Paediatrics Flashcards
When is the 6 in 1 vaccine given?
2, 3 and 4 months
When is the pneumococcus vaccine given?
2, 4 and 12 months
When is the Men B vaccine given?
2, 4 and 12 months
When is the rotavirus vaccine given?
2 and 3 months
When is the Hib/MenC booster given?
1 year
When is the MMR given?
1 year and 3 years 4 months
When is the HPV vaccine given?
12-13 years
When is the Men ACWY vaccine given?
14 years
New university students aged 19-25
Outline the management of DDH.
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)
Outline how hearing is tested in the neonate.
1st: evoked otoacoustic emission (EOEA) testing
If this is abnormal –> automated auditory brain stem (AABR) audiometry
Briefly outline the steps in the management of necrotising enterocolitis.
Stop oral feeding
Broad spectrum antibiotics (ceftriaxone and vancomycin)
Surgery if perforation/necrosis
TPN
What can be used to close a PDA?
IV indomethacin
Prostacyclin synthetase inhibitor
Ibuprofen
How is the bilirubin concentration measured in neonatal jaundice?
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)
What serum bilirubin levels suggests increased risk of developing kernicterus?
> 340 µmol/L in babies > 37 weeks
or rising rapidly > 8.5 µmol/L/hr
How often should serum bilirubin be measured in a neonate with jaundice?
Every 6 hours until it drops below the treatment threshold
Which investigations should be performed in a neonate who developed jaundice within 24 hours of birth?
Haematocrit Blood group of mother and baby DAT test FBC and blood film Blood G6PD level Blood/urine/CSF culture
Which antibiotics are used to treat meconium aspiration?
IV ampicillin and gentamicin
Which antibiotics may be used in the treatment of early-onset sepsis?
Benzylpenicillin and gentamicin
Which organism most commonly causes late-onset sepsis?
Coagulase-negative staphylococcus (e.g. Staphylococcus epidermidis)
How is neonatal meningitis treated?
3rd generation cephalosporin + amoxicillin/ampicillin
What is the paediatric sepsis 6?
- 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
How should neonatal conjunctivitis be treated?
Discharge and redness (staph or strep) - topical ointment (e.g. neomycin)
Gonococcus - 3rd generation cephalosporin
Chlamydia - erythromycin (2 weeks)
What should babies at risk of vertical hepatitis B transmission receive?
Hepatitis B immunoglobulin AND Hep B vaccine
How is gastro-oesophageal reflux in a breastfed infant treated?
1st line: Breastfeeding assessment
2nd line: trial of alginate therapy for 1-2 weeks
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
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
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
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
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
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
What should be monitored in children with Coeliac disease?
Annual review Weight, height and BMI Review symptoms Review diet and adherence Consider blood tests
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)
How is severe fulminating disease in UC managed?
IV corticosteroids (induce remission)
Consider IV ciclosporin
Surgery - colectomy with ileostomy or IJ pouch
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
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)
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)
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
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
Which antibiotics might you use in a patient with bacterial meningitis who has a severe beta-lactam allergy?
Vancomycin and moxifloxacin
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
How is HSV encephalitis treated?
High-dose IV aciclovir for 3 weeks
How is toxic shock syndrome managed?
ICU Surgical debridement of infected tissue Clindamycin (stops toxin production) Vancomycin or meropenem IVIG (neutralise the toxin)
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
How is periorbital cellulitis treated?
High-dose IV ceftriaxone
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)
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
What must young people with EBV avoid doing?
Contact sports
Which medications are occasionally used to treat CMV infection?
IV ganciclovir
Oral valganciclovir
Foscarnet
How long should a child stay away from school for after measles infection?
4 days after rash onset
How long should a child stay away from school for after mumps infection?
5 days after the development of parotitis
How are mumps and rubella diagnosed?
Oral fluid sample
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)
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
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
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
Which tests can you do to further investigate suspected cow’s milk protein allergy?
Skin prick testing
Specific IgE
What PRN treatment may be appropriate for patients with allergic rhinitis?
Aged 2-5: oral antihistamine
Everyone else: intranasal azelastine
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
How would you treat urticaria?
Identify and manage triggers
Oral antihistamine for up to 6 weeks
Severe - oral corticosteroid
Refractory - IgE antibody or LTRA
How is bacterial tonsillitis treated?
Penicillin V (10 days) Allergy: clarithromycin
Which medication should be avoided in tonsillitis?
Amoxicillin
Causes a widespread maculopapular rash in infectious mononucleosis
How is scarlet fever treated?
Penicillin V QDS for 10 days
Allergy: azithromycin, clarithromycin
How long should patients with strep throat/scarlet fever stay away from school?
24 hours after starting antibiotics
What is the first-line medical management for acute otitis media?
Amoxicillin 5-7 days
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)
Which severity of croup requires admission?
Anything worse than mild
I.e. anything worse than a barking cough on its own
How is croup treated?
0.15 mg/kg dexamethasone stat
This can be repeated after 12 hours if necessary
How is severe croup treated in an emergency?
High-flow oxygen
Nebulised adrenaline
How is acute epiglottitis managed?
Urgent hospital admission (ICU) Secure airway and supplemental oxygen Take blood culture IV cefuroxime Rifampicin prophylaxis for entire household
How is bronchiolitis treated?
Conservative
Supplemental oxygen if < 92%
Nasogastric/orogastric tube feeding if poor intake
Consider nebulised 3% saline
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
How is multiple trigger wheeze treated?
ICS or LTRA for 4-8 weeks
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
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
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
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
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
Which medication should a patient be given to take home after an acute asthma attack?
Oral prednisolone (3-7 days)
When should a patient with an asthma attack treated in hospital be followed-up?
Within 2 working days of discharge
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)
How is foreign body inhalation treated in an unconscious patient?
ABCDE
Secure the airway
Remove the foreign body (rigid/flexible bronchoscopy)
Which patients with whooping cough should be admitted?
< 6 months
Significant breathing difficulties
Outline the pharmacological treatment of whooping cough.
< 21 days after onset of cough: macrolide
(clarithromycin/azithromycin)
NOTE: use erythromycin in pregnant women
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
What are some treatment approaches for bronchiectasis?
Airway clearance techniques (physiotherapy)
Inhaled bronchodilator
Inhaled hypertonic saline
Antibiotic prophylaxis (e.g. azithromycin)
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
What is the first-line mucoactive agent for cystic fibrosis?
rhDNAse
2nd line: add hypertonic saline
Alternative: mannitol dry powder inhalation
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
What are the management approaches to the nutritional problems in CF?
Oral enteric-coated pancreatic replacement therapy
High calorie diet
Fat-soluble vitamin supplements
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
What is a treatment option for severe sleep disordered breathing in a child?
Adenotonsillectomy
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)
How should an inflamed nappy rash that is causing discomfort be treated?
Hydrocortisone 1% cream OD (max 7 days)
How should a nappy rash caused by candida be treated?
Do NOT use barrier protection
Prescribe topical imidazole (e.g clotrimazole)
How should a nappy rash caused by bacterial infection be treated?
Oral flucloxacillin for 7 days
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)
What treatments for seborrhoeic dermatitis could be used if conservative measures fail?
Topical imidazole cream
Hydrocortisone cream
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
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
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%
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)
Under which circumstances do bandages tend to be used in eczema?
For areas of chronically lichenified skin
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
How should infected eczema be treated?
Swab the affected area
Advice on good hygiene when using emollients
Flucloxacillin
How is eczema herpeticum managed?
Refer for same-day dermatology advice
Oral aciclovir
Consider ophthalmological review if around the eyes
How are viral warts treated?
Daily administration of salicylic acid, lactic acid paint or glutaraldehyde lotion
Cryotherapy
How is molluscum contagiosum managed?
Spontaneous resolution by 18 months
Avoid squeezing lesions
Avoid sharing towels/clothes
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
How are more severe ringworm infections managed?
Oral antifungals
1st line: terbinafine
2nd line: itraconazole
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
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%
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
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
List some agents that are used in the treatment of guttate psoriasis.
Coal tar preparations
Dithranol
Calcipotriol
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
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
What can be used as an alternative to oral antibiotics in girls with acne?
COCP
NOTE: POPs and progestin implants can worsen acne
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
When should a patient undergoing treatment for acne be reviewed?
At 8-12 weeks
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)
How are ASDs managed?
Secundum - percutaneous closure (cardiac catheterisation with insertion of an occlusive device)
Partial AVSD - surgical correction
When are symptomatic ASDs usually treated?
3-5 years
When do large VSDs and AVSD tend to be treated surgically?
3-6 months
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
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
What murmur is associated with ASD?
Ejection systolic murmur best heard at the upper left sternal edge and fixed wide split second heart sound
What murmur is associated with VSD?
Loud pansystolic murmur at the lower left sternal edge, quiet pulmonary second heart sound
Which defects require surgical correction in tetralogy of Fallot?
Close the VSD
Relive the right ventricular outflow obstruction
How may hypercyanotic spells in tetralogy of Fallot be treated?
Sedation and pain relief
IV propranolol
IV fluids
Which life-saving procedure may be performed for patients with transposition of the great arteries to enhance mixing of the blood?
Balloon atrial septostomy
How is tricuspid atresia treated?
Blalock-Taussig shunt
How is aortic stenosis treated?
Balloon valvulotomy
Aortic valve replacement
NOTE: same for pulmonary stenosis
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
How is acute rheumatic fever treated?
Bed rest and anti-inflammatory agents (e.g. aspirin)
Penicillin V if evidence of persistent infection
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
How is infective endocarditis treated?
Beta-lactam and gentamicin
Usually for 6 weeks
How would you treat an umbilical granuloma?
Regular application of salt to the wound
Cauterise with silver nitrate
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
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
Which clinical features are suggestive of an upper UTI?
Bacteriuria + fever
Bacteriuria + loin pain
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
How should simple cystitis be treated?
Oral antibiotics (e.g. trimethoprim) for 3 days
Which children should have an ultrasound after a UTI?
Children who have had an atypical UTI
Children < 6 months
Which children should have a DMSA and MCUG after a UTI?
< 6 months old presenting with atypical or recurrent UTI
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
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
List some causes of secondary enuresis.
UTI
Constipation
Diabetes
Psychological/Family problems
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
List some complications of nephrotic syndrome.
Hypovolaemia
Thrombosis
Infection
Hypercholesterolaemia
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
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
What are the most common causes of AKI in children?
HUS
ATN
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
What long-term follow-up should be offered to patients with HUS?
Check for persistent proteinuria, the development of hypertension and progressive CKD
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
How does CKD affect the growth of a child?
Delayed puberty
Subnormal pubertal growth spurt
How are hydroceles in children managed?
< 2 years = most resolve spontaneously
2-11 year = open/laparoscopic repair
11-18 years = conservative or surgical
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
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)
How is testicular torsion managed?
Urgent exploratory surgery (with orchidopexy/orchidectomy)
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
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
How are labial adhesions treated?
Topical steroids or oestrogens
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
Why should you check the serum bilirubin level 12-18 hours after stopping phototherapy?
Check for rebound hyperbilirubinaemia
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
How is biliary atresia managed?
Kasai procedure
Complications can be managed using ursodeoxycholic acid, fat-soluble vitamins and prophylactic antibiotics
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)
Which investigations are used for galactosaemia?
Galactose in urine
Measuring red cell Gal-1-PUT
How is galactosaemia treated?
Galactose-free diet
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
How is autoimmune hepatitis managed?
Prenisolone and azathioprine
Ursodeoxycholic acid can help in PSC
Liver transplant in severe cases
How is hepatic encephalopathy treated?
Supportive
Identify and correct precipitating factors (e.g. GI bleed)
Reduce nitrogenous load (dietary protein restriction, lactulose and rifaximin)
How long do patients with ALL tend to have chemotherapy for?
Girls - 2 years
Boys - 3 years
What is the cure rate for lymphoma?
80%
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)
What percentage of patients with Wilm’s tumour are cured?
80%
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
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
What is the definitive management for slipped upper femoral epiphysis?
Internal fixation across the growth plate
Which type of fluid should be given for maintenance requirements in children?
0.9% NaCl + 5% dextrose
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)
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
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
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
What are the aspects of treating a neonate with hereditary spherocytosis?
Supportive (maybe blood transfusion)
Folic acid supplementation
Phototherapy/exchange transfusion
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
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
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)
Outline the steps in the analgesic ladder.
Step 1: paracetamol
Step 2: cocodamol or NSAIDs (weak opioid)
Step 3: morphine, oxycodone
Which drug treatment can be used to reduce sickling in sickle cell patients?
Hydroxycarbamide
How is haemophilia treated?
Factor 8 concentrate (A)
Factor 9 concentrate (B)
Acute bleed - factor concentrates and anti-fibrinolytics
Which medications should be avoided in patients with haemophilia and von Willebrand disease?
IM injections
Aspirin
NSAIDs
Which treatment can be used for mild haemophilia A?
Desmopressin (stimulates endogenous release of vWF)
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
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
How can severe ITP be managed?
IVIG + corticosteroid + platelet transfusions
Antifibrinolytics (e.g. tranexamic acid) may be used
How is DIC treated?
Treat underlying cause
Supportive care
Replacement therapy (platelets and clotting factors)
Restoration of physiological coagulation pathways (e.g. using heparins)
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
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
How is chondromalacia patellae managed?
Physiotherapy for quadriceps strengthening
How is osteochondritis dissecans managed?
Pain relief
Rest and quadriceps exercise
Occasionally need surgery
How is subluxation of the patella treated?
Reduction and immobilisation
Rehab
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
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)
What are the aspects of managing juvenile idiopathic arthritis?
Multidisciplinary team Physiotherapist and occupational therapist NSAIDs Corticosteroids DMARDs (methotrexate is first-line)
How is vitamin D deficiency/Rickets treated?
Calcium and ergocalciferol
OR cholecalciferol
NOTE: pseudovitamin D deficiency is treated with alfacalcidol or calcitriol
How might you investigate a patient presenting with migraines?
Headache diary for 8 weeks to help identify triggers
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
Which medications are used to prevent migrianes?
Topiramate and propranolol
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
What should be tested in all children who have a seizure?
Blood glucose
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
What is the recurrence rate of febrile convulsions?
1 in 3
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)
What is the first-line AED for generalised seizures?
Valproate
NOTE: ethosuximide can be used for absence seizures
Name some antiepileptics that can worsen certain forms of epilepsy.
Lamotrigine - worsens myoclonic
Carbamazapine - worsens absence
What is the first-line AED for focal seizures?
Carbamazepine or lamotrigine
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
How is Guillain-Barre syndrome managed?
Supportive
Respiratory support
IVIG
Plasma exchange
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
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
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
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
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
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
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
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
How is paracetamol overdose managed?
Measure plasma paracetamol concentration at 4 hours and plot on normogram
Treat with IV N-acetylcysteine if necessary
How is carbon monoxide poisoning treated?
Presents with headache, nausea, confusion and drowsiness
High-flow oxygen
Hyperbaric oxygen therapy may be considered
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
How is TCA overdose treated?
Treat arrhythmias and give sodium bicarbonate
Support ventilation
How is ethylene glycol poisoning treated?
Presentation: intoxication, tachycardia, metabolic acidosis
Fomepizole inhibits the production of toxic metabolites (ethanol can also be used)
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
How is organophosphorus pesticide poisoning treated?
Supportive Atropine (large dose) Pralidoxime (reactivates actylcholinesterase)
NOTE: presentation is mainly cholinergic features
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
Describe the appearance and progression of cavernous haemangiomas.
Appears within the first month of life
Grows before shrinking and disappearing (before 5 years)
Describe the inheritance pattern of von Willebrand disease.
Type 1 and 2 = autosomal dominant
Type 3 = autosomal recessive
How should bladder outflow obstruction be investigatd?
MCUG
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
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
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
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
How is hydrocephalus treated?
Ventriculoperitoneal shunt
Outline the management of idiopathic intracranial hypertension.
Eliminate causal factors Weight-reduction Low-sodium diet and fluid restriction Acetazolamide Analgesia VP shunt
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
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
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
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
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
Which antibiotics are used to treat meningococcal sepsis?
Community: IM benzylpenicillin
Hospital: IV ceftriaxone
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
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
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
What should be monitored whilst a patient is receiving treatment for anaphylaxis?
Blood pressure
Pulse
Respiratory function
Outline the neonatal resuscitation guidelines.
- Dry the baby
- Within 30 seconds: assess tone, breathing and heart rate
- Within 60 seconds: if gasping or not breathing – give 5 inflation breaths
- Re-assess: if NO increase in heart rate, look for chest movement
- If chest NOT moving: recheck head position, consider 2-person airway control and other airway manoeuvres, repeat inflation breaths
- If NO increase in heart rate: look for chest movement
- When chest is moving: if heart rate is not detectable or slow (< 60/min) start compression with 3 compressions per breath
- Reassess heart rate every 30 seconds: if heart rate is not detectable or slow (< 60/min) consider venous access and drugs (e.g. atropine)
Outline the steps in paediatric basic life support.
- Are they unresponsive?
- Shout for help
- Open airway
- Look, listen and feel for breathing
- Give 5 rescue breaths
- Check for signs of circulation
- 15 chest compression: 2 rescue breaths (15:2)
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
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)
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)
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
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
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
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
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
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
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
List some conditions that are associated with Down syndrome.
Coeliac disease Hypothyroidism Alzheimer's disease Epilepsy Hearing and visual defects
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
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
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)
Which complications of T1DM require on going monitoring?
Thyroid disease - annually from diagnosis
Diabetic retinopathy, nephropathy and hypertension - annually from 12 years
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
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
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
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
Outline how the fluid deficit in DKA is estimated.
5% fluid deficit = mild-moderate DKA (> 7.1)
10% fluid deficit = severe DKA (< 7.1)
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
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
What should all fluids administered to patients with DKA contain?
40 mmol/L potassium chloride (unless renal failure)
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
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
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
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
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
List some clinical features of cerebral oedema.
Headache
Agitation or irritability
Unexpected fall in heart rate
Increased blood pressure
How is cerebral oedema resulting from DKA treatment managed?
IV mannitol or hypertonic sodium chloride
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
How is acute symptomatic hypocalcaemia managed?
IV calcium gluconate
NOTE: chronic is managed with oral calcium and high dose vitamin D analogues
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
Outline the management of an Addisonian crisis.
IV hydrocortisone
IV saline
IV glucose
Fludrocortisone may be needed
How is androgen insensitivity syndrome managed?
Investigation: karyotype Bilateral orchidectomy (risk of testicular cancer) Oestrogen therapy
For how long should children with measles or rubella be excluded from school?
4 days from the onset of the rash
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)
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)
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
What are some features of innocent murmurs?
Asymptomatic
Systolic
Louder during fever and exercise
Vary with respiration and posture
What counts as precocious and delayed puberty?
Precocious - Girls < 8, Boys < 9
Delayed - Girls > 13, Boys > 14
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
Which scoring system is used to assess the severity of croup?
Westley croup score
Define infantile colic.
> 3 hours total crying, for > 3 days in any week or > 3 weeks
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
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
Define global developmental delay.
Significant delay in 2 or more developmental domains
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
Which investigation is important to perform in any child who has had a non-febrile seizure?
ECG
List some causes of surfactant deficiency in newborns.
Prematurity male Sepsis Maternal diabetes Second twin Elective C-section
List some causes of speech delay.
Hearing impairment Expressive language disorder Late bloomer Cerebral palsy Autism spectrum disorder
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
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
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
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)
What are mucopolysaccharidoses?
Most common of the lysosomal storage disorders
Characterised by defective breakdown of glycosaminoglycans
Causes developmental regression, skeletal abnormalities, coarse facies, cardiomyopathy
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
What blood glucose level defines hypoglycaemia?
< 2.6 mmol/L
What is the inheritance pattern of congenital adrenal hyperplasia?
Autosomal recessive
NOTE: 21a-hydroxylase deficiency is most common
Describe the presenting features of an adrenal salt losing crisis.
Vomiting
Weight loss
Hypotonia
Circulatory collapse
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
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
Which children are most vulneralbe to periventricular leukomalacia?
Preterm babies
NOTE: periventricular leukomalacia is particularly associated with diplegic cerebral palsy
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
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
List some tests that may be used to assess language development.
Symbolic toy test (e.g. Kendall or McCormick)
Reynell test
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
Name some formalised tests that may be used to diagnose autism.
Autism diagnostic interview (ADI)
Autism diagnostic observation schedule (ADOS)
At what point in a child’s life would a squint be considered pathological?
3 months
Usually due to a refractive error
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
What are the limit ages for:
Fixes and follows
Pincer grip
Fixes and follows - 3 months
Pincer grip - 12 months
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
Name the primitive reflexes.
Moro Grasp Rooting Stepping Asymmetrical tonic neck reflex
NOTE: these will be replaced by postural reflexes (e.g. parachute)
Which intelligence tests may be used in children?
WPPSI (2-7 years)
WISC (6-16 years)
How is meconium ileus treated?
Gastrograffin enema (or N-acetylcysteine)
How should a congenital diaphragmatic hernia be managed?
Insert a large NG tube and apply suction to prevent distension of the intrathoracic bowel
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
How is a Meckel’s diverticulum diagnosed?
Technetium 99 pertechnetate scan
Which causes of gastroenteritis should be reported to the health protection unit?
- Campylobacter
- Listeria
- Shigella
- Salmonella
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
Describe the clinical features of congenital varicella syndrome.
Dermatomal skin scarring Neurological defects IUGR Limb hypoplasia Hydrops fetalis
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
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
What is the most common site of septic arthritis in children?
Hip
NOTE: plain film changes are apparent after 2-3 weeks
At what age can babies roll over?
4 months
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)
What is the most common site of osteomyelitis in children?
Lower femur or upper tibia (around the knee)
Tends to affect the metaphysis
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
Outline the definitive management of VUR.
Often improves with time
May require surgical corrections
Patients may receive prophylactic antibiotics
Which investigations are used to identify DDH?
< 5-6 months = ultrasound
> 6 months = X-ray
What is the typical blood gas picture that is seen in congenital cyanotic heart disease?
Metabolic acidosis
Hypoxia
Describe the appearance of a rash caused by parvovirus in adults.
Lacey rash
What is Gaucher disease?
Glycogen storage disease that is prevalent in Ashkanazi jews
NOTE: Tay Sachs is also common in this population
What are the five autism spectrum disorders?
Autism Asperger’s syndrome Rett syndrome Childhood disintegrative disorder Pervasive developmental disorder not otherwise specified (PDD-NOS)
Which disease in infants can present very similarly to necrotising enterocolitis?
Hirschsprung’s enterocolitis
What does ‘10% glucose’ mean with regards to fluids?
10 g glucose per 100 mL
What is the investigation of choice for suspected SUFE?
Frog-lateral hip X-ray
NOTE: they will have limited hip flexion and abduction
List some complications of Henoch-Schonlein Purpura.
Acute renal impairment Intussusception Arthritis involving ankles and knees commonly Testicular pain Pancreatitis
What is an important first step in the management of acute lymphoblastic leukaemia?
Prevent tumour lysis syndrome
Hyperhydration
Start allopurinol
Which antibiotic should be used for neutropaenic sepsis?
Tazocin
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)
Which age group is most likely to experience a febrile convulsion?
< 6 years
What are some key features of frontal seizures?
Involves motor and pre-motor cortex
May lead to clonic movements
What are some key features of temporal lobe seizures?
Aura
Automatisms (e.g. lip smaking)
Impaired consciousness
Which diagnostic tests can be used for mumps and rubella?
Serology from blood or oral fluid sample
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)
What are some complications of scarlet fever?
Otitis media Acute sinusitis/mastoiditis Streptococcal pneumonia Meningitis Endocarditis Osteomyelitis Rheumatic fever Streptococcal glomerulonephritis
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
Who will receive the children’s flu vaccine?
Annually as a nasal spray in september/october for all children aged 2-9 years
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
When is the 3 in 1 teenage booster given and which vaccines does it contain?
14 years
Diphtheria
Tetanus
Polio
What type of murmur does a VSD cause?
Pansystolic heard loudest at the lower left sternal edge (fifth intercostal space)
What type of murmur does a PDA cause?
Continuous machinery murmur inferior to the left clavicle
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
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
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
What is a key feature of CSF studies in Guillain-Barre syndrome?
High protein
What is myotonic dystrophy?
Autosomal dominant trinucleotide repeat (CTG) disorder
Presents with hypotonia, feeding difficulties, respiratory difficulties and abnormal facies (in older children)
List some causes of ataxia.
Friedreich ataxia Ataxia telangiectasia Cerebellar tumour Cerebellar agenesis/dysgenesis Post-infectious cerebellitis Toxins (e.g. ethanol)
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
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
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
What are the main features of NF2?
Multiple schwannomas
Meningiomas
Ependyomas
Bilateral acoustic neuroma
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
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
List some neurodegenerative conditions that occur in children.
Lysosomal storage disorders Peroxisome enzyme defects Wilson disease Tay Sachs Gaucher Niemann Pick
What is adrenoleukodystrophy?
Group of disorders caused by peroxisomal defects
Peroxisomes are important for breaking down fatty acids
Neonatal and X-linked forms
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
how does subluxation of the patella present?
Sudden lateral dislocation of the patella
Very painful
Feeling of instability/giving way
Requires reduction and immobilisation
How many joints must be affected to be classified as polyarticular juvenile idiopathic arthritis?
More than 4
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
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
List some clinical features of Patau syndrome.
Structural brain defects Small eyes Polydactyly Cardiac/renal malformations Death within 1 year
List some clinical features of Klinefelter’s syndrome.
Most common cause of male hypogonadism Small firm testes Tall Behavioural problems Delayed speech Gynaecomastia
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
How is fragile X syndrome inherited?
X-linked dominant
Female carriers may have mild learning difficulties
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
List some clinical features that are common to all congenital TORCH infection.
Low birth weight Prematurity Jaundice Microcephaly Seizures Anaemia Failure to thrive Encephalitis
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
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)
Which murmur is typically heard in tetralogy of Fallot?
Ejection systolic murmur heard best in the 3rd intercostal space with a single heart sound
Which tumour markers can be used for neuroblastoma?
Urinary catecholamines
What is a craniopharyngioma?
Rare type of brain tumour derived from pituitary gland embryonic tissue
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
Which tissue does nephroblastoma arise from?
Embryonal renal tissue
What is the most common type of malignant bone tumour in children?
Osteosarcoma
NOTE: Ewing is common in younger children
Describe the inheritance pattern of retinoblastoma.
Autosomal dominant
Located on chromosome 13
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
List some features of Fanconi syndrome.
Polydipsia and polyuria Salt depletion and dehydration Hyperchloraemic metabolic acidosis Rickets Faltering growth
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
Which congenital heart defect is most commonly seen in DiGeorge syndrome?
Interrupted aortic arch
Others include persistent truncus arteriosus, TOF and VSD
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
