloid without glucose.
| Replacement – isotonic crystalloid with glucose.
98
HYPERtonic dehydration
How is this caused?
Presentation?
How is it corrected?
Na+ >150
Can occur through severe, acute water loss.
More commonly occurs by parents making up feeds incorrectly
Child often hungry, but few signs of dehydration. Skin can be “doughy” and metabolic acidosis present.
Aim for correction over 48 hours and fall of <0.5 mmol/L Na+ per hour.
99
HYPOtonic dehydration
Na+ <130
Usually dilutional
Child lethargic and skin dry/inelastic
Give maintenance fluids plus deficit as calculated, checking U&Es every 4 hours.
100
how should hypertonic dehydration NOT be managed?
DO NOT manage hypertonic dehydration with fluid with no sodium – rapid drops in serum sodium causes a relatively high CSF sodium, attracting water into the CSF (can be fatal).
101
How is the foetal circulation different to "normal" circulation?
- Left atrium pressure is low, as little return from the lungs
- Right atrium pressure is high, as it receives all systemic blood (including from placenta).
- RA pressure keeps foramen ovale open, and blood flows from RA to LA.
- Blood also bypasses lungs by flowing through the ductus arteriosus.
102
How does the foetal circulation change at birth?
Decreased pulmonary resistance => more blood into LA => increased LA pressure
Loss of placental circulation => decreased RA pressure
Foramen ovale closes
Ductus arteriosus closes within the first few hours/days
103
What are "duct-dependent lesions?"
In severe left-sided obstructions, blood flow through the ductus arteriosus is critical for survival.
There will be a dramatic deterioration when the duct closes
=> Tetralogy Of Fallot, TGA, HLHS, aortic stenosis/coarctation
104
Congenital heart disease - Holes
atrial septal defect (ASD)
ventricular septal defect (VSD)
Atrioventricular septal defect (AVSD)
105
Congenital heart disease - pipes/valves
Patent ductus arteriosus (PDA)
Aortic/pulmonary stenosis
Coarctation of the aorta
106
Congenital heart disease - CYANOTIC diseases
```
Transposition of Great Arteries (TGA)
Tetralogy of fallot
Hypoplastic left heart
Tricuspid Atresia
Pulmonary Atresia
```
107
How can congenital heart conditions present?
1. Antenatal cardiac USS diagnosis (at 20 weeks)
2. Detection of a heart murmur
3. Heart failure
4. Cyanosis
5. Shock and collapse
108
Innocent heart murmurs
30% of children will have at some point (often during febrile illness/anaemia)
= Soft, Systolic, Sternal Edge, Asymptomatic, No thrills and normal CXR/ECG
109
Presentation of atrial septal defect
Child will generally be fairly well
Often presents when the child visits the GP for another reason and a murmur is heard = EJECTION SYSTOLIC murmur (split S2)
110
Why are ASD/VSDs and PDA not cyanotic diseases?
Left-to-right shunt => no cyanosis
111
Atrial septal defect - Investigations
ECG - potentially partial RBBB/right axis deviation
CXR - pulmonary oedema, cardiomegaly
112
Is patent foramen ovale an ASD?
It is a normal variation of the anatomy
Fairly common and goes unnoticed.
113
Atrial septal defect - Management
Usually no urgent treatment required
Can close spontaneously
Keyhole or surgical procedure to close it at ~4 years
114
What is the most common congenital heart defect?
VSD
| 2nd most common = coarctation of aorta
115
When is a VSD normally found?
1. Identified as incidental finding at 6-week baby check (by that point the right-sided pressure has come down and the murmur will be audible)
2. Failure to thrive.
116
What type of murmur occurs with a VSD?
= Pansystolic
The volume of the murmur does not correlate well with the size
117
Ventricular septal defect - Investigations
ECG – Right axis deviation and biventricular hypertrophy.
CXR – pulmonary oedema, cardiomegaly
118
Ventricular septal defect - Management
Can resolve spontaneously
Surgery to fix at <1 year.
119
What is Maladie de Roger ?
A very small VSD
| => High pitched squeaking sound
120
What is Eisenmenger's Syndrome?
Long standing left-to-right shunt (e.g. untreated VSD) causes right ventricular hypertrophy
Eventually this leads to reversal of the shunt to a cyanotic right-to-left shunt.
Incidence of Eisenmenger’s is decreasing - earlier diagnosis and management
121
What genetic condition is strongly associated with AVSD?
Trisomy 21
| 90% of AVSDs will be in children with Down’s
122
What type of murmur occurs with an AVSD?
= Pansystolic murmur
123
Atrioventricular septal defect - Investigations
ECG – superior (north-west) axis
CXR – pulmonary oedema, cardiomegaly
124
Atrioventricular septal defect - Management
Surgery <6 months
125
Patent Ductus Arteriosus
= Failure of duct closure by 1 month after estimated date of delivery (not pathological if pre-term).
Blood flows from the aorta to the pulmonary artery (i.e. a left-to-right shunt)
126
Presentation of PDA
Normally asymptomatic
Can have heart failure and pulmonary hypertension if duct is large
127
What type of murmur occurs with PDA?
A continuous, “machinery-like” murmur
128
Patent Ductus Arteriosus - Investigations
Echo – shows patent duct
CXR and ECG – usually normal
129
Patent Ductus Arteriosus - Management
Ibuprofen
Surgical tying
Closure with coil/occlusion device
130
What is aortic Stenosis?
| What can it be associated with?
= Narrowed/deformed aortic valve
Can be associated with bicuspid aortic valve, Turner’s Syndrome
131
When does aortic stenosis normally present?
If Critical – shortly after birth with collapse
If Mild – chest pain on exercise
132
Presentation of aortic stenosis
Inadequate cardiac output leads to shock presentation:
- Globally poor peripheral pulses
- Tachypnoea
- Mottled/grey appearance
133
Aortic stenosis - on examination
loud ejection systolic murmur,
Right sternal edge (2nd ICS) radiates to the carotid arteries
134
Aortic stenosis - Investigations
ECG – left axis deviation, left ventricular hypertrophy
135
What is pulmonary stenosis?
| What can it be associated with?
= Narrowed/deformed pulmonary valve.
Can be associated with Noonan’s Syndrome and William’s syndrome
136
Pulmonary stenosis - on examination
Murmur – ejection systolic murmur at left sternal edge
RV heave
137
Pulmonary stenosis - Investigations
ECG – RV hypertrophy
138
What is coarctation of the aorta?
| What can it be associated with?
= narrowing of the aorta,
usually distal to the branches supplying the upper limbs
Can be associated with bicuspid aortic valve and Turner’s syndrome
139
How does coarctation of the aorta present?
Affects lower limbs, not upper limbs – Absent femoral pulses
Lack of blood flow to the rest of the body => renal failure/gut ischaemia
Can cause back up of flow into LV, eventually causing cardiac failure
140
Coarctation - on examination
ejection systolic murmur, heard between shoulder blades
141
Coarctation - management
Resuscitation
| Cardiac catheterisation – balloon or stent
142
What is transposition of the great arteries?
Aorta connected to the RV, and pulmonary artery connected to the LV.
=> Incompatible with life, unless blood can mix.
There will be CYANOSIS when ductus arteriosus closes (around day 2)
143
transposition of the great arteries - on examination
Cyanosis without tachypnoea.
One loud, single 2nd heart sound.
May be a murmur if another defect is present, but often no murmur.
144
transposition of the great arteries - Investigations
Echo – visualise abnormalities
ECG – usually normal
CXR – “egg on a side” outline of heart, increased pulmonary markings
145
transposition of the great arteries - Management
Maintain duct patency – prostaglandin analogue
Balloon atrial septostomy – create hole in atrial septum to allow blood mixing.
Arterial switch procedure – pulmonary a. and aorta transected and switched, performed <4 weeks.
146
What are the four cardinal features of Tetralogy of Fallot?
1. Large VSD
2. Aorta overriding the ventricular septum (receives blood from both ventricles)
3. Sub-pulmonary stenosis (= RV outflow obstruction)
4. RV hypertrophy (as a result of RV outflow obstruction)
147
what genetic condition can Tetralogy of Fallot be associated with?
DiGeorge Syndrome
148
Tetralogy of Fallot - On Examination
CYANOSIS in 1st week
=> Variable – can have pink/blue fallot
Loud, harsh ejection systolic murmur (VSD and PS)
Clubbing (older children)
149
Tetralogy of Fallot - Investigations
Echo – shows cardinal features
CXR – small, “boot-shaped” heart
ECG – RV hypertrophy
150
Tetralogy of Fallot - Management
Maintain duct patency
Surgery at 6 months – close VSD, relieve RV outflow obstruction.
Treatment of hyper-cyanotic “TET spells”
- Knees to chest, sedation, pain relief (morphine)
- O2 +/- ventilation
- IV propranolol (to relax RV)
- IV fluids
151
What are TET SPELLS?
= worsening of right-to-left shunt
During exertion/crying/agitation causing increased pulmonary resistance
Causes cyanotic episodes
If severe = drowsy, seizures, death.
152
What is hypoplastic left heart and how does it present?
= underdeveloped left heart (including aorta).
Often detected antenatally so symptoms are prevented, but symptoms can be:
- Profound acidosis and CV collapse
- Weakness of all peripheral pulses
153
Hypoplastic Left Heart - management
Norwood procedure (3-stage surgery)
154
Cardiac Failure in children - signs and symptoms
What is an important differential to consider?
Signs and symptoms:
- Failure to thrive
- Breathlessness (especially when feeding)
- Sweating when feeding
- Ventricular Heave
- Backlog of pressure – crackles on lungs, big liver
Differential – SEPSIS!
155
Management of cardiac failure
Diuretics, inotropes, treat cause
156
Supraventricular Tachycardia - signs/symptoms
= HR 250-300bpm
Signs/Symptoms:
- Dizziness, palpitations, chest pains, SoB
- Tachyarrhythmia (abnormal, fast HR)
- Heart failure (pulmonary oedema, increased RR)
- Hydrops fetalis (abnormal fluid accumulation), causes intrauterine death.
157
Supraventricular Tachycardia - Investigations
- ECG – 250-300bpm and narrow QRS (P-waves often hidden)
| - Echo – to r/o structural problem
158
Supraventricular Tachycardia - Management
1. Acute:
• Circulatory and respiratory support
• Carotid sinus massage/ice pack on face (successful in ~80%)
• IV adenosine (induces AV block)
• Electrical cardioversion with synchronised defib shock.
2. Maintenance:
• Flecainide or Sotalol
• Radiofrequency ablation/cryoablation
159
What is myocarditis?
| What causes it?
= Inflammation of the myocardium
Usually due to infection, but also drug reaction/ chemicals/ radiation
Common viral causes = parvovirus, influenza, adenovirus, rubella, HIV
160
Myocarditis - signs/symptoms
Fever/malaise
Non-specific Sx of heart failure: SoB, cough, chest pain, oedema, pallor
161
Myocarditis - Management
Usually resolves spontaneously
Diuretics, ACEi, Beta blocker (carvedilol)
Severe cases may need heart transplant
162
What is Subacute Bacterial Endocarditis?
Most common cause in children?
Biggest risk factor in children?
= Slowly developing infection of the endocardium.
Alpha-haemolytic strep is the most common cause in children
Congenital heart defects are a BIG risk factor (especially VSD, PDA, coarctation)
163
Subacute Bacterial Endocarditis - symptoms
Fever, malaise and a NEW murmur
Also:
- Anaemia/pallor
- Arthritic/arthralgia
164
Subacute Bacterial Endocarditis - signs
Microscopic haematuria
+/- splinter haemorrhages
+/- Osler’s nodes, Janeway lesions, Roth spots
165
Subacute Bacterial Endocarditis - Investigations
Blood cultures (before starting Abx)
Echo – visualise vegetations
Bloods – anaemia, increased ESR/CRP
Urine dip – microscopic haematuria.
166
Subacute Bacterial Endocarditis - Management
Abx
| => high dose IV penicillin + gentamicin for 6 weeks
167
when is the neonatal period?
<28 days
168
When is a child considered pre-term?
Born <37 weeks
169
When is a child considered post-term?
Born >42 weeks
170
What is considered low birthweight?
<2500g
171
What is considered very low birthweight?
<1500g
172
What is considered extremely low birthweight?
<1000g
173
Respiratory Transition in the newborn
Alveoli will transition from fluid-filled to air-filled in order to survive without the placenta.
1. Fluid resorption starts before birth through lymphatics and circulatory system.
2. Some fluid is squeezed out during labour.
3. Breathing is stimulated by cold/touch/light and loss of placental gas transfer.
Babies take large, deep breaths (crying).
To keep the lungs open, you need to overcome the surface tension in the alveoli (surfactant is needed for this).
174
Positive end-expiratory Pressure (PEEP)
= the pressure in the lungs (alveolar pressure) above atmospheric pressure, that exists at the end of expiration
175
Extrinsic/Applied PEEP
applied by a ventilator
| = used for most mechanically ventilated patients to prevent end-expiratory alveolar collapse
176
Intrinsic/Auto PEEP
caused by incomplete exhalation
=> causes air trapping (hyper- inflation).
Develops commonly in hyperventilation, obstructed airway or increased airway resistance
177
What are causes of respiratory distress in neonates?
Respiratory Distress Syndrome
Meconium aspiration Syndrome
Infection
Pneumothorax
Congenital Causes
Transient tachypnoea of newborn (TTN)
178
Meconium aspiration Syndrome
Distress causes the baby to inhale meconium, which is toxic to the lungs.
Meconium is present at delivery
RFs - post-term delivery
179
What are the potential issues associated with prematurity?
Respiratory Distress Syndrome
Chronic lung disease
Retinopathy of prematurity
Intracranial haemorrhage
Long-term neurodisability
Temperature control (hypothermia)
Infection
Hypotension
Patent Ductus Arteriosus
Bradycardia
Metabolic problems (Hypoglycaemia, Hypocalcaemia, Metabolic bone disease of prematurity)
Feeding difficulties
Necrotising Enterocolitis
GORD
180
why is intracranial haemorrhage a problem in pre-term infants?
The pre-term brain is very susceptible to bleeding
181
What is early-onset sepsis?
| What organisms usually cause it?
Sepsis that starts near the time of delivery
Group B Streptococcus (Strep agalactaie)
E. coli
H. influenzae
Listeria monocytogenes
182
Group B strep infection of the newborn
= most common cause of neonatal infection (sepsis/meningitis/pneumonia)
Infection is prevented by giving antibiotics >2 hours before delivery
Infection is treated with benzylpenicillin and gentamicin
183
Investigations in suspected sepsis in a newborn
The “sepsis screen”:
- Blood culture
- Urine – SPA/clean catch
- Lumbar puncture
- CXR
- FBC, CRP
184
Why would a child need IV maintenance fluids?
if NBM or not taking enough orally
185
What is a risk with 0.9% saline that is less of a risk with Hartmann's solution
0.9% saline has increased risk of hyperchloraemia
186
How do you calculate a child's fluid deficit ?
Deficit (mL) = % dehydration x bodyweight (kg) x 10
187
"Bottom shufflers"
replacement for crawling
| Child may not have tolerated tummy time therefore less development of arms, neck and back.
188
what are the developmental red flags?
No responsive smile by 8 weeks
Not achieved good eye contact by 3 months
Not reaching for objects by 5 months
Not sitting unsupported by 9 months
Not walking unaided by 18 months
Not saying single words with meaning by 18 months
No two/three word sentences by 30 months
189
What are the neurodevelopmental conditions?
* ADHD
* ASD
* Intellectual disability
* Cerebral palsy
* Attachment disorders
* Mood disorders
* Anxiety Disorders
* Impulse control disorders
190
What is ADHD?
| What factors are needed for a diagnosis?
Inattention and hyperactivity/ impulsivity
Occurring <12 years and lasting >6 months
Occurring in 2 settings (e.g. home and school)
Interfering with social, academic or occupational functioning
Not explained by another disorder (e.g. oppositional defiant disorder)
Often also difficulties with emotional regulation and executive function
191
Epidemiology of ADHD
M>F 4:1 in childhood
50% of children with ADHD will have ADHD as an adult
=> M:F 1:1 in adulthood
Strong genetic component (multiple genes)
192
What FHx of ADHD increases the risk of a child having it?
1st degree relatives 4-5x more likely to have ADHD
10-fold risk among siblings of individuals with combined type
193
ADHD - pathophysiology
Linked to dysregulation of dopamine + noradrenaline in the brain
=> Dopamine involved in reward, risk-taking, impulsivity, mood.
=> NA involved in attention, arousal, mood
194
Pharmacological Rx of ADHD
1st line = methylphenidate (Equasym/Concerta/Ritalin)
=> MOA: blocks dopamine and noradrenaline transporters to increase the concentration within synapse
Taken around breakfast time; covers school hours
Prolonged action preparations available for home symptoms later in the day
195
What are the side effects of methylphenidate used in ADHD?
| How are these prevented/managed?
* Suppression of appetite, causing impaired growth (good breakfast, 3 meals + snacks; monitor weight + height)
* Hypertension (monitor BP)
196
Non-pharmacological Rx of ADHD
ADHD medication is unlikely to have significant impact on behaviour without parenting support and access to behavioural management advice
197
What 3 areas are impaired in autistic spectrum disorder?
1. Social & emotional interaction
2. Imagination and flexibility of thought
3. Social communication and language
May also have sensory difficulties (inc. touch, noise, light, smell, movement, food textures)
198
Autistic Spectrum Disorder - epidemiology
* Incidence – 1 in 100
* M>F 4:1 (but may be under-recognised in girls)
* Strong genetic component
* Can be a component of other conditions (e.g Rett syndrome, Fragile X, tuberous sclerosis, Down Syndrome, William Syndrome and many others)
199
Autistic Spectrum Disorder - management
No pharmacological Rx for autism itself (though melatonin may help with sleep disturbance)
Management centred around behavioural strategies, parental support and optimising environment to allow development
200
Intellectual disability
Affects ~1% of population
Variable severities of disability
Characterised by deficits in intellectual functioning (e.g. communication, learning, problem solving) and adaptive behaviour (e.g. social skills, routines, hygiene)
201
IQ testing to quantify intellectual impairment
Mild <70
Moderate <50
Profound <35
Severe <20
202
Intellectual disability - management
* Identification of co-existing medical conditions
* Family support
* Behavioural support
* Educational support
203
Educational, Health and Care Plans
A legal document
Describes a child’s educational, health and social care needs.
Details extra help that will be provided to meet those needs and how that help will support the child to achieve what they want to in their life.
204
Normal Pubertal Onset in F and M?
Girls – before age 8 is early, after 13 is late
Boys – before age 9 is early, after 14 is late.
205
How is bone age classically identified?
By an X-ray of the left wrist
206
Surgical assessment of acute abdominal pain
All patients require a full history and examination.
Girls - include a gynae history and pregnancy test if pubertal.
Boys - requires a testicular examination as it is a source of referred pain.
207
Management of acute abdominal pain
Most management plans will include analgesia, nil by mouth, IV access, bloods, fluids and imaging if necessary
208
Idiopathic abdominal pain
accounts for 30-40% of referrals to paediatric surgery,
It is a diagnosis of exclusion and requires careful review
209
Very common causes of acute abdominal pain
```
Gastroenteritis
Acute appendicitis
UTI
Constipation
Mesenteric Adenitis
```
210
Less common causes of acute abdominal pain
```
Strangulated Hernia
Intussusception
Pancreatitis
Intestinal Obstruction
Lower Lobe pneumonia
DKA
Henoch-schonlein Purpura
```
211
Rare causes of acute abdominal pain
```
Haemophilia
Lead poisoning
Acute porphyria
Sickle-cell anaemia
Herpes Zoster
```
212
What is the most common reason for acute surgical intervention?
Appendicitis
213
What is the typical presentation of appendicitis if the appendix is located in RIF, anterior to bowel?
* obvious RIF tenderness, guarding, rebound tenderness.
* In this position the diagnosis is typically easy to make and made early as it is the anterior peritoneum that is inflamed, and the symptoms localised.
214
What is the typical presentation of appendicitis if the appendix is located in RIF, posterior to bowel?
* Vague deep tenderness, can have guarding, may develop mass or perforation before diagnosis.
* This is a hard diagnosis to make and often delayed in presentation.
* The posterior peritoneum is inflamed and/or psoas muscle (giving the child the desire to limp to alleviate the pain).
* Few anterior abdominal wall signs.
215
What is the typical presentation of appendicitis if the appendix is located in the pelvis?
* Vague suprapubic tenderness, with no guarding.
* PR exam may illicit tenderness or a mass.
* Possible to have urinary/bowel symptoms due to irritation of these structures.
* Commonly perforated as it is hardest to diagnose and present late.
* Few anterior abdominal wall signs.
216
Management of suspected appendicitis?
Bloods and IV fluids,
Urine dip,
Good analgesia - Paracetamol and IV morphine if needed.
Consider NG tube,
Consider antibiotics
Contact the relevant on call surgical team.
=> the mainstay of treatment is surgery
217
What is the average morbidity from appendicitis?
10%
due to post operative collections, prolonged stay, wound infections, adhesional obstruction.
218
when are most abdominal wall defect discovered?
in antenatal scans
219
What is gastroschisis?
A congenital defect of the abdominal wall, usually to the right of the umbilical cord insertion.
Abdominal contents herniate into the amniotic sac, usually just involving the small intestine but sometimes also the stomach, colon and ovaries.
There is no covering membrane.
220
What is a risk factor for gastroschisis?
| Any associated conditions?
More commonly seen with younger maternal age
Commonly an isolated anomly however, it may be associated with Arthrogryposis
221
Gastroschisis - acute management
careful fluid balance, IV antibiotics and placing lower half of body in a protective bag.
=> Prevent temperature and fluid losses as well as reducing infection risk.
222
What is Exomphalos?
A congenital abnormality in which the contents of the abdomen herniate into the umbilical cord through the umbilical ring.
The viscera, which often includes the liver, is covered by a thin membrane consisting of peritoneum and amnion
223
Are any conditions associated with exomphalos?
Commonly associated with other abnormalities, therefore examination and investigation for genetic abnormalities may be required.
=> Associations with trisomy 13, 15 and 18 as well as Beckwith Wiedemann syndrome are well documented.
224
Exomphalos - acute management
* Careful fluid balance
| * IV antibiotics.
225
What is the estimated blood volume of a child?
= 80 mL/kg
226
What is the estimated weight of a child?
< 9 years = 2 x (age +4)
> 9 years = 3 x age
227
What might be considered a better fluid bolus in trauma?
10 mL/kg as there is a risk of disturbing a potential clot and exacerbating further bleeding.
228
Managing blood loss
Blood replacement:
- give type specific blood as a bolus and then reassess to see if more is required.
- When type specific blood isn’t available then O negative blood is adequate.
Before blood products are available then saline 0.9% is a good resuscitation fluid
229
What is intussusception?
| Where does it normally occur?
= a type of bowel obstruction, normally occurs in the ileocaecal region
occurs when one segment of the bowel invaginates into another segment just distal to it, the venous blood flow is restricted, swelling occurs and it becomes stuck. This causes obstruction.
230
Intussusception - presentation
Pain (80-90%):
- Often colicky in nature
Vomiting (90%):
- Will often progress to becoming bile stained.
```
Abdominal mass (70%)
- Sausage shaped mass in RUQ
```
Lethargy/hypotonia (70%)
- a non-specific symptom.
Shock
```
Altered Stool (55%):
- “Redcurrant jelly” stools (a late sign)
```
231
What are the 3 "food signs" of intussusception?
Sausage-shaped mass in RUQ
Donut sign on USS
Redcurrent jelly stools
232
Causes of intussuception
```
Idiopathic,
Meckel's diverticulum,
Polyps,
Peutz-Jegher's,
Tumours,
Inflamed appendix,
Foreign body,
Peyer's patch hypertrophy.
```
233
Intussuception - management
A-E Assessment – stabilise the child.
Surgical involvement is necessary – air reduction enema +/- laparotomy.
234
Congenital Diaphragmatic Hernia
when the normal diaphragm process is incomplete and the bowel herniates through and into the thoracic cavity.
This pressure can then stop the lung buds from being able to form into normal lungs (the earlier it occurs, the more impact it has on the developing lung)
235
When is the diaphragm normally fully formed during foetal development?
by 20 weeks, along with gut and lung formation
236
Do left or right sided diaphragmatic hernias have a better prognosis?
Left sided have a better prognosis than right sided (due to the solidity of the liver on the right side preventing the development of lung).
237
Can a congenital diaphragmatic hernia be detected on antenatal scans?
Mostly, yes
238
Congenital diaphragmatic hernia - management
Early recognition
intubation without bag and mask ventilation (to not inflate the stomach) and to watch and wait for the first 48 hours.
If the child is stable enough after this period of time then they will have the defect repaired surgically through either a lateral thoracotomy of through an abdominal approach.
239
Oesophageal Atresia / TOF
= incorrect development of the oesophagus so that the lumen is no longer patent.
As part of this anomaly the oesophagus can make an incorrect attachment to the trachea and create a tracheo-oesophageal fistula.
240
Can oesophageal atresia / TOF be identified antenatally?
Less well identified antenatally
often suspected when a newborn child either wont feed, vomits, has lots of salivary secretions and/or respiratory distress
241
Management of oesophageal atresia / TOF
Surgical intervention is performed to reconnect the two ends of the oesophagus and allow feeding to commence,
However when a child is unstable the most pressing issue is to disconnect the fistula.
=> Both procedures are typically performed through a right lateral thoracotomy.
242
Common causes of Neonatal Bowel Obstruction
Hirschsprung’s Disease
| Necrotising enterocolitis
243
Less common causes of Neonatal Bowel Obstruction
```
Small bowel atresia
Imperforate anus
Duodenal atresia/stenosis
Malrotation with volvulus
Meconium Ileus
```
244
Duodenal atresia
= narrowing of duodenum
Can have different types ranging from a narrowed lumen to several discrete atretic segments with separated blood supply.
245
bile stained vomit in duodenal atresia
If the atresia occurs prior to the midpoint of the second part of the duodenum then the vomiting won’t be bile stained.
=> This can sometimes delay the diagnosis by several days and result in a very sick child.
246
Duodenal atresia - management
1. resuscitate the child
| 2. intervene to remove the atretic segment and try and restore bowel continuity
247
Small Bowel Atresia
= narrowing of small bowel
This can be caused by thrombo-embolus, volvulus or intussusception
248
Small Bowel Atresia - management
| and outcome?
1. resuscitate the child
2. intervene to remove the atretic segment and try and restore bowel continuity.
=> Sometimes this isn’t possible and a stoma is formed.
If the child loses a significant portion of bowel then they may never be able to absorb enough nutrients and have a life dependant on total parenteral nutrition
249
what is necrotising enterocolitis?
Inflammation and necrosis of intestinal tissue
More common in premature babies
Results from immature guts not being able to stop translocation of gut flora.
Can cause sudden and dramatic deterioration and death
250
Necrotising enterocolitis - management
Early recognition => triple antibiotic therapy and NBM
Surgical intervention may be required to remove non-functional bowel and restore continuity or create a stoma.
251
what is Hirschsprung's disease?
Caused by defective nerve growth into the myenteric plexi of the bowel.
It can effect a small section of the bowel or the whole of the digestive tract.
=> the extent of the disease has a direct effect on severity of symptoms and time of diagnosis
252
How does Hirschsprung's disease present?
| How is it diagnosed and managed?
delayed passage of meconium and episodes of enterocolitis.
Dx is made via a rectal biopsy.
Mx - often requires removal of the non-functional bowel and to restore continuity prior to potty training age
253
Meconium ileus
born with very thick meconium and are unable to pass it
Commonly the child will pass the meconium (sometimes requiring a washout) and the symptoms will resolve.
One must think about the possibility of cystic fibrosis as a cause of thickened meconium and consider testing for it.
254
Where can testicular pain be referred to?
the abdomen
255
Testicular Torsion
Occlusion of the testicular blood vessels will affect the viability of the testis unless prompt action is taken.
It is more frequently seen in the left testis
256
Torted Hydatid of Morgagni
A small embryological remnant at the upper pole of the testis.
Torsion of the hydatid is of no consequence in itself except that it presents a similar picture to torsion of the testis.
The pain is usually less severe and of a longer duration than a torted testis.
Occasionally the torted hydatid may be palpable or visible as a 'blue dot' in the scrotum.
If in doubt, then one must explore surgically.
257
Torted Epididymal Cyst
This is a smooth, small fluid filled swelling that slowly develops in the epididymis.
They are often painless, but the affected testis sometimes ache or feel heavy.
It is not clear what causes cyst development, but they tend to be more common in middle aged men.
258
Epididymo-Orchitis
Inflammation of the epididymis and/or testis.
It is usually due to infection, most commonly from a urinary tract infection or a sexually transmitted infection.
A course of antibiotics will usually clear the infection. Most people make a full recovery without complication.
259
Idiopathic Scrotal Oedema
A self-limiting condition characterised by marked oedema +/- erythema.
Important to recognise to prevent unnecessary surgical exploration.
Most common under the age of 10.
Unknown aetiology.
Tends to resolve in 3 - 5 days.
Reassurance and analgesia are the mainstays of treatment
260
Trend of eGFR in children
Low in newborns
| Rises rapidly in 1-2 years.
261
Trend of plasma creatinine in children
Increases throughout childhood with height and muscle
262
DMSA scan
Static scan of renal cortex
Shows functional defects (e.g. scars), can help to give differential function between the two kidneys.
!! Can give false positives if done within 3 months of UTI.
263
What are congenital abnormalities of the kidneys?
1. Renal Agenesis
2. Multicystic Dysplastic Kidney (MCDK)
3. Polycystic Kidney Disease (PKD)
4. Horseshoe/Pelvic Kidney
5. Duplex Kidney
6. Posterior Urethral Valves
264
What is bilateral renal agenesis?
What does this lead to?
= absence of both kidneys
Results in oligohydramnios, due to no foetal urine.
Complications: FATAL potter syndrome.
265
What is Potter Syndrome?
= physical characteristics that can occur due to oligohydramnios
- distinctive facial features
- skeletal abnormalities
- lung hypoplasia
266
What is Multicystic Dysplastic Kidney (MCDK)?
What are the complications and management?
Caused by failed union of ureteric bud and renal mesenchyme.
Results in non-functioning kidney with variably sized fluid-filled cysts and narrow ureter.
Complications: Potter syndrome if bilateral.
Management: often no Tx needed, sometimes nephrectomy if remain large.
267
What is Polycystic Kidney Disease (PKD)?
How is it inherited?
What are the complications?
Always bilateral, but some/normal renal function is maintained.
Results in enlarged kidneys with separate, discrete cysts.
Either autosomal dominant or autosomal recessive.
Complications – HTN, haematuria, renal failure.
268
What is Horseshoe Kidney?
What are the complications?
Lower poles of the kidney are fused at the midline.
Complications – increased risk of obstruction/infection
269
what is Duplex Kidney?
what are the complications of this?
Either 2 renal pelvises or 2 complete ureters for 1 kidney
Complications – reflux in lower ureter, ectopic drainage/prolapse of upper ureter
270
What are Posterior Urethral Valves?
What are the complications?
How is this managed?
Abnormal flaps of tissue/membranes grown in the urethra, obstructing drainage from the bladder.
Complications:
- Hydronephrosis
- Small, dysplastic, non-functioning kidney
- Reduced amniotic fluid leading to Potter Syndrome
Mx: cystoscopic ablation of valves
271
What is the most common cause of bladder outlet obstruction in male newborns?
Posterior urethral valves
272
How might UTI present in an infant?
NON-SPECIFIC
```
Fever
Vomiting
Lethargic/irritable
Poor feeding/FTT
Jaundice
Sepsis
Offensive urine
```
273
How might UTI present in a child?
```
Fever
Abdo/loin pain
Frequency/accidents
Dysuria/haematuria
Lethargic
Vomiting/anorexia
Offensive urine
```
274
Pyelonephritis/upper UTI in children
Fever >38o
+ bacteriuria
+/- loin pain
275
Cystitis/lower UTI in children
bacteriuria WITHOUT systemic Sx
276
“Atypical” UTIs in children
* Severely ill/sepsis
* Poor urine flow
* Abdo/bladder mass
* No Tx response within 48h
* Increased creatinine
* Non-E.coli organism
* FHx of urinary tract abnormality
277
UTI - important points to gather from Hx?
- Exact symptoms
- Antenatal scan results
- What is the urinary stream like?
- Voiding history in general
- Bowel habit
- Drinking habits
- Previous episodes?
278
UTI - important points to gather from examination?
- Appears well/unwell?
- Fever?
- Any spinal lesion/ lower limb neurology?
- Any palpable bladder or kidneys?
- Is blood pressure normal?
279
Urine dip specificity/sensitivity for UTI
Nitrites = specific (+ve result = likely UTI)
Leucocytes = sensitive (-ve result = unlikely UTI, but +ve result may be other cause [e.g. other febrile illness or balanitis/vulvovaginitis])
280
When is urine MC&S required in children with ?UTI ?
ALWAYS required unless nitrites AND leucocytes are negative on urine dip
281
UTI - when are further investigations required?
Only if recurrent/atypical UTIs
USS – shows structural abnormalities, renal defects, obstruction
DMSA – for scarring/VUR/obstruction
282
UTI - Management
If <3 months – URGENT HOSPITAL ADMISSION
=> IV Abx
If >3 months with upper UTI/pyelonephritis:
=> PO Abx for 7-10 days
If >3 months with lower UTI/cystitis:
=> PO Abx for 3 days
283
Causes of vesicoureteric reflux
Familial – 30-50% risk if 1st degree relative
Bladder pathology – neuropathic bladder, urethral obstruction, post-UTI
284
Types of vesicoureteric reflux and their management
Mild – reflux into ureter only
=> usually resolves within the first few years of life
Severe – reflux into kidney
=> prophylactic Abx, surgery
285
vesicoureteric reflux - investigations
MCUS – shows dilated ureters and direction of flow (diagnostic – shows grade of reflux)
MAG3 – shows direction of flow.
USS – shows abnormalities/obstruction
286
Complications or presentation of vesicoureteric reflux
Incomplete bladder emptying = risk of UTI
Intrarenal reflux = risk of pyelonephritis
High voiding pressures = transmitted back to kidneys, damaging renal papillae.
287
What is enuresis?
= involuntary micturition
288
Primary nocturnal enuresis
= “Bed wetting”
- Common – 10% at 5 years, 5% at 10 years.
- Need to R/o underlying cause
- Mx – encourage child/reward system, etc.
289
Daytime enuresis
= Lack of bladder control during the day in a child old enough to be continent (3-5 years)
290
Causes of daytime enuresis
```
Lack of attention to bladder sensation
Detrusor instability
Bladder neck weakness
Neuropathic bladder
UTI
Constipation
Ectopic ureter
```
291
Daytime enuresis - Ix
Neuro exam (gait, sensation, reflexes)
Spinal X-ray
Urine MCS
Bladder USS/urodynamic studies
292
Daytime enuresis - Mx
Treat underlying cause.
Anti-cholinergics (to decrease bladder contractions)
Star charts, bladder trainings, “damp alarms”, etc.
293
Secondary/onset Enuresis
= Loss of previously achieved continence.
294
Causes of Secondary/onset Enuresis?
```
Emotional upset (most common)
UTI
Osmotic diuresis (DM, sickle cell, chronic renal failure)
```
295
Secondary/onset Enuresis - Ix
Urine dip - ?DM, ?UTI
Urine osmolality
USS renal tract
296
Nephrotic Syndrome
= Inflamed basement membrane allows passage of proteins into nephron.
1. Heavy proteinuria (>200mg/day)
2. Hypoalbuminaemia (<25g/L)
3. Oedema – periorbital, legs, scrotal/vaginal
Can be either Steroid-sensitive or Steroid-resistant (rare)
297
Cause of nephrotic syndrome?
The cause is unknown, but may be 2o to systemic disease (e.g. HSP, SLE, infections, etc)
298
Clinical features of nephrotic syndrome
- Periorbital oedema – especially on waking
- Leg, ankle, scrotal/vulval oedema
- Ascites
- Breathless (due to pulmonary oedema)
- Frothy urine (proteinuria)
299
Nephrotic Syndrome - investigations
- Urine dip – protein
- FBC, ESR/CRP
- U&Es, creatinine, albumin
- Urine MCS
- Complement levels
- Throat swab/anti-strep
- Hep B & C Screen
- Malaria screen
300
Management of steroid-sensitive nephrotic syndrome
Corticosteroids (8 weeks PO prednisolone)
• Tx resistant cases need renal biopsy
• Frequent relapses need high maintenance dose steroids
Fluid balance
Abx Prophylaxis (loss of Ig leads them susceptible to infection)
Refer to specialist if atypical features
301
Management of steroid-resistant nephrotic syndrome
Fluid Balance
• Restrict intake of water and salt
• Diuretics, ACEI, NSAIDs
302
Haematuria - glomerular
Brown Urine, deformed RBCs, protein
CAUSES:
- Acute/chronic glomerulonephritis
- IgA nephropathy
- Familial nephritis
- Goodpasture’s Syndrome (anti-basement membrane)
303
Haematuria - non-glomerular
Red urine, no protein
```
CAUSES:
Infection
Trauma (to genitals/urinary tract/kindeys)
Stones
Tumours
Renal vein thrombosis
Sickle cell disease
Bleeding disorders
```
304
Haematuria - investigations
For all patients with haematuria:
- Urine dip & MCS
- FBC, ESR/CRP, platelets, clotting screen
- Sickle cell screen
- U&Es, creatinine, calcium, phosphate, albumin
- USS KUB
For suspected glomerular haematuria:
- Complement levels (often low)
- Anti-DNA antibodies (present in vasculitis)
- Throat swab + Antistreptolysin O/ Anti-DNAse B
- Hep B/C Screen
305
What is Nephritic Syndrome?
What are the clinical features?
Inflammation causes increased glomerular cellularity, which restricts blood flow and decreases filtration.
1. Haematuria (>10 red cell casts)
2. Oliguria (<0.5-1 mL/kg/hour)
3. Proteinuria (>3.0g/day)
Clinical features:
- Oedema (especially periorbital)
- HTN, causing seizures.
306
Causes of Nephritic Syndrome
```
Post-infection (often streptococcal)
Vasculitis (HSP, SLE, Wegener granulomatosis)
IgA nephropathy
Membranoproliferative glomerulonephritis
Goodpasture Syndrome
```
307
Nephritic Syndrome - management
Maintain fluid and electrolyte balance
Treat infection if present
If rapid decrease in renal function – renal biopsy, immunosuppression, plasma exchange.
308
Post-infective Nephritis
Occurs 7-21 days after a sore throat or skin infection
Low complement, raised ASO/Anti-DNAse B
309
Henoch-Shonlein Purpura - pathophysiology
= Increased circulating IgA and IgG form complexes that deposit in organs (e.g. kidneys, skin, joints)
310
Henoch-Shonlein Purpura - Clinical Features
* Palpable purpuric rash – symmetrical over buttocks and extensor surfaces/ankles
* Arthralgia and periarticular oedema
* Colicky abdominal pain
* Glomerulonephritis – micro/macroscopic haematuria
* +/- fever and malaise
311
Henoch-Shonlein Purpura - risk factors
* 3-10 years
* M>F (2:1)
* Winter
* Preceding URTI
312
Henoch-Shonlein Purpura - Management:
- Normally self-resolved in 6 weeks
- Analgesia
- Corticosteroids – only if severe gut/joint involvement
- Follow-up for 1 year if renal involvement to ensure no CKD
313
IgA Nephropathy
“Berger’s Disease”
Similar pathology to HSP (IgA vasculitis), but ONLY affects the kidneys.
314
Glomerulonephritis
= Group of diseases causing immune-mediated inflammation of the glomerulus (increased permeability)
Causes nephrotic/nephritic syndrome
315
SLE - cause, RFs, Sx
involves dsDNA autoantibodies, decreased complement
Risk factors – adolescent girls/young women, Asian/afro-Caribbean
Symptoms - haematuria and proteinuria, malar rash, malaise, arthralgia
316
What is AKI?
What are the causes?
= a sudden, potentially reversible drop in renal function.
= Oliguria <0.5 mL/kg
PRE-RENAL
=> hypovolaemia, circulatory failure
RENAL
=> vascular/tubular/glomerular/interstitial
POST-RENAL
=> congenital/acquired obstruction
317
AKI - management
1. Correct fluid and electrolyte balance / any metabolic acidosis
2. TREAT CAUSE:
- Pre-renal - Urgent IV fluids and circulatory support
- Renal - depends on cause
- Post-renal - urine drainage
318
AKI - indications for dialysis
- Failed conservative Mx
- Hyperkalaemia or hypo/hypernatraemia
- Severe acidosis
- Pulmonary HTN/oedema
- Multi-system failure
319
What is Haemolytic uraemic syndrome (HUS)?
A triad of:
1. Renal Failure
2. Thrombocytopaenia
3. Microangiopathic haemolytic anaemia (damaged RBCs due to small vessel occlusion)
320
Typical vs. non-typical Haemolytic uraemic syndrome
95% “typical” (diarrhoeal) – good prognosis
5% “atypical” (non-diarrhoeal) – poor prognosis
321
Pathogenesis of Haemolytic uraemic syndrome
Secondary to gastroenteric infection (diarrhoeal prodrome) with verocytotoxin-producing E. coli 0157:H7
Toxin causes intravascular thrombogenesis in renal endothelial cells
Clotting cascade becomes activated.
Platelets are consumed and haemolytic anaemia occurs.
322
Haemolytic uraemic syndrome - investigations
- FBC – low Hb, low platelets, fragmented blood film
- U&Es
- Stool culture
323
Haemolytic uraemic syndrome - management
- Supportive – manage fluid balance
- Dialysis and plasma exchange
- Follow-up – for persistent proteinuria, HTN, progressive CKD
324
CKD in children
CKD = progressive loss of renal function
Very rare in children (~10 in 1 million)!
325
Causes of CKD in children
- Structural malformation
- Glomerulonephritis
- Hereditary neuropathies
- Systemic diseases
- Unknown
326
Clinical features of CKD in children
- Anorexia, lethargy
- Polydipsia & polyuria
- Hypertension
- Bone deformities
- FTT, poor/delayed growth (despite high GH levels)
- Anaemia (unexplained, normocytic)
327
CKD - management
Management with specialist paediatric nephrologist and MDT
Aim = to prevent metabolic complications and allow normal growth & development.
1. Diet – supplements, ?NG tube/gastrostomy
2. Prevent renal osteodystrophy – calcium and phosphate restriction, vitD supplements
3. Fluid and electrolyte balance
4. Anaemia – recombinant EPO
5. Hormone abnormalities – recombinant human GH.
Dialysis and transplantation for end-stage CKD.
Ideally child gets transplant before dialysis is needed.
328
Normal RR, HR, SBP in neonates <28 days
RR - 50-60
HR - 110-160
SBP - 50-70
329
Normal RR, HR, SBP in infants <1 year
RR - 30-40
HR - 110-160
SBP - 70-90
330
Normal RR, HR, SBP in children <5 years
RR - 25-35
HR - 95-140
SBP - 80-100
331
Normal RR, HR, SBP in children >12 years
RR - 20-25
HR - 80-120
SBP - 90-110
332
Normal RR, HR, SBP in children >12 years
RR - Adult
HR - Adult
SBP - 100-120
333
Paediatric A-E Assessment
- General condition
- Airway
- Breathing
- Circulation
- Disability
- Exposure/ENT
- Temperature
Need to assess systems for:
1. Effort
2. Efficacy
3. Effects on other systems
334
A-E Assessment - airway and breathing
1. Effort – RR, WOB, accessory muscles, recessions, added sounds, respiratory distress
2. Efficacy – talking, air entry, SaO2 (>92%)
3. Effects – skin colour, conscious level
Mx - Open and maintain airway, 5 initial rescue breaths, 100% high flow O2, Anaesthetist involvement for intubation
335
A-E Assessment - circulation
- Heart – rate, rhythm, pulse
- Blood pressure – hypotension (late sign)
- Capillary refill (<2 seconds)
- Peripheral temperature, colour
Mx - Chest compressions if needed, IV access, bloods,, catheterise, consider inotropes
336
A-E Assessment - disability
- Level of consciousness (AVPU / GCS)
- Pupils – size, reactivity
- Posture and tone
- Blood glucose!
337
A-E Assessment - exposure/ENT
- Rash, injuries, bruises
- Pain
- ENT exam
- Temperature
338
Causes of shock
Hypovolaemic
=> Bleeds, burns, fever, V&D, urinary losses
Distributive
=> Sepsis, intestinal obstruction
Obstructive
=> Cardiac tamponade, PE, tension PTX
Cardiogenic (rare)
=> Myocarditis, congenital heart disease)
339
Clinical signs of shock
```
Early:
• Tachypnoea, tachycardia
• Cap refill >2s
• Decreased skin turgor, sunken eyes/fontanelles
• Mottled, pale, cold skin
• Oliguria (<0.5 – 1 mL/kg/hour)
```
Late:
• Hypotension, bradycardia
• Metabolic acidosis
• Depressed cerebral state
340
Shock - management
1. FLUID RESUSCITATION = priority
- 0.9% saline bolus (20 mL/kg)
- 2nd bolus if necessary
2. If no improvement, then involve PICU
341
Septic Shock
Features of shock, plus:
- Fever, lethargy
- Poor feeding
- +/- purpuric rash (meningococcal sepsis)
342
Septic Shock - management
1. Fluid resuscitation
2. IV Abx ASAP (Ideally after cultures)
Keep reassessing, further support if needed
343
what is Anaphylaxis?
What are the causes?
= life-threatening hypersensitivity reaction (IgE) with rapid onset airway and circulatory problems.
Food allergy (85%), Insect stings, Drugs, Latex
344
Features of anaphylaxis
```
Difficulty breathing/swallowing
Stridor +/- wheeze
Swollen face/tongue
Urticaria
Pale, clammy
```
SHOCK may develop
345
What are some differentials of anaphylaxis?
DDx – asthma, panic attack, septic shock
346
Anaphylaxis - management
1. IM ADRENALINE 1:1000 – repeat in 5 mins if no improvement.
=> Adult and child >12 years = 0.5 mL
=> Child 6-12 years = 0.3 mL
=> Child <6 years = 0.15 mL
2. A-E assessment
3. Steroids and Antihistamines
=> Hydrocortisone and Chlorpheniramine
4. Monitor - pulse oximetry, ECG, BP
347
What is the dose of adrenaline used in anaphylaxis ?
IM ADRENALINE 1:1000
Adult and child >12 years = 0.5 mL
Child 6-12 years = 0.3 mL
Child <6 years = 0.15 mL
348
DKA - clinical features
- Polyuria & Polydipsia
- Weight loss
- Abdominal pain
- Vomiting
- Tachypnoea
- Confusion
349
DKA - management
1. A-E Assessment
=> Severity of dehydration
=> Evidence of acidosis (e.g. hyperventilation/Kussmaul breathing)
=> Assessment of conscious level
2. Initial investigations
=> Blood gas (pH), blood glucose, ketones, U&Es, ECG
3. IV fluid resuscitation and ongoing rehydration
=> Estimate dehydration and slowly rehydrate
=> 0.9% saline (10 mL/kg over 1 hour)
5. Insulin therapy and potassium replacement
=> 0.1 unit/kg/hour of insulin
350
Why is potassium replacement given alongside insulin therapy in DKA?
Insulin therapy causes a shift in potassium, which can cause hypokalaemia, so KCl given to replace potassium
351
What is the rate of insulin infusion for DKA?
0.1 unit/kg/hour of insulin
352
What is there a risk of when correcting fluids/sugar too rapidly?
How would you recognise this?
Risk of CEREBRAL OEDEMA
Sx:
headache, convulsions, abnormal posture, rising BP/falling pulse, poor respiratory effort, irritability/drowsiness, focal neurological signs, falling GCS, papilloedema, falling O2 sats.
353
How is cerebral oedema managed?
Inform senior immediately
Check blood glucose and r/o hypoglycaemia as a cause of neurological symptoms.
Mx: hypertonic saline/mannitol, reduce IV intake, transfer to PICU
354
Alcohol poisoning - effects
Hypoglycaemia
Coma
Respiratory Failure
355
Alcohol poisoning - management
Check blood alcohol levels
Monitor blood glucose
Ventilatory support
356
Acid/alkali poisoning - effects and management
Inflammation and ulceration of GI tract
Mx - Early endoscopy
357
Ethylene Glycol (anti-freeze) poisoning - effects
Tachycardia
Metabolic acidosis
Renal failure
358
Ethylene Glycol (anti-freeze) poisoning - management
Antidote – Fomepizole
| Haemodialysis
359
Paracetamol poisoning - effects
24-48 hours: abdo pain, vomiting
| 3-5 hours: liver failure
360
Paracetamol poisoning - management
Measure plasma paracetamol conc.
IV N-acetylcysteine
361
NSAID poisoning - effects
N&V, drowsiness, blurred vision, tinnitus
Hyperventilation
Acute renal failure
362
NSAID poisoning - management
Measure plasma conc.
Rapid BM, blood gas, creatinine, FBC, ECG
Supportive Tx (fluids, dialysis, etc.)
There is no antidote!
363
Iron poisoning - effects
Initial: V&D, haematemesis, melaena, gastric ulcers
Latent period
6 hours later: drowsy, coma, shock, convulsions, liver failure
364
Iron poisoning - management
Measure serum iron levels
IV Deferoxamine (acts by chelating the iron)
365
Methadone poisoning - management
Activated charcoal within 1 hour
IV Naloxone
366
Methadone poisoning - effects
Drowsiness, mitosis, vomiting
Tachypnoea/apnoea leading to respiratory acidosis
367
TCA poisoning - effects
Tachycardia, arrythmias
Dry mouth, blurred vision
Agitation, confusion, convulsions
Respiratory Depression
368
TCA poisoning - management
Ventilatory support
IV Sodium bicarbonate (if arrythmia / severe metabolic acidosis)
Diazepam (if convulsions)
369
General management of overdose/poisoning
1. Identify agent and amount taken
2. Can activated charcoal be used to decrease absorption?
=> Only if <1 hour
=> Ineffective for iron and pesticides
3. Investigations:
=> FBC, renal and liver function, ECG, ABG
4. Administer antidote:
=> If available/toxicity high enough
5. Supportive Tx:
=> Ventilatory support, IV fluids, etc.
370
What is Status epilepticus?
Seizure >30 minutes
OR
Successive seizures over 30 minutes with no recovery in between
371
Status epilepticus - management
1. A-E Assessment
- Open and maintain airway
- High flow oxygen
- Glucose to r/o hypoglycaemia
- Confirm it is an epileptic seizure (Hx, features, etc.)
2. Manage Convulsions
- IV lorazepam (again 5 mins later if no improvement)
- IV phenytoin
- Anaesthetist - intubation and PICU
372
what is an Apparent Life-Threatening Event?
= a frightening combination of symptoms (most common in <10 weeks).
- Apnoea
- Colour change
- Altered muscle tone
- Choking/gagging
373
Causes of Apparent Life-Threatening Event
* No cause identified (50%)
* Upper airway obstruction
* Infections (URTI)
* Seizures
* GORD
* Cardiac arrythmia
374
Apparent Life-Threatening Event - management
1. Detailed Hx and examination
2. Admission to hospital (basic investigations and overnight monitoring)
3. Discharge if normal and no high risk features.
375
What is sudden infant death syndrome?
What are the risk factors?
= sudden, unexplained death of an infant with no identifiable cause.
RFs:
Infant – age 1-6 months, preterm/LBW, Male
Parents – low income, poor/overcrowded housing, Parental smoking, unsupported single mum, maternal age <20 years.
Environment – co-sleeping, overheated baby, baby sleeping on tummy.
376
what is the most common cause of death/serious injury in childhood?
road traffic accidents
377
Complications of head injury
- Hypoxia – airway obstruction/decreased ventilation
- Hypo/hyperglycaemia
- Decreased cerebral perfusion – raised ICP/ decreased BP from bleed
- Haematoma
- Infection – open wound, CSF leak
378
Head injury - red flags
- LOC >5 mins
- Amnesia >5 mins
- Seizure
- ≥3x vomiting
- GCS <15
- Signs of fracture
379
How to assess surface area of burn?
Patient’s palm = 1% of body SA
Add 0.5% to each leg with every year >1 year old
Subtract 2% from head with every year >1 year old
380
Burns/scald - management
1. Burns first aid – cold water for 20 minutes and clingfilm
2. Analgesia – e.g. IV morphine
3. Fluid resuscitation – if shock/>10% SA
4. Wound care
381
Near drowning - management
Mouth-to-mouth resuscitation and CPR
Cover and keep warm
Hospital admission – monitor for resp. distress, pulmonary oedema, development of pneumonia
382
Choking/Aspiration - management
Unconscious – paediatric BLS
Conscious and ineffective cough – 5 back blows, 5 abdo thrusts (chest thrusts if <1 year)
383
what is the most common cause of death/serious injury in childhood?
road traffic accidents
384
Risk factors for infection
- Illness of family members
- Unimmunised/immunodeficient
- Recent travel abroad/contact with animals
385
Red flags in febrile child
- Fever >38oC and <3 months
- Fever >39oC and 3-6 months
- Pale/mottled/blue
- Cap refill >3 seconds
- Altered consciousness, seizures
- Stiff neck, bulging fontanelle
- Significant respiratory distress
- Bilious vomiting
386
Management of Febrile Child
- Not seriously ill – at home, clear instructions/safety netting, (+/- PO Abx)
- Significantly ill – further Ix (septic screen) and monitoring/treatment in hospital
- Seriously ill – immediate IV Abx (cefotaxime/ceftriaxone)
387
Safety netting for febrile child
```
Come back if:
• Poor oral intake
• Signs of dehydration
• Abnormal movement
• Breathing difficulties
• Rash
• Rigors/seizures.
```
388
Septic Screen
Blood cultures
FBC, CRP, VBG
Urine MC&S
Also consider - CXR, LP, antigen screen, U&E, LFT, PCR
389
Contraindications for Lumbar Puncture
CV shock
Focal neurological signs or seizures
Signs of raised ICP
Thrombocytopaenia
390
Indications for Lumbar Puncture in febrile child
Febrile and <1 year
| Suspected meningitis
391
Where is a LP performed in children?
Performed at or below the L4 level
392
Sudden onset purpura in a febrile child
Should be assumed to be meningococcal sepsis
(however, any severe sepsis can cause a non-blanching rash due to DIC).
393
Causes of meningitis in children
1. Viral Meningitis
- 2/3 of cases
- Less severe than bacterial
- Enterovirus, EBV, adenovirus, mumps
2. Bacterial Meningitis
- 10% mortality, 10% morbidity
- <3 months – GBS, E. coli, Listeria
- 1 month to 6 years – N. meningitidis, S. pneumoniae, H. influenzae
- >6 years – N. meningitidis, S. pneumoniae
3. TB Meningitis
- Rare in countries with low TB prevalence.
394
Symptoms of meningitis
```
FEVER
Non-blanching purpura (meningococcal)
Neck stiffness, bulging fontanelle
Arched back
Focal neuro signs, seizures
Altered consciousness
Signs of shock
+ve Brudzinski sign
+ve Kernig Sign
```
395
Positive Brudzinski sign
Flexion of neck causes hip and knee flexion
396
Positive Kernig Sign
With hips and knees flexed, there is pain extending the knees.
397
Meningitis presentation in infants
symptoms are often non-specific – e.g. poor feeding, vomiting, irritable, crying.
398
Complications of meningitis
- Sepsis
- Hearing impairment
- Cranial nerve palsies
- Seizures/epilepsy
- Hydrocephalus
- Cerebral abscess
399
Investigations in ?meningitis
- Lumbar puncture
- FBC, CRP, U&Es, LFTs, clotting
- Blood glucoses and gases (for pH and lactate)
- Blood, urine, stool, throat swab culture
- Rapid antigen test (on CSF or blood)
- PCR (of blood or CSF)
- If TB suspected – Mantoux, sputum, urine
400
What is the purpose of a LP in ?meningitis
To confirm Dx, also identify organism and sensitivity.
401
Normal CSF values
Clear Appearance
WCC <5/mm3
Protein - 0.15-0.4 g/L
Glucose - >50% that of blood level
402
What is Kawasaki Disease?
A rare disorder involving systemic small-to-medium vessel vasculitis (due to immune hyperactivity).
403
What should be suspected in children with prolonged fever?
Kawasaki Disease
404
Symptoms of Kawasaki Disease
```
Fever ≥38oC for >5 days PLUS 4 of:
• Non-purulent conjunctivitis
• Red mucous membranes
• Red/swollen/peeling skin (cracked lips, strawberry tongue)
• Rash
• Cervical lymphadenopathy
```
Infants may not present with all symptoms so must maintain high suspicion if prolonged fever.
405
Where is the rash normally located in Kawasaki Disease?
usually genital area, lips, palms, or soles of the feet,
406
Kawasaki Disease - Risk factors
- 6 months – 4 years old
- Asian or Afro-caribbean
- Covid-19
407
Kawasaki Disease - Investigations
History and Examination
Bloods – raised CRP, ESR, WCC and platelets
Echo – shows coronary aneurysms
408
What is the most important thing to rule out/identify in Kawasaki Disease?
Coronary Aneurysms
409
Kawasaki Disease - Initial Management
IV Immunoglobulin within 10 days – reduce risk of aneurysms
Aspirin
=> High dose until fever subsides, then low dose for 6 weeks.
=> Decrease risk of thrombosis
410
Kawasaki Disease - Management of long-term aneurysms
Long-term warfarin
411
Kawasaki Disease - Management if recurrence
Monoclonal antibodies/steroids/ciclosporin
412
What is infectious mononucleosis?
= a syndrome of symptoms maily caused by the host response to EBV.
413
Symptoms of infectious mononucleosis
• Fever, malaise
• Tonsillopharyngitis
=> Cannot eat/drink
=> Breathing may be compromised.
* Cervical lymphadenopathy
* Hepatosplenomegaly/jaundice
* Petechiae on soft palate
* Maculopapular rash (if <4 years).
414
infectious mononucleosis - investigations
Blood film – atypical lymphocytes
Monospot test – heterophile antibodies (may be negative in young children)
EBV antibodies – IgM and IgG
415
infectious mononucleosis - management
= self-resolving in 1-3 months
1. Symptomatic relief
2. Corticosteroids – if airway compromise
3. Abx ONLY if confirmed tonsilitis (but NOT AMOXICILLIN)
416
What treatment should be avoided in infectious mononucleosis?
AMOXICILLIN - causes rash
417
How quickly does infectious mononucleosis resolve?
self-resolving in 1-3 months
418
HIV in children - cause, presentation
Affects ~2 million children (mainly in sub-saharan Africa).
Majority occurs by transmission from mother (transplacental, at birth, breast-feeding).
Presents as opportunistic infections
=> Bacterial, candida, diarrhoea, PCP
419
Long-term problems of HIV infection in children
Failure to thrive, encephalopathy, malignancy
420
HIV in children - management
ART – based on clinical status, viral load, CD4 count
=> Infants start ART ASAP
Infection prophylaxis
=> co-trimoxazole for PCP
=> Vaccinations – flu, hep A/B, VZV
Regular follow-up – weight, neurodevelopment, signs of infection, adherence to Tx.
421
Cause of malaria infection
Caused by plasmodium falciparum parasite, which is transmitted by the female Anopheles mosquito.
422
Malaria - symptoms
occur ~7-10 days post-infection
* Fever, malaise, myalgia
* V&D, abdo pain
* Jaundice
* Anaemia
* Thrombocytopaenia
423
What is a very common feature of malaria in children?
Anaemia
424
Malaria - diagnosis
thick blood film
425
Malaria - management
Quinine
426
Malaria - prevention
Insect nets
Insect repellent
Abx prophylaxis
427
Cause of typhoid infection
Caused by salmonella typhi or paratyphi parasite
428
Typhoid - symptoms
occur ~7-10 days post-infection
* Worsening fever
* Headaches, malaise, myalgia
* Cough
* Abdo pain
* Anorexia, diarrhoea, constipation
429
Typhoid - signs
* Rose-coloured spots on trunk
* Splenomegaly
* Bradycardia
430
Typhoid - complications
* GI perforation
* Myocarditis
* Hepatitis, nephritis
431
Typhoid - management
Azithromycin or Ceftriaxone
432
What nutrients are required in children and why?
Protein - growth, repair, enzymes, antibodies
Energy - growth, moving, developing
=> Carbohydrate
=> Fat
Vitamins - metabolism, cell structure, defence
=> Water soluble, needed daily
=> Fat soluble, can be stored
Minerals - blood, bones, enzymes, compete for absorption
Water - metabolism, homeostasis, fluid balance
433
How much breast milk is needed to meed nutritional requirements of a child?
need 300mL/kg/day of Mature Breast milk
434
Advantages of breast milk
```
Ideal nutritional composition
Greater bioavailability of nutrients
Antibodies
Promote gut health
Lower rates of NEC in pre-term infants
```
435
How is faltering growth defined?
1. Weight deviating from usual centile/ falling through 2 centiles
2. Weight persistently more than 2 Centiles below height?
436
Which children are more at risk of faltering growth?
• Premature Infants
* Chronic Lung disease , cardiac problems
* Cleft Palate
* Cystic fibrosis
* Developmental delay
* ADHD
* Cerebral Palsy
* Coeliac Disease
* Cow’s milk protein Allergy
437
Faltering Growth - assessment
Nutrient intake compared to estimated requirements.
Feeding behaviour and feeding patterns.
Activity.
438
Faltering Growth - treatment
Dietary Manipulation
- Food fortification (FOOD FIRST)
- Milky drinks
- 2 course meals
Dietary Supplements
Tube Feeding
- NG
- Gastrostomy, PEG, Button, Jejenostomy
439
When is a jejunostomy used?
Only if need post-pyloric feeding.
440
what is cow’s milk protein allergy ?
= an immune reaction to the protein in milk
441
Cow’s milk protein allergy vs lactose intolerance
A food allergy, such as cow's milk allergy, is an immune reaction to the protein in milk.
A lactose intolerance is caused by the inability to break down lactose (the sugar in milk).
Lactose intolerance tends to be just uncomfortable, while cow’s milk protein allergy can have dangerous symptoms.
442
Symptoms of cow’s milk protein allergy
```
Failure to thrive
Frequent regurgitation
Vomiting, Diarrhoea
Refusal to feed
Blood in stools
```
Rash
Respiratory Sx
443
Symptoms of lactose intolerance
Diarrhoea
Abdominal pain
Reducing substances in stools
Small amounts usually tolerated.
444
What is coeliac disease?
When gluten is provoking a damaging immunological response in the proximal small intestine mucosa, causing loss of villi.
445
Coeliac disease - Sx
occur after introduction of wheat to diet
* FTT +/- weight loss/wasted buttocks
* Diarrhoea
* Abdo pain/distension
* Irritability/fatigue
* Anaemia
446
Coeliac disease - investigations
* Serological testing – IgA transglutaminase, anti-endomysial
* Small intestinal biopsy = diagnostic
447
Coeliac disease - management
* Gluten free diet for life.
| * If diagnosed <2 years, need gluten challenge later on to confirm Dx.
448
Severe combined immunodeficiency
= A group of rare disorders caused by mutations in different genes involved in the development and function of immune cells.
Infants with SCID appear healthy at birth but are highly susceptible to severe infections.
449
Primary immunodeficiency
= an intrinsic defect of the immune system.
Often X-linked or autosomal recessive.
Should be suspected in children with Severe, Prolonged, Unusual or Recurrent infections (SPUR)
450
Immunodeficiency - investigations
- FBC (including WCC breakdown)
- Immunoglobulins
- Complement proteins
- Specific genetic/molecular tests
451
Secondary immunodeficiency
= due to another disease or treatment.
```
E.g.:
• Concurrent bacterial/viral illness
• HIV
• Malignancy
• Malnutrition
• Immunosuppressive therapy
• Splenectomy
• Nephrotic Syndrome
```
More common than primary immunodeficiency.
452
Immunodeficiency - management
- Antimicrobial prophylaxis
- Prompt treatment of infection (longer Abx course)
- Ig-replacement therapy
- Bone marrow transplant
- Gene therapy (for SCID)
453
What are the ages of the Primary Series of vaccinations in the UK?
8 weeks old
12 weeks old
16 weeks old
454
What immunisations are given at 8 weeks?
= 1st set of vaccinations
6 in 1 - 1st dose
MenB - 1st dose
Rotavirus - 1st dose
455
What immunisations are given at 12 weeks?
* 6-in-1 2nd dose
* Pneumococcal (13 serotypes)
* Rotavirus 2nd dose
456
What immunisations are given at 16 weeks?
* 6-in-1 3rd dose
| * MenB 2nd dose
457
What immunisations are given at 1 year old?
* Hib and Men C
* Pneumococcal 2nd dose
* MMR 1st dose
* MenB 3rd dose
458
What immunisations are given yearly from 2 - 10 years old?
Influenza (every Winter)
459
What immunisations are given at 3 years 4 months old (or soon after)?
* Diphtheria, tetanus, pertussis, polio booster
| * MMR 2nd dose
460
What immunisations are given in secondary school age children?
12-13 years old
• HPV – two doses 6-24 months apart
14 years old (school year 9)
• Tetanus, diphtheria, polio booster
• Men ACWY
461
What vaccines are offered to pregnant women and when?
* Influenza (during flu season, at any stage of pregnancy)
| * Pertussis (from 16 weeks gestation)
462
Vaccines for Babies born to HepB +ve mother
HepB vaccine at birth, four weeks, 12 weeks
463
Which children are offered vaccines for TB?
Infants born in/with:
- areas of high TB incidence
- parent/grandparent born in area of high TB incidence
Receive BCG vaccine at birth.
464
Contraindications to Vaccines
Anaphylaxis
- To previous dose
- To a component of vaccine (neomycin, polymyxin B)
- Latex allergy
No live vaccines in certain groups
465
What groups are considered unable to receive live vaccines?
- Pregnancy
- Primary immunodeficiency
- Malignancy and solid tumour treatment
- Bone marrow transplant
- High-dose steroids / immunosuppressant drugs
- HIV
466
Egg Allergy and immunisation
Yellow fever vaccine is contraindicated in confirmed anaphylaxis to eggs.
Egg-free or low albumin Inactivated influenza vaccines are available – safe to give
MMR can safely be given to most children with egg allergy.
467
Vaccine hesitancy
= a delay in acceptance OR refusal of vaccines despite availability of vaccination services.
468
What is an allergy?
= an inappropriate, potentially harmful reaction by the immune system to a harmless stimulus
469
What is atopy?
personal or familial tendency to become sensitised and produce IgE antibodies in response to ordinary exposure to allergens.
470
What is an allergen?
= an antigen responsible for producing allergic reactions by inducing IgE antibody formation.
471
What is urticaria?
= an unexplained raised, itchy rash that appears spontaneously and responds to antihistamines.
Often no obvious cause can be found.
472
What is the prevalence of allergy in the UK?
~40% of all UK children have an allergic diagnosis
473
Pathogenesis of allergy
Mostly IgE-mediated
1. Allergen sensitisation - B-cells produce IgE specific to the allergen.
2. Upon re-exposure of allergen, stimulation of plasma cells causes release of the allergen specific IgE.
=> IgE stimulates mast cells, which degranulate and release histamine
474
What does a skin-prick test show?
shows the tissue release of histamine in response to allergens
475
Risk factors for allergy
- Hygiene Hypothesis
- FHx
- ?Caesarean birth/smoking in pregnancy/reduction in breast feeding
- ?In-utero exposure to maternal diet/late weaning to solids
- Childhood obesity
- Air pollution
476
Hygiene hypothesis
Suggests that the post-natal period of immune response is derailed by extremely clean household environments often found in the developed world.
This means failure to provide the necessary exposure to germs required to “educate” the immune system so it can create a response against infectious organisms.
477
How do Th1 and Th2 cells work in relation to infection and allergy in developed vs. developing countries?
Th1 cells help to fight infection – they are stimulated by viral/bacterial infection
=> in a developing country, children will have high Th1 cells, which suppresses Th2 cells.
Immunisation and clean environment mean that Th1 cells do not increase, so Th2 cells are not suppressed
=> in developed countries, Th2 cells are higher and this causes more allergies
478
Airborne allergens
Dust mites
Grass/tree/weed pollen
Pet dander/hair
Mould
479
Dust mite allergy
Causes perennial allergic rhino-conjunctivitis (present throughout the year).
Management:
=> antihistamines
=> regular hoovering/prevention of dust mites.
480
Grass/ tree/ weed pollen allergy
Causes seasonal allergic rhino-conjunctivitis (= hay fever)
Management – antihistamines, LTRAs, sometimes immunotherapy (Grasax).
481
Pet allergy
Dander, hair and saliva may induce an allergic reaction.
Tend to not be life-threatening reactions, and over prolonged exposure can develop tolerance.
In some children, it can trigger asthma
482
Common food allergens
Infants – cow's milk, egg, peanut
Older children – peanut, tree nut, fish, shellfish
483
Non-allergic food hypersensitivity
Experience discomfort after certain food.
=> D&V, abdo pain, failure to thrive
e.g. lactose intolerance
484
Food allergy
The immune system is specifically involved, and the immune responses can be measured.
Immediate onset of symptoms (10-15 mins after ingestion)
=> Rash, itching, swelling, etc.
=> Severe – wheeze, stridor, abdo pain, shock, collapse.
Responds to antihistamines.
485
Food allergy - Investigations
- History and examination
- Total and specific IgE antibodies
- Skin-prick test
- Exclusion of food (and careful reintroduction – “food challenge”)
486
Food allergy - Management
1. Avoid food triggers
2. Mild reaction = antihistamines
3. Severe reaction = IM adrenaline
487
Food allergy - when should a GP refer to allergy specialist?
- FTT/GI symptoms of food allergy
- No response to single elimination diet
- >1 systemic reaction
- >1 severe delayed reaction
- IgE associated food allergy & asthma
- Significant eczema – multiple food allergies suspected
488
Multi-system nature of allergy
- Respiratory symptoms – asthma, hoarseness
- Skin/mucous membrane symptoms – urticaria, angio-oedema, rhinitis, conjunctivitis
- CV symptoms – anaphylactic shock
- GI symptoms – abdominal pain, V&D
489
Acute Urticaria/angioedema
Acute (<6 weeks)
usually caused by food allergy, drug reactions (especially Abx), infection
490
Chronic Urticaria/angioedema
lasts >6 weeks
usually non-allergic (cold, heat, water, sunlight, sweating).
491
what is Tarsal Coalition ?
How can it present?
= lack of segmentation of small bones of feet => rigid and limited motion
presents as recurrent strains in adolescence.
492
"Normal" flat feet
<4 years old = normal
=> Due to flat medial longitudinal arch
=> Standing on tip-toe/extension of big toe demonstrates arch
493
"Abnormal" flat feet - presentation, Ix and Mx
Often painful, stiff and no arch on tip-toeing
Ix = X-ray, MRI/CT
Mx = specialised footwear/arch support, surgery for tarsal coalition.
494
DDx for flat feet
Achilles contracture,
tarsal coalition,
idiopathic juvenile arthritis,
infection.
495
Forefoot adduction
= “in-toeing”
Usually resolves by age 5.
Causes:
1. Metatarsus varus = highly mobile foot causes adduction deformity.
2. Medial tibial torsion = lack of lateral rotation of tibia.
3. Anteverted femoral neck = at hip => twisted forward more than normal.
496
What is Developmental Dysplasia of the Hip ?
| Why does it occur?
= a spectrum from dysplasia of the joint, to subluxation (partial dislocation) to frank dislocation.
Occurs due to shallow acetabulum.
497
What conditions are often associated with DDH?
Talipes equinovarus
| Torticollis
498
Risk factors for DDH
- Female
- Breech
- FHx
- 1st born
- Oligohydramnios
499
When is DDH most often identified?
At the Newborn examination / 6-8 week post-natal check
500
DDH - Diagnosis
1. Newborn examination / 6-8 week post-natal check - identifies most cases
=> Barlow and Ortolani test
=> If suspected or high-risk, refer to orthopaedics for USS of hip.
2. Later presentation with abnormal limp/gait
=> +/- shortened leg (Galeazzi’s sign) or limited abduction
=> +/- asymmetrical skin folds
=> needs X-ray to confirm diagnosis
501
Barlow and Ortolani tests
```
Barlow = posterior dislocation out of acetabulum
Ortolani = abductive relocation into acetabulum
```
After 2-3 months of age, the Ortolani test and Barlow manoeuvres are less sensitive
502
How can and degree of hip instability be described?
1. Dislocated and reducible (+ve Ortolani)
2. Dislocated and irreducible (-ve Ortolani)
3. Dislocatable (+ve Barlow)
4. Subluxed (a hip with mild instability or laxity with a -ve Barlow manoeuvre)
503
DDH - management
<6 months – Pavlik Harness
=> Maintains hip flexion and abduction until 12-18 months old
=> Monitor progress with USS/X-ray
>6 months or Tx failure – surgery (open reduction and 3 months in a cast).
504
What is Structural Scoliosis?
| What are the causes?
= lateral curvature of the spine.
1. Idiopathic (85%) – most often starts in girls at pubertal growth spurt.
2. Congenital – spinal defect (e.g. VACTERL, spina bifida)
3. Secondary – to cerebral palsy, muscular dystrophy, neurofibromatosis, Marfan’s, IJA
505
What is the most common cause of scoliosis?
idiopathic
506
Scoliosis - investigations
1. Examination of back
=> Irregular skin creases, different shoulder heights
=> If scoliosis disappears on leaning forwards = postural scoliosis
2. X-ray if more severe
=> To assess and monitor progression.
507
Scoliosis - management
- Mild – asymptomatic, resolves spontaneously.
- Severe – bracing
- Very severe or associated complication (e.g. chest deformity causing cardiorespiratory failure) – surgery.
508
what is Torticollis?
"wry neck"
= tilted head/neck position towards the affected muscle.
509
Causes of torticollis?
ACUTE
Sleep in unusual position
Neck sprain
```
CHRONIC
SCM tumour (most common cause in infants)
Muscle spasm
ENT infection
Spinal tumour
Cervical spine arthritis
```
510
SCM tumour
benign, mobile, non-tender nodule
resolves in 2-6 months.
511
Management of torticollis
= treat underlying cause!
```
Analgesia, heat packs, passive stretching.
Muscle relaxants (e.g. diazepam)
```
512
What is a skeletal dysplasia?
= genetic mutation (inherited or de novo) causing generalised developmental disorder of bone.
513
Types of skeletal dysplasia
1. Achondroplasia (Dwarfism)
2. Thanatophoric Dysplasia
=> Causes stillbirth of infant with large head, short limbs, small chest.
3. Cleidocranial Dysostosis:
=> Absence of part/all clavicles
=> Short stature
4. Osteogenesis Imperfecta (brittle bone disease)
514
What causes Osteogenesis Imperfecta ?
= disorders of collagen metabolism, causing bone fragility
515
Osteogenesis Imperfecta - symptoms
bowing, frequent fractures
516
Osteogenesis Imperfecta - management
bisphosphonates,
| splinting of fractures
517
Type 1 vs Type 2 Osteogenesis Imperfecta
Type 1 = most common
=> blue sclera, hearing loss
Type 2
Multiple fractures before birth => often stillborn
518
Causes of bone/joint infection
haematogenous spread,
spread from adjacent soft-tissue infection,
penetrating wound
519
bone/joint infection - risk factors
```
immunosuppressed,
DM,
extremes of age,
recent operation/injection,
wounds
```
520
bone/joint infection - complications
bone/cartilage necrosis,
chronic infection,
limb deformity,
amyloidosis
521
What is osteomyelitis?
= infection of the long bones.
522
Osteomyelitis - Sx
```
PAIN
Immobile limb
Fever
Swelling, tender, warmth
Erythema over bone
```
15% have co-existing septic arthritis
523
Osteomyelitis - Ix
Bloods – increased WCC, CRP, ESR
Blood cultures
X-ray – radiolucent area with hypodense border (due to new bone formation)
USS – ?co-existent joint effusion
MRI – subperiosteal pus
Bone scintigraphy – increased radionucleotide uptake
524
Osteomyelitis - Mx
1. IMMEDIATE IV ABX
=> Minimum 4 weeks.
=> IV initially then switch to PO
2. Supportive
=> Analgesia, bedrest, immobilise/splint then physio
3. Surgical
=> Decompression/aspiration or subperiosteal space if abscess or Tx failure.
525
What is septic arthritis?
= infection of the joint space
526
Septic arthritis - Sx
```
PAIN (on passive movement)
Immobile limb
Fever
Swelling, tender, warmth
Erythema over joint
+/- joint effusion
+/- limp
```
527
Septic arthritis - Ix
Bloods – increased WCC, CRP, ESR
Blood cultures
JOINT ASPIRATION & CULUTRE = DIAGNOSITIC
X-ray – normal
USS – shows joint effusion
MRI/Bone scintigraphy – show any co-existent osteomyelitis
528
Septic arthritis - Mx
1. IMMEDIATE ABX
=> Minimum 2 weeks.
=> IV at first then switch to PO
2. Supportive
=> Analgesia, bedrest, immobilise/splint then physio
3. Joint Drainage and Washout (lavage)
=> If deep-seated or Tx failure.
529
Limping Child - acute causes
```
Infection (OM/SA)
Transient synovitis (irritable hip)
Trauma/overuse injury
Malignancy
Slipped capital femoral epiphysis
Reactive arthritis/JIA
```
530
Limping Child - chronic causes
```
Congenital problem (DDH, talipes)
Tarsal coalition
Neuromuscular
JIA
Perthes Disease
Slipped capital femoral epiphysis
```
531
Likely Dx of limping child aged 0-2?
DDH
532
Likely Dx of limping child aged 2-4?
Transient synovitis (irritable hip)
533
Likely Dx of limping child aged 5-10?
Perthes Disease
534
Likely Dx of limping child aged 10-15?
Slipped capital femoral epiphysis
535
What is the #1 cause of hip pain in children?
Transient Synovitis (Irritable Hip)
536
Transient Synovitis - presentation
Follows/accompanies viral infection.
Symptoms:
- Pain on movement (not at rest)
- Decreased ROM (especially internal rotation)
- Afebrile/not ill
537
Transient Synovitis - investigations
Important to differentiate from septic arthritis => need bloods, blood cultures, normal X-ray
538
Transient Synovitis - management
Improves in 1 week => Bed rest and analgesia
Safety net – fever, unwell, non-weight bearing.
(3% develop Perthes' Disease)
539
What is Perthes Disease?
Idiopathic interruption of the blood supply to the femoral head,
=> causes avascular necrosis of capital femoral epiphysis, then revascularisation and re-ossification.
540
Perthes Disease - Sx
- Insidious onset (days)
| - Limp or hip/knee pain
541
Perthes Disease - Ix
- X-ray (AP and frog lateral) – femoral epiphyseal head flattened and fragmented, increased density.
- Bone scan
- MRI
542
Perthes Disease - Mx
Early/mild – AVOID INTENSIVE EXERCISE, bed rest, traction, analgesia.
Late/severe – plaster/calipers to maintain hip in abduction, femoral/pelvic osteotomy.
543
Perthes Disease - Prognosis
Poor prognostic factors - >5 years old, >50% epiphysis involved, deformed femoral head
=> Can cause later complication of osteoarthritis
544
what is Slipped Capital Femoral Epiphysis ?
Postero-inferior displacement of epiphysis
545
Slipped Capital Femoral Epiphysis - Risk factors
Adolescent males (growth spurt)
Obesity
Hypothyroidism
Hypogonadism
546
Slipped Capital Femoral Epiphysis - Sx
- Acute (post-trauma) or insidious
- Limp or hip/knee pain – often bilateral
- Restricted abduction and internal rotation of hip
547
Slipped Capital Femoral Epiphysis - Ix
X-ray (AP and frog-lateral) => lost Klein’s line, widened growth plate.
548
Klein's Line
= an arbitrary line drawn along the superior edge of the femoral neck
should normally intersect the lateral aspect of the superior femoral epiphysis
549
Trethowan sign
when the line of Klein passes above the femoral head.
indicates slipped capital femoral epiphysis
550
Slipped Capital Femoral Epiphysis - Mx
needs to be treated ASAP to prevent avascular necrosis
Conservative – analgesia, crutches
Surgical – pin fixation in situ.
551
Common pathogens in bone/joint infections
Staph. Aureus,
streptococcus,
H. influenzae,
TB (rare)
552
Incomplete Fractures
Common in children due to softer/ more flexible bones – the bones compress/bend rather than break.
Include:
1. Greenstick Fractures
2. Buckle/Torus Fractures
3. Hairline (stress) Fractures
553
Greenstick Fractures
= partial break in one side causes other side to bend.
Common in mid-shaft forearm and lower leg.
554
Buckle/Torus Fractures
= compression on one side of the bone causing bulging cortex.
Commonly distal metaphysis of radius
Following FOOSH
Appears as “base of pillar” and often no fracture line seen
555
Hairline (stress) Fractures
= small “cracks” that do not traverse entire bone.
Usually from overuse/repetitive stress-bearing motions
=>e.g. track runners, gymnasts, dancers.
556
Complete Fractures
1. Communicated Fracture = bone broken into >2 pieces/crushed into fragments.
2. Bucket-handle Fracture = fragmentation of corner of metaphysis.
=> Indicates non-accidental injury
557
Which type of fracture indicates non-accidental injury?
Bucket-handle Fracture
558
Growth Plate Fractures
Unique to children (common in growth spurt when physes are the weakest).
Typically caused by great force during sports or playground accidents.
Classified by Salter-Harries System
559
Salter Harris Fracture Type I
Straight across the growth plate.
Tx - Splinting or Casting
560
Salter Harris Fracture Type II
Into and above growth plate.
Tx - Splinting or Casting
561
Salter Harris Fracture Type III
Into and lower than growth plate
Tx - Open reduction and Internal Fixation
562
Salter Harris Fracture Type IV
Through growth plate
Tx - Open reduction and Internal Fixation
563
Salter Harris Fracture Type V
Erasure of growth plate / Crush
Tx - Surgery
564
Which Salter Harris type is the most common?
Type II
565
Which Salter Harris type is the most likely to affect bone growth?
Type V
566
What is the definition of Juvenile Idiopathic Arthritis?
= persistent joint swelling (>6 weeks) presenting before 16 years old in the absence of infection or other defined cause
567
How common is Juvenile Idiopathic Arthritis?
affects 1 in 1000
568
What is the most common subtype of Juvenile Idiopathic Arthritis?
Persistent Oligoarthritis (~50%)
569
What is Chronic anterior uveitis?
= inflammation of the uvea (middle layer of the eye).
It can cause eye pain and changes to vision.
570
Monoarthritis
Affects 1 joint
| e.g. septic arthritis, gout
571
Oligarthritis
Affects 4 joints or less
| e.g. reactive, psoriatic arthritis
572
Polyarthritis
Affects more than 4 joints
| e.g. rheumatoid, SLE
573
Symptoms of Juvenile Idiopathic Arthritis
Poor mood/decreased activity are indicators in infants
- Joint gelling – stiffness after rest
- Joint stiffness/pain – in mornings
- Joint swelling – may appear later.
574
Complications of Juvenile Idiopathic Arthritis
1/3 have active disease into adulthood (may need joint replacements)
1. Bone/joint deformities
2. Chronic anterior uveitis
3. Flexion contractures
4. Growth failure
5. Osteoporosis
6. Amyloidosis – proteinuria and renal failure (rare)
7. Side effects of Tx – growth suppression, immunosuppression
575
What are the subtypes of Juvenile Idiopathic Arthritis?
```
Persistent Oligoarthritis
Extended Oligoarthritis
RF -ve Polyarthritis
RF +ve polyarthritis
Systemic Arthritis
Psoriatic Arthritis
Enthesitis-related Arthritis
Undifferentiated Arthritis
```
576
JIA - Persistent Oligoarthritis
Affects max. 4 joints
Can have:
- Chronic anterior uveitis
- Leg length discrepancy
May have Antinuclear Antibody on tests
577
JIA - Extended Oligoarthritis
>4 joints
ASSYMETRICAL (large and small joints)
Can have:
- Chronic anterior uveitis
- Asymmetric growth
May have Antinuclear Antibody on tests
578
JIA - RF -ve or +ve Polyarthritis
SYMMETRICAL
Large and small joints
Marked finger involvement
+/- TMJ or cervical spine
If RF +ve then rheumatoid factor will be present on tests and patient may have rheumatoid nodules
If -ve:
Low grade fever
Chronic anterior uveitis
Late reduced growth
579
What age group is RF +ve polyarthritis likely to affect?
10-16 years
580
JIA - Systemic Arthritis
Symptoms and Blood results
Oligo/polyarthritis
Arthralgia/myalgia
Can have:
- Malaise, daily fevers
- Salmon-pink macular rash
- Lymphadenopathy
Bloods:
- Anaemia
- Raised CRP/ESR and platelets
581
JIA - Psoriatic Arthritis
ASSYMETRICAL
Large and small joints
Dactylitis
Can have:
- Psoriasis
- Chronic anterior uveitis
582
JIA - Enthesitis-related Arthritis
Affects large joints, mainly
There will be enthesitis (inflammation at tendon/ligament insertion)
583
JIA - Undifferentiated Arthritis
Overlapping patterns of subtypes
584
JIA - Management
MDT with rheumatology specialist.
Prevention and monitoring of complications
1. NSAIDs – relieve symptoms during flares.
2. Steroid injections
- 1st line for oligoarthritis, bridging Tx for polyarthritis
3. Methotrexate (tablet, liquid, injection weekly)
- 1st line for polyarthritis
4. Systemic corticosteroids
- Life-saving if severe systemic JIA
- Avoid if can as growth suppression/osteoporosis
5. Immunotherapy (e.g. anti-TNF)
- If methotrexate resistant
585
What is 1st line treatment for oligoarthritis?
Steroid injections
586
What is 1st line treatment for polyarthritis?
Methotrexate
587
What are side effects of methotrexate?
nausea, liver damage, bone-marrow suppression
588
What is 1st line treatment for severe systemic JIA?
systemic corticosteroids
589
What is the definition of reactive arthritis?
= transient (<6 weeks) joint swelling following extra-articular infection.
590
What infections can precede reactive arthritis?
Dysentery
STI - chlamydia, gonococcus
Viral illness
Other - mycoplasma, Lyme disease, Rheumatic fever
591
Symptoms of Reactive Arthritis
Reiter’s Syndrome
Dactylitis – swollen “sausage” fingers/toes
+/- mild fever
592
Reiter's Syndrome
Consists of:
Joint swelling
Conjunctivitis
Urethritis (dysuria)
593
Reactive Arthritis - Ix
To rule out other conditions:
* Bloods – normal or mild raise in ESR/CRP
* Rheumatoid factor – negative
* Urine/stool MC&S – bacterial/viral infections
* Joint aspiration and culture – no organisms
* X-ray – normal
594
Reactive Arthritis - Mx
NSAIDs – reduce inflammation
| Steroids – if severe
595
What are some other causes of Polyarthritis besides JIA?
1. Infection – bacterial, viral, reactive.
2. IBD – Crohn’s, UC
3. Vasculitis – HSP, Kawasaki
4. Haematological – haemophilia, Sickle Cell Disease
5. Malignancy – leukaemia, neuroblastoma
6. Connective Tissue Disorders – SLE, dermatomyositis, polyarteritis nododa
7. Other – Cystic fibrosis
596
Growing Pains
Wake in the night with pain (no daytime Sx)
Often worse after activity
Eased with massage
No abnormal physical signs
597
Idiopathic pain syndromes
```
Chronic fatigue Syndrome
Myalgic encephalomyelitis
Fibromyalgia
Diffuse idiopathic pain
Localised idiopathic pain
```
598
What haematological changes occur at birth?
Location of haematopoesis changes
=> from liver to the bone marrow.
Foetal Hb (HbF) is replaced by adult Hb (HbA)
Hb levels are high in newborns
=> Drops over the first few weeks.
599
HbF and HbA
HbF = 2alpha and 2gamma – higher oxygen affinity
HbA = 2alpha and 2beta – lower oxygen affinity.
600
what is Anaemia?
= Hb below the normal range for age.
- Neonate: < 14 g/dL
- 1-12 months: < 10g/dL
- 1-12 years: <11 g/dL
601
what are the 3 general causes of anaemia?
1. Reduced red cell production
2. Increased red cell destruction (haemolysis)
3. Increased red cell loss (bleeding)
602
Anaemia - Sx
Anaemia often asymptomatic, Sx normally only present when Hb quite low.
* Fatigue/weakness
* Pallor
* SoB/tachycardia
* Slow feeding, eating soil or chalk
603
DDx Macrocytic Anaemia
```
Alcohol and liver disease
B12 deficiency
Compensatory reticulocytosis (blood loss)
Drugs (cytotoxic)
Endocrine (hypothyroidism)
Folate deficiency
```
604
DDx Microcytic Anaemia
```
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
```
605
Anaemia - Ix
FBC – MCV (size of RBC) and MHC (Hb per RBC)
Iron studies – serum iron and ferritin
Blood film – size, shape, colour
Serum bilirubin
Hb high performance liquid chromatography or Hb electrophoresis
=> Shows amount of each Hb type (HbS, HbA, HbF)
606
What are causes of reduced RBC production
Iron deficiency anaemia
B12/folate deficiency
RBC Aplasia
607
What are causes of increased RBC destruction
Intrinsic/extrinsic haemolysis
INTRINSIC - hereditary spherocytosis, G6PD deficiency, thalassaemias, SCD
EXTRINSIC - autoimmune, infection, drugs, burns
608
What is the #1 cause of anaemia in children and why?
Iron Deficiency Anaemia
Due to high iron requirement of growth
609
Iron deficiency anaemia - Causes
Inadequate intake
=> Prolonged breastfeeding (>6 months), iron deficient diet
Malabsorption – Coeliac
Blood loss
610
Iron deficiency anaemia - Diagnosis
FBC – decreased MCV and MHC
Iron studies – decreased serum iron and serum ferritin
Blood film – abnormally shaped, small, pale RBCs
611
Iron deficiency anaemia - Management
Dietary advice
=> High iron foods
=> VitC helps iron absorption
=> Avoid too much cow’s milk
Oral iron supplements
=> Continue until Hb normal, PLUS 3 months.
NB – if no response to Tx, consider Ix for other causes (especially malabsorption)
612
What foods are high in iron?
```
red meat
pulses, beans, peas
leafy green veg
oily fish
fortified cereals
```
613
How long are iron supplements needed in iron deficiency anaemia?
Taken until Hb returns to normal, PLUS 3 months.
614
what are B12 and folate needed for ?
```
B12 = coenzyme needed for folate conversion
folate = needed for RBC synthesis
```
615
Causes of B12 and Folate Deficiency?
Low dietary intake
Malabsorption – e.g. Crohn’s
Low intrinsic factor – e.g. autoimmune
616
B12 and Folate Deficiency - diagnosis
FBC – increased MCV (= macrocytic)
| Iron and B12 studies – decreased B12, decreased serum folate, decreased cobalamin
617
B12 and Folate Deficiency - management
Dietary advice
=> B12 – eggs, fortified cereals, dairy
=> Folate – broccoli, peas, brown rice
Oral B12 and folic acid supplements
618
what is RBC Aplasia?
= failure of RBC synthesis
619
Causes of RBC aplasia?
Diamond-Black anaemia (rare congenital condition)
Transient erythroblastopaenia (triggered by viral infection)
Parvovirus B19 (ONLY causes RBC aplasia in children with inherited haemolytic anaemia)
620
RBC aplasia - diagnosis
Decreased Hb
Reduced reticulocytes
Normal bilirubin
Coombs test -ve
621
Haemolysis - clinical features
* Anaemia
* Hepatosplenomegaly
* Jaundice
622
Haemolysis - investigations
* FBC – low Hb
* Blood film – increased reticulocytes
* Bilirubin – increased unconjugated bilirubin and increased urinary urobilinogen
* Lactate dehydrogenase – increased LDH
623
What is the mode of inheritance of sickle cell disease?
autosomal recessive
624
Pathophysiology of sickle cell disease
Caused by a mutation in beta-globin gene causing abnormal HbS chain rather than HbA
= point mutation of glutamine to valine
Sickle-shaped RBCs have a decreased lifespan and also get stuck and occlude vessels.
625
Sickle cell disease - risk factors
Black, Afro-Caribbean
626
Sickle cell disease - types
Sickle Cell Anaemia (HbSS)
HbSC Disease (HbSC)
Sickle beta-thalassamia (HbSA)
Sickle Cell Trait (HbSA) - no symptoms, carrier
627
Sickle beta-thalassamia (HbSA)
1 HbS + beta-thalassaemia trait causing low HbA
628
Sickle cell disease - clinical presentation
Moderate anaemia
Jaundice
Infection (increased susceptibility)
Splenomegaly
Painful vaso-occlusive crises
Acute Anaemia
629
Symptoms of vaso-occlusive crises in sickle cell disease
Hands and feet – dactylitis and swelling
Bones of limbs – avascular necrosis of femoral head
Lungs – acute chest syndrome = EMERGENCY
Penis – priapism
630
Acute anaemia in sickle cell disease
Sudden drop in Hb +/- abdo pain, hepatosplenomegaly, circulatory collapse.
Triggered by infection, accumulation of sickle-cells in spleen
631
Long-term problems in sickle cell disease
Short stature and delayed puberty
Stroke and neuro damage
Heart failure and renal dysfunction
Pigment gallstones
632
Sickle cell disease - diagnosis
1. Screening – heel-prick test
2. Clinical presentation
3. FBC and iron studies
4. Blood film – sickle cells
5. Hb HPLC – HbS
633
Sickle cell disease - management
1. Infection prophylaxis – vaccines and daily PO penicillin through childhood
2. Daily folate supplements (lifelong)
3. Decrease risk of VO crises – dress warm, stay hydrated, avoid excessive exercise/stress
4. Treat VO crises – PO/IV analgesia, Abx and O2 if needed.
5. Hydroxyurea to increase HbF – if recurrent VO crises/acute chest
6. BM transplant – only cure
634
what is the only cure for sickle cell disease?
Bone marrow transplant
635
Beta-Thalassaemia
= autosomal-recessive disorder of beta-globin production, causing decreased HbA.
- b-thalassaemia major – no HbA = very severe
- b-thalassaemia minor – some HbA or HbF = mild/asymptomatic
636
Beta-Thalassaemia - prognosis
90% live beyond 40 years if treatment compliant
637
Prenatal diagnosis for Beta-Thalassaemia
Offered if one/both parents are affected.
if 2 affected parents = ¼ risk child affected
638
Beta-Thalassaemia - clinical features
Severe anaemia from 3-6 months – transfusion dependent
Jaundice and pallor
```
Hepatosplenomegaly (if untreated)
Characteristic facies (if untreated)
```
639
Beta-Thalassaemia - management
1. Lifelong monthly blood transfusion
2. Iron chelation (prevent Fe overload from transfusions)
3. BM transplantation = only cure
640
What is caused by iron deposition in the...
Heart
Liver
Pancreas
Skin
Heart – cardiomyopathy
Liver – cirrhosis
Pancreas – diabetes
Skin – hyperpigmentation
641
Alpha-Thalassaemia
= autosomal recessive disorder of alpha-globin production causing decreased HbA.
a-thalassaemia major / Hb Barts hydrops fetalis – deletion of all 4 globin genes
=> very severe (no HbA)
=> leads to death in utero
HbH disease – deletion of 3 a-globin genes
=> Some HbA – mild/moderate anaemia
=> May need transfusions
a-thalassaemia trait – deletion of 1 or 2 a-globin genes
=> asymptomatic (some HbA)
642
what is the mode of inheritance of Beta-Thalassaemia?
= autosomal-recessive
643
what is the mode of inheritance of Alpha-Thalassaemia?
= autosomal-recessive
644
Thalassaemia - investigations
1. FBC and iron studies – microcytic, hypochromic anaemia
2. Blood film – “target cells” or nucleated RBCs
3. Hb HPLC – proportions of HbA, HbF, HbA2
645
Hereditary Spherocytosis
= autosomal-dominant or de novo mutations for RBC membrane proteins.
Abnormal membrane causes spheroidal-shaped RBCs which are weak and hence destroyed in the spleen.
646
What is the mode of inheritance of Hereditary Spherocytosis?
autosomal dominant
OR de novo mutation
647
Hereditary Spherocytosis - clinical presentation
Jaundice – may be intermittent
Mild anaemia
Splenomegaly – due to haemolysis
Gallstones – decreased bilirubin excretion
APLASTIC CRISIS – new RBCs not made fast enough
=> Follows parvovirus B19, lasts 2-4 weeks
648
Hereditary Spherocytosis - diagnosis
Suspect if FHx
FBC and iron studies – microcytic anaemia
Blood film – microcytic spherical RBCs
649
What can happen in a patient with Hereditary Spherocytosis following parvovirus B19 infection?
aplastic crisis (RBC aplasia)
650
Hereditary Spherocytosis - management
Oral folic acid – increased demand as increased RBC synthesis
Tx for aplastic crisis – tranfusions until resolves
Splenectomy – if poor growth/troublesome Sx
=> Will need daily prophylactic ABX afterwards
651
G6PD Deficiency
= X-linked recessive mutation in G6PD gene – an enzyme involved in preventing oxidative damage to RBCs.
652
What is the mode of inheritence of G6PD deficiency?
= X-linked recessive
653
G6PD Deficiency - risk factors
African, Mediterranean, Middle Eastern
654
G6PD Deficiency - clinical presentation
Asymptomatic between haemolytic episodes
Neonatal jaundice – usually within first 3 days
Acute intravascular haemolysis – fever, pallor, malaise, dark urine
=> Precipitated by infection, certain drugs, fava beans
=> Hb drops <5g/dL within 24-48 hours
655
G6PD Deficiency - diagnosis
FBC – decreased Hb, increased reticulocytes
Unconjucated BR raised
LDH raised
Blood film – Heinz body inclusions (only seen during haemolytic crisis)
G6PD activity – reduced
656
Heinz body
= rounded structure protruding from the margin of an erythrocyte or as a small somewhat refractile spot within the cell, they are the result of oxidative damage to erythrocyte haemoglobin.
657
G6PD Deficiency - management
1. Safety net for signs of acute haemolysis
2. Advise on foods/drugs to avoid
=> Quinine, sulphonamides, nitrofurantoin, high dose aspirin, fava beans
3. May need blood transfusion during haemolytic crisis
658
Bleeding Disorders - History
Age of onset
Bleeding Hx/pattern
- Bleeds with previous surgery/dental work – inherited
- Mucous membranes – platelet disorder/vWD
- Haemarthrosis – haemophilia
Drug Hx - Anticoagulants?
659
Bleeding Disorders - Ix
FBC and blood film
PT and APTT
Thrombin time
Mixing studies – determine if factor deficient or inhibitor present
D-dimers
660
Inherited Bleeding disorders
Haemophilia A + B
| vWD
661
Acquired Bleeding disorders
Vitamin K deficiency
Thromcobytopaenia (immune thrombocytopaenia (ITP))
DIC
662
What is the mode of inheritance of haemophilia?
= X-linked recessive
663
What is haemophilia?
What factors are affected in Haemophilia A and B?
= X-linked recessive coagulation disorders.
Haemophilia A = factor VIII deficiency (more common)
Haemophilia B = factor IX deficiency
664
Haemophilia - clinical features
- Mild – bleed after surgery
- Moderate – Bleed after minor trauma
- Severe – spontaneous joint/muscle bleeds
40% of cases present in neonates with intracranial haemorrhage/post-circumcision bleed.
Commonly presents when start to crawl/walk.
665
Haemophilia - diagnosis
Increased APTT
| Low Factor VIII/IX
666
Haemophilia - management
Avoid NSAIDs and IM injections
For any acute bleeds – IV recombinant factor 8 or 9 (given ASAP, parents taught to administer at home).
For severe disease/before major surgery – regular prophylactic IV factor
For mild disease – desmopressin (antidiuretic that causes secretion of factor VIII and vWF into plasma)
667
Von Willebrand Disease (vWD)
= autosomal dominant deficiency in von Willebrand factor (vWF).
vWF facilitates platelet adhesion and is a carrier protein for factor VIII preventing its degradation.
668
What is the mode of inheritance of vWD?
autosomal dominant
669
vWD - clinical features
- Bruising
- Prolonged bleeding
- Mucosal bleeding – epistaxis, menorrhagia
670
vWD - diagnosis
Increased APTT
| Normal platelets and INR
671
vWD - management
Avoid NSAIDs and IM injections
Mild – desmopressin
Severe – plasma-derived factor VIII concentrate/vWF
672
Which coagulation factors need vitamin K?
factors 2, 7, 9, 10
673
Causes of vitamin K deficiency
Caused by inadequate intake, malabsorption, warfarin.
674
definition of thrombocytopaenia
= Platelets <150 x10^9 /L
- Mild = 50-150
- Moderate = 20-50
- Severe <20
675
thrombocytopaenia - clinical features
* Bruising, petechiae, purpura
* Mucosal bleeding
Less common = GI haemorrhage, haematuria, intracranial bleed.
676
what is the most common cause of thrombocytopaenia in children?
= immune thrombocytopaenia (ITP)
Caused by destruction of circulating platelets by IgG autoantibodies (following viral infection).
=> Onset 1-2 weeks post viral infection
677
immune thrombocytopaenia (ITP) - investigations
- Careful Hx and examination
- FBC and blood film
- BM examination – to exclude leukaemia/aplastic anaemia
678
immune thrombocytopaenia (ITP) - management
Benign, self-limiting – resolves in 6-8 weeks
=> If major bleed/persistent minor bleed – oral prednisolone, IV anti-D or IV Ig
=> If life-threatening haemorrhage – platelet transfusion
Chronic ITP – persistently low platelets for >6 months
=> If major bleed/persistent minor bleed – oral prednisolone, IV anti-D or IV Ig
=> If life-threatening haemorrhage – monoclonal antibodies, splenectomy
Avoid contact sports if platelet count is low.
679
Disseminated Intravascular Coagulation
coagulation pathway activation => diffuse fibrin deposition and consumption of coagulation factors and platelets.
680
DIC - causes
Severe sepsis
Trauma/burns
Malignancy
Toxins
681
DIC - Sx
Bruising
Purpura
Haemorrhage
682
DIC - diagnosis
CLOTTING SCREEN
- Low platelets, fibrinogen
- Raised D-dimer
- Low antithrombin
- Low protein C and S
- Raised APTT and PT
683
DIC - management
Treat underlying cause
| Supportive – e.g. FFP, platelets
684
What is a seizure?
| What types are there?
= a sudden disturbance of neurological function resulting in a change in behaviour.
EPILEPTIC - due to excessive, hyper-synchronous neuronal discharge in the cerebral cortex.
NON-EPILEPTIC - Seizures not involving abnormal electrical activity in the brain
685
Causes of epileptic seizures
Idiopathic (70-80%)
Secondary
- Cerebral dysgenesis
- Cerebral vascular occlusion
- Cerebral damage (e.g. hypoxia, infection)
- Cerebral tumour
Neurodegenerative disorders
Neurocutaneous syndromes
686
Causes of non-epileptic seizures
Febrile seizures
Metabolic
Head trauma
Infection - Meningitis/encephalitis
Poisons/toxins
Hypoxia/anoxia
=> Reflex anoxia/Cardiac arrythmia
687
Seizures - Investigations
1. History = MOST IMPORTANT
2. Examination
3. EEG
4. Imaging
5. Tests to r/o other causes (ECG, bloods)
688
Seizure - Hx
Frequency, triggers, length, symptoms
Any impairments, educational/psychological/social impacts
Video of seizure if possible
689
Seizure - Examination
CNS and PNS
CVS and resp
Skin markers for neurocutaneous syndromes
690
Seizure - Imaging
MRI/CT – if neuro signs between seizures (r/o tumour or CVD)
PET/SPECT – detect areas of hypo/hypermetabolism
691
What is epilepsy?
| How common is it?
= Chronic neurological disorder, characterised by recurrent, unprovoked seizures.
1 in 200 children affected
692
Types of epileptic seizures
1. Generalised
- Discharge from both hemispheres
- No warning
- Always have LOC
2. Focal:
- Discharge from ONE part of ONE hemisphere
- May have a preceding aura
- May or may not have LOC
- May lead to generalised tonic-clonic seizure if LOC
693
Generalised epileptic seizures
```
Tonic-clonic (Grand-mal)
Absence (petit-mal)
Myoclonic
Tonic
Atonic
```
694
Focal epileptic seizures
Temporal
Frontal
Occipital
Parietal
695
Tonic-clonic seizure
Tonic phase
=> May fall to ground, hold breath (cyanosis)
```
Clonic phase
=> Last seconds to minutes
=> Rhythmical jerking
=> Irregular breathing, cyanosis, salivation
=> Tongue-biting, incontinence
```
Post-ictal sleep/LOC (up to several hours)
696
absence seizure
“Blanking out” / staring
Absence of motor symptoms
Brief onset and termination
697
Myoclonic seizure
Repetitive, jerky movements
698
Tonic or atonic seizure
Tonic - Generalised increased tone => Fall
Atonic - Loss of muscle tone => fall
699
Complications of epileptic seizures in children?
Many have learning difficulties
Continuation into adulthood
Sudden unexplained death in epilepsy (SUDEP)
700
Sudden unexplained death in epilepsy (SUDEP)
Rare but must discuss with parents
Decrease risk by minimising seizures.
701
Epilepsy - Ix
As per seizure investigations
| EEG is always indicated if epilepsy is suspected
702
Epilepsy - Mx
Depends on likelihood of recurrence, severity and impact on life of seizures.
1. Education and Advice
2. Anti-epileptic Drugs
3. Rescue Therapy
703
Epilepsy - Education and Advice
Lifestyle – avoid deep baths/swimming alone, alcohol/sleep, only drive if no seizures for 1 year.
During seizure:
• DO – place pillow under head, move nearby objects, note time seizure began, place in recovery position afterwards.
• DO NOT – restrict/move child, give food/drink until complete recovery
704
For how long are Anti-epileptic Drugs taken?
Until seizure free for 2 years.
705
Principles of Anti-epileptic Drug therapy
- Drug depends on seizure type
- Aim for monotherapy at lowest effective dose.
- Must be counselled on side effects
- A single seizure does not indicate starting AEDs
706
What is the 1st line drug for generalised epileptic seizures?
What are the side effects of this drug?
Valproate
Weight gain, Hair loss, TERATOGEN
707
What is the 2nd line drug for generalised epileptic seizures?
What are the side effects of this drug?
Lamotrigine
Rash (SJS), Insomnia
708
Indication for Carbamazepine in epilepsy?
What are its side effects?
Focal seizures
SEs - Rash, Hyponatraemia, Ataxia, CYP140 inducer
709
Indication for Levetiracetam in epilepsy?
Focal seizures
710
Indication for Ethosuximide in epilepsy?
Absence seizures
711
Epilepsy - rescue therapy
BZDs
| => Given to terminate prolonged seizures (>5 mins)
712
Epilepsy Syndromes of Childhood
```
West Syndrome (Infantile spasms and hypsarrythmia)
Lennox-Gastaut
Benign Occipital
Benign + centrotemporal spikes
Childhood Absence
Juvenile Myoclonic
```
713
What is West Syndrome?
INFANTILE SPASMS
- violent spasms lasting 1-2s
- often on waking
- 4-6 months old
Associated with:
- Low IQ/developmental regression
- Poor social interaction
- Hypsarrhythmia EEG
714
Lennox-Gastaut Syndrome
- Drop attacks
- T-C
- atypical absence
- Neurodevelopmental arrest
715
Juvenile Myoclonic Syndrome
Occurs in Adolesence
Myoclonic jerks after waking
716
Definition of febrile seizures
= seizure accompanied by fever, caused by infection of extra-cranial origin.
DDx – sepsis, meningitis, brain abscess, gastroenteritis.
Usually occur at 6 months – 6 years old
717
febrile seizures - features
Onset = early in viral infection
Brief (<10 mins), generalised tonic-clonic
Rapidly rising temperature +/- other signs of infection
718
febrile seizures - complications
Further febrile seizures (30-40%)
Increased risk of epilepsy if complex seizure (>10 mins, focal, recurring in 24 hours, <18 months)
719
What is the chance of a child having further febrile seizures after their first?
30-40%
720
febrile seizures - Management
Reassurance – usually no long term complications
Education – high chance of another, seizure first aid, rescue Tx.
May give rescue Tx if >5 mins
Manage underlying infection – anti-pyrexials, fluids, etc.
721
What can be used for rescue Tx in seizures in children?
diazepam buccal/PR
OR
SL midazolam
722
What are reflex Anoxic Seizures?
| What triggers them?
Occur due to vagal inhibition caused by temporary cardiac asystole.
Triggers – pain, cold food, fear, fever.
Usually grow out of these by age 4-5
723
How can Cardiac Arrythmias cause seizures?
Prolonged Q-T causes collapse/syncope
May be exercise-induced
724
Psychogenic non-epileptic Seizures
Triggered by stress/emotions
More common in adolescent girls
725
Benign Sleep Myoclonus
Myoclonic jerks during REM sleep, no EEG changes
Typically <6 months old
726
Paroxysmal events
= Sudden onset symptoms mimicking seizures (“funny turns”)
Include:
- breath-holding attacks
- Neurally mediated Syncope/Faint
- Migraine
- Benign Paroxysmal Vertigo
727
Primary Headaches
1. Tension Type
2. Cluster
3. Migraine
728
Secondary Headaches
Raised ICP/space occupying lesions
Head/neck trauma,
IC haemorrhage,
Toxins (alcohol/drugs, etc),
Acute sinusitis
729
features of tension headache
Symmetrical “band” of pressure, gradual onset (usually evening).
730
features of cluster headache
One-sided, excruciating attacks of pain, often around one eye
731
features of migraine
= Headache associated with other symptoms
Common in children
Triggers – stress, exercise, tiredness, bright lights, cheese/chocolate, hormones.
Without aura (90%) – bilateral, pulsatile headache + N&V, abdo pain, photophobia, phonophobia => 1-72 hours.
With aura (10%) – preceding visual disturbance (hemianopia/scotoma/zig-zag lines) => several hours.
732
Features of raised ICP/space occupying lesions
Morning vomiting/headache,
Night-time waking/worse Sx lying down,
Altered mood/behaviour.
Visual field defect,
CN abnormalities,
Abnormal gait,
Papilloedema
733
Headaches- Ix
Thorough Hx – triggers, onset, duration, Sx, substance use, analgesia overuse.
Physical Examination – vision, sinus tenderness, pain on chewing, BP, CNS & PNS
734
Headaches - Mx
Education & advice:
=> Reassure that recurrent headaches are common and cause no long-term harm
=> No cure, but can relieve Sx.
Prophylaxis (if >2 per month):
=> 5-HT antagonist, beta-blockers, sodium channel blockers.
Rescue Tx:
=> Analgesia, anti-emetics, serotonin agonists (Sumatriptan, if >12 years).
Psychological Support:
=> If headaches triggered by particular stressor – e.g. bullying, exams.
735
What is cerebral palsy?
= disorder of movement and posture due to non-progressive disturbance in developing brain <2 years.
Often accompanied by disturbed cognition, communication, vision, perception, sensation, behaviour.
Associated with seizures and secondary MSK problems
736
what is the most common cause of motor impairment in children?
cerebral palsy
737
When can the causes of cerebral palsy occur?
- Antenatal (80%)
- Perinatal (10%)
- Postnatal (10%)
738
Antenatal causes of cerebral palsy
Hypoxia – cord prolapse, APH, maternal shock, rhesus disease, placental insufficiency (pre-eclampsia)
Abnormal development
Congenital infection – e.g. STIs, rubella, CMV
739
Perinatal causes of cerebral palsy
Hypoxic-ischaemic injury – prolonged labour/delivery, breech, C-section
740
Postnatal causes of cerebral palsy
Prematurity = big risk factor => intraventricular haemorrhage, infection, etc.
Metabolic disturbance – e.g. hyperbilirubinaemia
Head trauma
741
Cerebral Palsy - clinical presentation
Although non-progressive damage, Sx often emerge over time during development.
- Abnormal posture and tone
- Delayed motor milestones
- Feeding difficulties
- Asymmetric hand function
742
Types of cerebral palsy
1. SPASTIC (90%)
2. DYSKINETIC
3. ATAXIC/HYPOTONIC
743
Spastic Cerebral Palsy
```
Damaged UMN (corticospinal/pyramidal pathway)
=> causes spastic movements, hyperreflexia, +ve Babinski
```
Can be Hemiplegic, Diplegic, Quadriplegic
744
Dyskinetic Cerebral Palsy
Damaged basal ganglia or extra-pyramidal tracts
=> causes involuntary, uncontrolled movements and dystonia
```
Chorea = irregular, brief, fidgety movements.
Athetosis = slow, writhing movements in distal muscles
Dystonia = abnormal tone and muscle contraction causing twisting appearance.
```
745
Ataxic/hypotonic Cerebral Palsy
Damage to cerebellum => causes cerebellar signs
Hypotonia, poor balance, uncoordinated movements, intention tremor, ataxic gait.
746
Management of Cerebral Palsy
1. Therapies – physio, occupational therapy, speech and language therapy.
2. Medication
- Botulinum toxin injections – improve specific muscle tightness, stop drooling.
- Muscle relaxants – baclofen, diazepam (short-term only)
3. Surgery:
- Cut nerves to spastic muscles – may cause numbness
- Orthopaedic surgery – lengthen muscles, correct limb position.
747
Ataxia
= incoordination of muscle movement (gait, speech, posture) due to cerebellar/motor pathway problems.
748
Causes of ataxia
Medications/Drugs
Infection – varicella
Posterior fossa lesions
Genetic and neurodegenerative disorders
749
Symptoms of ataxia
Unsteady, wide-based gait
Dysdiadochokinesia and dysmetria
Intention tremor
Nystagmus
750
Genetic and neurodegenerative disorders causing ataxia
Friedrich Ataxia
| Ataxic Telangiectasia
751
Friedrich Ataxia
Autosomal recessive trinucleotide repeat (frataxin gene)
Diagnosed with genetic testing.
Complications – kyphoscoliosis and cardiomypoathy
752
Ataxic Telangiectasia
Autosomal recessive DNA repair disorder (ATM gene)
Diagnosed with genetic testing and raised AFP
Complications – increased risk of infection (IgA defect), malignancy (ALL)
753
Subdural Haematoma - causes
Non-accidental Injury (shaking/direct trauma)
| Direct trauma to head
754
Subdural Haematoma - symptoms
Altered mental state/seizures
Apnoea/breathing difficulty
Retinal haemorrhages often present
=> If a child presents like this consider NAI and safeguarding.
755
Spinal Muscular Atrophy
Caused by autosomal recessive degeneration of anterior horn cells.
Sx – progressive weakness and wasting of skeletal muscle.
3 types – type 1 is most severe form which presents in infancy and causes death by 1 year from respiratory failure.
756
Peripheral Neuropathies
Guillain-Barre
Chronic Inflammatory Demyelinating Polyneuropathy
Hereditary Motor Sensory Neuropathies
Bell Palsy
757
What is Guillain-Barre?
What are the symptoms?
Autoimmune demyelination of peripheral nerves following infection
2-4 weeks after URTI or campylobacter.
Sx:
- Ascending symmetrical weakness (over days-weeks)
- Loss of reflexes
- Distal paraesthesia
- Difficulty chewing/swallowing
- Difficulty breathing (if respiratory muscles affected)
- Autonomic dysfunction – tachy/bradycardia, HTN, urinary retention
758
Guillain-Barre - Ix
Lumbar puncture – increased protein, normal WCC
Nerve conduction studies – slowed
+/- spinal cord MRI
759
Guillain-Barre - Mx
Supportive – may need artificial ventilation
| Reassure 90% recover fully (may take 2 years)
760
Chronic Inflammatory Demyelinating Polyneuropathy
Thought to be autoimmune
Sx similar to Guillain-Barre, but slower progression.
Mx – supportive PLUS HIGH DOSE CORTICOSTEROIDS (prednisolone)
761
Hereditary Motor Sensory Neuropathies
Demyelination and attempted re-myelination of nerves.
762
What is the most common Hereditary Motor Sensory Neuropathy?
What are the Sx?
How is it managed?
Type 1 (CHARCOT-MARIE-TOOTH) = most common (dominant inheritance)
Sx:
- Progressive symmetrical distal muscle wasting.
- +/- distal sensory and reflex loss
- Physical deformities – stork leg, pes cavus, hammer toes.
Mx = long-term physio and OT to improve symptoms/prevent decline (no cure)
763
Bell Palsy
- Cause
- Symptoms
- Complications
- Managements
Isolated LMN paresis of CNVII (facial)
Occurs post-infection
Sx – complete hemiparesis of face
Complications – conjunctivitis as cannot close eye.
Mx –
Usually recover fully in several months
Corticosteroids in first week, eye patch.
764
Muscular Dystrophies
1. Duchenne Muscular Dystrophy
2. Becker Muscular Dystrophy
3. Congenital Muscular Dystrophies
765
What is the mode of inheritance of Duchenne Muscular Dystrophy?
What does the mutation lead to?
X-linked or de novo mutation – deletion at Xp21
=> Leads to reduced dystrophin protein, resulting in myofiber necrosis
766
What is the most common muscular dystrophy?
Duchenne Muscular Dystrophy
767
DMD - signs/symptoms
= Progressive muscular atrophy
Infants – Gower’s Sign, waddling gait, climb stairs 1-by-1, language delay.
Children – slow running, clumsiness, pseudohypertrophy of calves, non-ambulatory by 10-14 years.
768
Gower’s Sign
Use of their hands and arms to "walk" up their own body from a squatting position due to lack of hip and thigh muscle strength.
Indicates weakness of the proximal muscles
769
DMD - life expectancy
= early 20s
Due to complications - respiratory failure, cardiomyopathy, scoliosis
770
DMD - Ix
- Serum creatine phosphokinase – raised
- Genetic testing
- Muscle biopsy.
771
DMD - Mx
Corticosteroids – 10 days each month, to delay scoliosis and preserve mobility.
- Physiotherapy
- Scoliosis brace, foot and ankle boots/splints
- Surgery to lengthen Achilles tendon/correct scoliosis
- Respiratory support
772
How does Becker Muscular Dystrophy differ to DMD?
Less common
Some functional dystrophin produced => later onset and slower progression
Longer life expectancy
773
Congenital Muscular Dystrophies
Present at birth/early infancy
Mostly autosomal recessive
Caused by lack of extracellular matrix protein
Weakness, hypotonia, contractures +/- learning difficulties.
774
what is Myotonia ?
= delayed relaxation after sustained muscle contraction
e.g. difficulty releasing grip after handshake.
775
Myotonic dystrophy
Autosomal dominant inherited trinucleotide repeat.
Progressive muscle wasting and weakness.
Symptoms worsen down generations.
Symptoms:
- Infant – feeding and breathing difficulties
- Child – myotonia, facial weakness, learning difficulties
- Adult – cataracts, baldness, testicular atrophy, cardiomyopathy.
776
what is Hydrocephalus ?
| what types are there?
Accumulation of CSF in the brain
Types:
- Non-communicating – obstruction in ventricular system
- Communicating – failure to reabsorb CSF/overproduction
777
Hydrocephalus - presentation
- Disproportionately large and rapidly increasing head circumference
- Bulging fontanelles, distended scalp veins
- Fixed downwards gaze
- Will develop signs of raised ICP
778
Hydrocephalus - investigations
- May be diagnosed on antenatal USS
- Cranial USS (infants) or CT/MRI
- Monitor head circumference
779
Hydrocephalus - management
- Ventriculoperitoneal shunt
| - Ventriculostomy
780
Definition of macrocephaly and causes?
Head circumference >98th centile.
Causes:
- Tall stature
- Familial macrocephaly
- Raised ICP
- CNS storage disorders
- Neurofibromatosis
781
Definition of microcephaly and causes?
Head circumference <2nd centile
Causes:
- Familial microcephaly
- Autosomal recessive condition
- Congenital infection
- Acquired after insult to developing brain
782
Craniosynostosis
Premature fusion of one or more sutures leading to distorted head shape.
Types – localised / generalised
- Localised – sagittal suture, coronal suture, lamboid suture
- Generalised – multiple sutures
783
Trisomies
Trisomy 21 - Down's Syndrome
Trisomy 13 - Patau Syndrome
Trisomy 18 - Edward's Syndrome
784
Down's syndrome - diagnosis and prevalence
= Trisomy 21
>50% live to over 50 years
1 in 650
Diagnosis:
- Antenatal screening – combined/triple test + nuchal translucency
- Chromosome analysis – blood test
Prevalence increases with maternal age.
- Age 44 – 1 in 37
785
Down's syndrome - physical features
Round face, small mouth/ears, flat nasal bridge, protruding tongue.
Epicanthic folds, brushfield spots
Hypotonia
Single palmar crease
“Sandal gap” between toes
786
Down's syndrome - associated conditions
```
Congenital heart defects (40%)
Hearing and visual impairments
Leukaemia
Cognitive problems / Early-onset Alzheimer’s
Epilepsy
Coeliac Disease
Infections
```
787
Edward's syndrome
Trisomy 18
Physical features:
- IUGR / low birthweight
- Prominent occiput, small jaw
- Overlapping fingers
- Rocker-bottom feet
- Cardiac and renal malformations
Most die in infancy
788
Patau Syndrome
Trisomy 13
Physical features:
- Brain defects
- Microcephaly, Small eyes
- Cleft lip/palate
- Polydactyly
- Cardiac and renal malformations
Most die in Infancy
789
Turner’s Syndrome
45, X
Physical Features:
- Short stature
- Webbed neck
- Spoon-shaped nails
- Lymphoedema of hands/feet
Associated Conditions:
- Congenital heart defects (aortic coarctation, bicuspid valve)
- Delayed puberty
- Ovarian dysgenesis (infertility)
- Hypothyroidism
- Renal anomalies
790
Turner's Syndrome - diagnosis and treatment
Investigations:
- Blood karyotyping
- Buccal swab (for Barr Body = inactive X chromosome)
- LH and FSH
Treatment:
- Growth hormones
- Oestrogen replacement – develop 2o sexual characteristics but still infertile.
791
Klinefelter’s Syndrome
47, XXY
1 in 1000
Physical features:
- Hypogonadism/small testes (infertility)
- Gynaecomastia
- Lack of chest/facial hair
- Tall Stature
Tx – testosterone therapy may help.
792
examples of autosomal Dominant genetic disorders
Tuberous Sclerosis
Achondroplasia (dwarfism)
Marfan Syndrome
Neurofibromatosis (Skin changes and tumours along nerves)
Noonan Syndrome (Similar phenotype to Turner’s Syndrome, also mild learning difficulties)
793
examples of autosomal recessive genetic disorders
Phenylketonuria (PKU)
Sickle Cell Disease
Cystic Fibrosis
Tay-Sachs disease
794
examples of X-linked recessive genetic disorders
Males are affected, females are carriers (but may show mild disease.
```
Fragile X
Haemophilia A/B
Colour blindness
DMD/BMD
G6PD deficiency
```
795
examples of X-linked dominant genetic disorders
Very rare.
Both males and females will be affected (males usually die).
e.g. - Rett’s Syndrome
=> Severe speech, learning and coordination problems
796
Prader-Willi Syndrome
No paternal copy of chromosome 15q11-13.
```
Signs:
• Hypotonia
• Neonatal feeding difficulties
• Insatiable appetite later in childhood
• Narrow forehead
• Almond-shaped eyes
• Triangular mouth
```
```
Associated conditions:
• Poor growth
• Developmental delay
• Obesity/T2DM
• Learning difficulties
• Behavioural problems
```
797
Angelman Syndrome
No maternal copy of chromosome 15q11-13.
```
Signs:
• “Coarse” facial features
• Microcephaly
• Widely spaced teeth and wide mouth
• Prominent lower lip
```
```
Associated conditions:
• Intellectual disability
• Severe speech impairment
• Hyperactivity
• Ataxia
• Seizures
```
798
Russel-Silver Syndrome
Slow growth before and after birth, PLUS dysmorphic features.
Signs:
• LBW/FTT
• Head often disproportionate to small body
• Triangular face (prominent forehead, narrow chin)
• Curved 5th finger
Associated conditions:
• Short stature
• Delayed development
• Learning disabilities
799
William Syndrome
May be autosomal dominant or de novo
Signs:
• “Elfin” appearance – wide mouth, small upturned nose, short.
• Outgoing and friendly.
```
Associated Conditions:
• Mild learning disabilities
• Hypercalcaemia
• ADHD
• Aortic stenosis
```
800
Foetal stage of growth
30% of eventual height
Dependent on placental nutrition, maternal size/health
801
Infantile stage of growth (birth to age 2)
15% of eventual height
Dependent on nutrition, thyroid hormones, genes
802
Childhood stage of growth
40% of eventual height
Dependent on Nutrition, Thyroid and growth hormones, genes
803
Pubertal stage of growth
15% of eventual height
Dependent on Sex hormones, growth hormones, genes
804
Emotions and growth
All stages of growth depend on emotions/happiness as these impact hormone levels.
805
Why do boys end up taller than girls?
Girls start their puberty growth spurt ~2 years before boys (i.e. men get an extra 3-4 years of growth).
806
Role of GH in growth
Works directly at growth plate and indirectly via IGFs to increase organ size.
Peptide hormone (i.e. cannot be taken orally!)
Positive effect on GH release from catecholamines, serotonin, endorphins.
Negative effect on GH release by somatostatin
807
What is Laron Syndrome?
= GH insensitivity
808
Role of thyroid hormones in growth
Acts at growth plate.
Have effects on almost every cell in the body
In hypothyroidism, the growth plate is highly disorganised.
809
Role of sex hormones in growth
cause growth spurt, bone maturation and fusion of growth plates.
Once the growth plate closes, you will not grow anymore.
810
Measuring a child's growth
Weight, height (length if <2 years), head circumference
Plot serial growth measurements on growth chart
+/- Bone age, pubertal stage, BMI
811
Growth Indications for Urgent Specialist Referral
<0.4th centile or >99.6th centile.
Height markedly discrepant from weight.
Measurements cross centile line after 1 year.
Measurements cross 2 centile lines in 1st year.
812
Non-pathological causes of short stature
- Familial – short parents
| - Constitutional Delay of growth and puberty – late bone maturation and growth spurt.
813
Pathological causes of short stature
IUGR/extreme prematurity
Chromosomal disorders – Downs, Turner’s, Noonan’s, Russel-silver
Endocrine
=> associated with increase in weight
=> e.g. GH deficiency, excess corticosteroids, hypothyroidism
Nutritional/chronic illness
=> Associated with decrease in weight.
=> E.g. Coeliac, Crohn’s, chronic renal failure.
814
Investigations for abnormal growth
Height comparison:
- Against weight
- Against expected/mid-parental height
Hx, Examination, Hormone screen:
- Dysmorphic features
- Features of chronic/endocrine illness
- Hormones – GH, TFT, cortisol
- Birth Hx
- Nutritional Hx
815
Mid-parental height
((Father’s + Mother’s Height) / 2 )
+7cm for boys or -7cm for girls.
816
Causes of tall stature
Familial
Endocrine
=> Hyperparathyroidism, CAH, excess GH
Precocious puberty
Genetic Syndromes
=> Marfan’s, Klinefelter’s
817
Puberty age in females
9-11 years – 2o sex characteristics (breast development, then pubic hair)
10-12 years – growth spurt
11-13 years – menarche
818
Puberty age in males
10-14 years – 2o sex characteristics (increase in testicle size, then pubic hair, then dropping of scrotum and growth of penis)
12-14 years – growth spurt
819
Tanner Stage 1
= no signs of puberty
820
At what age is puberty considered early?
<8 years in girls,
| <9 years in boys.
821
At what age is puberty considered delayed?
>13/14 years in girls,
| >14/15 years in boys.
822
Causes of early/precocious puberty?
True/central – gonadotropin-dependent (premature activation of hypothalamic axis).
=> More common in girls
False/pseudo – gonadotropin-independent (excess sex steroids).
823
early/precocious puberty - investigations
- FHx, Examination, Hormone Screen
- Growth chart
- X-ray hand (determine bone-age)
- Orchidometer (if >4mm puberty has started)
- USS ovaries/uterus
- MRI (?tumours)
824
early/precocious puberty - management
Aim = prevent negative psychological impacts and conserve height potential (stop premature growth plate fusion).
Central PP – treat with GnRH analogues.
Pseudo PP – identify and treat underlying cause of excess sex hormones
825
Causes of delayed puberty
Constitutional delay in growth and puberty
Hypogonadotropic hypogonadism (decreased gonadotropins)
• Systemic disease (CF, anorexia, Crohn’s, increased exercise).
• Intracranial tumours/pituitary damage
• Syndromes – Kallman’s
```
Hypergonadotropic hypogonadism (increased gonadotropins)
• Syndromes – Turner’s, Klinefelters
```
826
delayed puberty - investigations
```
FHx, Examination, Hormone Screen (TFT, LH/FSH)
Growth Chart
X-ray hand (determine bone age)
Pubertal staging – testicular volume/USS
Karyotyping
```
827
delayed puberty - management
r/o or treat underlying causes
Reassure puberty will occur (Tx not usually needed)
IM testosterone/oestradiol
828
Cause of T1DM
Autoimmune destruction of pancreatic beta cells.
Genetic with environmental triggers.
=> Environmental triggers – enteroviral infection, cow’s milk, overnutrition.
829
T1DM - Symptoms
Polyuria, polydipsia, weight loss, lethargy.
+/- nocturnal enuresis, infections, signs of DKA.
830
Diabetes - diagnosis
= diabetes symptoms PLUS:
• Random blood glucose >11.1 mmol/L
• Fasting blood glucose >7.8 mmol/L
(also TFTs, coeliac screen, etc to r/o other causes)
831
T1DM - management
1. Intensive education for child and parents (and school)
• Injection of insulin and blood glucose monitoring
• Diet – carb counting, low GI carbs, adjustment for activity, alcohol
• “Sick-day” rules
• Signs of DKA/hypoglycaemia
2. Insulin
• S.c. pump = continuous infusion of rapid acting insulin (novorapid)
• Basal-bolus injection regimen = 2x LA at breakfast and night, and 1x SA before each meal.
3. Monitoring for complications = annual review
• Growth and pubertal development – risk of obesity/delayed puberty
• BP, renal function, eyesight, foot health
• Associated autoimmune conditions
832
Difficulties of glucose control in children
- Sugary foods/eat at odd times
- Infrequent/unreliable monitoring
- Poor family support
- Exercise
- Illness (increases insulin need)
833
Complications of DKA
Cerebral oedema
Hyper/Hypokalaemia
Hyponatraemia
Aspiration
834
Symptoms of hypoglycaemia
- Sweating, pallor, palpitations/tremor
- Headache, vision changes, confusion
- Hypotonia, poor feeding
- Drowsiness, seizures, coma
835
Causes of hypoglycaemia
- Neonatal hypoglycaemia
- Fasting/missed meals/ increased exercise
- Increased exogenous insulin dose
- Tumours (insulinoma)
- Drug-induced – sulphonyureas, alcohol
- Liver disease/glycogen storage disorder
- Addison’s, CAH, GH deficiency
836
Hypoglycaemia - Ix
- Careful Hx
- Physical Examination
- Blood glucose and ketones
- FBC, CRP, U&Es, LFT, TFT, blood gas
- Hormones – insulin, C-peptide, GH, cortisol
- Urinalysis – pH, ketones
837
Hypoglycaemia - Mx
- Oral fast-acting glucose – sugary drink/glucogel
- IV glucose – 2mL/kg dextrose bolus, then 10% dextrose infusion
- IM glucagon – if unconscious/fail to respond
838
In which situations should a child's blood glucose always be measured?
- Becomes septic/appears seriously ill
- Has a prolonged seizure
- Has an altered state of consciousness
839
Diabetes insipidus
= ADH disorder causing polydipsia and polyuria.
1. Central DI – insufficient ADH production
2. Nephrogenic DI – lack of kidney response to ADH = more common type in children
840
Diabetes insipidus - risk factors
Central:
=> Head injury, brain surgery, brain tumour.
Nephrogenic
=> Kidney disease/genetic mutation
=> Nephrotoxic medications (e.g. lithium)
841
Diabetes insipidus - management
Drink more to prevent dehydration, low salt diet.
Medication review (for nephrotoxics)
CENTRAL – manage with desmopressin (nephrogenic does not respond to desmopressin)
842
Congenital hypothyroidism - presentation
Initially asymptomatic - Most cases detected with heel-prick test (low T3/T4, high TSH)
Feeding problems, FTT
Prolonged jaundice
Constipation
Pale, cold, mottled skin
843
Why is it important to identify congenital hypothyroidism?
It is a preventable cause of severe learning difficulties
844
Acquired Hypothyroidism in children
Usually due to autoimmune thyroiditis
Girls > boys
RFs – Down’s, Turner’s, other autoimmine disorders
Presentation:
- Short stature
- Bradycardia
- Dry skin, thin hair
- Obesity, Constipation, Cold intolerance
- Delayed puberty
- +/- goitre
845
How is hypothyroidism managed?
Lifelong Levothyroxine
846
What is the most common cause of hyperthyroidism in children?
What are the symptoms?
Grave’s Disease = most common cause.
Symptoms are similar to in adults, but ALSO:
- Rapid growth in height
- Advanced bone maturity
- Learning difficulties/behavioural problems
- Eye signs are NOT common in children
847
Cushing's Syndrome in children
Most commonly due to long-term glucocorticoid Tx
Sx – Cushing Syndrome, growth suppression, osteopenia
848
Sx of Cushing's Syndrome
- Central obesity but short stature
- “moon face”
- Hirsutism
- HTN
- Depression
849
Definition of overweight/obesity in children
<12 years
Overweight = BMI >91st centile
Obese = BMI >98th centile
>12 years
Overweight = BMI >25
Obese = BMI >30
850
Risk factors for obesity
1. Lack of exercise/poor diet
2. Endocrine disorder – hypothyroidism, Cushing’s
3. Genetic syndrome – Prader-Willi
851
Rates of cancer in children
1 in 500 children <15 years develop cancer
Leukaemia and brain tumours are most common
852
Side Effects of Chemotherapy
1. Bone marrow suppression
=> Infection, anaemia, bleeding/bruising
2. GI disturbance
=> N&V, anorexia, undernutrition
3. Other
=> Hair loss, weight gain, mood
4. Long-term
=> Decreased fertility, late puberty
853
Acute Lymphoblastic Leukaemia (ALL)
- Incidence
- Pathophysiology
- RFs
- Prognosis
- 80% of childhood leukaemia
- Peak age 2-5 years
- Rapidly proliferating lymphocytes resulting in accumulation of abnormal, immature “blast” cells.
- RFs – Trisomy 21
- Prognosis = ~90% cure rate
854
Acute Lymphoblastic Leukaemia - symptoms
Onset over several weeks
• General – malaise, anorexia, LYMPHADENOPATHY, hepatosplenomegaly
* Anaemia
* Neutropaenia – infections/neutropenic sepsis
* Thrombocytopaenia – bruising, petechiae, nose bleeds
* Bone marrow infiltration – bone pain
* CNS infiltration – headaches, vomiting
855
Acute Lymphoblastic Leukaemia - Ix
1. FBC – low Hb, low platelets, low neutrophils, increased WCC
2. Peripheral Blood Film – blast cells
3. Clotting screen – 10% have DIC
4. Bone Marrow Biopsy = diagnostic and prognostic
5. CXR – mediastinal mass (characteristic of T-cell disease)
856
What should be avoided in children with a mediastinal mass?
NEVER sedate or anaesthetise someone with a mediastinal mass and orthopnoea
857
Acute Lymphoblastic Leukaemia - Mx
Before starting Tx – correct anaemia, thrombocytopaenia, treat any infections, renal protection (Allopurinol).
1. Remission Induction – eradication of blast cells with CHEMOTHERAPY and STEROIDS
2. Intensification – period of INTENSE CHEMO to consolidate remission (effective, but increased toxicity levels)
3. Continuation – chemo for 2-3 more years (2 in girls, 3 in boys)
4. Prophylactic Co-trimoxazole – given routinely to prevent PCP
5. Relapse – high-dose chemo and BM transplant
858
What forms of chemotherapy are given in ALL?
IV and intrathecal
859
Acute Lymphoblastic Leukaemia - poor prognostic factors
- Age <1 or >10
- WBC >50 x10^9 /L
- Cytogenic abnormalities in tumour cells
- CNS involvement
- Persisting blast cells after initial chemo
860
What is a lymphoma?
= solid malignancies of immune system
=> increased lymphocyte proliferation in LNs, spleen, thymus, BM
861
Lymphoma - Ix
* FBC & Blood film
* Lymph node biopsy – Reed-Sternberg cells in HL
* Bone marrow biopsy
* CT/MRI – assess nodal sites
862
Lymphoma - Mx
* Chemotherapy (+/- radiotherapy if HL)
| * PET to monitor Tx response
863
Hodgkin Lymphoma
- ~20% of lymphomas
- More common in adolescents
- Reed-Sternberg cells on LN biopsy (bi-nucleate “mirror cells”)
- Slightly better prognosis
864
What is Hodgkin's Lymphoma often associated with?
50% associated with EBV
865
Hodgkin Lymphoma - Sx
* Painless Lymphadenopathy – usually cervical, may cause mass effects (SC/bronchial obstruction)
* B-symptoms are less common
866
B- symptoms
fever,
drenching night sweats,
significant weight loss
Can be associated with Lymphoma
Also non-cancerous states such as TB, various inflammatory/rheumatologic conditions.
867
Non-Hodgkin's Lymphoma
- ~80% of lymphomas
- NO R-S cells on LN biopsy
- Slightly worse prognosis
- Can be high-grade/low-grade depending on speed of onset and symptoms.
868
Non-Hodgkin's Lymphoma - Sx
Painless Lymphadenopathy – cervical or abdominal, often cause mass effects (mediastinal mass).
Abdominal Symptoms
B symptoms are common
869
Brain tumour - presentation
Presentation depends on the location of tumour and will vary greatly during the process of growth and development.
General Sx:
- Headaches
- N&V
- Drowsy/irritable
- Seizures
If in posterior fossa/brainstem:
- Ataxia, abnormal eye movements
870
Brain tumour - Mx
= surgery, chemotherapy and radiotherapy and rehabilitation to:
- Remove tumour
- Prevent tumour recurrence
- Prevent further tumour growth
- Reverse deficits
- Minimise side effects
871
Neuroblastoma
= tumour arising from neural crest tissue in adrenal gland or SNS
=> 70% abdominal, 50% adrenal 20% thoracic
872
Neuroblastoma - Sx
- Abdominal mass:
=> usually adrenal gland, BUT could be anywhere along sympathetic chain from neck to pelvis
- Metastatic Sx
873
Neuroblastoma - Complications
Spinal cord compression (if paravertebral) – paraplegia, Horner’s syndrome
Adrenal tumour – HTN
Orbital disease – proptosis, “bruises” under eyes
874
Neuroblastoma - Ix
* FBC
* Urinalysis (increased catecholamines)
* USS, CT/MRI abdo
* Biopsy = diagnostic
* BM biopsy, MIBG scan – detect mets
875
Neuroblastoma - Mx
1. Non-metastatic = surgery
2. Metastatic = high-dose chemo + stem cell rescue
=> POOR PROGNOSIS
876
Wilm's Tumour
= nephroblastoma - a tumour of embryonal renal tissue.
877
Wilm's Tumour - Sx
Large abdominal mass – often only symptom
+/- abdo pain, anorexia, anaemia, haematuria, HTN
878
Wilm's Tumour - Ix
USS – intrarenal mass
+/- CT/MRI – assess for mets (esp. lungs)
879
Wilm's Tumour - Mx
Chemotherapy followed by nephrectomy
880
Soft tissue Sarcoma
= tumours of connective tissue (muscles, tendons, fat, nerves, etc.)
Rhabdomyosarcoma = most common => highly malignant cancer of skeletal muscle.
881
Soft tissue Sarcoma - Sx
depends on site:
Head and Neck – proptosis, nasal obstruction/bloody discharge
Genitourinary – dysuria, urinary obstruction, scrotal mass, bloody PV discharge
882
Soft tissue Sarcoma - Ix
Biopsy
| CT/MRI to assess metastases
883
Soft tissue Sarcoma - Mx
Chemotherapy + radiotherapy + surgery
884
What are the types of malignant bone tumour?
Osteosarcoma
| Ewing's Sarcoma
885
Osteosarcoma
- More common
- Usually affects hip, shoulder, knee
- Rapid growth and spread
886
Ewing's Sarcoma
- More common in younger children
| - Usually affects legs, back, pelvis, ribs, skull
887
Bone malignancy - Sx
* Persistent, localised bone pain
| * Mass on X-ray – often substantial soft tissue mass in Ewing’s
888
Bone malignancy - Ix
1. Bone X-ray
2. MRI & Bone scan
3. Chest CT – assess mets
4. BM sampling – exclude marrow involvement
889
Bone malignancy - Mx
= chemotherapy then surgery (avoid amputation where possible)
890
Retinoblastoma
= malignant tumour of retinal cells
Usually affects children <3 years
Caused by chromosome 13 mutation
=> All bilateral and 20% unilateral cases are INHERITED (autosomal dominant)
891
Retinoblastoma - Sx
- Strabismus
- Iris discolouration
- Leukocoria (white pupillary reflex instead of red)
- Vision changes
- Enlarged pupil
- Red/sore/swollen eye
892
Retinoblastoma - Ix
MRI and examination under anaesthetic
DO NOT BIOPSY as want to minimise damage to vision
893
Retinoblastoma - Mx
Chemo followed by laser treatment +/- radiotherapy
894
Gastro - HPC
Vomiting
- Colour?
- How much, how often?
- When?
- Projectile?
- Headaches? (early morning vomiting and headaches may indicate raised ICP)
Diarrhoea
- Appearance?
- Any blood/mucous?
- How many episodes?
```
Pain
Distension
Constipation
Jaundice
Swelling
```
895
Paeds Gastro - other relevant history
- Fever – how high? Pattern?
- Foreign travel
- Joint pain
- Skin rashes
- DHx
- Infectious contacts
- Allergies/intolerances
- Full systemic enquiry
896
Normal infant stool colour
Meconium – black and sticky
=> Important to ask when this was first passed
Day 3/4 – green
Day 4/5 – yellow (if breast-fed), peanut-butter (if formula fed)
897
Blood in stool
can be due to infection, injuries or allergies:
Normal stool tinged with red blood = often cow’s milk protein allergy.
Constipated stool with hint of red blood = tears of anus/ small haemorrhoids.
Diarrhoea mixed with red blood = infection
898
Causes of neonatal jaundice - unconjugated
<24 HOURS
Haemolysis
Infection
Haemorrhage
1 - 14 DAYS
Increased red cell turn over
Concentration Effect
Enzyme Deficiency
>2 WEEKS
Enzyme Deficiency
899
Causes of neonatal jaundice - conjugated
<24 HOURS
Thalassaemia (rare)
```
> 2 WEEKS
Hepatitis
Obstructive cause
Intrahepatic cholestasis
Bile transporter defect
Metabolic
```
900
What are the MOST COMMON causes of neonatal jaundice?
Rhesus disease,
ABO incompatibility,
Physiological jaundice,
Breast milk jaundice.
901
Physiological Jaundice
* Increased breakdown of red blood cells – HbF replaced by (adult) Haemoglobin
* Immature glucuronidation in Neonatal liver
902
Breast milk Jaundice
?due to fats in breast milk increasing enterohepatic circulation
Don’t stop the milk! (just affects duration, not severity)
903
Why and how is neonatal jaundice managed?
Unconjugated bilirubin can be neurotoxic
Treated with PHOTOTHERAPY
904
Phototherapy for neonatal jaundice
Wavelength 420nm to 480nm (i.e. BLUE light, not UV)
The light is absorbed by the skin and converts unconjugated bilirubin
This is done to reduce the ability of bilirubin to cross the BBB, thereby avoiding the neurotoxicity of high levels.
The baby will need eye protection and to have its temperature and hydration monitored
905
What is Biliary Atresia?
bile flow from the liver to the gallbladder is blocked
| => bile becomes trapped in the liver, causing damage and cirrhosis, and eventually failure.
906
How does biliary atresia present?
Symptoms appear ~2-8 weeks after birth.
- Jaundice
- Dark urine
- Pale stools
- Weight loss and irritability
907
Biliary Atresia - Ix
FBC, LFT, clotting
AXR – enlarged liver/spleen
Abdo USS – small/absent gall bladder or bile ducts.
Liver biopsy
Diagnostic surgery and operative cholangiogram (done at same time)
908
Biliary Atresia - Mx
= Kasai procedure (hepatoportoenterostomy)
Aim is to re-establish bile flow from the liver to the intestine.
Removal of damaged extra-hepatic ducts and attachment of a loop of intestine to the draining bile ducts of the liver.
If the Kasai procedure is unsuccessful, a liver transplant may be needed
909
Acute causes of liver disease in children
```
Viral Hepatitis
Poisoning (Alcohol, Paracetamol)
Wilson’s Disease
Tyrosinaemia (Probably not in this age group)
Autoimmune Hepatitis
```
910
Chronic causes of liver disease in children
```
Hep B, C
Autoimmune hepatitis
Drugs (non-steroidals)
Inflammatory Bowel Disease
Primary Sclerosing Cholangitis
Wilson’s
Alpha-1-antritrypsin
Cystic fibrosis
```
911
Wilson’s Disease
Rare recessive disorder involving copper metabolism
Presents with acute hepatitis, fulminant liver disease or cirrhosis
Copper in cornea – Kaiser-Fleischer Ring
912
Management of a child with severe malnutrition
Treat/prevent hypoglycaemia, hypothermia, dehydration
Correct electrolyte imbalance
Correct micronutrient deficiencies
Feeding
=> Begin feeding gradually over days 1-7
=> Then increase feeding to recover lost weight
913
Kwashiorkor malnutrition
= Severe protein deficiency but normal caloric intake
Oedema
Reduced s.c. fat
Fatty liver is common
Can have associated infections (loss of Ig)
Can be in combination with Marasmus
Worse prognosis
914
Marasmus malnutrition
Severe deficiency of all nutrients
Inadequate caloric intake
Fat and muscle wasting (absent s.c. fat)
Better prognosis
915
What is in the 6 in 1 vaccine?
```
Diphtheria,
Tetanus,
Pertussis,
Polio,
Haemophilus influenzae type b,
Hep B
```
916
When does the NIPE occur?
Within 72 hours post-birth
Again at 6-8 weeks (usually at GP)
917
What needs to be done if a baby is born breech / in breech position after 36 weeks gestation?
will need to have USS of their hips due to increased risk of developmental dysplasia of the hip.
918
What aspects of history are relevant to acquire before performing the NIPE?
Maternal/Birth Hx:
- Mum’s wellbeing after birth
- Pregnancy details – date/time and type of delivery/complications
- Breech presentation – do they need USS to r/o DDH?
- Risk factors for neonatal infection
- Abnormalities noted on antenatal scans
- Family history – first-degree relatives with hearing problems/hip dislocation/childhood heart problems/congenital cataracts/renal problems
Newborn Hx
- Feeding
- Wet nappies
- Passing of meconium
- Any parental concerns?
919
What is important to do before starting a NIPE?
Acquire relevant History
Check growth chart or measure weight
=> identify if small/normal/large for gestational age
920
parts to cover in NIPE
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General inspection
Head
Face
Upper limb
Chest (+neck)
Abdomen
Genitalia + anus
Lower limb
Back
Reflexes
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921
NIPE - general inspection
Colour:
- Jaundice
- Pallor
- Cyanosis
Posture and movement:
- Any gross abnormalities?
- Spontaneous movement of limbs
Birthmarks/bruising
TONE – floppy when picked up?
922
NIPE - head
Inspection
- SIZE – macrocephaly/microcephaly,
- SHAPE – sutures closely applied/ widely separated / normal.
Palpation of fontanelles
- Tense/bulging or Sunken or normal?
Measure head circumference
923
NIPE - face
Inspect face
- Facial features
- Asymmetry
- Trauma (bruising, lacerations)
- Patency of nasal passages
Eyes:
- Assess for red reflex
- Position and shape of eyes
- Subconjunctival haemorrhage,
- Discharge
Mouth:
- Cleft lip and palate,
- Suckling reflex, rooting reflex
- Tongue tie
Ears:
- Any asymmetry, skin tags, pits or the presence of accessory auricles.
924
NIPE - upper limb
Inspect neck:
- Normal length?
- Lumps
Brachial pulses (both arms)
Tone
- Move the baby’s arms
Inspect hands:
- Polydactyly
- Palmar creases
925
NIPE - chest
Inspection
- Asymmetry
- Obvious chest derformities
- Resp rate / signs of respiratory distress
Auscultate lungs
Auscultate heart sounds
926
NIPE - abdomen
Inspection
- Distension, hernias
- Umbilical cord – swelling, erythema, discharge
Palpation for organomegaly
- Liver, spleen, kidneys, bladder
927
NIPE - genitalia
Inspect
- Any ambiguity of genitalia?
MALE:
- Position of urethral meatus
- Palpate scrotum to identify if both testes are present
- Any swelling?
FEMALE:
- Inspect labia, clitoris, discharge
Inspect for patent anus
- Ask if meconium within 24 hours
928
NIPE - lower limbs
FEMORAL PULSES
Inspect for abnormalities:
- Asymmetry (esp. leg lengths)
- Oedema
- Digits (extra/missing) and ankle deformities
Assess tone
929
NIPE - hips
REMOVE NAPPY AND WARN PARENTS
Barlow’s Test
Ortolani Test
930
NIPE - back
Inspect for NTDs / asymmetry
Palpation of spine
931
NIPE - reflexes
Palmar grasp
(Sucking and rooting – done in mouth inspection)
Moro reflex
932
How to perform the Moro Reflex?
What might cause an abnormal moro reflex?
Support the infant’s upper back with one hand, then drop back once or twice into your other hand.
A normal Moro reflex involves the extension of the legs and head whilst the arms jerk upwards with the fingers extended. The arms are then brought together and the hands clench into fists, and the infant cries.
Asymmetry may be due to hemiparesis, brachial plexus injury or a fractured clavicle.
933
Mode of imaging for diagnosis of DDH
<4.5 months = USS
| >4.5 months = X-ray
934
features of fragile X syndrome
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Learning difficulties
Macrocephaly
Long face
Large ears
Macro-orchidism
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935
What murmur is commonly associated with Turner's Syndrome ?
Ejection systolic
| due to bicuspid aortic valve, associated with Turner's
936
Therapeutic Cooling
Involves cooling the neonate to around 33.5 - 34.5ºC for 72 hours within a six hour window of a hypoxia inducing event/birth.
Lowering core body temperature will slow the metabolic rate and allow cells more time to recover from the hypoxic insult.
937
When is the newborn heel prick test performed?
What does it test for?
~ 5 days
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CF
PKU
Sickle cell disease
Congenital hypothyroidism
Medium-chain acyl-Co-A dehydrogenase deficiency
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