Mx

Question 1

Chronic Lung Disease of Prematurity

Proph 1 resp support 3 medications 3

Answer 1

•	Prophylaxis:
o	Corticosteroids for women in suspected, diagnosed or established preterm labour
<34 weeks (consider if 34-36 weeks)
•	Respiratory support:
o	High flow oxygen
Via nasal cannula or incubator oxygen If >2L per min humidify oxygen
o	CPAP
o	Invasive ventilation
Give surfactant
•	Medications:
o	Dexamethasone
    If ≥ 8 days old and on ventilator
o	Caffeine citrate
If ≤ 30 weeks corrected gestational age. Start within 3 days of birth Consider if preterm and apneic
o	Nitric oxide
    Only if pulmonary hypoplasia or pulmonary hypertension

Question 2

Downs syndrome 3 Ix and 6 referrals incl. 1 support group

Answer 2

• Take blood for genetics and request urgent PCR for trisomy 21
• Full clinical examination paying particular attention to cardiovascular system
• Echocardiogram
o If abnormal then refer to cardiologist
o Congenital heart defects very common
Occur in 45% of Down syndrome babies (mainly AVSD)
• FBC and blood film
o 10% of Down syndrome babies will have transient abnormal myelopoiesis

    Baby is higher risk of leukaemia
•	Refer to haematologist
•	MDT approach
•	Hearing screening test
•	Monitor for associated problems
o	Duodenal atresia
o	Hirschsprung disease
o	Coeliac disease
o	Hypothyroidism
o	Epilepsy
o	Hearing and visual defects
o	Periodontal disease
o	Atlantoaxial instability
•	Speech and language therapist
o	If difficulty swallowing
•	Ophthalmologist (3-6 months)
•	Parental counselling and education
o	Recommend support groups to the parents
    Charity: Down’s Syndrome Association
o	Genetic counselling
•	Early intervention therapies in childhood, refer to
o	Physiotherapy
o	Occupational therapy for fine motor skills
•	Individualised educational plan (special schools)

Question 3

Obesity. When is treatment considered? Main goal? 4 lifestyle modifications and pharmacological interventions and indications

Answer 3

• Managed in primary care, only refer for specialist paediatric assessment if there are any complications or an endogenous cause is suspected
• Treatment is considered if child’s BMI is above 98th decile
• Weight maintenance is the main goal, with BMI reduction achieved as height increases
• Lifestyle modification
o Healthier diet – regular meals, decreased portions, eating together as a family, nutrient- rich foods etc.
o Increase physical activity – aim for fun activities, family participation is encourages, structured physical activity
o Limiting television and other small screen recreational activities to less than 2 hours per day
o Counselling
• Pharmacotherapy is indicated in a small subgroup of children, over the age of 12 with extreme obesity
o Orlistat – lipase inhibitor
• Bariatric surgery is generally not considered

Question 4

Septic arthritis

Answer 4

• Prolonged course of antibiotics (initially IV for 2 weeks, followed by 4 weeks of oral antibiotics)
o Suspected Gram-positive
Vancomycin
2nd line = clindamycin or cephalosporin
o Suspected Gram-negative
3rd generation cephalosporin (e.g. ceftriaxone) 2nd line = IV ciprofloxacin
• Joint aspiration - affected joints should be aspirated to dryness as often as required (through closed needle aspiration or arthroscopically)
• Washing out of the joint or surgical drainage may be required

Question 5

Obesity organic causes 5

Answer 5

-	Organic causes:
o	GH deficiency
o	Hypothyroidism
o	Down’s syndrome
o	Cushing’s syndrome
o	Prader-Willi syndrome

Question 6

Septic arthritis vs transient synovitis criteria

Answer 6

Kocher Criteria
Distinguishing transient synovitis from septic arthritis
More likely to be due to septic arthritis if:


•	Temperature >38.5C
•	Unable to bear weight on limb
•	ESR >40
•	WCC >12

0 = not likely
1 = 3% SA risk
2 = 40% SA risk
3 = 93% SA risk
4 = 100% SA risk


Question 7

Septic Arthritis definition and aetiology

Answer 7

Septic Arthritis *

• Infectious arthritis of the synovial joint (vs osteomyelitis of bone); hip = 75% of cases
o Most common in children <2yo; often presents late

Question 8

Septic arthritis MX

Answer 8

• Prolonged course of antibiotics (initially IV for 2 weeks, followed by 4 weeks of oral antibiotics)
o Suspected Gram-positive
Vancomycin
2nd line = clindamycin or cephalosporin
o Suspected Gram-negative
3rd generation cephalosporin (e.g. ceftriaxone) 2nd line = IV ciprofloxacin
• Joint aspiration - affected joints should be aspirated to dryness as often as required (through closed needle aspiration or arthroscopically)
• Washing out of the joint or surgical drainage may be required

Question 9

Meningitis: eponymous signs

Answer 9

 Kernig’s sign – pain on leg straightening

 Brudzinski’s sign – supine neck flexion -> knee/hip flexion

Question 10

Meningitis: cushings triad

Answer 10

o Raised ICP symptoms (late signs) = Cushing’s Triad:

 High BP + Low HR + Irregular RR

Question 11

Meningitis: Ix 8

Answer 11

o LP (CT head before LP if concerns of raised ICP – not routine however) – contraindications overleaf
o Blood culture (an LP would be done before this!)
o FBC, CRP, U&E and glucose
o Coagulation profile
o Further immunological analysis (complement deficiency) if >1 episode of meningococcal meningitis

Question 12

Meningitis: under 3 months over 3 months, 4 supportive therapies, Additional things 3

Answer 12

• <3 months old:
o Antibiotics: IV ampicillin/amoxicillin + cefotaxime
• >3 months old:
o IV ceftriaxone
• Supportive therapy:
o Analgesia and antipyretics
o Oxygen: reservoir rebreathing mask, unless
o IV fluids: 0.9% NaCl + 5% dextrose
o Vasopressors if hypotensive despite fluid resuscitation
• Presenting in primary care: single dose of IM/IV benzylpenicillin
o Arrange emergency medical transfer to hospital by telephoning 999
o Note: check for penicillin allergy (in which case you might consider moxifloxacin and vancomycin)
• Recent foreign travel: add vancomycin
• Dexamethasone may be given if >3 months old and presents with these in CSF analysis:
o Frankly purulent CSF
o CSF WBC > 1000/µL
o Raised CSF WBC + protein concentration > 1 g/L
o Bacteria on Gram stain
o Note: steroids should not be used in meningococcal septicaemia
• Notify the Health Protection Unit
• Discharge and Follow-Up
o All children should be reviewed by a paediatrician 4-6 weeks after discharge
o Offer formal audiological assessment
o Treating Contacts: ciprofloxacin is preferred over rifampicin
This includes anyone who has had close contact with the patients in the 7 days before onset

Question 13

Meningitis: special medication considerations 3 and after discharge 3 things

Answer 13

o Note: check for penicillin allergy (in which case you might consider moxifloxacin and vancomycin)
• Recent foreign travel: add vancomycin
• Dexamethasone may be given if >3 months old and presents with these in CSF analysis:
o Frankly purulent CSF
o CSF WBC > 1000/µL
o Raised CSF WBC + protein concentration > 1 g/L
o Bacteria on Gram stain
o Note: steroids should not be used in meningococcal septicaemia
• Notify the Health Protection Unit
• Discharge and Follow-Up
o All children should be reviewed by a paediatrician 4-6 weeks after discharge
o Offer formal audiological assessment
o Treating Contacts: ciprofloxacin is preferred over rifampicin
This includes anyone who has had close contact with the patients in the 7 days before onset

Question 14

PACES TIPS meningitis 6

Answer 14

• Explain the diagnosis (infection of the tissues surrounding the brain)
• Explain that it is a serious condition, but we have effective antibiotics that can treat the infection
• It will require hospital admission to administer the antibiotics and monitoring
• There can sometimes be long-term complications, the most common is hearing loss, and offer formal audiological assessment as follow up
• Follow-up with paediatricians in 4-6 weeks
• Offer ciprofloxacin prophylaxis for contacts
• Support: Meningitis Now

Question 15

HSP follow up

Answer 15

o Follow-up (weekly for 1 month, 2-weekly for 2 months, 3 months, 6 months, 12 months):
 BP measurements
 Urine dipstick (haematuria)

Question 16

UC scoring system 2

Answer 16

o	Severity graded using:
	Paediatric Ulcerative Colitis Activity Index (PUCAI)	N.B. be aware of coexistent depression
•	Severe = >65 points
•	Mild-Moderate = 10-64 points
	Truelove and Witts score

Question 17

CD / UC Ix 8

Answer 17

o FBC (including iron, B12 and folate
CRP and ESR
o Faecal calprotectin
Upper GI and small bowel contrast scan
Colonoscopy and biopsy (cobblestones)
Assess impact on daily functioning (anxiety, depression)
o Stopping smoking (reduce risk of relapse)
Assess risk of osteoporosis
biopsy = non-caseating epithelioid cell granulomata

Question 18

UC Mx Mild moderate steroid dependent and severe

Answer 18

•	Severity in children is assessed using the Paediatric Ulcerative Colitis Activity Index (PUCAI)
o	Severe > 65 points
o	Moderate - 35-64 points
o	Mild – 10-34 points
o	Remission – 0-9 points

• Mild UC:
o 5-ASA
o Can be used topically (suppository or enema) initially and PO if remission not achieved within 4 weeks
o For extensive disease- topical and high-dose PO treatment is 1st line
o Continue as maintenance if no relapse
o Relapse = use oral prednisolone and taper
If they relapse with steroids, then it is called steroid dependent disease
• Moderate UC:
o Oral prednisolone for 2-4wks and taper
o If good response = treat with oral 5-ASA and continue for maintenance
o If relapse frequent, then steroid dependent disease
o If bad response to oral prednisolone, then IV can be given. Should be tapered off to oral and maintain remission.
• Steroid dependent disease:
o #1: Thiopurine or infliximab
o If successful continue with medication as maintenance
o If inadequate:
Colectomy, adalimumab or vedolizmab Colectomy is the final treatment option
• Severe UC:
o Medical emergency
o High dose IV Methylprednisolone
o Oral 5-ASA should be stopped
o Antibiotics can be used with bacteraemia
o Parenteral nutrition needed until improvement or surgery
o Surgical Treatment
Colectomy with an ileostomy or ileojejunal pouch

Question 19

UC PACES tip 7

Answer 19

• Explain the diagnosis (condition with unknown cause that leads to inflammation of the bowel, which leads to symptoms)
• Explain that it isn’t common but is a well-known disease (1 in 420
• Explain that there is no cure, and it is a condition that tends to come and go in flare- ups every so often
• Reassure that there are medications that can be used to reduce the likelihood of flare-ups and to treat flare-ups when they happen
• Explain the complications (growth issues, bowel cancer)
• Explain that they will be seen by a gastroenterologist
• Support: Crohn’s and Colitis UK

Question 20

CD Mx inducing remission, if not tolerated, immunosuppressive/biologic therapies, maintaining remission, diet

Answer 20

• Assess impact of symptoms on daily functioning (anxiety, depression)
Replace diet with whole protein modular diet – excessively liquid, for 6-8 weeks
• Encourage stopping smoking (may reduce the risk of relapse)
• Assess risk of osteoporosis
• Inducing remission:
o Corticosteroid monotherapy (prednisolone, methylprednisolone or intravenous hydrocortisone): suitable for first presentation or first inflammatory exacerbation in a 12-month period
If not tolerated, budesonide or aminosalicylates (e.g. mesalazine) may be used If concerns about growth in children/young people, consider enteral nutrition as an alternative
o May add immunosuppressive drugs (azathioprine or methotrexate)
o Or biologic therapies: infliximab and adalimumab
o If disease is limited to the terminal ileum, consider resective surgery
• Maintaining remission:
o Offer azathioprine or methotrexate Including after surgery
• Using these medical therapies requires monitoring of certain biochemical measures (e.g. ferritin, B12, calcium and vitamin D)
• Educate about features of Crohn’s flare up (e.g. unintended weight loss)
• Patients on immunosuppressive therapies should not have live vaccines. They are at increased risk of influenza and pneumococcal infection so should receive these vaccines.

Question 21

Define intussusception and the most common location

Answer 21

• Invagination of proximal bowel into distal component; 95% ileum through to caecum through ileocecal valve (ilio-colic)

Question 22

o Most common cause of obstruction in infants of the neonatal period – 3 months to 2 years old (rarely <3m)

Answer 22

Intussusception

Question 23

Mx of intussusception

Answer 23

• ABCDE approach
• IV fluids and NG tube aspiration may be needed
• Unless there are signs of peritonitis, reduction of the intussusception by rectal air insufflation
(with fluoroscopy guidance) is usually attempted by a radiologist
o Success rate is 75%
o Remaining 25% require an operation
• Clinically stable with no contraindications to contrast enema reduction
o Fluid resuscitation
o Contrast enema (air or contrast liquid e.g. Barium or Gastrograffin)
o Contraindications
Peritonitis Perforation Hypovolaemic shock
o Broad-spectrum antibiotics
Clindamycin + gentamicin OR tazocin OR cefoxitin + vancomycin
o 2nd line: surgical reduction with broad-spectrum antibiotics
• If recurrent intussusception - consider investigating for a pathological lead point (e.g. Meckel’s diverticulum)

Question 24

Foreign body inhalation Mx conscious 5 unconscious 3

Answer 24

• Conscious
o Encourage coughing
o External maneuvers
Back blows (x 5)
Abdominal thrusts (Heimlich) (x 5)
• NOTE: these should NOT be done on infants and very young children
• Chest thrusts for infants (x 5)
o Removal of foreign body
1st line: Flexible bronchoscopy or rigid bronchoscopy
• Rigid bronchoscopy is preferred in cases of stridor, asphyxia, radio- opaque object seen on CXR, a history of foreign body aspiration associated with unilateral decreased breath sounds, localising wheeze, obstructive hyper-inflation, or atelectasis
• In all other cases, flexible bronchoscopy should be performed to confirm the diagnosis
• This is usually done with conscious sedation or general anaesthesia 2nd line: surgery, thoracotomy

• Unconscious
o Secure the airway immediately (endotracheal intubation)
Unless the foreign body can be seen and removed from the upper airway May need to do a cricothyroidotomy

o Removal of foreign body (as above)

Question 25

MMR counselling

Answer 25

Counselling I am glad you came in to discuss these things with me, I can see that you really want to do the best for your child and it is really difficult with so much information online and no way to police it, especially forums. I want to start by talking about finns development. I think that he is doing very well. Every child is different and that is why there are such variations in the time that we expect children to achieve different milestones. In terms of his walking, we would expect him to walk any time now and only look into this further if it gets to 18 months. The same is true of him feeding himself and saying 6 words with meaning. He was also born a bit early so we have to remember that when we look at him as an overall person. So in summary, for his development I don’t see anything to be concerned about and he seems like a very happy boy I want to address your concerns about the MMR vaccine. Can you tell me exactly what you understand about it and the link to autism? This link was first introduced by a man called Andrew Wakefield. He published a study that suggested a link between the vaccine and autism. However, the paper had a number of faults in it. In fact the paper has since been retracted, disproved by a number of large studies and the council that checks doctors are acting ethically actually took away his licence so he isn’t allowed to practice medicine in the UK any more. The specific issues with his study was that it only looked at 12 cases. He also had a lot to benefit from this – he had a patent for MMR alternative vaccines which means that if people used these vaccines more he would earn more money. He also didn’t include some of his data in his publication, this data went against his conclusions. There have since been larger studies which have disproved the link between autism and any link between MMR and bowel disease. The vaccine has never had mercury in it. There are sometimes additives put into the vaccine in tiny doses that are checked to be safe. The reason they are added is because they help the immune system see the virus and build a stronger level of immunity towards it. The concept of herd immunity is true however WHO say that 95% of people have to receive the MMR vaccine to ensure herd immunity. This has to include those who have weak immune systems that can’t tolerate a vaccine. In fact in 2018 only 91% of babies had received the vaccine so herd immunity doesn’t apply here unfortunately. Why MMR is important Protects against three illnesses in 2 separate doses. It protects against measles mumps rubella. These have a lot of nasty complications but the most worrying is called encephalitis. This is an infection of the brain tissue. Side effects of MMR - Ill for a few days – mild temperature

Question 26

Obesity Mx 5 and 1 Pharmacological

Answer 26

Most obese children can be managed in primary care. MDT approach Diet NICE does not recommend using a dietary approach alone.  Tailor any dietary changes to individual preferences. Allow a flexible, individual approach.  Do not recommend unduly restrictive, nutritionally unbalanced diets.  It may be helpful to keep a food diary (assists cognitive approach). Do not forget snacks and drinks.  It is very unpleasant being hungry and, rather than just cutting back on all food, it may be easier to move to a diet with less fat and more fibre in it.  NICE advises that calorie intake should be below energy expenditure, but gives no specifics about diet or numbers of calories. It emphasises the general benefits of healthy eating.  There may be occasions where there is benefit in referral to a dietician, particularly where there is a large amount of weight to be lost and caloric cut has to be balanced by adequate nutrition for ongoing developmental needs.  This is not easy for the patient and it is important to be positive and reinforcing. Exercise  The value of exercise is more than just the calories expended in the session. It tends to increase basal metabolic rate and, after vigorous exercise, metabolism is stimulated for the subsequent 36 hours. It has cardiovascular benefit, and reduces the risk of diabetes. It also promotes a sense of well-being.  Overweight children often shun exercise because of poor mobility, ready fatigue and "being no good at games". It is important to discuss the options to find something appropriate and sustainable. The age and aptitudes of the individual must be taken into account. It must be something that the individual will enjoy or he or she will not persevere. This is very important, as the ethos of exercise is not just for the duration of weight loss but for life.  NICE recommends a total of 60 minutes of at least moderate exercise each day (in one session, or more, shorter sessions lasting a minimum of 10 minutes). Overweight children may need more than 60 minutes.  Exercise need not always be 'formal' - walking, using stairs, cycling and active play all count.  Reduce time spent on inactive pursuits, such as watching television and playing video games.  It is very helpful to involve all the family in development of an active lifestyle ``` Behavioural therapy should involve  Stimulus control  Self-monitoring  Goal setting  Rewards for reaching goals  Problem solving ``` Pharmacological  Orlistat is the only licensed obesity drug in the UK – pancreatic lipase inhibitor  Reserved for children 12 years or older if co-morbidities present and lifestyle management is not sufficient Bariatric surgery is only considered once child has reached physical/psychological maturity , in extreme cases with complications and all other methods have failed

Question 27

Pyloric stenosis Mx 2

Answer 27

• IV fluid resuscitation o This is essential to correct the fluid and electrolyte disturbance before surgery o This should be provided at 1.5 x maintenance rate with 5% dextrose + 0.45% saline o Add potassium once urine output is adequate • Definitive treatment is by performing a Ramstedt pyloromyotomy o This involves dividing the hypertrophied muscle down to but not including the mucosa o Can be open or laparoscopic

Question 28

GOR Mx breast 2 formula 3 and positional 2

Answer 28

Gastro-oesophageal Reflux • Reassure o It is very common o Begins early (< 8 weeks) and may be frequent o It usually becomes less frequent with time o Treatment and investigation are not usually needed • Review infant or child if: o Projectile regurgitation o Bile-stained vomit or haematemesis o New concerns (e.g. faltering growth, feeding difficulties) o Persistent, frequent regurgitation beyond the first year of life • Same day referral if: haematemesis, melaena or dysphagia present • Initial Management o If breastfed: Carry out a breastfeeding assessment If issue persists despite advice, consider trial of alginate therapy for 1-2 weeks (stop at intervals to check whether the infant has recovered) o If formula-fed: Review feeding history Reduce feed volumes if excessive for infant’s weight (aim for 150-180 mL/kg/day) FIRST: Offer a trial of smaller, more frequent feeds SECOND: Offer a trial of thickened formula or anti-regurgitant formula THIRD: Offer alginate therapy without feed thickeners if the above hasn’t worked (stop at 2-week intervals to see if the infant has recovered) Trials are recommended to last 1-2 weeks o Positional: Advise about upright positioning after feeds and avoiding overfeeding Prone and left-lateral positioning helps but should be used when awake Do NOT use positional management in a sleeping infant (they should sleep on their back) o Pharmacological Management Consider 2-4-week trial of PPI or histamine antagonist in children who have 1 or more of the following: • Unexplained feeding difficulties (refusing feeds, choking) • Distressed behaviour • Faltering growth • No resolution respite 1-2 week trial of alginate therapy Consider specialist referral if still no resolution • Last Resort Options o Enteral feeding (if failure to thrive) o Nissen fundoplication The fundus of the stomach is wrapped around the intra-abdominal oesophagus Abdominal or laparoscopic procedure • If the child fails to respond to these measures, other diagnoses e.g. cow’s milk protein allergy should be considered

Question 29

Autism Mx 5 aspects

Answer 29

• Psychosocial Interventions o Aim to increase attention, engagement and reciprocal communication o Adjust to the child’s developmental level o Aim to increase carers’ and teachers’ understanding of patient’s patterns of communication and interaction o Include techniques to expand the child’s communication, interactive play and social routines o Consider CBT if patient has anxiety and has the verbal and cognitive ability to engage in therapy. • Speech and Language Therapy o Social skills training • Pharmacological o Consider antipsychotic medication if behavioural issues are making psychosocial interventions ineffective Review at 3-4 weeks Stop at 6 weeks if no clinical indication o Treat any comorbidities, e.g. ADHD according. See ‘ADHD’ section above • Families and Carers o Offer personal, social and emotional support o Offer practical support in the caring role (respite breaks and emergency plans) o Plan for the future (e.g. transition to adult care) o Offer carer’s needs assessment • Educational o Assess for learning disability o If needing extra support, discuss EHC plan (legal document that describes a child’s special education, health and care needs)

Question 30

PACES Autism tips 7

Answer 30

• Explain that autism is a spectrum, so it is difficult to predict the extent of the impact on the child’s life • Explain that it is characterised by difficulties in social interaction, language impairment and ritualistic behavioural tendencies • Explain that management involves: o Psychological interventions to reduce ritualistic behaviours o Speech and language therapy (with a focus on social skills) o Educational assessment and plan • Explain that the carer’s needs will also be attended to and link them to support websites: o National Autistic Society

Question 31

Down's 4 Ix 8 Mx

Answer 31

• Take blood for genetics and request urgent PCR for trisomy 21 • Full clinical examination paying particular attention to cardiovascular system • Echocardiogram o If abnormal then refer to cardiologist o Congenital heart defects very common Occur in 45% of Down syndrome babies (mainly AVSD) • FBC and blood film o 10% of Down syndrome babies will have transient abnormal myelopoiesis ``` Baby is higher risk of leukaemia • Refer to haematologist • MDT approach • Hearing screening test • Monitor for associated problems o Duodenal atresia o Hirschsprung disease o Coeliac disease o Hypothyroidism o Epilepsy o Hearing and visual defects o Periodontal disease o Atlantoaxial instability • Speech and language therapist o If difficulty swallowing • Ophthalmologist (3-6 months) • Parental counselling and education o Recommend support groups to the parents Charity: Down’s Syndrome Association o Genetic counselling • Early intervention therapies in childhood, refer to o Physiotherapy o Occupational therapy for fine motor skills • Individualised educational plan (special schools) ```

Question 32

Sign in Duchennes

Answer 32

o Gower’s sign – the need to turn prone to rise

Question 33

Duchenne Mx 9

Answer 33

• Physiotherapy helps prevent contractures • Exercise and psychological support are necessary • Tendoachilles lengthening and scoliosis surgery may be required • Glucocorticoids (e.g. prednisolone) may help delay wheelchair dependence • Ataluren is a drug that restores dystrophin synthesis and has conditional licensing for patients 5 years and over • Dietician for gastric feeding indicated in some patients. Vitamin D and calcium supplementation may be necessary to prevent and treat bone fragility. • Weakness of intercostal muscles may lead to nocturnal hypoxia o This presents with daytime headache, irritability and loss of appetite • Overnight CPAP may be indicated for respiratory support • If the left ventricular ejection fraction drops, cardioprotective drugs (e.g. carvedilol) and left ventricular assist devices may be considered

Question 34

• Choice of Antiepileptic Drug

Answer 34

• Choice of Antiepileptic Drug o Generalised Tonic-Clonic • 1st line: Sodium valproate for boys and girls who are not of childbearing potential. Otherwise, offer lamotrigine o Alternatives: carbamazepine, oxcarbazepine o NOTE: these can exacerbate myoclonic (lamotrigine) and absence (carbamazepine and oxcabazepine) seizures • Adjunctive treatment: clobazam, lamotrigine, levetiracetam, valproate, topiramate Absence • 1st line: ethosuximide (preferred for girls of childbearing potential) or valproate o Alternative: lamotrigine • Adjunctive treatment: consider a combination of 2 of these 3 - ethosuximide, lamotrigine, valproate Myoclonic • 1st line: valproate o Alternatives: levetiracetam, topiramate • Adjunctive treatment: levetiracetam, valproate, topiramate o Focal 1st line: carbamazepine, lamotrigine (preferred for girls of childbearing potential) • Alternatives: levetiracetam, oxcarbazepine, valproate Adjunctive therapy: carbamazepine, clobazam, gabapentin, lamotrigine, oxcarbazine, valproate, topiramate

Question 35

Epilepsy Mx 2 + 4 advice

Answer 35

* Urgently refer all patients suspected of having a first epileptic seizure to a neurologist (first fit clinic) * Epilepsy specialist nurse may assist families by providing education and continuing lifestyle advice • Advice o Advise parents and carers how to recognise a seizure o Advise parents to record any future episode of possible seizures (e.g. by video) o Advise that the patient avoid dangerous activities until the diagnosis is confirmed (e.g. swimming, bathing) o Advise the parent to seek help if another seizure occurs before the referral • Decisions on treating epilepsy is dependent on the risk of recurrence, how dangerous or impairing the seizures are and how upsetting further seizures would be to the patient's life • Treatment is NOT usually given for childhood rolandic epilepsy • Treatment of childhood absence epilepsy is aimed at maximising their educational potential and supporting social development • Antiepileptic Drug (AED) Therapy o Not all children with epileptic seizures require AED o Decisions to treat should be based on seizure type, epilepsy type, frequency and social/educational consequences against the possibility of unwanted effects of antiepileptic drugs o Choose an appropriate AED for the seizure and epilepsy (e.g. carbamazapine can make absence and myoclonic seizures worse) o Monotherapy should be used, at the minimum dosage required to prevent seizures, to reduce the risk of adverse effects o All AEDs have potential adverse effects o AED levels are not checked regularly but may be measured to check adherence o Children with prolonged epileptic seizures (convulsive seizures with loss of consciousness > 5 mins) are given rescue therapy to keep with them This is usually buccal midazolam o AED therapy may be discontinued after 2 years free of seizures • Choice of Antiepileptic Drug o Generalised Tonic-Clonic • 1st line: Sodium valproate for boys and girls who are not of childbearing potential. Otherwise, offer lamotrigine o Alternatives: carbamazepine, oxcarbazepine o NOTE: these can exacerbate myoclonic (lamotrigine) and absence (carbamazepine and oxcabazepine) seizures • Adjunctive treatment: clobazam, lamotrigine, levetiracetam, valproate, topiramate Absence • 1st line: ethosuximide (preferred for girls of childbearing potential) or valproate o Alternative: lamotrigine • Adjunctive treatment: consider a combination of 2 of these 3 - ethosuximide, lamotrigine, valproate Myoclonic • 1st line: valproate o Alternatives: levetiracetam, topiramate • Adjunctive treatment: levetiracetam, valproate, topiramate o Focal 1st line: carbamazepine, lamotrigine (preferred for girls of childbearing potential) • Alternatives: levetiracetam, oxcarbazepine, valproate Adjunctive therapy: carbamazepine, clobazam, gabapentin, lamotrigine, oxcarbazine, valproate, topiramate

Question 36

Cerebral palsy RF ante peri post natal 3 5 2

Answer 36

• Risk Factors o Antenatal: Chorioamnionitis, maternal respiratory or GU infection o Perinatal: Preterm birth, LBW, neonatal encephalopathy, neonatal sepsis, maternal infection (e.g. respiratory or genito-urinary) o Postnatal: Meningitis, head trauma prior to 3 years

Question 37

• Signs of Cerebral Palsy 8

Answer 37

o For children at risk: provide clinical and developmental follow-up programme by an MDT for children up to 2 years o Possible early motor features of CP Unusual fidgety movements or abnormality of movement (including asymmetry or paucity of movement) Abnormalities of tone (includes hypotonia, spasticity or dystonia (fluctuating tone)) Abnormal motor developing (including late head control, rolling and crawling) Feeding difficulties o Delayed motor milestones (correct for gestational age) Not sitting by 8 months Not walking by 18 months Hand preference before 1 year o Refer all children with persistent toe walking

Question 38

• RED FLAGS for other neurological disorders not CP 6

Answer 38

o Absence of risk factors o Family history of progressive neurological disorder o Loss of already attained cognitive or developmental abilities o Development of unexpected focal neurological signs o MRI findings suggestive of progressive neurological disorder o MRI findings not in keeping with CP

Question 39

CP MDT 10

Answer 39

• Multi-Disciplinary Team (MDT) o Main Members: paediatrician, nurse, physiotherapist, occupational therapist, speech and language therapist, dietetics, psychology o Supplementary Members: orthopaedics, orthotics, visual and hearing

Question 40

CP Mx 7

Answer 40

• Summary o Physiotherapy – encourage movement, improve strength and stop muscles from losing range of motion o Speech therapy – improve language abilities o Occupation therapy – identify everyday tasks that may be difficult and help make these tasks more accessible o Medication • Stiffness – baclofen, diazepam • Sleeping – melatonin • Constipation – laxatives • Drooling – anticholinergic

Question 41

Counselling CP 5

Answer 41

* Explain the diagnosis (damage to the brain that would have occurred early in development) * The damage to the brain doesn’t get worse, but the way it manifests will change as the child gets older * Refer to paediatrician specialising in developmental disorders * Long-term management will include physiotherapy, speech and language therapy and special educational needs * Medications can also be given to help with symptoms

Question 42

Bronchiolitis Mx 4

Answer 42

``` • Respiratory management o Humidified oxygen If saturation is persistently < 92% o CPAP If impending respiratory failure ``` o Upper airway suction Do not perform routinely Perform if upper airway secretions are causing respiratory distress or feeding difficulties o Fluids By nasogastric/orogastric tube if they cannot take enough fluid by mouth Give IV fluids if cannot tolerate nasogastric or orogastric fluids or have impending respiratory failure

Question 43

Atypical UTI Ix 5

Answer 43

o Renal USS o DMSA scan (Tc-99m) – detect scarring & functional defects, sensitive so only >2m after last UTI (gives false +ve) o MCUG (Micturating Cystourethrogram) – visualise anatomy, see VUR and urethral obstruction, not past infancy o MAG3 renogram (Tc-99m) – dynamic scan to see MAG3 excreted from blood into urine – use furosemide  See graph image o Genetic karyotyping -> Potter sequence (a sequence – renal problems -> oligohydramnios -> problems seen)

Question 44

Atypical UTI signs 6

Answer 44

o Atypical UTI:  Poor UO Abdominal/bladder mass Raised creatinine  Sepsis Failure to respond <48 hours Infection with non-E. coli organisms

Question 45

Ix atypical UTI

Answer 45

o 1st -> renal USS for…  All children with an atypical UTI USS during acute infection  Recurrent UTI; <6m USS during acute infection  Recurrent UTI; >6m USS urgent USS (<6w)  First UTI; <6m, responds to treatment (<48 hours) USS urgent USS (<6w) o 2nd -> DMSA scan for… (cannot be <2m after last UTI)  All children with a recurrent UTI DMSA routine (4-6m)  Atypical UTI; <3yo DMSA routine (4-6m) o 3rd or VUR -> MCUG/VCUG scan for…  VUR suspected on USS Male, recurrent UTI Female, <3yo, 1st UTI  Obstruction, trauma Female, pyelonephritis, recurrent UTI Female, <5yo, febrile UTI

Question 46

Mx UTI 4 age brackets , 2 Mx for stable and unstable

Answer 46

Neonates and infants <6wks All to receive IV ampicillin and gentamicin or cefotaxime All to be admitted to hospital after full sepsis screen Oral therapy after clinical response + blood/CSF culture being negative ``` Infants >6wks to <2yrs Oral cefixime for 7-10d (Co-amoxiclav as an alternate) IV ceftriaxone for 7-10d If symptoms don’t resolve then IV ampicillin and gentamicin can be used ``` Children 2-13yrs Oral cefixime for 7-10d (Co-amoxiclav as an alternate) IV ampicillin and gentamicin for 7-10d If symptoms don’t resolve then IV cefepime can be use Adolescents >13yrs Oral cefixime for 3d IV ampicillin and gentamicin for 3d If symptoms don’t resolve then IV cefepime can be used

Question 47

G6PD Causes 4

Answer 47

o Causative drugs:  Antimalarials (i.e. quinine) Analgesics (i.e. high-dose aspirin)  Antibiotics (i.e. nitrofurantoin) Chemicals (i.e. fava beans, naphthalene moth balls)

Question 48

G6PD Mx

Answer 48

• Parents should be advised on the signs of acute haemolysis (jaundice, pallor, dark urine) • Parents should also be given a list of drugs, chemicals, and food to avoid • Acute Haemolysis o Supportive care + folic acid o Blood transfusion and renal support may be given in cases of severe anaemia with renal impairment • Neonates with prolonged hyperbilirubinaemia o Follow treatment graphs for phototherapy and exchange transfusion

Question 49

Mx brain tumour MDT 10 Mx 3

Answer 49

• Management: o MDT: paediatrician, neurologist, SN, OT, PT, SALT, psychology, radiologist, oncologist, CLIC Sargent  CLIC Sargent = Cancer and Leukaemia in Children social worker o 1st line: Surgery (maximal safe resection to obtain and extensive excision with minimal damage to the patient)  Resectability is dependent on the location, site and number of lesions  Craniotomy -> debulking (subtotal and complete resections)  Open biopsies -> inoperable but approachable tumours  Stereotactic biopsy -> open biopsy not indicated o Radiotherapy -> used for… low and high-grade gliomas, metastases o Chemotherapy -> used for… high-grade gliomas (temozolomide)  Biological agents (EGFR inhibitors, PD-1 inhibitors etc.)

Question 50

HSP MX

Answer 50

• Most cases will resolve spontaneously within 4 weeks • Joint pain can be managed using paracetamol or ibuprofen • If there is scrotal involvement or severe oedema or severe abdominal pain, oral prednisolone may be given o Rest, hydration and elevation of the affected area needed • IV corticosteroids are recommended in patients with nephrotic-range proteinuria and those with declining renal function • In rapidly progressive nephritis: o IV corticosteroids + oral prednisolone + cyclophosphamine o Renal transplant or dialysis may be considered • Follow-up to check blood pressure and renal function

Question 51

HSP S/s 4

Answer 51

o Purpuric Rash (100%)  Extensor surface of legs, arms, buttocks, ankles  Urticarial -> maculopapular; spares trunk o Arthralgia and periarticular oedema (60-80%):  Large Joints  Joint pain and swelling of knees and ankles o Abdominal pain (60%)  Colicky abdominal pain  Haematemesis, melena, intussusception o Glomerulonephritis (20-60% -> 97% within 3m of onset):  Microscopic or macroscopic haematuria  Nephrotic syndrome (rare)

Question 52

Complications of HSP 5

Answer 52

o Testicular pain (rare) Arthritis of knees o Intussusception Acute renal failure / CRF (as an adult) o Pancreatitis

Question 53

o Special cases in chicken pox 4

Answer 53

:  Admit if serious complications (e.g. pneumonia, encephalitis, dehydration) • Secondary bacterial superinfection -> sudden high fever: toxic shock, necrotising fasciitis • Encephalitis (ataxic with cerebellar signs; better prognosis than HSV-encephalitis) • Purpura fulminans -> large necrotic loss of skin from cross-activation of antiviral ABs -> inhibit the inhibitory coagulation proteins factors C and S -> increased clotting and purpuric skin rash • Dehydration (severe)

Question 54

Chicken pox general advice 5

Answer 54

• General advice: o Encourage adequate fluid intake o Dress appropriately to avoid overheating or shivering o Wear smooth, cotton fabrics o Keep nails short to minimize damage from scratching o Oral paracetamol and topical emollient (e.g. calamine lotion) to reduce itching

Question 55

Chicken pox Mx severe, neonatal, immunocompromised treatment and prevention

Answer 55

• Severe disease: o Consider oral aciclovir 800 mg 5 times a day for 7 days if adolescent (aged 14 years or older) who presents within 24 hours of rash onset • Neonatal chickenpox: o Seek immediate specialist advice • Immunocompromised children: o IV aciclovir for 7 days if they present with 24 hours of the onset of the rash OR if the chickenpox is severe o PO valaciclovir may be substituted • Prevention in Immunocompromised children: o Human varicella zoster immunoglobulin should be used in high-risk immunocompromised individuals with deficient T cell function following contact with chickenpox

Question 56

Movicol dosage in disimpaction and maintenance regimes in 3 different age brackets

Answer 56

Disimpaction Child under 1 year: ½ to 1 sachet daily Child 1 to 5 years: 2 sachets on 1st day, then 4 sachets daily for 2 days, then 6 sachets daily for 2 days, then 8 sachets daily Child 5 to 12 years: 4 sachets on 1st day, then increased in steps of 2 sachets daily to maximum of 12 sachets daily Ongoing maintenance (chronic constipation, prevention of faecal impaction) Child under 1 year: ½ to 1 sachet daily Child 1 to 6 years: 1 sachet daily; adjust dose to produce regular soft stools (maximum 4 sachets daily) (for children under 2) Child 6 to 12 years: 2 sachets daily; adjust dose to produce regular soft stools (maximum 4 sachets daily)

Question 57

Behavioural interventions in constipation 5

Answer 57

behavioural interventions o Bowel habit diary: for monitoring o Scheduled toileting: after meals and before bedtime o Reward systems/star charts: given for attempting to poo, not actually pooing o Address any anxieties about going to the toilet, staying calm and reassuring o Posture: make sure both feet are flat on the floor

Question 58

Migraine Mx acute management 4 steps

Answer 58

• Assess the severity and frequency of attacks, and the impact on the patient's life: o Quality of attacks - intensity and site of pain, associated symptoms o Timing and frequency - when they start, reason for consultation, how often they occur, temporal pattern, how long they last, time off school o Possible causes - suspected triggers or emotional problems (e.g. bullying) o Other factors - general health in between attacks • Consider using a headache diary for a minimum of 8 weeks to identify triggers • Acute Management (in 12-17 year olds) o Step 1: Simple analgesia (paracetamol or ibuprofen) Only consider aspirin if > 16 (risk of Reye’s syndrome) o Step 2: Nasal sumatriptan NOTE: oral triptans are NOT licensed in people < 18 years o Step 3: Combination therapy with nasal triptan and NSAID/paracetamol Consider adding anti-emetic e.g. metoclopramide or prochlorperazine o Arrange follow-up within 1 month, but ask them to return sooner if symptoms get worse • Prophylactic Treatment o Offer topiramate or propranolol – specialist referral required NOTE: topiramate has a risk of foetal malformations

Question 59

• Delayed puberty = absence of pubertal development by… | in males and females

Answer 59

• Delayed puberty = absence of pubertal development by… o Males, no testicular development (volume ≤4mL) by age 14 years o Females, no breast development by age 13 years OR Females, no periods by age 15 years

Question 60

Aetiology of delayed puberty 3 categories 3 in each

Answer 60

o Functional (most commonly):  Constitutional Delay of Growth and Puberty (GDGP; commonest in boys) • S/S: low bone age, no puberty signs, no organic causes • FHx; M > F – usually FHx of same delay in parent of same sex  Chronic disease, malnutrition  Psychiatric – excessive exercise, depression, anorexia nervosa ``` o Hypogonadotrophic (low LH and FSH) hypogonadism:  Hypothalamo-pituitary disorders – panhypopituitarism, intercranial tumours  Kallmann’s syndrome (LHRH deficiency and anosmia), Prader-Willi syndrome  Hypothyroidism (acquired) ``` ``` o Hypergonadotrophic (high LH and FSH) hypogonadism:  Congenital – cryptorchidism, Klienfelter’s syndrome (47 XXY), Turner’s syndrome (45 XO)  Acquired – testicular torsion, chemotherapy, infection, trauma, autoimmune ```

Question 61

Ix for delayed puberty 11

Answer 61

• Investigations: o Initial examination:  Charting (Height and weight plots, mid-parental height) and note dysmorphic features  Prader’s orchidometer (see picture) for boys; Tanner’s staging for girls o Bloods:  Gonadotrophin-dependant vs independent -> LH and FSH levels (GnRH stimulation given if <12yo)  TSH, prolactin, testosterone o Imaging -> bone age (from wrist X-ray), MRI brain o Karyotyping

Question 62

CDGP Mx

Answer 62

• Boys o 1st line: observation - most do NOT need treatment o 2nd line: short course of oxandrolone or testosterone (3-6 months) This can help increase growth velocity • Girls o 1st line: observation - most do NOT need treatment o 2nd line: short course of oestrogen (3-6 months) * Organic or permanent causes can be treated by inducing puberty using either testosterone or oestrogen * Persistent hypogonadism would require testosterone and ovarian supplementation

Question 63

CF Mx 4 aspects

Answer 63

o Pulmonary – physiotherapy, mucolytics o Infection – prophylactic antibiotics, monitoring o Nutrition – enzyme tablets, high-calorie diet, monitor growth o Psychosocial – provide support for child and carers • Δ F508 mutation o This specific mutation can be treated with lumacaftor/ivacaftor (increase CFTR protein trafficking to cell membrane) • Pulmonary complications management • Common complications: o Pneumonia o Nasal polyps o Sinusitis (prevalence increases with age) • General prophylactic management o airway clearance techniques o mucoactive agents 1st line rhDNase 2nd line hypertonic sodium chloride +/- rhDNase 3rd line mannitol dry powder for inhalation • Staphylococcus aureus pneumonia o Flucloxacillin (as prophylaxis) From diagnosis to 3rs age Can be given up to 6 yrs age • Pseudomonas aeruginosa pneumonia o Acute infection 1st line: treat as per local guidelines with oral/ inhaled antibiotics • Consider IV antibiotics if unwell o Chronic/ persistent infection 1st line: nebulised/ inhaled Colistimethate sodium • Plus oral antibiotic/ 2 IV antibiotics of different classes if clinically unwell with a pulmonary disease exacerbation 2nd line: Tobramycin DPI • Immunomodulatory agents for patients with deteriorating lung function o Azithromycin o Oral corticosteroids • Gastrointestinal complications management o Common complications: Underweight/ malnutrition Intussusception meconium ileus (affects 1 in 7 newborn babies) fat-soluble vitamin deficiencies (including vitamins A, D, E and K) o High calorie diet o Pancreatic enzyme replacement therapy (e.g Creon) Insufficiency of pancreatic enzymes can be tested with faecal elastase o H2 receptor antagonist or PPI If malabsorption persistent despite optimal pancreatic enzyme replacement therapy

Question 64

Aetiology of precocious puberty o Gonadotrophin-Dependant Precocious Puberty [GDPP] 4

Answer 64

o Gonadotrophin-Dependant Precocious Puberty [GDPP]  Premature activation of HPG axis  Idiopathic (no cause found in 80% girls and 40% boys)  CNS abnormalities (tumours, trauma, central congenital disorders)

Question 65

Aetiology of precocious puberty | o Gonadotrophin-Independent Precocious Puberty [GIPP] – 20% of PP: 6

Answer 65

 Early puberty from increased gonadal activation independent of HPG  Ovarian – follicular cyst, granulosa cell tumour, Leydig cell tumour, gonadoblastoma  Testicular – Leydig cell tumour, testotoxicosis (defective LH-R function; a familial GIPP)  Adrenal – CAH, Cushing’s syndrome  Tumours – b-hCG-secreting tumour of liver, tumours of ovary, testes, adrenals  McCune-Albright syndrome – a multiple endocrinopathy of thyrotoxicosis, Cushing’s, acromegaly • S/S: polyostotic fibrous dysplasia, café-au-lait spots, ovarian cysts  Exogenous hormones – COCP, testosterone gels

Question 66

Ix of previous puberty General 4 Males 3 Females 1

Answer 66

o Females [normally not of concern] → pelvic USS  Premature onset of normal puberty -> multicystic ovaries and enlarging uterus  Rule out gonadal tumour, cysts o Males [organic cause] → examination of testes, MRI (intracranial tumours), GnRH-stimulated LH/FSH  Most commonly has an organic cause • N.B. if LH and FSH are normal, any virilisation has a primary cause (i.e. adrenal hyperplasia) • CAH -> initial growth spurt (tallest in class) -> premature bone fusion (smallest in class)  Prader’s orchidometer measurement and examination of testes: • Bilateral enlargement → GDPP (intercranial lesion; i.e. optic glioma in NF1) • Unilateral enlargement→ gonadal tumour • Small testes → tumour or CAH (adrenal cause) o General investigations:  GOLD-STANDARD: GnRH stimulation test – suppressed LH/FSH if G-independent • FSH, LH low = GIPP -> other tests… • FSH, LH high = GDPP -> other tests…  Wrist XR (non-dominant) for skeletal age  General hormone profile -> basal LH/FSH, serum testosterone and oestrogen  Urinary 17-OH progesterone if CAH suspected

Question 67

Mx precocious puberty dependent and independent

Answer 67

• Gonadotrophin-Dependent Precocious Puberty o 90% in females has NO identifiable cause o Manage associated brain neoplasms (e.g. optic nerve gliomas) o GnRH agonist (e.g. leuprolide) can suppress puberty via negative feedback o GH therapy (as GnRH agonists can stunt growth) o Cryproterone (anti-androgen) is used by specialists • Gonadotrophin-Independent Precocious Puberty o McCune Albright or Testotoxicosis: ketoconazole or cyproterone, GnRH agonist, aromatase inhibitors o Congenital Adrenal Hyperplasia: adjustment of hydrocortisone therapy, GnRH agonist o Tumours: specialist referral

Question 68

Wilms associations

Answer 68

o Beckwith-Wiedemann syndrome (specific body parts overgrow; usually presents at birth; islet cell hyperplasia) o WAGR syndrome (Wilm’s tumour, Aniridia, Genitourinary malformations, (mental) Retardation) o Hemihypertrophy o 33% with a loss-of-function mutation in the WT1 gene on chromosome 11

Question 69

Pneumonia | Immediately refer for hospital admission if: 7

Answer 69

o Persistent SpO2 < 92% on air o Grunting, marked chest recession, RR > 60/min o Cyanosis o Child looks seriously unwell, does not wake, or does not stay awake if roused or does not respond to normal social cues o Temperature > 38 degrees in a child < 3 months o Consider admission if: dehydration, decreased activity, nasal flaring, predisposing diseases (e.g. chronic lung disease) o Whilst awaiting hospital admission Give controlled supplemental oxygen if SpO2 < 92%

Question 70

Pneumonia Mx hospital admsission and non

Answer 70

• If hospital admission is not needed: o Most children can be managed at home Bacterial and viral pneumonia are difficult to differentiate so all children should be given antibiotics. o Amoxicillin is first-line for 5 days for low-moderate severity If penicillin allergic, give clarithromycin Macrolides can be added at any stage if there is no response to first-line treatment o Co-amoxiclav for high severity Add clarithromycin if atypical organism suspected o Paracetamol or ibuprofen can be used as antipyretics o Keep adequate hydration o Seek medical advice if RR increases, dehydration occurs or worsening of fever • If admission required: o PO antibiotics if tolerated. Otherwise, IV and review after 48h to consider switching to PO.