Paediatrics Flashcards
1
Q
When is the 6 in 1 vaccine given?
A
2, 3 and 4 months
2
Q
When is the pneumococcus vaccine given?
A
2, 4 and 12 months
3
Q
When is the Men B vaccine given?
A
2, 4 and 12 months
4
Q
When is the rotavirus vaccine given?
A
2 and 3 months
5
Q
When is the Hib/MenC booster given?
A
1 year
6
Q
When is the MMR given?
A
1 year and 3 years 4 months
7
Q
When is the HPV vaccine given?
A
12-13 years
8
Q
When is the Men ACWY vaccine given?
A
14 years
New university students aged 19-25
9
Q
Outline the management of DDH.
A
If < 2 months, observation and serial examination and ultrasound is recommended (every months)
If it persists/worsens, hip abduction orthosis (splint) or Pavlik harness are recommended (serial follow-up and plain X-ray at 6 months)
10
Q
Outline how hearing is tested in the neonate.
A
1st: evoked otoacoustic emission (EOEA) testing
If this is abnormal –> automated auditory brain stem (AABR) audiometry
11
Q
Briefly outline the steps in the management of necrotising enterocolitis.
A
Stop oral feeding
Broad spectrum antibiotics (ceftriaxone and vancomycin)
Surgery if perforation/necrosis
TPN
12
Q
What can be used to close a PDA?
A
IV indomethacin
Prostacyclin synthetase inhibitor
Ibuprofen
13
Q
How is the bilirubin concentration measured in neonatal jaundice?
A
If < 24 hours or < 35 weeks gestation = serum bilirubin
If > 24 hours or > 35 weeks gestation = transcutaneous bilirubin (if this is > 250 µmol/L - check serum bilirubin)
14
Q
What serum bilirubin levels suggests increased risk of developing kernicterus?
A
> 340 µmol/L in babies > 37 weeks
or rising rapidly > 8.5 µmol/L/hr
15
Q
How often should serum bilirubin be measured in a neonate with jaundice?
A
Every 6 hours until it drops below the treatment threshold
16
Q
Which investigations should be performed in a neonate who developed jaundice within 24 hours of birth?
A
Haematocrit Blood group of mother and baby DAT test FBC and blood film Blood G6PD level Blood/urine/CSF culture
17
Q
Which antibiotics are used to treat meconium aspiration?
A
IV ampicillin and gentamicin
18
Q
Which antibiotics may be used in the treatment of early-onset sepsis?
A
Benzylpenicillin and gentamicin
19
Q
Which organism most commonly causes late-onset sepsis?
A
Coagulase-negative staphylococcus (e.g. Staphylococcus epidermidis)
20
Q
How is neonatal meningitis treated?
A
3rd generation cephalosporin + amoxicillin/ampicillin
21
Q
What is the paediatric sepsis 6?
A
- Supplemental oxygen
- Gain IV or IO access and order blood cultures, blood glucose and arterial/capillary/venous gasses
- IV/IO broad-spectrum antibiotics
- IV fluids (be cautious about fluid overload)
- Experienced senior clinicians should be involved early
- Vasoactive inotropic support (e.g. adrenaline) should be considered early
a. Considered if normal physiological parameters are NOT achieved after > 40 ml/kg of fluid resuscitation
22
Q
How should neonatal conjunctivitis be treated?
A
Discharge and redness (staph or strep) - topical ointment (e.g. neomycin)
Gonococcus - 3rd generation cephalosporin
Chlamydia - erythromycin (2 weeks)
23
Q
What should babies at risk of vertical hepatitis B transmission receive?
A
Hepatitis B immunoglobulin AND Hep B vaccine
24
Q
How is gastro-oesophageal reflux in a breastfed infant treated?
A
1st line: Breastfeeding assessment
2nd line: trial of alginate therapy for 1-2 weeks
25
How is gastro-oesophageal reflux in a formula fed infant treated?
1st line: review feeding history (check for overfeeding)
2nd line: offer trial of smaller more frequent feeds
3rd line: offer trial of thickened formula
4th line: offer trial of alginate therapy
26
If conservative measures to treat GORD in an infant fail, what should you do?
Consider a 4-week trial of a PPI or histamine antagonist
27
State an example of an antibiotic regimen that may be used to eliminate H. pylori.
Amoxicillin + metronidazole/clarithromycin
| This is given as a 7-day triple therapy with a PPI
28
How are the maintenance fluid values for children calculated?
0-10 kg = 100 ml/kg/day
10-20 kg = 1000 mL + 50 ml/kg/day
20+ kg = 1500 mL + 20 ml/kg/day
29
How is the amount of fluid required when giving a bolus to a child calculated?
20 ml/kg of NaCl in < 10 mins
NOTE: use 10 mL/kg if DKA, trauma, fluid overload or heart failure
30
What precaution must be taken when rehydrating a child with hypernatraemic dehydration?
Replace fluid deficit over 48 hours and measure plasma sodium regularly
Rapid reduction in plasma sodium can lead to seizures and cerebral oedema
31
What should be monitored in children with Coeliac disease?
```
Annual review
Weight, height and BMI
Review symptoms
Review diet and adherence
Consider blood tests
```
32
What are the management options in a UC patient with:
Mild proctitis
Mild left-sided and extensive disease
Maintainin remission
Mild proctitis
- Oral/topical aminosalicylates
Mild left-sided and extensive disease
- Oral aminosalicylates (consider topical aminosalicylate or oral steroid)
Maintaining remission
- Aminosalicylates
- Consider oral azathioprine or mercaptopurine
NOTE: if aminosalicylates are ineffective after 4 weeks, consider adding oral prednisolone (if this is ineffective, consider oral tacrolimus)
33
How is severe fulminating disease in UC managed?
IV corticosteroids (induce remission)
Consider IV ciclosporin
Surgery - colectomy with ileostomy or IJ pouch
34
What is a major risk of UC and how are patient's monitored for it?
UC is associated with bowel cancer
| Regular colonoscopic screening performed after 10 years of diagnosis
35
How is constipation with faecal impaction treated?
1 - DISIMPACTION REGIME
Movicol Paediatric Plain
If not effective - senna
If not tolerated - senna + lactulose
2 - MAINTENANCE LAXATIVES
Movicol with or without senna (carry on for several months and titrate dose based on stools )
3 - BEHAVIOURAL METHODS (e.g. star charts)
36
How are anal fissures in children treated?
Ensure ease of passing stool (consider constipation treatment, advise increase in fluid intake and dietary fibre)
Offer simple analgesia
Advise sitting in a shallow, warm bath to reduce discomfort
Adult treatments (topical diltiazem or GTN)
37
How is threadworm infection treated?
If > 6 months: single dose mebendazole for child and all household contacts and hygiene measures (for 2 weeks)
If < 6 months: 6 weeks of hygiene measures
38
Which antibiotic is used in the management of bacterial meningitis in hospital?
IV ceftriaxone
N. meningitidis - 7 days
H. influenzae - 10 days
S. pneumoniae - 14 days
39
Which antibiotics might you use in a patient with bacterial meningitis who has a severe beta-lactam allergy?
Vancomycin and moxifloxacin
40
How should a patient with bacterial meningitis be followed-up?
Discuss potential long-term effects and pattern of recovery (e.g. hearing problems)
Offer formal audiological assessment
Consider testing for complement deficiency if more than one episode of meningococcal septicaemia
41
How is HSV encephalitis treated?
High-dose IV aciclovir for 3 weeks
42
How is toxic shock syndrome managed?
```
ICU
Surgical debridement of infected tissue
Clindamycin (stops toxin production)
Vancomycin or meropenem
IVIG (neutralise the toxin)
```
43
How is impetigo treated?
Localised infection = topical fusidic acid (3-4/day for 7 days)
Extensive Infection = oral flucloxacillin (QDS for 7 days)
Clarithromycin if penicillin allergy
44
How is periorbital cellulitis treated?
High-dose IV ceftriaxone
45
When is a child with chickenpox considered infectious?
Most infectious 1-2 days before the rash
| Infectious until all the lesions have crusted over (usually 5 days after onset)
46
Which groups of patients should children with chickenpox avoid?
Pregnant women
People who are immunocompromised
Infants < 4 weeks old
NOTE: avoid school until lesions have crusted over
47
What must young people with EBV avoid doing?
Contact sports
48
Which medications are occasionally used to treat CMV infection?
IV ganciclovir
Oral valganciclovir
Foscarnet
49
How long should a child stay away from school for after measles infection?
4 days after rash onset
50
How long should a child stay away from school for after mumps infection?
5 days after the development of parotitis
51
How are mumps and rubella diagnosed?
Oral fluid sample
52
How is Kawasaki disease managed?
High-dose aspirin (7.5-12.5 mg/kg QDS for 2 weeks or until afebrile, then 2-5 mg/kg once daily for 6-8 weeks)
IVIG (2 g/kg daily for 1 dose)
Echocardiogram (check for coronary artery aneurysms)
53
What steps can be taken to reduce the risk of vertical transmission of HIV?
Intrapartum zidovudine infusion
Elective C-section (if high viral load)
Zidovudine treatment for neonate (up to 6 weeks)
Avoidance of breastfeeding
54
Outline the management of food allergy.
Avoidance
Provide an allergy action plan for managing an allergic attack
Mild reactions - non-sedating antihistamine (e.g. fexofenadine)
Severe reactions - provide an EpiPen
55
How is Cow's milk protein allergy managed?
Breastfed - advise mother to exclude dairy from her diet (consider prescribing vitamin D and calcium supplements)
Formula-fed - use extensively hydrolysed formula
Trial for at least 6 months, and consider gradually reintroducing dairy following a milk ladder under medical supervision
56
Which tests can you do to further investigate suspected cow's milk protein allergy?
Skin prick testing
| Specific IgE
57
What PRN treatment may be appropriate for patients with allergic rhinitis?
Aged 2-5: oral antihistamine
| Everyone else: intranasal azelastine
58
What preventative treatment may be used in patients with allergic rhinitis?
if main issue is nasal blockage or polyps - intranasal corticosteroid (e.g. beclometasone)
If main issue is sneezing/nasal discharge - oral antihistamine or intranasal corticosteroid
59
How would you treat urticaria?
Identify and manage triggers
Oral antihistamine for up to 6 weeks
Severe - oral corticosteroid
Refractory - IgE antibody or LTRA
60
How is bacterial tonsillitis treated?
```
Penicillin V (10 days)
Allergy: clarithromycin
```
61
Which medication should be avoided in tonsillitis?
Amoxicillin
| Causes a widespread maculopapular rash in infectious mononucleosis
62
How is scarlet fever treated?
Penicillin V QDS for 10 days
| Allergy: azithromycin, clarithromycin
63
How long should patients with strep throat/scarlet fever stay away from school?
24 hours after starting antibiotics
64
What is the first-line medical management for acute otitis media?
Amoxicillin 5-7 days
65
How should sinusitis be managed?
< 10 days: reassure that it is usually viral and self-resolving
> 10 days: high-dose intranasal steroids (if > 12 years)
Consider back-up antibiotic prescription if not improved by 7 days (pen V)
66
Which severity of croup requires admission?
Anything worse than mild
I.e. anything worse than a barking cough on its own
67
How is croup treated?
0.15 mg/kg dexamethasone stat
This can be repeated after 12 hours if necessary
68
How is severe croup treated in an emergency?
High-flow oxygen
| Nebulised adrenaline
69
How is acute epiglottitis managed?
```
Urgent hospital admission (ICU)
Secure airway and supplemental oxygen
Take blood culture
IV cefuroxime
Rifampicin prophylaxis for entire household
```
70
How is bronchiolitis treated?
Conservative
Supplemental oxygen if < 92%
Nasogastric/orogastric tube feeding if poor intake
Consider nebulised 3% saline
71
What are the first and second line treatment options for viral-induced wheeze?
1st line: SABA (up to 10 puffs every 4 hours)
| 2nd line: Intermittent LTRA or ICS
72
How is multiple trigger wheeze treated?
ICS or LTRA for 4-8 weeks
73
Outline the management steps for asthma in someone < 5 years.
1) SABA
2) 8-week trial of moderate-dose ICS
After 8 weeks:
- If symptoms resolve but recur < 4 weeks = restart low-dose ICS
- If symptoms resolve but recur > 4 weeks = repeat 8-week trial of moderate-dose
3) Add LTRA
4) Refer to specialist
74
Outline the management steps for asthma in someone > 5 years.
1) SABA
2) Low-dose ICS
3) Add LTRA (review in 4-8 weeks)
4) Stop LTRA, add LABA
5) Change to MART
6) Increase ICS to moderate-dose
7) Refer to specialist
75
List some non-pharmacological aspects of asthma management.
Assess impact on life
Provide personalised asthma action plan (Asthma UK)
Advise about trigger avoidance
Ensure clear explanation of peak flow and inhaler technique
76
Which investigations would you request in a patient having an asthma attack?
Obs (HR and RR are particularly important)
PEFR
SaO2
VBG/ABG
Examine for signs of increased respiratory effort
77
Outline the management of an acute asthma attack.
Supplemental oxygen
Nebulised SABA
If ineffective, add nebulised ipratropium bromide
Monitor PEFR and SaO2
NOTE: if mild-to-moderate, SABA can be given through a large volume spacer
78
Which medication should a patient be given to take home after an acute asthma attack?
Oral prednisolone (3-7 days)
79
When should a patient with an asthma attack treated in hospital be followed-up?
Within 2 working days of discharge
80
How is foreign body inhalation treated in a conscious patient?
ABCDE
Encourage coughing
Back blows
Heimlich manoeuvre (NOT in very young children)
Remove object (rigid/flexible bronchoscopy)
81
How is foreign body inhalation treated in an unconscious patient?
ABCDE
Secure the airway
Remove the foreign body (rigid/flexible bronchoscopy)
82
Which patients with whooping cough should be admitted?
< 6 months
| Significant breathing difficulties
83
Outline the pharmacological treatment of whooping cough.
< 21 days after onset of cough: macrolide
(clarithromycin/azithromycin)
NOTE: use erythromycin in pregnant women
84
How is pneumonia in children treated?
1st line: amoxicillin 7-14 days
2nd line: add macrolide
ALL children with a clinical diagnosis of pneumonia should be treated with antibiotics
85
What are some treatment approaches for bronchiectasis?
Airway clearance techniques (physiotherapy)
Inhaled bronchodilator
Inhaled hypertonic saline
Antibiotic prophylaxis (e.g. azithromycin)
86
What are the aspects of managing the respiratory issues in cystic fibrosis?
Pulmonary monitoring (every 2 months in children, every 3 months in adults)
Airway clearance techniques (physiotherapy)
Mucoactive agents
87
What is the first-line mucoactive agent for cystic fibrosis?
rhDNAse
2nd line: add hypertonic saline
Alternative: mannitol dry powder inhalation
88
What are the management approaches to the infection risk associated with cystic fibrosis?
Continuous prophylactic antibiotics (flucloxacillin and macrolides)
Prompt and vigorous IV therapy for infections
End-stage disease: bilateral lung transplantation
89
What are the management approaches to the nutritional problems in CF?
Oral enteric-coated pancreatic replacement therapy
High calorie diet
Fat-soluble vitamin supplements
90
What are the main domains of management in cystic fibrosis?
Pulmonary management (regular chest physiotherapy)
Infection management
Nutritional management (high calorie and high fat, vitamin supplementation, enzymes)
Psychological management
91
What is a treatment option for severe sleep disordered breathing in a child?
Adenotonsillectomy
92
What is some general conservative advice given to parents of an infant with a nappy rash?
Use high absorbency nappy
Leave nappy off as much as possible to help the skin dry
Clean the skin/change the nappy every 3-4 hours and ASAP after soiling/wetting
Bath the child gently
Use barrier protection (e.g. sudocrem)
93
How should an inflamed nappy rash that is causing discomfort be treated?
Hydrocortisone 1% cream OD (max 7 days)
94
How should a nappy rash caused by candida be treated?
Do NOT use barrier protection
| Prescribe topical imidazole (e.g clotrimazole)
95
How should a nappy rash caused by bacterial infection be treated?
Oral flucloxacillin for 7 days
96
What is the first-line treatment of seborrhoeic dermatitis?
Regular washing of the scalp with baby oils and baby shampoo (gently brush to remove the scales)
97
What treatments for seborrhoeic dermatitis could be used if conservative measures fail?
Topical imidazole cream
| Hydrocortisone cream
98
What advice would you give a patient regarding emollient use for eczema?
Use in large amounts and often
Apply on the whole body
Use as a soap substitute
99
What advice would you give regarding how to apply topical steroids for eczema?
Use once or twice daily and only apply to areas of active eczema
100
Give an example of a mild, moderate and potent topical steroid used for eczema.
Mild - hydrocortisone 1%
Moderate - betamethasone valerate 0.025% or clobetasone butyrate 0.05%
Potent - betamethasone valerate 0.1%
101
Which treatment would be recommended for children > 2 years with eczema that has failed to respond to topical steroids?
Topical calcineurin inhibitors (e.g. pimecrolimus)
102
Under which circumstances do bandages tend to be used in eczema?
For areas of chronically lichenified skin
103
When are antihistamines used in eczema?
1 month trial of non-sedating antihistamine (e.g. fexofenadine) if severe itching or urticaria
1-2 week trial of sedating antihistamine (e.g. promethazine) if flare is disturbing sleep
104
How should infected eczema be treated?
Swab the affected area
Advice on good hygiene when using emollients
Flucloxacillin
105
How is eczema herpeticum managed?
Refer for same-day dermatology advice
Oral aciclovir
Consider ophthalmological review if around the eyes
106
How are viral warts treated?
Daily administration of salicylic acid, lactic acid paint or glutaraldehyde lotion
Cryotherapy
107
How is molluscum contagiosum managed?
Spontaneous resolution by 18 months
Avoid squeezing lesions
Avoid sharing towels/clothes
108
What is the first-line treatment option for mild ringworm?
Topical antifungals (terbinafine cream)
NOTE: hydrocortisone 1% may be added if there is extensive inflammation
109
How are more severe ringworm infections managed?
Oral antifungals
1st line: terbinafine
2nd line: itraconazole
110
What is the first-line management option for tinea capitis?
Oral griseofulvin (or oral terbinafine)
NOTE: any animal source of the infection would also need treatment
111
What is the first line treatment option for scabies?
Topical permethrin 5% cream
Apply on the whole body (chin downwards) and was off after 8-12 hours
Second application is required 1 week later
2nd line: malathion aqueous 0.5%
112
What advice should be given to patients with scabies?
Members of the household and close contacts should be treated
Bedding and clothes should be washed at high temperature
Treat post-scabeitic itch with crotamiton 10% cream
Nighttime sedative anti-histamine may be useful to help sleep
113
How should head lice be treated?
Wet combing with a fine-tooth comp every 3-4 days for 2 weeks
Dimeticone 4% lotion
Alternative: malathione 0.5% lotion
114
List some agents that are used in the treatment of guttate psoriasis.
Coal tar preparations
Dithranol
Calcipotriol
115
What are the treatment options for mild-to-moderate acne?
Benzoyl peroxide
Duac (benzol peroxide + clindamycin)
Adapalene (topical retinoid - CI in pregnancy and breastfeeding)
Azelaic acid
116
Outline the treatment options for moderate acne.
Consider oral antibiotics (lymecycline or doxycycline) for a maximum of 3 months
Change to alternative antibiotic after 3 months if no improvement
NOTE: topical benzoyl peroxide or retinoid should be co-prescribed to reduce the risk of antibiotic resistance
117
What can be used as an alternative to oral antibiotics in girls with acne?
COCP
NOTE: POPs and progestin implants can worsen acne
118
When might you consider dermatology referral for a patient with acne?
If not responding to 2 courses of antibiotics or if there is scarring, refer to dermatology for consideration of isotretinoin
119
When should a patient undergoing treatment for acne be reviewed?
At 8-12 weeks
120
How is heart failure in an infant managed?
```
Diuretics such as frusemide (reduce preload
Enhance contractility (e.g. dopamine)
Reduce afterload (e.g. ACEi)
Improve oxygen delivery (beta-blockers)
```
121
How are ASDs managed?
Secundum - percutaneous closure (cardiac catheterisation with insertion of an occlusive device)
Partial AVSD - surgical correction
122
When are symptomatic ASDs usually treated?
3-5 years
123
When do large VSDs and AVSD tend to be treated surgically?
3-6 months
124
How can a PDA be closed?
Medical: indomethacin (or other NSAID)
Surgical: cardiac catheterisation and coil/occlusive device insertion
NOTE: surgical management usually happens at around 1 year
125
How should a cyanosed neonate presenting within the 1st week of life be managed?
Stabilise the airway, breathing and circulation
Artificial ventilation if necessary
Start prostaglandin infusion
Surgery
126
What murmur is associated with ASD?
Ejection systolic murmur best heard at the upper left sternal edge and fixed wide split second heart sound
127
What murmur is associated with VSD?
Loud pansystolic murmur at the lower left sternal edge, quiet pulmonary second heart sound
128
Which defects require surgical correction in tetralogy of Fallot?
Close the VSD
| Relive the right ventricular outflow obstruction
129
How may hypercyanotic spells in tetralogy of Fallot be treated?
Sedation and pain relief
IV propranolol
IV fluids
130
Which life-saving procedure may be performed for patients with transposition of the great arteries to enhance mixing of the blood?
Balloon atrial septostomy
131
How is tricuspid atresia treated?
Blalock-Taussig shunt
132
How is aortic stenosis treated?
Balloon valvulotomy
Aortic valve replacement
NOTE: same for pulmonary stenosis
133
How is SVT managed?
1 - vagal manoeuvres
2 - IV adenosine (DC cardioversion if this fails)
3 - maintenance therapy with fleicainide or sotalol
90% of children have no further attacks
134
How is acute rheumatic fever treated?
Bed rest and anti-inflammatory agents (e.g. aspirin)
| Penicillin V if evidence of persistent infection
135
What is the most effective prophylaxis for rheumatic fever?
Monthly injections of benzathine penicillin
Alternative: oral penicillin OD
NOTE: prophylaxis recommended for 10 years after last episode of rheumatic fever or until 21 years old
136
How is infective endocarditis treated?
Beta-lactam and gentamicin
Usually for 6 weeks
137
How would you treat an umbilical granuloma?
Regular application of salt to the wound
| Cauterise with silver nitrate
138
List some contraindications for MMR.
Severe immunosuppression (high dose steroids leave you immunocompromised for 3 months)
Allergy to neomycin
Received another live vaccine by injection within 4 weeks
Pregnancy should be avoided for at least 1 month afterwards
IG therapy within the past 3 months
139
How should children < 3 months with a UTI be managed?
```
Admit to hospital immediately
IV antibiotics (e.g. amoxicillin) for at least 5-7 days
```
140
Which clinical features are suggestive of an upper UTI?
Bacteriuria + fever
| Bacteriuria + loin pain
141
How should an upper UTI be treated?
```
Oral antibiotics (e.g. trimethoprim for 7 days)
If this cannot be used, give IV antibiotics (e.g. coamoxiclav) for 2-4 days and discharge with oral antibiotics
```
142
How should simple cystitis be treated?
Oral antibiotics (e.g. trimethoprim) for 3 days
143
Which children should have an ultrasound after a UTI?
Children who have had an atypical UTI
| Children < 6 months
144
Which children should have a DMSA and MCUG after a UTI?
< 6 months old presenting with atypical or recurrent UTI
145
How should enuresis in < 5 year olds be managed?
Reassure that this usually resolves without investigation
Ensure easy access to the toilet at night
Encourage bladder emptying before bed
146
How should enuresis in > 5 year olds be managed?
If infrequent (< 2 weeks) reassure and watch-and-wait
1st line if < 7: enuresis alarm and positive reward system
2nd line: desmopressin
Desmopressin may be used first line if rapid short-term control is necessary, or if > 7 years old
147
List some causes of secondary enuresis.
UTI
Constipation
Diabetes
Psychological/Family problems
148
How is nephrotic syndrome treated?
Oral prednisolone for 4 weeks
Wean and stop after 4 weeks
If the child does not respond or has atypical features, consider renal biopsy
149
List some complications of nephrotic syndrome.
Hypovolaemia
Thrombosis
Infection
Hypercholesterolaemia
150
How is Henoch-Schonlein purpura managed?
Most resolve spontaneously within 4 weeks
Joint pain can be managed with paracetamol/ibuprofen
IV corticosteroids are recommended for nephrotic-range proteinuria or declining renal function
Oral prednisolone may be given for severe scrotal oedema or abdominal pain
151
How are urinary tract calculi managed?
Conservative - fluids, analgesia, antiemetics
Bacterial infection - co-trimoxazole/nitrofurantoin or surgical decompression
Small stones - tamsulosin
Large stones - ESWL or uteroscopy
152
What are the most common causes of AKI in children?
HUS
| ATN
153
How is haemolytic uraemic syndrome managed?
```
Admit to hospital
Monitor urine output and fluid balance
Maintain adequate hydration status
Monitor BP (treat with CCB if necessary)
Some will need dialysis
```
154
What long-term follow-up should be offered to patients with HUS?
Check for persistent proteinuria, the development of hypertension and progressive CKD
155
Outline the aspects of managing CKD in a child.
Diet - calorie supplements often necessary
Prevention of renal osteodystrophy - phosphate restriction, calcium and vitamin D supplements
Control of salt and water balance
Anaemia - recombinant EPO
Hormonal - human GH for GH resistance
156
How does CKD affect the growth of a child?
Delayed puberty
| Subnormal pubertal growth spurt
157
How are hydroceles in children managed?
< 2 years = most resolve spontaneously
2-11 year = open/laparoscopic repair
11-18 years = conservative or surgical
158
How should unilateral undescended testicles be managed?
Undescended at birth --> review at 6-8 weeks
Undescended at 6-8 weeks --> review at 3 months
Undescended at 3 months --> seen by urologist by 6 months
NOTE: if descended but retractile at 3 months, advise annual follow up due to risk of ascending testes through childhood
159
How should bilateral undescended testicles at birth be managed?
Urgent referral to a senior paediatrician within 24 hours (genetic or endocrine testing may be necessary)
160
How is testicular torsion managed?
Urgent exploratory surgery (with orchidopexy/orchidectomy)
161
How is torsion of the appendix testis managed?
Exploratory surgery is often performed because it may be difficult to distinguish from testicular torsion
Otherwise conservative
162
How are hypospadias managed?
May not require treatment
May require surgery (from 3 months) for cosmetic/functional purposes
IMPORTANT: do NOT circumcise any child with a hypospadia
163
How are labial adhesions treated?
Topical steroids or oestrogens
164
When should phototherapy for neonatal jaundice be stopped?
Once the serum bilirubin is at least 50 µmol below the treatment threshold
Patients should be given folic acid supplements
165
Why should you check the serum bilirubin level 12-18 hours after stopping phototherapy?
Check for rebound hyperbilirubinaemia
166
When is IVIG used in neonatal jaundice?
Used alongside intensified phototherapy in cases of rhesus or ABO haemolytic disease where the serum continues to rise by > 8.5 µmol/L per hour
167
How is biliary atresia managed?
Kasai procedure
Complications can be managed using ursodeoxycholic acid, fat-soluble vitamins and prophylactic antibiotics
168
How is alpha-1 antitrypsin deficiency managed?
Advise against smoking and drinking
Pulmonary manifestations are managed like COPD
Liver manifestations are managed similar to other liver diseases (e.g. monitoring for coagulopathy, diuretics for ascites)
169
Which investigations are used for galactosaemia?
Galactose in urine
| Measuring red cell Gal-1-PUT
170
How is galactosaemia treated?
Galactose-free diet
171
How is Hep A managed?
Close contacts should be vaccinated within 2 weeks of onset of illness
Unvaccinated patients with recent exposure should receive IVIG or the hepatitis A vaccine
172
How is autoimmune hepatitis managed?
Prenisolone and azathioprine
Ursodeoxycholic acid can help in PSC
Liver transplant in severe cases
173
How is hepatic encephalopathy treated?
Supportive
Identify and correct precipitating factors (e.g. GI bleed)
Reduce nitrogenous load (dietary protein restriction, lactulose and rifaximin)
174
How long do patients with ALL tend to have chemotherapy for?
Girls - 2 years
| Boys - 3 years
175
What is the cure rate for lymphoma?
80%
176
How is neuroblastoma treated?
Localised primaries can be cured by surgery alone
| Metastatic disease will require chemotherapy (it may also require stem cell transplantation and radiotherapy)
177
What percentage of patients with Wilm's tumour are cured?
80%
178
How is retinoblastoma treated?
Chemotherapy to shrink tumours
Laser treatment of the retina
Radiotherapy maybe used in more advanced disease
NOTE: 90% cure rate but many will be visually impaired
179
Which antibiotics are used for suspected bacterial meningitis in children?
< 3 months = IV cefotaxime + amoxicillin
> 3 months = IV cefotaxime
If > 1 month and caused by H. influenzae, give dexamethasone
180
What is the definitive management for slipped upper femoral epiphysis?
Internal fixation across the growth plate
181
Which type of fluid should be given for maintenance requirements in children?
0.9% NaCl + 5% dextrose
182
How are dehydration corrections calculated when administering fluids?
```
Usually given over 24 hours
Give maintenance + % dehydration
Weigh the child if possible (1 kg loss = 1000 mL)
Estimate clinically (e.g. each kg = 1000 mL, so 3% weight loss in 20 kg child = 3 x 200 mL = 600 mL)
```
183
How should iron deficiency anaemia be treated?
Oral ferrous sulphate 200 mg tablets (2-3/day)
| Continue for 3 months after iron deficiency has corrected
184
What advice would you give to someone who is taking iron tablets for iron deficiency anaemia about side-effects?
May experience adverse effects (constipation, diarrhoea, faecal impaction)
Discomfort could be minimised by taking the iron supplement with food
185
How should treatment for iron deficiency anaemia be monitored?
Re-check Hb after 2-4 weeks (expect 20 g/L rise)
| FBC every 3 months for 1 year
186
What are the aspects of treating a neonate with hereditary spherocytosis?
Supportive (maybe blood transfusion)
Folic acid supplementation
Phototherapy/exchange transfusion
187
Outline the aspects of treating hereditary spherocytosis in older children and adults.
Supportive (maybe blood transfusion)
Folic acid supplementation
Splenectomy and vaccination regimen for encapsulated bacteria
Cholecystectomy for gallstones
188
How can complications of sickle cell disease be prevented?
Immunisation against encapsulated organisms
Daily oral penicillin
Daily folic acid
Minimise exposure to cold, dehydration, excessive exercise and hypoxia
189
Outline the treatment of acute sickle cell crises.
```
Oral and IV analgesia
Good hydration
Antibiotics if necessary
Oxygen
Exchange transfusion (for acute chest syndrome, priapism and stroke)
```
190
Outline the steps in the analgesic ladder.
Step 1: paracetamol
Step 2: cocodamol or NSAIDs (weak opioid)
Step 3: morphine, oxycodone
191
Which drug treatment can be used to reduce sickling in sickle cell patients?
Hydroxycarbamide
192
How is haemophilia treated?
Factor 8 concentrate (A)
Factor 9 concentrate (B)
Acute bleed - factor concentrates and anti-fibrinolytics
193
Which medications should be avoided in patients with haemophilia and von Willebrand disease?
IM injections
Aspirin
NSAIDs
194
Which treatment can be used for mild haemophilia A?
Desmopressin (stimulates endogenous release of vWF)
195
What medical treatment can be used for type 1 von Willebrand disease?
Desmopressin
NOTE: risk of hyponatraemia
More severe disease is treated with factor 8 concentrate
196
Describe the usual natural course of ITP.
In 80% it is an acute, benign and self-limiting disease
Resolves spontaneously within 6-8 weeks
Mild/asymptomatic disease can be managed with observation alone
197
How can severe ITP be managed?
IVIG + corticosteroid + platelet transfusions
| Antifibrinolytics (e.g. tranexamic acid) may be used
198
How is DIC treated?
Treat underlying cause
Supportive care
Replacement therapy (platelets and clotting factors)
Restoration of physiological coagulation pathways (e.g. using heparins)
199
How is acute osteomyelitis managed?
High-dose IV empirical antibiotics (for 2-4 weeks)
Switch to oral once clinically recovered (continue for 6 weeks)
Immobilise the affected area
Surgical debridement may be necessary
Possible antibiotics used include flucloxacillin and penicillin
200
How would you counsel a patient with Osgood-Schlatter disease?
```
Stop or reduce sporting activity
Analgesia - paracetamol and NSAIDs
Intermittent ice packs
Protective knee pads
Stretching
```
201
How is chondromalacia patellae managed?
Physiotherapy for quadriceps strengthening
202
How is osteochondritis dissecans managed?
Pain relief
Rest and quadriceps exercise
Occasionally need surgery
203
How is subluxation of the patella treated?
Reduction and immobilisation
| Rehab
204
Outline the treatment of Perthes disease.
o Acute Pain - supporting care with simple analgesia
o < 5 years
• Mobilisation and monitoring (healing potential is good at this age)
• Non-surgical containment using splints
o 5-7 years
• Mobilisation and monitoring
• Surgical containment
o 7-12 years
• Surgical containment
• Salvage procedure (remodel the acetabulum)
o 12+ years
• Salvage procedure
• Replacement arthroplasty
205
How is septic arthritis treated?
Antibiotics (initially IV for 2 weeks, followed by 4 weeks oral)
Suspected Gram-positive
Vancomycin + joint aspiration
2nd line = clindamycin or cephalosporin + joint aspiration
Suspected Gram-negative
3rd generation cephalosporin (e.g. ceftriaxone) + joint aspiration
2nd line = IV ciprofloxacin + joint aspiration
Affected joints should be aspirated to dryness as often as required (through closed needle aspiration or arthroscopically)
206
What are the aspects of managing juvenile idiopathic arthritis?
```
Multidisciplinary team
Physiotherapist and occupational therapist
NSAIDs
Corticosteroids
DMARDs (methotrexate is first-line)
```
207
How is vitamin D deficiency/Rickets treated?
Calcium and ergocalciferol
OR cholecalciferol
NOTE: pseudovitamin D deficiency is treated with alfacalcidol or calcitriol
208
How might you investigate a patient presenting with migraines?
Headache diary for 8 weeks to help identify triggers
209
Outline the steps in the acute management of a migraine.
Step 1: simple analgesia
Step 2: nasal sumatriptan
Step 3: nasal sumatriptan and NSAID/paracetamol
Step 4: consider adding prochlorperazine or metoclopramide
210
Which medications are used to prevent migrianes?
Topiramate and propranolol
211
How should you manage a febrile convulsion during the seizure?
Protect them from injury
Do not restrain
When the seizure stops, check the airway and place in the recovery position
If > 5 mins --> rectal diazepam (can be done twice) or buccal midazolam (only one dose)
An ambulance should be called if the seizure continues
212
What should be tested in all children who have a seizure?
Blood glucose
213
Which children who have had a febrile convulsion require hospital assessment by a paediatrician?
First febrile convulsion
Diagnostic uncertainty about the cause of the seizure
Focal features of the seizure
Seizure recurs within the same febrile illness (or within 24 hours)
< 18 months old
Parents are anxious
No apparent focus of infection
214
What is the recurrence rate of febrile convulsions?
1 in 3
215
What are some cardinal features of childhood rolandic epilepsy?
```
Unilateral facial sensorimotor symptoms
Oropharyngeal ictal manifestations
Arrest of speech
Hypersalivation
NOTE: this is not usually treated and children will grow out of it (around 14-18 years)
```
216
What is the first-line AED for generalised seizures?
Valproate
| NOTE: ethosuximide can be used for absence seizures
217
Name some antiepileptics that can worsen certain forms of epilepsy.
Lamotrigine - worsens myoclonic
| Carbamazapine - worsens absence
218
What is the first-line AED for focal seizures?
Carbamazepine or lamotrigine
219
What advice should you give to parents with regards to activities that may be dangerous with epilepsy?
Avoid situations where having a seizure could lead to injury or death (e.g. swimming unsupervised)
Driving is allows only after 1 year free of seizures
The school should be made aware of the diagnosis
220
How is Guillain-Barre syndrome managed?
Supportive
Respiratory support
IVIG
Plasma exchange
221
Which milestones would you expect the average child to have reached by 7 months?
Gross Motor: sits without support
Fine Motor: transfers objects from hand to hand
Hearing, Speech and Language: turns to voice, polysylabic babble
Social/Emotional: finger feeds, fears strangers
222
Which milestones would you expect the average child to have reached by 1 year?
Gross Motor: stand independently
Fine Motor: pincer grip (10 months), points
Hearing, Speech and Language: 1-2 words, understands name
Social/Emotional: drinks from cup, waves
223
Which milestones would you expect the average child to have reached by 15-18 months?
Gross Motor: walks independently
Fine Motor: immature grip of pencil, random scribble
Hearing, Speech and Language: 6-10 words, points to 4 body parts
Social/Emotional: feeds self with spoon, beginning to help with dressing
224
Which milestones would you expect the average child to have reached by 2.5 years?
Gross Motor: runs and jumps
Fine Motor: draws
Hearing, Speech and Language: 3-4 word sentences, understands 2 commands
Social/Emotional: parallel play
225
List the median ages for gross motor development milestones.
6-8 weeks: raises head to 45 degrees when prone
6-8 months: sits without support (first with round back, then straight back)
8-9 months: crawling
10 months: cruising
12 months: walks unsteadily
15 months: walks steadily
226
List the median ages for vision and fine motor milestones.
6 weeks: follows moving object or face by turning head
4 months: reaches out for toys
4-6 months: palmar grasp
7 months: transfers toys from one hand to another
10 months: mature pincer grip
16-18 months: makes marks with a crayon, tower of 3
3 years: draws a circle
227
List the median ages for hearing, speech and language development.
3-4 months: vocalises alone or when spoken to, coos and laughs
7 months: turns to soft sound out of sight
10 months: sounds used discriminately to parents (mama/dada)
12 months: 2-3 words other than mama or dada
18 months: 6-10 words, shows two parts of the body
20-24 months: use 2 or more words to make simple phrases
2.5-3 years: talks constantly in 3-4 word sentences
228
List the median ages for social, emotional and behavioural development.
```
6 weeks: smiles responsively
6-8 months: puts food in mouth
10-12 months: waves bye bye, plays peek-a-boo
12 months: drinks from cup
18 months: uses spoon
18-24 months: symbolic play
2 years: dry by day
2.5-3 years: parallel play, takes turns
```
229
How is paracetamol overdose managed?
Measure plasma paracetamol concentration at 4 hours and plot on normogram
Treat with IV N-acetylcysteine if necessary
230
How is carbon monoxide poisoning treated?
Presents with headache, nausea, confusion and drowsiness
High-flow oxygen
Hyperbaric oxygen therapy may be considered
231
How is salicylate poisoning treated?
Presentation: vomiting, tinnitus, respiratory alkalosis
Measure plasma salicylate concentration at 2-4 hours
Alkalinisation of urine with sodium bicarbonate increases urinary excretion
Consider haemodialysis
232
How is TCA overdose treated?
Treat arrhythmias and give sodium bicarbonate
| Support ventilation
233
How is ethylene glycol poisoning treated?
Presentation: intoxication, tachycardia, metabolic acidosis
| Fomepizole inhibits the production of toxic metabolites (ethanol can also be used)
234
How is iron overdose treated?
Presentation: vomiting, diarrhoea, haematemesis, late drowsiness/coma/shock/hypoglycaemia
Serum iron level 4 hours after ingestion is the best measure of severity
IV desferoxamine chelates iron
235
How is organophosphorus pesticide poisoning treated?
```
Supportive
Atropine (large dose)
Pralidoxime (reactivates actylcholinesterase)
```
NOTE: presentation is mainly cholinergic features
236
What is a port wine stain?
Capillary malformation in the dermis that is present from birth and persists for life
If in the trigeminal nerve distribution, some children may have Sturge-Weber syndrome and should have an MRI
237
Describe the appearance and progression of cavernous haemangiomas.
Appears within the first month of life
| Grows before shrinking and disappearing (before 5 years)
238
Describe the inheritance pattern of von Willebrand disease.
Type 1 and 2 = autosomal dominant
| Type 3 = autosomal recessive
239
How should bladder outflow obstruction be investigatd?
MCUG
240
What are the two different types of polycystic kidney disease and how do they differ?
Autosomal Dominant - mainly in older children/adults, cysts are large
Autosomal Recessive - presents in childhood with bilateral renal masses, respiratory distress due to pulmonary hypoplasia and congenital hepatic fibrosis with pulmonary hypertensio
241
What are the aspects of managing Duchenne muscular dystrophy?
```
Physiotherapy to prevent contractions
Exercise and psychological support
Surgery (e.g. tendoachilles lengthening)
CPAP for nocturnal hypoxia
Glucocorticoids may slow defeneration
```
242
What's the main difference between Duchenne and Becker muscular dystrophy?
Duchenne - no dystrophin - severe symptoms (LE: 20-30 years)
| Becker - abnormal dystrophin - milder symptoms
243
What are some consequences of neural tube defects?
Paralysis and muscle imbalance (needs physiotherapy)
Sensory loss (can lead to accidental damage)
Neuropathic bladder
Bowel denervation
Scoliosis
Hydrocephalus
244
How is hydrocephalus treated?
Ventriculoperitoneal shunt
245
Outline the management of idiopathic intracranial hypertension.
```
Eliminate causal factors
Weight-reduction
Low-sodium diet and fluid restriction
Acetazolamide
Analgesia
VP shunt
```
246
List some clinical features of a child at high-risk of sepsis.
```
Behaviour:
• No response to social cues
• Appears ill
• Does not wake, or if roused does not stay awake
• Weak, high-pitched and continuous cry
Heart Rate:
• Tachycardia (different at different ages)
• < 60 bpm at any age
Respiratory Rate
• Tachypnoea (different at different ages)
• Grunting
• Apnoea
• SpO2 < 90% on air
Mottled or ashen appearance
Cyanosis of the skin, lips or tongue
Non-blanching rash
Aged < 3 months with temperature > 38 degrees
Temperature < 36 degrees
```
247
Which investigations constitute a septic screen?
```
FBC
Blood culture
CRP
Urinalysis
LP
CXR
Also do a VBG
NOTE: if < 1 month, all children should have an LP
```
248
Outline the role of a VBG in managing a child with moderate to high risk of sepsis.
> 2 mmol/L or evidence of AKI --> treat as high-risk
| < 2 mmol/L = repeat assessment at least hourly, ensure review by senior clinician, identify a cause and manage it
249
Outline the non-pharmacological steps taken in the management of high risk sepsis.
```
Immediate review by senior clinician
VBG (gas, glucose, lactate, FBC, U&E, creatinine, clotting)
Broad spectrum antibiotics immediately
Monitor continuously
Monitor mental state using GCS or AVPU
```
250
Outline how the lactate guides treatment in high risk sepsis.
> 4 mmol/L = IV fluid bolus without delay, refer to critical care
2-4 mmol/L = IV fluid bolus without delay
< 2 mmmol/L = consider IV fluids
251
Which antibiotics are used to treat meningococcal sepsis?
Community: IM benzylpenicillin
Hospital: IV ceftriaxone
252
Outline the ABC approach to anaphylaxis.
Airway - look for and relieve any obstruction, intubate if necessary
Breathing - check whether it is normal
If unresponsive/not breathing normally - start CPR
Circulation: check pulse and blood pressure
Everything Else: check skin and inside of the mouth for urticaria and angio-oedema
253
Outline the use of adrenaline in anaphylaxis.
IM adrenaline 1:1000 (as per age-related guidelines) into thigh
Assess response after 5 mins
Repeat IM injection at 5 min interval until there has been a response
NOTE: IV adrenaline is only used for advanced life support
254
Aside from adrenaline, what else should be given to a patient in anaphylaxis?
High flow oxygen
IV fluids (titrate against blood pressure)
Chlorphenamine 10 mg IV
Hydrocortisone 200 mg IV
NOTE: if there is a wheeze, may require bronchodilators
255
What should be monitored whilst a patient is receiving treatment for anaphylaxis?
Blood pressure
Pulse
Respiratory function
256
Outline the neonatal resuscitation guidelines.
1. Dry the baby
2. Within 30 seconds: assess tone, breathing and heart rate
3. Within 60 seconds: if gasping or not breathing – give 5 inflation breaths
4. Re-assess: if NO increase in heart rate, look for chest movement
5. If chest NOT moving: recheck head position, consider 2-person airway control and other airway manoeuvres, repeat inflation breaths
6. If NO increase in heart rate: look for chest movement
7. When chest is moving: if heart rate is not detectable or slow (< 60/min) start compression with 3 compressions per breath
8. Reassess heart rate every 30 seconds: if heart rate is not detectable or slow (< 60/min) consider venous access and drugs (e.g. atropine)
257
Outline the steps in paediatric basic life support.
1. Are they unresponsive?
2. Shout for help
3. Open airway
4. Look, listen and feel for breathing
5. Give 5 rescue breaths
6. Check for signs of circulation
7. 15 chest compression: 2 rescue breaths (15:2)
258
List some possible presenting features of cerebral palsy.
```
Unusual fidget movements (e.g. asymmetry, paucity)
Abnormalities of tone (e.g. hypotonia)
Delayed motor milestones
Feeding difficulties
Persistent toe walking
```
259
Outline the aspects of managing cerebral palsy.
MDT approach
Physiotherapy - encourage movement, build strength
Speech and Swallow - ensure safe swallow, improve language abilities
Occupational therapy
Medications (baclofen for stiffness, melatonin for sleep, laxatives for constipation and anticholinergics for drooling)
260
List some comorbidities that are commonly associated with cerebral palsy.
```
Learning disability (1 in 2)
Behavioural difficulties (2 in 10)
Chronic constipation (3 in 5)
Visual impairment (1 in 2)
Hearing impairment (1 in 10)
Low bone mineral density
Epilepsy (1 in 3)
```
261
What are the three main types of cerebral palsy?
Spastic (hemiplegic, quadriplegic, diplegic)
Dyskinetic
Ataxic
NOTE: injuries causing brain damage up to 2 years can be classified as cerebral palsy
262
Outline the aspects of management of autism spectrum disorders.
Psychosocial interventions (increase attention and reduce repetitive/ritualistic behaviours)
Speech and language therapy
Pharmacological (antipsychotics may be considered for difficult behaviour)
Attend to family/carers needs
Assess for learning disability and discuss EHC plan
NOTE: < 10% can function independently as adults
263
What is the first-line management of ADHD?
ADHD-focused group parent-training programme
Offer individualised training programmes if the needs are too complex
NOTE: consider up to 10-week watch and wait period before this
264
Outline the step by step medical management of ADHD.
Methylphenidate (6 week trial)
If unsuccessful, consider lisdexamphetamine (or dexamphetamine)
if unsuccessful, consider atomoxetine or guanfacine
265
What is an important thing to do before starting patients on medication for ADHD?
Establish baseline physical state (especially HEIGHT) and perform an ECG
The medications can cause loss of appetite and stunted growth and the development of tics (they are also cardiotoxic)
NOTE: recommend yearly off medication trials
266
How should medical treatment of ADHD be monitored?
Consider using symptom rating scales (e.g. Conner's)
Measure height every 6 months
Measure weight every 3 months
Monitor HR and BP every 6 months
267
Outline the aspects of managing Down syndrome.
MDT
Screen for abnormalities - AVSD, duodenal atresia
Parental counselling and education
Individualised education plan
Genetic counselling about future pregnancies
268
List some conditions that are associated with Down syndrome.
```
Coeliac disease
Hypothyroidism
Alzheimer's disease
Epilepsy
Hearing and visual defects
```
269
What are the three types of insulin therapy?
Multiple Daily Injection Basal-Bolus: injections of short-acting insulin before meals + 1 long-acting insulin (generally the 1st option for new diagnoses)
Continuous SC Insulin Infusion: regular and continuous insulin delivered through a pump
1, 2 or 3 injections per day: mix of short-acting and long-acting insulin
270
How often should capillary glucose be measured in patients with T1DM?
At least 5/day
Fasting/through the day target: 4-7
After meals: 5-9
271
In which patients might you consider continuous glucose monitoring?
Frequent severe hypoglycaemia
Hypoglycaemia unawareness
Inability to recognise or communicate symptoms of hypoglycaemia (e.g. cognitive impairment)
272
Which complications of T1DM require on going monitoring?
Thyroid disease - annually from diagnosis
| Diabetic retinopathy, nephropathy and hypertension - annually from 12 years
273
How should mild-to-moderate hypoglycaemia be managed?
Fast-acting glucose by mouth (e.g. lucozade)
Check blood glucose in 15 mins (repeat glucose if necessary)
As symptoms improve, switch to oral complex long-acting carbohydrate
274
How should severe hypoglycaemia be managed?
In Hospital: IV 10% glucose (max 500 mg/kg of body weight)
Community: IM glucagon (500 µg < 8 yrs or 1 mg > 8 yrs) or glucogel
Once symptoms improve, give oral complex long-acting carbohydrate
275
Which observations should be recorded in a patient with DKA?
* Level of consciousness
* Vital signs (HR, BP, Temp, RR)
* History of nausea or vomiting
* Clinical evidence of dehydration
* Body weight
276
Which biochemical parameters should be measured in a patient with DKA?
```
pH and pCO2
Plasma sodium, potassium, urea and creatinine
Plasma bicarbonate
Blood glucose
Blood ketones
```
277
Outline how the fluid deficit in DKA is estimated.
5% fluid deficit = mild-moderate DKA (> 7.1)
| 10% fluid deficit = severe DKA (< 7.1)
278
Outline how maintenance fluid requirements are calculated in patients with DKA.
< 10 kg = 2 ml/kg/hour
10-40 = 1 ml/kg/hour
40+ = 40 ml/hour
These are lower than standard maintenance fluid calculations because of the risk of cerebral oedema
279
Which fluids should be given when rehydrating patients with DKA?
0.9% saline ONLY until plasma glucose < 14 mmol/L
Then change to 0.9% saline + 5% dextrose
Rehydrate over 48 hours
Consider switching to oral fluids once the child is alert, ketosis is resolving and they can tolerate oral fluids
280
What should all fluids administered to patients with DKA contain?
40 mmol/L potassium chloride (unless renal failure)
281
Describe how insulin therapy should be given in DKA.
Start IV insulin infusion 1-2 hours after beginning IV fluid therapy
Use soluble insulin at 0.05-0.1 units/kg/hour (disconnect insulin pump if present)
Consider increasing insulin dose if no reduction in blood ketones after 6-8 hours
282
When can SC insulin be started in a patient with DKA?
Consider if ketosis is resolving, child is alert and can tolerate oral fluids
Start SC insulin at least 30 mins before stopping IV insulin
If using an insulin pump, start the pump at least 60 mins before IV insulin is stopped
283
How should a child be monitored whilst receiving treatment for DKA?
Measure at least HOURLY
• Capillary blood glucose
• Vital signs (HR, BP, Temp, RR)
• Fluid balance with fluid input and output charts
• Level of consciousness (using modified GCS)
NOTE: if severe DKA or < 2 years, monitor every 30 mins
284
What else should be monitored in a patient receiving IV therapy for DKA?
Continuous ECG (detect hypokaleemia)
NOTE: if K+ < 3 mmol/L, consider temporarily stopping the insulin and discuss with paediatric critical care
285
What should be measured 2 hours after starting treatment for DKA and at least every 4 hours afterwards?
Glucose (laboratory)
Blood pH and CO2
Plasma sodium, potassium and urea
Beta-hydroxybutyrate
NOTE: every 4 hours, review clinical status, blood results, ECG trace and fluid balance
286
List some clinical features of cerebral oedema.
Headache
Agitation or irritability
Unexpected fall in heart rate
Increased blood pressure
287
How is cerebral oedema resulting from DKA treatment managed?
IV mannitol or hypertonic sodium chloride
288
How is congenital hypothyroidism treated?
Start thyroxine treatment within 2-3 weeks of age and continue throughout life
With adequate treatment, intelligence and development should be normal
289
How is acute symptomatic hypocalcaemia managed?
IV calcium gluconate
NOTE: chronic is managed with oral calcium and high dose vitamin D analogues
290
Outline the aspects of managing congenital adrenal hyperplasia.
Corrective surgery (usually at puberty)
Life-long glucocorticoids
Mineralocorticoids (if salt loss)
Monitor growth, skeletal maturity, plasma androgens and 17a-hydroxyprogesterone levels
291
Outline the management of an Addisonian crisis.
IV hydrocortisone
IV saline
IV glucose
Fludrocortisone may be needed
292
How is androgen insensitivity syndrome managed?
```
Investigation: karyotype
Bilateral orchidectomy (risk of testicular cancer)
Oestrogen therapy
```
293
For how long should children with measles or rubella be excluded from school?
4 days from the onset of the rash
294
Which investigations should be requested in a patient with suspected non-accidental injury?
Skeletal survey
CT head scan
Bloods and bone profile (rule out leukaemia, ITP)
Fundoscopy (retinal haemorrhages)
295
Who should be contacted in cases of suspected non-accidental injury?
Senior colleagues
Named doctor for child protection
Social services
Consider contacting the police (Child Abuse Investigation Team)
Consider contacting Mutli-Agency Safeguarding Hub (MASH)
296
What are the differences between diplegic, hemiplegic and quadriplegic cerebral palsy?
Diplegic: both legs and arms are involved, legs are affected more than arms, associated with periventricular leukomalacia and preterm babies, do NOT tend to have severe learning difficulties/epilepsy
Hemiplegic: affects one side of the body, arms more than legs
Quadriplegic: most severe form, all four limbs are severely affected, associated with learning difficulties, epilepsy and swallowing problems
297
What are some features of innocent murmurs?
Asymptomatic
Systolic
Louder during fever and exercise
Vary with respiration and posture
298
What counts as precocious and delayed puberty?
Precocious - Girls < 8, Boys < 9
| Delayed - Girls > 13, Boys > 14
299
Which investigations might be used for delayed puberty?
```
Pubertal staging (Tanner)
Accurate height and weight measurements
Bone age
Gonadotrophin levels
Visual field examination
CT/MRI head scan
Karyotype
```
300
Which scoring system is used to assess the severity of croup?
Westley croup score
301
Define infantile colic.
> 3 hours total crying, for > 3 days in any week or > 3 weeks
302
What are some key factors that distinguish Noonan syndrome from Turner syndrome?
Noonan can affect males
| Noonan is associated with mental retardation and pulmonary valve stenosis
303
Describe the prognosis for patients with minimal change disease.
1/3 have only a single episode
1/3 have occasional relapses
1/3 have frequent relapses stopping at adulthood
304
Define global developmental delay.
Significant delay in 2 or more developmental domains
305
Which investigations should be requested in suspected global developmental delay?
```
Chromosomal analysis (Down, Di George, Williams)
Fragile X testing
Creatine kinase
U&E
Lead level
Urate
Full blood count
Ferritin
TFTs
Biotinidase level
```
306
Which investigation is important to perform in any child who has had a non-febrile seizure?
ECG
307
List some causes of surfactant deficiency in newborns.
```
Prematurity
male
Sepsis
Maternal diabetes
Second twin
Elective C-section
```
308
List some causes of speech delay.
```
Hearing impairment
Expressive language disorder
Late bloomer
Cerebral palsy
Autism spectrum disorder
```
309
List some potential first-line investigations for suspected inherited disorders of metabolism.
```
Amino acids and acylcarcinitine profile
Ammonia
Lactate
Organic acids
Amino acids
Very long chain fatty acids
```
310
Which diseases are tested for in the Guthrie test?
```
Sickle cell anaemia
Cystic fibrosis
Congenital hypothyroidism
MCAD deficiency
PKU
Maple syrup urine disease
Isovaleric acidaemia
Homocystinuria
Glutaric aciduria type I
```
311
Which acid-base features would raise suspicion of an inborn error of metabolism?
Acidosis out of keeping with clinical picture
Abnormalities persist despite standard treatment
Raised anion gap
312
Outline the pathophysiology of lysosomal storage disorders and describe the clinical manifestations.
The lysosome is the recycling centre of the cell
Deficiency of enzymes within the lysosome leads to the accumulation of toxic proteins resulting hepatosplenomegaly and CNS involvement
This can manifest with developmental regress and seizures
Examples: mucopolysaccharidoses, oligosaccharidoses, mucolipidoses, sphingolypidoses (Fabry disease)
313
What are mucopolysaccharidoses?
Most common of the lysosomal storage disorders
Characterised by defective breakdown of glycosaminoglycans
Causes developmental regression, skeletal abnormalities, coarse facies, cardiomyopathy
314
Briefly outline the pathophysiology of lipid storage disorders.
Enzyme deficiency leads to lipid accumulation in cells and tissues
Excessive fat storage leads to permanent cellular and tissue damage
NOTE: Gaucher disease is the most common
315
What blood glucose level defines hypoglycaemia?
< 2.6 mmol/L
316
What is the inheritance pattern of congenital adrenal hyperplasia?
Autosomal recessive
NOTE: 21a-hydroxylase deficiency is most common
317
Describe the presenting features of an adrenal salt losing crisis.
Vomiting
Weight loss
Hypotonia
Circulatory collapse
318
List some causes of global developmental disorders.
```
Chromosomal (e.g. Down, fragile X)
Congenital hypothyroidism
Inborn errors of metabolism (e.g. PKU)
Congenital infection (e.g. rubella, CMV)
Hypoxic brain injury
Kernicterus
Meningitis
```
319
List some causes of abnormal motor development.
```
Central motor deficit (e.g. cerebral palsy)
Congenital myopathy (e.g. DMD)
Neural tube defect
Global developmental delay
```
320
Which children are most vulneralbe to periventricular leukomalacia?
Preterm babies
NOTE: periventricular leukomalacia is particularly associated with diplegic cerebral palsy
321
Describe the main features of dyskinetic cerebral palsy.
Characterised by the presence of involuntary, uncontrolled movements that are more obvious on active movement or stress
May feature chorea, athetosis or dystonia
Usually due to damage to the basal ganglia
322
Describe the main features of ataxic (hypotonic) cerebral palsy.
Most are genetically determined
Characterised by trunk and limb hypotonia, poor balance and delayed motor development
Later features include intention tremor and ataxic gait
323
List some tests that may be used to assess language development.
Symbolic toy test (e.g. Kendall or McCormick)
| Reynell test
324
What are the three main features of autism spectrum disorders?
Impaired social interaction
Speech and language disorder
Imposition of routines and ritualistic and repetitive behaviours
325
Name some formalised tests that may be used to diagnose autism.
Autism diagnostic interview (ADI)
| Autism diagnostic observation schedule (ADOS)
326
At what point in a child's life would a squint be considered pathological?
3 months
Usually due to a refractive error
327
```
What are the limit ages for:
Head control
Sits unsupported
Stands independently
Walks independently
```
Head control - 4 months
Sits unsupported - 9 months
Stands independently - 12 months
Walks independently - 18 months
328
What are the limit ages for:
Fixes and follows
Pincer grip
Fixes and follows - 3 months
| Pincer grip - 12 months
329
What are the limit ages for:
Saying 6 words with meaning
Joining words
3-word sentences
Saying 6 words with meaning - 18 months
Joining words - 2 years
3-word sentences - 2.5 years
330
Name the primitive reflexes.
```
Moro
Grasp
Rooting
Stepping
Asymmetrical tonic neck reflex
```
NOTE: these will be replaced by postural reflexes (e.g. parachute)
331
Which intelligence tests may be used in children?
WPPSI (2-7 years)
| WISC (6-16 years)
332
How is meconium ileus treated?
Gastrograffin enema (or N-acetylcysteine)
333
How should a congenital diaphragmatic hernia be managed?
Insert a large NG tube and apply suction to prevent distension of the intrathoracic bowel
334
How should neonatal hypoglycaemia be managed?
Early and frequent milk feeding
IV dextrose in refractory cases (and consider glucagon and hydrocortisone)
Aim for blood glucose > 2 mmol/L
335
How is a Meckel's diverticulum diagnosed?
Technetium 99 pertechnetate scan
336
Which causes of gastroenteritis should be reported to the health protection unit?
* Campylobacter
* Listeria
* Shigella
* Salmonella
337
Which blood tests may be considered at a coeliac disease review
```
Coeliac serology
FBC
TFT
LFT
Vitamin D, B12, folate and serum calcium
U&E
```
338
Describe the clinical features of congenital varicella syndrome.
```
Dermatomal skin scarring
Neurological defects
IUGR
Limb hypoplasia
Hydrops fetalis
```
339
Describe the clinical features of congenital CMV infection.
Mental handicap
Visual impairment
Progressive hearing loss
Psychomotor retardation
At birth: hydrops, IUGR, exomphalos, microcephaly, hydrocephalus, hepatosplenomegaly, thrombocytopaenia
340
Which organism most commonly causes septic arthritis in children?
Staphylococcus aureus
NOTE: in sickle cell patients, salmonella is also common but S. aureus is still the most common
341
What is the most common site of septic arthritis in children?
Hip
NOTE: plain film changes are apparent after 2-3 weeks
342
At what age can babies roll over?
4 months
343
What does the child protection team do once they have been informed about a case of suspected NAI?
Convene a case conference (includes parents, police officer, paediatrician, GP, health visitor and social worker)
Place the child's name on the child protection register
Give support to the parents
Ensure regular monitoring by health visitor/social worker
Arrange regular follow-up with paediatrician (especially growth and development)
344
What is the most common site of osteomyelitis in children?
Lower femur or upper tibia (around the knee)
| Tends to affect the metaphysis
345
When should an MCUG and DMSA be performed in a child who has had an atypical UTI?
MCUG can be done as soon as the current infection is under control
DMSA should be performed 3-4 months after the infection
346
Outline the definitive management of VUR.
Often improves with time
May require surgical corrections
Patients may receive prophylactic antibiotics
347
Which investigations are used to identify DDH?
< 5-6 months = ultrasound
| > 6 months = X-ray
348
What is the typical blood gas picture that is seen in congenital cyanotic heart disease?
Metabolic acidosis
| Hypoxia
349
Describe the appearance of a rash caused by parvovirus in adults.
Lacey rash
350
What is Gaucher disease?
Glycogen storage disease that is prevalent in Ashkanazi jews
NOTE: Tay Sachs is also common in this population
351
What are the five autism spectrum disorders?
```
Autism
Asperger’s syndrome
Rett syndrome
Childhood disintegrative disorder
Pervasive developmental disorder not otherwise specified (PDD-NOS)
```
352
Which disease in infants can present very similarly to necrotising enterocolitis?
Hirschsprung's enterocolitis
353
What does '10% glucose' mean with regards to fluids?
10 g glucose per 100 mL
354
What is the investigation of choice for suspected SUFE?
Frog-lateral hip X-ray
NOTE: they will have limited hip flexion and abduction
355
List some complications of Henoch-Schonlein Purpura.
```
Acute renal impairment
Intussusception
Arthritis involving ankles and knees commonly
Testicular pain
Pancreatitis
```
356
What is an important first step in the management of acute lymphoblastic leukaemia?
Prevent tumour lysis syndrome
Hyperhydration
Start allopurinol
357
Which antibiotic should be used for neutropaenic sepsis?
Tazocin
358
List some clinical features of space-occupying lesions.
```
Headaches are worse when lying down
Morning vomiting
Headaches may cause night-time waking
Change in mood or personality
Change in educational performance
Focal neurology (e.g. weakness, visual field defect)
```
359
Which age group is most likely to experience a febrile convulsion?
< 6 years
360
What are some key features of frontal seizures?
Involves motor and pre-motor cortex
| May lead to clonic movements
361
What are some key features of temporal lobe seizures?
Aura
Automatisms (e.g. lip smaking)
Impaired consciousness
362
Which diagnostic tests can be used for mumps and rubella?
Serology from blood or oral fluid sample
363
Which investigations may be used in a child with short stature?
```
MId-parental height
Random GH measurement
Insulin tolerance test
CT/MRI head scan
Bone age (using DEXA or write X-ray)
```
364
What are some complications of scarlet fever?
```
Otitis media
Acute sinusitis/mastoiditis
Streptococcal pneumonia
Meningitis
Endocarditis
Osteomyelitis
Rheumatic fever
Streptococcal glomerulonephritis
```
365
In an acute asthma attack, which other medications may be used if the patient is unresponsive to nebulised SABA and ipratropium bromide?
Magnesium sulphate
Aminophylline
IV salbutamol
IMPORTANT: ECG and electrolytes should be monitored
366
Who will receive the children's flu vaccine?
Annually as a nasal spray in september/october for all children aged 2-9 years
367
When is the 4 in 1 preschool booster given and which vaccines does it contain?
```
Around 3 years and 4 months
Diphtheria
Tetanus
Pertussis
Polio
```
368
When is the 3 in 1 teenage booster given and which vaccines does it contain?
14 years
Diphtheria
Tetanus
Polio
369
What type of murmur does a VSD cause?
Pansystolic heard loudest at the lower left sternal edge (fifth intercostal space)
370
What type of murmur does a PDA cause?
Continuous machinery murmur inferior to the left clavicle
371
What should you do first if a child presents to paediatric A&E with infectious diarrhoea, whooping cough or symptoms suggestive of MMR?
Put in a side room
372
What is spinal muscular atrophy?
Autosomal recessive disorder of anterior horn cells
Leads to progressive weakness and wasting of skeletal muscles
Due to mutation in SMN1
NOTE: Werdnig-Hoffman disease is type 1 SMD
373
What is Charcot-Marie-Tooth disease?
Hereditary motor sensory neuropathy
Causes symmetrical, slowly progressive distal muscle wasting
May present in preschool children with tripping from bilateral foot drop
374
What is a key feature of CSF studies in Guillain-Barre syndrome?
High protein
375
What is myotonic dystrophy?
Autosomal dominant trinucleotide repeat (CTG) disorder
| Presents with hypotonia, feeding difficulties, respiratory difficulties and abnormal facies (in older children)
376
List some causes of ataxia.
```
Friedreich ataxia
Ataxia telangiectasia
Cerebellar tumour
Cerebellar agenesis/dysgenesis
Post-infectious cerebellitis
Toxins (e.g. ethanol)
```
377
What is Friedreich ataxia and how does it present?
Autosomal recessive triplet repeat in FXN gene
Worsening ataxia and dysarthria
Distal wasting of lower limbs
Absent reflexes
Pes cavus (associated with Charcot-Marie-Tooth)
Impairment of proprioception
378
What are the main clinical features of ataxia telangiectasia?
```
Telangiectasia in the conjunctiva
Mild delay in motor development
Oculomotor problems
Coordination problems
Complex eye movement disorders
```
379
What are the main features of NF1?
```
6 or more cafe au lait spots
Neurofibromas
Axillary freckling
Optic glioma
Lisch nodules
1st degree relative with NF1
```
380
What are the main features of NF2?
Multiple schwannomas
Meningiomas
Ependyomas
Bilateral acoustic neuroma
381
List the main clinical features of tuberous sclerosis.
Cutaneous: ash leaf depigmentation, shagreen patches (roughened), angiofibromata (across the bridge of the nose)
Neurological: developmental delay, epilepsy, intellectual disability
382
What are the main features of Sturge-Weber syndrome?
Port wine stain in the distribution of the ophthalmic division of the trigeminal nerve
Similar lesion intracranially
May have epilepsy, intellectual disability and contralateral hemiplegia
383
List some neurodegenerative conditions that occur in children.
```
Lysosomal storage disorders
Peroxisome enzyme defects
Wilson disease
Tay Sachs
Gaucher
Niemann Pick
```
384
What is adrenoleukodystrophy?
Group of disorders caused by peroxisomal defects
Peroxisomes are important for breaking down fatty acids
Neonatal and X-linked forms
385
What are some clinical features of growing pains?
Age 3-12
Symmetrical pain not limited to joints
Pain never present at the start of the day
Physical activities not limited (no limp)
Normal physical examination
386
how does subluxation of the patella present?
Sudden lateral dislocation of the patella
Very painful
Feeling of instability/giving way
Requires reduction and immobilisation
387
How many joints must be affected to be classified as polyarticular juvenile idiopathic arthritis?
More than 4
388
Describe the difference in the iron content of breast milk and cows milk.
Breast milk - low iron content, high absorption
| Cows milk - high iron content, low absorption
389
List some clinical features of Edwards syndrome.
```
Low-set ears
Small chin
Microcephaly
Overlapping fingers
Rocker-bottom feet
Cardiac defects (VSDs)
Renal anomalies
Learning disability
90% death within 1 year
```
390
List some clinical features of Patau syndrome.
```
Structural brain defects
Small eyes
Polydactyly
Cardiac/renal malformations
Death within 1 year
```
391
List some clinical features of Klinefelter's syndrome.
```
Most common cause of male hypogonadism
Small firm testes
Tall
Behavioural problems
Delayed speech
Gynaecomastia
```
392
List some clinical features of fragile X syndrome.
```
Moderate learning difficulties (most common genetic cause of learning difficulties)
Macrocephaly
Large testes
Large ears
Long face
Prominent mandible and forehead
```
393
How is fragile X syndrome inherited?
X-linked dominant
| Female carriers may have mild learning difficulties
394
What is Still's disease?
Systemic form of JIA thought to be of autoimmune origin
Intermittent pyrexia
Salmon-link rash
Aches and pains of joints and muscles
395
List some clinical features that are common to all congenital TORCH infection.
```
Low birth weight
Prematurity
Jaundice
Microcephaly
Seizures
Anaemia
Failure to thrive
Encephalitis
```
396
Which medication is used to chelate lead in lead poisoning?
EDTA (ethylene diamine tetraacetic acid)
NOTE: it present with encephalopathic features (e.g. behavioural problems, pica) and causes a hypochromic microcytic anaemia with basophilic stippling
397
Which other condition can present very similarly to acute epiglottitis?
```
Retropharyngeal abscess (causes fever, drooling, dysphagia and stridor)
Typically has a longer history than acute epiglottitis (over a few days)
```
398
Which murmur is typically heard in tetralogy of Fallot?
Ejection systolic murmur heard best in the 3rd intercostal space with a single heart sound
399
Which tumour markers can be used for neuroblastoma?
Urinary catecholamines
400
What is a craniopharyngioma?
Rare type of brain tumour derived from pituitary gland embryonic tissue
401
Which tissue does neuroblastoma arise from?
Neural crest tissue in the adrenal medulla and sympathetic nervous system
NOTE: MIBG scans are used to identify neurblastoma tissue
402
Which tissue does nephroblastoma arise from?
Embryonal renal tissue
403
What is the most common type of malignant bone tumour in children?
Osteosarcoma
NOTE: Ewing is common in younger children
404
Describe the inheritance pattern of retinoblastoma.
Autosomal dominant
| Located on chromosome 13
405
What is a common benign cause of proteinuria in children?
Orthostatic (postural) proteinuria
| Proteinuria is only found when the child is upright during the day
406
List some features of Fanconi syndrome.
```
Polydipsia and polyuria
Salt depletion and dehydration
Hyperchloraemic metabolic acidosis
Rickets
Faltering growth
```
407
List some causes of heart failure in neonates.
Hypoplastic left heart syndrome
Critical aortic valve stenosis
Severe coarctation of the aorta
Interruption of the aortic arch
408
Which congenital heart defect is most commonly seen in DiGeorge syndrome?
Interrupted aortic arch
| Others include persistent truncus arteriosus, TOF and VSD
409
What are some features suggestive of atypical UTI?
```
Seriously ill
Poor urine flow
Abdominal mass
Raised creatinine
Septicaemia
Failure to respond to antibiotic treatment
Infection with non-E. coli organisms
```
