Paeds - Management Flashcards

Question

Cerebral palsy other things you need to treat ``` o Low bone mineral density o Pain/discomfort/distress o Sleep disturbance o Mental health problems o GORD o Chronic constipation o Epilepsy ```

Answer 1

``` o Low bone mineral density  Assess dietary intake of calcium + vitamin D  Active movement programme  Active weight bearing  Nutritional support, calcium + vitamin D supplementation  If low-impact fracture • DEXA scan • Consider bisphosphonates ``` o Pain/discomfort/distress  Causes – MSK problems,  muscle tone (dystonia, spasticity), muscle fatigue + immobility, constipation, vomiting, GORD  Treat reversible causes  In the absence of an identifiable cause of pain – Consider a stepped approach trial of simple analgesia (e.g. paracetamol +/or ibuprofen) for mild to moderate pain o Sleep disturbance  Optimise sleep hygiene  Manage treatable causes - OSA, seizures, pain, need for repositioning bc of immobility, poor sleep hygiene, night-time interventions, hunger, thirst  Trail of melatonin o Mental health problems  Refer o GORD – refer to specialist o Chronic constipation – laxatives o Epilepsy – anticonvulsants

Answer 2

• Children w RF for development of CP | o Enhanced clinical + developmental follow-up programme by MDT for children up to 2y (corrected for GA)

Answer 3

o Urgent assessment  If at increased risk of developing CP  If abnormal features  If new or worsening seizures

Answer 4

if red flags for other neurological disorders other than CP are present  Absence of known RF  FHx pf progressive neurological disorder  Loss of already attained cognitive or developmental abilities  Development of unexpected focal neurological signs  MRI findings suggestive of a progressive neurological disorder/ not in keeping with clinical signs of cerebral palsy

Answer 5

o refer to dietician (1st line), refer for enteral tube feeding (2nd line)

Answer 6

refer To a specialist for treating dysphagia  Specialist MDT may consider video fluoroscopy  Create individualised plan for managing eating, drinking and swallowing (e.g. postural management, modifying textures, feeding techniques and equipment)

Answer 7

Emollients * E45 * Cetraben * Diprobase * Aveeno

Answer 8

* Emollients * Mild potency topical steroids with continued treatment until >48hrs after flare has been controlled * Routine follow-up not normally needed Mild topical CS • Hydrocortisone 1%

Answer 9

* Emollients * Moderate potency topical corticosteroids with continued treatment until >48hrs after flare has been controlled * Consider maintenance regimen of topical CS to control areas of skin prone to frequent flares * Topical calcineurin inhibitors (e.g. tacrolimus) – 2nd line * If severe itching/urticaria – 1/12 trial non-sedating antihistamine * If flare + sleep disturbance + >6m – 7-14d sedating anti-histamines * If itching * Bandages Moderate topical CS • Betamethasone valerate 0.025% • Clobetasone butyrate 0.05% Non-sedating antihistamines • Cetirizine • Loratadine • Fenoxfenadine Sedating antihistamines • Chlorphenamine

Answer 10

* Emollients * Potent topical corticosteroids with continued treatment until >48hrs after flare has been controlled * Prescribe a maintenance regimen of topical CS * Systemic therapy – If severe extensive eczema causing psychological distress – consider a course of CS PO • Topical calcineurin inhibitors (e.g. tacrolimus) – 2nd line • Phototherapy if other options have failed • Non-sedating/sedating antihistamine Cetirizine Loratadine Fexofenadine • Bandages Potent topical CS • Betamethasone valerate 0.1% • Mometasone

Answer 11

o Identify + education of triggers (e.g. food allergens, contact allergens, inhalational allergens, irritants like soap) o Avoid scratching (keep nails short, use of anti-scratch mittens)

Answer 12

o Treatment should be started as soon as signs + symptoms appear o Treatment should be continued for approx. 48h after symptoms subside

Answer 13

o E45, cetraben, diprobase, aveeno o Basis of management o Frequent + liberal use – in large amounts + often o Unperfumed emollients for moisturising, washing, bathing instead of soap/ detergent-based wash products/ shampoos (in children <12m) o Should be used on the whole body both when atopic eczema is clear + while using all other treatments  Different products should ideally be applied one at a time with several minutes between applications where practical  Preferences of child + parent or cares should determine which product should be applied first o Smooth emollients onto skin rather than rubbing them in

Answer 14

o Benefits outweigh possible harms when they are applied correctly – explain that they are topical and not systemic o Should be prescribed for applications only OD or BD o Only a short-course is required  Duration can vary from 3-14 days depending on how long the skin takes to respond  Better to use 1-2 weeks short course to clear up eczema than let a child suffer for months o Only apply to areas of active atopic eczema (or eczema that has been active within the past 48h), which may include areas of broke skin o If a mild or moderately potent topical CS has not controlled atopic eczema within 7-14 days  Exclude secondary bacterial or viral infection  >12m – use potent topical corticosteroids • For as short time as possible • No >14 days • Do not use on the face or neck • Should not be used in children <12m without specialist dermatological supervision • If this does not control atopic eczema  review diagnosis  refer child for specialist dermatological advice o Face and neck  Mild potency  Moderate potency for severe flares but short-term use (3-5 days) o Axillae + groin  Moderate potency  Potent for short periods only (7-14 days) o Do not use without specialist dermatological advice  Potent CS in children <12m  Very potent preparations in children o Controlled eczema but frequent flares (2 or 3 per month)  Treat problem areas with topical CS for 2 consecutive days per week to prevent flares instead of treating flares as they arise  Review within 3-6m to assess effectiveness o If topical CS deemed ineffective/ Tachyphylaxis (= appearance of progressive decrease in response to a given dose after repetitive administration)  consider a different topical CS of the same potency before increasing potency o If severe + extensive + causing psychological distress – oral CS o CS  Mild Potency – hydrocortisone 1%  Moderate Potency – betamethasone valerate 0.025%, clobetasone butyrate 0.05%  Potent – betamethasone valerate 0.1%, mometasone Apply thinly to affected area only might cause skin hypopigmentation

Answer 15

o Mild atopic eczema – not recommended o 2nd line treatment  Only in children >2  If eczema has not been controlled by topical CS (= disease that has not shown a satisfactory clinical response to adequate use of the maximum strength + potency that is appropriate for the patient’s age + the area being treated)  Or where there is a serious risk of important adverse effects from further topical CS use, particularly irreversible skin atrophy o Only apply to areas of active atopic eczema, which may include areas of broke skin o Should not be used under occlusion (bandages, dressings) o Topical tacrolimus  Moderate to severe atopic eczema  >2y/o o Pimecrolimus  2nd line treatment  Moderate atopic eczema on the face and neck  2y-16y o If facial atopic eczema requires long-term or frequent use of mild topical CS – consider stepping up treatment to topical calcineurin inhibitors

Answer 16

o Infected eczema - Occlusive medicated dressings + dry bandages should not be used o Chronic lichenified atopic eczema OR flares  Localised medicated dressings or dry bandages with emollients and topical CS  7-14 days  May be impregnated with zinc paste +/- tar paste  Worn overnight or for 2-3 days at a time until skin has improved o Wet stockinette wraps  Also used with diluted topical steroids + emollients mixed in for 7-14 days o Whole body (limbs and trunk) occlusive dressings (incl. wet wrap therapy)  Should not be used as first line  Should only be initiated by a HCP trained in their use  … with CS should only be used to treat atopic eczema in children for 7-14 days – can be continued with emollients alone until the atopic eczema is controlled

Answer 17

o Should not be used routinely in the mx of atopic eczema o If severe itching/ urticaria  Offer 1-month trial of a non-sedating antihistamine  Treatment can be continued while symptoms persist  Review every 3 months o If acute flare of eczema and sleep disturbance  7-14 day trial of an age-appropriate sedating antihistamine  Children >6 months  Treatment can be repeated during subsequent flares if successful Non-sedating antihistamines • Cetirizine • Loratadine • Fenoxfenadine Sedating antihistamines • Chlorphenamine

Answer 18

o Take viral (HSV) + bacterial (staph, strep) swabs from wet papules o Good hygiene when using emollients and other creams  Use spatula  Do not leave them open  Parent should obtain new supplies of topical atopic eczema medications after treatment for infected atopic eczema – products in open containers can become contaminated with microorganisms + act as a source of infection o 1st line  Flucloxacillin (oral if extensive, topical if local)  Erythromycin or clarithromycin (if penicillin allergic)  <2 weeks o Recurrent infections  Antiseptics – chlorhexidine – can be used to decrease bacterial load  Do not use in the long term topical steroid remains the first-line treatment for infected eczema. https://dermnetnz.org/topics/topical-steroid

Answer 19

 If a child’s infected atopic eczema fails to respond to treatment with abx + an appropriate CS  If lesion on skin suspected to be HSV – acyclovir PO even if infection is localised

Answer 20

 Admit  Oral acyclovir ASAP  Referral for same day specialist dermatological advice  If secondary bacterial infection suspected (golden crust in vesicles) – systemic antibiotics (e.g. IV clindamycin for MRSA)  If around eyes – refer for same day ophthalmological + dermatological advice  If sepsis suspected – IV ceftriaxone

Answer 21

• Phototherapy + systemic treatments o For the treatment of severe atopic eczema when other management options have failed or are inappropriate + where there is a significant negative impact on QOL

Answer 22

o Not adequately assessed in clinical studies o Topical CS are deliberately added to some herbal products intended for use in children with atopic eczema o Liver toxicity has been associated with the use of some Chinese herbal medicines intended to treat atopic eczema o Inform HCP if they are using/intend to use complementary therapies – should keep using using emollients as well – regular massage with emollients may improve the atopic eczema

Answer 23

• Eczema herpeticum (+ peri-orbital skin involvement)  immediate (same day) dermatologist referral (+same day ophthalmologist referral) • Urgent (within 2 weeks) referral for specialist dermatological advice if o Severe atopic eczema that has not responded to optimum topical therapy after 1 week o Treatment of bacterially infected atopic eczema has failed • Refer if o Diagnosis is/has become uncertain o Mx has not controlled atopic eczema satisfactorily (e.g. child having 1-2 flares per month or is reacting adversely to many emollients) o Atopic eczema on the face has not responded to appropriate treatment o Child/carer may benefit from specialist advice on treatment application (e.g. bandaging techniques) o Contact allergic dermatitis is suspected (e.g. persistent atopic eczema or facial/eyelid/hand atopic eczema) o Atopic eczema giving rise to significant social or psychological problems for the child or carer (e.g. sleep disturbance, poor school attendance) o Atopic eczema is associated with severe recurrent infections esp. deep abscesses or pneumonia o Psychological advice – if psychosocial wellbeing as not improved after eczema improvement o If moderate or severe atopic eczema + suspected food allergy – specialist ix + mx of atopic eczema + allergy o Children with eczema + failure to thrive  should be referred for specialist advice relating to growth

Answer 24

* Dietician for gastric feeding indicated in some patients * Early screening + Regular monitoring for cardiac complications • Ataluren o Recommended for treating DMD resulting from a nonsense mutation in the dystrophin gene o Restores dystrophin synthesis o Has conditional licensing for patients >5 who can walk ``` Initial management • Information + support for the family • Genetic diagnosis + counselling • Referral to a specialist + MDT • Immunisations – influenza + pneumococcal ``` With few exceptions, all generalised muscle disease can be managed using principles for managing DMD • Cardioprotective drugs • Respiratory muscle aids • Corticosteroids

Answer 25

• CS o Prednisolone or deflazacort o Improves muscle strength + function in the short term o May help delay wheelchair dependence – Can delay loss of independent ambulation by approx. 3 years o Intermittent dosing to avoid complications (every other day or 10 days on, 10 days off) • Physiotherapy + exercise + psychological support o To reverse musculotendinous contractures o To strengthen muscles o Hamstring and Achilles tendon lengthening o Cessation of regular exercise result sin quick fall-off in strength to baseline or below o Psychological support  Discourage over-protection of the patient to encourage maturation  Encourage goal-orientated activities  Facilitate adherence by introducing + planning further therapeutic option * Knee-foot-ankle orthoses may help prolong walking * Serial casting of ankles – may prevent need for surgical release of Achilles tendon • Surgery for contractures o If musculotendinous contractures • Optimise bone health o Vitamin D + Ca supplementation may be necessary to prevent + treat bone fragility o Bisphosphonates if vertebral fracture occurs • Air stacking + maximal insufflations o If contractures of the lungs and chest wall o Air stacking – receiving consecutively delivered volumes of air to fill the lungs beyond the inspiratory capacity with the goal of approaching the predicted inspiratory capacity • Cardioprotective drugs o If LVEF <40-50% o BB + ACEi  Carvedilol + perindopril or lisinopril o Spironolactone o Anticoagulation if severe cardiac impairment – at risk of thromboembolism o Typically results in an increase in LVEF of 20-40% • Cardiac defibrillator o Once LVEF decreases <20% - risk of CHF + sudden death

Answer 26

• Supportive therapies to maintain ADL o Standard and motorised wheelchairs o If minimal hip + knee contractures  standing motorised wheelchairs • Psychological support • Respiratory care o Respiratory function tends to decline after the child needs a wheelchair o Monitoring e.g. FVC, cough peak flows, pulse oximetry o Treat chest infections promptly o PT o IPPV o MAC • Maintenance of optimal nutrition o Might require indwelling gastrostomy tubes • Air stacking + maximal insufflations o If contractures of the lungs and chest wall • Surgery for scoliosis o If spinal curvature >40 o Bracing  Ineffective as scoliosis in DMD is caused by collapsing spine caused by increasing paraspinal muscle weakness • Cardioprotective drugs o If LVEF <40-50% • Cardiac defibrillator o Once LVEF decreases <20% - risk of CHF + sudden death * Support + adaptations for school * Counselling and support with adolescence

Answer 27

• Inspiratory + expiratory respiratory muscle assistance o IPPV – intermittent positive pressure ventilation  Can be used in both the daytime + for nocturnal rest or support  As patients weaken + require more ventilatory support, they continue nocturnal IPPV into daytime hours + eventually depend on it 24h a day o For patients who are symptomatic for nocturnal hypoventilation with some combination of fatigue, morning headaches, daytime drowsiness, dyspnoea o MAC – mechanically assisted coughing – to clean airway secretions o “unweanable” DMD patients can be extubated to continuous, full setting, non-invasive ventilatory support + use mechanically assisted coughing to clear airway secretions • Maintenance of optimal nutrition o DMD patients become wheelchair dependent between 7-12 years of age o Due to limb weakness + musculotendinous contractures • Psychological support • Air stacking + maximal insufflations o If contractures of the lungs and chest wall • Cardioprotective drugs o If LVEF <40-50% • Cardiac defibrillator o Once LVEF decreases <20% - risk of CHF + sudden death * Respite care for the family * Palliative care * Planning ahead + end-of life directives – deterioration can occur suddenly in later stages of DMD

Answer 28

• Hormonal therapy (CS or ACTH) or Vigabatrin – 1st line o Prednisolone (CS) – 1st line o Tetracosactide (ACTH) o Vigabatrin (anticonvulsant)  1st line in children with tuberous sclerosis • Switch to alternative first line therapy – 2nd line • Pyridoxine or pyridoxal phosphate – 3rd line o If refractory to treatment + unknown aetiology • Alternative anticonvulsant or ketogenic diet – 4th line o Valproic acid, BZD (clonazepam, nitrazepam), topiramate, levetiracetam • Surgery if focal operable lesion – indications o Infantile spasm refractory to medical management o Focal EEG abnormalities o Focal abnormality on neuroimaging o No evidence of metabolic or degenerative disease • AN genetic testing may be available for future pregnancies if a previous child with infantile spasms has been diagnosed with a specific genetic disorder

Answer 29

* Child systemically unwell and/or features suggesting severe dehydration and/or progression to shock * Intractable or bilious vomiting * Acute-onset painful, bloody diarrhoea in previously healthy children or confirmed shiga toxin producing Escherichia coli (STEC) infection 0157 * There is a suspected serious complication – HUS, sepsis

Answer 30

* Clinical features suggest a potentially life threatening/serious alternative diagnosis * Inadequate response to ORS / child can’t take ORS orally * Red flag symptoms/signs despite use of ORS * RF for developing dehydration * Parents unable to monitor the child’s condition or to provide appropriate management safely at home

Answer 31

• Rehydration (oral/NG ORS or IVF depending on degree of dehydration) over 4h + o In severe dehydration this is given as a bolus over 1h • Fluid compensation with ORS solution after each episode of D+V + • Maintenance fluids with ORS solution over 24h o Both ongoing losses and maintenance fluids are calculated together and given evenly over 24h

Answer 32

First 10kg 100ml/kg 4ml Second 10kg 50ml/kg 2 ml Each kg thereafter 20 ml/kg 1ml

Answer 33

* 50ml/kg body weight for fluid deficit replacement over 4h * Maintenance volume ORS solution * BF can continue * Do not give oral fluids other than ORS solution

Answer 34

• 200ml ORS after each loose stool in addition to the child’s normal fluid intake

Answer 35

• 200-400ml ORS solution after every loose stool

Answer 36

• 5ml/kg ORS after each watery stool to prevent recurrence of dehydration

Answer 37

• Amount of ORS per episode of D+V o <10kg – 60-120mL ORS o >10kg – 120-240mL ORS • Daily maintenance requirement • Age-appropriate diet continued o Continue BF or other milks o Encourage fluid intake o Discourage the drinking of fruit juices/carbonated drinks o Offer oral rehydration salt (ORS) solution to those at  risk of dehydration

Answer 38

o Low-osmolarity ORS (240-250 mOsm/l) for oral rehydration therapy  Frequently + in small amounts  Consider giving it via NG tube if they are unable to drink/vomit persistently  Give it to rehydrate children (incl. those with hypernatremia), unless IVF is indicated • 50mL/kg ORS over 4hrs • Amount of ORS per episode of D+V o <10kg – 60-120mL ORS o >10kg – 120-240mL ORS • Daily maintenance requirement with ORS solution • Age-appropriate diet continued o Supplementation with their usual fluids if they refuse to take ORS + do not have red flag symptoms o Monitor response o If symptoms/signs suggesting shock  immediate transfer to secondary care

Answer 39

• Oral rehydration therapy not contraindicated o 100mL/kg ORS over 4hrs (oral or via NG) o Amount of ORS per episode of D+V  <10kg – 60-120mL ORS  >10kg – 120-240mL ORS • Oral rehydration therapy contraindicated* o IVF – deficit should be replaced in 4h • Daily maintenance requirement • Age-appropriate diet continued

Answer 40

* Medical emergency * Immediate hospital admission + IV resuscitation • IVF rehydration 20ml/kg over 1h normal saline or Ringer’s lactate o IV bolus may be given until haemodynamically stable o Monitor + Reassess vital signs on a regular basis • Daily maintenance requirement • Rehydration therapy per episode of D+V (oral or IV) o <10kg – 60-120mL ORS (oral), IVF (IV) o >10kg – 120-240mL ORS (oral), IVF (IV) * Age-appropriate diet continued once stable * Or BF infants, BF to be continued throughout, even during the initial rehydration phases

Answer 41

o Shock is suspected or confirmed o Child with red flag symptoms/signs shows clinical evidence of deterioration despite oral rehydration therapy o Child persistently vomits the ORS solution, given orally or via NG tube

Answer 42

o Suspected/confirmed shock – rapid IV infusion of 20ml/kg of 0.9% NaCl over 5-10 mins o If child remains shocked  Immediately give another rapid IV infusion 20ml/kg at 0.9% NaCl solution and  Consider possible causes of shock other than dehydration o When symptoms and/or signs of shock resolve after rapid IV infusions  start rehydration with IVF therapy o If IVF required for rehydration (+ child not hypernatremic at presentation)  Isotonic solution for both fluid replacement + maintenance – 0.9% NaCl or 0.9% NaCl + 5% glucose  If required initial IVF boluses for suspected/confirmed shock – add 100ml/kg for fluid deficit replacement to maintenance fluid requirements  No shock at presentation – add 50ml/kg for fluid deficit replacement to maintenance fluid requirements

Answer 43

 Obtain urgent expert advice on fluid management  Isotonic solution for both fluid replacement + maintenance – 0.9% NaCl or 0.9% NaCl + 5% glucose  Replace the fluid deficit slowly – typically over 48h  Monitor plasma sodium frequently – reduce it at a rate of less than 0.5 mmol/h

Answer 44

o Attempt early + gradual introduction of oral hydration therapy during IVF therapy o If tolerated, stop IVF + complete rehydration wit oral rehydration therapy o Continue BF, offer more frequent feedings o Do not give solid foods o In children with red flag symptoms/signs – do not give oral fluids other than ORS solution o In children without red flags/signs  Do not routinely give oral fluids other than ORS solution  Consider supplementation with the child’s usual fluids if they consistently refuse ORS solution

Answer 45

o Give full strength milk straight away o Encourage BF + other milk feeds o Encourage fluid intake o Reintroduce the child’s usual solid food o Avoid giving fruit juices + carbonated drinks until the diarrhoea has stopped o Restart oral rehydration therapy if dehydration recurs after rehydration o Nutritional intake needs to be increased after diarrhoeal illness • Children at increased risk of dehydration (see complications) – consider giving 5ml/kg of ORS solution after each large watery stool

Answer 46

o Not routinely required to treat gastroenteritis (even if the cause is bacterial) o Consider abx if  Suspected/ confirmed septicaemia  Extra-intestinal spread of bacterial infection  <6m with salmonella gastroenteritis  Malnourished/immunocompromised with salmonella gastroenteritis  C. difficile associated pseudomembranous enterocolitis, giardiasis, dysenteric shigellosis, dysenteric amoebiasis, cholera

Answer 47

o Antidiarrhoeal drugs o Antiemetics- prochlorperazine CI in children <2y due to risk of respiratory depression, or in children <9kg o Zinc supplements o Probiotics

Answer 48

o Wash hands with soap in warm running water – after going to the toilet (children) / changing nappies, before preparing, serving, or eating food o Towels used by infected children should not be shared o Children excluded from school until 48h after the last episode of D+V o Children should not swim in swimming pools for 2 weeks after the last episode of diarrhoea o Seek medical advice if  Symptoms/signs of dehydration  Red flag symptoms – get immediate help  Any new features requiring stool cultures – blood, mucus, pus in stool (?dysentery)  Symptoms that do not resolve within the expected timeframe • D – 5-7d, in most it stops within 2w • V – 1-2d, in most it stops within 3d o Give advice listed above o Sources of information and support – NHS leaflets, patient.info leaflets

Answer 49

• Seek specialist orthopaedic opinion • 1st line – Observation o <2m with normal physical examination + mild dysplasia without instability on IS  Serial examinations and US on a monthly basis o Neonate with hip subluxation  Observation for up to 3 weeks (bmj) (or 6 w in specialties guide + patient.info) – most will experience spontaneous resolution  If >3w + persistent subluxation  treatment for dislocated hip ``` • 2nd line – Hip abduction orthosis (splint) + further evaluation at 6 months o If dysplasia persists or worsens o Spint/Pavlik harness  Keeps hip flexed and abducted  Left in place at all times  Enhances optimum hip development  CI in children older than 4.5-6 months when the hip is irreducible o F/U – Plain XR at 6 months of age ```

Answer 50

• 3rd line – Surgery o Indications  Children who have failed splinting  Children >6m o Reduction with spica casting (with adductor or psoas tenotomy to decrease adduction contracture) o Formal closed reduction under GA with orthographic confirmation with spica casting – 1st line o Open reduction with spica casting  If closed reduction has failed  If child >18m o Cast immobilisation should be continued until hip stability has been achieved (around 3-4 months) o Then, transition from cast to splint o Discontinuation of splint decided by the orthopaedist o Femoral/pelvic osteotomy may be performed at the time of an open reduction

Answer 51

• Safe swaddling can prevent DDH o Infants hips should be positioned in slight flexion + abduction during swaddling o Knees should also be maintained in slight flexion o Avoidance of forced or sustained passive hip extension + adduction in the first few months of life – essential for proper hip development

Answer 52

• 4th line – salvage osteotomies o For older children, >6y, where there is little potential for remodelling and hips are not amenable to open reduction surgery + reconstruction o Increase the weightbearing surface of the joint – decrease potential for degenerative changes

Answer 53

• Non-surgical treatment – Benign, Self-limiting • Acute pain o Paracetamol or ibuprofen o Ice packs o Protective pad over the tibial tubercle o Short period of rest o Followed by period of limited activities and/or sports until the pain resolves and hip motion is restored o If severe symptoms – abduction plaster • Activity continuation + monitoring o Up to 7 years • Physical therapy o Stretching of the quadriceps + hamstring muscles o Strengthening of the quadriceps o Encourage hip abduction ``` • Surgical containment o 5-12 years o If pt fail to respond to conservative treatment o Bring an increased area of femoral head articular cartilage under the weight-bearing portion of the acetabulum = ensures maximal contact between the immature femoral head and the acetabulum during the period of growth o Operative treatments include  Femoral or pelvic osteotomy  Valgus or shelf osteotomies  Hip arthroscopy  Hip arthrodiastasis (controversial) ``` • Salvage procedure o If over >12 (limited re-modelling potential) o Address either the acetabulum to recreate or deepen the socket or aim to improve congruence between the weight-bearing areas by altering the femoral head orientation with a femoral valgus osteotomy – aim to increase the load bearing area by attempting to improve congruence • Education about exacerbations and management

Answer 54

Self limiting o Activity modification  Do not usually need to stop all sporting activity – reduction in activity may be sufficient to control pain  If mild pain + no weakness – can continue sport but seek advice if sx worsen  If person is having difficulty tolerating their usual exercise • Modify exercise for a limited period to a level needed to decrease the symptoms to an acceptable level o Reduce duration/frequency/intensity of exercise o Change type of exercise to activities that avoid/limit the amount of running + jumping (e.g. swimming, cycling) requiring powerful quadriceps contraction if they cannot tolerate normal activity o As symptoms decrease, gradually  exercise as tolerated  If person cannot tolerate modified exercise programme * As symptoms decrease, introduce low-impact quadriceps exercises before gradually increasing the intensity of the exercise * If symptoms recur – advise them to stop exercises or reduce their intensity – person can gradually re-establish exercise or increase exercise intensity on the basis of their symptoms o Cold pack/compress to anterior knee – 10-15 mins up to TDS, incl. after exercise o NSAIDs – ibuprofen, naproxen o Protective knee pads – may relieve pain when kneeling o Physiotherapy referral  Started after acute symptoms abate  Stretching  Strengthening exercises to strengthen the quadriceps and hamstrings and iliotibial band as well as improve flexibility about the knee  Reducing muscle imbalance of the quadriceps, hamstrings, calf muscles, iliotibial band • Education and advice o Signpost to sources of information --> useful stretches o Reassure that symptoms usually resolve over time – within a few months, can take longer – may persist until the end of the growth spurt (14-18 years of age) o Importance of preventative strategies  Regular quadriceps + hamstring stretching  Cross training  Stretching before and after exercise o Gradual and judicious increase in intensity and duration of athletic activity

Answer 55

• Refer for specialist assessment to a paediatrician/orthopaedic surgeon if o Symptoms do not improve/worsen despite appropriate mx o Sx persist into adulthood

Answer 56

• Surgery o If conservative management fails  Progressive or late stage  Persistence of pain into late adolescence or adulthood o Excision of the affected part of the tibial tubercle to relieve symptoms o Should not be performed until after the patient reaches skeletal maturity (around 25 years of age)

Answer 57

• Mild + self-limiting • Admission o Persistent or severe headache or fever o Myoclonus with sleep disturbances o Confusion, weakness, lethargy, drowsiness, irritability, generalized seizures, coma (?encephalitis) o Signs of significant dehydration – signs of dehydration:  U/O, lethargy, cold peripheries, reduced skin turgor • Self-care o Adequate fluid intake o Soft diet if mouth ulcers are painful + foods that are hot/spicy/salty/acidic may cause oral pain o Paracetamol or ibuprofen to reduce fever + pain o Topical anaesthetics – lidocaine topical for small, sparse mouth ulcers o Keep blisters clean + apply non-adherent dressings to erosions • Advice o Measures to reduce the risk of transmission o Do not necessarily need exclusion from school o F/U not routinely required o Safety netting – Seek medical advice if  Person becomes dehydrated/ more unwell  Oral ulcers persist for >3w • Do not prescribe antibiotics/antiviral medication

Answer 58

``` Exposure • Child - Reassure if o Exposure to chickenpox not significant o Have had a hx of chickenpox o Are known to be immune to chickenpox ``` NICE CKS Neonates • Seek immediate specialist advice regarding further management • Give advice to the parents/carers about contact with other people Children • Admit if serious complications o Pneumonia o Encephalitis o Dehydration o Bacterial superinfection (High temperature (particularly after initial improvement), Redness + pain surrounding the chickenpox lesions) o Purpura fulminans (acute, often fatal thrombotic disorder which manifests as blood spots, bruising, discolouration of the skin resulting from coagulation in small blood vessels within the skin + rapidly leads to skin necrosis + DIC) * <14  acyclovir not recommended for otherwise healthy children with chickenpox * >14 presenting within 24h of rash onset  Acyclovir PO 800mg 5x day for 7/7 o improves the time to healing of cutaneous lesions + decreases duration of fever in adolescents and adults • Symptomatic treatment o Adequate fluid intake to avoid dehydration o Dress appropriately to avoid overheating or shivering o Wear smooth, cotton fabrics o Keep nails short to minimise damage from scratching  Paracetamol if pain or fever are causing distress (avoid NSAIDs) - not licensed for use in children <2m  Topical calamine lotion to alleviate itch  Chlorphenamine for treating itch associated with chickenpox if >1 year • Advice o Most infectious period is 1-2 days before the rash appears o Infectivity continues until all the lesions are dry + have crusted over (usually about 5 days after the onset of the rash) o Person to avoid contact with  School  Immunocompromised people e.g. those receiving cancer treatment or high doses of oral steroids, or those with conditions that reduce immunity  Pregnant women  Infants <4w o Children should be kept away from school or nursery until all the vesicles have crusted over o Safety netting – seek urgent medical advice if condition deteriorates or complications develop (see above) o Offer sources of information Immunocompromised children • IV acyclovir for 7 days if they present within 24h of rash onset or if the chickenpox is severe • PO valaciclovir may be substituted • Prevention o Human varicella zoster immunoglobulin – in high-risk immunocompromised individuals with deficient T cell function following contact with chickenpox

Answer 59

• Notifiable disease – notify the HPU (health protection unit) o Notification should be based on clinical suspicion + should not await laboratory confirmation • Admission o Sx of mumps encephalitis (altered level of consciousness, loss of consciousness, focal neurological signs, seizures) o Sx of mumps meningitis (severe headache, neck ache, high fever, lethargy, vomiting) • Advice o Mumps is usually a self-limiting condition o Will resolve in 1-2 weeks with no long term consequences o Antibiotic treatment is not required o Self care – rest, drink adequate fluids o Paracetamol or ibuprofen for symptomatic relief (aspirin only if >16) o Apply warm or cold packs to the parotid glands – may ease discomfort o Stay off school or work for 5 days after the initial development of parotitis o Find out immunisation status of close contacts + tell them to watch out for symptoms of mumps o Safety netting – seek medical advice if you develop symptoms of  Meningitis – severe headache, vomiting, neck stiffness (urgent attention if altered consciousness or convulsions)  Epididymo orchitis – swollen painful testicles • Follow up o 1 week after the onset of parotitis o Check that symptoms have resolved/are resolving adequately o Ensure person is UTD with immunisations o If following epididymo-orchitis (particularly if bilateral) – man has an abnormal semen analysis or is experiencing infertility – seek specialist advice or refer to a fertility specialist • Contacts o Ask about immunisation status of close contacts o Offer MMR vaccine to people who are not fully immunised (have not had 2 doses of the MMR) o Advise person to seek advice if symptoms develop • Pregnancy o Manage pregnant women the same way as otherwise healthy people o MMR vaccine is contraindicated in pregnancy • Immunocompromised o If no hx of mumps exposure + not fully immunised  MMR to people +/- moderate (but not severe) immunosuppression

Answer 60

• Prevention – vaccine • Notifiable disease – notify the HPU (health protection unit) o Notification should be based on clinical suspicion + should not await laboratory confirmation • Admission – if person develops serious complications of measles o Pneumonia o Neurological problems e.g. convulsions, encephalitis o Children with fever who are otherwise at serious risk o Respiratory support can be given if pneumonia or neurological support in case of encephalitis • Suspected measles advice o Self-limiting, unpleasant symptoms will usually resolve over the course of about a week o Self-care – rest, adequate fluids, paracetamol or ibuprofen for symptomatic release (no aspirin if <16) o Stay away from school/work for at least 4 days after initial development of rash (ideally until full recovery to reduce the risk of infective complications) o Avoid contact with susceptible people o Find out immunisation status of close contacts + tell them to watch out for symptoms of mumps o Safety netting – seek urgent medial advice if signs and symptoms of measles complications  SOB  Uncontrolled fever  Convulsions or altered consciousness o Provide written advice about measles o Ribavirin may be of use in immunocompromised patients • Vitamin A o PO, for 2 days o Recommended in all serous cases of measles + <2 • F/U o Not always necessary o Consider contacting the person about a week after the rash to ensure that symptoms have resolved/are resolving adequately o When the person has sufficiently recovered from the acute symptoms – encourage them to undergo any outstanding vaccinations • Contact management o Determine risk – immunisation status + whether they have had significant contact with a possible case of measles o If person is susceptible to measles infection + is not <1/immunosuppressed/pregnant + has no other contraindications  Offer immediate vaccination if no CI  MMR should be given within 3 days of contact with a possible case of measles + repeated after an interval of at least 1 month

Answer 61

Pulmonary mx • Prophylactic – physiotherapy, mucolytics o Airway clearance techniques (physiotherapy)  Offer training ^  Give salbutamol before airway clearance  Moderate/severe lung disease + cannot clear their lungs using standard airway clearance techniques  consider using non-invasive ventilation o Mucolytic agents  Offer to people with CF + clinical evidence of lung disease  rhDNase (dornase alfa; recombinant human deoxyribonuclease) – 1st line  Hypertonic sodium chloride +/- rhDNase – 2nd line  Mannitol dry powder for inhalation – 3rd line • Pulmonary infection – prophylactic antibiotics, monitoring o Staph aureus  Prophylactic flucloxacillin – from dx to 3y, consider continuing up to 6y  If on prophylactic abx but resp. sample positive for Staph aureus • Start treatment dose abx • Restart prophylaxis after treatment o Pseudomonas aeruginosa  Acute infection • Commence eradication therapy (PO or IV abx.) + inhaled abx • Follow this with an extended course of PO + inhaled abx  Chronic/persistent infection 1st line • Nebulised colistimethane sodium  If eradication therapy unsuccessful 2nd line • nebulised aztreonam, nebulized tobramycin or tobramycin DPI o Immunomodulatory agents – if deteriorating lung function or repeated pulmonary exacerbations  Long-term treatment with azithromycin – 1st line  Oral CS – 2nd line  Do not offer ICS

Answer 62

Pulmonary monitoring • Review children at least every 8/52 • At each routine review o Clinical assessment – review of clinical hx + medicines adherence, physical examination with measurement of weight + length/height o O2 sats. measurement o Respiratory secretion samples for microbiological ix o Spirometry  If normal – consider measuring lung clearance index • At each annual review o In addition to everything carried out in the routine reviews: o Physiotherapy assessment o CXR o Blood tests – WCC, CRP, aspergillus serology, serum IgE • During or after an exacerbation of lung disease – monitor treatment by assessing whether the symptoms + signs have resolved o Respiratory secretion samples for microbiological ix o Spirometry o O2 sats.

Answer 63

• Monitor growth + optimise nutrition o Monitor stooling habits, incl. quantity + quality + presence of GORD • Weight loss/ inadequate weight gain o  calorie intake ( portion size + eating high-energy foods) – 1st line o Oral nutritional supplements – 2nd line o 3rd line  Supplementation with enteral tube feeding or  Short-term trial of an appetite stimulant (up to 3m) • Exocrine pancreatic insufficiency o Oral pancreatic enzyme replacement  Creon, pancreatin = lipase + protease + amylase  Given before snacks and meals o H2 receptor agonist or PPI  For people with persistent symptoms/signs of malabsorption despite optimal pancreatic enzyme replacement therapy  Provides a more alkaline environment for pancreatic enzyme supplemental therapy, thus improving enzyme function • Fat soluble vitamins – ADEK o Vitamin A + ergocalciferol/colecalciferol + alpha tocopherol + phytomenadione • Meconium ileus = in neonate • DIOS = after neonatal period o Involves blockage of the small intestines by thick stool due to abnormal salt + water balance in the intestine o DIOS is similar to constipation, but the blockage is higher up in the intestines * PO or IV fluids * NG decompression (in meconium ileus) * Diatrizoate meglumine + diatrizoate sodium solution (Gastrografin) (PO or enteral tube) – 1st line * Iso-osmotic polyethylene glycol + electrolyte (PEG) solution (macrogols) (PO or enteral tube) – 2nd line * Surgery – 3rd line • To reduce risk of distal intestinal obstruction syndrome recurring o Encourage people to drink plenty of fluids o Optimise pancreatic enzyme replacement therapy o Regular treatment with stool-softening agent e.g. lactulose, PEG solution

Answer 64

• Meconium ileus = in neonate • DIOS = after neonatal period o Involves blockage of the small intestines by thick stool due to abnormal salt + water balance in the intestine o DIOS is similar to constipation, but the blockage is higher up in the intestines * PO or IV fluids * NG decompression (in meconium ileus) * Diatrizoate meglumine + diatrizoate sodium solution (Gastrografin) (PO or enteral tube) – 1st line * Iso-osmotic polyethylene glycol + electrolyte (PEG) solution (macrogols) (PO or enteral tube) – 2nd line * Surgery – 3rd line • To reduce risk of distal intestinal obstruction syndrome recurring o Encourage people to drink plenty of fluids o Optimise pancreatic enzyme replacement therapy o Regular treatment with stool-softening agent e.g. lactulose, PEG solution

Answer 65

• If LFTs abnormal o Liver US o Ursodeoxycholic acid treatment (stop if LFTs return to normal + no evidence of liver disease (clinical assessment, US)) • If ursodeoxycholic acid is stopped – monitor for liver disease re-emergence • Refer to liver specialist if o Persistently abnormal LFTs despite treatment with ursodeoxycholic acid o Chronic progressive liver disease – based on clinical assessment, US liver, LFTs o Liver failure o Portal hypertension, haematemesis, splenomegaly

Answer 66

• ΔF 508 mutation homozygous o >2 years – Lumacaftor/ivacaftor ( CFTR protein trafficking to cell membrane) o >6 years – Tezacaftor/ivacaftor o >12 years – Elexacaftor/ Tezacaftor/ivacaftor heterozygous/homozygous

Answer 67

o Positive/ equivocal sweat test result o Normal results but assessment suggests CF o >1 CF mutations revealed on gene testing

Answer 68

o Information about their condition, management options, comorbidities, care pathway o Online support groups specialist psychological support o Inform them of the risk of cross-infection + how to avoid it o Immunizations – annual influenza vaccine • Offer information on genetic counselling if considering having more children

Answer 69

• Care for pt w CF should be provided by specialist CF MDT based at specialist CF centre o Can provide outreach care o Specialist CF centre should have a point of contact available 24/7 for urgent enquiries from people wit CF + their family members/carers o Specialist CF centre should have access to social workers  Help with adjusting to long-term treatment  Education  Employment  Government benefits  Respite care ``` • MDT o Specialist paediatricians o Social workers o Specialist nurses – coordinate care, act as advocates for people with CF + their carers o Specialist physiotherapists o Specialist dietitians o Specialist pharmacists o Specialist clinical psychologists ```

Answer 70

• Comprehensive annual review o Pulmonary assessment o Assessment of nutrition + intestinal absorption o Assessment for liver disease - LFTs o Testing for CF related diabetes, from 10 years of age o Assessment of other potential or existing CF complications o Psychological assessment o Assessments by specialist nurse, physiotherapist, pharmacist, social worker o Review of their exercise programme

Answer 71

``` • Routine reviews o <1m – weekly o 1-12m – every 4/52 o 1-5y – every 6-8/52 o >5 – every 8-12/52 o Adults – every 3-6/12 ```

Answer 72

o Stimulant laxatives = senna, bisacodyl, sodium picosulfate, docusate sodium (NICE) o Osmotic laxative = lactulose, Movicol (Movicol) o Bulk forming = fybogel, methylcellulose o Stool softner = arachis oil, docusate dosium

Answer 73

• Polyethylene glycol 3350 + electrolytes = Movicol (macrogol) ``` o 1st line o Faecal disimpaction o Osmotic laxative o Using an escalating dose regimen* o With dietary/lifestyle modification o Adjust the dose according to symptoms + response ``` * If Movicol doesn’t lead to disimpaction after 2 weeks  Add a stimulant laxative (Senna, 2nd line) * If stools are hard  add lactulose or docusate • Disimpaction treatment failure o If all PO medications for disimpaction have failed  sodium citrate enemas o If <1y + dx of idiopathic constipation that does not respond to optimum treatment within 4 weeks  urgent referral for DRE o If optimum treatment with oral + rectal medications has failed  manual evacuation of bowel under anaesthesia ``` • Summary – disimpaction o Movicol (osmotic laxative)  senna  sodium citrate enema  phosphate enema  manual evacuation under anaesthetic ```

Answer 74

o Paediatric formula  <1y/o – ½-1 sachet daily  1-5 y/o – 2 sachets (daily, 1 day)  4 sachets (daily, 2 days)  6 sachets (daily, 2 days)  8 sachets (daily)  5-12 y/o – 4 sachets on 1st day  increased in steps of 2 sachets daily to a maximum of 12 daily o Adult formula  12-18 y/o – 4 sachets on 1st day   increased in steps of 2 sachets daily to a maximum of 8 daily o Review pt. undergoing disimpaction within 1 week

Answer 75

o Started if impaction not present/has been treated o Osmotic laxative (Movicol) + lifestyle/dietary modification o Children with a history of disimpaction  Maintenance dose is half the disimpaction dose o Continue maintenance dose for several weeks after regular bowel habit is established o Children who are toilet training  should remain on laxatives until toilet training is well established o Once normal bowel routine established, gradually reduce dose o Do not stop medication abruptly – gradually reduce the dose over a period of months in response to stool consistency + frequency

Answer 76

o Started as soon as the child’s bowel is disimpacted + continue medication at maintenance dose for several weeks after regular bowel habit is established o Paediatric formula  <1y/o – ½-1 sachet daily  1-6 y/o – 1 sachet daily  adjust dose to produce regular soft stools (max. 4 sachets daily)  6-12 y/o – 2 sachets daily  adjust dose to produce regular soft stools (max. 4 sachets daily) o Adult formula  12-18 y/o – 1-3 sachets daily in divided doses according to response o Reassess pt frequently during maintenance treatment to ensure that they do not become reimpacted + assess issues in maintaining treatment e.g. taking medicine, toileting

Answer 77

• Do not use dietary interventions alone as first-line treatment for idiopathic constipation * Balanced diet should include – adequate fluid intake + adequate fibre * Adequate fibre = fruit, vegetables, high-fibre bread, baked beans, wholegrain breakfast cereals

Answer 78

* Scheduled toileting + support to establish a regular bowel habit – after meals, before bedtime * Maintenance + discussion of a bowel diary – for monitoring * Information on constipation * Use of encouragement and rewards systems – given for attempting to poo, not actually pooing * Posture – make sure both feet are flat on the floor

Answer 79

o Disimpaction treatment can initially increase symptoms of soiling + abdominal pain o Laxatives may have to be taken for several months o Offer point of contact with specialist HCP, incl. school nurses who can give ongoing support o Constipation to be treated with laxatives + a combination of  Dietary modifications to ensure a balanced diet + sufficient fluids are consumed • Do not use dietary interventions alone as first-line treatment for idiopathic constipation • Balanced diet should include – adequate fluid intake + adequate fibre • Adequate fibre = fruit, vegetables, high-fibre bread, baked beans, wholegrain breakfast cereals  Daily physical activity  Behavioural interventions suited to the child or young person’s stage of development • Scheduled toileting + support to establish a regular bowel habit – after meals, before bedtime • Maintenance + discussion of a bowel diary – for monitoring • Information on constipation • Use of encouragement and rewards systems – given for attempting to poo, not actually pooing • Posture – make sure both feet are flat on the floor o Give verbal information supported by written or website information in several formats about  How the bowels work  Red flag symptoms  How to take their medication  What to expect when taking laxatives  How to poo  Criteria to recognise risk situations for relapse (such as worsening of any symptoms, soiling etc.)  Importance of continuing treatment until advised otherwise by the HCP

Answer 80

o Review pt. undergoing disimpaction within 1 week o Reassess pt frequently during maintenance treatment to ensure that they do not become reimpacted + to assess adherence + response to treatment e.g. taking medicine, toileting

Answer 81

• IVF + electrolyte replacement o Fluid and electrolyte disturbance must be corrected before surgery  If alkalosis is not corrected – risk of postoperative respiratory depression o IVF should be provided at 1.5x maintenance rate + 5% dextrose + 0.45% saline o Should not contain potassium until the urine output is adequate (1-2ml/kg/h) • Ramstedt Pyloromyotomy o Must be delayed until hypovolaemia + electrolyte disturbances are corrected o Involves dividing the hypertrophied muscle down to but not including the mucosa o Can be open or laparoscopic

Answer 82

o Haematemesis (not caused by swallowed blood from nosebleed or ingested from a cracked maternal nipple) o Melaena o Dysphagia

Answer 83

• Reassure parents o See epidemiology section • Provide sourced of additional information • Keep an eye on the vomitus + note colour • Review if o Regurgitation becomes persistently projectile – requires admission o Bile stained vomiting or haematemesis – requires admission o New concerns – marked distress, feeding difficulties, faltering growth – suggest GORD + further mx will be required o Persistent, frequent regurgitation beyond the first year of life • If BF – give advice re. technique, positioning, attachment

Answer 84

• Positional o Advise about upright positioning after feeds o Avoid overfeeding o Prone + left lateral position helps but should be used when awake o Do not use positional management in a sleeping infant (they should sleep on their back)

Answer 85

Offer assessment  alginate therapy o BF assessment + advice o If symptoms persist  1-2 week trial of alginate therapy (e.g. Gaviscon Infant) o If symptoms improve --> Continue treatment + Stop at regular intervals (e.g. every 2 weeks) to assess improvement o If symptoms remain troublesome --> 4-week PPI (omeprazole suspension) or H2 receptor antagonist (ranitidine)

Answer 86

Review feeding history  smaller, more frequent feeds  thickeners  alginate therapy o Stepped care approach  Review feeding history, then  Reduce volume of feeds only if this is excessive for the child’s weight • Recommended total volume – 150ml/kg over 24 hours, 6-8 x a day  1st – Offer a 1-2 week trial of smaller, more frequent feeds (while maintaining an appropriate total daily amount of milk unless feeds are already small + frequent, then  2nd – Offer a 1-2 week trial of feed thickeners or anti-regurgitant formula – pre-thickened formula or thickener that can be added to usual infant formula o If stepped care approach is unsuccessful  Stop thickened formula  3rd – Offer 1-2 week trial of alginate therapy (Gaviscon Infant) added to formula o If symptoms improve after 1-2 week trial of alginate therapy  Continue treatment  Stop at regular intervals (e.g. every 2 weeks) in order to see if symptoms have improved + if it is possible to stop treatment completely

Answer 87

o 4 week trial of PPI or H2Ras o If symptoms do not resolve or recur after stopping treatment – refer to a paediatrician or a paediatric gastroenterologist for assessment + specialist management

Answer 88

o If symptoms remain troublesome (unexplained feeding difficulties refusing feeds, choking), distressed behaviour, faltering growth) despite 1-2w alginate therapy trial in both breastfed + formula fed infants o 4th – 4-week PPI (omeprazole suspension) or H2 receptor antagonist (ranitidine)  Omeprazole suspension is the only PPI liquid preparation available for administration to infants o Referral to a specialist for possible endoscopy if symptoms do not resolve or recur after stopping the treatment – refer to a paediatrician or a paediatric gastroenterologist for assessment + specialist management

Answer 89

o Enteral feeding (if failure to thrive) o Nissan fundoplication  Fundus of stomach is wrapped around the intra-abdominal oesophagus  Abdominal or laparoscopic procedure • If child rails to respond to these measures – consider alternative dx e.g. cow’s milk protein allergy

Answer 90

• Refer to secondary care if o Complications of impetigo – sepsis, glomerulonephritis, deeper soft tissue infection o Person is immunocompromised + infection is widespread • Consider referral or seek specialist advice if o Uncertain dx o Bullous impetigo, particularly in babies (<1y) o Have impetigo that recurs frequently o Are systemically unwell o Are at high risk of complications

Answer 91

o Explain dx + provide written info o Reassure – heals completely + without scarring o Advice re. good hygiene measures  Wash affected areas with soap + water  Wash hands regularly, in particular after touching a patch of impetigo  Avoid scratching affected areas + keep nails short  Avoid sharing towels, bathwater, face cloths and other personal care products  Thoroughly clean potentially contaminated toys + play equipment o Children and adults to stay away from school + other childcare facilities or work until lesions are healed, dry and crusted over or 48h after initiation of antibiotics o Ensure optimal treatment of any pre-existing skin conditions e.g. eczema, head lice, scabies, insect bites

Answer 92

o Hydrogen peroxide 1% cream (apply 2-3x OD for 5/7) for people who are not systemically unwell or at high risk of complications o If this is unsuitable – short course (5/7) of topical abx  Fusidic acid 2% (apply 3x/day for 5/7)  Mupirocin 2% (apply 3x/day for 5/7) [if fusidic acid resistance is suspected or confirmed]  Length of course can be increased to 7 days if required – based on clinical judgement, depending on severity/number of lesions o Safety netting – seek help if  Symptoms worsen rapidly or significantly at any time  Symptoms have not improved after completing a course of treatment

Answer 93

o Short course of topical or oral abx for people who are not systemically unwell or at high risk of complications  Topical Fusidic acid 2% (apply 3x/day for 5/7) or  Topical Mupirocin 2% (apply 3x/day for 5/7) OR  PO flucloxacillin for 5/7 or  Clarithromycin for 5/7 if penicillin allergic or  Erythromycin for 5/7 if 8-17 o Do NOT offer combination treatment with topical + oral abx o Length of course can be increased to 7 days if required o Safety netting

Answer 94

o Short course of PO abx  PO flucloxacillin for 5/7 or  Clarithromycin for 5/7 if penicillin allergic or  Erythromycin for 5/7 if 8-17 o Length of course can be increased to 7 days if required o Safety netting

Answer 95

• Follow up o If sx worsen rapidly/significantly or have not improved after 7 days/ completing a course of treatment  Reassess the person + consider alternative dx, underlying causes, previous abx use  Check compliance w treatment + hygiene measures  If topical hydrogen peroxide 1% was used offer • Localised – topical abx • Widespread – topical or oral abx  If topical abx was used, offer • Short course of oral abx and • Consider sending a skin swab for microbiological testing  If oral abx was used – consider sending a swab for microbiological testing + adjust treatment as appropriate based on swab results o If recurrent impetigo  Send a skin swab for microbiological testing and  Consider taking a nasal swab and starting treatment for decolonisation o Review the choice of abx when swab results are available and change the abx according to results using a narrow-spectrum abx if possible

Answer 96

• IVIG – 1st line o Single dose o 2g/kg single infusion over 10-12 hours o Second dose at 36-48 hours after completion of first dose if pt fails to defervesce o Reduced fever + myocardial inflammation, prevents or ameliorates cardiac sequalae o Delay immunisation with live vaccines for 3 months • CS – 2nd line o IV methylprednisolone or low dose PO prednisolone o If 2 IVIG infusions have been given without effect on the fever and/or acute inflammatory markers • Infliximab – 2nd line o In pt refractory to IVIG and methylprednisolone o TNF-α antagonist monoclonal antibody • Other immunomodulatory drug (cyclosporin, anakinra, cyclophosphamide) or plasma exchange – 3rd line • + high-dose aspirin with every treatment o Reduces risk of thrombosis o Can be continued 48-72h after the fever stops or until the ESR/CRP normalise o Switch to lose dose aspirin until 6-8 weeks from the onset of the disease o Continue past 8 weeks depending on the long-term risk of MI – low, moderate, high • Cardiology follow up o Perform at dx + routinely repeat it at 1-2w + 4-6w after treatment o If coronary artery abnormalities are significant during the acute illness – perform echo at least 2ice per week

Answer 97

• Low dose aspirin for 6-8 weeks | o Continue past 8 weeks depending on the long-term risk of MI – low, moderate, high

Answer 98

Carry out risk assessment for myocardial ischaemia + coronary artery aneurysms • Low risk – no further medications after 8w of aspirin • Moderate risk o Low dose aspirin until aneurysm regression is demonstrated o ECG + echo F/U annually ``` • High risk o Low dose aspirin long term o ECG + echo F/U 2ice a year o May need long term warfarin (INR target 2-3) o May need clopidogrel ``` Follow up • Echo – determine whether or not there have been any coronary artery complications • Severe cases with cardiac complications – PCI, CABG o PCI – intracoronary thrombolysis, balloon angioplasty, cardiac stents, ablation therapy • Long term antiplatelet/ anticoagulant therapies will be required, usually aspirin + warfarin/LMWH • Recommended in later life – long term cardiac implications

Answer 99

o In 90% of females, no cause is found o In the majority of boys, an underlying aetiology is contributory  identify + treat o Manage associated brain neoplasms (e.g. optic nerve gliomas) o If sexual abuse – change environment o Gonadotrophin releasing hormone agonists  Leuprorelin, triptorelin, goserelin (depot)  Histrelin (implantable)  Suppresses puberty via negative feedback  Continuous exposure to GnRH suppresses puberty as it is only pulsatile exposure that triggers pubertal progression – overstimulation of the pituitary gland – causes desensitisation + thereby less release of LH+FSH  +ves – improves adult height of children in children <6, suppression of puberty is reversible  -ves – uterine bleeding in girls, menopausal symptoms, reduction in bone mineral density  Stop treatment once an acceptable age of puberty is reached – gonadotrophin secretion recommences approx. 3-4 months after stopping treatment, with normal pubertal progress + fertility ``` o GH (somatotrophin)  GnRH agonists can lead to a reduction of the growth rate (reduction in GH + IGF1 concentrations) ``` o Cryoproterone (anti-androgen) is used by specialist

Answer 100

o McCune-Albright syndrome or testotoxicosis  Ketoconazole + GnRH agonist – 1st line • Ketoconazole o inhibitor of steroid synthesis, suppresses both gonadal + adrenal steroid production o SE – liver injury, adrenal insufficiency o CI in people with liver disease o If used – monitor liver + adrenal function before + during treatment • GnRH agonist  Aromatase inhibitor + anti-androgen + GnRH agonist – 2nd line • Testolactone/ Anastrozole/ Letrozole + Spironolactone/ Bicalutamide/ Cryproterone • To prevent synthesis or action of gonadal steroids • Aromatase inhibitors o Reduce growth rate + bone age advance o Prevent aromatization = conversion of androgens to oestrogens o Can cause infertility, adrenal insufficiency, kidney failure, liver dysfunction, hair loss • If aromatase inhibitors are used, anti-androgen agents may be required to reduce testosterone effects on pubic hair + genital development o CAH  Adjustment of ongoing hydrocortisone treatment + GnRH agonist • Hydrocortisone or prednisolone or dexamethasone +/- fludrocortisone o Tumours  Specialist referral + treatment  Tumours of the ovary/testes/adrenals – surgery  Human chorionic gonadotrophin tumours – combination surgery, chemo, radiation o Exogenous oestrogens or androgens  Identification + discontinuation of exogenous agent – e.g. COCP, testosterone gels, environmental exposure of oestrogens in cosmetics

Answer 101

o In GIPP, GnRH agonists are used because Prolonged sex steroid exposure in these conditions  2o GDPP (sex steroids may have a direct maturational effect on the hypothalamus + accelerate the onset of centrally mediated puberty)

Answer 102

• Treatment should be stopped once an acceptable age of puberty is reached

Answer 103

``` Monitoring during treatment • Growth • Skeletal maturity • Plasma androgens • Response to treatment  17α hydroxyprogesterone ```

Answer 104

Happens in CAH, Addison's disease due to low aldosterone • Acute (salt losing crisis) o IV saline (0.9% NaCl) – to correct hypotension + dehydration – administer 1L rapidly + a further 2-4L over the first 24h to correct hypotension o IV hydrocortisone 50-100mg IV every 6-8h for 1-3d o IV dextrose 5% to correct hypoglycaemia

Answer 105

Prenatal therapy – when a pregnant woman has classical CAH • Dexamethasone treatment to suppress fetal HPA axis + reduce the genital ambiguity of affected female infants • When both parents are carriers – risk of having an effected infant is 1 in 8 Neonatal period • 2/3 of people with classic CAH are salt losers • Neonates are particularly vulnerable to hypovolaemia, electrolyte disturbances, hypoglycaemia

Answer 106

* May not need treatment * Treatment recommended only for those with symptoms * GC treatment indicated in children with androgen excess * Adult women may need female hormones – COCP, adjuvant antiandrogen therapy (e.g. flutamide) * Do not need stress doses of hydrocortisone unless they have iatrogenic suppression of their adrenal glands by GC treatment * Treatment can be stopped when symptoms resolve

Answer 107

• Genital surgery in girls (pt w uterus + ovaries) o Feminising genitoplasty, clitoroplasty – removes the redundant erectile tissue while preserving the sexually sensitive glans clitoris o Usually delayed until puberty • Glucocorticoid o Hydrocortisone o To suppress ACTH levels (+hence testosterone) + replace cortisol o Lifelong o During periods of stress (e.g. surgery, febrile illness, shock)  2-3x the normal dose GC PO or IM  Up to 5-10x the usual daily dose may be required during surgical procedures  Carry emergency corticosteroid dosing (medical alert bracelet or necklace) o Hypoglycaemia may occur with exercise, illness or fasting   intake of carbohydrates + glucose • Mineralocorticoid – if there is salt loss o Fludrocortisone – replaces aldosterone or o Sodium chloride  To achieve adequate sodium repletion + normalisation of plasma renin activity  Used in infants with salt losing CAH  Usually not needed after the first 6-12 months of life  Additional salt intake may be needed with exposure to hot weather or with intense exercise * GH therapy (somatropin)– improves growth rate + final height, reducing height deficit * GnRH analogues (leuprorelin, histrelin)– if precocious puberty * Oral contraceptive to treat hirsutism that doesn’t improve with glucocorticoid monotherapy

Answer 108

Constitutional delay Boys • Observation + monitoring – 1st line • 3-6m of oxandrolone or testosterone – 2nd line o Oxandrolone – weak androgen, can improve growth velocity o Testosterone – activated puberty, puberty will progress spontaneously despite withdrawal of treatment Girls • Observation + monitoring • 3-6 months of oestradiol Organic (permanent cause) Boys • Pubertal induction with testosterone o Carried out gradually with increasing doses of testosterone therapy until adult levels are reached o Dose gradually increased every 6m + interval between injections is decreased until an adult dose is reached o Check serum testosterone levels 1 week after therapy to asses whether the doses are therapeutic o SE – irritability, aggression, hypersexuality Girls • Pubertal induction with oestrogen o Estradiol (PO or transdermal – transdermal avoids first pass metabolism) o Should not use contraceptive pills or patches  doses of osterogen are too high + androgenic progestogens impair optimal breast development • + cyclic progesterone after breakthrough bleeding or adequate oestrigenisation o To promote endometrial shedding • Turners o Growth hormone +/- oxandrolone  GH to be started from the time they drop off the normal growth curve until the age of 12-14 years  If dx is made late – oxandrolone to be added to promote linear growth If confirmed hypogonadism post-puberty – need lifelong HRT after induction of puberty Boys  testosterone Girls  estradiol + progesterone

Answer 109

• Patients should be managed by a specialist paediatric rheumatology MDT • Goals o Relieve immediate pain o Prevent joint damage • Intra-articular CS o Avoid if possible due to risk of growth suppression + osteoporosis * Methotrexate (in children >2y) * PT, OT, psychology * Inactivity – deconditioning, disability, decreased bone mass  encourage patients to participate in activities such as swimming, cycling

Answer 110

• 1st line o NSAID – may be give for 2 months for relief of joint pain and/or swelling if a child has low disease activity, no contractures and no poor prognostic features • If residual disease after 2 months’ treatment  intra-articular CS injections o Intra-articular CS – may be used alone o DMARD - Methotrexate • Started initially for patients with high disease activity + poor prognostic features • After initial CS Injections for pt w High disease activity but without poor prognostic features Moderate disease activity + poor prognostic features • folic acid + anti-emetics concomitantly • Avoid alcohol + pregnancy • Before starting – FBC, serum Cr, LFTs, (+ HBV, HCV if at risk of infection) Repeat measurements every 3-4 months during treatment raised ASP or ALT above 2x upper limit – temporary suspension of methotrexate – re-start after normalisation of LFTs o Supportive care • 2nd line o TNF-α inhibitor – adalimumab or etanercept • Rarely needed • Considered in pt with moderate/high disease activity + poor prognostic features after 3 months’ treatment with methotrexate at max. tolerated dose + intra-articular steroids

Answer 111

``` • DMARD o Initial therapy o Methotrexate – 1st line Children >2 y PO, IM or SC Folic acid to be given concomitantly to decrease SE Anti-emetics o Leflunomide – 2nd line o Sulfasalazine – 2nd line ``` • Supportive care o Physical + psychological o PT, OT • Biological therapy (inflammatory cytokine blockade) o Adalimumab • >=2y • If disease has not responded adequately to >1 DMARD • TNF-a inhibitor o Etanercept • >=2y • if disease has responded inadequately to or if pt is intolerant to methotrexate • TNF-a inhibitor o Tocilizumab– 1st line • >=2y • if disease has responded inadequately to previous therapy w methotrexate • IL-6 inhibitor o Tofacitinib (NICE) • >=2y • If TNFa inhibitor is not suitable or does not control the condition well enough • JAK inhibitor o Abatacept • >=6y • If disease has responded inadequately to other DMARDs incl. at least 1 TNFa inhibitor • Inhibits T cell activation • NSAID o Ibuprofen or naproxen or meloxicam o To control pain + stiffness in children with polyarticular JIA o SE – renal impairment, GI sx (N, D, constipation, abdominal pain), headache, rash • Intra-articular CS o Triamcinolone acetonide or methylprednisolone acetate o To relieve pain and/or swelling while systemic therapies take effect o Procedure after administration of Entonox or GA o Relief expected to last for at least 4 months – repeat injections every 4 months as needed • PO CS o Prednisolone o To relieve sx for up to 3 months in patients with high/moderate disease activity whilst DMARDs or biological therapies take effect

Answer 112

• 1st line o PO or IV CS o Supportive care o NSAIDs • 2nd line o Tocilizumab or canakinuab or anakinra • If inadequate response to CS + NSAIDs

Answer 113

* If acute onset or regression of previously acquired skills  consider acquired disability due to intracranial pathology * Parental concerns re. hearing + vision – should be referred to the relevant specialist * Refer to paediatrician • MDT assessment – paediatrician or child psychiatrist + allied professionals e.g. psychologist + SALT, OT, PT o Clinical psychologist – to identify the child’s cognitive ability (IQ to be compared with their chronological age) o SALT – to assess the child’s expressive + receptive language abilities + compare these with the overall level of cognitive ability – some children have a discrepancy between cognitive + language abilities (e.g. children w autism sometimes have relatively superior non-verbal skills compared with verbal performance) o OT if motor co-ordination difficulties resulting in physical problems with writing e.g. developmental co-ordination disorder, dyspraxia • Educational assistance o Can include children with disabilities in mainstream education with additional support from teachers or Learning support assistants o If cognitive disability or complex neurodevelopmental disorders – may be better provided for in specialist schooling * Family counselling * Behavioural intervention * Manage associated conditions

Answer 114

Referrals • Refer immediately  Sudden onset chorea, ataxia, dystonia for neurological assessment • Do not routinely refer children with simple motor tics that are not troublesome to the child * Referral to mental health services  if tic disorder is associated with sx. of anxiety or OCD * Referral to neurodevelopmental team  if tic disorder associated with sx. of ASD, ADHD * Referral for neurological assessment  if the tic disorder is severe

Answer 115

Bio-psycho-social approach • Screen + Manage comorbid conditions • Indicated only for those patients whose tics are interfering with activities of daily living or social interactions or who experience significant tic-related physical pain Social – 1st line • Education + support for the patient’s family + school o Reassure about benign nature of tics o Inform about tendency for tics to increase in times of stress, anxiety, excitement o Accept/ignore minor tics o Accommodate for other tics where possible by * Using objects (rugs, foam) to dampen sounds from motor tics * Replace words (saying shoot instead of shit) o Tic is an involuntary movement disorder  Child should not be punished Psycho – 2nd line • Behavioural therapy • Habit reversal therapy (HRT) – behavioural treatment used to reduce repetitive behaviours • Comprehensive behavioural intervention for tics (CBIT) • CBT • Performed by trained professionals, incl. psychologists, OT, SALT Bio • In cases of mild to moderate tics after or in addition to behavioural therapy • Do not offer medicine for motor tics in children without specialist referral and advice • Pharmacotherapy primarily alpha-2 agonists or antipsychotics o Clonidine or Guanfacine– 1st line • Screen for cardiac arrhythmias + monitor for orthostatic hypotension • Rebound hypertension can occur if clonidine is stopped abruptly or not tapered • Risk of daytime fatigue • No long-term potential SE o Antipsychotics – 2nd line • When clonidine is ineffective/poorly tolerated • Increased risk of adverse SE w antipsychotics • Aripiprazole or risperidone – 1st line • Olanzapine – 2nd line

Answer 116

* Pain relief (paracetamol or ibuprofen) * Rest + quadriceps exercises * Sometimes surgical intervention is needed (to remove intra-articular loose bodies)

Answer 117

``` Stable lesion without malalignment • Conservative management o Pain relief • Ibuprofen (<12) or Naproxen (>2) +/or • paracetamol (<12) ``` o Short term immobilisation o activity modification o protected weight bearing * Phase 1 * 4-6w immobilisation * Crutch-protected partial weight bearing * Phase 2 * 6 weeks weight bearing as tolerated * Gentle strengthening programme without immobilisation * No sport or repetitive impact activities * Phase 3 * Supervised sport readiness programme * Transchondral or retroarticular drilling in skeletally immature patients to stimulate vascular ingrowth and subchondral bone healing * Osteotomy to unload the involved compartment– if malalignment exists + the weight bearing line passes through the involved compartment * Unstable lesion without full-thickness defects of articular cartilage – loose fragments that are not fragmented  arthroscopic assisted internal fixation * Unstable lesion with full-thickness defects of articular cartilage  arthroscopy and salvage techniques

Answer 118

• Conservative management – 1st line o NSAIDs – ibuprofen or naproxen +/or paracetamol o Avoid sport or other aggravating activities for 3-6w until symptoms subside o PT once symptoms abated • Arthroscopy + surgical intervention – 2nd line o Removal of intra-articular loose bodies  If persistent or worsening symptoms despite 6w of conservative care, loose bodes, evidence of instability

Answer 119

• Conservative management o RISE o Temporarily protected weight bearing o If giving way – short term immobilisation with an orthosis o NSAIDs • Surgical intervention o If failed conservative management, intra-articular loos bodies, lesion becomes unstable

Answer 120

• If on abx – check bloods at least weekly while on IV abx to monitor for adverse effects – FBC, U+Es, LFTs, CRP Acute osteomyelitis • Admit + IV abx (High-dose empirical abx for at least 3-4 w (start IV, switch to PO)) o Take blood cultures before abx  regimen altered once results arrive o 3m-5y  IV cephalosporin (ceftriaxone) o >5y  IV flucloxacillin or IV cefazolin o If RF for atypical organisms (e.g. sickle cell disease)  ceftriaxone +/- flucloxacillin or clindamycin o If MRSA prevalence >10-15% • <2y  add clindamycin (1st line), vancomycin or linezolid (2nd line) • >2y  give clindamycin alone o If suspecting Pseudomonas infection (penetrating foot injury – walking barefoot) o Piperacillin/tanzobactam o Ciprofloxacin if allergic to penicillin • Supportive care o Immobilise affected limb o Analgesia – paracetamol or ibuprofen -1st line, morphine sulphate – 2nd line • Surgery o If the limb deteriorates or imaging suggests progressive bone destruction – surgery to prevent progression to chronic osteomyelitis o Surgical debridement if dead bone or biofilm • Although acute osteomyelitis often responds to antibiotics alone if it can be treated promptly and aggressively, once dead bone or a biofilm has become established, antibiotics alone cannot cure the infection and thorough surgical debridement is required o Can switch from IV to PO after 2-4 days if child • Has been afebrile for 24-48h • Shows clinical improvement with  pain, inflammation, improved mobility • CRP  by at least 30% of the highest value • Pseudomonas abx cover o In children with osteomyelitis, consider surgery in the following situations • Persistent or recurring fever after 3-4 days • Periosteal abscess with persistent fever + raised CRP • Sequestration • MRSA S. aureus • Chronic osteomyelitis • Prosthetic material

Answer 121

* If on abx – check bloods at least weekly while on IV abx to monitor for adverse effects – FBC, U+Es, LFTs, CRP * Referral to specialist team – paediatric orthopaedic surgeon + infectious diseases consultant Cierny-Mader classification to stage the patient’s condition * Optimise patient’s condition – manage comorbidities * IV antibiotics * Consider limited surgery to reduce infective load + long term abx suppression • Consider curative surgery + IV abx o Staged reconstruction o Debridement + excision of all infected tissue o Microbiological + histological sampling early during the procedure – dx of causative organism, rule out malignancy o Dead space mx to prevent haematoma formation o Stabilisation of the bone with an external fixator if there is instability or fracture o Immediate soft-tissue coverage with healthy vascularised tissue that can deliver systemic abx o Optimal duration of abx after surgery if ostemyelitic bone has been fully resected  2-6w • Functional rehabilitation

Answer 122

• Immediate ambulance transfer to A+E if systemic symptoms or suspected anaphylaxis +/- angio-oedema • Refer to an allergy specialist if o >1 episodes of systemic symptoms consistent with a severe reaction o Person is at increased risk of anaphylaxis e.g. • Hx of food allergy + co-morbid persistent/poorly controlled asthma • Severe reaction to a trace amount of food allergen (airborne or contact through skin only o Allergy testing is needed to confirm the dx or to assess whether tolerance has developed to a specific food allergen o Dx is uncertain o There are multiple suspected food allergies o Significant atopic eczema, where multiple or cross-reactive food allergies are suspected o There is persistent food allergy beyond the age of expected tolerance, or adult-onset allergy o There is persistent parental/carer suspicion of food allergy despite a lack of supporting history, or persistent anxiety about the dx of food allergy • Referral to a paediatric dietician if o Concerns about faltering growth, excessive weight gain, nutritional status, inappropriate dietary restriction – advice on replacement foods or supplements can be given o Advice on specific food allergen avoidance is needed o Advice on food reintroduction is needed * Tolerance often develops in steps with cooked forms tolerated before raw equivalents * Egg allergy  start with well-cooked + baked foods (e.g. cake), working up through food with lessening degree of heating (e.g. scrambled egg)

Answer 123

``` • Dietary treatment o Exclusion of offending food from diet o Allergy clinic referral o Paediatric dietician referral o Dietary exclusion in mother considered if mother is breast feeding ``` • If confirmed dx of food allergy o Individualized written allergy action plan o Written self-management plans + training o Provide Epi-pens for home use  advise to keep 2 doses with them at all times Single dose: • <30kg = 0.15mg • >30kg = 0.30mg • Sources of information and support o Allergy UK factsheets, British dietetic association • Advice on prompt recognition + mx of acute sx following accidental or new exposures o Severe reactions (with cardiovascular, laryngeal, bronchial involvement (anaphylaxis)) – IM adrenaline (EpiPen) + salbutamol if bronchospasm occurs o Mild or moderate sx (no cardiorespiratory sx) • Immediate use of an oral non-sedating antihistamine (cetirizine, loratadine) PRN • Infants, <2 – sedating antihistamine (chlorphenamine) can be taken instead o Oral allergy syndrome – advise on the additional mx of co-morbid allergic rhinitis sx

Answer 124

• Advice + education on food allergen avoidance o How to check + interpret food labels + recognise food allergens in ingredients of food products * Alternative terms for specific food allergens e.g. ovalbumin or ovomucoid for egg * Loose foods (e.g. brought from markets or open bakeries) + foods imported from outside the EU, may not have food ingredient labelling – avoid o Awareness of situations when accidental exposure is most likely – nursery/school, eating out, birthday parties, friends’ houses, when travelling * Update school/nursery on the person’s allergen mx plan * Wipe eating surfaces before meals to avoid cross-contamination * Airline needs to be informed before a flight – cabin staff should be aware of the food allergy on the day of the travel * Emergency medication to be carried in the aircraft cabin or train carriage within easy reach * Give leaflet from Allergy UK • Ensure that o Co-morbid conditions e.g. asthma are optimally managed in primary care o The person is reviewed by a specialist or in primary care at least annually * Arrange specialist allergy clinic referral e.g. to repeat allergy testing to assess whether tolerance has developed to a specific food allergen * Arrange dietician referral for monitoring growth + nutrition, advice on food reintroduction * Update the individualized written allergy management plan

Answer 125

Rapidly developing angio-oedema • Emergency admission • Slow IV or IM chlorphenamine + hydrocortisone • Review after discharge • Referral to an immunologist or dermatologist for confirmation of the diagnosis if suspecting o Idiopathic angio-oedema – i.e. the cause of angio-oedema is not identifiable or avoidable o HAE/AAE • Provide additional information on angio-oedema – • If awaiting specialist review + at risk of anaphylaxis (see complications) o Epi-pen

Answer 126

* Identify cause * Mild sx – tx not needed • For people with sx that need treatment o Non-sedating antihistamine – cetirizine, fexofenadine, loratadine for up to 6w • Severe sx o Short course of PO CS (e.g. prednisolone 40mg OD for up to 7/7) o Non-sedating antihistamine • Safety-netting – seek immediate medical help if symptoms progress rapidly or if anaphylaxis develops • Review + assess response to treatment o if symptoms don’t improve/ get worse (increased swelling, development of other features e.g. abdominal pain, vomiting, rhinitis)  admission if symptoms improve and o it is likely that symptoms will be persistent/recurrent – daily antihistamine for 3-6/12, then review o long hx of urticaria + angio-oedema – daily antihistamine for 6-12/12 with gradual withdrawal over a period of weeks o sx were short-lived and frequent recurrence it thought unlikely – treatment PRN or prophylactically (e.g. prior to occasions when sx would be most unwelcome e.g. business meetings) • Referral to an immunologist or dermatologist for confirmation of the diagnosis if suspecting o Idiopathic angio-oedema – i.e. the cause of angio-oedema is not identifiable or avoidable o HAE/AAE • Provide additional information on angio-oedema • If awaiting specialist review + at risk of anaphylaxis (see complications) o Epi-pen

Answer 127

Medical emergency – call ambulance, ask for help • ABCDE approach o Look for + relieve airway obstruction – intubate if necessary o Check for normal breathing o If person is unresponsive/ not breathing normally * CPR * Ensure help is on its way bc advanced life support is essential o If no CPR required • Examine chest for signs of upper or lower airway obstruction • Check pulse + BP for signs of circulatory collapse • Check the skin + inside of mouth for angio-oedema + urticaria • Position o Fatality can occur within minutes if the person stands, walks or sits up suddenly – pt must not walk or stand during acute reactions o If AB problems – semi-recumbent position o Low BP – lie flat +/- leg elevation o If breathing normally + unconscious – recovery position, monitor breathing continuously, prepare to intervene if this changes • IM adrenaline 1:1000 o Assess response after 5 mins – vital signs + auscultate for wheeze • Repeat IM adrenaline every 5 mins until adequate response o Do not give IV adrenaline in primary care – however it may be given in cases of cardiopulmonary arrest • Remove trigger if possible – e.g. stinger after a bee sting, attempt to make the person vomit are not recommended • High flow O2 o O2 at highest concentration possible using a non-rebreather mask o Adjust O2 concentration to achieve an oxygen saturation of 94-98% • Attach monitoring o BP, pulse oximeter, ECG o Will help assess the person’s response to adrenaline • IV fluids o If shock/hypotension/poor response to an initial dose of adrenaline o Obtain IV access o Give rapid IV bolus (e.g. Hartmann’s or normal saline) o 10ml/kg in a child o Give further fluids as necessary • If wheezy/ known asthma o Consider inhaled salbutamol or ipratropium therapy * IV chlorphenamine + IV hydrocortisone * Arrange review after person has been discharge from hospital

Answer 128

• Refer to specialist allergy service • Offer o 2 adrenaline auto-injectors (EpiPen, Jext, Emerade) • Advice o Sx of anaphylaxis o Risk of biphasic reaction o Carry 2 adrenaline auto-injectors at all times + check expiry dates + obtain replacements before they expire o Bracelet (medicalert) that provides info on hx of anaphylactic reaction o Trigger avoidance o Importance of referral to a specialist allergy service • Safety net – if anaphylactic rxn occurs o Use one adrenaline auto-injector + call 999 – ask for an ambulance, state “anaphylaxis” even if the symptoms are improving o Lie flat with the legs raised in order to maintain blood flow o If breathing difficulties – sit up o If sx don’t get better 5-15 mins after the first injection – use second auto-injector • Information leaflets o NHS website o Allergy UK – national charity dedicated to supporting allergy sufferers

Answer 129

o <6m – 0.1-0.15ml (100-150μg), o 6m-6y – 0.15 ml (150μg) o 6-12y – 0.3 ml (300μg) o >12y – 0.5ml (500 μg) but 0.3ml in a child who is small or pre-pubertal https://cks.nice.org.uk/topics/angio-oedema-anaphylaxis/prescribing-information/emergency-drug-doses/

Answer 130

o <6m – 250μg + 25mg o 6m-6y – 2.5mg + 50mg o 6-12y – 5mg + 100mg o >12y – 10mg + 200mg https://cks.nice.org.uk/topics/angio-oedema-anaphylaxis/prescribing-information/emergency-drug-doses/

Answer 131

* Most cases are self-limiting or resolve with symptomatic treatment within 4 weeks * Refer to nephrologist Mild/moderate nephritis • CS (mild/moderate nephritis) o Mild nephritis – Prednisolone PO o Moderate nephritis – prednisolone and/or methylprednisolone PO or IV • Immunosuppressants o Azathioprine or mycophenolate mofetil or cyclophosphamide IV (in moderate/severe nephritis) o Corticosteroid-sparing agents or a 2nd line treatment without overlap with oral prednisolone • ACEi – enalapril, lisinopril or Angiotensin II receptor antagonist (e.g. losartan) o Considered --> prevent/limit glomerular injury in patients with persistent proteinuria

Answer 132

* Most cases are self-limiting or resolve with symptomatic treatment within 4 weeks * Refer to nephrologist • Skin rash o Conservative management o If severe (blistering or necrotic area) – add topical CS • Joint pain o Ibuprofen or paracetamol • Mild to moderate abdominal pain o Managed with paracetamol, rest, supportive care • Orchitis/ scrotal involvement or severe oedema or Severe rash or moderate to severe MSK or GI involvement o CS +/or immunosuppressant prescribed for mild nephritis o Oral prednisolone o Rest, hydration, elevation of the affected area needed • Persistent proteinuria o Continued therapy with an immunosuppressant is appropriate o ACEi or ATII receptor antagonist • F/U to check BP + renal function o BP + urinalysis monitored for 6-12 months even if normal

Answer 133

• Admission to hospital if severe nephritis • IV cyclophosphamide – 1st line • PO or IV prednisolone/methylprednisolone (can be combined with cyclophosphamide to induce remission) • ACEi • Azathioprine or mycophenolate mofetil – 2nd line • Renal transplant o May be required in pt who progress to end-stage renal disease

Answer 134

• Specialist referral for further evaluation + to determine the extent of urethral involvement • Possible surgery o Not mandatory o Can be performed >3m of age on functional or cosmetic grounds • Functional – to allow boys to pass urine in a straight line whilst standing and to have a straight erection o May need postoperative urethral stent for up to 2w after surgery depending upon severity o Mild forms might not need surgery • Incomplete prepuce o Should not undergo circumcision in the neonatal period o Foreskin can be reconstructed or a circumcision can be performed at the time of urethroplasty • Important – boys with hypospadias should not be circumcised before repair, because the skin is important in the repair

Answer 135

• Phimosis <12 y/o, congenital, physiological o Reassurance + hygiene • Expectant management • No need to forcibly retract the foreskin for cleaning until natural separation of the foreskin occurs • Routine cleaning of the external skin ``` • Acquired or pathological phimosis e.g. cicatrix, balanitis xerotica obliterans, lichen sclerosis o Requires treatment regardless of age o Topical CS • Betamethasone dipropionate • 4-6w • Course may be repeated once ``` o Preputial surgery • Circumcision • If the do not respond to topical CS ``` • Phimosis >12 y/o o Topical CS • Betamethasone dipropionate • 6-week trial topical CS in the phimotic ring may allow retraction of the foreskin • Course may be repeated once o Preputial surgery ```

Answer 136

o With ischaemia or necrosis  emergency surgery o Chronic without ischaemia or necrosis  surgical reduction followed by circumcision o Acute but without ischaemia or necrosis • Manual reduction with topical anaesthesia – 1st line + Sugar • If there is difficulty with manual reduction, several therapeutic measures can be attempted incl. pressure, sugar, hyaluronidase injection, puncture technique • Sugar – osmotic agent, applied in liberal amounts, fluid flows down a concentration gradient – hypotonic fluid in penis flows to hypertonic agent outside skin + reduces oedema * Needle puncture – 2nd line + hyaluronidase * reduction of oedema before manual reduction is attempted * 26-g needle is used to make about 20 punctures in the oedematous ring of foreskin • Hyaluronidase induces osmotic diuresis, reducing oedema * Preputial surgery – 3rd line * If conservative measures fail * Surgical reduction followed by circumcision * Dorsal slit or circumcision

Answer 137

• Renal o Renal cell carcinoma – partial or total nephrectomy o HTN secondary to renal complications - antihypertensives o AMLs – mTOR inhibitor (1st line), embolization of artery supplying the lesion (2nd line), partial nephrectomy (3rd line) o Renal failure secondar to AMLs or polycystic renal disease – dialysis * Intracranial aneurysm – surgical or radiological intervention * Infantile spasms – anti-convulsant (vigabatrin) * Seizures– anti-convulsant, ketogenic diet, early evaluation + provision of surgical interventions (focal + multifocal cortical resection, corpus callosotomy, vagus nerve stimulation) • SEGA (giant cell astrocytoma) o Periodic neuroimaging (1- to 3- year intervals) -1st line o Surgical resection or mTOR inihibitor (everolimus) – 2nd line • Medical therapy with everolimus or sirolimus may be used to induce tumour remissin or size reduction before resection • Arrhythmias secondary to rhabdomyoma – anti arrhythmic ``` • Lymphangioleiomyomatosis o Supportive treatment until lung transplantation can be provided o mTOR inhibitor – 1st line o oxygen + bronchodilator o lung transplant – 2nd line ``` • Impaired cognitive function or autism o Early educational support – reinforcing pro-social skills, discouraging aberrant behaviours, assisting in verbal + non-verbal communication skills o Antipsychotic or antidepressant in some patients • Skin lesions o Laser therapy or topical sirolimus o Surgical resection

Answer 138

• Self-limiting – no treatment required • No antibiotics (Sterile) • Symptomatic relief o NSAID – 1st line – naproxen, ibuprofen, indometacin o CS – 2nd line/severe – prednisolone [intra-articular injections in monoarticular or oligoarticular forms, or systemic CS if many joints are affected] o Systemic CS – ocular manifestations e.g. iritis o Topical CS – skin involvement (e.g. circinate balanitis, keratoderma belonnorrhagicum) • Refractory disease: oral glucocorticoids, steroid-sparing agents e.g. sulfasalazine • Chronic disease – DMARD (sulfasalazine) – considered when NSAIDs fail or when more aggressive treatments are needed

Answer 139

Supportive treatment • Activity restrictions • Short period of bed rest • Gentle skin traction – 2kg of weight applied to skin, usually at night • Analgesia o Ibuprofen or paracetamol o Naproxen if >2y • Ibuprofen has been shown to decrease duration of sx • NSAIDs – discontinue with any GI sx • Paracetamol if pt unable to take NSAIDs

Answer 140

Surgical emergency • Refer immediately to orthopaedics • US for joint aspiration + possible surgical debridement • Synovial fluid sample + blood cultures • IV Abx o Start empirically immediately but after synovial fluid + blood cultures are taken, narrow down when microscopy + gram stain results are back o IV for 2/52 + then switch to PO for further 4/52 o Suspected Gram +ve – vancomycin (1st line), clindamycin or cephalosporin (2nd line) o Suspected Gram -ve – ceftriaxone (3rd generation cephalosporin – 1st line), ciprofloxacin (2nd line) • Joint aspiration o Aspirated to dryness as often as necessary o Using closed needle aspiration or arthroscopy o Do not aspirate a prosthetic joint – refer to an orthopaedic surgeon • Joint lavage (washing out of the joint) or surgical drainage may be required – arthroscopic washouts indicated if temperature + inflammatory markers do not improve after initial aspiration + treatment • Analgesia – paracetamol or ibuprofen • If suspected infection in any joints + systemic involvement  follow local protocol for suspected sepsis • Send joint aspirates for MC+S (urgent gram stain + culture) • If worsening symptoms (fever, confusion, pain, swelling) – review from a senior colleague

Answer 141

• Don’t let patient walk, analgesia, immediate orthopaedic referral • Surgical repair o In situ pinning to stabilise femoral head in order to prevent further slipping o In situ screw fixation across the growth plate o Surgical fixing screws + pins through the growth plate to the femoral head o Period of rest w limited weight bearing until hip heals + becomes stable • Unstable SCFE o Urgent surgical repair  Percutaneous decompression of the hip joint  Incidental repositioning of the slip  Fixation with 2 screws o Prophylactic fixation of contralateral hip • Stable SCFE o In situ screw fixation of the epiphysis with a single screw– 1st line  Screw is placed in the centre of the epiphysis both on the AP + lateral aspects  Easy technique, low further slip rate, prevention of complications  Post-op, toe-touch weight bearing permitted for 2 weeks followed by weight bearing as tolerated o Open reduction + internal fixation with surgical hip dislocation – 2nd line o Bone graft epiphysiodesis – 3rd line o Prophylactic fixation of contralateral hip • Late deformity o Corrective surgery

Answer 142

``` Neurolastoma Abdominal mass (palpable) or enlarged abdominal organ (unexplained) in children Very urgent referral (for an appointment within 48 hours) for specialist assessment ``` Wilm's tumour Abdominal mass (palpable) or enlarged abdominal organ (unexplained) in children Haematuria (visible and unexplained) in children very urgent referral (for an appointment within 48 hours) for specialist assessment Retinoblastoma Absent red reflex in children Urgent referral (for an appointment within 2 weeks) for ophthalmological assessment

Answer 143

• Very urgent referral (appt. within 48h) for specialist assessment for neuroblastoma in children with o A palpable abdominal mass or o An unexplained enlarged abdominal organ • Low risk disease (localised primaries without metastatic disease, in some infants may resolve spontaneously) o Observation +/- surgery  Observation with serial US every 3-6 weeks or • If tumour enlargement is noted during the observation period, consider surgery  Surgery is the initial treatment of choice for pt with localised disease who are able to have >50% of their tumour safely removed  May be done at the time of biopsy in some symptomatic patients with localised disease o Chemotherapy  Only used in cases where the tumour progresses following surgery or if the patient is experiencing severe symptoms from mass effect of the tumour e.g. airway compromise, spina cord compression, bowel obstruction  Carboplatin + etoposide + cyclophosphamide + doxorubicin • Intermediate-risk disease (metastatic disease) o Chemotherapy  Carboplatin + etoposide + cyclophosphamide + doxorubicin  Usually given for 4-8 cycles o Surgery  Attempt at gross total resection is recommended after chemotherapy if possible o Radiotherapy  If chemotherapy is not effective • High risk disease (aggressive + metastatic disease) o Induction  Chemotherapy • Carboplatin + etoposide + cyclophosphamide + doxorubicin • Should be started in all patients using an intense induction regimen that is usually given for 5-6 cycles • Higher doses of drugs compared to those given to low- or intermediate- risk disease  Surgery • Surgical removal of the primary tumour once chemo has decreased the initial tumour volume • Remove as much tumour as possible, while limiting morbidity o Consolidation  High dose chemotherapy + autologous BM transplant • Patients given myeloablative doses of chemotherapy followed by an infusion of their own stem cells that were harvested earlier in the treatment process  Radiotherapy • For local control of the tumour + may prevent recurrence of metastases • Typically given after autologous bone marrow translant o Maintenance  Isotretinoin • Mainstay of maintenance therapy • Promotes differentiation of neuroblastoma cells into normal cells • Usually given for 2/52 every month for 6/12  Immunotherapy with Dinutuximab or dinutuximab beta • Chimeric anti-glycolipid disialoganglioside that binds to the surface of neuroblastoma cells • Patients aged >12m • Relapsed or refractory disease o HD-ICE  High dose isosfamide, carboplatin, etoposide o Radiation o Immunotherapy

Answer 144

• Urgent referral for ophthalmological assessment for retinoblastoma in children with absent red reflex • With vitreous seeding (tumour cells floating within the vitreous cavity) o Enucleation  Surgical removal of eye without resecting the lids or extraocular muscles  Orbital implant o Post-operative systemic chemotherapy  If post-op histopathological examination of the enucleated specimen by an experienced ocular pathologist shows the presence of tumour extension into the cut section of the optic nerve/ if scleral /iris/ ciliary body invasion/ if surgical margin invasion  Carboplatin + etoposide + vincristine • Without vitreous seeding o (Focal laser ablation alone if <2 disc diameters) o Systemic or intra-arterial chemo  Carboplatin + etoposide + vincristine  6-9 cycles  Patients undergo regular, frequent EUA to assess the response to treatment o Plus focal therapy  Concurrent laser ablation or cryotherapy o External beam radiotherapy – 2nd line  If pt develop vitreous seeding after chemotherapy + focal therapy o Periocular carboplatin therapy – 3rd line o Enucleation– 4th line • FHx of retinoblastoma/detected at birth o Usually treated by laser alone o Followed by an EUA every month for at least 1 year • Metastatic disease – depends on location o Optic nerve/ choroidal invasion – chemo o Orbital invasion – chemo + radio o CNS invasion – chemo + intrathecal chemo + craniospinal irradiation o BM/ bone/ soft tissue invasion – chemo + stem cell rescue +/- radiation • Recurrence o Brachytherapy  Recurrence post globe salvaging therapy  for focal, non-macular, circumscribed tumours with no associated vitreous seeds appearing after treatment with other modalities o external beam radiotherapy +/- systemic chemo (carboplatin + vincristine + etoposide)  post-enucleation

Answer 145

• Very urgent referral for specialist assessment if any of the following o Palpable abdominal mass o Unexplained enlarged abdominal organ o Unexplained visible haematuria Stages I-II • Surgery o Radical nephrectomy o If tumour unresectable – open biopsy with lymph-node sampling • Postoperative chemo o If surgically feasible, any residual disease that remains needs to be resected after week 6 of chemo if intra-abdominal or after week 12 of chemo if pulmonary Stage III + IV • Surgery • Post-operative chemotherapy • Radiotherapy – either flank or whole abdomen ``` Stage V • Pre-operative chemotherapy • Surgery • Postoperative chemo • Renal transplant ``` ``` Tumour recurrence • Preoperative chemo o If previously treated o Regimens should include etoposide + cyclophosphamide or carboplatin o If previously untreated, jump straight to surgical resection • Surgical resection • Postoperative chemo • Radiotherapy ``` Multiple adverse prognostic factors or multiple relapses • BM transplant – autologous SCT may be considered

Answer 146

If suspected unilateral undescended testis • At birth o Re-examine infant at 6-8w of age o If both testes are normally descended, no further action is required • At 6-8w of age o Re-examine at 4-5 months of age • At 4-5 months of age (corrected for GA) o If both testes are normally descended, no further action is required o If testis remains undescended, arrange referral to paediatric surgery or urology for specialist management to be seen by 6 months of age o If there is any uncertainty in differentiating between a possible undescended testis + retractile testis  arrange referral for clarification of dx o if both testes are in the scrotum but one or both are retractile  annual follow up + re-examination until after puberty as there is a significant risk of ascending testis • Unilateral or bilateral undescended palpable testicles at 6m– orchidopexy o Orchidopexy = placement of testis in the scrotum o Treatment of choice for a palpable testicle that has not descended into the dependent portion of the scrotum by 6 months of age o Cosmetic o Reduced risk of trauma and torsion o Fertility (particularly important if bilateral) o Malignancy ( risk in undescended testis) o Ideally, surgery should be performed <12-18m of age • Unilateral non palpable testis o EAU (1st line) +/- inguinal exploration + diagnostic laparoscopy (2nd line) to locate an intra-abdominal testis + perform subsequent orchidopexy or orchidectomy o If the testis is palpable within the inguinal canal or at the deep inguinal ring, a single-stage operation may be successful o Alternatively, a two-stage procedure may be needed, involving intra-abdominal high ligation of of the testicular vessels and then mobilisation of the testis into the scrotum • Surgical treatment o Preferred treatment of undescended testes – most effective + reliable method o Orchidopexy (freeing of undescended testis + implanting it into the scrotum) using an inguinal approach

Answer 147

If suspected disorder of sexual development and/or bilateral undescended testes identified at birth • Urgent referral to a paediatrician within 24h – child might need urgent endocrine or genetic investigation - refer immediately for an evaluation wit karyotype + biochemical workup for a difference of sex development o Possibility of disorder of sexual development (e.g. ambiguous genitalia or hypospadias) • If disorder of sexual development is excluded + testes are not present in the scrotum by 4-5 months of age  refer to paediatric surgeon/urology to be seen by 6 months of age If suspected bilateral undescended testes at 6-8 weeks of age • urgent referral to a paediatrician to be seen within 2 weeks Unilateral or bilateral undescended palpable testicles at 6m– orchidopexy • Orchidopexy = placement of testis in the scrotum • Treatment of choice for a palpable testicle that has not descended into the dependent portion of the scrotum by 6 months of age • Cosmetic • Reduced risk of trauma and torsion • Fertility (particularly important if bilateral) • Malignancy ( risk in undescended testis) • Ideally, surgery should be performed <12-18m of age • Surgical treatment o Preferred treatment of undescended testes – most effective + reliable method o Orchidopexy (freeing of undescended testis + implanting it into the scrotum) using an inguinal approach

Answer 148

* referral to paediatric surgery, paediatric urology, urology * orchidopexy +/- biopsy or orchiectomy * advise boys + young men with a hx of undescended testis to perform regular testicular self-examination during + after puberty owing to the increased risk of developing testicular cancer

Answer 149

* Ascended testes (newly acquired testicles)  refer to a surgical specialist * Retractile testicle  annual follow-up examination – any development of cryptorchidism or testicular asymmetry should be treated with orchidopexy

Answer 150

• MDT approach • Assessment +/- specialist consult • Cryptorchidism – refer to urology, surgery • Congenital heart abnormalities o Comprehensive cardiovascular evaluation o Optimisation of cardiac function o Pulmonary valve stenosis/ dysplasia – may respond to balloon valvuloplasty, may require valve replacement o Hypertrophic cardiomyopathy – BB, CCB, surgical myomectomy o Cardia care should continue into adulthood – ongoing morbidity + mortality • Poor growth o Refer to an endocrinologist o Somatotropin o Treatment effect monitored by the growth response + IGF-1 levels (insulin-like growth factor 1) • Coagulation abnormalities – refer to haematologist for further assessments

Answer 151

• Assessment +/- specialist consult for medical conditions associated with DS • Parental counselling o Refer to geneticist and/or genetic counsellor o Will review the chromosome results and explain the rate of recurrence in future pregnancies and availability of testing options o Refer to local parent support groups o Connect to a local DS clinic • Early interventional therapies o Focus on gross motor, fine motor, language, social development o PT o OT – fine motor skills, mastering self-help skills for independence (feeding, dressing, writing, playing), oral-motor exercise in infants with feeding issues o SALT • Individualised educational plan o Carry out a comprehensive psycho-educational evaluation of the child’s cognitive, achievement and adaptive skills to determine strengths and challenges • Identification of ongoing complications +/- specialist consult o Hearing evaluation, ear examination, TFTs, ophthalmic evaluation dental examination, polysomnography • Monitor growth using DS-specific growth charts • Referral to paediatric otolaryngologist if tympanic membrane is not visualised and/or otitis media recures frequently in childhood

Answer 152

• Main aims of treatment o Optimisation of height o Induction and maintenance of pubertal development o Treatment of ongoing ovarian hormone deficiency o Screening for and treatment of comorbidities or complications • Screening o TFTs, LFTs, BP, fasting glucose, lipids annually o TtIgA every 3-5 years o Baseline BMD • CV assessment at time of initial dx + evaluation for surgery Poor growth o Somatotropin – treatment effect monitored by the growth response + insulin-like growth factor 1 levels (IGF-1) o Failure of a good growth response commonly due to – hypothyroidism, coeliac disease, non-compliance o Risks -  ICP, SUFE, scoliosis, pancreatitis, T1DM • Pubertal delay/arrest (age >12y, if no spontaneous breast development has occurred by the age of 11-12 years + serum FSH is ) o Low-dose oestrogen o Cyclic progesterone – once there is breakthrough bleeding or when the pt is on a full adult does of oestrogen to induce menstruation • After establishment of cyclical bleeding o Ovarian HRT  Oestrogen therapy to reduce osteoporosis, CV disease, urogenital atrophy and to improve QOL  HRT after induction + development of puberty until about 50 years f age  Combine with progesterone o Low bone mineral mass – oestrogen HRT, vitamin D, weight bearing exercises, adequate ca in diet, not bisphosphoates o Breast implants o Monitoring + education on reproductive issues  Fertility rapidly declines with age  offer fertility treatment at a young age  Routine oocyte retrieval not recommended in

Answer 153

• Education o Bedwetting is not the child’s fault o Occurs because the volume of urine produced at night exceeds the capacity of the bladder to hold it + the sensation of a full bladder does not wake the child • Reassurance o <5 – usually resolves without treatment as children get older o >5 – if bedwetting is infrequent (<2/week) reassure + offer watch + see approach o They develop an  bladder capacity and/or produce less urine at night and/or learn to wake to the sensation of a full bladder Advice • Diet + fluid intake o Healthy diet – do not restrict it as a form of treatment for bedwetting o Adequate daily fluid intake o Avoid caffeine based drinks before sleep (cola, coffee, tea) o 5-8y – 1000-1400ml o 9-13y – 1200-2100ml (f), 1400-2300ml (m) o 14-18y – 1400-2500ml (f), 2100-3200ml (m) • Toileting patterns o Encourage child to empty bladder regularly during the day + before sleep (bn 4-7x in total) o Waterproof mattress + duvet cover, absorbent quilted sheets, bed pads o Easy access to a toilet e.g. potty near the bed o If child has been toilet trained by day for >6m – consider trial of at least 2 nights in a row without nappies (with appropriate waterproof mattress protection) o A longer trial can be considered in older children/ children who achieve a reduction in wetness / children whose family circumstances allow the trial to continue • Lifting and waking o Lifting or waking the child during the night does not promote long-term dryness o If child wakes at night they should be taken to toilet o Young people with bedwetting who have not responded to treatment may try self-instigated waking (e.g. alarm clock) • Positive reward systems o Dry nights o Drinking recommended levels of fluid during the day o Using toilet before bed o Engaging in mx (e.g. taking medication, helping change sheets) o Do not penalise Do not punish – might humiliate the child + reduce their self esteem • Provide additional sources of information + support o ERIC the children’s bowel and bladder charity o The bladder + bowel community o NHS website

Answer 154

Treatment if conservative mx fails Rapid/short-term mx (e.g. sleepovers, school trips) • Desmopressin ``` Long term mx • Enuresis alarm o First line o Use a reward system along side it • Desmopressin o Second line o Restrict fluid 1h before + 8h after desmopressin (A total of 1 regular glass of water may be drunk in this time) o Less preferred, may be considered if an alarm is considered undesirable/inappropriate ``` Non-response to tx • If bedwetting has not responded to at least 2 complete courses of treatment with either alarm or desmopressin (may be 1 course of each treatment or 2 of the same) • Refer to secondary care/ enuresis clinic/ community paediatrician • Further assessment required for factors that may be associated with a poor response – OAB, underlying disease, social and emotional factors • TCA (e.g. imipramine) or antimuscarinics (e.g. oxybutynin) may be initiated in secondary care or primary care following assessment by a HCP with expertise in managing bedwetting

Answer 155

Enuresis alarms o Aims of treatment  Recognise the need to pass urine  Wake to go to the toilet or hold on  Learn overtime to hold on or to wake spontaneously + stop wetting the bed o How the alarm works  Sensor pads that sense wetness  Sensor linked to an alarm that wakes the child if it becomes wet  2 main types • Bedside – noise box placed next to the bed + sensor pad positioned under a draw sheet • Body worn – tiny sensor attached to the child or young persons pants + alarm on pyjama top o How to obtain an alarm  Cannot be prescribed on the NHS  Can be borrowed from local enuresis adviser or bought privately

Answer 156

• Contraindications o Child or parents don’t want to use one o Child wets bed <1-2 a week o Parents have emotional difficulty coping with the burden of bedwetting o Parents express anger, negativity, blame towards the child o Child is <7 + is not able to use an alarm - decision to use an alarm in a child <7 is based on the child’s maturity, their understanding of the alarm + their motivation

Answer 157

o High long-term success rates but may not suit all families – requires significant commitment  Child + parents require training on how to use the alarm  Alarm can disrupt sleep  Parents may need to help child wake up to alarm  Keep record of child’s progress (if + when they wake up, how wet they are) o Reward system should be used with the alarm  Waking up when the alarm goes off  Going to the toilet after the alarm has gone off  Returning to bed + resetting the alarm

Answer 158

• Assess response after 4 weeks o Response = smaller wet patches/ fewer wetting episodes per night/ fewer wet nights/ alarm going off later + fewer times per day  May take a few weeks for the early signs of a response to the alarm to occur  Dry nights may be a late sign of response to the alarm + may take weeks to achieve o If no signs of response stop treatment o If there are signs of a response – continue alarm treatment until a min. of 2 weeks’ uninterrupted dry nights have been achieved o If complete dryness is not achieved after 3 months only continue alarm if bedwetting is still improving + the child/parents are motivated to continue • No response to initial alarm treatment o Desmopressin +/- alarm  Partial response to combination treatment of an alarm + desmopressin – consider offering desmopressin alone • Relapse following response to alarm treatment o Start using alarm again o No need to consult a HCP

Answer 159

• Second line • Indications o Rapid onset in improvement or a short-term improvement is required (e.g. sleepovers, school trips)  Trial of at least 1 week before the school trip or sleepover to determine the effectiveness of treatment o Child/parents are unable/unwilling to use an enuresis alarm as first line treatment o Children around 5-7y who are not mature enough to use an enuresis alarm o Child/parents currently using an enuresis alarm + want to stop

Answer 160

• Advice on how decompression works o Reduces the amount of urine the body produces at night – mimics the action of the body’s own ADH o In most children, levels of ADH rise overnight + reduce the volume of water excreted by kidneys compared with during the daytime • What to expect o Usually there is a rapid response to treatment o May children will experience a reduction in wetness o Relapse when treatment is withdrawn – repeated courses may be used • Advice on how to take desmopressin o Should be taken at bedtime o Fluid intake restricted to sips only from 1h before taking desmopressin to 8h afterwards  A total of 1 regular glass of water may be drunk in this time  Fluid restriction required to avoid potential for fluid overload + hyponatraemia which can lead to hyponatraemic convulsions  Stop Desmopressin if child or young person has V,D until fluid balance is normal  OTC NSAIDs e.g. ibuprofen to be avoided – cause water retention +  risk of hyponatraemia • Low dose Desmopressin PO, initially at bedtime o If there is response, continue treatment o If complete dryness not achieved after 1-2 weeks, increase dose

Answer 161

• Assess response 4w after starting treatment o If sx of a response  Continue treatment for 3m  Stop for 1 week to check whether dryness has been achieved  Repeated courses of desmopressin may be used o Partial response  Increase dose of desmopressin  Take 1-2h before bedtime  Continue treatment for another 6m – bedwetting may improve for up to 6m after starting treatment o No signs of response  Stop treatment or  Continue + recommend that it is taken 1-2h before bedtime + fluid restriction for 1h before desmopressin is taken • Review child regularly if desmopressin is being used long term

Answer 162

• If enuresis alarm was successful o Restart alarm or o Alarm + desmopressin (if >1 recurrence of bedwetting) • If desmopressin was successful o Repeat course of desmopressin  Withdraw treatment at regular intervals (for 1 week every 3 months) to check whether dryness has been achieved o Withdraw gradually instead of stopping suddenly  An increase of “no medication days” over an 8-week period o Use an enuresis alarm instead

Answer 163

• Refer to secondary care or an enuresis clinic for further investigations and management

Answer 164

• Manage underlying cause in primary care – UTI, constipation • Arrange referral to a paediatrician or an enuresis clinic if cause cannot be managed in primary care/ is not clearly identified – the following are likely to need a specialist referral o Diabetes o Recurrent UTI o Psychological problems o Family problems o Developmental, attention, learning difficulties o Known or suspected physical or neurological problems

Answer 165

``` fever in • Infants <1m • All infants 1-3m who appear unwell • Infants 1-3m with o WCC <5x10^9 /l o WCC >15x10^9 /l ``` • Always perform LP before the administration of antibiotics

Answer 166

• Immediately life-threatening illness e.g. sepsis, CNS infection – refer immediately for emergency medical care by the most appropriate means of transport (usually 999 ambulance) • Remote assessment or primary care assessment o Red features – urgent f2f assessment by HCP within 2h – urgent referral to paediatric specialist o Amber features – f2f assessment by HCP – discharge with safety netting or refer to paediatric specialist o Green features –assess + manage any underlying cause of fever, can be cared for at home, safety netting

Answer 167

* ABC * Consider sepsis * Assess child using the traffic light system

Answer 168

Immediate IV fluid bolus 20ml/kg 0.9% NaCl  Monitor + give further fluid boluses as necessary o Fever + shock/unrousable/ signs of meningococcal disease – review urgently + consider referral to PICU

Answer 169

 Immediate parenteral abx if shocked/ unrousable/ signs of meningococcal disease  Consider immediate parenteral abx if reduced level of consciousness (seek for sx of meningitis + HSV encephalitis)  Parenteral abx  cefotaxime or ceftriaxone (3rd generation cephalosporin) until cultures are available • Cefotaxime/ceftriaxone – antibiotics should be directed against – N. meningitidis, Strep. Pneumoniae, E.coli, Staph aureus, HIB  If <3m - cefotaxime/ ceftriaxone + ampicillin/ amoxicillin (abx active against listeria) • Abx – Do not prescribe PO abx in fever without an apparent source HSV encephalitis - IV acyclovir

Answer 170

o >3m + fever without apparent source – consider observation in hospital +/- ix – to help differentiate non-serious from serious illness o reassess all children in hospital with amber or red features after 1-2h to detect possible deterioration

Answer 171

o Do not prevent febrile convulsions + should not be used specifically for this purpose o Do not routinely treat children with fever who are otherwise well o Do not use aspirin if <16 o Paracetamol or ibuprofen only if child appears distressed o Do not rely on a decrease (or lack of decrease) in the child’s temperature after 1-2h following the use of antipyretic therapy, to differentiate between serious + non-serious illness  Paracetamol given every 4-6h  Ibuprofen given every 8h  Continue as long as the child appears distressed  Consider changing to the other agent if the child’s distress is not alleviated  Do not give both agents simultaneously  If ibuprofen monotherapy and paracetamol monotherapy are ineffective, consider alternating these agents if the distress persists or recurs before the next dose is due  Consider use of treatment diary to record the drug + the time it was given to avoid medication errors  Stop antipyretic drug treatment once the child is comfortable + not distressed

Answer 172

• Advice to parents o Do not use oral/rectal routes to measure body temperature o If child does not need admission but no dx has been reached – safety net if any “red” or “amber” features are present  Information on warning symptoms  Arrange follow up  Liaise with other HCP to ensure direct access for child if further assessment is required o Antipyretics o Offer regular fluids + encourage a higher fluid intake, continue BF o How to detect signs of dehydration o Do not overdress/ underdress the child o Check child regularly + incl. during the night o To keep their child away from nursery/ school while the child’s fever persist but to notify the school/nursery of the illness o Seek further help if  Child has a fit  Develops non blanching rash or other signs of CNS infection  Parent/carer feels that the child becomes less well  Child becoming dehydrated + self-management measures are not helping  Fever >5 d  Parent distressed or concerned that they are unable to look after their child o Provide advice on sourced of information + support

Answer 173

• Insulin therapy o Three types of insulin therapy  Multiple daily injection basal-bolus regimen = short-acting or rapid-acting insulin analogue before meals + >1 separate daily injections of intermediate-acting insulin or long-acting insulin  Once, twice or three times daily mixed insulin injections = short-acting or rapid acting insulin analogue + intermediate-acting insulin  Continuous SC insulin infusion (insulin pump therapy) = programmable pump + insulin storage device that gives regular or continuous amounts of insulin (usually rapid-acting insulin or short-acting insulin) by a SC cannula o Multiple daily injection basal bolus insulin regimen – 1st line  Adjust dose if appropriate after each blood glucose measurement  Inject rapid-acting insulin analogues before eating o Continuous SC insulin infusion (CSII or pump therapy) – 2nd line • Oral medicines o Do not offer children + young people with T1DM acarbose or sulphonylureas (glibenclamide, gliclazide, glipizide, tobutamide) in combination with insulin  might increase the risk of hypoglycaemia without improving blood glucose management

Answer 174

o Offer level 3 carbohydrate counting education from diagnosis and to family members  Counting the carbohydrates in your food and drink, so you can make sure you are injecting the right amount of insulin o To reduce the risk of CVD  Eating foods with low glycaemic index (GI) – fruit, vegetables  5 portions of fruit and vegetables a day  Appropriate types + amounts of fats o Low GI diet may help to improve blood glucose control + reduce the risk of hyperglycaemic episodes o Encourage exercise  Regular exercise may lower glucose levels as exercise increases the amount of glucose used by the muscles for energy o Encourage monitoring of blood glucose levels before and after exercise o Watch out for exercise induced hypoglycaemia  Can occur several hours after prolonged exercise  Should have extra carbohydrates + carbohydrate-based foods to avoid hypoglycaemia during and after exercise  Should consume additional carbohydrates esp. if plasma glucose levels are <7mmol/l before they exercise  Carbohydrate based foods should be readily available during + after exercise o They may need to alter their insulin dose or carbohydrate intake if they change their daily exercise patterns o Children have the same basic nutritional needs as other children so their food should provide sufficient energy + nutrients for optimal growth + development o Support to help optimise body weight

Answer 175

• Monitoring o Perform at least 5 capillary blood glucose tests per day • Blood glucose targets o Fasting plasma glucose on waking – 4-7 mmol/l o Plasma glucose before meals – 4-7 mmol/l o Plasma glucose after meals – 5-9mmol/l o Plasma glucose when driving – >5 mmol/l • HbA1c targets o <48 mmol/mol (6.5%) o Measure HbA1c 4x a year o Any reduction in HbA1c reduces their risk of long-term complications – benefits of safely achieving + maintaining the lowest attainable HbA1c o Achieving + maintaining blood glucose levels towards the lower end of the target optimal ranges will help them to achieve the lowest attainable HbA1c

Answer 176

o Offer ongoing real-time continuous glucose monitoring with alarms if  Frequent severe hypoglycaemia or  Impaired hypoglycaemia awareness that is associated with adverse consequences (seizures, anxiety)  Cannot recognise or communicate symptoms of hypoglycaemia (e.g. cognitive or neurological disabilities) o Consider ongoing real-time continuous glucose monitoring with alarms for  Babies, infants, pre-school children  Children + young people with high levels of physical activity  Comorbidities (e.g. AN) or who are having treatments (e.g. CS) that can make blood glucose mx difficult o Intermittent (real time or retrospective) continuous glucose monitoring if children continue to have hyperglycaemia despite insulin adjustment

Answer 177

 Continue insulin as usual  Check blood glucose every 1-2h incl. through the night  Check blood ketones every 3-4h incl. through the night • If blood ketone level is elevated (>3 mmol/L) – contact GP or diabetes care team immediately  Maintain normal meal pattern • Normal meals could be replaced with carbohydrate containing drinks (milk, milk shakes, fruit juices, sugary drinks)  Maintain adequate fluid intake to prevent dehydration • Water or sugar free fluids • If blood glucose levels are low  drinks containing glucose or person should take carbohydrates • If glucose levels are high  maintain fluid intake with sugar-free fluids • Avoid carbonated drinks  Weigh yourself every day – losing weight without trying is a sign of high blood glucose  Seek urgent advice if violently sick, drowsy, unable to keep fluids down – IVF may be required o Test for ketonemia if they are ill or have hyperglycaemia - offer blood ketone testing strips + a meter o Seek for urgent medical advice if the child  Is unable to eat or drink, is dehydrated or at risk of dehydration  Has persistent vomiting  Has hypoglycaemia that cannot be managed in primary care

Answer 178

o Immediate admission  Immediate risk of DKA  Moderate ketonaemia (1.5-2.9 mmol/L) +/- hyperglycaemia + the person cannot eat or drink – risk of DKA  Child does not improve rapidly with insulin therapy o Consider admission or urgent refer to specialist if  Underlying condition is not clear  Child dehydrated  Vomiting >2h  Child becoming exhausted, confused, dehydrated, is hyperventilating, has severe abdo pain  <3 + co-existing medical condition  Can’t keep glucose level >3.5mmol/L  Child is on continuous SC insulin pump therapy

Answer 179

• Associated conditions + Complications Monitor for o Thyroid disease – at dx + then annually o Nephropathy – Moderately increased albuminuria (A: Cr ratio 3-30 mg/mmol) to detect diabetic kidney disease, annually from 12 years o HTN, annually from 12 years o Retinopathy, annually from 12 years • Screening for eye disease o Children should have an eye examination by an optometrist every 2y until the ag of 12 o Background retinopathy is often found through screening + improved blood glucose mx will  th risk of this progressing to significant diabetic retinopathy o It will help them keep their eyes healthy + prevent problems with their vision o Diabetic retinopathy screening  Begins at 12  Annual monitoring is important – early treatment will improve outcome + reduce the risk of slight loss • Diabetic kidney disease screening o Monitoring for moderately increased albuminuria (ACR 3–30 mg/mmol)  Begins at 12  Annual using the first urine sample of the day ('early morning urine') o If the initial albumin: creatinine ratio is 3-30 mg/mmol, confirm the result by repeating the test on 2 further occasions using first urine samples of the day ('early morning urine') before starting further investigation and therapy  If microalbuminuria (urine ACR 3-30 mg/mmol) is detected, improving blood glucose control should reduce the risk of progression to significant diabetic kidney disease o Investigate further if initial albumin: creatinine ratio is >30 mg/mmol (proteinuria) o monitoring is important – early treatment will improve outcome • Diabetic foot problems screening o <12 – basic foot care advice  Check daily the entire surface of both feet, including areas between the toes  Foot emergencies and who to contact  Footwear advice, including to avoid shoes that are too tight, have rough edges, or uneven seams; to avoid tight-fitting socks and change socks daily.  Nail-cutting advice  How to recognize foot ulcers and pre-ulcerative signs. o 12-17 – annual assessment

Answer 180

• Psychological interventions o Difficulties with diabetes related family conflict – behavioural family systems therapy o Health related quality of life – CBT o Adherence to diabetes treatment – motivational interviewing o Blood glucose management if high HbA1c levels (>69 mmol/mol (8.5%)) – multisystemic therapy o Screen for depression + anxiety o T1DM + ED – joint management involving their diabetes team + child mental health professionals

Answer 181

• Immunisations o Annual immunisation against influenza starting when they are 6m o Immunisation against pneumococcal infection if they are taking insulin or oral hypoglycaemic medicines

Answer 182

• How to receive treatment o Home-based care + support from local paediatric diabetes team (incl. 24h telephone access) o Initial inpatient mx if children <2y or if social/ emotional factors make home-based mx inappropriate and children who live far away from the hospital • Attend clinic 4x a year

Answer 183

• Education o Insulin therapy (aims + how it works), delivery (rotating injection sites within the same body region), dosage adjustment o Blood glucose monitoring incl. HbA1c + targets o How diet, physical activity, intercurrent illness affect blood glucose level o Sick day rules incl. monitoring of blood ketones o Detecting + mx hypoglycaemia, hyperglycaemia, ketosis o Regular contact with the diabetes team will help them maintain optimal blood glucose levels o Medical bracelet o Honeymoon period = partial remission phase when they start insulin, where they only need a low dosage of insulin (0.5 u/kg/day) to maintain HbA1c <48mmol/mol (6.5%) o Explain the sx of DKA – NV, abdo pain, hyperventilation, dehydration, reduced consciousness • Support o Provide this information at the time of dx + regularly thereafter o Information about support groups + organizations – Diabetes UK website o Offer emotional support after dx o Offer timely and ongoing access to mental health professionals with an understanding of diabetes  children may experience psychological problems (e.g. anxiety, depression, behavioural + conduct disorders, family conflict) pr psychosocial difficulties that can impact on the management of diabetes + wellbeing

Answer 184

• Talk about o HbA1c monitoring targets o How diet, physical activity, body weight, intercurrent illness affect glucose levels o How metformin can help + possible adverse effects o Complications of T2DM + how to prevent them

Answer 185

Annual monitoring for o HTN starting at dx o Dyslipidaemia starting at dx o Moderately increased albuminuria (albumin: cr [ACR] 3-30 mg/mmol) to detect diabetic kidney disease, starting at dx o Diabetic retinopathy screening from 12 years

Answer 186

• HbA1c targets + monitoring o <48 mmol/mol (6.5%) o Measure HbA1c every 3 months o Any reduction in HbA1c reduces their risk of long-term complications – benefits of safely achieving + maintaining the lowest attainable HbA1c o Achieving + maintaining blood glucose levels towards the lower end of the target optimal ranges will help them to achieve the lowest attainable HbA1c

Answer 187

Referral • Very urgent direct access XR to be performed within 48h to assess for bone sarcoma in children + young people with unexplained bone swelling or pain • Very urgent referral (appt. within 48h) for specialist assessment if bone XR suggests the possibility of bone sarcoma Information + support • Explain to people that they are being referred to a cancer service – reassure them that most people referred will not have a dx of cancer + discuss alternative dx with them • Give the person information on the possible dx in accordance with their wishes for information • Information to be shared with the patient o Where the person is being referred to o How long they will have to wait for the appointment o How to obtain further information about the type of cancer suspected or help before the specialist appointment o What to expect from the service the person will be attending o What type of tests may be carried out, and what will happen during diagnostic procedures o How long it will take to get a diagnosis or test results o Whether they can take someone with them to the appointment o Who to contact if they do not receive confirmation of an appointment o Other sources of support • Assess pt’s need for continuing support while waiting for their referral appointment o Invite pt to contact their healthcare professional again if they have more concerns or questions before they see a specialist • If cancer suspected in a child – discuss referral decision + information to be given to child with the parents

Answer 188

Low grade disease • Wide surgical resection + reconstruction High grade disease • Surgery o Complete surgical resection of primary tumour o Wide resection of the involved bone o Replacement with a prosthetic implant or cadaveric bone graf • Adjuvant chemotherapy o Methotrexate + calcium folinate + doxorubicin + cisplatin o 42w • +/- Neoadjuvant chemotherapy o No significant difference in disease-free + overall survival times between regimens that use neoadjuvant chemo and those that use adjuvant chemo

Answer 189

Metastatic disease 1st line • Neoadjuvant chemotherapy o Methotrexate + calcium folinate + doxorubicin + cisplatin + ifosfamide • Surgery o Complete surgical resection of metastatic foci with wide clear margins • Adjuvant chemotherapy o Methotrexate + calcium folinate + doxorubicin + cisplatin + ifosfamide o 34w • +/- Radiotherapy 2nd line • Neoadjuvant chemotherapy o Methotrexate + calcium folinate + doxorubicin + cisplatin + ifosfamide • Radiotherapy alone o If surgical resection is not a viable option following neoadjuvant chemotherapy o If surgery is declined by the patient • Adjuvant chemotherapy o Methotrexate + calcium folinate + doxorubicin + cisplatin + ifosfamide Recurrent disease 1st line • Surgery • Chemo 2nd line • Palliation with supportive care, radioactive samarium 153 +/or radiotherapy

Answer 190

o Refer to a paediatric specialist care for treatment with parenteral abx o Send urine sample for M+C o Manage in line with the NICE guideline on fever  Parenteral abx  Ceftriaxone/ cefotaxime + ampicillin/amoxicillin (according to NICE guidelines for fever <5)

Answer 191

manage according to urine dipstick results o Leukocytes -ve, nitrites -ve  Do not start abx  Do not send urine sample for M+C unless see ix o Leukocytes or nitrites or both positive  Start abx  Send urine sample for culture

Answer 192

o Consider referral to paediatric specialist o First line abx  Cefalexin PO 7-10 days  Co-amoxiclav PO (only if culture results available + susceptible)  Co- amoxiclav IV (only in combination or if culture results available and susceptible)  Cefuroxime IV  Ceftriaxone IV  Gentamicin IV  Amikacin IV

Answer 193

``` o 3 days of o First line  Trimethoprim  Nitrofurantoin o Second line  Nitrofurantoin – if not used as first line  Amoxicillin (only if culture results available + susceptible)  Cefalexin ```

Answer 194

Start abx if LE+ve, N+ve - sample for M+C if high/intermiediate risk of serious illness +/or PMH of previous UTI LE-ve, N+ve - sample for M+C Do not start abx unless there is a good evidence of UTI LE+ve, N-ve - sample for M+C

Answer 195

o <3m or high risk of serious illness  urgent referral to paediatric specialist o >3m  Teat current UTI  Specialist advice re. daily abx prophylaxis if behavioural + personal hygiene measures alone are not effective or appropriate • Review within 6m + at least every 6m • Remind parents/carers about behavioural + personal hygiene measures + self-care assessments • Seek medical help if sx of acute UTI • Trimethoprim – 1st line • Nitrofurantoin – 1st line • Cefalexin – 2nd line • Amoxicillin – 2nd line

Answer 196

o Come back if symptoms have not resolved within 24-48h o Importance of completing course of abx o Paracetamol for pain relief o Adequate hydration o Access to clean toilets, should not be expected to delay voiding o UTI recurring  need to seek prompt treatment from HCP o Give leaflets

Answer 197

• Follow up o In 24-48h if they do not respond to treatment  Consider alternative dx  Send sample for MC o If no imaging investigations or if imaging results are normal  no need for F/U o If recurrent UTI or abnormal imaging results  refer to paediatric specialist for assessment  if renal parenchymal defects assessment should incl. height, weight, BP + routine testing for proteinuria o if bilateral renal abnormalities, impaired kidney function, raised BP +/or proteinuria should receive monitoring + appropriate mx by a paediatric nephrologist to slow the progression of CKD o if AN diagnosed renal abnormality +/or HTN  paediatric nephrologist follow up

Answer 198

* Treatment of underlying disorder * Order coagulation screen * Do a risk assessment for DIC (see above) * Platelet transfusion * Consider when <20x10^9/L or <50 x10^9/L with active bleeding * Coagulation factors (FFP) * Significant bleeding or fibrinogen <100mg/dl or <2.94 micromol/L * Risk – blood borne disease, febrile reactions, hypotension * Recombinant factor VII – to control severe refractory haemorrhagic episodes * Cryoprecipitate * Protein C concentrates may be used, particularly in purpura fulminans due to meningococcal septicaemia or congenital protein deficiency (in neonates) * Restoration of physiological coagulation pathways (e.g. heparin – however use is controversial)

Answer 199

* Treatment of underlying disorder * Thrombotic signs > hyperfibrinolytic signs + no evidence of significant bleeding * Heparin * Augments antithrombin III activity + inhibits conversion of fibrinogen  fibrin * Thrombotic signs < hyperfibrinolytic signs * Antifibrinolytic agents – tranexamic acid, aminocaproic acid * Indications * Hyperfibrinolysis associated with APML – acute promyelocytic leukaemia * Severe refractory bleeding resistant to conventional replacement therapy

Answer 200

* Genetic counselling * Iron advice – avoid iron supplementation for their anaemia unless they are actually iron deficient * Prenatal diagnosis via chorionic villus sampling

Answer 201

• Regular transfusion or transfusions at times of symptomatic anaemia o Do not usually require regular transfusions (non-transfusion dependent thalassaemia) o May require transfusion at times of major stress to the body – perioperatively, during a serious illness or infection o If profound anaemia then patients may require regular transfusions to prevent growth + development impairment, changes in appearance + habitus • Iron monitoring + chelation o Deferasirox PO or desferrioxamine SC o Serial measurement of liver iron concentration (LIC) over time is an accurate + reliable means of monitoring the progression of iron loading + the efficacy of chelation therapy o Should be started when LIC is >5mg Fe/g dry weight + stopped when it falls to <3 o Chelation therapy should be temporarily suspended if there is any suspicion of a bacterial or fungal illness until this is appropriately managed • Genetic counselling • +/- • Splenectomy o 2 weeks prior to splenectomy • pneumococcal vaccine, Haemophilus influenzae b vaccine, quadrivalent meningococcal polysaccharide vaccine • NHS vaccines – Neiserria meningitidis, Haemophilus influenza, Strep. Pneumoniae Pneumococcal revaccination every 5 years o Penicillin prophylaxis for at least 2 years + vaccination against pneumococcus • Assessment for SCT o Only therapy that offers cure

Answer 202

• Regular transfusion o Mainstay of treatment o Goal – maintain a Hb level that allows normal growth + development + suppress ineffective erythropoiesis (Hb >95-100 g/L at all times) o Transfusion typically starts between the age of 18m + 2y with 1u of packed RBC every 3-4w o Adults are typically transfused with 2u every 2-3w o If not SCT – regular transfusions will be continued lifelong • Iron monitoring + chelation o Deferasirox PO or desferrioxamine SC o For young children chelation is not used until 2y of age – before that do an MRI to obtain a baseline measurement of LIC o Chelation therapy should be temporarily suspended if there is any suspicion of a bacterial or fungal illness until this is appropriately managed • Genetic counselling • Splenectomy o Performed if the spleen is massively enlarged with risk of spontaneous or traumatic rupture o Or if the transfusion requirement is so high that the resulting iron overload would not be adequately managed by regular chelation – splenectomy may reduce transfusion requirements by 20-30% o 2 weeks prior to splenectomy • pneumococcal vaccine, Haemophilus influenzae b vaccine, quadrivalent meningococcal polysaccharide vaccine • NHS vaccines – Neiserria meningitidis, Haemophilus influenza, Strep. Pneumoniae Pneumococcal revaccination every 5 years o Penicillin prophylaxis for at least 2 years + vaccination against pneumococcus • Assessment for SCT

Answer 203

• Most children can be managed at home • Indications for treatment o Major bleeding – intracranial or GI bleeding o Persistent minor bleeding that affects daily life (e.g. excessive epistaxis)

Answer 204

• IVIG + CS (prednisolone) + plt transfusion o Emergency treatment may take 1-5 days to have an effect o Usually lasts for 2-4w o IVIG can prolong plt survival o CS prevent immune-mediated destruction of plt • Antifibrinolytic o CI in pt with haematuria – clots in the collecting system of the kidneys can lead to outlet obstruction o Aminocaproic acid or tranexamic acid o Inhibit fibrinolysis to help stabilise clots that have already formed

Answer 205

Newly diagnosed child Asymptomatic or with minor bleeding sx (e.g. bruising, petechiae, purpura) • Observation (most will achieve a normal plt count eventually) • Reduce trauma risk • Avoid antiplatelet medications (e.g. aspirin, NSAIDs) • Most manifestations are limited to the skin Major bleeding sx (e.g. mucosal bleeding) • CS – 1st line • IVIG or anti-D immunoglobulin – if CS are CI o Anti-D only in pt who are Rh-+ve + non-splenectomised

Answer 206

(ITP 3-12m after dx (persistent), ITP >12m (chronic)) treatment goal – increase plt count to control clinically relevant bleeding or minimise risk of major bleeding with minimum toxicity while awaiting for spontaneous remission or amelioration 1st line • Mycophenolate mofetil • Rituximab • Thrombopoietin receptor antagonist (e.g. eltrombopag, romiplostim) 2nd line • Splenectomy (+ treatment to achieve target platelet level) o If persistent/chronic disease + unresponsive to /intolerant of pharmacological interventions o Preoperative plt cout >20x10^9/l considered safe o IVIG +/or PO CS may be used a few days in advance of the surgery to increase plt levels o 2 weeks prior to splenectomy • pneumococcal vaccine, Haemophilus influenzae b vaccine, quadrivalent meningococcal polysaccharide vaccine • NHS vaccines – Neiserria meningitidis, Haemophilus influenza, Strep. Pneumoniae o Pneumococcal revaccination every 5 years o Post splenectomy – prophylactic phenoxymethylpenicillin for at least 2 years, ideally until they are 5

Answer 207

Education • Risk of acute events • Education on complications • Genetic counselling • HbH – seek medical help if increased fatgue, SOB, jaundice, dark urine • Post-splenectomy sepsis – immediate medical evaluation in the case of febrile illness * Normal Hb levels * Avoidance of unnecessary iron supplementation * Supportive care * Genetic counselling

Answer 208

Education • Risk of acute events • Education on complications • Genetic counselling • HbH – seek medical help if increased fatgue, SOB, jaundice, dark urine • Post-splenectomy sepsis – immediate medical evaluation in the case of febrile illness * Mild asymptomatic anaemia * Avoidance of unnecessary iron supplementation * Supportive care * Genetic counselling

Answer 209

• At risk of complications – see below Education • Risk of acute events • Education on complications • Genetic counselling • HbH – seek medical help if increased fatgue, SOB, jaundice, dark urine • Post-splenectomy sepsis – immediate medical evaluation in the case of febrile illness * Folic acid supplementation * Supportive care * RBC transfusion • Iron chelation therapy o Deferasirox PO or desferrioxamine SC • Splenectomy + preoperative vaccination + postoperative phenoxymethylpenicillin + antiplatelet agents o Splenectomy – if pt develops painful splenomegaly, hypersplenism with associated pancytopenia, an increase in transfusion requirement, poor growth and development due to worsening anaemia, lack of availability of transfusion or chelation therapy o Splenectomy may be particularly helpful in raising Hb level o 2w Prior to splenectomy – immunisation with pneumococcal vaccine, Haemophilus influenzae b vaccine, quadrivalent meningococcal polysaccharide vaccine Pneumococcal revaccination every 5 years o Post splenectomy – prophylactic phenoxymethylpenicillin for at least 2 years, ideally until they are 5 • HSCT for severe treatment dependent disease – only curative therapy available

Answer 210

* Risk of acute events * Education on complications * Genetic counselling * HbH – seek medical help if increased fatgue, SOB, jaundice, dark urine * Post-splenectomy sepsis – immediate medical evaluation in the case of febrile illness

Answer 211

``` Acute haemolytic episodes • Identification of cause • Monitoring • Folic acid supplementation • RBC transfusion ``` Transient aplastic crisis • Self limiting • RBC transfusion

Answer 212

* Avoid cold, fever, dehydration, stress * Increase fluid intake as dehydration might prolong the panful episode * Distracting techniques e.g. games, computers, television to deal with the pain • Analgesia o Offer within 30 minutes of presentation o Paracetamol used to treat mild pain o NSAIDs used to treat mild to moderate pain – used with caution in patients with mild hepatic/renal impairment o Weak opioids  <13 – dihydrocodeine  >13 – codeine phosphate • Supportive care + correction of cause o Oxygen if O2 <92% o IVF or oral rehydration to help reverse dehydration o Deep breathing exercises to help reverse hypoxia, acidosis * Abx if there is evidence of infection * Exchange transfusion – indicated for life-threatening vaso-occlusive events (acute chest syndrome, priapism, stroke), symptomatic anaemia, acute organ dysfunction • Hydroxycarbamide (hydroxyurea) o Can reduce the frequency of painful crises in sickle cell disease o Given if there are 6+ episodes of vaso-occlusive crisis per year • Folic acid – in severe haemolysis or pregnancy • Safety net – parents to seek help If o Fever o Respiratory sx or other signs of infection o Priapism o Unusual pallor o Weakness (without pain), tingling, loss of speech, any neurological complications

Answer 213

• Education o Should very rarely have symptoms o Risk of vaso-occlusive episode if they become oxygen deprived • Adequate hydration * Avoidance of excessive fluid loss * Avoidance of severe heat * Avoidance of high altitudes – e.g. long haul flights, mountain climbing * Inform anaesthetist that they are sickle cell carriers if they are going to have an anaesthetic * Malaria prophylaxis if they are visiting an area where malaria is endemic * Recurrent splenic sequestration is an indication for splenectomy Refer if • Haematuria • Sx of renal medullary cancer – haematuria, wight loss, loin pain, fever, abdominal pain – 2ww

Answer 214

• Attend hospital if priapism persists for >1h o Pain relief, hydration, oxygenation, alpha-adrenergic agent e.g. etilefrine * Hydration + analgesia * Minor episodes – bladder emptying, gentle exercise, warm baths, analgesia, keeping warm, keeping hydrated at bedtime • Prevention o Alpha adrenergic agents e.g. etilefrine o Anti-androgens

Answer 215

* Enuresis is common in people with sickle cell disease because they produce large quantities of dilute urine * most cases resolve spontaneously. * Give general advice on the management of enuresis * Children w sickle cell disease tend not to respond to behavioural management techniques, such as star charts and mattress alarms

Answer 216

* Monitor annually for sx + signs of renal disease (e.g. UTI, haematuria), for HTN, for presence/progression of albuminuria, proteinuria, declining renal function * Advise a good fluid intake to prevent dehydration * Refer children with HTN * Urgent referral if sx suggestive of medullary cancer – haematuria, abdominal or back pain, weight loss – 2ww * Ix new-onset haematuria regardless of age to exclude malignancy

Answer 217

• Oral hydroxyurea Hydroxycarbamide (hydroxyurea) o Considered in patients aged >2 years with sickle cell anaemia who have recurrent hospital admissions (>3 in 12 months) for acute chest syndrome o Stimulates HbF production o Decreases the frequency of sickle cell crises, reduces transfusion requirements, decreases risk of acute chest syndrome o Monitor for WBC suppression (SE of hydroxycarbamide) • Daily antibiotic prophylaxis o Penicillin prophylaxis  Phenoxymethylpenicillin  Daily  Start at 3 months + continue until 5y/o o Erythromycin prophylaxis if allergic to pencillin o Lifelong prophylactic antibiotics – offered to people considered at continued high risk of pneumococcal infection • Immunisation against encapsulated organisms (S. pneumoniae, H. influenzae type B) o Pneumococcal vaccine at 2y + every 5 years o Influenza vaccine annual from 6m o Meningitis ACWY vaccine * Daly Folic acid if diet does not contain adequate folic acid or if there is proven folate deficiency * Avoid exposure to cold, dehydration, excessive exercise, undue stress, hypoxia * Malaria prophylaxis if travelling to an area where malaria is endemic

Answer 218

* Regular psychological support for children — cognitive behavioural therapy may also be offered to children experiencing frequent pain episodes and emotional difficulties. * Elective blood transfusion — for example, for people with pulmonary hypertension, acute priapism, or severe anaemia. * Hydroxycarbamide treatment — for example, for people who have had recurrent hospital admission for acute chest syndrome or acute painful crises. * Surgery — for example, to relieve pain due to avascular necrosis, or splenectomy for people who have had two or more episodes of acute splenic sequestration. * Stem cell transplantation — this is the only cure for sickle cell disease and may be considered in children and some adults with severe sickle cell disease

Answer 219

* When to seek urgent medical assistance. * How to examine their child for pallor and an enlarging spleen. * The importance of keeping warm, avoiding sudden changes in temperature, and avoiding dehydration (by maintaining a good fluid intake) * The importance of adherence to prophylactic antibiotics and the immunization schedule. * When to seek early medical advice, for example if the child has a fever, respiratory symptoms, signs of infection, unusual pallor, an enlarged (or enlarging) spleen, signs of stroke (weakness, tingling, or loss of speech), or painful crisis and dactylitis. * To report any concerns, for example about a child's development or any deterioration in school achievement. * To seek advice about intended travel to a foreign country, so that travel immunization and malarial prophylaxis are provided.

Answer 220

• Repeated blood transfusions o Common prophylactic treatment to maintain HbS below 30% o Required for severe anaemia or to reduce the proportion of HbS if there are lung or CNS complications, severe symptoms refractory to treatment (haemodynamic instability due to hypovolaemic shock) o Iron overload is a common complication – chelation should be started in all children receiving regular blood transfusions (SC deferoxamine, oral deferasirox, oral deferiprone) • Splenectomy + preoperative vaccination + postoperative phenoxymethylpenicillin o Splenectomy – if pt develops painful splenomegaly, hypersplenism with associated pancytopenia, an increase in transfusion requirement, poor growth and development due to worsening anaemia, lack of availability of transfusion or chelation therapy o Splenectomy may be particularly helpful in raising Hb level– splenectomy may reduce transfusion requirements by 20-30% o Prior to splenectomy – immunisation with pneumococcal vaccine, Haemophilus influenzae b vaccine, quadrivalent meningococcal polysaccharide vaccine o Post splenectomy – prophylactic phenoxymethylpenicillin for at least 2 years, ideally until they are 5 ((Penicillin prophylaxis should be offered to all children with sickle cell disease, started by 3 months of age and continued until the child is 5 years old. )) • Bone marrow transplantation o 2nd line, considered in children with severe complications of sickle cell anaemia (e.g. stroke, recurrent acute chest syndrome) who are unresponsive to first-line therapies • Stroke o Transcranial Doppler US performed annually in children aged 2-16 years o Regular blood transfusions considered in those with abnormal findings on transcranial Doppler US to keep their sickle hb percentage <30% • OSA  refer for sleep studies • Eye problems o No retinopathy – Pt should be evaluated every 2-3 years by an ophthalmologist o Retinopathy – annual review o Ocular problems most common in people aged 15-30

Answer 221

• Red flag – refer urgently o Severe pain not controlled by simple analgesia or low dose opioids. o Dehydration caused by severe vomiting or diarrhoea. o Severe sepsis.  Temperature over 38.5°C, or over 38°C in children aged under 2 years, temperature less than 36°C, or hypotension. o Symptoms or signs of acute chest syndrome including tachypnoea, oxygen saturation more than 5% below steady state, signs of lung consolidation. o New neurological symptoms or signs. o Symptoms or signs of acute fall in haemoglobin. o Acute enlargement of spleen or liver over 24 hours, particularly in young children. o Marked increase in jaundice. o Any change in vision. o Haematuria. o Fulminant priapism lasting more than 2 hours or worsening of recurrent episodes.

Answer 222

• Impaired growth o Zinc supplementation o Refer to paediatric dietician if child is hospitalized frequently o Refer to paediatric endocrinologist if there are no physical signs of puberty by 14 years of age in a girl and by 14y+6m in a boy o Reassure children with delayed growth + delayed puberty that they will reach a normal adult height

Answer 223

From 12 y onwards • Address underlying cause o If dietary deficiency – person to maintain adequate balanced intake of iron-rich foods e.g. dark green vegetables, iron-fortified bread, meat, apricots, prunes, raisins) + consider referral to dietician • 200mg Ferrous sulphate PO OD o 65mg elemental iron o Should be continued for 3/12 after iron deficiency is corrected to allow stores to be replenished o If not tolerated – reduce dose to one tablet on alternate days o Parenteral iron if PO iron CI, ineffective, not tolerated o Do not wait for ix to be carried out before prescribing supplements

Answer 224

• Monitor the person to ensure that there is an adequate response to treatment o Recheck Hb levels (FBC) within the first 4 weeks of iron supplement treatment o The Hb concentration should rise by about 20 g/L over 3-4 weeks o If there is a response  check FBC in 2-4m to ensure that Hb levels have returned to normal o If no response  address compliance issues • Once Hb concentration + red cell indices are normal o Continue iron treatment for 3 months to aid replenishment of iron stores, then stop o Then monitor the person’s FBC periodically e.g. 3-monthyl for 12m, then 6 monthly for 2-3 years o If Hb or red cell indices drop below normal – prescribe iron supplements

Answer 225

``` • Prophylactic iron supplementation o Dose – 200mg ferrous sulfate OD o Indications • An iron-poor diet – e.g. vegans • Malabsorption – e.g. coeliac disease • Recurring anaemia • Gastrectomy • Undergoing haemodialysis ```

Answer 226

• Referral o coeliac serology is positive — refer to gastroenterology. o profound anaemia with signs of heart failure o unable to tolerate, or are not responding to, oral iron treatment o Who have initially responded to iron treatment but develop anaemia again without an obvious underlying cause o When the type of anaemia is in doubt o When further haematological investigations, such as bone marrow examination or an investigation of bleeding state, cannot be carried out in primary care

Answer 227

``` Education • Adverse effects of iron tablets o Constipation/ diarrhoea o Epigastric pain o Faecal impaction o GI irritation o Nausea ``` • Safe storage of iron tablets – accidental OD can be fatal • GI adverse effects o May be minimised by taking the iron supplements with or after food (this may decrease iron absorption by 40-66%), o or reducing the dose frequency to alternate days • explain monitoring requirements

Answer 228

Factor concentrates • Haem A  recombinant factor VIII • Haem B  recombinant factor IX Life-threatening bleed (CNS bleed, GIB, airway bleed, pseudotumor) • Factor concentrate – either VIII (A) or IX (B) • Supportive care + subspecialty consultations o ABC o If intracranial haemorrhage – anticonvulsant therapy • Antifibrinolytic agent – tranexamic acid or aminocaproic acid o Tranexamic acid CI in the treatment of haematuria + in the setting of thoracic surgery o Antifibrinolytic agents should not be given concomitantly with factor VIII If inhibitor present • High dose factor concentrate +/- • Bypassing agent o Contains variable amounts of activated + precursor vitamin K dependent clotting factors (II, VII, IX, X) which generate thrombin by bypassing the coagulation cascade

Answer 229

Factor concentrates • Haem A  recombinant factor VIII • Haem B  recombinant factor IX * Factor concentrate * Analgesics – paracetamol * Physiotherapy evaluation * Orthopaedic team evaluation – if risk of contracture or muscle atrophy due to recurrent bleeds at the same time * Pain team evaluation If inhibitor present • High dose factor concentrate +/- • Bypassing agent o Contains variable amounts of activated + precursor vitamin K dependent clotting factors (II, VII, IX, X) which generate thrombin by bypassing the coagulation cascade

Answer 230

Haem A • Desmopressin + supportive care – 1st line o Stimulates the endogenous release of factor VIII and VWF o Desmopressin should produce a 2- to 4-fold rise in the levels of factor VIII o Patients advised to limit water intake in order to reduce the risk of developing hyponatraemia (<1.5L for 24h after desmopressin administration) o Should not be used in children <2 • On demand factor VIII concentrate + supportive care – 2nd line o Factor VIII concentrate given on demand every 12-24h until sx have resolved • Antifibrinolytic agent ``` Haem B or moderate-severe haem A • On demand factor concentrate • Supportive care • Antifibrinolytic agent • (desmopressin not effective in Haem B as factor IX levels are not influenced by it) ``` If inhibitor present • High dose factor concentrate +/- • Bypassing agent o Contains variable amounts of activated + precursor vitamin K dependent clotting factors (II, VII, IX, X) which generate thrombin by bypassing the coagulation cascade

Answer 231

Prophylaxis in severe haemophilia A or B • Regular, continuous IV factor replacement given for at least 45 weeks/year in anticipation of and to prevent bleeding o Primary prophylaxis = therapy initiated in young patients (aged <3) with haemophilia, prior to clinically detectable joint damage + before the second major joint bleed o Secondary prophylaxis = therapy initiated after >2 bleeds into large joints and before the onset of joint disease o Tertiary prophylaxis = therapy initiated after development of joint disease ``` • Haemophilia A o Prophylactic factor VIII o 2-3x weekly o Reduces the risk of chronic joint damage o Emicizumab ``` • Haemophilia B o 2x weekly • Recurrent bleeds into a single specific (target) joint  radioactive synovectomy

Answer 232

* IM injections * Aspirin * NSAIDs

Answer 233

• Admit + treat as an emergency (A-E approach) • Initial mx if suspected viral aetiology o Immunocompetent  IV Acyclovir • All cases of suspected community – acquired viral encephalitis are started empirically on acyclovir until the cause is determined • Why? Because most cases of sporadic viral encephalitis are secondary to HSV  Supportive care • Admit until full evaluation • Patient might need to be in ICU • ETT + mechanical ventilation • IVF – Circulatory + electrolyte support • Prevention + mx of secondary bacterial infections • DVT prophylaxis • Decrease ICP if elevated – with CS + mannitol o Immunocompromised  Combination antiviral therapy – IV ganciclovir + IV foscarnet + IV acyclovir • Ganciclovir + foscarnet for CMV • Acyclovir for HSV  Supportive care

Answer 234

* HSV – IV acyclovir (2-3/52) * VZV – IV acyclovir (2/52) or ganciclovir (2-3/52) * CMV – IV ganciclovir + IV foscarnet • EBV – IV acyclovir or IV ganciclovir or IV cidofovir o Aciclovir is first line in suspected viral encephalitis, but once the diagnosis of EBV is confirmed, ganciclovir or cidofovir are possible alternatives * HBV - IV ganciclovir (1) or IV acyclovir(2) or IV valaciclovir(2) (2-3/52) * HHV-6 – IV ganciclovir or IV foscarnet * Non-herpes virus – supportive care +/- antiviral therapy • Non-viral aetiology - supportive care + treatment of underlying aetiology o Autoimmune encephalitis – immune-modulating therapy – IV CS or IVIG or plasma exchange o Syphilis – benzylpenicillin o Listeria – ampicillin + gentamicin o Mycoplasma – doxycycline or erythromycin

Answer 235

• Follow up o Monitor for the development of a seizure disorder or hydrocephalus o Monitor for the development of ADHD + cognitive problems o Neuropsychological testing o Rehabilitation – cognitive/ behavioural rehabilitation + motor/ambulatory rehabilitation

Answer 236

• Reassure parents o Not a serious condition o Does not trouble infant o Typically resolves spontaneously within a few months – usually by 8m • Cradle cap o Conservative  Massage topical emollient/olive/vegetable oil onto scalp to loosen scales  Brush gently with a soft brush  Wash off with shampoo  Thicker scales can be soaked overnight with olive/vegetable oil or petroleum jelly and then shampooed in the morning o Topical imidazole cream  Clotrimazole 1% cream – 2-3x daily for up to 4 weeks  Miconazole 2% cream – BD for up to 4 weeks • other areas affected (incl. nappy area) o Conservative  bathe infant using emollient as a soap substitute  avoid soaps/detergents + vigorous cleansing  frequent nappy changes + use of barrier emollients o topical imidazole (clotrimazole 1% or miconazole) – treat for up to 4w until sx resolve • Recurrence – repeat a course of treatment

Answer 237

``` • Seborrheic dermatitis of the face and body o Imidazole cream  Clotrimazole (2-3x/day)  Miconazole BD  For up to 4 weeks o Mildly potent topical CS for flares  Hydrocortisone 0.5% or 1%  Should only be used for short term (1w)  SE – thinning of skin o Antifungal shampoo  Ketoconazole 2%  Only in adolescents – >12y can be used as body wash  Leave on for 5 mins before rinsing off  Use 2-3x day until sx disappear o Other treatments (specialties guide)  Selenium sulphide shampoo  Coal tar  Salicylic acid ointment  Pyrithione zinc  Ciclopirox o Safety netting + referral same as in infants ``` • Widespread seborrheic dermatitis o Consider serious underlying causes and alternative dx o Discuss urgently with paeds or derm

Answer 238

Infants + children + adolescents • Safety net – return if o Response to treatment is poor o Symptoms worsen despite treatment o Signs of infection (e.g. crusting, oozing, bleeding) develop • Referral to a dermatologist/ paediatrician if o Severe or widespread seborrheic dermatitis – consider possible serious underlying conditions such as immunodeficiency o Diagnostic uncertainty o Failure to respond to routine treatment – after 4w o Eyelid involvement

Answer 239

• Parent education o Nappy with high absorbency o Ensure that nappy fits properly o Leave nappies off for as long as possible to help skin drying of the nappy area o Clean skin + change nappy every 3-4h or asap after wetting or soiling to  skin exposure to urine or faeces  Water or fragrance free + alcohol free baby wipes  Dry gently after cleaning  Bath child daily but avoid excessive bathing (>2x a day)  Do not use soap/ bubble bath lotions/ talcum powder/ topical antibiotics which can have an irritant effect • Should settle with appropriate management in primary care • Typically lasts about 3 days • Mild erythema + asymptomatic child o Barrier preparation to protect skin  OTC zinc and castor oil ointment, metanium ointment, soft white paraffin ointment o Apply thinly at each nappy change • Inflamed rash + discomfort o >1m – topical hydrocortisone 1% OD  Until symptoms settle or for a max. of 7 days  topical hydrocortisone first + wait a few minutes before applying barrier preparation o Barrier preparation • Persisting rash + candida infection suspected or confirmed on swab o Topical imidazole cream – e.g. clotrimazole, econazole, miconazole o Do not use barrier preparation until candida infection has settled • Persisting rash + bacterial infection suspected or confirmed on swab o Flucloxacillin PO 7/7 o Clarithromycin PO 7/7 if allergic to penicillin • Review • Treatment failure o Manage underlying cause of treatment failure  Review adherence + self-care advice  Skin swab for candida or bacterial secondary infection  Consider alternative cause for the rash o If rash persists following treatment for secondary infection  Adjust the choice of topical imidazole or PO abx if indicated or  Seek specialist advice from medical microbiologist o Consider referral o paediatric dermatologist if  Uncertainty about the diagnosis  Rash persists despite optimal treatment in primary care  There are recurrent, severe unexplained episodes

Answer 240

• Do not need treatment • Can be mistaken for bruises and thus child abuse/safeguarding issues o So should be recorded on baby’s medical records from birth

Answer 241

• Reassure o Do not require treatment if immunocompetent o Self-limiting condition o Spontaneous remission within 18m • Advice o Contagious lesions – avoid sharing towels, clothing, bedding o Do not scratch or squeeze lesions – avoid spread of infectious material +  risk of superinfections o Exclusion from school/ gym/ swimming not necessary – cover lesions with waterproof bandages before swimming • Sx management o Imiquimod 5% cream  Apply 3x/week and wash of 6-10h later o Podophyllotoxin 0.5%  Usually used to treat anogenital warts but effective  Apply 2x/day for 3/7  Repeat after a week if needed o Cryotherapy o Chemical or physical destruction may be done by a specialist only if a lesion has become symptomatic o Eczema or inflammation can develop around lesions prior to resolution  treat appropriately (e.g. emollients, steroids, abx) o Secondary bacterial infection – topical antibacterial • Support o NHS website • Referral to o Ophthalmology for people with eyelid-margin or ocular lesions + associated red eye o Dermatologist – dx uncertainty, immunocompromised, extensive painful lesions (although inflamed lesions may indicate resolution)

Answer 242

``` Asymptomatic • Education + reassurance o undergo involution o do not necessarily need treatment o needs to be looked after as they can bleed when scratched ``` Functional impairment (near eyes, nose, mouth) or cosmetic disfigurement • BB PO or topical o Propranolol (PO), timolol (topical) o Propranolol – Rebound growth after cessation => treatment continued through the time of theoretical involution or around 12m o Topical – for the treatment of superficial infantile haemangiomas • +/- CS PO o Prednisolone o Systemic CS – 6-12m • Surgical excision o Cryotherapy, electrotherapy, vascular laser surgery o If conservative treatment inadequate o If presence of ulceration or bleeding o To improve cosmetic appearance after involution is complete Ulceration • Astringents + barrier protection o Barrier protection o Burow’s solution compresses OD or BD for gentle debridement o Petrolatum-impregnated gauze – discomfort from lesions in the perineal area • Topical abx o Metronidazole o PO if secondary infection is present • BB if haven’t used previously • Analgesia – paracetamol, topical lidocaine • Topical becaplermin o Recombinant human plt derived GF o Promotes wound repair, cell proliferation, granulation tissue formation • Pulsed dye laser

Answer 243

``` • No treatment required • Needle removal (doesn’t require LA) • Other mx o Cryotherapy o Laser treatment o Dermabrasion o Chemical peeling ```

Answer 244

``` • Malaria is a medical emergency • Discuss with specialist • Notifiable disease – notify to PHE • Emergency admission + specialist treatment if o Severe or complicated malaria o Falciparum malaria o Child/ pregnant/ >65 ``` • Advice o Warn other members of family/ travel group o Acute episode from malaria will not protect against future attacks – will still need malaria chemoprophylaxis in the future o Relapses of malaria can occur (from treatment failure due to drug resistance or poor absorption) – must be reported • SE o Medications associated with neurological + psychiatric sx o Treatment failures are rare but have been reported ``` • Children o Notify all cases to HPT/ PHE o Admit o Observe for at least 24h o If you don’t know species treat as p. falciparum ``` ``` o Uncomplicated P. falciparum malaria  Artemisinin combination therapy (e.g. artemether + lumefantrine or artenimol + piperaquine phosphate) • 1st line  Quinine • 2nd line  Quinine + doxycycline • 2nd line • Doxycycline should not be given to children <12  risk of dental hypoplasia + permanent discolouration of teeth  Atovaquone-proguanil hydrochloride ``` o Severe or complicated P. falciparum malaria  HDU or PICU  IV artesunate over min. of 24h followed by oral treatment (artemisinin combination therapy)  IV quinine 2nd line o Non-falciparum malaria (P. vivax + less commonly P. ovale/ malariae/ knowlesi)  Artemisinin combination therapy or chloroquine

Answer 245

Suspected • Supportive care – antipyretics, IVF • IV Ceftriaxone + PO azithromycin Confirmed • Supportive care – antipyretics, IVF • Ciprofloxacin PO 7-14/7 • 4-5 days of treatment without response  add azithromycin PO • Encephalopathic complications  high dose dexamethasone

Answer 246

• Notification to relevant authorities ``` WHO group A (no warning signs) • Home mx • Oral fluids • Monitoring o Monitor for warning signs o Daily blood counts • Tepid sponging +/or paracetamol for fever ```

Answer 247

• Notification to relevant authorities WHO group B (developing warning signs) • Hospital admission • Oral or IVF 0.9% saline for 24-48h • Monitoring o Vital signs, peripheral perfusion, fluid balance o Hct, plt count o U/O, T, BG, LFTs, renal profile, coagulation profile • Hospital discharge planning o Once pt. remains afebrile for 48h with a  plt count + stable Hct, the pt has been discharged

Answer 248

• Notification to relevant authorities • Emergency medical intervention – admission to ICU • Rapid administration of IVF o IV 0.9% NaCl – maintenance + 5% fluid deficit  100ml//kg for first 10kg  50ml/kg for next 10kg  20ml/kg for every kg after 20kg  5% fluid deficit  50ml/kg o For 24-48h • Monitoring o Vital signs, peripheral perfusion, fluid balance o Hct, plt count o U/O, T, BG, LFTs, renal profile, coagulation profil • Blood transfusion o If not improving + Hct falls  suspect internal bleeding • Hospital discharge planning o Once pt. remains afebrile for 48h with a  plt count + stable Hct, the pt has been discharged

Answer 249

Primary care • 999 emergency transfer to secondary care • Suspected meningitis without non blanching rash o Emergency transfer without abx o If urgent transfer not possible – abx • Suspected meningococcal disease (meningitis with non-blanching rash or meningococcal septicaemia) o IM or IV benzylpenicillin immediately  Only withhold if clear hx of anaphylaxis after a previous dose, a hx of rash following penicillin is not a CI (consider moxifloxacin + vancomycin) o Urgent transfer to hospital

Answer 250

• Bacterial meningitis o <3 m  IV cefotaxime/ceftriaxone + amoxicillin or ampicillin for at least 14/7  ceftriaxone CI in premature babies, babies with jaundice, hypoalbuminaemia, acidosis – may exacerbate hyperbilirubinemia o >3 m  IV ceftriaxone for at least 10/7 • If recent travel outside the UK/ prolonged/multiple exposure to abx  add vancomycin to all the abx

Answer 251

``` • Dehydration o PO fluids or o IV 0.9% NaCl + 5% glucose (children) / 10% glucose (neonates) • If  ICP – restrict fluids • Monitor fluid administration + UO + electrolytes + blood glucose • Do not give CS if <3m • Dexamethasone if LP o Frankly purulent CSF o CSF WBC >1000/μl o  CSF WBC + protein >1g/l o Bacteria on gram stain ```

Answer 252

• Shock o 20ml/kg NaCl 0.9% over 5-10 mins (IV or IO) o Reassess immediately o If shock persists  2nd bolus 20ml/kg NaCl .9% or human albumin 4.5% over 5-10 mins o If shock persists after the first 40ml/kg  3rd bolus 20ml/kg NaCl .9% or human albumin 4.5% over 5-10 mins  Call for anaesthetic assistance for urgent tracheal intubation + mechanical ventilation  Vasoactive drugs – IV adrenaline or IV noradrenaline or both  Give further boluses based on clinical signs + appropriate laboratory ix incl. U+Es • Steroids should not be used in meningococcal septicaemia

Answer 253

Respiratory support • Signs of respiratory distress  15-L face mask O2 via a reservoir rebreathing mask • Threatened loos of airway patency  open airway, bag-valve mask ventilation, prepare for tracheal intubation • Indications for tracheal intubation and mechanical ventilation o Threatened (e.g. loss of gag reflex) or actual loss of airway patency o Need for any form of assisted ventilation o Increasing work of breathing o Hypoventilation or apnoea o Resp failure – irregular respiration, hypoxia, hypercapnia o Continuing shock following a total of 40ml/kg of resuscitation fluid o Signs of  ICP o Impaired mental status – GCS <9 or drop of >3 o Control of intractable seizures o Need for stabilisation + mx to allow brain imaging or transfer to PICU or another hospital

Answer 254

Contact management • Consider prophylaxis against meningococcal disease for close contacts regardless of meningococcal vaccination status o Oral ciprofloxacin > rifampicin o Close contact with case during 7 days before onset of illness o People who have been exposed to respiratory secretion o Abx prophylaxis should be given asap (ideally within 24h) after dx of the index case • Offer meningococcal vaccination when serotype results are available, incl. booster doses

Answer 255

Follow up/ parent education • Review in 4-6w with results of hearing rest + to assess recovery • Discuss possible complications • Inform GP, health visitor, school nurse • Offer sources of support • Formal audiological assessment asap, preferably before discharge within 4w of being fit to test • Severe/profound deafness  cochlear implants • Testing for complement deficiency if o >1 episode of meningococcal disease or o 1 episode of meningococcal disease caused by serogroups other than B or o Meningococcal disease caused by any serogroup + hx of other recurrent or serious bacterial infections • If recurrent episodes of meningococcal disease  refer to infectious disease specialist or immunologist

Answer 256

• No active treatment is an option for certain patients with mild symptoms alone, provided that they are monitored closely for disease complications and progression Inducing remission • First presentation or Single inflammatory exacerbation of CD in a 12-month period o Prednisolone or methylprednisolone or IV hydrocortisone – 1st line o Budesonide – 2nd line  Less effective than a conventional glucocorticosteroid but may have fewer side-effects o 5-ASA/ Aminosalicylate (mesalazine, sulfasalazine) – 2nd line   frequency of relapses  Useful for mild to moderate disease  More commonly used in UC  Less effective than conventional glucocorticoid/budesonide but fewer side-effects • Children/ young people o Enteral nutrition o Considered as an alternative to a conventional GC to indue remission for children + young adults in whom there is concern about growth Add-on treatment Add to conventional GC or budesonide if: • >2 inflammatory exacerbations in a 12m period or GC dose cannot be tapered o Add azathioprine or mercaptopurine– 1st line  Immunomodulator therapy  Before offering azathioprine or mercaptopurine assess thiopurine methyltransferase (TPMT) activity  Monitor for neutropenia  Thiopurines may  the person’s risk of non-melanoma skin cancer – sun protection advice + monitor for skin cancer o Add methotrexate – 2nd line Severe active CD not responding to conventional therapy • Infliximab for children 6-17y/o o anti tumour necrosis factor-α (anti-TNFα) monoclonal antibody o Very effective at inducing and maintaining remission o Usually reserved for refractory Crohn’s • Severe active CD = very poor general health with >1: weight loss, fever, severe abdominal pain, frequent (>3-4) diarrhoeal stools

Answer 257

``` Maintaining remission • Can choose no treatment o Follow-up o Educate on signs of relapse – unintended weight loss, abdominal pain, general ill health o No smoking • Can choose treatment o Azathioprine (purine synthesis inhibitor) or mercaptopurine – 1st line o Methotrexate – 2nd line • After surgery o Azathioprine or mercaptopurine ``` This is both to avoid steroid-related complications (e.g. diabetes, cataracts, osteoporosis) + because steroids do not modify disease or induce sustained mucosal healing

Answer 258

Surgery • If disease is limited to terminal ileum • Consider surgery as an alternative to medical treatment early in the course of the disease or before or early in puberty for people who have o Growth impairment despite optimal medical treatment and/or o Refractory disease • Resection of affected bowel + stoma formation • Temporary solution – other portions of the bowel will ultimately become affected too – risk of disease recurrenc • Strictures o Balloon dilation

Answer 259

* Refer to secondary care/ paediatric gastroenterologist– dx of CD + initiation of treatment should be done in secondary care * MDT to address – concerns about disease and its treatment, concerns about body image, living with a chronic illness, attending school, higher education Primary care F/U • Analgesia for pain o Paracetamol o Opiates may be needed for additional sx relief o Avoid NSAIDs – may aggravate CD • Symptomatic treatment o Anti-diarrhoeal drugs (e.g. loperamide) o Anti-spasmodic drugs for pain relief (e.g. mebeverine, dicycloverine, hyoscyamine) o Bulk forming laxatives (e.g. ispaghula husk) o *** do not prescribe symptomatic treatment for people with active colitis, people who are systemically unwell, have abdominal tenderness or sigs of intestinal obstruction   risk of toxic megacolon • Assess impact of sx on daily functioning – home, work, school, leisure activities, anxiety, depression • Encourage stopping smoking (may  risk of relapse) • Information and support (Crohn’s and Colitis UK) • Assess risk of osteoporosis incl. dietary calcium intake + extent of CD + hx of small bowel resection • Colonoscopy screening for colorectal cancer if sx started 10y ago + CD affecting >1 segment • Monitoring – serum ferritin, vitamin B12, folate, calcium, vitamin D • Assess for clinical features suggesting disease flare up o BMI, unintended weight loss, signs of malnutrition o CRP • If on immunosuppressive or biologic therapy o Live vaccines are CI o Vaccines can be given before the start of specialist treatment or else postponed for at least 6m after stopping therapy o Increased risk of influenza and pneumococcal infection so should receive those vaccines

Answer 260

Acute exacerbation • Admission if o Severe diarrhoea (>6-8 stools/day) o Fever, dehydration, tachycardia, hypotension o Suspected intestinal obstruction or intra-abdominal or perianal abscess o Cachexia or BMI <18.5 kg/m2 or unintended sudden weight loss o Persistent sx despite optimal mx in primary care • If hospital admission not indicated o Check adherence o Consider urgent specialist gastroenterology review appt or seek specialist advice o Consider dietician referral if signs of unintended weight loss or malnutrition

Answer 261

Primary care • Urgent referral to paediatric gastroenterologist for specialist Ix to confirm dx + initiate specialist treatment • Do not prescribe anti-diarrhoeal drugs if dx uncertain- may precipitate toxic megacolon • Referral to appropriate specialist team for extra-intestinal manifestations (rheumatology, dermatology, ophthalmology dietician) • Regular reviews • Assess impact of sx on daily functioning – home, work, school, leisure activities, anxiety, depression • Advice + sources of information and support o Lifelong condition, unpredictable relapses and remissions, specialist drug treatments to induce remissions, surgery may be needed o Encourage healthy lifestyle measures – healthy weight, regular exercise o Crohn’s and colitis UK • Ensure person has follow-up arranged with a gastroenterologist and/or specialist nurse if appropriate + encourage person to attend appointments regularly • Inform of the need of colorectal cancer surveillance – frequency is a specialist decision • Monitor growth of children (height and weight) + pubertal development regularly • Monitoring – serum ferritin, vitamin B12, folate, calcium, vitamin D • Assess for clinical features suggesting disease flare up o BMI, unintended weight loss, signs of malnutrition o CRP • If on immunosuppressive or biologic therapy o Live vaccines are CI o Vaccines can be given before the start of specialist treatment or else postponed for at least 6m after stopping therapy o Increased risk of influenza and pneumococcal infection so should receive those vaccines • Symptom mx o Do not prescribe anti-diarrhoeal o Only prescribe bulk forming laxatives (ispaghula husk, methylcellulose, sterculia) o Abdominal pain – paracetamol, No NSAIDs (may aggravate colitis sx),no opioids (may  risk of developing megacolon)

Answer 262

• Emergency hospital admission if severe sx or pt unstable • If hospital admission not indicated o Check adherence o Consider urgent specialist gastroenterology review appt or seek specialist advice • Consider dietician referral if signs of unintended weight loss or malnutrition ``` Step 1 therapy • IVF • IV CS to induce remission – 1st line • Stop oral 5-ASA • Parenteral nutrition needed until improvement or surgery • Surgical mx – colectomy with an ileostomy or ileojejunal pouch • IV ciclosporin – 2nd line • ``` Step 2 therapy • Add IV ciclosporin if o Little/no improvement within 72h of starting IV CS o Symptoms worsen at any time despite CS treatment • Infliximab for treatment of acute exacerbations of severely active UC only in pt in whom ciclosporin is CI or clinically inappropriate

Answer 263

* Topical aminosalicylate (mesalazine, olsalazine, sulfasalazine) – 1st line * Add PO aminosalicylate if remission not achieved within 4 weeks – 2nd line * Add time-limited topical/ PO CS – 3rd line * Ass calcineurin inhibitors (tacrolimus or ciclosporin) if inadequate response to PO CS after 2-4w

Answer 264

• Topical aminosalicylate (mesalazine, olsalazine, sulfasalazine) – 1st line • If remission not achieved within 4 weeks – 2nd line o Add high-dose PO aminosalicylate or o Switch to high dose PO aminosalicylate + time limited topical CS • Stop topical treatments, offer PO aminosalicylate + time-limited course of PO CS • Ass calcineurin inhibitors (tacrolimus or ciclosporin) if inadequate response to PO CS after 2-4w

Answer 265

• Topical aminosalicylate + high dose PO aminosalicylate – 1st line • If remission not achieved within 4 weeks – 2nd line o Stop topical aminosalicylate o Offer high dose PO aminosalicylate + time-limited course of PO CS • Add calcineurin inhibitors (tacrolimus or ciclosporin) if inadequate response to PO CS after 2-4w

Answer 266

• Increased likelihood of needing surgery if o Stool frequency >8/day o pyrexia o tachycardia o AXR showing colonic dilatation o Low albumin, low Hb, high plt count or  CRP

Answer 267

o Colectomy – Remove the portion of the affected bowel o Potentially curative – removal of affected bowel + elimination of risk of colonic adenocarcinoma o Proctocolectomy with ileostomy (past) o IPAA – ileal pouch-anal formation/anastomosis (now)  Pouch of ileum is used to fashion a rectum + connected to the anus to allow normal defecation  Post-IPAA complications: leakage and pelvic abscess, pouchitis  First line therapy for pouchitis: metronidazole or ciprofloxacin

Answer 268

Monitoring children and young adults • Monitor bone health o During chronic active disease o After treatment with systemic CS o After recurrent active disease • Monitor growth and pubertal development o Height + body weight monitoring  Every 3-6m if • They have an inflammatory exacerbation + are approaching or undergoing puberty • There is chronic active disease • They are being treated with systemic CS  Every 6m during pubertal growth if the disease is inactive  Every 12m if none of the criteria above are met o Pubertal development monitoring  Using principles of Tanner staging  Asking screening questions  Carrying out a formal examination • Consider referral to a secondary care paediatrician for pubertal assessment + ix of underlying cause if young person with UC o Has slow pubertal progress o Has not developed pubertal features appropriate for their age

Answer 269

Maintaining remission Proctitis + proctosigmoiditis • Topical aminosalicylate alone (daily or intermittent) or • PO aminosalicylate + topical (daily or intermittent) or • PO aminosalicylate alone L-sided + extensive UC • PO aminosalicylate alone All extents • PO azathioprine or PO mercaptopurine if o >2 inflammatory exacerbations in 12m that require treatment with systemic CS or o If remission is not maintained by aminosalicylates o To maintain remission after a single episode of acute severe UC

Answer 270

Moderate UC • Oral prednisolone for 2-4weeks + taper • If good response = treat with 5-ASA + continue for maintenance • If bad response = IV prednisolone Steroid dependent disease = pt with UC who are unable to stop CS within 3 months without recurrent active disease or who have a relapse requiring CS within 3 months of stopping them • Thiopurine or infliximab • If successful continue with medication as maintenance • If inadequate o Colectomy – final treatment option

Answer 271

• Strangulation or intestinal obstruction  emergency hospital admission • <18 years o Urgent referral to paediatric surgeon to be seen within 2w • Presenting in the first few months of life  urgent repair (highest risk of strangulation) • >1y o Reducible  manual reduction + elective surgery (herniotomy without implantation of mesh) o Incarcerated  emergency manual reduction + repair after 48h to allow oedema to settle • Neonates + premature infants kept in hospital overnight  recognised  risk of post operative apnoea

Answer 272

• Small and asymptomatic o Observation until 4 or 5 years of age (spontaneous closure in up to 80%) o If it incarcerates during this period  Manual reduction + surgical repair within 24h  If it can’t be reduced  emergency surgery o If it persists beyond 4-5 years  elective outpatient surgical repair (risk of incarceration) • Large (>1.5-2cm) or symptomatic (intermittent sx of incarceration or recurring pain) o Elective repair at 2-3 years • Incarcerated o Immediate attempt at reduction (in the absence of signs of peritonitis) o Reduced  admit + observe for peritonitis  surgical repair the following day o Cannot be reduced  emergency repair

Answer 273

Newly diagnosed ALL • Induction therapy o First phase of treatment o Prednisolone/dexamethasone + cyclophosphamide + vincristine + doxorubicin +/- L-asparaginase o Dexrazoxane – to prevent cardiotoxicity o Goal – achieve complete remission (CR – eradication of leukaemia determined by morphological criteria) o MDR (minimal residual disease) – should be tested after induction therapy  If positive  augment induction + referral for post-induction therapy allogenic SCT • CNS prophylaxis or treatment o Intrathecal methotrexate +/- intrathecal cytarabine + intrathecal hydrocortisone (triple intrathecal therapy) o If CNS disease is present, the recommended treatment regimen is the same as the prophylactic regimen except the frequency of intrathecal injections may be increased • Supportive care o Prevention of TLS – IVF + Hypouricaemic agents (e.g. allopurinol, rasburicase) o Platelet transfusions – if bleeding/low plt – to prevent or treat bleeding complications  Blood products should be irradiated, leukocyte-depleted, CMV -ve o Prophylactic use of Antibiotics/ antifungals/ antivirals – to prevent or treat infections + febrile neutropenia o Prophylactic use of GCSF (filgrastim) or GMCSF (sargramostim) in those at risk of febrile neutropenia o Norethisterone given to female patients to supress periods during therapy + periods of thrombocytopenia • If CD 20+ o Rituximab • If Ph + o TKI inhibitor – imatinib, dasatinib

Answer 274

Consolidation +/- Maintenance therapy • Continuing chemotherapy of moderate-high intensity continued for a relatively long time after remission (up to 3 years) • Consolidation therapy o Second phase of treatment o Cyclophosphamide + crisantaspase + cytrabine + idarubicin + etoposide + methotrexate or o Re-administration of the induction regimen o Goal – eliminate clinically undetectable residual leukaemia, preventing relapse + the development of drug-resistant cells • Maintenance therapy or allogenic SCT o Third phase of treatment o Mercaptopurine + methotrexate o TPMT + NUDT15 phenotype should be assessed at dx to allow individual dosing of mercaptopurine o Allogeneic SCT in high-risk disease  Suitable patients – suitable donor, absence of comorbidities, good performance status • CNS prophylaxis o Should be received at every stage of the treatment (induction, consolidation, maintenance) o Intrathecal methotrexate +/- intrathecal cytarabine + intrathecal hydrocortisone (triple intrathecal therapy) o During consolidation therapy – intensive systemic therapy with high-dose cytarabine or high-dose methotrexate to ensure good BBB penetration • Supportive care o See above

Answer 275

• Salvage chemotherapy or immunotherapy +/- SCT o Immunotherapy – Blinatumomab, inotuzumab ozogamicin, tisagenlecleucel • CNS prophylaxis or treatment o Intrathecal methotrexate +/- intrathecal cytarabine + intrathecal hydrocortisone (triple intrathecal therapy) • Supportive care

Answer 276

• Chemotherapy (ABVD (Adriamycin (Doxorubicin) + Bleomycin + Vinblastine + Decarbazine) +/- radiotherapy o Favourable disease = 2 cycles + 20 Gy radiation o Unfavourable disease  4 cycles + 30 Gy radiation or  BEACOPP regimen (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisolone) + 2 cycles ABVD + radiotherapy o Advanced disease = 6-8 cycles of ABVD or 6 cycles of BEACOPP • Low-dose radiotherapy to involved regions after favourable response to induction • Refractory and relapsed disease after 1st line therapy o High dose chemotherapy + autologous SCT o Brentuximab vedotin – anti CD30 antibody – if SCT fails/unsuitable o Nivolumab and Pembrolizumab (both block PD-1) – last line

Answer 277

General • Senior paediatric/ child protection review should be undertaken • Consider skeletal survey • Look for other features in the child’s history/ presentation/interactions with parents • Gather information from other agencies – explain to the parents that this information is needed to make an overall assessment of the child or young person • Document o In the child’s clinical record exactly what is observed and/or heard, from whim and when o All the concerns + any decisions made/ actions taken relating concerns o Information shared or reasons for not sharing information • !!!! Identify the safety of other children living with or in contact with the suspected perpetrator o Who else lives at home? o Does the child have any siblings? • Sources of information and support for the family if you are suspecting NAI, it is always safe to admit the child

Answer 278

Consider admission • Discuss concerns with o A more experienced colleague o A community paediatrician o CAMHS colleague o Named or designated professional for safeguarding children • Follow up o Ensure review of the child at a date appropriate to concern + look out for repeated presentation of this or any other alerting features o Check that support services are in place + that initial concerns have been addressed appropriately

Answer 279

Admit • Obtain consent from child/parents to share confidential information with the relevant agencies unless this will increase the risk of harm to the child/young person • Refer to children’s social services following local multi-agency safeguarding arrangements o May trigger a child protection investigation o Supportive services may be offered to the family following assessment o Alternative explanations may be identified • If child is thought to be in immediate danger o Refer to child’s social care +/or the police o The child ay be admitted to a paediatric ward as a place of safety whilst a social worker makes urgent enquiries and puts a safety plan in place • If child is not in immediate danger o Contact children’s social care to discuss the need for a referral to them, using multi-agency safeguarding procedures

Answer 280

o No intimate examination, general examination appropriate to assess person’s general health + look for other injuries o Refer urgently for the collection of forensic evidence were appropriate o DNA can be gathered for up to 7 days after vaginal penetration, up to 2 days in oral penetration and for up to 3 days in anal/penile penetration irrespective of washing or bathing o Asses the need for contraception + STI prophylaxis o STI prophylaxis  GUM clinic

Answer 281

o Admit if A+E o Assess Gillick competence o Will need to inform seniors/ safeguarding team o Will probably inform social services/ police depending on crime

Answer 282

* Senor colleagues * Named doctor for child protection * Contact social services + make a formal referral * Consider contacting the police (Child Abuse Investigation Team (CAIT)) * Consider contacting Multi-Agency Safeguarding Hub (MASH)

Answer 283

• Mild to moderate hypoglycaemia o Treat immediately o Oral fast acting glucose PO (10-20g, usually liquid carbohydrate e.g. Lucozade) o May need to be given in small amounts if vomiting o Blood glucose concentrations should rise within 5-15 mins. o Recheck blood glucose levels within 15 mins o Give more fast-acting glucose if they still have hypoglycaemia o As symptoms improve or blood glucose levels return to normal, give oral complex long-acting carbohydrate to maintain blood glucose levels

Answer 284

• Severe hypoglycaemia In hospital + IV access possible  10% IV glucose/dextrose infusion (max. 500mg/kg (5ml/kg))  A bolus of 10% can be given before glucose infusion • To rapidly increase the plasma-glucose concentration in order to allow glucose to cross the BBB and alleviate neuroglycopenia • SE – pain and phlebitis may occur during administration Not in hospital or IV access not possible, unresponsive or PO route cannot be used o IM glucagon or  >8y / >25kg – 1mg  <8y/ <25kg – 500mcg  Note – glucagon will be ineffective in patients whose liver glycogen is depleted, therefore should not be used in anyone who has fasted for a prolonged period, has adrenal insufficiency, chronic hypoglycaemia or alcohol-induced hypoglycaemia  Glucagon may be less effective in children taking sulfonylurea – use IV glucose instead o Concentrated oral glucose solution (e.g. Glucogel – glucose 40% gel)  Do not use oral glucose solution if they have reduced consciousness o As symptoms improve or blood glucose levels return to normal, give oral complex long-acting carbohydrate (e.g. 2 biscuits, 1 banana) to maintain blood glucose levels

Answer 285

* Should always have access to an immediate source of fast-acing glucose + blood glucose monitoring equipment (incl. parents + school nurses) * Parents should always be equipped with IM glucagon for emergency use in severe hypoglycaemic attacks – seek medical assistance if glucagon is not effective within 10 minutes as IV glucose is required

Answer 286

Asymptomatic neonatal hypoglycaemia • Feeding interventions o Increasing BF frequency o Supplement with a breast milk substitute (i.e. formula) o Buccal glucose gel may be used in conjunction with a feeding plan o Re-check blood-glucose concentrations in 1h to ensure there has been a response • IV glucose therapy 10% o 1st line or If feeding interventions are not effective o Re-check glucose in 15 mins

Answer 287

Symptomatic neonatal hypoglycaemia/ severe hypoglycaemia (sugar <1.5 mmol/l) • Admit to neonatal unit • Treat immediately with IV glucose – 1st line • Buccal glucose gel or IM glucagon – 2nd line o If glucose <1mmol/l buccal glucose should only be used as a interim measure while arranging treatment with IV glucose • Frequently recheck glucose until stable o Aim for 3-4 mmol/l

Answer 288

Prevention • Feed baby within 30 mins of birth • Subsequent frequent milk feeding (every 2-3h)

Answer 289

Under 16 • Carbimazole using a titration regimen – 1st line o Required for at least 2 years o Review need for treatment every 2 years o Warn parents about risk of neutropenia – seek urgent medical attention + blood count if a sore throat or fever occur whilst on treatment o If agranulocytosis develops, stop + do not restart treatment • Radioiodine treatment or surgery (total or partial thyroidectomy) – 2nd line 16 and over • Radioactive iodine > carbimazole – 1st line • Carbimazole o Can be used instead of radioiodine if remission is likely to be achieved with antityroid drugs or if radioactive iodine + surgery are unsuitable o 12-18m course o Block and replace (fixed high dose carbimazole + levothyroxine) or titration (dose based on TFTs) o If persistent/ relapsed hyperthyroidism on antithyroid drug treatment  radioactive iodine or surgery • Propylthiouracil o If SE to carbimazole or are pregnant/trying to conceive within the following 6m/ hx of pancreatitis • If agranulocytosis develops, stop + do not restart treatment • Symptomatic mx o Can use BB if >16

Answer 290

Under 16 • Carbimazole using a titration regimen 16 and over Multiple nodules • Radioactive iodine– 1st line • Total thyroidectomy or lifelong antithyroid drugs – 2nd line o Carbimazole > propylthiouracil o Propylthiouracil – If SE to carbimazole or are pregnant/trying to conceive within the following 6m/ hx of pancreatitis Single nodule • Radioactive iodine or hemithyroidectomy– 1st line • Lifelong antithyroid drugs – 2nd line

Answer 291

o TSH, FT4, FT3 every 3 months (<16) or every 6 months (>16) o Consider stopping TSH measurements if TSH level stabilises (2 similar measurements within the reference range 3-6m apart)

Answer 292

o TSH, FT4, FT3 within 8 weeks of stopping the drug o TSH, FT4, FT3 every 3 months for the first year o TSH every 6 months for the second year o TSH once a year

Answer 293

o Offer levothyroxine o Measure TSH + FT4 at 2m  6m • Monitoring of antithyroid drugs o TSH, FT4, FT3 every 6w until TSH is within reference range o TSH every 3m until antithyroid drugs are stopped

Answer 294

o TSH, FT4, FT3 every 6 weeks for the first 6 months until TSH is within reference range  then measure at 9 + 12 months o After 12 months, measure TSH every 6 months unless they develop hypothyroidism o If hyperthyroidism persists  antithyroid drugs until the 6-month appointment  if it still persists 6m after radioactive iodine treatment  consider further treatment o If hypothyroidism develops  levothyroxine replacement therapy

Answer 295

<2 • Consider levothyroxine if o TSH >20 mIU/l OR o TSH 10-20 mIU/l on 2 separate occasions 3 months apart or o TSH 5-10 mIU/l on 2 separate occasions 3 months apart and  Thyroid dysgenesis  Signs or symptoms of thyroid dysfunction >2 • Consider levothyroxine if >10mIU/l >16 • Consider levothyroxine sodium if >10mIU/l on 2 separate occasions 3 months apart • If TSH <10mIU/l on 2 separate occasions 3 months apart  consider a 6-month trial of levothyroxine sodium treatment o If symptoms do not improve  remeasure TSH + if level remains elevated, adjust the dose o If symptoms persist when the serum TSH is within the reference range  consider stopping levothyroxine + monitor (see below)

Answer 296

Can consider stopping TSH + FT4 measurement if TSH level has stabilised (2 similar measurement within the reference range 3-6m apart) • <2 – FT4 + TSH measurements o Every 1-2m • >2 y – if TSH <10 mIU/l, FT4 + TSH measurements o Every 3-6m if thyroid dysgenesis or  thyroid autoantibodies o Every 6-12m if no features of underlying thyroid disease

Answer 297

• Levothyroxine o Thyroxine treatment should be started within 2-3w of age to  risk of impaired neurodevelopment o Treatment is life-long with PO levothyroxine titrating dose to maintain normal growth, TSH + T4 levels o If TSH levels were very high before starting treatment or for children with a prolonged period of untreated disease, the TSH level can take up to 6m to return to the reference range

Answer 298

• <2y – FT4 + TSH measurements o Every 4-8w until the TSH level has stabilised (2 similar measurements within the reference range 2 months apart) o Every 2-3m during the first year of life then o Every 3-4 months during the second year of life • >2 y – FT4 + TSH measurements o Every 6-12w until the TSH level has stabilised (2 similar measurements within the reference range 3 months apart) o Every 4-6m until after puberty then o Once a year • >16 – TSH o Every 3m until stable o Then yearly

Answer 299

Mx • Observation • Elective closure at 4-5y if large, symptomatic, haemodynamically significant, RV dilation • Open heart surgery or transcatheter closure • Open heart surgery o Primum ASD, Secundum ASD (commonest), sinus venosus defect, coronary sinus defect o Performed through an incision at the front of the chest o Cardiopulmonary bypass o Open RA to gain access to the interatrial septum o Repair defect using pericardial or synthetic patch or direct suture o Patents usually stay in hospital for several days after the operation • Endovascular/Transcatheter closure of an ASD o Secundum ASD only o Relative contra-indications  Defects with a maximum diameter >40mm  Defects with inadequate margins to support the device  Interference of the device with AV valve function or pulmonary or systemic venous drainage o Small incision in the groin  introduction of guidewire + delivery sheath into the femoral vein o An occlude device is then introduced through the delivery sheath on a semi-rigid cable + expanded within the ASD to close it o People can usually go home the next day o Small residual shunts after the procedure  often resolve as endothelial tissue grows around the device

Answer 300

• Observe o Small shunts will close spontaneously – disappearance of murmur, normal echo • Medical management o Diuretics (furosemide) for HF o High-energy feeds to improve calorie intake (if VSD is affecting feeding + growth) o ACEi (captopril)-  afterload  promotes direct systemic flow from the LV  reduces the shunt o Digoxin – inotropic effect o Prevention of bacterial endocarditis while VSD is present  Prophylactic amoxicillin to patients at risk  Maintain good dental hygiene o Any pt needing significant medical management – should be offered surgical assessment • Surgical management o Indications  Large VSD Qp: Qs >1.5  Uncontrolled HF  Manage poor growth  Infundibular defects even if asymptomatic (bc of their location)  Development of aortic valve prolapse + aortic regurgitation in perimembranous VSDs  Prevent Eisenmenger syndrome (permanent lung damage from pulmonary hypertension and high blood flow) o Endovascular/Catheter closure  Muscular + perimembranous VSDs  Only if normal AV + ventriculoarterial connection + in the absence of any AV or arterial valve override  Insertion of an occluder into the heart from the RV usually through the tricuspid valve  Echocardiographic and fluoroscopic guidance are used to guide the occlude device as it is advanced through the delivery sheath and expanded to close the defect o Large muscular VSDs that are difficult to see or multiple holes (Swiss cheese septum) in infants  Palliation – pulmonary artery banding  Followed by corrective surgery many months later + removal of the pulmonary artery band o AVSD  Treat HF  Surgical repair at 3-6 months • Exercise is permitted if o Small VSD + normal heart o Or after closure if  Normal pulmonary arterial pressure  No significant disturbance of rhythm during stress testing + ambulatory 24h monitoring  Normal ECF  No evidence of aneurysm of the septal wall on echo

Answer 301

• Medical management o Ibuprofen, Indometacin o Diuretics if HF • PDA closure indications o Symptomatic (with exclusion of fixed high pulmonary vascular resistance) o Asymptomatic with left heart volume load o Closure recommended to abolish lifelong risk of bacterial endocarditis + of pulmonary vascular disease • Asymptomatic well infants o Wait until 1 year of age o Regular echo to check for spontaneous closure of the PDA o If PDA >1 year of age – endovascular occlusion • Infants with HF or pulmonary HTN o Urgent surgery o Percutaneous catheter device closure • Open surgery o Standard treatment (according to NICE) o Standard surgical procedure – ligation + division of the ductus through L posterolateral thoracotomy without cardiopulmonary bypass o Stich and/or clip is placed around both ends of the ductus arteriosus (ligation) o Ductus arteriosus cut in half if there is enough length (ligation + division) • Endovascular/transcatheter closure o Catheter passed through a vein/artery into the heart +coil or occlusion device is introduced into the ductus through the catheter under X-ray guidance o Pressure measurements + angiograms may be performed to assess the size + shape of the ductus o Small residual shunts after the procedure often resolve as endothelial tissue grows over and around the device

Answer 302

• If AN dx – delivery in a unit with ability to provide immediate + corrective mx • PG E1 infusion – to maintain PDA o Once cyanotic heart disease is suspected o Do not delay while waiting for echo • Maintain body temperature • Correct acidosis + hypoglycaemia if present • Prompt transfer to a cardiac centre  Urgent atrial septostomy o If not clinically stable – may need mechanical ventilation during transfer • Endovascular/ Balloon atrial septostomy (BAS) o Done in neonates o Prolongs survival until definitive surgery can be performed o Procedure used to enlarge the foramen ovale – breaks the flap valve of foramen ovale o Makes a connection between the R and the L atria o Bolus of heparin given before BAS o Simple balloon septostomy o Static balloon atrial septostomy  When simple balloon septostomy is unsuccessful or contraindicated  In older children or adults with a thick septum in whom there is no atrial communication • Arterial switch operation (ASO) o Definitive corrective procedure o Done in the first 2 weeks of life o Focuses on achieving a physiological rather than an anatomical correction of circulation in TGA o Note – coronary arteries also need to be transferred to the new aorta

Answer 303

• PG E1 infusion – in neonates to open the ductus + achieve improved haemodynamic stability when pt presents acutely • Symptom management o Diuretics + inotropes – CCF o IE prophylaxis • Open chest surgery o Standard treatment for  Native coarctation – carried out in the first treatment  Recoarctation – if previous surgical or angioplastic treatment fails and coarctation recurs o May include  Resection of the coarctation site and end-to-end anastomosis repair  Arch reconstruction with patch aortoplasty  L SC flap angioplasty  Bypass graft repair  Older patients may require stent insertion or surgical resection • Balloon angioplasty of aortic coarctation +/- stent insertion o For native coarctation + recoarctation o Neonates  Symptomatic – usually used as a preliminary procedure to buy time for surgery  Preferred to treat recoarctation following surgery • Long-term follow up – careful monitoring for development of complications + possible ongoing pharmacological therapy • Advice o Avoid excessively vigorous physical activity, contact sports + exercise that involves straining e.g. weightlifting o Regular gentle anaerobic exercise

Answer 304

``` • O2 • PG E1 • Mild – F/U • Moderate to severe o PBPV (percutaneous balloon dilation pulmonary valvulopasty) – 1 o Surgical valvuloplasty – 2 o Endocarditis prophylaxis – 6m postop ```

Answer 305

• If fetal critical aortic stenosis - AN percutaneous balloon valvuloplasty at 21-32w • PN balloon valvuloplasty o Palliative until SAVR/TAVR • SAVR/TAVR (Surgical/ transcatheter aortic valve replacement) • HLHS – staged reconstruction • Long-term anitcoag. if mechanical valves – Warfarin (not DOACs)

Answer 306

Ix • Echo – absence of tricuspid valve • CXR – massive RA enlargement (wall-to-wall heart) Mx • PGE infusion • Surgery o Neonatal period – Blalock-Taussig shunt (shunt bn subclavian artery + pulmonary artery), pulmonary artery banding o 3-6m – Glenn shunt (shunt bn SVC + Pulmonary artery) o Preschool (2-5y) – Fontan procedure (permanent corrective surgery) (shunt bn IVC + Pulmonary artery)

Answer 307

• The condition will resolve over a few days/week • Paracetamol (10-15mg/kg every 4-6hrs) or ibuprofen (5-10mg/kg every 4-6hrs) for symptomatic relief • Advise to maintain adequate hydration • Explain the risk of febrile seizures • School exclusion is NOT needed

Answer 308

• Infection should not alter the MOD • BF ok • First dose of vaccine within 24h after birth • Hep B monovalent vaccine at birth + 4w + 1 year of age • Heb B hexavalent vaccine at 8w, 12w, 16w • HBIG given simultaneously with vaccine but at a different site if o Mother is HBsAg +ve (even if she is HBeAg negative) o Mother had acute hepatitis B during pregnancy o Mother had an HBV DNA level > 1x10^6IUs/ml in any antenatal sample during the current pregnancy • Acute infection  supportive care

Answer 309

``` • Conservative mx if o CS o Recurrent HSV • 1st/2nd trimester acquisition o PO/IV acyclovir if disseminated o Daily suppressive acyclovir from 36w to delivery o SVD • 3rd trimester acquisition (>28w) o PO/IV acyclovir if disseminated o Daily suppressive acyclovir until delivery o CS • Recurrent o Daily suppressive acyclovir from 36w to delivery o SVD ``` Mx of neonate CS/ recurrent HSV infection  conservative SVD w primary HSV within previous 6w • See ix • IV acyclovir until evidence of infection is ruled out • BF ok

Answer 310

Mother – spiramycin 3/52 Fetus – sulfadiazine + pyrimethamine up to 1y after delivery Newborn • Sulfadiazine + • Pyrimethamine + • Folinic acid For 1 year Monitor FBC + LFTs every 4-6w • Ophthalmology + audiology assessment

Answer 311

• Supportive treatment Pregnancy >20w – congenital rubella syndrome does not develop No effective treatment to prevent development of congenital rubella syndrome

Answer 312

* AN – amniocentesis  US/MRI of feal brain  serial US every 2-3w until delivery + cerebral MRI at 28-32w * Hyperechogenic bowel on US * CMV must be confirmed at birth in the first 21d of life * Urine/oral swab/ Guthrie card PCR for CMV

Answer 313

* Supportive care * Notify the local Health Protection Unit (HPU) * Rest and take in adequate fluids * Consider admitting if there is a serious complication such as haemorrhagic complications (caused by thrombocytopaenia) or encephalitis

Answer 314

• Prednisolone PO o 60mg/m2 OD 4-6w until proteinuria ceases o 40mg/m2 alternate days for 4-6w o Then withdraw by reducing gradually • Fluid restriction + low-salt diet • Albumin + Furosemide o For massive oedema o Furosemide given IV with each albumin infusion to promote diuresis • Cyclophosphamide or ciclopsorin or tacrolimus or mycophenolate mofetil o Used as corticosteroid – sparing agents or as immunosuppressants o Used in conjunction with CS therapy to minimise CS dose o In patients with intermittent relapses (<3 episodes a year) o In patients with frequent relapses (>2 episodes of nephrotic syndrome in 6 months or >3 episodes in 1 year) o In patients with CS dependence (relapses occur during CS tapering) • If frequent relapses continue despite adding CS-sparing therapy  renal biopsy to confirm histological dx • Prophylactic trimethoprim/ sulfamethoxazole o Given in the early phase of induction therapy (i.e. when the intensity of immunosuppression is deemed high) to prevent PCP • Transition to an adult nephrologist recommended for all children approaching adulthood

Answer 315

Mild Hydrocortisone Hydrocortisone acetate Moderate Clobetasone butyrate Triamcinolone acetonide ``` Potent Betamethasone valerate Betamethasone dipropionate (cream, ointment, gel) Mometasone furoate Diflucortolone valerate Hydrocortisone 17-butyrate Methylprednisolone aceponate ``` Very potent Clobetasol propionate Betamethasone dipropionate (in an optimised vehicle)

Answer 316

Mild disease (= isolated haematuria, minimal/no proteinuria, normal GFR) • Treatment of underlying cause (e.g. antibiotics, antivirals, withdrawal of the causative drug • Supportive measures • If poststreptococcal GN  Phenoxymethylpenicillin or azithromycin/clarithromycin if penicillin allergic Moderate – severe disease (= haematuria, proteinuria, reduced GFR) • ACEi or ATII receptor antagonists – ramipril/enalapril, losartan • Furosemide o For patients who remain hypertensive despite ACEi therapy or ATII receptor antagonists • If poststreptococcal GN  Phenoxymethylpenicillin or azithromycin/clarithromycin if penicillin allergic

Answer 317

Anti-GBM • Plasmapheresis + prednisolone + cyclophosphamide • Prophylactic trimethoprim/ sulfamethoxazole o Given in the early phase of induction therapy (i.e. when the intensity of immunosuppression is deemed high) to prevent PCP Immune complex (no SLE) • Corticosteroids o Prednisolone/ methylprednisolone Immune complex (with SLE) • Immunosuppressants o Cyclophosphamide or mycophenolate mofetil or rituximab • Prophylactic trimethoprim/ sulfamethoxazole o Given in the early phase of induction therapy (i.e. when the intensity of immunosuppression is deemed high) to prevent PCP

Answer 318

Anti-GBM • Plasmapheresis + prednisolone + cyclophosphamide • Prophylactic trimethoprim/ sulfamethoxazole o Given in the early phase of induction therapy (i.e. when the intensity of immunosuppression is deemed high) to prevent PCP Immune complex (no SLE) • Corticosteroids o Prednisolone/ methylprednisolone Immune complex (with SLE) • Immunosuppressants o Cyclophosphamide or mycophenolate mofetil or rituximab • Prophylactic trimethoprim/ sulfamethoxazole o Given in the early phase of induction therapy (i.e. when the intensity of immunosuppression is deemed high) to prevent PCP

Answer 319

``` • Supportive treatment o Paracetamol +/- ibuprofen  For fever + arthralgia  Paracetamol – 10-15 mg/kg every 4-6h  Ibuprofen – 5-10mg/kg every 4-6h o Hydration + rest o Ibuprofen for arthritis • Persistent infection (>3 weeks) o IVIG for 5/7 o RBC transfusion ```