Bedside Clinical Guidelines Partnership In association with

Bedside Clinical Guidelines Partnership Paediatric Guidelines In association with 2006

Paediatric Guidelines 2006 Paediatric Guidelines 2013–14 Paediatric These guidelinesISBN: ar 978-0-9567736-1-6e advisory, not mandatory. Every effort has been made to ensure accuracy. The authors cannot accept any responsibility for These guidelinesadverse outcomes.are advisory, not mandatory. Guidelines Every effort has been made to ensure accuracy. SuggestionsThe authors for cannotimprovement accept andany responsibilityadditional for Theseguidelines guidelines would ar adverseebe advisory most outcomes. welcome, not mandatory by the . Every efPartnersfort has in been Paediatrics made toCoor ensurdinatore accuracy, . Suggestions for improvement and additional guidelines The authors cannot accept any responsibility for Tel. 01782would 552002 be most or welcomeEmail nicky by.smith [email protected] in Paediatrics, please contact viaadverse http://www.networks.nhs.uk/nhs-networks/ outcomes. 2013-14 partners-in-paediatrics/guidelines Suggestions for improvement and additional Paediatric Guidelines 2013–14 guidelines would be most welcome by the Partners in Paediatrics Coordinator, Tel. 01782 552002 or Email [email protected] ISSUE 5

Printed by: Sherwin Rivers Ltd, Waterloo Road, Stoke on Trent ST6 3HR Tel: 01782 212024 Fax: 01782 214661 Email: [email protected] This copy belongs to: Name: Further copies can be obtained from Partners in Paediatrics via http://www.networks.nhs.uk/nhs-networks/partners-in-paediatrics/guidelines

Published by the Bedside Clinical Guidelines Partnership and Partners in Paediatrics NOT TO BE REPRODUCED WITHOUT PERMISSION

Partners in Paediatrics comprises: Birmingham Children’s Hospital NHS Foundation Trust Burton Hospitals NHS Foundation Trust Dudley Clinical Commissioning Group East Cheshire NHS Trust George Eliot Hospital NHS Trust Heart of England NHS Foundation Trust Mid Staffordshire NHS Foundation Trust Robert Jones & Agnes Hunt Orthopaedic Hospital NHS Foundation Trust Shropshire Community NHS Trust South Staffordshire & Shropshire Healthcare NHS Foundation Trust The Royal Wolverhampton Hospitals NHS Trust The Shrewsbury & Telford Hospital NHS Trust University Hospital of North Staffordshire NHS Trust Walsall Healthcare NHS Trust

The Bedside Clinical Guidelines Partnership comprises:

Ashford & St Peter’s Hospitals NHS Trust (Surrey) Barnet and Chase Farm Hospitals NHS Trust (Middlesex) Burton Hospitals NHS Foundation Trust The Dudley Group NHS Foundation Trust East Cheshire NHS Trust (Macclesfield) The Hillingdon Hospital NHS Foundation Trust (Hillingdon) Ipswich Hospitals NHS Trust Mid Cheshire Hospitals NHS Trust (Leighton, Crewe) Mid Staffordshire NHS Foundation Trust North Cumbria University Hospitals NHS Trust The Pennine Acute Hospitals NHS Trust (Greater Manchester) The Princess Alexandra Hospital NHS Trust (Harlow, Essex) The Royal Wolverhampton Hospitals NHS Trust Salford Royal NHS Foundation Trust Sandwell and West Birmingham Hospitals NHS Trust The Shrewsbury and Telford Hospital NHS Trust University Hospitals Birmingham NHS Foundation Trust University Hospital of North Staffordshire NHS Trust Walsall Healthcare NHS Trust Wye Valley NHS Trust (Hereford) CONTENTS • 1/3

Preface...... 6 Acknowledgements...... 8 A: ANAESTHETICS AND CRITICAL CARE

APLS – Cardiorespiratory arrest...... 9 APLS – Recognition and assessment of the sick child ...... 12 Intraosseous infusion...... 16 Apparent life threatening event (ALTE)...... 18 Anaphylaxis...... 20 Pain assessment...... 23 Analgesia...... 24 Sedation...... 28 IV Fluid therapy ...... 31 Long line insertion...... 32 Pre-op fasting...... 35 Post GA monitoring ex-premature infants...... 36 B: BREATHING (RESPIRATORY DISEASE)

Asthma – acute management...... 37 Bronchiolitis...... 41 Croup...... 44 Cystic fibrosis – Admission...... 46 Cystic fibrosis – Exacerbation...... 48 Cystic fibrosis – Microbiology...... 50 Cystic fibrosis – Distal intestinal obstruction syndrome (DIOS)...... 52 Pneumonia ...... 53 Pleural effusion...... 56 Pneumothorax ...... 59 C: CARDIOVASCULAR DISEASE

Cyanotic congenital heart disease...... 61 Heart failure and weak pulses...... 63 ECG interpretation...... 65 Tachycardia and bradycardia...... 69 Endocarditis prophylaxis...... 74 D: DRUGS AND POISONING

Poisoning and drug overdose...... 75 Alcohol poisoning...... 78

Issue 5 Issued: May 2013 Expires: May 2014 3 CONTENTS • 2/3

Iron poisoning...... 80 Paracetamol poisoning...... 82 Phenothiazine poisoning/side effects...... 87 Salicylate poisoning...... 88 Tricyclic poisoning...... 90 E: ENDOCRINE/METABOLISM

Diabetes and fasting...... 92 Diabetic ketoacidosis...... 96 Diabetes new (non-ketotic)...... 103 Hypoglycaemia...... 105 Ketone monitoring...... 111 Steroid dependence...... 112 G: GASTROENTEROLOGY

Abdominal pain...... 114 Constipation...... 117 Diarrhoea and vomiting...... 122 Nutritional first line advice ...... 128 Failure to thrive...... 131 Jaundice...... 134 Vitamin D deficiency NEW ...... 137 H: HAEMATOLOGY

Blood and platelet transfusions...... 138 Febrile neutropenia...... 140 Henoch-Schönlein purpura...... 143 Immune thrombocytopenic purpura (ITP)...... 145 Haemophilia...... 147 I: INFECTION

Antibiotics...... 150 Bites...... 152 Cervical lymphadenopathy...... 153 Fever ...... 157 Fever of unknown origin NEW ...... 161 Hepatitis...... 163 HIV and hepatitis B post-exposure prophylaxis (PEP)...... 164 HIV testing...... 166

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Immunodeficiency...... 168 Kawasaki disease...... 170 Malaria ...... 173 Meningitis...... 176 Notifiable infectious diseases and food poisoning...... 180 Orbital cellulitis...... 182 Osteomyelitis and septic arthritis...... 183 Petechial/purpuric rashes...... 186 Septicaemia (including meningococcal)...... 187 Tuberculosis...... 191 N: NEUROLOGY

Facial palsy ...... 195 Epilepsy ...... 196 Status epilepticus ...... 201 Neuromuscular disorders...... 202 Glasgow coma score ...... 204 R: RENAL

Glomerulonephritis...... 205 Haemolytic uraemic syndrome...... 207 Hypertension...... 209 Nephrotic syndrome...... 215 Renal calculi...... 219 Renal failure...... 223 Renal investigations...... 226 Urinary tract infection ...... 230 R: RHEUMATOLOGY

Arthritis...... 235 Limping child...... 237 S: SAFEGUARDING

Child protection...... 240 Self harm...... 246

Index...... 248

Issue 5 Issued: May 2013 Expires: May 2014 5 PREFACE • 1/2

This book has been compiled as an aide-memoire for all staff concerned with the management of general medical paediatric patients, especially those who present as emergencies. Guidelines on the management of common medical conditions No guideline will apply to every patient, even where the diagnosis is clear-cut; there will always be exceptions. These guidelines are not intended as a substitute for logical thought and must be tempered by clinical judgement in the individual patient.

The guidelines are advisory, NOT mandatory

Prescribing regimens and nomograms

The administration of certain drugs, especially those given intravenously, requires great care if hazardous errors are to be avoided. These guidelines do not include all guidance on the indications, contraindications, dosage and administration for all drugs. Please refer to the British National Formulary for Children (BNFc).

Practical procedures

DO NOT attempt to carry out any of these Practical procedures unless you have been trained to do so and have demonstrated your competence.

National guidelines

Where there are different recommendations the following order of prioritisation is followed: NICE > NPSA > SIGN > RCPCH > National specialist society > BNFC > Cochrane > Meta-analysis > systematic review > RCT > other peer review research > review > local practice.

Evidence base

These have been written with reference to published medical literature and amended after extensive consultation. Wherever possible, the recommendations made are evidence based. Where no clear evidence has been identified from published literature the advice given represents a consensus of the expert authors and their peers and is based on their practical experience.

Supporting information

Where possible the guidelines are based on evidence from published literature. It is intended that the evidence relating to statements made in the guidelines and its quality will be made explicit. Where supporting evidence has been identified it is graded I to V according to standard criteria of validity and methodological quality as detailed in the table below. A summary of the evidence supporting each statement is available, with the original sources referenced (ward-based copies only). The evidence summaries are being developed on a rolling programme which will be updated as each guideline is reviewed.

Issue 5 Issued: May 2013 6 Expires: May 2014 PREFACE • 2/2 Level of evidence Strength of evidence I Strong evidence from at least one systematic review of multiple well-designed randomized controlled trials II Strong evidence from at least one properly designed randomized controlled trial of appropriate size III Evidence from well-designed trials without randomization, single group pre-post, cohort, time series or matched case-control studies IV Evidence from well-designed non-experimental studies from more than one centre or research group V Opinions of respected authorities, based on clinical evidence, descriptive studies or reports of expert committees

JA Muir-Gray from Evidence Based Healthcare, Churchill Livingstone London 1997

Feedback

Evaluating the evidence-base of these guidelines involves continuous review of both new and existing literature. The editors encourage you to challenge the evidence provided in this document. If you know of evidence that contradicts, or additional evidence in support of the advice given in these guidelines please contact us.

The accuracy of the detailed advice given has been subject to exhaustive checks. However, if any errors or omissions become apparent contact us so these can be amended in the next review, or, if necessary, be brought to the urgent attention of users. Constructive comments or suggestions would also be welcome.

Contact

Partners in Paediatrics, via www.networks.nhs.uk/nhs-networks/partners-in-paediatrics or Bedside clinical guidelines partnership via e-mail: [email protected]

Issue 5 Issued: May 2013 Expires: May 2014 7 ACKNOWLEDGEMENTS • 1/1

We would like to thank the following for their assistance in producing this edition of the Paediatric guidelines on behalf of the Bedside Clinical Guidelines Partnership and Partners in Paediatrics Contributors Paediatric Editors

Mona Abdel-Hady Andrew Cowley John Alexander Loveday Jago Maggie Babb Paddy McMaster Kathryn Bailey Sue Bell Bedside Clinical Guidelines Karen Davies Partnership Shireen Edmends Paddy McMaster Sarah Goddard Naveed Mustfa Helen Haley Melissa Hubbard Prakash Kamath Clinical Evidence Librarian Deirdre Kelly David Rogers Jackie Kilding Stephen Parton Aswath Kumar Uma Kumbattae Warren Lenney Partners in Paediatrics Paddy McMaster Andrew Cowley Andy Magnay Loveday Jago David Milford Julia Greensall Tess Mobberley Lesley Hines Angela Moore Ros Negrycz Tina Newton Partners in Paediatrics Anna Pigott Networks Parakkal Raffeeq Members of the West Midlands Child George Raptis Sexual Abuse Network Martin Samuels (Led by Ros Negrycz & Jenny Hawkes) Shiva Shankar Members of the West Midlands Children Ravi Singh & Adolescent Rheumatology Network Sarah Thompson (Chaired by Kathryn Bailey)

Pharmacist Members of the West Midlands Paediatric Anaesthesia Network Helen Haley (Co-chaired by Richard Crombie & Rob Alcock)

Microbiology reviewer Members of the West Midlands Gastroenterology Network Krishna Banavathi (Chaired by Anna Pigott)

Issue 5 Issued: May 2013 8 Expires: May 2014 APLS - CARDIORESPIRATORY ARREST • 1/3 MANAGEMENT Breathing (B)

G Stimulate patient to assess for signs G Self-inflating bag and mask with of life and shout for help 100% oxygen G Establish basic life support: Airway – G Ventilation rate Breathing – Circulation G unintubated: 2 inflations for every 15 G Connect ECG monitor: identify rhythm compressions and follow Algorithm G intubated:10–12/min, with continuous G Control airway and ventilation: compressions preferably intubate G Consider foreign body or G Obtain vascular access, peripheral or pneumothorax intraosseous (IO) Circulation (C) G Change person performing chest compressions every few minutes G Cardiac compression rate: Airway (A) 100–120/min depressing lower half of sternum by at least one third: push G Inspect mouth: apply suction if hard, push fast necessary G Peripheral venous access: 1 –2 G Use either head tilt and chin lift or jaw attempts (<30 sec) thrust G Intraosseous access: 2–3 cm below G Oro- or nasopharyngeal airway tibial tuberosity (see Intraosseous G Intubation: infusion guideline) G endotracheal tube sizes G Use ECG monitor to decide between: G term newborns 3–3.5 mm G a non-shockable rhythm: asystole or G aged 1 yr 4.5 mm pulseless electrical activity (PEA) i.e. G aged >1 yr: use formula [(age/4) electromechanical dissociation + 4] mm for uncuffed tubes; 0.5 OR smaller for cuffed G a shockable rhythm: ventricular G If airway cannot be achieved, fibrillation or pulseless ventricular consider laryngeal mask or, failing tachycardia that, cricothyrotomy Algorithm for managing these rhythms follows: G If arrest rhythm changes, restart Algorithm G If organised electrical activity seen, Adrenaline doses for asystole check pulse and for signs of circulation Route Aged <12 yr Aged 12 yr – adult Notes 10 microgram/kg 1 mg Initial and If given by (0.1 mL/kg (10 mL of usual intraosseous route of 1:10,000) 1:10,000) subsequent flush with sodium dose chloride 0.9% IV rapid bolus/ intraosseous 100 microgram/kg 5 mg Exceptional Maximum dose (0.1 mL/kg of 1:1000 (5 mL of circumstances 5 mL of 1:1000 or 1:10,000) (e.g. beta- 1 mL/kg of 1:10,000) blocker overdose) 100 microgram/kg 5 mg - Endotracheal (0.1 mL/kg of 1:1000 (5 mL of 1:1000) tube (ETT) or 1 mL/kg of 1:10,000)

Issue 5 Issued: May 2013 Expires: May 2014 9 APLS - CARDIORESPIRATORY ARREST • 2/3 SAFETY Approach with care Free from danger?

STIMULATE Are you alright?

SHOUT for help

Airway opening manoeuvres

Look, listen, feel

5 rescue breaths

Check for signs of life Brachial pulse aged <1 yr Check pulse Carotid pulse aged >1 yr Take no more than 10 sec

CPR 15 chest compressions: 2 ventilations

VF/ Asystole/ pulseless VT Assess PEA DC Shock 4 J/kg Continue CPR 2 min CPR rhythm 2 min CPR

High flow oxygen High flow oxygen IV/IO access IV/IO access If able – intubate Return of If able – intubate spontaneous circulation rd Adrenaline after 3 DC (ROSC) Adrenaline immediately shock and then every – see Post- and then every 4 min alternate DC shock resuscitation 10 microgram/kg 10 microgram/kg management IV or IO IV or IO

Amiodarone after 3 rd and 5 th DC shock only 5 mg/kg IV or IO Consider 4 Hs and 4 Ts Hypoxia Tension pneumothorax Hypovolaemia Tamponade If signs of life, check rhythm Hyperkalaemia Toxins If perfusable rhythm, check pulse Hypothermia Thromboembolism

Modified from ALSG 2010, reproduced with permission

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Defibrillation G Exceptions include: G hypothermia (<32ºC) G Use hands-free paediatric pads in G overdoses of cerebral depressant children, may be used anteriorly and drugs (successful resuscitation has posteriorly occurred with 24 hr CPR) G Resume 2 min of cardiac G Discuss difficult cases with consultant compressions immediately after giving before abandoning resuscitation DC shock, without checking monitor or feeling for pulse POST-RESUSCITATION G Briefly check monitor for rhythm MANAGEMENT before next shock: if rhythm changed, check pulse Identify and treat underlying cause G Adrenaline and amiodarone are given rd th after the 3 and 5 DC shock, and Monitor then adrenaline only every other DC shock G Heart rate and rhythm G Automatic external defibrillators (AEDs) G Oxygen saturation do not easily detect tachyarrythmias in G infants but may be used at all ages, CO 2 monitoring ideally with paediatric pads, which G Core and skin temperatures attenuate the dose to 50–80 J G BP PARENTAL PRESENCE G Urine output G Arterial blood gases G Evidence suggests that presence at G their child’s side during resuscitation Central venous pressure enables parents to gain a realistic Request understanding of efforts made to save their child. They may subsequently G Chest X-ray show less anxiety and depression G Arterial and central venous gases G Designate one staff member to G Haemoglobin and platelets support parents and explain all actions G Group and save serum for G Team leader, not parents, must crossmatch decide when it is appropriate to stop resuscitation G Sodium, potassium, urea and creatinine WHEN TO STOP G Clotting screen RESUSCITATION G Blood glucose G Unless exceptions exist, it is G LFTs reasonable to stop after 30 min of G 12-lead ECG CPR if you find: G no detectable signs of cardiac output and G Transfer to PICU G G no evidence of signs of life (even if Hold a team debriefing session to ECG complexes) reflect on practice

Issue 5 Issued: May 2013 Expires: May 2014 11 APLS - RECOGNITION AND ASSESSMENT OF THE SICK CHILD • 1/4 SUMMARY OF RAPID CLINICAL G Once airway (A), breathing (B) and ASSESSMENT circulation (C) are clearly recognised as being stable or have been Assessment stabilised, definitive management of underlying condition can proceed Airway (A) and Breathing (B) G Reassessment of ABCDE at frequent intervals necessary to assess progress G Effort of breathing and detect deterioration G respiratory rate G Hypoglycaemia: glucose 10% 2 mL/kg followed by IV glucose infusion G recession G use of accessory muscles CHILD AND PARENTS G additional sounds: stridor, wheeze, G grunting Give clear explanations to parents and child G flaring of nostrils G Allow and encourage parents to G Efficacy of breathing remain with child at all times G chest movement and symmetry RECOGNITION AND G breath sounds ASSESSMENT OF THE SICK G SpO in air 2 CHILD Circulation (C) Weight G Heart rate G Pulse volume Anticipated weight in relation to age G peripheral Age Weight (kg) G central (carotid/femoral) Birth 3.5 G Blood pressure 5 months 7 G Capillary refill time 1 yr 10 G Skin colour and temperature Weight can be estimated using following Disability (D) formulae: G 0–12 months: wt (kg) = [age (m) / 2] + 4 G Conscious level G 1–6 years: wt (kg) = [age (y) + 4] x 2 G Posture G 7–14 years: wt (kg) = [age (y) x 3] + 7 G Pupils Airway Exposure (E)

G Fever Primary assessment of airway G Skin rashes, bruising G Vocalisations (e.g. crying or talking) Don’t Ever Forget Glucose indicate ventilation and some degree (DEFG) of airway patency G Assess patency by: G Glucose stix G looking for chest and/or abdominal Actions movement G listening for breath sounds G Complete assessment should take G feeling <1 min for expired air G Treat as problems are found

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Re-assess after any airway G wheeze, predominantly expiratory, opening manoeuvres indicates lower airway obstruction G volume of noise is not an indicator of G Infants: a neutral head position; other severity children: ‘sniffing the morning air’ G Grunting: G Other signs that may suggest upper G a sign of severe respiratory distress airway obstruction: G can also occur in intracranial and G stridor intra-abdominal emergencies G intercostal/subcostal/sternal recession G Accessory muscle use Breathing G Gasping (a sign of severe hypoxaemia and can be pre-terminal) Primary assessment of G Flaring of nostrils breathing Exceptions G Assess G Increased effort of breathing DOES NOT occur in three G effort of breathing circumstances: G efficacy of breathing G exhaustion G effects of respiratory failure G central respiratory depression Effort of breathing (e.g. from raised intracranial depression, poisoning or G Respiratory rates ‘at rest’ at different encephalopathy) ages G neuromuscular disease (e.g. Age (yr) Resp rate (breaths/min) spinal muscular atrophy, muscular dystrophy or poliomyelitis) <1 30–40 1–2 25–35 Efficacy of breathing 3–5 25–30 6–12 20–25 G Breath sounds on auscultation: >12 15–20 G reduced or absent G bronchial G Respiratory rate: G symmetrical or asymmetric G tachypnoea: from either lung or G airway disease or metabolic acidosis Chest expansion and, more importantly in infants, abdominal ‘see- G bradypnoea: due to fatigue, raised sawing’ intracranial pressure, or pre-terminal G Pulse oximetry G Recession: G intercostal, subcostal or sternal Effects of respiratory failure on recession shows increased effort of other physiology breathing G Heart rate: G degree of recession indicates severity G of respiratory difficulty increased by hypoxia, fever or stress G G in child with exhaustion, chest bradycardia a pre-terminal sign movement and recession will decrease G Inspiratory or expiratory noises: G stridor, usually inspiratory, indicates laryngeal or tracheal obstruction

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G Skin colour: Effects of circulatory G hypoxia first causes vasoconstriction inadequacy on other and pallor (via catecholamine release) organs/physiology G cyanosis is a late and pre-terminal sign G some children with congenital heart G Respiratory system: disease may be permanently cyanosed G tachypnoea and hyperventilation and oxygen may have little effect occurs with acidosis G Mental status: G Skin: G hypoxic child will be agitated first, G pale or mottled skin colour indicates then drowsy and unconscious poor perfusion G pulse oximetry can be difficult to G Mental status: achieve in agitated child owing to G agitation, then drowsiness leading to movement artefact unconsciousness Circulation G Urinary output: G <1 mL/kg/hr (<2 mL/kg/hr in infants) G Heart rates ‘at rest’ at different ages indicates inadequate renal perfusion Age (yr) Heart rate (beats/min) Features suggesting cardiac <1 110–160 cause of respiratory inadequacy 1–2 100–150 3–5 95–140 G Cyanosis, not relieved by oxygen therapy 6–12 80–120 G Tachycardia out of proportion to >12 60–100 respiratory difficulty Pulse volume G Raised JVP G Gallop rhythm/murmur G Absent peripheral pulses or reduced central pulses indicate shock G Enlarged liver G Absent femoral pulses Capillary refill Disability G Pressure on centre of sternum or a digit for 5 sec should be followed by Primary assessment of return of circulation in skin within disability 2 sec G can be prolonged by shock or cold G Always assess and treat airway, environmental temperatures breathing and circulatory problems G not a specific or sensitive sign of before undertaking neurological shock assessment: G should not be used alone as a guide G respiratory and circulatory failure will to response to treatment have central neurological effects BP G central neurological conditions (e.g. meningitis, raised intracranial G Cuff should cover >80% of length of pressure, status epilepticus) will have upper arm both respiratory and circulatory G expected systolic BP = 85 + (age in consequences yrs x 2) G Hypotension is a late and pre-terminal sign of circulatory failure

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Neurological function Circulatory effects

G Conscious level: AVPU (Figure 1 ); a G Raised intracranial pressure may painful central stimulus may be induce: applied by sternal pressure or by G systemic hypertension pulling frontal hair G sinus bradycardia G Posture: G hypotonia G decorticate or decerebrate postures may only be elicited by a painful stimulus G Pupils – look for: G pupil size, reactivity and symmetry G dilated, unreactive or unequal pupils indicate serious brain disorders Respiratory effects

G Raised intracranial pressure may induce: G hyperventilation G Cheyne-Stokes breathing G slow, sighing respiration G apnoea

Figure 1: Rapid assessment of level of consciousness

A Alert

- responds to Voice V this level is equivalent to 8 or less on GCS P - responds to Pain

U Unresponsive

Issue 5 Issued: May 2013 Expires: May 2014 15 INTRAOSSEOUS INFUSION • 1/2

INDICATIONS PROCEDURE

G Profound shock or cardiac arrest, Preferred sites when immediate vascular access needed and peripheral access not possible (maximum 2 attempts) Avoid fractured bones and G allows rapid expansion of circulating limbs with fractures proximal to volume possible sites G gives time to obtain IV access and facilitates procedure by increasing Proximal tibia venous filling G Identify anteromedial surface of tibia EQUIPMENT 1–3 cm below tibial tuberosity G Direct needle away from knee at G Intraosseous infusion needles for approx 90º to long axis of tibia manual insertion or EZ-IO drill and G needles (<40 kg: 15 mm pink; >40 kg: Needle entry into marrow cavity 25 mm blue) on resuscitation trolley accompanied by loss of resistance, sustained erect posture of needle G Alcohol swabs to clean skin without support and free fluid infusion G 5 mL syringe to aspirate and confirm G Connect 5 mL syringe and confirm correct position correct position by aspirating bone G 10 mL sodium chloride 0.9% flush marrow contents or flushing with G 20 or 50 mL syringe to administer sodium chloride 0.9% 5 mL without fluid boluses encountering resistance G Infusion fluid G Secure needle with tape G Use 20 or 50 mL syringe to deliver Manual insertion is painful, bolus of resuscitation fluid use local anaesthetic unless patient unresponsive to pain. Infiltrate with lidocaine 1% 1–2 mL (maximum dose 0.3 mL/kg) and wait 90 sec

Figure 1: Access site on proximal tibia – lateral view

Issue 5 Issued: May 2013 16 Expires: May 2014 INTRAOSSEOUS INFUSION • 2/2 Figure 2: Access site on proximal tibia Figure 3: Access site on distal tibia – oblique view

Distal tibia COMPLICATIONS

G Access site on medial surface of tibia G Bleeding proximal to medial malleolus G Infection Distal femur G revert to central or peripheral venous access as soon as possible G If tibia fractured, use lower end of G Compartment syndrome femur on anterolateral surface, 3 cm G above lateral condyle, directing observe and measure limb needle away from epiphysis circumference regularly G palpate distal pulses and assess perfusion distal to IO access site G Pain from rapid infusion: give lidocaine 2% 0.5 mg/kg slow infusion

Issue 5 Issued: May 2013 Expires: May 2014 17 APPARENT LIFE THREATENING EVENT (ALTE) • 1/2

DEFINITION G Examination abnormal G Severe ALTE A sudden, unexpected change in an infant’s behaviour that is frightening to Immediate the observer and includes changes in G two or more of the following: FBC G G Breathing: noisy, apnoea U&E, blood glucose G G Colour: blue, pale Plasma lactate G G Consciousness, responsiveness Blood gases G G Movement, including eyes Blood culture G Muscle tone: stiff, floppy Urgent

INVESTIGATION OF FIRST ALTE G Nasopharyngeal aspirate for virology G Per-nasal swab for pertussis Clinical history G Urine microscopy and culture (microbiology) G Feeding G G Sleeping Urine biochemistry: store for possible further tests (see below) G Infant and family illness and medicines G Chest X-ray G Gestation at delivery G ECG Examination If events recur during admission, discuss with senior role of further G Full examination including signs of investigations (see below) non-accidental injury MANAGEMENT Assessment Admit for observation G SpO 2 G Fundoscopy by paediatric G SpO 2, ECG monitoring ophthalmologist if: G Liaise with health visitor (direct or via G recurrent liaison HV on wards) G severe events (e.g. received CPR) G Check if child known to local authority G history or examination raises child children’s social care or is the subject safeguarding concerns (e.g. of a child protection plan inconsistent history, blood in nose/mouth, bruising or petechiae, After 24 hr observation history of possible trauma) G If event brief and child completely well: G anaemic G reassure parents and offer Investigations resuscitation training G discharge (no follow-up appointment) Indicated if: G Aged <1 month old G <32 weeks gestation G Previous illness/ALTE

Issue 5 Issued: May 2013 18 Expires: May 2014 APPARENT LIFE THREATENING EVENT (ALTE) • 2/2

G All patients in following categories should have consultant review and be offered Care of Next Infant (CONI) Plus programme and/or home SpO 2 monitoring: G parents remain concerned despite reassurance G recurrent ALTE G severe ALTE (e.g. needing cardiopulmonary resuscitation/PICU) G <32 weeks gestation at birth G a sibling was either a sudden unexplained death (SUD) or had ALTEs G family history of sudden death If events severe (e.g. CPR given) or repeated events

G Multi-channel physiological recording

Further investigations

Exclude following disorders: Gastro-oesophageal reflux pH study +/- contrast swallow Seizures EEG Intracranial abnormalities CT or MRI brain Cardiac arrhythmias ECG and 24 hr ECG Upper airway disorder Sleep study Hypocalcaemia Ca and bone screen Metabolic assessment Urinary amino and organic acids Plasma amino acids and acylcarnitine Abuse Skeletal survey (including CT brain) Blood and urine toxicology (from admission) Continuous physiological or video recordings

Issue 5 Issued: May 2013 Expires: May 2014 19 ANAPHYLAXIS • 1/3

DEFINITION G ABC approach: provide BLS as needed Sudden onset systemic life-threatening G if airway oedema, call anaesthetist for allergic reaction to food, medication, potential difficult airway intubation contrast material, anaesthetic agents, G if not responding to IM adrenaline, insect sting or latex, involving either: give nebulised adrenaline 1:1000 G Circulatory failure (shock) (1 mg/mL) 400 microgram/kg (max G Difficulty breathing from one or more 5 mg) of following: G treat shock with sodium chloride 0.9% G stridor 20 mL/kg bolus G G bronchospasm monitor SpO 2, non-invasive blood pressure and ECG (see Algorithm ) G rapid swelling of tongue, causing difficulty in swallowing or speaking G Repeat IM adrenaline after 5 min if no (hoarse cry) response G associated with GI or neurological Do not give adrenaline disturbance and/or skin reaction intravenously except in Document cardiorespiratory arrest or in resistant shock (no response to G Acute clinical features 2 IM doses) G Time of onset of reaction SUBSEQUENT MANAGEMENT G Circumstances immediately before onset of symptoms G Observe for a minimum of 6 hr to IMMEDIATE TREATMENT detect potential biphasic reactions and usually for 24 hr, especially in following situations: Widespread facial or peripheral G severe reactions with slow onset oedema with a rash in absence caused by idiopathic anaphylaxis of above symptoms do not justify G reactions in individuals with severe adrenaline or hydrocortisone. asthma or with a severe asthmatic Give chlorphenamine orally component G reactions with possibility of continuing Management of anaphylaxis G See absorption of allergen algorithm G patients with a previous history of G Remove allergen if possible biphasic reactions G Call for help G patients presenting in evening or at G IM adrenaline: dose by age (see night, or those who may not be able Algorithm ) or 10 microgram/kg: to respond to any deterioration G 0.1 mL/kg of 1:10,000 in infants (up to G patients in areas where access to 10 kg = 1 mL) emergency care is difficult

G 0.01 mL/kg of 1:1000 (max 0.5 mL G Monitor SpO 2, ECG and non-invasive = 0.5 mg) BP, as a minimum G give in anterolateral thigh

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G Sample serum (clotted blood – must G Discharge with an emergency plan, get to lab immediately) for mast cell including 2 adrenaline pen auto- tryptase if clinical diagnosis of injectors after appropriate training anaphylaxis uncertain and reaction G If still symptomatic give oral thought to be secondary to venom, antihistamines and steroids for up to drug or idiopathic at following times 3 days and send to immunology: G Refer as out-patient to a consultant G immediately after reaction paediatrician with an interest in G 1–2 hr after symptoms started when allergy levels peak G >24 hr after exposure or in convalescence for baseline G If patient presenting late, take as many of these samples as time since presentation allows G Write mast cell tryptase on immunology lab request form with time and date of onset and sample to allow interpretation of results DISCHARGE AND FOLLOW-UP

G Discuss all children with anaphylaxis with a consultant paediatrician before discharge G Give following to patient, or as appropriate their parent and/or carer: G information about anaphylaxis, including signs and symptoms of an anaphylactic reaction G information about risk of a biphasic reaction G information on what to do if an anaphylactic reaction occurs (use adrenaline injector and call emergency services) G a demonstration of correct use of the adrenaline injector and when to use it G advice about how to avoid suspected trigger (if known) G information about need for referral to a specialist allergy service and the referral process G information about patient support groups

Issue 5 Issued: May 2013 Expires: May 2014 21 ANAPHYLAXIS • 3/3

Management of anaphylaxis Remove allergen

Partial Adrenaline IM Complete obstruction/ Nebulised adrenaline obstruction stridor Repeat nebuliser every Intubation Assess 10 min as required or surgical airway A Hydrocortisone

No problem Adrenaline IM Nebulised salbutamol Bag-mask ventilation Repeat salbutamol as Apnoea Wheeze Adrenaline IM Assess required Hydrocortisone B Hydrocortisone Consider salbutamol IV or aminophylline IV No problem

No pulse Adrenaline IM Basic and advanced Assess Shock Crystaloid life support C Adrenaline IV infusion

No problem

Reassess No problem Antihistamine 48 hr to ABC prevent recurrence

Drugs in Dosage by age anaphylaxis <6 months 6 months–6 yr 6–12 yr >12 yr Adrenaline IM: pre-hospital 150 microgram 300 microgram 500 microgram practitioners (0.15 mL of 1:1000) (0.3 mL of 1:1000) (0.5 mL of 1:1000) Adrenaline IM: 10 microgram/kg in-hospital 0.1 mL/kg of 1:10,000 (infants and young children) OR practitioners 0.01 mL/kg of 1:1000 (older children) 1 Adrenaline IV 1 microgram/kg = 0.01 mL/kg of 1:10,000 over 1 min Crystalloid 20 mL/kg Hydrocortisone (IM or slow IV) 25 mg 50 mg 100 mg 200 mg

1 Strength of IM adrenaline not intended to be prescriptive, 1:1000 or 1:10,000 is used depending on what is practicable: e.g. use of 1:1000 involves drawing up too small volumes when used in infants

ALSG: APLS Anaphylaxis Algorithm: Updated January 2010 reproduced with permission

Issue 5 Issued: May 2013 22 Expires: May 2014 PAIN ASSESSMENT • 1/1

FLACC SUGGESTED AGE GROUP: 2 months to 7 years Behavioural SCORING CATEGORIES 01 2 Face No particular expression or Occasional grimace or frown, Frequent to constant quivering smile withdrawn, disinterested chin, clenched jaw Legs Normal position or relaxed Uneasy, restless, tense Kicking, or legs drawn up Activity Lying quietly, normal position, Squirming, shifting back and Arched, rigid or jerking moves easily forth, tense Cry No cry (awake or asleep) Moans or whimpers, occasional Crying steadily, screams or complaint sobs, frequent complaints Consolability Content, relaxed Reassured by occasional Difficult to console or comfort touching, hugging or being talked to, distractible Each of the five categories: (F) Face; (L) Legs; (A) Activity; (C) Cry; (C) Consolability; is scored from 0 - 2 which results in a total score between 0 and 10 (Merkel et al, 1997) WONG AND BAKER PAIN ASSESSMENT – SELF REPORT

G Suggested age group ≥4 yr G Point to each face using the words to describe the pain intensity G Ask child to choose a face that best describes their own pain and record the appropriate number

0 1 2 3 4 5 6 7 8 9 10 From Wong D.L., Hockenberry-Eaton M., Wilson D., Winkelstein M.L., Schwartz P.: Wong's Essentials of Pediatric Nursing, ed. 6, St. Louis, 2001, p. 1301. Copyrighted by Mosby, Inc. Reprinted by permission

Analgesic interventions Analgesic ladder Play Specialist Increasing Pain Intervention by play staff 0=No pain Preparation aid used: doll, verbal 1–3 = Mild pain Severe 4–7 = Moderate pain Explanation , photos 8–10 = Severe pain Paracetamol, Distraction: toys, bubbles, music, multi sensory, NSAID + books potent opioid Moderate e.g. Morphine Refer all in need of analgesia and with (PCA / NCA) Paracetamol, behavioural concerns NSAID + weak opioid Mild e.g. Codeine

Paracetamol, +NSAID Slight Paracetamol

NB: Check BNFc for contraindications/interactions/precautions etc

Issue 5 Issued: May 2013 Expires: May 2014 23 ANALGESIA • 1/4

G For combination of analgesics to use, see Analgesic ladder in Pain assessment guideline TOPICAL

Age group Preparation Time to onset Comments <1 month Glucose syrup on pacifier During procedure For venepuncture or (available as a tootsweet) cannulation >1 month Ametop 30 min Causes itch, lasts 4 hr LMX4 30 min Wait 5 min after removing cream before cannulation EMLA 1 hr Remove after 1 hr >5 yr Ethyl chloride Immediately If cannot wait for cream

MILD PAIN (pain score 1 –3)

Drug and Dose Maximum dose Comments preparation Paracetamol G First dose 20 mg/kg Max total dose in 24 hr G For mild [oral/nasogastric THEN G Aged <1 month: pain (NG)] G aged 1–3 months: 30–60 mg 60 mg/kg/day G Increase G Suspensions: 8-hrly G Aged ≥1 month–18 yr: dose interval in G 120 mg/5 mL G aged 3–6 months: 60 mg 90 mg/kg (max 4 g) G renal G 250 mg/5 mL aged 6–24 months: 120 mg impairment G G Tablets/soluble aged 2–4 yr: 180 mg G Avoid large 500 mg G aged 4–6 yr: 240 mg doses in G aged 6–8 yr: 250 mg dehydration, G aged 8–10 yr: 375 mg malnutrition, G aged 10–12 yr: 500 mg hepatic impairment G aged 12–16 yr: 750 mg G aged >16 yr: 500 mg –1 g Paracetamol G First dose 30 mg/kg G Max total dose in 24 hr: G As for oral (rectal) THEN G aged <3 months: paracetamol G Suppositories G birth–3 months: 20 mg/kg 60 mg/kg G For mild G 60 mg 8-hrly G aged pain when G G aged 3 months–12 yr: ≥3 months: 90 mg/kg oral/NG 125 mg route not G 250 mg 20 mg/kg 4 -hrly for 48 hr then 60 mg/kg G G possible G 500 mg aged >12 yr: 500 mg–1 g aged >12 yr: 4 g 4-hrly G Suspension G 1 g can be given rectally

Paracetamol (IV) G <10 kg: 10 mg/kg 6-hrly G Aged <1 month: G As for oral 10 mg/mL G 10 –50 kg: 15 mg/kg 6-hrly 30 mg/kg/day paracetamol G >50 kg: 1 g 6-hrly G <50 kg: G For mild (<33 kg use 60 mg/kg/day pain when 50 mL vial G >50 kg: oral/NG/PR via burette or in 60 mg/kg/day route not syringe) G Up to 4 g daily possible Prescribe in mg G Give over (not mL) 15 min

Issue 5 Issued: May 2013 24 Expires: May 2014 ANALGESIA • 2/4

MODERATE PAIN (pain score 4–7)

Drug and preparation Dose Maximum dose Comments

Ibuprofen G Aged 3 months–12 yr: G Aged <12 yr: G If aged <3 months G Liquid 100 mg/5 mL 5 mg/kg max 30 mg/kg/day or <5 kg use only if G Tablets 200 mg and 6–8 hrly G Aged ≥12 yr: recommended by 400 mg G Aged ≥12 yr: max 2.4 g/day consultant 200–600 mg G Avoid in renal 6–8 hrly dysfunction G Contraindications: G shock G bleeding disorders G hypersensitive to aspirin or other NSAID G Can be given to asthmatics if no history of NSAID- induced wheeze and chest clear on auscultation G Caution in hypertension, heart failure Diclofenac G Aged >6 months: G Max 150 mg/day G As ibuprofen G Tablets: 300 microgram– G 1 mg/kg dispersible 50 mg 8-hrly (can be used to give smaller doses) G enteric coated 25 mg and 50 mg G Suppositories 12.5 mg, 25 mg, 50 mg and 100 mg Codeine G Aged <12 yr: G Max 240 mg/day G For moderate pain G Liquid 25 mg/5 mL 500 microgram– G Caution in hepatic G 1mg/kg impairment Tablets 15 mg, 4–6 hrly 30 mg and 60 mg G If aged <1 yr, use G Aged ≥12 yr: only if recommended 30–60 mg by consultant 4–6 hrly G Repeated doses increase risk of respiratory depression G Caution if renal impairment, obstructive or inflammatory bowel disease, raised ICP, compulsive disorders G Contraindications: G acute respiratory depression G paralytic ileus G Not to be given with other opioids G Prescribe laxatives if given for >24 hr

Issue 5 Issued: May 2013 Expires: May 2014 25 ANALGESIA • 3/4

SEVERE PAIN IN CHILDREN AGED >1 YR (pain score 8–10)

In head injuries/respiratory difficulties/upper airway obstruction, use opioids only with consultant advice. Monitor children needing oxygen and parenteral opioids with SpO 2 +/- TcCO 2 in an HDU setting

Analgesic method and Dose Monitoring technique Oral morphine G Aged >1–12 yr: G Respiratory rate , maintain: G Single dose before painful G 200–300 microgram/kg 4-hrly G aged 1–2 yr, >16 breaths/min procedure may be useful G Aged >12 yr: G aged 2–9 yr, >14 breaths/min G Use if no IV access or for G 5–10 mg 4-hrly (max 10 mg) G aged 10–16 yr, weaning from IV opioid >12 breaths/min G If to be taken regularly G if rate reduced, contact consider use of medical staff prophylactic laxative

Morphine patient/nurse- G If loading dose required: Hourly observations controlled analgesia G experienced staff only G (PCA/NCA) Pain score G 50–100 microgram/kg G Sedation score G PCA suitable for children G Background infusion if used G Pump displays aged >5 yr (understand G and will press button); 4–10 microgram/kg/hr G Syringe movement NCA otherwise G Bolus dose G Respiratory rate G G Nurses must be certified 10–20 microgram/kg G SpO 2 if needed G competent in use of Lockout time G TcCO 2 if needed PCA/NCA G 5–30 min G Use anti-reflux valve G Maximum dose in 4 hr of 4 hourly observations unless dedicated cannula 400 microgram/kg G Vomiting/itching G Use morphine 1 mg/kg G Urinary retention made up to 50 mL with sodium chloride 0.9% G Inspection of IV site G maximum of 50 mg/50 mL

Morphine infusion G Loading dose of Hourly observations G Use for severe pain when 100 microgram/kg given G Pain score unable to use PCA/NCA over 5–20 min (max 5 mg) G Sedation score G G Use anti-reflux valve Continuous infusion of G Respiratory rate (as above) 10–30 microgram/kg/hr unless dedicated cannula G SpO monitoring G G Start at 20 microgram/kg/hr 2 Use anti-siphon valve on G Syringe movement line except after major surgery G IV site for infection G when start at Use morphine 1 mg/kg G Urinary retention made up to 50 mL with 30 microgram/kg/hr and sodium chloride 0.9% adjust according to pain and sedation scores G max of 50 mg/50 mL IV intermittent morphine G Give slowly over 5 min Hourly observations G Infusion preferable G Aged 1–12 yr: G Pain score G 100 microgram/kg 4-hrly G Sedation score G Aged >12 yr: G Respiratory rate (as above) G 2.5–5 mg 4-hrly G SpO 2 monitoring

SC intermittent morphine G Flush with sodium chloride G Pain score G IV preferable 0.9% 0.3 mL G Sedation score G Site 22/24 G SC cannula G Prime cannula with G Respiratory rate (as above) at time of surgery or using morphine solution EMLA cream G Morphine: G suitable sites: uppermost G 100–200 microgram/kg 4-hrly arm, abdominal skin G max 6 times in 24 hr

Issue 5 Issued: May 2013 26 Expires: May 2014 ANALGESIA • 4/4

SEVERE PAIN IN CHILDREN AGED <1 YR (pain score 8–10)

In head injuries/respiratory difficulties/upper airway obstruction, only use opioids with consultant advice. Monitor children requiring oxygen and parenteral opioids with SpO 2 +/- TcCO 2 in an HDU setting

Analgesic method and Dose Monitoring technique Oral morphine G Aged 1–6 months: G Pain score G Use if no IV access or for 50–100 microgram/kg 4-hrly G Sedation score weaning from IV opiate G Aged 6–12 months: G Respiratory rate , maintain: 100–200 microgram/kg 4-hrly G if aged <6 months, >20 breaths/min G if aged ≥6 months, >16 breaths/min G if rate reduced, contact medical staff

G SpO 2 as appropriate Morphine infusion G Aged <1 month: Hourly observations G Use anti-reflux valve 50 microgram/kg over 5 min G Pain score unless dedicated cannula then 5–20 microgram/kg/hr G Sedation score G Use anti-siphon valve on G Aged 1–12 months: G Respiratory rate (as above) line G 100 microgram/kg over G SpO monitoring G 2 Use morphine 1 mg/kg 5 min then G Syringe movement made up to 50 mL with 10–30 microgram/kg/hr G sodium chloride 0.9% (0.5–1.5 mL/hr) Site for infection G G thus 1 mL/hr = G Adjust in increments of Urinary retention 20 microgram/kg/hr 5 microgram/kg/hr according to response

IV intermittent morphine G Aged <1 month: G Hourly observations for G Infusion preferable 50 microgram/kg 6-hrly 24 hr then 4-hrly if stable G Aged 1–12 months: G Pain score 100 microgram/kg 4-hrly G Sedation score G Respiratory rate (as above)

G SpO 2 monitoring G SC intermittent opiate Flush with sodium chloride G Pain score G IV preferable 0.9% 0.3 mL G Sedation score G Site 24 G SC cannula at G Morphine: G Respiratory rate (as above) time of surgery or using G aged <1 month: G SpO 2 as appropriate EMLA cream 100 microgram/kg 6-hrly G suitable sites: uppermost arm, G aged 1–6 months abdominal skin 100–200 microgram/kg 6-hrly (aged ≥6 months 4–6 hrly)

Issue 5 Issued: May 2013 Expires: May 2014 27 SEDATION • 1/3

ASSESSMENT PREPARATION FOR SEDATION

Sedation and anaesthesia belong to the Information required same spectrum of impaired consciousness G Age G In sedation, patient maintains the G Weight following vital functions without G assistance: Procedure for which sedation required G G protection of airway, swallowing, Previous sedation history cough reflex G Other drugs being taken G respiration G Other major diagnoses and implications in terms of respiratory G cardiovascular stability function and upper airway Cautions competence G Current health, including coughs, Discuss with anaesthetist before colds, pyrexia sedation if any of following present: G Oral intake status G Abnormal airway (including large tonsils) Consent for sedation (all cases) G Sleep apnoea Discuss with parent(s): G Respiratory failure G Unpredictable response to medication G Respiratory disease with significant G functional compromise Paradoxical excitation G G Active respiratory tract infection Failure of sedation (may need repeat dose or general anaesthetic at future G Cardiac failure date) G Raised intracranial pressure G Over-sedation (maintaining airway, G Decreased conscious level aspiration) G Neuromuscular disease Fasting for moderate–heavy G Bowel obstruction sedation G Significant gastro-oesophageal reflux G There should be the following interval G Renal impairment before procedure: G Liver impairment G after a full meal: 6 hr G Previous adverse reaction to sedation G after milk: 4 hr G Very distressed child G after clear fluids: 2 hr Potential difficulties For short, painless procedures Sedation can be difficult in children: (e.g. CT or X-ray), give infants G Taking anti-epileptics (can result in aged <4 months normal milk feed increased or reduced effect of only and allow them to sleep sedating drug) naturally G Already taking sedating drugs G With behavioural difficulties

Issue 5 Issued: May 2013 28 Expires: May 2014 SEDATION • 2/3

EQUIPMENT

G Portable oxygen G Portable suction G Appropriately sized face mask and self-inflating resuscitation bag G Two healthcare professionals trained in airway management with patient during sedation

DRUG CHOICE Sedation drugs

Drug Route Onset Duration Dose Comments Chloral G Oral 30 min–1 hr 1–2 hr G Night sedation G More efficacious hydrate G Rectal 30 mg/kg in infants <15 kg G Pre-anaesthesia or aged 50 mg/kg <18 months G Scans 70 mg/kg G max dose 2 g Melatonin G Oral 15–30 min 2–5 hr G Aged ≤5 yr: 5 mg G Use for sedation G Aged >5 yr: 10 mg before EEG G Use 5 mg initially, if no response, give further 5 mg Temazepam G Oral 45–90 min up to G 0.5 mg/kg G Only if aged ≥2 yr 4 hr G Up to 1 mg/kg for scans G CT, MAG3 scan G Max 30 mg Midazolam G Oral 15–30 min 1–2 hr G Aged 1 month–18 yr: G Have flumazenil 500 microgram/kg ready to give (max 20 mg) G Rectal G Aged 6 months–12 yr: 300–500 microgram/kg (max 20 mg) G Buccal G Aged 6 months–10 yr: 200–300 microgram/kg (max 5 mg) G Aged >10 yr: 6–7 mg G IV 2–3 min G 25–50 microgram/kg G IV cannulation over 2–3 min, (+ EMLA or local 5–10 min before anaesthetic) procedure (aged G More suitable for 1–6 yr max 2 mg; older children (not aged 6–12 yr max 6 mg; suitable for aged 12–18 yr max infants) 7.5 mg) G Not for CT scan Morphine G Oral 15 min 2–3 hr G Aged >1 yr: G May be combined sulphate 200–300 microgram/kg with midazolam (max 20 mg) 500 microgram/kg oral for painful procedures

Issue 5 Issued: May 2013 Expires: May 2014 29 SEDATION • 3/3

MONITORING

G Keep under direct observation G Once asleep or if <1 yr, monitor saturation continuously G Record saturation, heart rate and colour every 15 min G Discontinue once conscious level returned to normal SUBSEQUENT MANAGEMENT

Failed sedation

G Repeat maximum dose of initial drug used after expected period of onset G If repeat dose fails: G call anaesthetist who may give IV sedation (apply EMLA), or G reschedule procedure for later date under general anaesthetic Paradoxical excitement

G Do not attempt further drug dose G Discuss with anaesthetist. If unavailable that day, reschedule procedure for later date under general anaesthetic

Issue 5 Issued: May 2013 30 Expires: May 2014 IV FLUID THERAPY • 1/1

For previously well children aged 1 month–16 yr (excluding renal, cardiac, endocrinology, diabetic ketoacidosis and acute burns patients)

Hyponatraemia IfIf shockshock present,present, administer sodium chloride 0.9% 20 mL/kg or Symptomatic may develop as a compoundcompound sodium lactate (Hartmann’s) (10 mL/kg in the setting hyponatraemia is complication of of trauma)trauma) a medical any fluid regime RRepeatepeat if necessary and call for senior help emergency Consider blood or colloid if relevant

(VWLPDWHEstimate DQ\any ÀXLGfluid GH¿FLWdeficit DQGand UHSODFHreplace asDV sodiumVRGLXP chlorideFKORULGH 0.9% (with ZLWK orRU withoutZLWKRXW glucoseglucose 5%) OR compound sodium lactate (Hartmann’s) over over a a minimum minimum of of 24 24 hr hr Check plasmaplasma electrolytes

Calculate volume of maintenance and replacement fluids and select fluid type

VOLUME OF INTRAVENOUS MAINTENANCE FLUID TYPE OF INTRAVENOUS MAINTENANCE FLUID

<10 kg: 100 mL/kg/day In following circumstances, administer isotonic fluids 10–2010 20 kg:kg: 1000 mL + 50 mL/kg/day for each kg >10 kg such as sodium chloride 0.9% with potassium 0.15% >20 kg: 1500 mL + 20 mL/kg/day for each kg >20 kg x Plasma sodium <135 mmol/L x Intravascular volume depletion x Up to a maximum of 2500 mL/day (males) or x CNS infection 2000 mL/day (females) x Peri- and post-operative patients Use bags with potassium chloride premixed x Hypotension Check serum potassium x Head injury x Bronchiolitis VOLUME OF INTRAVENOUS REPLACEMENT FLUID x Sepsis (to replace losses, reassess every 4 hr) x Excessive gastric or diarrhoeal losses x Salt wasting conditions e.g. diabetes, CF x Fluids used to replace ongoing fluid losses should x Hypernatraemic dehydration (Na >160 mmol/L) reflect the composition of fluid being lost. Sodium Otherwise, children may be safely administered chloride 0.9% or sodium chloride 0.9% with potassium sodium chloride 0.45% with glucose 5% and 0.15% will be appropriate in most cases potassium 0.15%

Patients requiring both maintenance fluids and replacement of ongoing losses should receive a single isotonic fluid such as sodium chloride 0.9% with potassium 0.15% or sodium chloride 0.9% with glucose 5%

Monitoring x Weigh patient before starting fluid therapy, then daily if possible x Check plasma electrolytes before commencing infusion, except before majority of elective surgery x Check plasma electrolytes every 24 hr whilst intravenous fluids are being administered x if abnormal or if plasma sodium <130 mmol/L measure every 4–64 6 hrhr x Check plasma electrolytes immediately if clinical features suggestive of hyponatraemia; features include nausea, vomiting, headache, irritability, altered level of consciousness, seizure or apnoea x Maintain fluid balance chart to record input and output. Oliguria may be due to inadequate fluid, renal failure, obstruction or effect of ADH x 6RPH DFXWHO\ LOO FKLOGUHQ ZLWK LQFUHDVHG $'+ VHFUHWLRQ PD\ EHQHILW IURP UHVWULFWLRQ RI PDLQWHQDQFH IOXLGV WR Ҁ RI normal recommended volume x Contact senior paediatrician, PICU or paediatric anaesthetist if uncertain or ongoing fluid losses When using volumetric pump to administer IV fluids G Do not leave bag of fluid connected (blood components excepted) G Nurse to check following hourly: infusion rate infusion equipment site of infusion G Close all clamps and switch off pump before removing giving set

Issue 5 Issued: May 2013 Expires: May 2014 31 LONG LINE INSERTION • 1/3 INDICATIONS G Alcoholic chlorhexidine (or other skin antiseptic) G ‘Short’ long lines in patients requiring G 1 injectable bung 5–14 days IV therapy either in G 3 wide Steri-strips ® (optional to hospital or at home secure line) G Peripherally inserted central catheter G Sterile untoothed forceps (to feed line (PICC) for drugs that have to be given up butterfly) centrally (e.g. if they cause phlebitis), if risk of infection high (e.g. parenteral PROCEDURE nutrition) or for access >14 days EQUIPMENT PICC line preparation

G Assistant G Long line G ‘Short’ long line: G Leaderflex 22 G (2.5 F) line 8 or 20 cm G PICC: G Vygon PICC 3, 4 or 4.5 F 60 cm G Assess whether patient will need Lifecath (expert silver coated) sedation. Rarely, children with needle G Vygon Nutriline 2, 3 or 4 F 30 cm phobia will need the line inserted G Vygon Neocath or Epicutaneo-cave under general anaesthetic. Arrange catheter 2 F (23 G) 15, 30 or 50 cm appropriate person to administer has different insertion technique, not sedation recommended except neonates G If necessary, shave arm to avoid hair plucking when dressing removed DO NOT ATTEMPT INSERTION G Specify exactly where you would like UNLESS YOU ARE FULLY topical local anaesthetic cream sited. TRAINED Basilic vein (medial) is usually best. Use whichever line you have been Apply anaesthetic cream to chosen trained to use veins (3 sites) at least 1 hr before starting procedure G Flush solution: sodium chloride 0.9% G A BP cuff inflated to 80 mmHg is a 5 mL more reliable tourniquet than either an G Single dressing pack elastic strip or a nurse’s squeeze G G Sterile gloves Check patient’s notes for comments about previous line insertions. Some G Sterile scissors veins can be particularly difficult and G 2 extra sterile towels patient can often provide guidance G 5 mL syringe/green needle G Check whether blood samples are G Tape measure required G Sterile clear dressing (e.g. G Gather all necessary equipment Opsite ®/Tegaderm ®) including a spare line (unopened) G Incontinence pad G 2 extra packs gauze swabs

Issue 5 Issued: May 2013 32 Expires: May 2014 LONG LINE INSERTION • 2/3 Consent G Insert peelable cannula until blood flowing freely (it is not necessary to G Explain procedure and reassure patient thread needle into vein) in some patients this will come quite quickly so G Obtain and record consent have catheter ready Premedication and position of G Ask assistant to release tourniquet to patient reduce blood flow G Taking the PICC line in forceps, pass G Position patient seated in chair or it up through cannula. At about 5 cm, lying with his/her arm stretched out you will reach tip of the cannula. If and supported by table or bed (on a line passes easily beyond 6 cm, you utility drape) have probably succeeded. Resistance G ensure patient in position and at any point usually indicates failure comfortable, and lighting optimal to thread vein, or curling of line. G Measure distance from site of insertion Rotating butterfly needle so that the to sternal notch (if inserting in arm) or bevel faces downwards may help to xiphisternum (if inserting in leg) so introduce line into vein if it will not catheter tip is placed outside heart thread more than 5 cm G Insert line to previously measured Aseptic non touch technique distance from site of insertion (ANTT) G When tip of line is judged to be in correct position, carefully withdraw G Wash hands, and put on apron/gown sheath and remove from around line and sterile gloves by pulling apart the two blue wings G Clean patient’s skin thoroughly with G Pressing firmly on insertion site with a alcoholic chlorhexidine and allow to piece of gauze, remove cannula dry in area of planned insertion G Without releasing pressure on entry G Drape sterile sheet to expose only site (it may bleed for a few minutes), chosen vein, and cover surrounding reassemble line and flush with sodium areas to provide working room and a chloride 0.9% 2 mL flat surface on which to rest your line, G With sterile scissors, cut rectangle of forceps and flush gauze (1 x 2 cm) to prevent hub of Nutriline PICC line line rubbing skin G Check all connections are firmly G Assemble line fully and flush with tightened. Coil any unused line next sodium chloride 0.9%1 mL to ensure to insertion site and secure with Steri- patency strips ® G Place everything you will need onto G Cover entry site, connections and all sterile sheet within reach exposed line with one piece of clear G Ask assistant to apply tourniquet (or dressing (e.g. Opsite ®) squeeze patient’s arm), but remain G X-ray line with 0.5 mL of contrast (e.g. ready to release Omnapaque 240) in the line to check G Check patient is ready for you to start tip position if near heart or if no blood flushes back up line. Do not draw G Be careful: introducer for the PICC blood back up line (this increases risk line is much stiffer than a standard of line blockage) cannula and more likely to perforate G the entire vein Flush once more and line is then ready to use

Issue 5 Issued: May 2013 Expires: May 2014 33 LONG LINE INSERTION • 3/3

Leaderflex lines LONG LINE CARE

G These are inserted using Seldinger G Keep dressing clean and intact technique G Maintain aseptic technique for G Cannulate target vein with either accessing system and dressing needle provided or a blue cannula changes. Before accessing system, G Feed guidewire into vein through disinfect hub and ports with cannula sheath and remove sheath disinfectant compatible with catheter leaving wire in situ (e.g. alcohol or povidone-iodine) G Feed line over guidewire and into vein G Assess site at least daily for any signs with a gentle twisting action. It is of infection and remove if signs of important that, at any time, operator is infection are present (only short-term able to grasp directly either free end CVCs) of wire or wire itself as it passes G Replace administration sets every through skin, to ensure that it does 24 hr and after administration of not pass entirely into vein blood, blood products and lipids. G Remove guidewire and secure line in Routine catheter replacement is place unnecessary G It is not necessary to verify position of G Assess need for device daily and 8 cm lines radiologically remove as soon as possible G Document insertion and all Use an aseptic technique when interventions in patient notes accessing the system or for dressing changes

AFTERCARE

G Aim to insert to 20 cm and tape remaining silastic length to skin with an adhesive dressing e.g. Steri-strip ® G Place a folded half gauze swab under the blue hub before taping down with adhesive, then cover with transparent dressing, minimising contact between gauze and transparent dressing in case removal is required for troubleshooting G Flush after each use with sodium chloride 0.9% 2 mL

Issue 5 Issued: May 2013 34 Expires: May 2014 PRE-OP FASTING • 1/1

PRINCIPLES Infants/children aged <1 yr

G Do not fast patients for longer than Morning operating lists necessary for their safety under general anaesthesia G Last formula milk feed before 0230 hr G Do not deny fluids for excessively G Last breast milk feed before 0430 hr long periods; allow patients to drink G Water or diluted squash to finish within these guidelines before 0630 hr G Use theatre time efficiently Afternoon operating lists Ideally give all children (especially those aged <2 yr) clear fluids up G Last formula milk feed before 0700 hr to 2 hr pre-operatively. Liaise G Last breast milk feed before 0900 hr closely with theatre to discover G Water or diluted squash to finish approximate time of patient’s before 1100 hr operation Nursing and medical staff should ensure that all children are POLICY encouraged to drink clear fluids (e.g. water or diluted squash) until G Solid food and milk (including 2 hr before anaesthesia/surgery formula) up to 6 hr before elective surgery G Breast milk up to 4 hr before elective surgery G Encourage patients to take clear oral fluids up to 2 hr before elective surgery. Thereafter, sips of water may be taken to enable tablets to be swallowed G clear fluids do not include fizzy drinks PROCEDURE

All children aged ≥1 yr

Morning operating lists

G No solid food after midnight G Water or diluted squash to finish before 0630 hr Afternoon operating lists

G Light breakfast (including toast, or small bowl of cereal), to finish before 0700 hr G Water or diluted squash to finish before 1100 hr

Issue 5 Issued: May 2013 Expires: May 2014 35 POST GA MONITORING EX-PREMATURE INFANTS • 1/1

G Risk of apnoea after general Subsequent post-GA anaesthetic (GA) management G increased if anaemic G with chronic lung disease who have G High dependency nursing care required oxygen treatment within last G Monitoring to include: 6 months G continuous pulse oximetry MANAGEMENT G continuous ECG G continuous respiratory rate Pre-operative G transcutaneous CO 2 G Check haemoglobin G If apnoea >15 sec: G if Hb <90 g/L, arrange transfusion G immediate respiratory support by nurse (airway manoeuvres, bag and G Arrange overnight stay for post- mask ventilation) operative monitoring if age (weeks) <[3 x (38 – gestational age in weeks)] G contact on-call SpR e.g. baby born at 30 weeks gestation G liaise with anaesthetist responsible for would be kept overnight after GA if patient <24 weeks old. A 36 week baby G review period of HDU care would be allowed home after GA if >6 weeks old DISCHARGE AND FOLLOW-UP

Immediate post-GA period G Discharge patient home same day or next day as calculated by above G Transfer patient with oxygen supply, formula providing there have been no continuous SpO 2 monitoring and full apnoeic episodes resuscitative equipment G Admit patient to a designated HDU ward area

Issue 5 Issued: May 2013 36 Expires: May 2014 ASTHMA – ACUTE MANAGEMENT • 1/4

RECOGNITION AND Life-threatening ASSESSMENT G Cyanosis/pallor Definition G Decreased air entry/silent chest G Poor respiratory effort Asthma is a chronic inflammatory disorder G of the airways with reversible obstruction Altered conscious level G Irritable/exhausted Symptoms and signs G SpO 2 <92% in air G Breathlessness G PEF ≤33% in those aged ≥7 yr G Wheeze Patients with severe or life- G Cough threatening attacks may not be G Nocturnal cough distressed and may not have all G Tight chest these abnormalities. Presence of any one of these should alert G Bilateral wheeze doctor G Symptoms and signs tend to be: Differential diagnosis G variable G Foreign body G intermittent G Pneumonia G worse at night G Pneumothorax G provoked by triggers, including exercise G Aspiration Mild/moderate G Cystic fibrosis G Normal vital signs G Tracheobronchomalacia G Mild wheeze G Gastro-oesophageal reflux G Speaks in complete sentences or Assessment feeding G G SpO 2 >92% in air Record: G PEF >50% in patient aged ≥7 yr G respiratory rate and effort G Severe recession G heart rate G Too breathless to talk/feed G air entry G Tachypnoea (>40 breaths/min if aged G oxygen saturation in air <5 yr; >25 breaths/min if aged >5 yr) G if aged ≥7 yr, peak expiratory flow G Tachycardia (>140 beats/min if aged (PEF) <5 yr; >125 beats/min if aged >5 yr) G conscious level G Use of accessory muscles, recession subcostal and intercostal, flaring of Do not take any samples for routine alae nasi blood tests or routine blood gases.

G SpO 2 <92% in air Routine chest X-ray is unnecessary G Peak expiratory flow (PEF) ≤50% in a child with asthma predicted/best

Issue 5 Issued: May 2013 Expires: May 2014 37 ASTHMA – ACUTE MANAGEMENT • 2/4

IMMEDIATE TREATMENT Drug doses

G Follow algorithm Management of G Salbutamol nebulised, driven by acute wheezing in children 6–8 L/min oxygen: Senior assessment G aged <5 yr, 2.5 mg G aged >5 yr, 2.5 –5 mg If you are worried about child’s conscious level or there is no response G Ipratropium bromide (Atrovent ®) to nebulised salbutamol or poor nebulised: respiratory effort: G aged <12 yr, 250 microgram G Call senior doctor for further G aged >12 yr, 500 microgram assessment G Site an IV line G Prednisolone 0.5 mg/kg oral: G Initial dose of salbutamol IV over G aged <2 yr = max 10 mg once daily 5 min (max 250 microgram) G aged 2–5 yr = max 20 mg once daily G aged <2 yr: 5 microgram/kg G aged >5 yr = max 30 mg once daily G aged >2 yr: 15 microgram/kg G G Using 500 microgram/mL injection Hydrocortisone slow IV injection: preparation dilute to a concentrate of G aged <2 yr, 4 mg/kg (max 25 mg) 6-hrly 50 microgram/mL with sodium G aged 2–5 yr, 50 mg 6-hrly chloride 0.9% G aged 5–18 yr, 100 mg 6-hrly G e.g. withdraw 250 microgram = 0.5 mL and make up to a total volume Monitoring of 5 mL using sodium chloride 0.9% = 250 microgram in 5 mL G Record heart rate and respiratory rate every 10 min Not responding within 15 min G Continuous SpO 2 G Salbutamol 1–2 microgram/kg/min G Cardiac monitoring continuous infusion G Baseline U&Es (capillary blood gas G use 1 mg/mL solution for IV infusion for potassium) dilute 10 mg (10 mL) to concentration SUBSEQUENT MANAGEMENT of 200 microgram/mL made up to 50 mL with sodium chloride 0.9% Follow the algorithm Management of G If not responding increase up to acute wheezing in children 5 microgram/kg/min for 1 hr then reduce back to 2 microgram/kg/min Previous history G If requiring >2 microgram/kg/min G When recovering, ask about: admit to PICU G previous episodes of wheeze, similar G Use TcCO monitor 2 episodes G Continue with high flow oxygen and G triggering factors, seasonal variation continuous salbutamol nebuliser while waiting G nocturnal cough G family history of asthma, hay fever, eczema, other atopy G smokers in the family (including child)

Issue 5 Issued: May 2013 38 Expires: May 2014 ASTHMA – ACUTE MANAGEMENT • 3/4

G days off school because of asthma Discharge treatment G number of courses of prednisolone used in last year G Prescribe beta-agonist with spacer G pets G Give prednisolone 0.5 mg/kg daily for G drug history (device and dose) 3–5 days (if already on oral especially any bronchodilators/inhaled prednisolone maintenance therapy corticosteroids and their effect, speak to respiratory consultant/nurse) particularly need to use beta-agonists G Educate on use of PEF meter if aged >6 yr (not if child has never used one DISCHARGE AND FOLLOW-UP before) G Discuss follow-up in either nurse-led Discharge criteria asthma clinic or consultant clinic

G SpO 2 in air >94% Chronic management G Respiratory rate: <40 breaths/min G Give inhaled corticosteroid if any of aged <5 yr; <30 breaths/min aged following: >5 yr G frequent episodes G Heart rate: <140 beats/min aged <5 yr; <125 beats/min aged >5 yr G bronchodilators used most days G Peak flow: ≥75% predicted/best in G nocturnal and/or exercise-induced those aged >7 yr symptoms G Stable on 4-hrly treatment G other atopic symptoms and strong family history of atopy Discharge home same day if: G If recurrent upper respiratory tract problems or allergic rhinitis triggering G Child has made a significant attacks, give oral antihistamines +/- improvement and has remained steroid nasal spray stable for 4 hr G Parents: G understand use of inhalers G have a written personal asthma action plan G have a written discharge/weaning salbutamol information leaflet G know how to recognise signs of deterioration and the actions to take

Issue 5 Issued: May 2013 Expires: May 2014 39 ASTHMA – ACUTE MANAGEMENT • 4/4 Algorithm: Management of acute wheezing in children L m / I V = ? 0 d . i , ? m 9 l a u % n i t L c e s e d r o m o 2 t t m s l o o g u 5 o s a r n 0 t a a d i a o k o h e e i k m - d u e n / r b w s n b g l i m m y o u u ) u u i w f L t ( o d m l 1 l p h g m i ? i c i s L r k i s t m / u / h h h / e m w g e a c / s I m m t r l d V i h d / x o i m h o n m L ) L ( t , ] l m h r k e d 2 f L o 2 o c / i o u o m t g C d I n i h r f - h a u V n s n m i h e Y l d r R I / x m i i 4 i o i M e r t o u n h E t e N m o E o r I a 5 s n j w L P S . s i U k e r O c A r d t e i ( i i R e C i g n h c c h m n 1 = d p T m n e S s m n i / r g e t O a g l g 0 a i l a a g c S o A o i o l l i i 0 o u a n c t m L m n y n l n V L 2 l E r g i c m M 2 u . n r t e r r d i 1 g k d I 9 u d S u o m r L L y 2 N s O t n S / i O 5 a O y i % p l m e s c g / ) C S e ± = S C o t G a b s p 1 d m A r r i v r C I H m L n s n O p t o A e l E i O ± r . a r e L o e t 5 o a t y o r o g l e p m i L = i & 3 r B u o o B c r f H ‡ ‡ ‡ t h l ( c n e r d a l r / 0 ± B c t 2 r d v i / O 5 r o / o 0 i t a o a n m r o t l 0 1 a w o i 0 i a i U a 0 i r o m . p n n ? s s o o g L g f k ± 8 p 5 t n 0 L i k e t n m g i i u e T t t a n g r m U Y m i u s 1 2 m o p h e o L a / i f o m s A t o E w m i n o p S d m a n g o / m i c u 1 c [ m n S M t r f e o ( i r t r e r h c s o r 0 e i a e i o i b r r c o . n e r O r s . w g g r s o r k g g g n C E R o e o p A 2 A R C E B . a r L g g . r n a t m 2 C g o e o 5 l a g g C a e o r b i U c 0 i = I = s M ‡ ‡ ‡ m I 5 a r a r n p t 0 ‡ ‡ U c 0 t 2 a M ‡ ‡ ‡ ‡ n n m G n e e s c n i N a G a o u e o o . d 0 s m o t e s . 5 n g f e O o 9 f d d 2 0 t s s i O m 9 t c n l u F e u i n i n a u 0 d a a l n r % i 0 . a n s m a i % e c N s n s U 3 d u n c N t t m ) u 0 b i g n m e i i t a i e g u e o I o o e o b o S v o I o h d T n d o m m T 1 n u n n n l t i e a 5 u v I e o U c O m t l a m O O t s i r d t 0 e ) s i a t r r o a l a c o R o a i e 6 0 : N R S r d n v n r t S r t t g e i 0 o I o s d i i p o I m N n p s t r r N g l s a n a u t a g m G i r e G t m c m a t e o r h a r m 2 - f o e r a k a i l n 2 g h e s y n o r 0 r r 5 d a I f a u , y 0 f V m i 5 d r p 4 r r s 0 7 m m e s c , r l . t i h • t a o s v 9 o i g I r c p 2 n w e f % ) 5 r i n n p o r h 6 0 b h a u o g - r b S o r 3 t l s o r a a i l o y l n y h 0 ± a s a r u N W g - r j r m l y s s e S O m e l c b I O y n a V c d r m r o u i e R o C a l t n b v i t b g g o t S n H a m s u e e f l u r n e E o ( A o m 6 t r a s l a g e b N R w i N a m v m 5 s e e s T o m v u I E p l I i G 6 = - e e N o v l , a V g e H l m l b o A o = i d e E d G s / r 1 y r R c m k = l y S y i r i e ) r n 5 0 u o y 3 g r s S a < g - E d a 1 4 0 0 m s S n ” 3 L y i , E 9 2 2 X : ( A s v 0 5 1 a Y s % m I c 2 S 0 i k o I e ± ± 8 l F p T u - c M V b m a y % / x S I a < > < 2 5 h r l E E n M i r l u t P a y n l x Y i a b 1 1 2 l o a A C S P A S I P n N t o T g P t i / m a r a M n o 2 2 n n s r k t 2 r r O s i o l p E y e y R I L l o h m l t S i C B C t i l a i g b N L P i 5 e t s u e r d n a o O y y a n n M F p o O a a l u l / m o h T e n o r r r s e G n r o f i A A A A A 8 s i b C R r ‡ ‡ ‡ ‡ r n e V S t u o u n e O s t l y o = = e o r a e ± l g g g g g N d d I m x e s f s s s o e e c d s N M I s d m I H C 6 I ‡ ‡ H ‡ ‡ ‡ I 2 C e e e e e M y 2 5 O i t s s h n p n p i / r i p P c d A G 0 o s S e d d d d d g o i o o 5 0 g p i o b e f r t e P o g o g e I / o i I B x a n e c n n 0 0 a i l r n s M C a R n h n r n r h r o t o n C t s s t a u t m r s O s P U s i m m : m a m r n o i f t o c d Y e t l R o V u o o p i u i i a u i i s e E : c o r d O s I w f i o g s N y o u r r S t u e s V u o e b n n g m G a s n e . h s E g d y e e i , n n f e v o r M f s b a e e a b o c i E r p u m u r k o o t n N h m l m i x e s y T y b e l i s h a d ( g u - d u i m r x e f e l l T n l i y i p a / n s L i y a n h l e / r s 8 a e d a ± t a m e l m h d c g y g y a I d ) / / f / n m m / / o 0 k r 6 h m m p o v n g . n - i i r 5 o i c n n u i i a l 4 n n y h o o l - ± a a a C r r m r h a a l A t g g y R r r ¼ i g g N m s a d l e e e b b D m i s y I e e 0 5 p o O T s r s d d r r p , g M i d d 4 r e e k 5 n E g o m v p b b e a m < > 4 2 1 1 a a e ± e x R 6 o I < > e e a t g T 5 5 0 5 2 > > > > y t t n m r g n v I S a a k 5 5 h h o A g i 6 s y y t t a s s b E 2 g p p e t s s y y e = r r M o y . / i S V r n r r m 5 k u = y e E 2 Y r 6 E g a r m D R d e a 5 4 5 M M n s l y y B = R b I E A n s ( s 3 ” 0 2 0 5 5 1 S e r r m b P R D p d u k E i A o 0 ± % 1 ± ± ± 8 1 s C b r = = i m y T y y i O t p x s a S < > < > < 2 5 I o 0 r o o m u a H r m r r i O y a n r x N i 2 S m 1 p 0 5 5 1 2 l r m l c a A = = o s i g a M a t j i < . s 0 r l E 2 C o e n r 2 n t o 5 n f i y m e e R I s o o e r d S 9 m 5 2 S V e H r c a 5 w r p e o y k d n l G f y g d e 2 0 f t o S s o O r e I u , p a a i i A A A A A A A A A A r M , E % s c o l a - r b a f o i a = x c D F C N R e t u g g g g g g g g g g f n l P P f e m u e s r l R s T S U R H Y I v H S + S ‡ ‡ I ‡ ‡ O m c ‡ ‡ ‡ I s ‡ ‡ ‡ i m 2 b e e e e e e e e e e o e c 5 : i L f f R r e d l n e o l E E o / i s p ( 0 d d d d d d d d d d u a a s e e n p i o 0 A n s m p o a / a O g 1 r n S Q t o b e O t b l n a s a t e w 0 c e y T i o r b k m < 2 > a h 0 i V n s 2 a e p r n n i r U a e O o b u r o m 2 5 0 o m u t 2 t I f l s i g u u e t N E e l r h r r t f R 0 m N l d r 0 o s t e a t o e l y y i n N a G r a r a o a c a t w O n r r t a l e m T i e a t C r c o d n n h i e t s H l o = = r n c y r h e c t i Y i t : o p g c / g y E c e e l . n m m i o r e o h O r e R d s o r u a r s d n a a a s s F < > A a f g r m t s a s l t o s x x h o 5 5 l r P e n e i e a r e , t l w 1 3 Y : o y o y , y y ) m r 0 0 t a r r g m t ) t a p a a i m m 1 o s v m u v l y g g ± p k 0 e l , g g e i s e e a / e 0 g c f r d d e 3 o o a / 0 / / a m l L 0 k m m / e ± 0 e n n k t m g V . e s i d c c 5 i i 7 M n t n n 1 d S i e e ( o • n 5 I i y m N f a a g p d d r m m g g i b g r a a v e e e e b g g i i e 0 5 o l l d d d a r y 0 4 o o e n a n t b < > 1 Y s 4 1 1 n n c a n i e b s 0 ± / 5 5 E e 2 c c < < < < t r a h o e e S e 3 < p y y d t s l p D s o u a 0 r r u 9 d d 2 a - r I n p t e f 4 S a a i h l f e t M y d s % i i C p s D h e - l l y 7 y y n - 5 A l H ) r r I ( r I - 5 • i L l m 2 S ± i S s y A v = % n y % 0 D e < 2 > i o C S i R r f D a i R a 0 : m a n 2 5 l / E 5 a S H = o G i M e p > l I s E i n r h l > o S f r S 0 t a a n y p r E t A 9 2 a m m O w 9 h e 2 S t r x r e C O 2 h l R f g m 4 d e a = a D a A A A % E 1 H o e C P A C - % i G d c x s c a 0 E V g g g s m A r g o h N M S S P k v t E S m + O O a r ‡ ‡ ‡ e i o t 2 e e e S R H P S E R e C c R ‡ ‡ ‡ ‡ c n r i e m e R r o a p p E n l o / v e 0 d d d a i R e o d r p e t x n o e e i a s l c A o e H l x a c G e O e a i d r u e F p l m a y / Y O a c n d a s a p k g b m n v r b T b a a O i m l g 3 t e E t p k o r e i r i w i e e u f t m > p r e r r l i e k E d t o a i e 0 e n M i g o a t d n t e r l d h s i p w y e C a l l n e n e Issue 5 h a r l t l u t a o e E e o o a v m s t n g a + R t o r e e l i n e o r i w m r p o e l t t l n

s Issued: May 2013 y y o I e i z a r t a o s n n T a 4 r o y l e r l m c c o e p n g

40 E n M c 3 Expires: May 2014 s t r o e r a i c e a r a R a o a i n r e D t a g d c i l t m m l I n s e ( t n a h I A a d o L u t : e s p y n 2 e s a r V l n M l y t t i n e i c q a h S l t y a t o d E u n m e ) n u i e t o T s / a c s r r n c s e a e u a h e l n r t c a h s t t r e i e e g o n ) r e n a c e p l p e s y l t a t o e n r r f e e d i n g BRONCHIOLITIS • 1/3

G RECOGNITION AND SpO 2 <92% in >50% inspired oxygen ASSESSMENT G severe respiratory distress G Avoid tests that do not contribute to Definition immediate management. Perform following only for specific indications: G Acute viral inflammatory illness of G viral nose swab for influenza for small airways that occurs in winter oseltamivir if admission required epidemics and affects children aged when flu prevalence high <2 yr, with peak incidence at around G 6 months nasopharyngeal aspirate for respiratory virus immunofluorescence Symptoms and signs in severely immunocompromised patient to plan antiviral treatment G Coryzal symptoms for 2 –5 days G chest X-ray if there are localising before presentation signs, cardiac murmur or atypical G Cough (sometimes paroxysmal) presentation (e.g. aged >18 months) G Intermittent wheeze G U&E if there is a plan for IV fluids G Irritability and poor feeding G blood cultures if signs of sepsis or G Mild pyrexia – rarely higher than temperature >38.5ºC 38.5ºC IMMEDIATE TREATMENT G Respiratory distress with progressive tachypnoea, flaring of alae nasi and G Nurse in cubicle, or in bay with intercostal recession children with same diagnosis G Apnoea or hypoventilation G Strict hand washing to support infection control and use apron for G Hyperinflated chest on examination patient contact G Widespread fine crackles and wheeze G Nurse head up to reduce splinting of over both lung fields diaphragm Differential diagnosis G Clear airway by suction of nares and mouth G Recurrent viral-induced wheeze G Use sodium chloride 0.9% nose drops G Early asthma before suction G Cystic fibrosis G Nebulised sodium chloride 3% 4 mL G Pertussis 6-hrly G Recurrent aspiration Respiratory G Foreign body in trachea G If oxygen saturation ≤92% in air, give G Congenital lung anomaly oxygen via face mask with a reservoir Investigations bag G humidify oxygen G SpO 2 while breathing air G if mask not tolerated, use nasal G Capillary blood gas if: prongs for oxygen flow up to 1 L/min in children ≤5 kg body weight or up to G respiratory rate >80 breaths/min 2 L/min in children >5 kg G transcutaneous PCO >6 kPa 2 G use humidifier if available to warm oxygen

Issue 5 Issued: May 2013 Expires: May 2014 41 BRONCHIOLITIS • 2/3 G In patients with impending respiratory G IV fluids (as above) if: failure, review hourly. Consider G persistent respiratory rate additional respiratory support with >80 breaths/min CPAP or humidified high flow G cannulae (e.g. Vapotherm) if two or persistent vomiting more of following are present: G oxygen saturation <92% despite G respiratory rate >60 breaths/min or supplemental oxygen bradypnoea G deterioration of respiratory status G severe intercostal recession and during nasogastric feeding indrawing G marked increase in work of breathing G need for >2 L/min oxygen via nasal with poor coordination of sucking, prongs: SpO 2 <90% in >50% oxygen swallowing and breathing or cyanotic episodes despite Drug treatment supplemental oxygen (except cyanotic congenital heart disease) G In immunocompetent patients, drug G rising PaCO 2 (>3 kPa from baseline) treatment and physiotherapy (in acute G respiratory acidosis (pH <7.20) phase) are ineffective. Do not routinely prescribe salbutamol, Circulation and hydration ipratropium bromide (Atrovent ®), adrenaline, antibiotics or G Assess circulation and treat shock if corticosteroids present G For babies aged <6 weeks or patients G Correct dehydration if present with temperature >39ºC, discuss G Use IV fluids if oral fluids not tolerated antibiotics with consultant or significantly increased work of breathing G If symptoms <48 hr and influenza test positive or high prevalence influenza G restrict intake to 80% of estimated (see www.hpa.org.uk) and risk factors maintenance requirements (see I V (chronic respiratory, renal, liver, fluid therapy guideline) using sodium neurological or cardiovascular disease, chloride 0.9% with 10 mmol diabetic or immunocompromised) give potassium chloride per 500 mL oseltamivir G check U&E at least once every 12 hr while giving intravenous fluids (more Criteria for admission frequently if abnormal), and adjust volume and potassium content Absolute accordingly Feeds G Apnoea G Underlying cardiac defects, especially G Normal feeds (breast, bottle, solids) if large left to right shunt tolerated G SpO 2 <92% in air in a child in the G NG tube feeds if: early phase of the illness G oral intake by normal route insufficient G and Inadequate feeding G Dehydration G airway protective reflexes test normal on suctioning G Diagnostic uncertainty and G patient well enough to tolerate NG feeds

Issue 5 Issued: May 2013 42 Expires: May 2014 BRONCHIOLITIS • 3/3

Relative SUBSEQUENT MANAGEMENT

G Re-attends A&E in <48 hr G Fluid balance G Aged <6 weeks (corrected gestational G Oxygen support: age) G test the need for support 6-hrly G Unsatisfactory family circumstances G keep oxygen saturation ≥92% in and impaired ability to care for unwell recovery phase child G wean from nasal prongs to air as G Younger children (i.e. aged <6 tolerated months), presenting earlier in illness (<3 days symptoms) DISCHARGE AND FOLLOW-UP G Pre-existing lung disease, including G chronic lung disease, ex-preterm, Discharge home when: cystic fibrosis: inform speciality G fully fed orally consultant G SpO 2 >92% in air G Other pre-existing chronic disease G Hospital follow-up if: (e.g. neurodegenerative) G ventilated on PICU MONITORING TREATMENT G consolidation on chest X-ray (first reassess clinically, do not request G Standard nursing observations ‘routine’ follow-up X-ray) G Continuous oxygen saturation G ex-preterm with chronic lung disease monitoring if patient requires G supplemental oxygen GP follow-up in all other cases

G Transcutaneous CO 2 monitoring if patient using oxygen via nasal prongs at ≥2 L/min (approximately ≥60% oxygen) or has history of apnoea or colour changes G Continuous heart and respiratory rate monitoring if patient requires additional respiratory support

Issue 5 Issued: May 2013 Expires: May 2014 43 CROUP • 1/2

DEFINITION G Symptoms worse at night G Child does not look toxic G Acute viral inflammation of upper airway causing oedema of larynx and Assessment trachea and presenting with stridor G Record croup severity: G Causative agent: parainfluenza virus G (sometimes influenza, respiratory C – Cyanosis syncytial virus, rhinovirus) G R – Recession of chest Aetiology G O – Oxygen saturations (keep >92%) G UP – Upper airway obstruction e.g. G Aged 6 months–6 yr (peak age 2 yr) stridor G Seasonal peak: Spring and Autumn G respiratory rate G Transmission: usually by droplet G heart rate spread G level of consciousness G Incubation period 2–6 days G Do not examine throat as it may cause acute severe/total Differential diagnosis of stridor obstruction

Acute G Do not distress child G Any clinical concerns call consultant G Croup paediatrician immediately G Epiglottitis (rare since immunisation Severity against Haemophilus influenzae type B) G Bacterial tracheitis Mild croup G Foreign body G Barking cough Chronic G Stridor G Allergic airways disease (recurrent G No recession croup) G No cyanosis G Congenital abnormality e.g. laryngeal Moderate croup haemangioma G Laryngomalacia G Intermittent stridor at rest G Foreign body G Mild recession G Laryngeal papilloma G Alert and responsive CROUP Severe croup

Symptoms and signs G Stridor at rest G Cyanosis G Preceding coryzal illness G Oxygen saturation <92% in air G Fever G Moderate to severe recession G Harsh bark/seal-like cough G Apathetic/restless G Hoarse voice G Inspiratory stridor

Issue 5 Issued: May 2013 44 Expires: May 2014 CROUP • 2/2

Investigations G High flow oxygen 15 L/min via mask with reservoir bag G No investigations necessary, do not G Contact on-call consultant attempt to take blood or put in cannula paediatrician urgently to assess G If diagnosis unclear, or child severely clinical situation unwell, call consultant as an G discuss whether to involve on-call emergency measure paediatric anaesthetist and ENT surgeon IMMEDIATE MANAGEMENT G If no sustained improvement with adrenaline and dexamethasone: Mild to moderate croup G secure airway in theatre by G Antipyretics experienced anaesthetist G Adequate fluid intake G transfer to PICU G Leaflet on croup and reassurance DISCHARGE AND FOLLOW-UP G Oral dexamethasone 150 microgram/kg, can be repeated G Leaflet on croup 12 hr later if symptoms persist G Antibiotics, antitussives and G Admit/observe for 4 hr and reassess humidified air do not help G If better, discharge with 1 dose of G Advise paracetamol to control fever dexamethasone 150 microgram/kg and encourage oral fluid intake oral, telling parents to use if G Advise parents to seek help urgently symptoms persist 12–24 hr later. If if any of the following are present: dexamethasone not available as TTO, G drooling discharge with prednisolone 1 mg/kg G as a single dose 12–24 hr after laboured breathing dexamethasone if symptoms persist G persistent fever G biphasic/worsening stridor If parents do not clearly G understand what to do, do not cyanosis discharge G reduced level of consciousness/confusion Severe croup G No need for follow-up of croup

G Keep child and parents calm: do not upset child e.g. by forcing oxygen mask onto face or examining throat; nurse on parents lap and in position they find comfortable G Nebulised adrenaline 400 microgram/kg to max 5 mg (0.4 mL/kg to max 5 mL 1:1000) relieves symptoms, but short duration of action G Dexamethasone 150 microgram/kg oral (or if child refuses to swallow oral medication, nebulised budesonide 2 mg)

Issue 5 Issued: May 2013 Expires: May 2014 45 CYSTIC FIBROSIS – ADMISSION • 1/2

ARRANGING ADMISSION G U&E, creatinine, chloride, calcium, magnesium, phosphate, albumin, total G Via CF nurse specialist with ward protein, alkaline phosphatase, sister bilirubin, AST/ALT, GGT, CRP G Refer to admission plan in notes or G Glucose clinic letter If aged >5 yr G Always admit to a cubicle All of the above plus: ADMISSION PROCEDURE G If symptoms could be caused by allergic G Plot baseline weight, height bronchopulmonary aspergillosis, specific IgE to aspergillus and G Perform flow volume loop spirometry aspergillus precipitins. Also total IgE on admission day (aged ≥6 yr) G If diabetic, HbA G Write up drug chart before parents 1c leave If aged ≥10 yr G Check whether annual bloods could conveniently be taken now (see G Add glucose tolerance test (at 0, 60 Annual bloods ) and 120 min) G Ask nursing staff to inform G Baseline DEXA scan (repeated every physiotherapist and dietitian on day of 2–3 yr) admission Chest X-ray G Check specific aspects of management or investigations, as G Most children have a chest X-ray described by CF team every 6–12 months so another may G for IV antibiotics, see Cystic fibrosis not be necessary – Exacerbation guideline G Check when latest was taken and, if G for bowel blockage, see Cystic fibrosis in doubt, discuss with CF consultant – Distal intestinal obstructive Lung function and oxygen syndrome (DIOS) guideline saturation INVESTIGATIONS G Measure FVC and FEV 1 using ward Annual bloods spirometer (physiotherapist/trained nurse can take these measurements G All children attending CF clinics have if requested): annual blood screening G in all children who can blow reliably G Perform annual bloods if admission (usually from aged 6 yr) within a month of annual screening G on admission and at least weekly, (usually at time of birthday): preferably before ward rounds G during insertion of a long line or Port- G towards the end or after completion of a-cath needle, or when checking a course of IV antibiotics, take tobramycin level measurements before and after inhalation of salbutamol MDI All ages 4–8 puffs via a spacer G G FBC and film Monitor oxygen saturation overnight for first 2 nights after admission G Vitamins A, D, E G if saturations <91%, give oxygen via G Parathyroid hormone nasal cannulae or face mask

Issue 5 Issued: May 2013 46 Expires: May 2014 CYSTIC FIBROSIS – ADMISSION • 2/2

Microbiology OR G Creon 10,000 one-quarter (2500 units G In hospital, request twice weekly lipase) to one-half capsule (5000 units sputum/cough swab lipase) per 120 mL milk or breast feed G usually performed by physiotherapist G Children but check this has been done G starting dose Creon 10,000 2 Cystic G If new pathogen found, see capsules per meal, 1 capsule per fibrosis – Microbiology guideline snack and cross-infection G Dose titrated with fat content of meals Screening for hyperglycaemia and snacks to control symptoms of malabsorption About 8% of children with CF G maximum 10,000 units lipase/kg/day, develop diabetes after age 10 yr, higher doses can result in colonic usually manifests as weight loss; strictures ketoacidosis is rare Signs of malabsorption G Fatty pale stools, frequent, smelly, G If taking regular oral corticosteroids, orange oil, excess flatulence, screen for glucose intolerance at abdominal pains admission G discuss with CF team G During first 24 hr after admission, request glucose stick profile before H2-receptor antagonists breakfast, 1–2 hr after every meal, G If taking large doses of pancreatic and at 0200 hr if on overnight feeds enzymes (e.g. >10,000 units lipase), G If prednisolone started or dosage discuss with CF team need for increased during admission, repeat concurrent ranitidine to reduce glucose stick profile deactivation of pancreatin G If blood glucose elevated, discuss with CF team Vitamins A, D and E NUTRITION Starting dosage for newly-diagnosed G Infants G Always involve dietitians G 0.6 mL Dalivit ® or/and 0.5 mL (50 mg) G Weigh twice weekly, in nightwear Vitamin E and before breakfast (weigh babies naked if possible) G Children G Continue normal supplements G 1.2 mL Dalivit ® or 3 BPC Pancreatic enzyme supplements multivitamins capsules and 100 mg Vitamin E (2 x 50 mg capsule) G Continue same type and dose of OR pancreatic supplement as already G continue dose as prescribed in CF prescribed clinic Starting dosage for newly G Vitamin levels are checked annually and dosage adjusted accordingly diagnosed child Oral sodium chloride G Infants 1 G Creon Micro for children /2 scoop G Only if prescribed by CF team (2500 units lipase) to 1 scoop G Often needed in first year of life after (5000 units lipase) per 120 mL milk or diagnosis has been made breast feed

Issue 5 Issued: May 2013 Expires: May 2014 47 CYSTIC FIBROSIS – EXACERBATION • 1/2

RESPIRATORY G Take into account any past allergic INFECTION/EXACERBATION reactions First-line regimen If unusual symptoms, such as haemoptysis, abdominal pain (distal G Sputum culture intestinal obstruction syndrome), or G Pseudomonas aeruginosa: bleeding varices, discuss urgently with CF consultant ceftazidime 50 mg/kg 8-hrly (max 3 g/dose) and tobramycin 10 mg/kg Symptoms and signs once daily (max 660 mg) given over 30 min G Increasing cough and sputum G no Pseudomonas aeruginosa: G Increasing dyspnoea cefuroxime 50 mg/kg 8-hrly (max G Weight loss with loss of appetite 1.5 g/dose) G Thick, tenacious sputum G Courses usually last 2 weeks G Coarse crepitations G For cephalosporins (but not tobramycin), aim to use whole vials G Haemoptysis by rounding doses +10% considering G Signs of right heart failure vial size Investigations G After satisfactory tobramycin blood levels established, CF team will teach G See investigations in Cystic fibrosis parents to give antibiotics at home. – Admission guideline Discuss with pharmacy as well Differential diagnosis Nebulised antibiotics

G Non-CF bronchiectasis G Give children colonised with Pseudomonas , colomycin 1 million G Chronic obliterative bronchiolitis units made up to 4 mL with sodium ADDITIONAL ADMISSION chloride 0.9%, nebulised 12-hrly PROCEDURE Oral antibiotics G If IV antibiotics required, discuss with G Children are rarely given oral CF team re procedure: antibiotics during admission but may G agree a clear individualised procedure resume an oral agent on discharge for every patient Bronchodilators G discuss with CF team whether anaesthetic team needed for needle- G Prescribe salbutamol by MDI and phobic patients spacer before chest physiotherapy in G Trained nursing staff needed to hospital needle Port-a-cath Inhaled corticosteroids IMMEDIATE TREATMENT G There is no evidence these are of G Use IV antibiotic regimen suggested benefit. Discuss with CF team re following discussion with CF team stopping G If no discussion possible, stop oral antibiotics and give first-line regimen (see below)

Issue 5 Issued: May 2013 48 Expires: May 2014 CYSTIC FIBROSIS – EXACERBATION • 2/2

TOBRAMYCIN MONITORING DISCHARGE AND FOLLOW-UP

Once daily regimen: G On advice of CF team G Trough level immediately before 2 nd th Self-administration of IV and 8 dose antibiotics – home IV therapy G Should be <1 mmol/L G High levels need to be discussed G Service managed by CF nurse in with CF consultant conjunction with hospital pharmacy G No need to determine peak G Discuss fully with CF nurse before making any changes or arrangements G Always discuss dose changes with CF team beforehand Criteria for home administration G Do not check tobramycin dose via of IV antibiotics Port-a-cath or long line Ensure that: SUBSEQUENT MANAGEMENT G CF team and ward staff happy for patient to be discharged G Do not change antibiotics before entirely happy, discussing with CF team G Patient and parents confident and competent to Oral corticosteroids administer IV antibiotics at home G Patient/parent has been assessed G If no chest improvement after a week before discharge by CF team of IV antibiotics, consider starting 7 day course of prednisolone G Parents have written guidelines and 24 hr 1 mg/kg/day contact numbers G If already taking alternate-day G If patient considered responsible prednisolone at lower dosage, review enough to self-administer IV dosage needed at discharge antibiotics, important that parent/carer also has adequate instruction and G For children with allergic broncho guidance pulmonary aspergillosis (ABPA), continue prednisolone for longer (e.g. G Anaphylaxis kit at home and family at least one month) know how to use G Notify CF liaison nurses of any patient Dornase alfa (DNAse) discharged on home antibiotic therapy so they can arrange support at home G Discuss need for dornase alfa with or at school if necessary CF team G CF liaison nurse will visit patient at G Indications for use are: home during his/her course of IV G cough productive of sputum or therapy, to monitor progress sputum difficult to expectorate G Feedback any concerns to CF team G Give dornase alfa (2.5 mg/daily) via nebuliser after morning physiotherapy G Patients should bring their own nebuliser (usually a modified Sidestream ®) and compressor into hospital

Issue 5 Issued: May 2013 Expires: May 2014 49 CYSTIC FIBROSIS – MICROBIOLOGY • 1/2

In addition to standard precautions and Pseudomonas chronic infection hand hygiene, the following precautions are required for patients G Defined as 3 or more isolations in infected/colonised with transmissible 6 months from sputum/cough swab pathogens samples taken at least 1 month apart G Do not share equipment between patients Nebulised antibiotics G Nurse children with CF in a cubicle G If chronically infected with G Prevent contact between CF patients Pseudomonas , give colomycin 1 million units made up to 4 mL with PATIENT NEWLY DIAGNOSED sodium chloride 0.9%, nebulised WITH CF 12-hrly G Prophylaxis with flucloxacillin 125 mg G Nebulised tobramycin to be decided oral 12-hrly until aged 2 yr by CF team G If newly diagnosed CF patient has BURKHOLDERIA CEPACIA chest infection requiring IV antibiotics: COLONISATION G commence cefuroxime IV for Report any new cases to CF team 2 weeks, then co-amoxiclav oral for G immediately 3–4 weeks B. cepacia G Subsequent treatment depends on G Nurse children with microbiology colonisation in a cubicle on a separate ward from other CF children PSEUDOMONAS AERUGINOSA G Use separate spirometer with disposable filters First isolations in sputum or cough/throat swabs MRSA COLONISATION Report any new cases to CF team G If asymptomatic with first isolation G immediately from sputum/cough swab: G ciprofloxacin: aged 1 month–18 yr G Use normal spirometer with a disposable filter 20 mg/kg oral 12-hrly (max 750 mg) and colomycin aged <2 yr 1 million CHICKENPOX AND CF units 12-hrly, aged ≥2 yr 2 million units 12-hrly via nebuliser for G Varicella infection can have serious 3 months consequences in immunosuppressed G If recurrent isolation from children sputum/cough swab: G CF patients taking oral corticosteroids G ciprofloxacin and colomycin or are at high risk intravenous antibiotic – discuss with G If no history of chickenpox and no CF team antibodies, vaccinate G If symptomatic: G tobramycin and ceftazidime IV for 2 weeks, followed by: ciprofloxacin and colomycin as directed by CF team

Issue 5 Issued: May 2013 50 Expires: May 2014 CYSTIC FIBROSIS – MICROBIOLOGY • 2/2

Exposure PORT-A-CATH

G Ask about exposure to a known case: G Use in children requiring frequent IV G being in the same room (e.g. in the antibiotics house, classroom or hall in school) for G Manufacturer's instructions found on ≥15 min ward G face-to-face contact, for example G Observe sterile precautions whenever whilst having a conversation Vascuport accessed G If exposure significant, check notes to G Accessed only by trained nursing staff determine immune status (history of chickenpox or antibody status before Routine flushing of Port-a-cath corticosteroids) (usually by nursing staff) G If non-immune and taking a high dose G Every 4 weeks (coincide with clinic of oral corticosteroid (prednisolone appointment where possible) 1 mg/kg/day for one month or G 2 mg/kg/day for 1 week), and Use a straight Port-a-cath needle and exposure occurred <1 week earlier, 4 mL heparinised sodium chloride ® give varicella-zoster immunoglobulin 0.9% 100 units/mL (e.g. Canusal , ® (VZIG) aged <6 yr 250 mg; aged not Hepsal ), withdrawing needle 6–10 yr 500 mg; aged 11–14 yr while injecting last mL 750 mg; aged >15 yr 1 g, or IV immunoglobulin 0.2 g/kg G If non-immune and taking a modest dose of oral corticosteroid (prednisolone <1 mg/kg/day), give aciclovir prophylaxis 6-hrly: aged <2 yr 200 mg; aged >2 yr 400 mg 7–21 days after exposure Infected

G If chickenpox appears in a child not taking oral corticosteroid, give aciclovir 10 mg/kg oral 6-hrly for 7 days and a course of oral antibiotics (e.g. amoxicillin and flucloxacillin) INFLUENZA AND PNEUMOCOCCAL VACCINE

G Influenza vaccine every October G Conjugate pneumococcal vaccine (Prevenar13 ®) G Usually prescribed by patient’s own GP but obtainable from pharmacy

Issue 5 Issued: May 2013 Expires: May 2014 51 CYSTIC FIBROSIS – DISTAL INTESTINAL OBSTRUCTION SYNDROME (DIOS) • 1/1

RECOGNITION AND G Start early in the morning and ASSESSMENT continue until stools are yellow, watery and free of solid matter G Faeces can accumulate in distal ileum G 2 L in first instance, increasing to 3 or and caecum causing varying degrees 4 L depending on response, age and of intestinal obstruction size of child (most children with DIOS G Patients present with intermittent will be teenagers) abdominal pain, constipation and G Withhold food but, if success not faecal masses, usually in right or left achieved after 12 hr, stop, give an iliac fossa evening meal and resume following morning MANAGEMENT G Monitor effectiveness with pre- or G If symptoms mild, prescribe daily post-plain abdominal X-ray before macrogol laxative (e.g. Movicol) see and after lavage BNFc and encourage fluids G If signs of complete intestinal G Consider adjusting pancreatic obstruction, stop lavage, give IV fluids enzymes – but discuss with CF team and discuss contrast enema with CF team G If unresponsive, or symptoms more severe: G single dose of sodium amidotrizoate (Gastrografin): see dose and treatment practice in BNFc G repeat dose after 12–18 hr, encourage drinks, monitor fluid balance and allow food G If no effect after 24–48 hr or if patient deteriorates, give balanced electrolyte solution (discuss with CF team and gastro team) G Bowel lavage with Klean-Prep ® (usually requires a nasogastric tube) G 1 sachet Klean-Prep ® in 1 L give: 10 mL/kg/hr for 30 min then 20 mL/kg/hr for 30 min then 25 mL/kg/hr up to max total dose of 100 mL/kg or 4 L

Issue 5 Issued: May 2013 52 Expires: May 2014 PNEUMONIA • 1/3

If aged <1 month-old, refer to Investigations Neonatal guidelines G Pulse oximetry RECOGNITION AND G CXR ASSESSMENT G Full blood count, blood culture G Serum electrolytes (may have Definition hyponatraemia owing to SIADH), CRP G G Inflammation and consolidation of the If mycoplasma pneumonia suspected, lung caused by a bacterial, viral or mycoplasma titre (indicate date of mycoplasma infection onset on request form) G G Absence of clinical signs AND Sputum if able to provide good quality negative CXR makes pneumonia specimen unlikely G Nasopharyngeal aspirate or nasal swab in viral transport medium for G Up to 35% of lower respiratory tract infections have single virus as respiratory viruses causative organism G If pertussis suspected, pernasal swab in charcoal transport medium G Can be presenting illness in cystic fibrosis and immunodeficiency states G Pleural fluid culture and PCR if aspirated Symptoms and signs G If severe pneumonia, pneumococcal antigen in urine G Cough G Fever Differential diagnosis G Irritability G Bronchiolitis with atelectasis (usually G Poor feeding aged <1 yr) G Vomiting G Foreign body aspiration G Tachypnoea at rest (most useful sign) G Tumour (‘round’ pneumonia) G Beware: awake or unsettled Empyema/lung abscess infants can have high respiratory G Tracheobronchitis rate on a single measurement; G Whooping cough measure at rest and repeat IMMEDIATE TREATMENT Table 1: WHO definition of tachypnoea See Flowchart Age Counted breath rate Pleural effusion <2 months ≥60/min 2–11 months 50/min ≥ G See Pleural effusion guideline 1–5 yr ≥40/min

G Bronchial breathing, inspiratory crackles G Recession G Abdominal pain (referred pleural pain) G Aged >5 yr, headache, arthralgia, sore throat (suggests mycoplasma)

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SUBSEQUENT MANAGEMENT DISCHARGE AND FOLLOW-UP

G Change from IV to oral within G Follow-up within 6–8 weeks with CXR 24–48 hr if: G Total antibiotic course 5–7 days G lobar collapse G If atypical or staphylococcal G significant pleural effusion pneumonia, treat for 14 days G ‘round’ pneumonia on CXR uncomplicated CAP and 14–21 days G for severe CAP previous lower respiratory tract infections G Physiotherapy once cough productive G failure to thrive G important if neuromuscular G impairment results in poor clearance GP follow-up for all others within 6–8 weeks G Maintain hydration G Convalescent mycoplasma titre can G oral fluids if tolerated be obtained at this visit (indicate date G if unable to take oral fluids and Na of onset on request form) >135 mmol give sodium chloride 0.45% with glucose 5% and potassium chloride 10 mmol/500 mL via IV infusion. If Na <135 mmol use sodium chloride 0.9% with glucose 5% with potassium G restrict IV fluid replacement to 80% maintenance G monitor electrolytes MONITORING TREATMENT

G Continuous SpO 2 monitoring if needing oxygen G 1–4 hrly observation depending on severity of illness G If no improvement in 24–48 hr, review diagnosis (repeat chest X-ray) or treatment

Issue 5 Issued: May 2013 54 Expires: May 2014 PNEUMONIA • 3/3 Flowchart: Management of community acquired pneumonia in a previously well patient aged >1 month-old

ANY of following apply: *'Very unwell' implied by: G Aged <3 months G Drowsiness/lethargy G SpO <92% in air 2 G G Intermittent apnoea/grunting Lower chest indrawing G G Tachypnoeic Nasal flare G Pleural effusion G Poor feeding/dehydrated G Very unwell* NO G Consider out-patient management YES G If aged <1 yr, arrange review by senior doctor before discharge Admit to hospital

G Poor perfusion G Altered level of consciousness YES G Resuscitate G Respiratory failure: hypoxia, G Discuss case with PICU hypercapnia, acidosis

NO

G Oxygen sats <92% in air: Give oxygen G Gentle suctioning to clear nasal secretions G Paracetamol for pyrexia

YES Pleural effusion? Pleural effusion guideline

G Oral amoxicillin (or if pencillin allergy give macrolide e.g. azithromycin, clarithromycin) G If vomiting, IV benzylpenicillin G If severe symptoms, IV co-amoxiclav + macrolide G FBC, U&E G Fluid balance/observations

YES Pneumonia with influenza Oseltamivir + co-amoxiclav

YES Add flucloxacillin Suspected Staph. aureus e.g. bullae on CXR

YES Add metronidazole Aspiration

YES Change to piperacillin/tazobactam Hospital acquired G Change from IV to oral antibiotics YES G Discharge Improves in 24-48 hr G Total antibiotic course for 5–7 days NO G Follow up within 6–8 weeks. See Discharge and follow-up G Discuss with consultant G Review chest X-ray G ?organism

Issue 5 Issued: May 2013 Expires: May 2014 55 PLEURAL EFFUSION • 1/3

RECOGNITION AND G If risk factors for coagulopathy or ASSESSMENT thrombocytopenia check and correct before drain insertion Symptoms and signs G Pleural fluid analysis for: Gram stain and bacterial culture G Investigate for effusion if persistent differential cell count pyrexia or unwell 48 hr after treatment started for pneumonia AAFB and TB PCR and culture G LDH, protein, glucose and pH (via Differential diagnosis blood gas analyser) at same time, G Uncomplicated pneumonia blood samples for FBC, clotting screen, U&E, LDH, G Malignancy protein, albumin, glucose G Heart failure G CRP G Pancreatitis G Blood cultures G Pulmonary embolism G Sputum culture, if possible Investigations G If recurrent infections, investigate for immune deficiency (first line: FBC, G Chest X-ray PA or AP (no need for IgG, A, M, functional antibodies and lateral) HIV antibody) G Ultrasound (US) scan to: It is not necessary to obtain confirm presence of effusion sample for pleural fluid culture ascertain volume routinely before chest drain ask radiologist to mark optimal insertion if cause likely to be position for chest drain infective. If alternative cause differentiate between simple and suspected, try to avoid complicated effusion (e.g. loculations, unnecessary chest drain insertion heterogeneous material) by obtaining diagnostic aspirate G If history, chest X-ray or US of pleural fluid for cytology suggestive of malignancy, request CT chest

Table: Fluid/serum protein and LDH ratios are best discriminators between transudate and exudate Transudate Exudate Appearance Serous Cloudy, bloody Leucocyte count <10,000/mm 3 >50,000/mm 3 Protein ≤30 g/L >30 g/L Fluid/serum protein ratio ≤0.5 >0.5 2 LDH ≤200 IU >200 IU or > /3 local upper limit of serum LDH Fluid/serum LDH ratio ≤0.6 >0.6 Glucose ≥3.3 mmol/L <3.3 mmol/L pH ≥7.4 ≤7.3 Gram stain/culture No organisms Organisms on stain or culture

Issue 5 Issued: May 2013 56 Expires: May 2014 PLEURAL EFFUSION • 2/3

IMMEDIATE TREATMENT Refer to respiratory paediatrician Supportive G Remember that underlying cavitating disease may lead to bronchopleural G ABC fistulae. Assess likelihood of this G Oxygen and fluid resuscitation as problem before inserting any chest indicated drain G Analgesia G Small effusions (<2 cm deep) which are not enlarging or compromising respiratory function do not need to be drained G Early active treatment reduces length of illness Antibiotic therapy

Type of effusion suspected Choice of antibiotics Effusion following community-acquired Co-amoxiclav IV + clindamycin IV or oral pneumonia Effusion following hospital-acquired Piperacillin/tazobactam pneumonia or in immune-compromised child Effusion possibly tuberculous Discuss with TB team

G Narrow antibiotic spectrum with Chest drain management culture results Chest drain insertion G Ensure nursing staff trained in care of children with chest drains G Drain inserted by experienced team G Attach chest drain to low level suction G Discuss with respiratory team, (5–10 cm H 2O) via underwater seal consultant paediatrician, paediatric G If altitude chest drainage system anaesthetic team (usually GA used) used, set wall suction to G support may also be required from 160 mmHg/22 kPa and set dial cardiothoracic team +/- interventional on drainage system to 20 radiologist G Keep underwater seal below level of G Consider simultaneous insertion of chest at all times long line during general anaesthetic, if G After 10 mL/kg has been drained, possible clamp chest drain for 1 hr G Ensure vascular access before G Never clamp a bubbling chest drain starting procedure – this indicates presence of G CXR after drain insertion pneumothorax G If clamped and chest pain or breathlessness, unclamp immediately G Ensure adequate analgesia (see Analgesia guideline) and encourage patient to move freely when well enough

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Intrapleural fibrinolytics G Continue IV antibiotics at least until afebrile. Change to oral co-amoxiclav G Indicated if thick fluid with loculations when clinical improvement obvious. or pus Complete minimum 14 days antibiotics G Instill urokinase in all patients, as G Continue antibiotics until CRP <10 follows: G Encourage early mobilisation and G ≥10 kg, urokinase 40,000 units in exercise 40 mL sodium chloride 0.9% Non-resolution G <10 kg, urokinase 10,000 units in 10 mL sodium chloride 0.9% G Non-resolution of effusion after 3 days G administer via chest drain 12-hrly for or further complications occur, 3 days (total 6 doses) consider CT scan of chest G clamp chest drain for 4 hr after G If no fluid draining, check for instillation of urokinase, then drain for obstruction by flushing 8 hr G If drain can not be unblocked, remove G Record fluid volumes into and out of and replace if significant effusion pleural space carefully and accurately remains G Discuss referral for thoracotomy with SUBSEQUENT MANAGEMENT respiratory paediatrician Act on response to treatment and Surgery clinical assessment of patient G Monitor symptoms and re-examine G Discuss with paediatric thoracic patient to assess progress surgeon if: G Repeat CRP as needed G effusion has not resolved G if falling rapidly, continue with current G child is still septic regimen DISCHARGE AND FOLLOW-UP G if not falling after 72 hr, treat as non- resolution (see below) G Arrange review by respiratory G Chase pleural fluid aspirate results paediatrician, initial appointment 6 weeks after discharge (CXR on G if unexpected organisms grown, arrival) adjust antibiotic therapy with antibiotic sensitivities G if symptoms persist or recur, early referral to respiratory paediatrician G if differential cell count shows lymphocytosis, discuss with TB team, send aspirate for cytology and consider CT scan of chest G Chase blood and sputum culture results – if no growth, continue empirical treatment until patient improves G Remove chest drain when drainage minimal and in agreement with respiratory paediatrician: appose skin with Steristrips ® rather than sutures

Issue 5 Issued: May 2013 58 Expires: May 2014 PNEUMOTHORAX • 1/2 RECOGNITION AND Spontaneous pneumothorax ASSESSMENT G Sudden onset, occasionally at rest Symptoms and signs G Chest pain (unilateral) G Dyspnoea Tension pneumothorax G Resonance on percussion, with (very rare) reduced vocal fremitus and breath sounds (if moderate-large) G Severe dyspnoea Investigations G Circulatory compromise G Trachea +/- apex beat displaced G PA chest X-ray Treat immediately G If findings are unclear on PA, lateral (if possible, decubitus) film may help G Give oxygen 15 L/min with mask with G If findings obscured by surgical reservoir bag emphysema or complex bulla G Insert a large bore cannula of at least disease, CT scan may help 4.5 cm in length into 2 nd anterior intercostal space, midclavicular line BEWARE: suspected basal G Insert intercostal tube pneumothorax usually implies a G Remove emergency cannula when bulla. CT scan will differentiate bubbling in underwater seal system bullae from pneumothorax confirms intercostal tube system functioning

IMMEDIATE TREATMENT

Chest X-ray

Small collapse Large collapse Rim of air <2 cm Rim of air ≥2 cm

Yes No Significant dyspnoea Aspirate Chronic lung disease Successful? No No (asymptomatic) Intercostal tube drainage Yes

Observe for 4 hr No Yes In-patient Chronic lung disease Follow-up observation

Issue 5 Issued: May 2013 Expires: May 2014 59 PNEUMOTHORAX • 2/2

Management of intercostal drains

1 Chest X-ray next morning

Yes Re-expanded? No

Still bubbling? Yes Yes Still bubbling or swinging, or surgical emphysema? No No Wait 24 hr

If no bubbling remove drain 2 Check drain and underwater seal 3 Repeat X-ray

Collapsed again? Yes

No

Follow-up 4 Respiratory opinion 5

G 4: Follow-up: Do not clamp chest tube unless G at clinic in 7–10 days advised by respiratory G patient given discharge letter and paediatrician or thoracic surgeon. written advice to return immediately if If clamped and chest pain or deteriorates breathless unclamp immediately G no air travel until chest X-ray resolved G 1: Chest X-ray 5: Respiratory paediatrician’s G G keep underwater seal below level of opinion: chest at all times G if no re-expansion consider air leak, displaced/blocked tube, G 2: Removal of chest drain: bronchopleural fistula, underlying G bubbling stopped for at least 24 hr pulmonary disease G cut drain-securing suture G use of high volume/low pressure G withdraw tube while patient holds suction, 1–2 kPa/Barr, (8–16 mmHg; breath in expiration 8–20 cm H 2O) G close wound with remaining sutures G if altitude chest drainage system used, set wall suction to 160 mmHg/22 kPa G 3: Check drain: and set dial on drainage system to 20 G if lung not re-inflated and no bubbling G early thoracic surgery. Refer when in underwater bottle: Try to remove pneumothorax fails to resolve after block or kink 5 days of above management or after G if unsuccessful, remove drain. Insert 3 days if patient has chronic lung new drain through clean incision disease

Issue 5 Issued: May 2013 60 Expires: May 2014 CYANOTIC CONGENITAL HEART DISEASE • 1/2

RECOGNITION AND G tricuspid atresia ASSESSMENT G severe Fallot’s tetralogy G transposition of the great arteries Symptoms and signs without septal defect G single ventricle anatomy G Central cyanosis may be respiratory or cardiac in origin Acute pulmonary outflow G Respiratory illness producing obstruction (cyanotic episodes) cyanosis will usually have signs of G Fallot’s tetralogy or other complex respiratory distress (e.g. cough, congenital cyanotic heart disease tachypnoea, recession and added G severe pallor respiratory sounds) G loss of consciousness G Cardiac decompensation may occur with a respiratory infection: they may G convulsions co-exist Physical examination G Cyanosis more likely due to cardiac disease if: G Remember to check femoral pulses G G SpO 2 responds poorly to high flow If coarctation of the aorta suspected: oxygen (15 L/min) via face mask and check BP in upper and lower limbs – reservoir bag normal difference <15 mmHg G marked tachycardia Investigations G enlarged heart (clinically or on CXR) G gallop rhythm/murmur If infant cyanosed or in heart G enlarged liver/raised JVP failure, discuss urgency of G basal crackles investigations with consultant G absent femoral pulses G finger clubbing occurs after a few SpO 2 months (also consider endocarditis) G Check pre- (right arm) and post-ductal Causes of cardiac cyanosis (lower limbs) G when breathing air before oxygen Significant right-to-left shunt given G after giving 15 L/min oxygen by mask G Transposition with inadequate mixing, with a reservoir bag for 10 min pulmonary or tricuspid atresia G Fallot’s tetralogy: hypercyanotic Chest X-ray episodes follow emotional or painful G upset For cardiac conditions, specifically record: Duct-dependent pulmonary G cardiac situs (normal or right side of circulation chest) G G Commonly presents in first 10–14 aortic arch left or right-sided days of life G bronchial situs (is right main bronchus G severely blue, breathless or shocked on the right?) G G pulmonary atresia cardiac size and configuration G G critical pulmonary valve stenosis size of pulmonary vessels and pulmonary vascular markings

Issue 5 Issued: May 2013 Expires: May 2014 61 CYANOTIC CONGENITAL HEART DISEASE • 2/2

Electrocardiogram Acute pulmonary outflow obstruction (cyanotic episodes) See ECG interpretation guideline. G Nitrogen washout in cyanosed Immediate treatment before transfer babies to a paediatric cardiac centre: G do not upset child G G Monitor SpO 2 in air then in headbox give morphine 50–100 microgram/kg after breathing 100% oxygen for 10 min IV over 5 min or IM G in cyanotic congenital heart disease, G provide high concentration face mask oxygen (15 L/min with reservoir bag) PaO 2 will remain below 20 kPa with G SpO 2 unchanged if Fallot’s tetralogy has been diagnosed by echocardiography, G not as reliable as echocardiogram discuss with cardiologist use of IV Echocardiogram beta-blocker SUBSEQUENT MANAGEMENT G Locally, if available, or refer to regional paediatric cardiac centre G On advice of consultant and IMMEDIATE TREATMENT paediatric cardiac centre

If infant cyanosed or in heart failure, discuss urgency of referral to local paediatric cardiac surgical centre with consultant

Duct-dependent congenital heart disease

G Immediate treatment before transfer to a paediatric cardiac centre: G open duct with prostaglandin E1 (alprostadil) or E2 (dinoprostone) same dose: G 5–10 nanogram/kg/min IV infusion to start G increasing in steps of 5–10 nanogram/kg up to max 100 nanogram/kg/min G then reducing to lowest dose needed G May cause apnoea and patients may need ventilation G Beware of giving high concentrations of oxygen as this encourages duct closure

Issue 5 Issued: May 2013 62 Expires: May 2014 HEART FAILURE AND WEAK PULSES • 1/2

CAUSES Recognition of cardiogenic shock G Congenital heart malformations G G aortic stenosis For definition of shock see Septicaemia guideline G coarctation of the aorta G Following cardiopulmonary G hypoplastic left heart resuscitation with adequate fluid G Cardiomyopathies replacement in patients with: G Pericardial effusion G septic shock that fails to improve after adequate fluid replacement (e.g. G Myocarditis ≥40 mL/kg) G Arrhythmias G a known heart condition and shock G Hypoxia G a large heart on chest X-ray but G Hypovolaemia previously well G Acidosis G shock, who have a history of poisoning G Toxins G a murmur or pulmonary oedema, or both RECOGNITION AND ASSESSMENT INVESTIGATIONS G Presentation Check BP in upper and lower limbs (normal <15 mmHg difference)

G Usually during first few weeks of life SpO 2 G Later triggered by an intercurrent infection, with associated myocarditis G Check pre- (right arm) and post-ductal or prolonged arrhythmia (lower limbs) G In air and after giving oxygen Symptoms and signs Chest X-ray G Failure to thrive G For cardiac conditions, specifically G Rapid weight gain record: G Sweating G cardiac situs (normal or right side of G Breathlessness, particularly during chest) feeding G aortic arch left- or right-sided G Tachypnoea G bronchial situs (is right main bronchus G Tachycardia on the right?) G Absent or low volume peripheral or G cardiac size and configuration central pulses G size of pulmonary vessels and G Enlarged heart pulmonary vascular markings G Prominent cardiac impulses Electrocardiogram G Quiet heart sounds in pericardial ECG interpretation effusion G See guideline G Thrill Echocardiogram G Gallop rhythm G Locally, if available, or refer to local G Enlarged liver paediatric cardiac centre

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MONITORING If cardiogenic shock present 1. Monitor CVP and ensure adequate G ECG monitor pre-load: give Human Albumin G Non-invasive BP Solution (HAS) 4.5% 10 mL/kg as IV bolus or, if HAS not available, G Pulse oximetry sodium chloride 0.9% 10 mL/kg as IV G Core-skin temperature difference bolus G Daily weights 2. If shock severe (see Septicaemia G Urine output ( ≥1 mL/kg/hr) guideline), start mechanical ventilation with positive end-expiratory pressure G If shocked or ≥40 mL/kg fluid early; if pulmonary oedema present, resuscitation: start urgently G intra-arterial BP monitoring 3. If shock severe, give early inotropic G CVP drug support: dopamine, dobutamine, THERAPEUTIC MEASURES adrenaline or noradrenaline as per NNU/PICU protocols In all children with heart failure DUCT-DEPENDENT 1. If breathless, elevate head and trunk CONGENITAL HEART DISEASE 2. If infant not feeding well, give G nasogastric feeds May present in first two weeks of life 3. In moderate-to-severe failure or if Duct-dependent systemic patient hypoxic or distressed, give circulation oxygen therapy via nasal cannulae (up to 2 L/min) or via a face mask G Breathless, grey, collapsed, poor with reservoir bag (up to 15 L/min) pulses 4. Diuretics: furosemide 1 mg/kg oral or G severe coarctation of the aorta by slow IV injection (max 4 mg/min) G critical aortic stenosis and amiloride 100 microgram/kg G hypoplastic left heart syndrome (max 10 mg) oral 12-hrly (doses can be repeated if not responding to Duct-dependent pulmonary initial dose) circulation 5. If serum potassium <4.5 mmol/L, give additional potassium chloride G Blue, breathless or shocked 1 mmol/kg 12-hrly enterally G pulmonary atresia 6. Correct acidosis, hypoglycaemia and G critical pulmonary valve stenosis electrolyte imbalance G tricuspid atresia 7. Relieve pain with morphine: loading G severe Fallot’s tetralogy dose 100 microgram/kg IV over 5 min, followed by 50 microgram/kg G transposition of the great arteries IV 4–6 hrly over 5 min or Treatment 10 microgram/kg/hr via IV infusion (doses can be doubled if necessary) G See Cyanotic congenital heart 8. If anaemic (Hb <100 g/L), correct with disease guideline infusion of packed cells over 4 hr to bring Hb to 120–140 g/L

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G All ECGs, check: Right atrial hypertrophy (RAH) G P-wave size and axis G axis of QRS complex G Increased P wave amplitude in leads G R-S pattern in chest leads II, V1, and V4R G P-R, QRS and Q-T intervals Causes G P- and T-wave configuration G size of QRS in chest leads G Pulmonary hypertension PAPER SPEED G Pulmonary stenosis G Pulmonary atresia G ECG normally recorded at 25 cm/sec G Tricuspid atresia G 1 mm (1 small square) = 0.04 sec Left atrial hypertrophy (LAH) G 5 mm (1 large square) = 0.2 sec P WAVE G Biphasic P wave (later depolarization of LA) G Reflects atrial activity G Duration shorter than in adults Causes G infants: 0.04–0.07 sec G Mitral valve disease G adolescents: 0.06–0.1 sec G LV obstruction and disease G Height ≤2.5 mm G Varying P wave morphology may indicate wandering atrial pacemaker P-R INTERVAL

G Atrial depolarization varies with age and rate Normal range of P-R interval (time in sec) Heart rate P-R interval (sec) 0–1 month 0–12 months 1–12 yr 12–16 yr <60 - - - 0.1–0.19 60–99 - - 0.1–0.16 0.1–0.17 100–139 0.08–0.11 0.08–0.12 0.1–0.14 - 140–180 0.08–0.11 0.08–0.12 0.1–0.14 - >180 0.08–0.09 0.08–0.11 - - Prolonged interval Variable interval

G Normal G Wandering atrial pacemaker G Wenckebach phenomenon G Myocarditis G Ischaemia QRS COMPLEX G Drugs G Ventricular activity G Hyperkalaemia G Duration: 0.06–0.08 sec Short interval Prolonged G Ventricular hypertrophy G Wolff-Parkinson-White syndrome G Bundle branch block G Lown-Ganong-Levine syndrome G Electrolyte disturbance G Glycogen storage disease G Metabolic disease G Drugs (e.g. digoxin)

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Normal range of R and S waves (height in mm) Age R and S waves (height in mm) V4-R V1-R V1-S V5-R V6-R V6-S Birth 4–12 5–20 0–20 2–20 1–13 0–15 6–12 months 2–7 3–17 1–25 10–28 5–25 0–10 1–10 yr 0–7 2–16 1–12 5–30 5–25 0–7 >10 yr 0–6 1–12 1–25 5–40 5–30 0–5

Q WAVE Short interval

G Normal in II; III; aVF; V5-6 G Hypercalcaemia G Depth 2–3 mm G Digitalis effect G pathological if >4 mm (i.e. septal T WAVE hypertrophy) G May be found in other leads in: G Ventricular repolarization G anomalous coronary arteries Normal G hypertrophic obstructive cardiomyopathy G T inversion V4R/V1 (from third day of G transposition of great arteries (with life until 10 yr) opposite polarity) G Amplitude is 25–30% of R-wave Q-T INTERVAL G Aged <1 yr: V5 ≤11 mm; V6 ≤7 mm G Aged >1 yr: V5 ≤14 mm; V6 ≤9 mm Inversely proportional to rate G Adolescence reduces amplitude G Calculate ratio of Q-T interval to R-R interval Peaked T wave G QTc = Q-T G Hyperkalaemia R-R 1 G LVH G QTc is usually less than 0.44 s G Cerebrovascular episode G prolonged QTc is associated with G sudden death: alert consultant Post-MI immediately Flat T wave Prolonged interval G Normal newborn G Hypocalcaemia G Hypothyroidism G Myocarditis G Hypokalaemia G Jervell-Lange-Nielsen syndrome G Hyper/hypoglycaemia G Romano-Ward syndrome G Hypocalcaemia G Head injuries or cerebrovascular G Peri/myocarditis episodes G Ischaemia G Diffuse myocardial disease G Digoxin effect G Antiarrhythmics

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MEAN QRS AXIS Causes include

Vertical plane (limb leads) G Aortic stenosis G Aortic regurgitation Normal axis in vertical plane G Hypertension G Moderate VSD G Birth +60° to +180° (av +135°) G Hypertrophic obstructive G Aged 1 yr +10° to +100° (av +60°) cardiomyopathy G Aged 10 yr +30° to +90° (av +65°) G Patent ductus arteriosus Right axis deviation G Mitral regurgitation RIGHT VENTRICULAR G Right ventricular hypertrophy (RVH) HYPERTROPHY G Left posterior hemiblock G Ostium secundum atrial septal defect Diagnosis (ASD)/right bundle branch block (RBBB) G RAD and RV1 > SV1 (aged >1 yr) G SV6 above maximum for age: Left axis deviation G 0–6 months 15 mm G Left ventricular hypertrophy (LVH) G >6 months 10 mm G Ostium primum ASD (+ RBBB) G >12 months 7 mm G Often in conduction defects G 10 yr 5 mm G R waves in V4R/V1 >normal Horizontal plane G T wave changes (anterior chest leads) G upright in V1/V4R (aged from 3 days to 10 yr) Normal Causes include G Transition at around V3 G Pulmonary stenosis/atresia Clockwise rotation G Transposition of great arteries G S>R in V4 = RA/RV hypertrophy G Pulmonary regurgitation G Total anomalous pulmonary drainage Anticlockwise rotation G Tricuspid regurgitation G R>S in V2 = cardiac shift (e.g. G Fallot's tetralogy pneumothorax) G Pulmonary hypertension LEFT VENTRICULAR BIVENTRICULAR HYPERTROPHY HYPERTROPHY

Diagnosis Diagnosis

G SV1 + RV5 ≥40 mm (30 mm aged G R + S >50 mm in V3-V4 <1 yr) G LVH + bifid R <8 mm in V1 G +/- prolonged QRS G RVH + LV strain G Flat T wave G Q waves V3-V6 imply septal G T wave inversion V5-V6 (LV strain) hypertrophy G Left bundle branch block

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TYPICAL ECG ABNORMALITIES

Heart lesion ECG abnormalities PDA LVH > RVH; LAH VSD LVH > RVH; +/- RBBB; T inv LV. leads ASD Secundum RAD; RBBB; +/- increased P-R; AF Primum LAD; RBBB; BVH; RAH Eisenmenger’s RVH; P pulmonale Aortic stenosis LVH + strain Aortic regurgitation LVH Coarctation Newborn: RVH Older: Normal or LVH +/- strain; RBBB Mitral regurgitation LVH Pulmonary stenosis RVH; RAH Ebstein’s anomaly Prolonged P-R interval; gross RAH; RBBB Fallot's tetralogy Newborn: Normal or T +ve V1 Older: RVH; RAH Pulmonary atresia RAH Tricuspid atresia LAD; RAH; LVH

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SUPRAVENTRICULAR G Failure to respond to adenosine can TACHYCARDIA be used to distinguish origin of a tachycardia in a stable patient

Early diagnosis and effective Causes of tachyarrhythmias management of supraventricular G Re-entrant congenital conduction tachycardia (SVT) are vital as pathway abnormality (common) there is a small risk of mortality G Poisoning RECOGNITION AND G Metabolic disturbance ASSESSMENT G After cardiac surgery G Cardiomyopathy Symptoms and signs G Long QT syndrome G Recurrent condition ECG DIAGNOSIS G family may identify as ‘another attack’ G Infants Infants G gradual onset of increasing G tachypnoea Majority have a P wave before every QRS complex, usually by >70 msec G poor feeding (2 mm at 25 mm/sec) G pallor G QRS complexes are generally normal G occasionally more dramatic but may be wide presentation with a rapid onset of G Accessory pathway frequently severe cardiac failure capable of anterograde as well as G Toddlers retrograde conduction G recurrent episodes of breathlessness, G this will be revealed during normal cold sweats and pallor sinus rhythm by short P-R interval G Older children and presence of a delta wave (classic Wolff-Parkinson-White syndrome) G recurrent palpitations, episodes of dizziness and pallor Older children Investigations G Nodal tachycardias become more common with increasing age G Confirm diagnosis with 12-lead ECG G characterised by fast, regular, narrow G Continuous ECG monitoring is QRS complexes without visible P essential waves G Assess for cardiac failure G Wide QRS complex or bundle branch Differential diagnosis block in childhood is rare G changes also present in sinus rhythm G Sinus tachycardia, particularly in G infants, can be >200/min. However, review previous ECGs rates of 220–300/min are more likely to be SVT If in doubt, seek more experienced help G If first presentation, check for any other cause of cardiac failure

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IMMEDIATE TREATMENT Other drugs

G Resuscitate (ABC) first G If adenosine ineffective, seek advice G If first presentation, refer to consultant from a paediatric cardiologist G See following Algorithms G In refractory Wolff-Parkinson-White tachycardia, flecainide is particularly Vagal manoeuvres useful G In refractory atrial tachycardia, These may include: amiodarone is useful G Diving reflex G wrap infants in a towel and immerse Do not use verapamil and their whole face into iced water for propranolol in same patient, as about 5–10 sec both have negative inotropic G in children, place a bag or rubber effects. Do not use verapamil in glove containing iced water over face children aged <1 yr G One side carotid massage G Valsalva manoeuvre G Where possible, maintain ECG monitoring and recording during all procedures

Do NOT use eyeball pressure because of risk of ocular damage

Adenosine

G Drug of choice as it has a rapid onset of action and not negatively inotropic G Very short half-life (10–15 sec) giving short-lived side-effects (flushing, nausea, dyspnoea, chest tightness) G Effective in >80% of junctional tachycardias and will not precipitate ventricular tachycardias into ventricular fibrillation G Can be used in broad-complex tachycardia of uncertain origin G Must be given as a rapid bolus IV via a large peripheral or central vein and followed by sodium chloride 0.9% flush G In patients with sinus tachycardia, heart rate will slow to bradycardia but will rapidly increase again

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Supraventricular tachycardia

Shock present Yes No

Vagal manoeuvres (if no delay) Vagal manoeuvres

Establish vascular Yes Adenosine access if quicker G aged <1 yr: 150 microgram/kg than obtaining defib G aged 1–12 yr: 100 microgram/kg G aged >12 yr: 3 mg No

Synchronous DC shock Repeat adenosine every 1–2 min 1 J/kg aged <12 yr increasing doses by 50–100 microgram/kg aged >12 yr 6 mg, then 12 mg Synchronous DC 2 J/kg shock

Max dose adenosine aged <1 month: 300 microgram/kg Amiodarone aged >1 yr: 500 microgram/kg max 12 mg

G Adenosine may be used in preference G Discuss with cardiologist to electrical shock G Consider: G An anaesthetic must be given for DC G synchronous DC shock shock if patient responsive to pain G other anti-arrhythmics (seek advice)

WIDE COMPLEX TACHYCARDIA Causes

RECOGNITION AND G Underlying cause (e.g. myocarditis, ASSESSMENT cardiomyopathy, or patient with congenital heart disease) G Definition Poisoning (e.g. phenothiazines, tricyclic antidepressants, quinidine and procainamide) G Ventricular tachycardia G Electrolyte disturbance (e.g. G ≥3 successive ectopic ventricular hypokalaemia, hypomagnesaemia) beats G Ventricular tachycardia can G sustained if it continues >30 sec degenerate into ventricular fibrillation

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Diagnosis G Seek advice G Ventricular tachycardia not always G Wide-QRS SVT (SVT with aberrant obvious on ECG, clues are: conduction) is uncommon in infants G rate varies between 120 and and children. Correct diagnosis and 250 beats/min (rarely 300 beats/min) differentiation from VT depends on G careful analysis of at least a 12-lead QRS complexes are almost regular ECG +/- an oesophageal lead though wide G G Assess patient and obtain family QRS axis abnormal for age (normal history to identify presence of an for aged >6 months is <+90º) underlying condition predisposing to G no preceding P wave, or A-V stable ventricular tachycardia dissociation G SVT or VT can cause haemodynamic G fusion beats (normally conducted instability: response to adenosine can QRS complex merges with an help identify underlying aetiology of abnormal discharge) the arrhythmia, but adenosine should be used with extreme caution in haemodynamically stable children with wide-complex tachycardia because of the risk of acceleration of tachycardia and significant hypotension. This should not delay definitive treatment in children with shock

IMMEDIATE TREATMENT

Ventricular tachycardia

No VF protocol Pulse present

An anaesthetic must be Yes given for DC shock if patient responsive to pain

Hypotensive Yes No

Amiodarone Synchronous DC shock 5 mg/kg (max 300 mg) over 30 min 1 J/kg

Consider: G synchronous DC shock Synchronous DC shock G seek advice 2 J/kg

Amiodarone

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G Treatment of haemodynamically BRADYARRHYTHMIAS stable child with ventricular tachycardia should always include G Urgently manage: early consultation with a paediatric G cardiologist. They may suggest pre-terminal event in hypoxia or amiodarone: can cause hypotension, shock which should be treated with volume G raised intracranial pressure expansion G vagal stimulation G Use synchronous shocks initially, as Investigations these are less likely than an asynchronous shock to produce G ECG to look for: ventricular fibrillation. If synchronous shocks are ineffectual, and child is G conduction pathway damage after profoundly hypotensive, subsequent cardiac surgery attempts will have to be G congenital heart block (rare) asynchronous G long QT syndrome G Treatment of torsade de pointes ventricular tachycardia is magnesium Management sulphate 25–50 mg/kg (up to 2 g) G diluted to 100 mg/mL in sodium ABC approach: ensure adequate chloride 0.9% over 10–15 min. Can oxygenation and ventilation be repeated once if necessary G If vagal stimulation is cause, give G Amiodarone 5 mg/kg (max 300 mg) atropine 20 microgram/kg (min may be given over 3 min in ventricular 100 microgram; max tachycardia if child in severe shock 600 microgram) G Consider IV isoprenaline infusion G Contact paediatric cardiologist for advice G fax ECG to cardiologist

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MANAGEMENT Endocarditis prophylaxis is not recommended unless undergoing G Patients at risk of endocarditis should gastro-intestinal or genito-urinary be: tract procedure at a site where G advised to maintain good oral hygiene infection is suspected G told how to recognise signs of infective endocarditis, and advised INDICATIONS when to seek expert advice G Investigate promptly any infection in Cardiac risk factors patients at risk of endocarditis and treat appropriately to reduce the risk G Acquired valvular heart disease with of endocarditis stenosis or regurgitation G If patients at risk of endocarditis are G Valve replacement undergoing a gastro-intestinal or G Structural congenital heart disease, genito-urinary tract procedure at a including surgically corrected or site where infection suspected seek palliated structural conditions, but senior advice excluding isolated atrial septal defect, fully repaired ventricular septal defect or fully repaired patent ductus arteriosus, and closure devices judged to be endothelialised G Previous infective endocarditis G Hypertrophic cardiomyopathy

If there is uncertainty, seek advice from cardiology team at local paediatric cardiac surgical centre

Issue 5 Issued: May 2013 74 Expires: May 2014 POISONING AND DRUG OVERDOSE • 1/3 The poisoned Poison(s)/drug(s) information

G Toddlers (accidental poisoning) G Ask patient, relatives, GP, ambulance crew. Retain any containers found G Older children, particularly girls (intentional self-poisoning) G if identification doubtful, send parents home for poison The poisoners G Ask about visitors to the house/visits to other houses (e.g. grandparents) G Most childhood poisonings are accidental G Quantity ingested: Difficult to quantify but parents may know how full a G Intentional poisoning may be by the bottle should have been child or an adult G assume child has ingested something G Inadvertent poisoning may occur in a even if found with a few tablets and medical setting an empty bottle The poison G Time of ingestion, including multiple doses G Children will eat and drink almost G Other possible poisons/drugs taken anything Investigations RECOGNITION AND ASSESSMENT G U&E G Blood gases and acid-base Symptoms and signs G Save blood and urine for toxicological analysis. Urgent measurement of G Depressed respiration suggests plasma/serum concentrations essential centrally-acting drug in diagnosis and management of G Skin blisters (between knees/toes) poisoning with ethylene glycol, iron, common after barbiturates and lithium, methanol, paracetamol, tricyclics theophylline and salicylate. With exception of paracetamol, no need to G Hypothermia after exposure or measure concentrations of these barbiturates substances unless clear history of G Venepuncture marks and pinpoint ingestion. Specify which drugs pupils suggest opioid overdose suspected or urine for ‘drugs of abuse’ G Burns around mouth Request plasma paracetamol Life-threatening features concentration in all unconscious patients in whom drug overdose G Coma considered G Cyanosis Seek advice G Hypotension G Paralytic ileus G Use Toxbase: www.toxbase.org access and password available in A&E G if further information required, contact National Poisons Information Service (0844 892 0111)

Issue 5 Issued: May 2013 Expires: May 2014 75 POISONING AND DRUG OVERDOSE • 2/3 G Activated charcoal in patients who Always admit a child who have ingested life-threatening amounts is symptomatic or who has of a toxic agent up to 1 hr previously, ingested iron, digoxin, aspirin or provided patient conscious or airway a tricyclic antidepressant. can be protected. Give 1 g/kg (max If child not admitted always 50 g) oral (disguised with soft consult on-call paediatric SpR drink/fruit juice) or via nasogastric tube. before sending home Activated charcoal does not affect absorption of acids, alkalis, alcohols, IMMEDIATE TREATMENT cyanide, ethylene glycol, iron or lithium G Do not give ipecacuanha, it does not Separate guidelines give more empty the stomach reliably and can detailed advice on management be dangerous of overdose with alcohol, iron, G Stop any regular medication that paracetamol, phenothiazines, might enhance effect of substance salicylates and tricyclic taken in overdose antidepressants SUBSEQUENT MANAGEMENT Assess airway, breathing and G If unconscious, admit to a high- circulation dependency nursing area and attach an ECG monitor G Maintain airway G Supportive care alone required for G if airway not protected, may need majority of acutely poisoned patients intubation and ventilation G If deliberate self harm, refer to G if cyanosed or rate and depth of CAMHS – see Self harm guideline respiration obviously low, arterial blood gases indicated MONITORING TREATMENT G if PaCO high or rising, mechanical 2 G ventilation indicated Monitor conscious level, temperature, respiration, pulse and BP until these G Correct hypotension return to normal G raise foot of bed G No need to monitor drug concentrations G if in haemodynamic shock, give IV other than to guide use of measures to bolus of sodium chloride 0.9% enhance drug elimination (20 mL/kg over 10 min). Assess and G If unconscious, make full head injury repeat if still in shock observations G consider need for central venous G Record pulse, respiratory rate, BP, pressure (CVP) monitoring pupil size and reaction, and level of Neurological consciousness hourly for at least 4 hr then increase interval if stable G Control convulsions PSYCHIATRIC REVIEW G if unconscious, treat as head injury until proved otherwise G Offer all patients admitted after deliberate acute self-poisoning or Drug absorption drug overdose an interview with the psychiatric priority referral team within G Give antidote if appropriate 24 hr of admission or regaining consciousness

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DISCHARGE AND FOLLOW-UP

G When discharged from hospital patients should have: G been conscious and alert with normal vital signs for at least 6 hr G no evidence of significant organ dysfunction as a result of poisoning/drug toxicity G been interviewed by a member of the psychiatric priority referral team where indicated G follow-up appointment in psychiatric clinic (if recommended by psychiatrist) G follow-up appointment in paediatric clinic (if persistent sequelae of poisoning require review)

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Refer all children with features of alcohol intoxication to hospital

RECOGNITION AND ASSESSMENT Symptoms and signs

Table 1: Assessment of alcohol poisoning Mild toxicity G Impaired visual acuity and co-ordination G Emotional lability Moderate toxicity G Slurred speech, diplopia, blurred vision, ataxia, lack of co-ordination, blackouts, sweating, tachycardia, nausea, vomiting, incontinence G Acidosis, hypoglycaemia, hypokalaemia Severe toxicity G Cold clammy skin, hypothermia, hypotension, stupor, coma, dilated pupils, depressed or absent tendon reflexes G Severe hypoglycaemia, convulsions, respiratory depression, metabolic acidosis G Cardiac arrhythmias (e.g. atrial fibrillation, atrio-ventricular block) Potentially fatal G Deep coma, respiratory depression or arrest, circulatory failure

Alcoholic drinks/preparations G check blood glucose hourly until consciousness regained G Spirits are particularly dangerous G Correct hypotension (see Poisoning and drug overdose G Alcopops guideline) G Perfumes, colognes and aftershaves G Correct acid-base and metabolic disturbance G Mouth washes (some) G Correct hypothermia using G Methylated spirit conventional means (e.g. Bair Hugger, G Hand gels blankets) G Detergents G Control convulsions with IV lorazepam G Fake vodka may contain propranolol G If blood ethanol >5 g/L (108.5 mmol/L) and methanol or if arterial pH <7.0, consider G Other drugs often taken too but not haemodialysis disclosed G discuss with National Poisons IMMEDIATE TREATMENT Information Service (0844 892 0111) Investigations G Ensure clear airway and adequate ventilation G Blood glucose G Gut decontamination is unlikely to be G In moderate to severe toxicity of benefit G U&E G activated charcoal does not G arterial blood gases significantly reduce rate of absorption G blood ethanol concentration G Correct hypoglycaemia as quickly as possible G toxicology blood and urine for drugs of abuse G if awake, give oral glucose G 12-lead ECG G if drowsy or unconscious, give glucose 10% 2 mL/kg IV

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Assessment of severity

G Blood ethanol is a guide to severity of poisoning G 0.2–1.0 g/L (4–22 mmol/L) mild toxicity G 1–2 g/L (22–43 mmol/L) moderate toxicity G >2–4 g/L (>43 mmol/L) severe toxicity and potentially fatal G Observe for at least 4 hr if >0.4 mL/kg body weight of absolute ethanol had been ingested (i.e. 1 mL/kg 40% spirit, 4 mL/kg 10% wine or 8 mL/kg 5% beer) G Monitor pulse, blood pressure and body temperature SUBSEQUENT MANAGEMENT

G See Poisoning and drug overdose guideline G Follow guidance on www.toxbase.org (password from A&E) G Further advice from National Poisons Information Service (0844 892 0111)

Issue 5 Issued: May 2013 Expires: May 2014 79 IRON POISONING • 1/2 RECOGNITION AND ASSESSMENT Symptoms and signs

Time Symptoms and signs <6 hr after ingestion G Nausea, vomiting, abdominal pain and diarrhoea G Vomit and stools often grey or black G Polymorph leucocytosis and hyperglycaemia suggest toxicity but their absence does not exclude it 6–12 hr after ingestion G Early features improve in mild cases G Possibly persistent hyperglycaemia/metabolic acidosis in more serious cases >12 hr after ingestion G In serious cases, evidence of hepatocellular necrosis appears with jaundice, bleeding, hypoglycaemia, encephalopathy and metabolic acidosis. Hypotension may occur 2–5 weeks after ingestion G Gastric stricture or pyloric stenosis may start to cause obstructive symptoms Investigations G Serum iron taken at 4 hr after ingestion is best laboratory measure G If ingested dose >20 mg/kg elemental G <3 mg/L (55 micromol/L) mild toxicity iron, measure serum iron 4 hr after G 3–5 mg/L (55–90 micromol/L) ingestion moderate toxicity G U&E, creatinine G >5 mg/L (90 micromol/L) severe toxicity G INR G Absence of visible tablets on X-ray G Blood glucose does not eliminate possibility of G If presenting within 2 hr of ingestion, ingestion request plain abdominal X-ray Action G tablets are sometimes visible in the stomach or small bowel G Discuss all cases with National G if patient could be pregnant, do NOT Poisons Information Service (NPIS) X-ray (0844 892 0111) for advice Assessment of severity IMMEDIATE TREATMENT

Review both clinical and Unconscious or in shock laboratory features G Assess airway, breathing, circulation G Estimate ingested dose of elemental G Secure airway, treat shock, control iron, BNFc lists quantity of elemental seizures iron in various preparations G IV fluids to replace losses G <20 mg/kg mild or no toxicity G Commence desferrioxamine IV (see G ≥20 mg/kg toxicity likely Desferrioxamine ) G >200 mg/kg severe toxicity, possibly G if this is before time when serum iron fatal should be taken (4 hr), take sample G Coma and shock indicate severe for serum iron immediately before poisoning: urgent treatment required commencing desferrioxamine G do not delay starting desferrioxamine IV

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G Monitor cardiac rhythm, BP and urine Desferrioxamine output G Check U&E, FBC, blood glucose, G Before starting treatment, contact LFTs, INR and arterial blood gases NPIS (0844 892 0111) for advice G Discuss whole bowel irrigation with G Starting dose is 15 mg/kg/hr NPIS if ingested dose >60 mg/kg G Reduce after 4–6 hr and/or tablets seen on X-ray and child presents within 1 hr G maximum 80 mg/kg in 24 hr G do not use activated charcoal as it G desferrioxamine commonly causes does not adsorb iron hypotension if infused more rapidly than recommended rate and turns Conscious and not in shock urine red/orange but rarely causes rashes or anaphylactic reactions G Check serum iron concentration at 4 hr SUBSEQUENT MANAGEMENT G Interpret serum iron concentration in G See Poisoning and drug overdose view of child’s clinical condition and guideline history G If slow release preparations ingested, Moderate poisoning: repeat serum iron after further 6–8 hr serum iron 3–5 mg/L G In patients with severe toxicity: G Repeat serum iron measurement after G arterial blood gases and correct further 2 hr, even if asymptomatic acidosis G If concentration falling, no further G If metabolic acidosis persists despite treatment required correction of hypoxia and adequate fluid resuscitation, give IV sodium G If concentration rising and child bicarbonate 1 mL/kg 8.4% symptomatic, give desferrioxamine IV bicarbonate diluted in glucose 5% or Desferrioxamine (see ) sodium chloride 0.9% 500 mL at Severe poisoning: 2–3 mL/kg/hr serum iron >5 mg/L G monitor renal and liver function G be alert for evidence of gut G If asymptomatic, repeat serum iron perforation or infarction after 2 hr: if concentration falling treatment unlikely to be required G Follow guidance on www.toxbase.org (password from A&E) G If symptomatic, give desferrioxamine IV (see Desferrioxamine ) G Further advice from NPIS (0844 892 0111)

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RECOGNITION AND Investigations ASSESSMENT G Plasma paracetamol 4–16 hr (but not Symptoms and signs outside this interval) is a reliable guide to the need for treatment after G Common: nausea and vomiting single overdose ingested of <60 min G Rare: coma and metabolic acidosis G If patient presents >8 hr after single overdose; or after staggered G Late: abdominal pain overdose; request baseline: Management for G FBC, INR G U&E, liver function, phosphate G Paracetamol dose >6 g or >75 mg/kg G acid-base (venous sample) G Staggered overdose [including chronic therapeutic excess IMMEDIATE TREATMENT >75 mg/kg/d (>60 mg/kg in neonate)] G Symptomatic G Compare plasma paracetamol with treatment graph ( Figure 1 ) OR G if above, or on, the ‘treatment line’, G INR >1.3 or ALT >upper limit of give IV acetylcysteine in glucose 5% normal, or abnormal acid/base or bicarbonate G Time interval is critical in assessing need for treatment. Detailed G Follow guidance on www.toxbase.org questioning essential (password from A&E) G Further advice from National Poisons If there is doubt about timing or Information Service (NPIS) 0844 892 need for treatment, treat 0111 G If there is absolute certainty that a single dose of paracetamol of <6 g and <75 mg/kg has been ingested, plasma paracetamol need not be measured and child requires no antidote Time from overdose (hr) Guidance on use of acetylcysteine Give activated charcoal 1 g/kg (max 50 g) oral or via nasogastric tube (gastric <1 lavage is not indicated) Await paracetamol level if available <8 hr from ingestion. Treat if level ≥ 4–7 ‘treatment line’ OR if biochemical tests (INR, ALT) suggest acute liver injury 8–14 Give at once while awaiting paracetamol concentration result. Cease if concentration well below appropriate ‘treatment line’ and ALT within normal limit, INR ≤1.3 15–24 Give at once. Cease at 24 hr after ingestion if patient asymptomatic, and INR ≤1.3, and ALT 24 Give if paracetamol still detectable in the blood (>5 mg/L), or INR >1.3 or ALT >twice upper limit of normal, or symptomatic. If patient has, or is at risk of developing, fulminant hepatic failure (see life- threatening features below), continue to give 50 mg/kg in 500 mL every 8 hr Discuss with NPIS and follow Toxbase guidance

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Acetylcysteine dosage (see BNFC*)

Weight <20 kg G First phase: 150 mg/kg IV in 3 mL/kg glucose 5% over 60 min, then (including G Second phase: 50 mg/kg IV in 7 mL/kg glucose 5% over 4 hr, then neonates) G Third phase: 100 mg/kg IV in 14 mL/kg glucose 5% over 16 hr G Then careful review at 16 hr, in case of need to continue third infusion phase Weight G First phase: 150 mg/kg IV in 100 mL glucose 5% over 60 min, then 20–40 kg G Second phase: 50 mg/kg IV in 250 mL glucose 5% over 4 hr, then G Third phase: 100 mg/kg IV in 500 mL glucose 5% over 16 hr G Then careful review at 16 hr, in case of need to continue third infusion phase Weight >40 kg G First phase: 150 mg/kg IV (max 16.5 g) in 200 mL glucose 5% over 1 hr, then (dose capped G Second phase: 50 mg/kg IV (max 5.5 g) in 500 mL glucose 5% over 4 hr, then at 110 kg G Third phase: 100 mg/kg IV (max 11.0 g) in 1000 mL glucose 5% over 16 hr body weight) G Then careful review at 16 hr, in case of need to continue third infusion phase * BNFC dose calculation and prescription method is simple to prescribe and give. Alternatively, the dosage calculation described in Toxbase yields the same drug dose but is prepared differently and prescribed by volumes

Alternative dosage chart for body weight >40 kg

G See Adult acetylcysteine dose and administration in the BNF

Adult acetylcysteine prescription (each ampoule = 200 mg/mL acetylcysteine) Regimen First infusion Second infusion Third infusion

Infusion fluid 200 mL glucose 5% or 500 mL glucose 5% or 1000 mL glucose 5% or sodium chloride 0.9% sodium chloride 0.9% sodium chloride 0.9%

Duration of Infusion 1 hr 4 hr 16 hr

Drug dose 150 mg/kg 50 mg/kg 100 mg/kg acetylcysteine acetylcysteine acetylcysteine Patient weight Ampoule Infusion Ampoule Infusion Ampoule Infusion volume rate volume rate volume rate

kg mL mL/hr mL mL/hr mL mL/hr

40–49 34 234 12 128 23 64

50–59 42 242 14 129 28 64

60–69 49 249 17 129 33 65

70–79 57 257 19 130 38 65

80–89 64 264 22 131 43 65

90–99 72 272 24 131 48 66

100–109 79 279 27 132 53 66

≥110 83 283 28 132 55 66

G If for any reason glucose 5% unsuitable, substitute sodium chloride 0.9%

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Prepare and check infusion bags carefully. Administration errors are common Acetylcysteine can cause a pseudo-allergic reaction (wheezing, flushing, hypotension) that is usually relieved by stopping infusion but occasionally chlorphenamine and hydrocortisone are required. Once reaction has subsided, recommence infusion at lower rate of 50 mg/kg/hr to complete the 150 mg/kg (max 16.5 g), then start the second phase infusion of 50 mg/kg (max 5.5 g) over 4 hr

MONITORING TREATMENT

G Severe liver damage in the context of paracetamol poisoning has been defined as a peak plasma ALT activity exceeding 1000 iu/L Time of presentation after overdose Monitoring/continued treatment Discharge policy (hr)

<8 G INR, AST/ALT, creatinine, G Discharge if INR ≤1.3, AST/ALT and bicarbonate 24 hr after overdose or plasma creatinine normal at 24 hr when antidote treatment complete after overdose, or after antidote G if INR >1.3 or creatinine raised, or treatment complete, with warning to patient acidotic, repeat third infusion return if vomiting or abdominal pain phase until INR <1.3 occur G recheck INR and U&E 12-hrly until clearly falling G Do NOT correct INR with vitamin K without prior discussion with tertiary liver unit, see below for management of life-threatening conditions including use of FFP 8–15 G INR, AST/ALT, creatinine, G If INR ≤1.3, AST/ALT and plasma bicarbonate and phosphate 24 hr creatinine normal: after overdose or when antidote G discharge asymptomatic patients treatment complete 12 hr after antidote treatment with G if INR >1.3 or creatinine raised, or warning to return if vomiting or patient acidotic, repeat third infusion abdominal pain occur phase until INR <1.3 G if INR >1.3 and rises, and/or plasma G recheck INR and U&E 12-hrly until creatinine raised after antidote clearly falling treatment, monitor and observe until G discuss with NPIS these indices are falling and INR <2.0

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Time of presentation Monitoring/continued treatment Discharge policy after overdose (hr)

≥16 G Observe for signs of G If INR ≤1.3, AST/ALT and plasma encephalopathy (mental confusion, creatinine normal: drowsiness, spatial disorientation, G discharge asymptomatic patients asterixis) 12 hr after end of antidote treatment G Urine output (maintain good flow †) with warning to return if vomiting or G Capillary blood glucose 4-hrly abdominal pain occur G Blood gases and acid-base daily G if INR >1.3 and rises, and/or plasma G INR, AST/ALT, creatinine, creatinine raised after antidote bicarbonate and phosphate 24 hr treatment, monitor and observe until after overdose or when antidote these indices are falling and INR treatment complete <2.0 G if INR >1.3 and rises, or creatinine G Patients presenting 24–36 hr after raised, or patient acidotic, repeat overdose can develop hepatic third infusion phase until INR <2.0 dysfunction after this time, even if G recheck INR and U&E 12-hrly until INR, ALT and creatinine normal at INR clearly falling and creatinine time of presentation: repeat these <10% higher than start value indices 12 hr later

Life-threatening features Psychiatric review

G A poor prognosis indicated by: G Offer all patients admitted after acute G INR >3.0 self-poisoning or deliberate drug G serum creatinine >200 µmol/L overdose an interview with a member G blood pH <7.3 of the psychiatric priority referral team within 24 hr of admission or regaining G signs of encephalopathy consciousness G If any of these features are present after overdose, seek advice from DISCHARGE AND FOLLOW-UP local tertiary liver unit G †insert urinary catheter to monitor G See Poisoning and drug overdose urine flow and rehydrate to maintain guideline urine output >2 mL/kg/hr or 100 mL/hr G Advise all patients to return to whichever is smaller hospital if vomiting or abdominal G if unresponsive to IV fluids, give pains develop or recur furosemide and consider low-dose dopamine G insert CVP line to monitor response to IV fluids only if INR normal G Patients with incipient or established hepatic failure may be candidates for liver transplantation G Treat haemorrhage with fresh frozen plasma G Hypophosphataemia usually occurs after paracetamol poisoning and correlates well with degree of hepatic damage

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Figure 1: Treatment graph for paracetamol overdose

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RECOGNITION AND G Correct hypotension (see Poisoning and drug overdose ASSESSMENT guideline) G Correct hypothermia using Symptoms and Signs conventional means (e.g. Bair Hugger, blankets) G Drowsiness G Correct acid-base and metabolic G Confusion disturbance with bicarbonate infusion (see Salicylate poisoning guideline) Common preparations G Control convulsions with IV lorazepam G Chlorpromazine G Levomepromazine Treatment of extrapyramidal side effects G Perphenazine G Prochlorperazine G Procyclidine orally G Promazine G in severe reactions give IV or IM G Trifluoperazine G subsequent oral doses may be G Metoclopramide needed for 2–3 days Extrapyramidal side effects G if procyclidine not available, give trihexyphenidyl hydrochloride or Not dose-related diazepam G Dystonia (e.g. oculogyric crises, MONITORING spasmodic torticollis) TREATMENT/SUBSEQUENT G Dyskinesia MANAGEMENT G Appear after only a few doses G See Poisoning and drug overdose Complications guideline G Follow guidance on www.toxbase.org G Convulsions (password from A&E) G Hypothermia G Further advice from National Poisons G Hypotension Information Service (0844 892 0111) G Arrhythmias (e.g. sinus tachycardia, QT and QRS prolongation, VT/VF, bundle branch/atrio-ventricular block) G Respiratory depression G Rhabdomyolysis G Renal failure IMMEDIATE TREATMENT

G If patient presents within 1 hr of ingesting a potentially toxic dose, give activated charcoal 1 g/kg (max 50 g) G Maintain clear airway and adequate ventilation

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RECOGNITION AND Investigations ASSESSMENT G U&E, creatinine Symptoms and signs G INR G Arterial blood gases Common features G Blood glucose (capillary) G G Vomiting In asymptomatic patients with a reliable history of ingestion of G Dehydration <125 mg/kg of aspirin, plasma G Tinnitus salicylate not required G Vertigo G In those who have ingested G Deafness >125 mg/kg, measure plasma G Sweating salicylate level G Warm extremities with bounding pulse G repeat after 2 hr: if rising, repeat G Increased respiratory rate levels every 3 hr until falling G Hyperventilation G If coincident paracetamol overdose, G Acid-base disturbance: check salicylate level before G aged >4 yr usually mixed respiratory administration of N-acetylcysteine alkalosis and metabolic acidosis with G Urine pH normal or high arterial pH Assessment of severity G aged <4 yr usually a dominant metabolic acidosis with low arterial pH G Severity cannot be assessed from Uncommon features plasma salicylate concentrations alone G Haematemesis G Neurological features (e.g. confusion G Hyperpyrexia and impaired consciousness), G Hypoglycaemia metabolic acidosis, and high G Hypokalaemia salicylate concentrations indicate severe poisoning G Thrombocytopenia G G Increased INR/PTR Risk factors for death include: G Intravascular coagulation G aged <10 yr G Renal failure G CNS features G Non-cardiac pulmonary oedema G acidosis G Confusion G hyperpyrexia G Disorientation G late presentation G Coma G pulmonary oedema G Convulsions G salicylate concentration >5.1 mmol/L Preparations IMMEDIATE TREATMENT G Aspirin tablets G If ingested >125 mg/kg salicylate G Methyl salicylate (Oil of Wintergreen), within previous hour, give oral very toxic activated charcoal 1 g/kg (maximum G Choline salicylate (dental gels) 50 g), mixed with soft drink/fruit juice G Numerous over-the-counter if necessary to disguise taste analgesics/antipyretics contain aspirin G Rehydrate orally (IV if vomiting)

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Interpretation of plasma G repeat salicylate levels and potassium salicylate concentrations level every 1–2 hr G Clinical presentation is most important Do not use volumes of IV fluids factor above maintenance requirements G Late presenting patient may have a (forced diuresis) – they do not subtoxic salicylate concentration, but increase salicylate elimination and serious acid-base or CNS disturbances can cause pulmonary oedema G If levels still rising, repeat oral activated charcoal Haemodialysis G Plasma salicylate <350 mg/L (2.5 mmol/L) and mild clinical effects: Use in patients with severe poisoning G continue maintenance management G Plasma concentrations >700 mg/L G Plasma salicylate 350–700 mg/L (5.0 mmol/L) (2.5–5.0 mmol/L) and moderate G Renal failure clinical effects: G Congestive cardiac failure G continue maintenance management G Non-cardiogenic pulmonary oedema and start alkaline diuresis in children G Convulsions aged <5 yr G CNS effects not resolved by G if plasma salicylate >500 mg/L correction of acidosis (3.6 mmol/L) in children aged ≥5 yr G Persistently high salicylate start alkaline diuresis concentrations unresponsive to G Plasma salicylate >700 mg/L urinary alkalinisation (5.0 mmol/L) and severe clinical effects G Severe metabolic acidosis G haemodialysis use G Children aged <10 yr who have an Children aged <10 yr have an increased risk of salicylate toxicity increased risk of salicylate toxicity MONITORING and may require haemodialysis at TREATMENT/SUBSEQUENT an earlier stage MANAGEMENT G See Poisoning and drug overdose Alkaline diuresis guideline G During alkaline diuresis, check U&E, G If serum potassium low, give blood glucose, acid-base hourly potassium chloride 1 mmol/kg oral G Repeat plasma salicylate 2-hrly until G or if not tolerated sodium chloride falling 0.45%/glucose 5% with 20 mmol G potassium in 500 mL at 100% if plasma salicylate continues to rise, maintenance consider a second dose of activated charcoal G If serum potassium within normal G range, alkalinise urine to enhance Continue therapy until patient salicylate excretion (optimum urine improving and plasma salicylate falling pH 7.5–8.5) G Follow guidance on www.toxbase.org G give sodium bicarbonate 8.4% (password from A&E) 1 mL/kg (1 mmol/kg) in 500 mL sodium G Further advice from National Poisons chloride 0.9% or glucose 5% at Information Service (0844 892 0111) 2–3 mL/kg/hr, and repeat if necessary to maintain urine pH 7.5–8.5

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RECOGNITION AND IMMEDIATE TREATMENT ASSESSMENT If a benzodiazepine has also been Symptoms and signs taken, do NOT give flumazenil

Early in poisoning G Correct any hypoxia G if PaCO 2 >6 kPa in respiratory failure G Anticholinergic effects (tachycardia, arrange assisted ventilation hot, dry skin, dry mouth and tongue, G If high dose (e.g. amitriptyline dilated pupils, urinary retention) >4 mg/kg) within previous hour, give G Ataxia, nystagmus activated charcoal 1 g/kg (max 50 g) G Drowsiness either oral (mixed with soft drink/fruit juice if necessary to disguise taste) G Metabolic acidosis or, if drowsy or unconscious, by G Hypokalaemia nasogastric tube (provided airway can Severe cases be protected) G Admit to HDU G Hypotension G Treat arrhythmias by correction of G Increased tone, hyperreflexia hypoxia and acidosis G Coma G sodium bicarbonate 8.4% diluted in an G Seizures equal volume of glucose 5% and give a ‘calculated’ dose: dose (in mmol) = G Respiratory depression desired change in base deficit G Cardiac arrhythmias (current-target) x 0.3 x weight (kg) to a maximum of 50 mmol (ideally via Preparations central vein). For rapid correction administer over 20 min, otherwise G Amitriptyline administer at a rate of 1 mmol/min. G Amoxapine Caution required if solution to be G Clomipramine given by peripheral venous line, as irritant to veins and can cause local G Dosulepin necrosis in cases of extravasation G Doxepin G if unresponsive discuss using lipid G Imipramine emulsion with National Poisons G Lofepramine Information Service (NPIS) G Nortriptyline (0844 892 0111) G Trimipramine G do not use arrythmics G consult local paediatric cardiac team Investigations G Hyperpyrexia (>39ºC): treat with ice G U&E bags and sedation: if persists give dantrolene, discuss with NPIS G Arterial blood gas G 12-lead ECG, large doses cause Prolonged resuscitation prolongation of P-R and QRS (up to 1 hr) may be successful intervals after cardiac arrest

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MONITORING TREATMENT

G See Poisoning and drug overdose guideline G Cardiac monitor for at least 6 hr G asymptomatic patients with normal ECG after 6 hr are unlikely to develop late complications SUBSEQUENT MANAGEMENT

G See Poisoning and drug overdose guideline G Follow guidance on www.toxbase.org (password from A&E) G Further advice from NPIS (0844 892 0111) G Consider second dose of charcoal after 2 hr if: G sustained-release formulation taken G CNS/respiratory depression G In severe cases, correct hypotension by raising foot of bed or, if necessary, expanding intravascular volume G Control convulsions with lorazepam IV G If patient hypothermic, rewarm slowly using conventional means (e.g. Bair Hugger, blankets) G Treat skin blisters as burns G monitor for rhabdomyolysis (look for coca-cola coloured urine testing positive for blood, measure creatine kinase) G Forced diuresis, haemodialysis or haemoperfusion are of no value G Agitation and visual and auditory hallucinations are common during recovery and may require treatment with high doses of diazepam

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Management of children with diabetes First on the afternoon list undergoing surgery and other procedures that require fasting G Advise usual doses of insulin evening PRINCIPLES before procedure G Advise child to have normal breakfast Reasons to control diabetes well in peri- no later than 0700 hr operative period G Breakfast insulin dose G To prevent hyperglycaemia and keto- acidosis, resulting from: G if using multiple daily injection (MDI) regimen, give usual breakfast insulin G omission of insulin G stress hormone response to surgery G if using twice daily insulin regimen give one-third of total morning dose as G catabolic state rapid-acting insulin (e.g. Novorapid ®) G To minimise risk of infection, enhanced by hyperglycaemia G Allow clear fluids until 3 hr before G To prevent hypoglycaemia, resulting operation from: G Measure and record capillary blood G starvation pre- and post-operatively glucose on arrival in theatre G anorexia post-operatively G Measure and record capillary blood glucose hourly once nil-by-mouth and Careful regular monitoring of half-hourly during operation blood glucose is required G throughout peri-operative period If any concerns (e.g. vomiting, prolonged operation), start IV fluids G Diabetic patients should preferably be and insulin as for Major surgery (see first on morning list below) G Give usual insulin evening before Post-operative care procedure G Recommend normal age-dependent G Monitor capillary blood glucose in Pre-op fasting fasting – see guideline recovery and then hourly for 4 hr If any concerns, contact diabetes G If well on return to ward and using team multiple daily injection (MDI) regimen G give dose of rapid-acting insulin MINOR SURGERY (able to eat appropriate for carbohydrate content within 4 hr of procedure) of next meal (if advice needed, contact diabetes team) and Pre-operative care G give next dose of long-acting insulin at usual time First on the morning list G If using twice daily premixed insulin G Advise usual doses of insulin on night regimen and able to eat by lunch on before procedure same day of procedure, give one-third G of total morning dose as rapid-acting On day of procedure omit insulin and ® breakfast insulin (e.g. Novorapid ) G Allow clear fluids, including sweet G with meal drinks, up to 0600 hr G teatime on same day of procedure, it G Measure and record capillary blood may be appropriate to give child’s glucose pre-operatively and usual insulin dose or a reduced dose: half-hourly during operation contact diabetes team for advice

Issue 5 Issued: May 2013 92 Expires: May 2014 DIABETES AND FASTING • 2/4 G If any concerns (e.g. vomiting or G Allow clear fluids until 3 hr before prolonged operation), start IV fluids operation Major surgery and insulin as for (see G Measure and record capillary blood below) glucose on arrival in theatre G Post- when child ready to eat, see G operative care Insert 2 IV cannulae if possible. These can be inserted in theatre, if necessary MAJOR SURGERY (unable to G Start a glucose and sliding scale eat within 4 hr of start of insulin infusion in theatre – see below procedure G Measure and record capillary blood glucose hourly once nil-by-mouth and Pre-operative care half-hourly during operation G Admit on day before surgery EMERGENCY SURGERY G Check pre-meal and bedtime capillary blood glucose measurements on ward Emergency procedures differ from First on the morning list elective ones as children run the risk of developing ketoacidosis if they are ill. G If using multiple daily injections (MDI), Prolonged starvation associated with give usual mealtime short-acting insulin delayed surgery poses additional but half usual dose of long-acting complications insulin evening before procedure Pre-operative care G If using twice-daily insulin regimen, give usual doses of insulin with meal G Inform diabetes team immediately evening before procedure G Do not give any SC insulin while child G On day of procedure, omit insulin and starved breakfast G Check venous U&E, glucose, blood G Allow clear fluids, including sweet gas when child cannulated drinks, up to 0600 hr G G Commence glucose and sliding scale Insert 2 IV cannulae if possible. These below can be inserted in theatre, if necessary insulin infusion – see G Start a glucose and sliding scale G Measure and record capillary blood insulin infusion in theatre – see below glucose hourly once nil-by-mouth and G Measure and record capillary blood half-hourly during operation glucose pre-operatively and half-hourly during operation If patient ill or diabetes not well controlled, follow Diabetic First on the afternoon list ketoacidosis guideline and postpone operation until patient G Advise usual doses of insulin evening stabilised. before procedure Operate once patient rehydrated, G Advise child to have a normal with stable blood pressure, serum breakfast no later than 0700 hr sodium and potassium within G Breakfast insulin dose normal range and blood glucose G if using multiple daily injection (MDI) <17 mmol/L regimen, give usual breakfast insulin G if using twice daily insulin regimen, give one-third of total morning dose as rapid-acting insulin (e.g. Novorapid ®)

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INTRAVENOUS INSULIN AND G Insulin sliding scale should always to be given in conjunction with a FLUIDS glucose infusion Maintenance fluid infusion G If blood glucose >15 mmol/L, check for ketones, if positive, contact doctor G Use premixed 500 mL bags of sodium chloride 0.9% and glucose 5% with Do not switch off insulin and/or 20 mmol/L of potassium chloride maintenance fluids in transit to G If blood glucose >14 mmol/L give and from theatre sodium chloride 0.9% with 20 mmol/L Do not give ANY SC insulin potassium chloride until child ready to come off G If blood glucose <5 mmol/L stop sliding scale insulin, recheck after 10–15 min and if still <5 mmol/L change fluid to glucose Monitoring 10% with sodium chloride 0.9% and 20 mmol/L of potassium chloride G Adjust insulin infusion rate according Table 1. Insulin infusion to blood glucose – see Aim to keep blood glucose between 5 and 10 mmol/L G Add 50 units soluble insulin (e.g. Actrapid ®) to 50 mL of sodium chloride G Check capillary blood glucose 0.9% to make a 1 unit/mL solution half-hourly during surgery and adjust insulin infusion according to Table 1 G Administer via syringe pump, do not add directly to fluid bag G Administer via a Y connector with one- way valve (e.g. Vygon-Protect-a-Line 2 extension) G Determine infusion rate from hourly capillary blood glucose results, according to sliding scale – see Table 1

Table 1 Capillary blood glucose (mmol/L) Insulin infusion rate (mL/kg/hr) ≥28.1 Call Doctor 18.1–28 0.1 12.1–18 0.075 8.1–12 0.05 4.1–8 0.025 ≤4 Stop insulin, treat hypo . Recheck blood glucose after 30 min and follow sliding scale

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Post-operative care Patients unlikely to resume eating and drinking G Check capillary blood glucose half-hourly for the first 2 hr and then G If after 48 hr, patient still unable to eat hourly or drink enough post-operatively: G Continue glucose and sliding scale G assess for enteral or parenteral insulin infusion until taking adequate feeding – contact nutrition support oral fluids and snacks. While on team insulin sliding scale, child may safely G contact diabetic nurse specialist for eat and drink advice on prescribing regular SC insulin G If taking adequate oral fluids and snacks and using multiple daily injection (MDI) regimen G give dose of rapid-acting insulin appropriate for carbohydrate content of next meal (if advice needed, contact diabetes team) and G give next long-acting insulin dose at usual time. If child was treated using a sliding scale overnight, and usual dose of long-acting insulin was omitted previous night, give half usual dose of long-acting insulin with breakfast if ready to eat G If using twice daily premixed insulin regimen and taking adequate oral fluids and snacks by: G lunch on same day of procedure, give one-third of total morning dose as rapid-acting insulin (e.g. Novorapid ®) G teatime on same day of procedure or breakfast on next day, it may be appropriate to give child’s usual insulin dose or a reduced dose – contact diabetes team for advice

Give non-analogue insulin 30 min before meal. Give analogue insulin 5 min before meal. After 30 min stop infusion

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RECOGNITION AND Investigations ASSESSMENT G Insert IV cannula (as large as Symptoms and signs appropriate for child) All cases G Thirst G Weight loss G Capillary blood glucose G Polyuria G FBC G Abdominal pain/vomiting G Blood glucose G Tachypnoea G Blood gas

G Sighing respiration (Kussmaul G Haemoglobin A 1c breathing) G Blood osmolality, sodium, potassium, G Odour of ketones urea, bicarbonate, creatinine, pH G Dehydration G Urine ketones on urinalysis G Drowsiness G Blood ketones G Coma G Infection screen: blood and urine G Biochemical signs: culture; if meningism consider lumbar puncture G ketones in urine or blood G elevated blood glucose (>11 mmol/L) Moderate and severe cases G acidaemia (pH <7.3) G Liver function tests and amylase Assessment G Group and save

G Airway, breathing, circulation Newly diagnosed case G record respiratory rate, heart rate, BP, G Thyroid and coeliac disease antibody peripheral pulse volume screen G Conscious level: look for signs of G Islet cell antibodies cerebral oedema (see Glasgow coma score ) G GAD antibodies G Headache, confusion, irritability, G Thyroid function tests, TSH, Free T4 abnormal movements, slow pulse, G Immunoglobulin A high BP, papilloedema, small and irregular pupils G Presence of infection G Height, weight Degree of dehydration

G Assessment degree of dehydration as 3%, 5% and 8% (for most children use 5–8% dehydration to calculate fluids)

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ALGORITHM (cross-referenced to text)

G Remember paediatric type 2 patients can present in DKA Dehydration <5% Shock Diabetic ketoacidosis Call senior staff Clinically well See 2 for definition Immediate treatment 1 Tolerating fluid orally pH >7.3

Dehydration >5% Resuscitation 2 Vomiting or Therapy G Airway + NG tube Biochemically acidotic G Start with SC insulin G Breathing (100% G oxygen) Give oral fluids G Circulation (sodium Intravenous therapy 3 chloride 0.9% No improvement 10 mL/kg repeated G Calculate fluid requirements Blood ketones rising until circulation G Correct over 48 hr, never less Looks unwell restored, max than 12 hr Starts vomiting 3 doses) G Sodium chloride 0.9% G Use premixed bags with KCl G Insulin 0.1 units/kg/hr infusion (after first hour of fluids) starting dose

Neurological No improvement Monitoring 5 deterioration G Blood glucose half-hourly, then G Warning signs: hourly G Re-evaluate G Neurological status at least headache, irritability, G Fluid balance + IV hourly slowing heart rate, therapy G Fluid input/output hourly reduced conscious level G If continued acidosis, G Electrolytes 2 hr after start of IV may require further therapy, then 4-hrly G Specific signs resuscitation fluid G raised intra-cranial G Check insulin dose Blood glucose <14 mmol/L pressure correct G Consider sepsis

Subsequent management 6 Exclude hypoglycaemia G Change to sodium chloride 0.9%/0.45% + glucose 5% ? cerebral oedema G Use premixed bags containing KCl G Continue monitoring as above

Management 7 Improvement 8 G Give sodium chloride 2.7% G Clinically well, drinking 5 mL/kg or 0.5–1 g/kg well, tolerating food mannitol G Blood ketones <1.0 mmol/L G Call senior staff Insulin 7 8 or pH normal G Restrict IV fluids by half Start SC insulin then G Urine ketones may still be G Move to PICU stop IV insulin positive G CT scan when stabilised

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G IMMEDIATE TREATMENT 1 altered state of consciousness G AND acidosis Inform senior staff G with or without hypotension G Give sodium chloride 0.9% 10 mL/kg Admission rapidly and reassess; repeated until circulation restored, max 3 doses G If alert and not shocked, admit to ward/HDU If still shocked despite giving G If shock or GCS <8, admit to PICU 2 boluses of sodium chloride 0.9% G Discuss with PICU if: 10 mL/kg, discuss with consultant G pH <7.1 and marked hyperventilation G When no longer shocked and G aged <2 yr circulated blood volume has been General restored, calculate volume of fluid required (see below) G Nil-by-mouth for first 8–12 hr INTRAVENOUS FLUIDS 3 G if vomiting, abdominal pain, no bowel sounds or decreased GCS, insert nasogastric tube Volume of fluid G Place on weigh-bed (if available) G Total fluid requirement is the addition G Strict fluid balance: catheterise of four categories: children requiring HDU or PICU G fluid to re-expand circulating volume if G Start flow-sheet to record shocked biochemistry and blood gases G maintenance fluids G Monitor ECG for T wave changes G deficit G Initiate IV fluids and insulin (see below) G continuing losses, do not include Shock and resuscitation 2 continuing urinary losses at this stage Maintenance fluids G Patient is shocked (very rare in DKA): G tachycardia G Patient will be nil-by-mouth and will G reduced peripheral pulse volume need normal fluid requirement IV G mottled cool peripheries G prolonged capillary refill time (poor sign)

Weight (kg) Rate (mL/kg/24 hr) Rate (mL/kg/48 hr)

0–12.9 80 160 13–19.9 65 130 20–34.9 55 110 35–59.9 45 90 >60 35 70

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Fluid deficit Total Amount

G Estimated amount of fluid patient has G Hourly rate of fluid replacement = lost, (i.e. how dehydrated – see (48 hr maintenance requirements + Assessment ) deficit – resuscitation fluid already given)/48 G Calculate from weight loss G Weight should rise gradually with G Most accurate method rehydration G weigh child and compare with recent G If available use weigh-bed to record weight weight hourly to obtain accurate G gives good estimate of fluid loss (1 kg assessment weight loss = 1 L fluid deficit) Type of fluid G Clinical assessment G Initially use sodium chloride 0.9% with G deficit in mL = % dehydration x body potassium chloride and continue this weight (kg) x 10 (e.g. for a 10 kg child concentration for at least 12 hr with 5% dehydration, the deficit is commercially premixed bag 5x10x10 = 500 mL) G Use G G do not use more than 8% dehydration Maximum rate potassium in calculations 0.2 mmol/kg/hr (ward) G If femoral line used give dalteparin 100 units/kg/day (max 5000 units)

Table 1 K+ <3.5 K+ 3.5–5.5 K+ >5.5

500 mL sodium chloride 0.9% Sodium chloride 0.9% with Sodium chloride 0.9% with potassium chloride potassium chloride 40 mmol/L 40 mmol via central line

If serum potassium <2.5 mmol/L, transfer to PICU. Discuss with consultant whether to give potassium chloride 0.2 mmol/kg in sodium chloride 0.9% by separate infusion over 1 hr. Before infusing bag containing potassium, connect patient to cardiac monitor. If possible, use commercially premixed bag. Only in exceptional circumstances (with consultant agreement and 2 doctors checking procedure) should potassium chloride be added on the ward to a bag of sodium chloride 0.9% (mix well)

G Further fluid and K + as dictated by the patient’s condition and serum K + (Table 1 ), repeated until glucose fallen to 14 mmol/L, then move to Subsequent management

YOU MUST obtain consultant authorisation before using bicarbonate infusion (not recommended)

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Insulin infusion 4 MONITORING TREATMENT 5

G Start 1 hr after IV fluids G Hourly capillary blood gas and G Soluble insulin (e.g. Actrapid ®) glucose infusion 1 unit/mL in sodium chloride G Check U&E, glucose, osmolality pH 0.9% via IV syringe pump at and capillary ketones 2-hrly until 0.1 units/kg/hr (or 0.05 units/kg/hr if improving, then 4-hrly local policy) G Neurological status, heart rate and G If no fall in glucose after 2 hr (very blood pressure hourly unusual, check pump and patency of IV cannula), increase by 20%. If no G Complete DKA summary sheets fall after 4 hr, consult senior medical SUBSEQUENT MANAGEMENT 6 staff and re-evaluate (e.g. sepsis, insulin errors) G If blood glucose falls exceeds After a minimum 12 hr of initial 5 mmol/L/hr, reduce insulin infusion intravenous therapy, if plasma rate by 20% sodium level stable or increasing, G Do not stop insulin infusion. Check change sodium concentration to capillary glucose in 1 hr sodium chloride 0.45% G If IV fluids and insulin given through When blood glucose falls below same cannula use anti-reflux valve 14 mmol/L add glucose to fluid Do not give insulin bolus. Do not G Maintenance fluid dependent on add insulin directly to fluid bags sodium, glucose and potassium:

Table 2: Sodium Blood sodium Fluid: with glucose (see Table 3 ) and potassium chloride (see Table 4 ) First 12 hr and if Sodium chloride 0.9% falling after 12 hr After 12 hr if stable Sodium chloride 0.45% or increasing Table 3: Glucose Blood glucose Fluid: sodium chloride (see Table 2 ) with potassium chloride (see Table 4 ) and 0–8.0 Glucose 10% 8.1–14.0 Glucose 5% >14 No glucose Table 4: Potassium Blood potassium Fluid: sodium chloride (see Table 2 ) and glucose (see Table 3 ) and K+ <3.5 Discuss with consultant K+ 3.5–5.5 Potassium chloride 20 mmol in 500 mL K+ >5.5 No potassium

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G If pH >7.3 reduce insulin infusion rate G Give 5 mL/kg of sodium chloride 2.7% to 0.05 units/kg/hr over 5–10 min G Blood glucose may rise as a result, G if not available give mannitol 0.5 g/kg but do not revert to sodium (2.5 mL/kg of 20%) over 20 min, chloride 0.9% unless plasma pH repeat mannitol once or twice after falls 2 hr if required G if pH falls, reassess fluid deficit and G restrict IV fluid intake to half regimen maintenance and replace deficit over G If glucose falls below 4 mmol/L, give 72 hr 2 mL/kg glucose 10% IV. Reduce G if patient unconscious, insert urethral insulin infusion rate by 20%. Check catheter capillary glucose in 1 hr G admit to PICU G To make glucose 10% with sodium G consider CT scan/MR scan chloride 0.45% (with or without potassium): remove 50 mL from Converting to SC insulin 8 500 mL bag of glucose 5%, sodium chloride 0.45% (with or without G Inform diabetes team (consultant, potassium) and add 50 mL of diabetic nurse and dietitian) glucose 50% G Children usually require insulin G Continue with IV fluids and insulin 0.25–1.0 units/kg/day (pre-pubertal infusion until urine is negative for usually 0.6–0.8 units/kg/day; higher in ketones and child tolerating oral fluids puberty) and food G If converting to multiple daily dose regimen: G Continue IV insulin pump after first SC dose of insulin for 1 hr if SC dose G give 40% as long-acting insulin at was soluble (e.g. Humulin S ®) or night 10 min if SC dose of insulin was G 20% short-acting insulin with each of aspart or lispro (e.g. Novorapid ®) the 3 main meals If acidosis not improving, G Adjust ratio if necessary, depending consider: on child’s eating patterns G If converting to biphasic insulin G Insufficient insulin to switch off ketones regimen divide total daily dose as follows: G Inadequate resuscitation 2 G Sepsis G /3 dose 20 min before breakfast (give G Hyperchloraemic acidosis insulin analogues 5 min before meal) 1 G Salicylate or other prescription or G /3 dose 20 min before evening meal recreational drugs (give insulin analogues 5 min before meal) Cerebral oedema 7 G Choose insulin preparation most G Observe for headache, any change in suitable for child: discuss with symptoms, pH <7.2, or persistently diabetes team low serum sodium as glucose G Continue IV insulin pump after first corrects SC dose of insulin for 60 min if SC ® G Exclude hypoglycaemia dose was soluble (e.g. Actrapid ) or 10 min if SC dose of insulin was G If cerebral oedema suspected, inform aspart or lispro (e.g. consultant immediately Novorapid ®/Humalog ®)

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DISCHARGE AND FOLLOW-UP

G Prescribe following as TTO for all new patients: G brand and strength of regular insulin, specify if pre-filled pen or cartridges G brand of soluble insulin, specify if pre- filled pen or cartridges G needles 5 mm G 1 pack hypostop triple pack G 1 packet glucose tablets G 1 box lancets (e.g. Microfine plus) G GlucaGen HypoKit (glucagon) 1 kit 500 microgram <25 kg, 1 mg ≥25 kg (if local policy) G 1 box blood glucose strips appropriate to blood glucose monitor G 1 box Ketostix (ketones in urine) G 1 box blood ketone testing strips (particular to local policy) G Organise out-patient follow-up

Issue 5 Issued: May 2013 102 Expires: May 2014 DIABETES NEW (NON-KETOTIC) • 1/2 G Any child or young person haemoglobin A 1c presenting to GP or A&E G FBC with symptoms suggestive of G cholesterol and triglycerides diabetes should be referred (by G TSH and FT4 phone) immediately to paediatric G immunoglobins A, G and M diabetes team G autoantibody screen for thyroid, RECOGNITION AND coeliac GAD and islet cell antibodies ASSESSMENT G Urine G ketones Definition G glucose Elevated blood glucose with no G C-peptide if considering type 2 diabetes ketonuria/blood ketones G Symptoms + random plasma glucose Do not arrange a fasting blood ≥11 mmol/L glucose or glucose tolerance test G Or symptoms + fasting plasma IMMEDIATE TREATMENT glucose ≥7 mmol/L G No symptoms but random plasma G Admit under admitting consultant of glucose ≥11 mmol/L day/week G No symptoms but fasting plasma G Inform diabetes team, consultant or glucose ≥7 mmol/L on 2 tests on diabetes nurse specialist 2 separate days G Start on SC insulin, total daily dose of Symptoms and signs 0.4 units/kg G If starting on multiple daily dose G Change in school performance regimen: G Thirst G give 40% as long-acting insulin at G Weight loss night G G Thrush 20% short-acting insulin with each of the 3 main meals G Polyuria G Adjust ratio if necessary, depending G Nocturia on child’s eating patterns G May be absent G If starting on biphasic insulin G if obese, no ketonuria or evidence of regimen divide total daily dose as insulin resistance (e.g. acanthosis follows: nigricans), consider type 2 diabetes 2 G /3 of total dose 10–20 min before Investigations breakfast (give insulin analogues 5 min before meal) G 1 Height and weight G /3 of total dose 10–20 min before G Blood: evening meal (give insulin analogues G glucose 5 min before meal) G G electrolytes For advice on which insulin to use, discuss with consultant with special G pH interest in diabetes G ketones

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SUBSEQUENT MANAGEMENT

G If tolerating food, allow patient to eat according to appetite for first 24–48 hr G Adjust insulin according to child’s eating habits G Refer to dietitians MONITORING TREATMENT

G Glucose stick monitoring pre-meals and at 0000 and 0400 hr DISCHARGE AND FOLLOW-UP

G Out-patient appointment to see consultant 1–2 weeks after discharge G Prescribe as TTO: G brand and strength of regular insulin, specify if pre-filled pen or cartridges G brand of soluble insulin, specify if pre-filled pen or cartridges G needles 5 mm G 1 pack glucogel triple pack G 1 packet glucose tablets G 1 box lancets (e.g. Microfine plus) G GlucaGen hypoKit (glucagon) 1 kit 500 microgram <25 kg, 1 mg >25 kg (dependent on local policy) G 1 box blood glucose sticks appropriate to blood glucose monitor G 1 box ketostix (ketones in urine) G if appropriate, 1 box blood ketone sticks (particular to local policy)

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Unexplained and prolonged G access to glycopenic agents (e.g. metformin) RECOGNITION AND G ASSESSMENT oral hypoglycaemics G nutritional intake Definition Investigations

G For the purposes of this guideline, hypoglycaemia defined as a blood Certain pointers to cause of glucose <2.6 mmol/L in child aged unexplained hypoglycaemia are >1 month-old detectable only during episode. Symptoms and signs Take blood samples BEFORE correcting blood glucose G Neuroglycopenia: G lethargy Immediate samples G lassitude G Before treating hypoglycaemia, take G tremulousness venous blood for assay using correct G loss of consciousness blood bottles ( Table 1 ) G seizure G once samples have been obtained, G Autonomic effects: correct hypoglycaemia. See Immediate treatment G sweating G inform laboratory immediately so G shaking samples arrive as quickly as possible G trembling (within 20 min) G tachycardia G Ensure first voided urine specimen G anxiety after hypoglycaemia episode is obtained to test for ketone bodies, G hunger organic/amino acid metabolites Previous history and reducing substances. Check with laboratory G Ask about: Table 1: Total blood requirement G antenatal history (e.g. small-for-dates) (5 mL minimum) G prematurity Fluoride 1.3 mL G history of hypoglycaemia on the neonatal unit Lithium heparin 1.3 mL G early or prolonged jaundice Clotted 2.6 mL G family history of sudden death (MCAD, LCAD) G history of neuroglycopenia/autonomic symptoms when glucose intake decreased, (e.g. during minor illnesses) G development, especially developmental regression G medication

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Investigations Physcial examination

G In all prolonged unexplained G Height and weight hypoglycaemia: G Midline defects, micropenis, optic G glucose sticks nerve hypoplasia (pituitary disorder) G capillary blood gas G Dysmorphic features: macroglossia, G true glucose macrosomia, ear lobe crease (Beckwith-Wiedemann) G lactate G Skin hyperpigmentation (adrenal G ACTH insufficiency) G growth hormone G Hepatomegaly (glycogen storage G insulin disorder) G C-peptide G cortisol G urea and electrolytes G urinary ketones G 17 O HP in infant if hyponatraemia present

G Store blood and urine for these investigations depending on above results: G IGF1 G beta-hydroxybutyrate G free fatty acids G carnitine G urinary-reducing substances G organic and amino acids

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Differential diagnosis

First-line investigations before correcting glucose: GInsulin GGrowth hormone GC-peptide GCortisol GUrinary ketones GACTH

Ketones absent Ketones present

Urinary non-glucose-reducing substances See algorithm on next page

Present Absent

Serum insulin elevated Serum insulin not elevated

Serum insulin Serum insulin Fatty acid >5–10 micro units/mL >100 micro units/mL oxidation or carnitine defect

Fasting C-peptide glucagon test (refer to specialist centre) High Low

Hereditary fructose Hyperinsulinaemia Insulinoma Exogenous intolerance or insulin galactosaemia

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Algorithm: Ketones present

Ketones present

Growth Cortisol <50 mmol/L +/- growth hormone and hormone <10 mmol/L in presence of cortisol normal confirmed hypoglycaemia

Hepatomegaly ACTH level

Yes No Low High

Glycogen Hypopituitarism Adrenal storage Ketotic insufficiency disease hypoglycaemia

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IMMEDIATE TREATMENT

Glucose sticks <2.6 mmol/L

GCS ≥8 GCS <8 and well (seek help)

IV access

G Take blood for hypoglycaemia investigation and obtain urine sample for G Take blood for hypoglycaemia ketones investigation and obtain urine sample for ketones G If available check blood for ketones G If available check blood for ketones

Feed or, if not interested in Glucose 10% 2 mL/kg IV bolus followed by solids, give Lucozade ® infusion of glucose 10% and sodium (flat) or Glucogel chloride 0.45%* at 4–5 mL/kg/hr

Continue Recheck glucose sticks Recheck glucose sticks after 10 min glucose after 10 min infusion

Reassess ABCD and ≥2.6 mmol/L <2.6 mmol/L <2.6 mmol/L ≥2.6 mmol/L discuss further management with consultant

Continue to reassess. Discuss further management with consultant

G Failure of blood glucose to respond to extra glucose suggests possible underlying metabolic problem related to either: G excessive insulin production or exogenous insulin G inability to utilise glucose owing to hypopituitarism or adrenal insufficiency G In either case further therapeutic manoeuvres need to be used – see Subsequent management

* Remove 50 mL from 500 mL sodium chloride 0.45% and glucose 5%, add 50 mL glucose 50%

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SUBSEQUENT MANAGEMENT

<2.6 and ketone body production not known

Bolus dose of hydrocortisone 4 mg/kg IV

G Continue glucose infusion Recheck glucose sticks after 10 min G Reassess ABCD G Correct electrolyte imbalance G discuss with consultant re <2.6 mmol/L >2.6 mmol/L further management (e.g. hydrocortisone oral 4 mg/m 2 8-hrly or hydrocortisone IV Consider hydrocortisone infusion at boluses at 4 mg/kg 6-hrly) 25 mg/24 hr for <10 kg, 50 mg/24 hr if 10–20 kg, 100 mg/24 hr >20 kg

If still no response in blood glucose

Increase glucose content to 14–20% (>14% needs to go through a central line)

A high glucose load (>10 mg/kg/min) suggestive of hyperinsulinism

G Endocrine opinion G Consider: G octreotide G diazoxide G glucagon

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Applicable for all subcutaneous insulin regimes and insulin pump therapy

Blood glucose ≥14 mmol/L, or blood glucose <14 mmol/L with associated dehydration or symptoms of illness Test blood ketones (beta-hydroxybutyrate)

0.1–1.0 1.1–3.0 >3.0 mmol/L mmol/L mmol/L

Acceptable levels Need to reduce blood ketones to Are signs and Treat high ≤1 mmol/L symptoms of blood glucose DKA present? Contact on-call If unwell refer to paediatric SpR to NO YES sick day dose children’s flowchart diabetes team

Start DKA protocol

Give additional rapid acting insulin by SC injection and refer to sick day dose flowchart Dose calculation: either (a) 10% of total daily dose to maximum of 10 units/dose OR (b) insulin 0.1 unit/kg body weight If pump user, administer by insulin pen or syringe, then replace infusion set and check insulin pump

Retest blood glucose and blood ketones in 1 hr Contact on-call Blood ketones paediatric Blood ketones not SpR or falling falling children’s diabetes team G Repeat further Recheck blood SC insulin dose ketones in 1 hr as above G ≤1.0 >1.0 Blood ketones should fall by mmol/L mmol/L 1 mmol/L/hr

Blood Blood ketones Recheck blood ketones not falling ketones in 1 hr falling

If unsure, especially if child aged <5 yr, contact children’s diabetes team

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Pituitary-adrenal axis impairment G if no plan for corticosteroid manipulation, prescribe RECOGNITION AND hydrocortisone 2–4 mg/kg IV at ASSESSMENT induction then 6-hrly until child capable of taking oral medication, Definition then give double usual daily maintenance dosage of G Children with the following conditions hydrocortisone for subsequent 48 hr are corticosteroid-dependent with a G Continue usual medication with: depressed or absent pituitary-adrenal axis: G fludrocortisone G hypopituitarism G growth hormone G adrenal insufficiency G levothyroxine G congenital adrenal hyperplasia G desmopressin G growth hormone insufficiency Acute illness G prolonged corticosteroid use for immunosuppression G During illness, corticosteroid- dependent children can usually be G severe asthma requiring oral managed at home corticosteroids or high-dose inhaled corticosteroids G Moderate illness with temperature ≤38ºC give double hydrocortisone When shocked or stressed dose, if temperature >38ºC give treble corticosteroid-dependent children hydrocortisone dose cannot mount an appropriate G if unable to take oral corticosteroids adrenal response (e.g. vomiting or acute collapse), parents to administer IM hydrocortisone 2 mg/kg or aged <1 yr G Corticosteroid-dependent children are 25 mg, 1–6 yr 50 mg, 100 mg encountered in a number of ways: thereafter G at presentation and first diagnosis G If IM hydrocortisone required, hospital G for elective surgical and investigative assessment necessary with training of procedures parents to administer IM G for emergency surgery or when G Continue usual dose of other acutely unwell medication G with hyponatraemia, hyperkalaemia G failure to do so may lead to +/- hypoglycaemia and hypotension hypoglycaemia MANAGEMENT G Some units do not prescribe IM hydrocortisone. Check locally Elective surgical and G Patients must carry a steroid card investigative procedures

G Check whether pre-operative discussion of endocrine management has taken place

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Algorithm for the management of unwell corticosteroid-dependent children

Resus ABC BP, GCS

IV access

G Take blood for G glucose stick If glucose stick Correct electrolyte G <2.6 give bolus of FBC abnormalities (see glucose 10% G blood culture Diarrhoea and 2 mL/kg G renal profile vomiting guideline) G blood gas

Hydrocortisone 4 mg/kg IV bolus

Calculate maintenance + deficit fluids (see Diarrhoea and vomiting guideline) Commence infusion of glucose 10%* and sodium chloride 0.45% Once electrolytes are known, it may be necessary to change to glucose 10% and sodium chloride 0.9%

G Regularly monitor BP and blood glucose Commence hydrocortisone G Titrate infusion IV 1–3 mg/hr (2–4 mg/kg accordingly, i.e. low 6-hrly) BP and/or glucose stick then increase infusion rate

G Once able to tolerate oral fluids, convert to oral corticosteroids (at double the patient’s normal daily dose) G consider treble usual corticosteroid dose initially G for simplicity, double patient’s highest dose of the day (as may be different doses throughout day)

G Continue double/triple normal daily dose of corticosteroid until 2 –3 days after recovery from acute episode

* Glucose 10% can be made by adding 50 mL glucose 50% to a 500 mL bag of glucose 5% with sodium chloride 0.9% or 0.45%

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RECOGNITION AND G On examination: ASSESSMENT G a sausage-shaped mass crossing midline in the epigastrium or behind Symptoms and signs umbilicus G may be associated with Henoch- G Pain may be localised or generalised Schönlein purpura G Vomiting G abdominal distension and G Anorexia hypovolaemic shock late signs G Fever Mid-gut volvulus G Crying and irritability G Presents mainly in neonatal period Typical features of some G History of: important causes of acute G bowel obstruction abdominal pain in children G abdominal pain Appendicitis G distension G bilious vomiting G History of localised pain with G increased severity on RIF On examination G G On examination: abdominal distension, tenderness G fever Pneumonia and empyema G mid-abdominal pain migrating to RIF G History of fever and cough G guarding and rebound tenderness G On examination: G pain on percussion G tachypnoea G young children may not have typical G features recession +/- focal signs at one base G decreased breath sounds and Intussusception dullness to percussion

G Typical age of presentation: Differential diagnosis 2 months–2 yr G History of intermittent colicky Surgical problems abdominal pain 2–3 times/hr initially with increasing frequency G Acute appendicitis G Looks pale with pain G Intussusception G Lethargic between episodes of pain G Intestinal obstruction G Vomiting prominent feature G Torsion of ovary or testis G Diarrhoea common G Meckel’s diverticulitis G Passage of blood and/or mucus per G Hydronephrosis rectum (redcurrant jelly stools) late G Renal or biliary calculus sign G Enterocolitis secondary to G Follows respiratory or diarrhoeal Hirschprung’s disease illness G Clinical features of intestinal obstruction

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Medical problems – relatively G If child stable and suspect common appendicitis, intussusception, torsion of ovary or testis, renal problems, G Mesenteric adenitis pancreatitis or cholecystitis: ultrasound scan of abdomen G Constipation G Gastroenteritis G If respiratory symptoms: chest X-ray G Inflammatory bowel disease G Do not delay surgical review awaiting G Lower lobe pneumonia scans if acute surgical problem G Acute pyelonephritis suspected (e.g. torsion of testis, intussusception) G Henoch-Schönlein purpura G Hepatitis MANAGEMENT G Acute cholecystitis G Gastritis/peptic ulcer G If present, treat hypotension and shock Medical problems – rare but G Stop feeding if you suspect surgical important problem G Lead poisoning G IV access if surgical cause likely G Diabetes G Nasogastric tube free drainage if G Sickle cell crisis bowel obstruction G Acute porphyria G IV antibiotics if perforation (e.g. G Pancreatitis cefuroxime and metronidazole) G Primary peritonitis Indications for surgical review G Non-accidental injury Gynaecological problems G Localised right iliac fossa pain G Rebound tenderness/pain on G Ectopic pregnancy percussion G Torsion of ovarian cyst G Migration of pain G Miscarriage G Redcurrant jelly stools and bleeding G Pelvic inflammatory disease (PID) per rectum G Mittelschmerz pain G Bile-stained vomiting INVESTIGATIONS G Marked abdominal distension G Urine testing and analysis G Inguino-scrotal pain or swelling G FBC, ESR G Increasing abdominal pain with G Blood and stool culture progressive signs of deterioration G CRP, U&E, amylase, glucose, LFT Observation G Consider group and save G Consider pregnancy test in G If stable, period of observation may adolescent females (inform patient) be useful to make diagnosis Imaging Analgesia G Only if bowel obstruction or G Do not withhold analgesia pending abdominal perforation suspected: surgical review: opioids may be X-ray necessary (see Analgesia guideline)

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Management of acute abdominal pain

G History Indications for surgical Refer for surgical G Yes Physical review opinion examination No

Urine dipstick: Urinary tract Yes See positive for leucocytes infection guideline and nitrites

No

Diarrhoea +/- fever or Fever No vomiting

Yes Yes

Consider: Consider: G Gastroenteritis G Gastroenteritis G Urinary tract infection (UTI) G Inflammatory bowel G Pneumonia No disease G Mesenteric lymphadenitis G Appendicitis G Appendicitis

No Consider: G Inflammatory bowel Is there blood in Yes disease stools? G Haemolytic uraemic syndrome No G Gastroenteritis G Intussusception

History of infrequent Adolescent girl bowel motions

Consider pregnancy test Consider constipation

DISCHARGE AND FOLLOW-UP

G Discharge usually within 24 hr of symptoms settling (e.g. fever, abdominal pain) G Follow-up usually appropriate in primary care/GP

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RECOGNITION AND KEY POINTS IN PHYSICAL ASSESSMENT EXAMINATION

Definition G Weight and height G Abdominal examination to look for G Constipation: infrequent bowel abdominal distension, faecal loading evacuation of hard faeces or G Lower limb neuromuscular difficult/painful defecation for examination in long standing cases ≥1 month G Spinal examination G Faecal soiling (overflow as a result G of faecal impaction): passage of loose Inspection of perianal area for and offensive stools in child’s appearance, position of anus or underwear over which child has no evidence of streptococcal infections control Symptoms and signs G Encopresis (functional non-retentive suggestive of organic soiling): inappropriate passage of constipation (‘red flags’) normal stools in inappropriate places. Often associated with behavioural G Early onset of constipation (first few problems weeks of life) G Faecal incontinence: soiling in the G Failure to thrive/growth failure presence of an anatomical or organic G Neuropathic bowel: lesion G lack of lumbosacral curve G Faecal impaction: hard faecal mass G pilonidal dimple or tuft of hair in lower abdomen, a dilated rectum impacted with stool or excessive stool G sacral agenesis in the colon identified radiologically G flat buttocks KEY POINTS IN HISTORY G patulous anus G absent cremasteric reflex/absent anal G Frequency, volume and type of stool wink using Bristol stool chart G decreased lower extremity tone G Overflow soiling in older children and/or strength G Distress and/or straining on opening G absence or delay in relaxation phase bowels of lower extremity deep tendon reflex G Holding behaviour (crossing legs, G urinary symptoms back arching or tiptoeing) G Hirschsprung’s disease G Time of passing meconium after birth G delayed passage of meconium for G Bleeding per rectum more than 24 hr after birth in a term G Any trigger factors i.e. diet change, baby infection, potty training or starting G abdominal distension nursery/school G tight empty rectum in presence of palpable faecal mass G gush of liquid stool and air from rectum on withdrawal of finger G rarely causes soiling

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G Anteriorly displaced anus Abdominal X-ray G Anal stenosis: G tightness or stricture felt when per G Has little or no value in the diagnosis rectum digital examination done using of idiopathic constipation lubricated fifth finger in newborn and G lower spine X-ray may be useful in an infants up to 6 months encopretic child with no faecal G Dairy protein intolerance in first 3 yr masses on abdominal and rectal of life examination DIFFERENTIAL DIAGNOSIS When to consider referral for rectal biopsy G Idiopathic functional constipation (90–95%). Most common cause of G History of delayed passage of constipation beyond neonatal period meconium G Constipation since neonatal period Organic constipation (suspected in presence of red flags) G History of abdominal distension and vomiting G Constipation secondary to anal G Failure to thrive or faltering growth anatomic malformation (ano-rectal G Family history of Hirschprung’s examination required) G Neurogenic constipation due to spinal MANAGEMENT OF cord anomalies or trauma, FUNCTIONAL CONSTIPATION neurofibromatosis and tethered cord G See Constipation management (lower limb neurological examination flowchart required) G G Constipation secondary to Refer children with organic cause to endocrine/metabolic disorders gastroenterologist (hypothyroidism, hypercalcaemia, Principles of treatment hypokalemia, CF) G Constipation induced by drugs G Education (opioids) G Diet and lifestyle G Coeliac disease G Behavioural management INVESTIGATIONS G Medication G Supporting child and family G Most children with chronic constipation require minimal Education investigation: G Give parents clear explanation of G careful history and physical pathophysiology of constipation and examination will help determine soiling appropriate investigation G Consider testing for coeliac disease Diet and lifestyle and thyroid function in intractable cases G Use in combination with laxatives G Ensure adequate fluid intake G High fibre diet is recommended G Encourage physical activities

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Behavioural management Medication

G Use of behavioural management in G Disimpaction in the presence of combination with medications impacted stools decreases time to remission DISIMPACTION G regular toileting: unhurried time on the toilet after meals Aged <1 yr, refer to paediatric G correct toilet position gastroenterologist G maintain diaries of stool frequency combined with reward system 1. A macrogol laxative [polyethylene G regular review and positive glycol (e.g. Movicol paediatric plain)]; reinforcement faecal impaction dose, see below up to a maximum of 7 days G discourage negative responses to soiling from family 2. Use stimulant laxative, senna or sodium picosulphate (Picolax) if no G encourage older children to take result with macrogol or if not tolerated responsibility 3. Review all children within/after one G May need counselling or a week of disimpaction (in hospital or psychology referral in case of by GP) motivational or behavioural problems

Disimpaction dosage Age (yr) Day 1 Day 2 Day 3 Day 4 Day 5 Day 6 Day 7

Number of Movicol paediatric plain sachets daily divided into 2–3 doses

1–5 2 4 4 6 6 8 8 5–12 4 6 8 10 12 12 12 Number of adult Movicol preparation for children aged >12 yr

>12–18 4688888

Rectal disimpaction Manual evacuation (only if oral disimpaction fails) G If all above have failed, consider G Sodium citrate micro-enemas (Relaxit) manual evacuation under general G Small volume sodium citrate enemas anaesthetic. Consult with paediatric (Microlax) is preferable to large volume gastroenterologist or paediatric phosphate enemas surgeon G Phosphate enemas (only if oral MAINTENANCE THERAPY medications and Microlax enemas failed). Use only under specialist G After disimpaction, or if child had no supervision. Consider sedation if child impaction, focus treatment on is distressed prevention of recurrence and establishment of a regular bowel habit to allow bowel to regain normal tone and sensation

Issue 5 Issued: May 2013 Expires: May 2014 119 CONSTIPATION • 4/5

G Continue maintenance therapy for 4–6 G Use outreach nursing support if months then reduce dosage gradually available G half the dismpaction dose of Movicol is G Liaise with the child’s health visitor, a useful guide for initial maintenance community paediatric nurse and/or dose school nurse. Send copies of consultations with parental agreement Laxatives to help provide a unified approach G Use macrogols as first line G Child psychology support when 1 available is invaluable maintenance treatment ( /2–1 sachet daily in children aged <1 yr) Withdrawal of laxatives G If not improved within a month or to prevent recurrence of impaction, add G Once regular bowel habit has been a stimulant laxative such as senna, established for a few months, and child bisacodyl or sodium picosulphate has good sensation to pass stools, syrup. Using stimulants is gradually withdraw laxatives over a recommended only for short periods period of months of time and intermittently. Use with INDICATIONS FOR SEEKING faecal softener e.g. sodium docusate and/or fibre ADVICE OF PAEDIATRIC GASTROENTEROLOGIST G Aim for soft/loose stools initially daily G High doses (up to 4–6 sachets daily of G Organic cause of constipation macrogols) may be required and suspected doses may need frequent adjustment G Disimpaction orally/rectally by child and parent to maintain a unsuccessful regular bowel action. Advise parents to G Soiling/abdominal pain continues reduce doses gradually and to despite treatment increase again if no bowel action in 3 days G Children aged <1 yr with faecal impaction or not responding to G If macrogols not tolerated, use sodium maintenance therapy docusate or lactulose G Aged <6 months: G give infant glycerol suppository once/day G change milk to hydrolysed formula if dairy intolerance Supporting child and family

G Organise review within a week then regular and frequent local contact and by telephone to prevent re-impaction G Provide a contact telephone number for parents if available G discuss timing of doses for convenience with bowel action G emphasise need for good compliance

Issue 5 Issued: May 2013 120 Expires: May 2014 CONSTIPATION • 5/5

CONSTIPATION MANAGEMENT

G History Yes Evaluate G Physical ‘Red flags’ for underlying organic disease? further examination No FUNCTIONAL CONSTIPATION

Is there faecal impaction?

Yes No G Treatment: G education G Disimpact orally G diet and lifestyle G Only use rectal preparations if oral G oral medication medication fails . Ensure child consents and is not distressed G behavioural therapy G close follow-up

G Movicol or docusate for 5 days to soften stools if G Use Movicol +/- stimulant not used previously G Aim initially for one or more ‘sloppy’ stools per day

Yes Effective? Treatment effective? Is there faecal impaction? No Yes No Yes

G Re-assessment Reduce dose after few G Compliance weeks and monitor closely G Re-education No G Change medication Yes

Relapse? Treatment effective? Yes No No G Wean G Blood tests: G Observe G T4 and TSH G Coeliac antibodies

Yes Abnormal blood tests? Evaluate further

No

Consultation with paediatric gastroenterologist and/or paediatric surgeon

Issue 5 Issued: May 2013 Expires: May 2014 121 DIARRHOEA AND VOMITING • 1/6

RECOGNITION AND G underlying problems e.g. low birth- ASSESSMENT weight, malnutrition, neuro-disability Inform public health if outbreak of Definition of diarrhoea gastroenteritis suspected G Passage of loose watery stools at least three-times in 24 hr Assessment G Most common cause is acute infective gastroenteritis G Weight, including any previous recent weight Diarrhoea and vomiting in infants G Temperature, pulse, respiratory rate may be a sign of sepsis G Degree of dehydration (see Table 1 ) and/or calculate from weight deficit Symptoms and signs G Complete systemic examination to rule out other causes of D&V G Sudden onset of diarrhoea (D) or G Children aged <1 yr are at increased vomiting (V), or both (D&V) risk of dehydration G Fever, malaise, lethargy Calculating fluid deficit G Abdominal cramps G Loss of appetite G Deficit in mL = % dehydration x weight (kg) x 10 Patient history G e.g. for a 10 kg child with 5% G Ask about: dehydration deficit is 5 x 10 x 10 = 500 mL G duration of illness G frequency of stools and associated Calculating maintenance fluids vomiting (>6 stools more likely to Weight Fluid volume become dehydrated) (kg) G colour of vomit (if green bilious vomit, <10 100 mL/kg/day consider obstruction) 10–20 1000 mL + 50 mL/kg/day for each G nature of stools, including presence of kg >10 kg blood in stool >20 1500 mL + 20 mL/kg/day for each G feeds (fluid and food intake) kg >20 kg G urine output (number of wet nappies) G contacts/exposure to infection G recent travel abroad G recent antibiotic use G symptoms of other causes of D&V (e.g. high pyrexia, shortness of breath, severe/localised abdominal pain or tenderness, symptoms of meningitis/septicaemia) G weight loss

Issue 5 Issued: May 2013 122 Expires: May 2014 DIARRHOEA AND VOMITING • 2/6 Table 1: Assessment of degree of dehydration Increasing severity of dehydration No clinically Clinical dehydration Clinical shock detectable 5–10% dehydrated >10% dehydration dehydration (<5%)

Appears well Appears to be unwell e – t e

o or deteriorating c ) t a m f n e -

r Alert and responsive Altered responsiveness Decreased level of e o ( t

m - (e.g. irritable, lethargic) consciousness s s e s c m e a o Normal urine output Decreased urine output

f – t s

p s d a n m Skin colour unchanged Skin colour unchanged Pale or mottled skin a y

S Warm extremities Warm extremities Cold extremities

Alert and responsive Altered responsiveness Decreased level of (e.g. irritable, lethargic) consciousness

Skin colour unchanged Skin colour unchanged Pale or mottled skin

) Warm extremities Warm extremities Cold extremities t n

e Eyes not sunken Sunken eyes – m s

s Moist mucous membranes Dry mucous membranes – e

s (except for ‘mouth (except after a drink) s s

n breather’) a

g i e c S Normal heart rate Tachycardia Tachycardia a f -

o Normal breathing pattern Tachypnoea t Tachypnoea - e

c Normal peripheral pulses Normal peripheral pulses Weak peripheral pulses a f ( Normal capillary refill time Normal capillary refill time Prolonged capillary refill time

Normal skin turgor Reduced skin turgor –

Normal blood pressure Normal blood pressure Hypotension (decompensated shock)

Investigations G If severe dehydration, possible hypernatraemic dehydration (see Hypernatraemic dehydration G If vomiting a major feature or vomiting below) alone, or if baby aged <3 months: or diagnosis in doubt: urine for MC&S G FBC, U&E, chloride, glucose, blood G If septicaemia suspected, child and urine cultures. Blood gas or immunocompromised, or if stools venous bicarbonate bloody, mucous or chronic diarrhoea G if decreased level of consciousness present, send stools for MC&S and consider lumbar puncture, especially virology in babies G If recent antibiotics, send stool for Clostridium difficile toxin

Issue 5 Issued: May 2013 Expires: May 2014 123 DIARRHOEA AND VOMITING • 3/6

IMMEDIATE TREATMENT G Rehydrate orally using ORS (prescribe sachets and give clear See Flowchart – Management of instructions: if genuinely not tolerated, acute gastroenteritis in young parents may substitute with diluted children (aged <4 yr) sugar containing juice) G calculate fluid deficit and replace over General advice to parents 4 hr with frequent small volumes (5 mL every 1–2 min) G Adequate hydration important G continue to supplement with ORS for G Encourage use of oral rehydration each watery stool/vomit (10 mL/kg per solution (ORS) watery stool) G ‘clear fluids’ (water alone/homemade G Do not withhold food unless vomiting solutions of sugar and fruit) lack adequate sodium content and are G full feeding appropriate for age well inappropriate tolerated with no adverse effects G sugar, fruit juices and cola have a Step 2: Moderate dehydration high osmolar load and little sodium, (6–10%) and can worsen diarrhoea G Recommend early re-feeding with G If improving after 4 hr observation, resumption of normal diet (without can be managed at home provided restriction of lactose intake) after 4 hr social circumstances are rehydration appropriate/parents are happy. Otherwise, admit G Do not use anti-diarrhoeal agents G Calculate deficit and aim to replace G Anti-emetics (e.g. ondansetron melts) with ORS 50 mL/kg oral over 4 hr can be given for vomiting G Give small frequent feeds (5 mL every Continue breastfeeding 1–2 min) throughout episode of illness, G If not tolerating oral rehydration ORS can be given in addition (refuses, vomits, takes insufficient volume), use NG tube Treatment of dehydration G Review after 4 hr G Admit if: G when rehydrated start a normal diet, and continue maintenance fluids and G patient ≥10% dehydrated supplementary ORS for each watery G failure of treatment (e.g. worsening stool or vomit (10 mL/kg per watery diarrhoea and/or dehydration) stool) G other concerns (e.g. diagnosis G if dehydration persists, continue the uncertain, child aged <3 months, same regimen but replace fluid deficit irritable, drowsy, potential for surgical with ORS over the next 4 hr cause) G if this fails, e.g. vomiting ORS, Step 1: Mild dehydration (<5%) consider IV rehydration (see below) G If improving move to Step 1 G Can be managed at home G Emphasise to parents importance of adequate hydration

Issue 5 Issued: May 2013 124 Expires: May 2014 DIARRHOEA AND VOMITING • 4/6

Step 3: Severe dehydration Hypernatraemic dehydration (>10%) – see flowchart (Na >150 mmol/L)

G In hypernatraemic dehydration, there Beware hypernatraemic are fewer signs of dehydration dehydration. See Hypernatraemic G dehydration section skin feels warm and doughy, child lethargic and irritable/jittery with G If child in shock, first resuscitate with hypertonia and hyperreflexic. They sodium chloride 0.9% (20 mL/kg) and may have seizures reassess G if in shock, resuscitate with sodium G If >10% dehydration, obtain IV chloride 0.9% 20 mL/kg bolus access, especially if child drowsy G if Na >170 mmol/L, contact PICU G Calculate deficit using recent normal G if child has passed urine, add weight if available potassium to IV fluid – initially at G If alert, rehydrate orally with ORS, 10 mmol/500 mL, adjust according to replacing deficit (plus maintenance blood results when available requirement) over 4 hr In hypernatraemic dehydration, G Use NG tube if necessary aim to reduce sodium by no more G If oral/NG rehydration not possible, than 10 mmol/L in 24 hr replace deficit with sodium chloride 0.9% with glucose 5% over 24 hr G After initial resuscitation, give ORS: G give isotonic fluid e.g. sodium chloride replace deficit (+ maintenance) over 0.9% or sodium chloride with glucose 48 hr – via NG if necessary 5% G Check U&E after 1 hr G if hypoglycaemic or at risk of G If ORS not tolerated or sodium drops hypoglycaemia use sodium chloride >0.5 mmol/L/hr, start IV rehydration 0.9% with glucose 5% and potassium with sodium chloride 0.9%, replacing chloride deficit (+ daily maintenance) over 48 hr G start normal diet as soon as tolerated G Recheck U&E after 1–4 hr (depending G continue to replace ongoing losses on rate of drop of serum sodium and with ORS for each watery stool or starting value) vomit (5 mL/kg per watery stool) G If sodium dropping by >0.5 mmol/L/hr, G when improves move to Step 2 reduce rate by 20% G Once rehydrated, start normal diet including maintenance fluids orally

Issue 5 Issued: May 2013 Expires: May 2014 125 DIARRHOEA AND VOMITING • 5/6

MANAGEMENT OF SEVERE DEHYDRATION

Yes Shock Sodium chloride 0.9% 20 mL/kg

No Reassess

No Oral/NG tube Start sodium chloride 0.9% rehydration possible with potassium chloride IV

Yes

Low/normal (<150 mmol/L) Measure Maintenance and Rehydrate orally or serum sodium replacement over 24 hr via NG tube

High (>150 mmol/L)

Maintenance and replacement over 48 hr

DISCHARGE AND FOLLOW-UP G Do not withhold food, (especially breast milk), full feeding appropriate G If dehydration was >5%, ensure child for age if well tolerated after initial has taken and tolerated two breast or rehydration bottle feeds, or at least one beaker of G Advise parents how to prevent fluid transmission to other family members G Check child has passed urine and contacts G Tell parents diagnosis and advise on G patient should not share towels with management and diet others G Explain nature of illness, signs of G hand-washing with soap and warm dehydration, and how to assess and water after using toilet or changing deal with continuing D&V (explain nappy. Dry hands properly flagged symptoms in table of G Exclude from school/nursery until dehydration) 48 hr from last episode of diarrhoea G Emphasise importance of adequate or vomiting hydration. If dehydration recurs will G Exclude from swimming for 2 weeks need further rehydration following last episode of diarrhoea G If symptoms persisting, aged <1 yr or G Give open access if appropriate, low birth weight, continue to ensure parents aware of how to seek supplement with ORS at 5 mL/kg per help if needed watery stool or vomit G If diarrhoea persists for >10 days, advise to return for medical reassessment

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MANAGEMENT OF ACUTE GASTROENTERITIS IN YOUNG CHILDREN (AGED <4 YR)

Detailed history and examination

Clinician estimates % dehydration and current weight

G Hospitalise G Give sodium chloride 0.9% IV bolus if shock One or more of G Re-evaluate and repeat if necessary – following present? Yes G >10% dehydration see Management of severe dehydration G Signs of shock G Begin ORS, replacing deficit (up to G Patient drowsy 100 mL/kg) over 4 hr plus replacement of ongoing losses (oral/NG)

No Begin ORS, replacing Is patient 6–9% deficit (up to 100 mL/kg) dehydrated by weight Yes over 4 hr plus loss or by clinical replacement of ongoing estimation? losses (oral/NG)

No Begin ORS, replacing Is patient 3–5% Yes deficit (up to 50 mL/kg) dehydrated by weight over 4 hr plus loss or by clinical replacement of ongoing estimation? losses (oral/NG)

No Patient Yes Continue ORS Patient with diarrhoea tolerating for 4–6 hr or and <3% dehydration on ORS until rehydrated clinical estimation/current No weight

Yes G NG rehydration G Consider IV infusion G Continue child’s regular diet G Consider adding ORS to replace ongoing losses G Continue breastfeeding G Resume foods G Replace ongoing losses with ORS

Issue 5 Issued: May 2013 Expires: May 2014 127 NUTRITIONAL FIRST LINE ADVICE • 1/3 Initial guide to feeding when child not able to eat normally and dietitian not available

If patient nil-by-mouth see IV fluid therapy guideline before starting total parenteral nutrition (TPN) to ensure hypotonic fluids are not used if contraindicated

Gut functioning? NO YES

Total parenteral nutrition (TPN) Malabsorption Normal gut Pharmacy manufacturing Mon–Fri Pre-digested/ Whole protein hydrolysed feed protein feed If not available, use sodium chloride 0.45% + glucose 5% with 10 mmol potassium chloride in 500 mL Aged ≥1 yr Aged >6 yr Aged Aged ≥1 yr Aged >6 yr (use commercial Aged <1 yr Wt 8–20 kg Wt >20 kg <1 yr Wt 8–20 kg Wt >20 kg pre-mixed bags) <8 kg – monitor U&E

Breast milk or Paediasure Peptisorb, Breast Nutrini Tentrini up Nutramigen 1 Pepdite Peptamen – milk/standard Paediasure to 45 kg If medium chain Peptamen peptide infant Frebini Paediasure triglycerides junior based formula. original up to 30 kg (MCT)* needed, Pepdite 1 + If above not If failure to Frebini use Peptijunior/ tolerated: thrive or fluid original up Pregestimil – Elemental restriction, to 30 kg OR use see below** feeds above are <1 yr feeds 028 Extra-L Over 20 kg: peptide based until dietitian – amino Nutrison If not tolerated, review acids Standard try Fresubin Nutramigen AA original or Neocate LCP Osmolite both contain L-amino acids

G Contact dietitian to assess individual requirements and appropriate feed at the first available opportunity Monday–Friday G Feeds in bold must be prescribed G Hospital pharmacy will advise which feed is used locally (all similar composition for ages but different manufacturer) G See Table 1 for daily fluid and nutritional requirements

* Indications for MCT: malabsorption, or problems with digestion, absorption or transport of long chain fats e.g. cholestasis, short gut, pancreatic insufficiency ** If failure to thrive or fluid restricted: G If using breast milk, dietitian to advise on fortification of breast milk G nutriprem breast milk fortifier 1 sachet (2.1 g) per 50 mL expressed breast milk (EBM) can be added until dietitian advice given G If using standard infant formula, change to Similac High Energy or Infatrini

Issue 5 Issued: May 2013 128 Expires: May 2014 NUTRITIONAL FIRST LINE ADVICE • 2/3 Table 1: Nutritional and fluid requirements Age Average Fluid Energy Energy Protein Sodium Potassium weight mL/kg per *EAR/day Kcal/kg g/kg per mmol/kg mmol/kg (kg) day per day day per day per day 0–3 months 6 150 100–115 2.1 1.5 3.4 4–6 months 7.5 130 95 1.6 1.6 2.8 7–9 months 9 120 95 1.5 1.6 2.0 10–12 10 110 95 1.5 1.5 1.8 months 1–3 yr 95 1.1 1.7 1.6 female 12 1165 95 male 12.5 1230 95 4 yr 95 1.1 1.9 1.6 female 16 1460 87 male 16.5 1520 94 5 yr 95 1.1 1.9 1.6 female 18 1550 82 male 18.5 1720 88 6 yr 85 1.1 1.9 1.6 female 20.5 1620 76 male 21 1810 84 7–10 yr 75 1.0 1.8 1.8 female 27 1740 70 male 27 1970 70 11–14 yr 55 1.0 1.6 1.8 female 43 1845 47 male 40 2220 55 15–18 yr 50 1.0 female 56 2110 40 1.3 1.6 male 62.5 2755 45 1.0 1.4 *EAR - estimated average requirements = BMR (basal metabolic rate) x 1.4–1.5 Nutritional composition of milks – for further information use BNFc

Per 100 mL Kcal Protein g Fat g CHO g Na mmol K mmol Osmolality Breast milk (mature) 70 1.3 4.2 7.2 0.6 1.5 276 Cow & Gate Premium 67 1.4 3.5 7.5 0.8 1.7 330 Infatrini 100 2.6 5.4 10.4 1.0 2.6 310 Similac High Energy 101 2.6 5.4 10.3 1.1 2.3 333 SMA High Energy 91 2.0 4.9 9.8 1.0 2.3 415 Nutramigen 1 68 1.9 3.4 7.4 1.4 1.9 290 Peptijunior 67 1.8 3.6/50% MCT 6.9 1.4 1.9 190 Neocate LCP 71 2.0 3.5 6.6 0.9 1.6 360 Nutramigen AA 68 1.9 3.6 7.0 1.4 1.9 348 Nutrini 100 2.8 4.5 12.2 2.3 2.6 250 Paediasure 100 2.8 5.0 11.2 2.6 2.8 320 Nutrison Standard 100 4.0 3.9 12.3 3.5 3.5 310 Osmolite 100 4.0 3.4 13.6 3.83 3.8 288 Paediasure Pepdite 100 3.0 4.0/50% MCT 13.0 3.0 3.9 320 Peptamen junior 100 3.0 3.9/60%MCT 13.8 2.9 3.5 260 Pepdite 1+ 100 3.1 3.9/35% MCT 13.0 2.1 3.0 465 Peptisorb 100 4.0 1.7/47% MCT 17.6 4.3 3.8 520 Peptamen 100 4.0 3.7/70% MCT 12.7 2.6 2.8 240 Aged >6 yr Elemental Extra 028 85 2.5 3.5/35% MCT 11 2.7 2.4 700

Issue 5 Issued: May 2013 Expires: May 2014 129 NUTRITIONAL FIRST LINE ADVICE • 3/3

How to calculate energy Monitoring requirements for tube feeds G Check plasma electrolytes daily with G Choose appropriate feed for age. If particular reference to phosphate, very underweight for age, use potassium and magnesium: correct appropriate feed for actual accordingly. Stop once clinical bodyweight condition stable G Calculate amount of feed to use in G Re-feeding syndrome may occur in 24 hr based on: the first few days of re-feeding but G Kcal/kg in children aged <1 yr can occur up to 2 weeks after. Continue biochemical monitoring for G estimated average requirements 2 weeks or until electrolyte (EAR) for age/weight for aged >1 yr parameters are stable G Calculate fluid requirement; if restricted, continue to use feeds above until reviewed by dietitian G if extra fluid required, give water G Feeding method depends on clinical condition of child: G if child at risk of re-feeding syndrome (e.g. anorexia nervosa, Crohn’s), introduce feed slowly over 3–4 days starting at 25% of Kcal intake day 1. Increase daily by 25% until full feeds at day 4 G Bolus feed can be given 1, 2, 3, 4 hourly intervals depending on tolerance G If on continuous feeds (i.e. over 24 hr), start feed at a quarter of final hourly requirement. Increase to half requirement, three-quarters, and full every 4–6 hr as tolerated. When full feeds tolerated, aim to give full requirement over 20 hr

Issue 5 Issued: May 2013 130 Expires: May 2014 FAILURE TO THRIVE • 1/3 RECOGNITION AND G Perform direct observation of child at ASSESSMENT mealtimes: G oral, motor, co-ordination, behaviour Definition (e.g. crying, tantrums), appetite, family interaction G An infant or older child who fails to Family gain weight as expected nd G Growth below the 2 percentile or a Ask about socio-emotional factors change in growth that has crossed G downwards 2 major growth percentiles Family composition (other children, age, FTT?) in a short time (approximately 4 months, or longer period in older G Ask parental ages, health, educational child) status G Associated features include: G were either parents in care during G developmental delay childhood? G apathy G do parents have a history of G misery psychiatric illness or depression (including post-natal depression) or Symptoms and signs had learning disability? G G Gastrointestinal problems parents with inadequate social or problem solving skills? G vomiting G Has the family any support network G voracious appetite (e.g. grandparents)? G anorexia G Social isolation? G diarrhoea G Is there a lack of money in the home G Physical examination or unemployment? G dysmorphic features G Other sources of stress (e.g. divorce)? G heart murmurs G Substance abuse? G abdominal distension G G wasting Domestic violence? G bruising Measurements Patient and family history Measurements must be carried out properly and checked if there is doubt Child G Record birth weight and gestation G Take a full feeding history G some ‘light-for-dates’ infants fail to G type of milk given (breast milk, baby catch up, and grow parallel but below milk, cow’s milk) the 2 nd percentile G volume given at each feed G Measure and plot G frequency of feeding G weight (unclothed) G method of making up feeds (correct G head circumference strength) G length or height G introduction of solids: age and type of G solid body mass index and plot on appropriate chart (useful if height or G any difficulty with feeding process weight below 0.4 th centile) (e.g. breathless, uncomfortable)

Issue 5 Issued: May 2013 Expires: May 2014 131 FAILURE TO THRIVE • 2/3

G Infant may be a small, normal child Differential diagnosis growing below but parallel to the 2 nd percentile G Low genetic growth potential: G parents are often also small G familial G record height of parents and G ‘light-for-dates’ baby grandparents G genetic syndrome Single set of measurements of G Social factors: limited value and does not justify G maternal depression complex investigations. G poor parenting skills Serial measurements of more G abuse value and should be plotted on G Malabsorption: percentile charts G pancreatic insufficiency: CF, Swachman-Diamond syndrome Investigations G enteropathy: coeliac, cow’s milk protein allergy Routine tests G inflammatory bowel disease (IBD) G G Faeces: culture and sensitivity, carbohydrate intolerance: lactose, microscopy for ova, cysts and sucrose, post-enteritis syndrome parasites G infective: Giardia, bacterial overgrowth G Urinalysis for protein, nitrites and blood G others (rarer): abetalipoproteinaemia, lymphangiectasia G Haemoglobin, blood film (for signs of iron deficiency), WBC and ESR G Vomiting/severe regurgitation G Biochemical profile including G Any chronic underlying disorder: creatinine, bicarbonate, calcium and G renal failure albumin G liver disease G Coeliac screen (anti-tTG and IgA) G congenital heart disease Further tests G severe asthma G immunodeficiency G If underlying pathology indicated by G other rare conditions e.g. history, clinical examination or results chromosomal or metabolic conditions of routine investigations, request if dysmorphic features present further tests, such as: G chest X-ray MANAGEMENT G bone age G Most patients can be managed as G sweat test/cystic fibrosis (CF) gene out-patient G Further gastrointestinal investigation G record height and weight at each visit or management of malabsorption G seek dietitian opinion disorders should be undertaken by referral to specialist gastroenterology G seek child psychologist opinion and team as appropriate: evaluation G endoscopy G if treatable cause identified, treat appropriately G gastrointestinal imaging

Issue 5 Issued: May 2013 132 Expires: May 2014 FAILURE TO THRIVE • 3/3

G If social problems responsible, consider: G admission to ward to demonstrate good weight gain out of home environment G significant weight gain after admission (>180 g/week in infant) supports parenting issues as cause G health visitor support G social work support G child psychology consultation, referral and/or intervention (evaluation of: child’s cognitive development, food refusal etc; parents’ perception of the child; family/child disturbances of affect expression and family dynamics) G day care and nursery provision G case conference G care proceedings

Issue 5 Issued: May 2013 Expires: May 2014 133 JAUNDICE • 1/3

Jaundice in neonates after discharge G Alpha-1-antitrypsin deficiency from maternity unit G Galactosaemia RECOGNITION AND G TPN-induced cholestasis ASSESSMENT Investigations Symptoms and signs All G Yellow colouration of skin in a pale- G skinned infant observed in natural If bilirubinometer light ≥250 micromol/L, total bilirubin G G Yellow conjunctivae in dark-skinned Conjugated bilirubin on all babies with infants very light yellow/pale stools Assess Jaundice in first 24 hours of life or requiring treatment G Pallor (haemolysis) G Urgent bilirubin (result within 2 hr) G Poor feeding, drowsiness G (neurotoxicity) Full blood count and film G G Hepatosplenomegaly (blood-group Baby’s blood group and direct incompatibility or cytomegalovirus) Coombs test G G Splenomegaly (spherocytosis) Mother’s blood group and antibody status (should be available from G Stools (pale, chalky) and urine colour maternal case notes) (dark, stains nappy: conjugated G hyperbilirubinaemia) Full infection screen (in an ill baby) G G6PD concentration (if indicated by Causes ethnic origin: Mediterranean, Middle Eastern, South East Asian, and local G Physiological hospital policy) G Prematurity Persistent jaundice aged >14 G Increased bilirubin load (e.g. bruising, days old term/preterm infants blood group incompatibility) G G6PD deficiency and other red cell G Total and conjugated bilirubin enzyme deficiencies G Liver function test (ALT, albumin, G congenital spherocytosis GGT) G cephalhaematoma G FBC, blood group, direct Coombs test G Rarely infection (e.g. UTI, congenital and coagulation profile infection) G Urine MC&S G Metabolic disorder G Document stool colour Persistent jaundice after 14 days G Check routine metabolic screening of age has been performed

G Breast milk jaundice G Hypothyroidism G Liver disease (e.g. extra hepatic biliary atresia and neonatal hepatitis)

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Second line investigations G Metabolic investigations: if indicated by associated G blood galactose-1-phosphate problems G urine for reducing substances G G If conjugated bilirubin >25 mmol/L urine for amino acid and organic acid seek specialist advice G alpha-1-antitrypsin G G6PD screen in African, Asian or Mediterranean patients If conjugated bilirubin elevated (>20% of total or >20 micromol/L), G Thyroid function tests: ask for ‘FT 4 discuss with consultant urgently priority and then TSH’ G Congenital infection screen: CMV PCR: in urine first 2 weeks life, later test newborn blood spot card G toxoplasma ISAGA-IgM and G throat swab for HSV PCR

Limits (micromol/L) for phototherapy and exchange transfusion for infants ≥38 weeks gestation

Age Repeat transcutaneous Consider Phototherapy Exchange (hours) bilirubin/serum bilirubin phototherapy # transfusion (6–12 hours)* 0 >100 >100 6 >100 >112 >125 >150 12 >100 >125 >150 >200 18 >100 >137 >175 >250 24 >100 >150 >200 >300 30 >112 >162 >212 >350 36 >125 >175 >225 >400 42 >137 >187 >237 >450 48 >150 >200 >250 >450 54 >162 >212 >262 >450 60 >175 >225 >275 >450 66 >187 >237 >287 >450 72 >200 >250 >300 >450 78 >212 >262 >312 >450 84 >225 >275 >325 >450 90 >237 >287 >337 >450 96+ >250 >300 >350 >450

* Result in this category repeat transcutaneous measurement in 6–12 hr # Result in this category repeat serum bilirubin measurement in 6 hr whether or not phototherapy started

Issue 5 Issued: May 2013 Expires: May 2014 135 JAUNDICE • 3/3

TREATMENT <7 DAYS MONITORING TREATMENT

G Adequate fluid and energy intake G If haemolysis present, check bilirubin G Phototherapy 4–6 hrly until rate of rise flattens G If bilirubin concentration approaching Jaundice presenting in first threshold for exchange transfusion, or 24 hours of life rising rapidly (>10 micromol/hr), check 4-hrly G Visible jaundice can be treated with phototherapy after sample taken for SUBSEQUENT MANAGEMENT bilirubin measurement G Bilirubin >100 micromol/L: repeat in G When bilirubin concentration has 6–12 hr fallen below threshold for phototherapy (see above), discontinue After first 24 hours phototherapy G If jaundice persists after 14 days of G Commence phototherapy according to age, review and treat cause following equation: G for infants <37 weeks, start if serum DISCHARGE AND FOLLOW-UP bilirubin (micromol/L) ≥ phototherapy level [(gestational age in completed G GP follow-up with routine examination weeks x 10) – 100] at 6–8 weeks G G for infants ≥37 weeks, start if serum If exchange transfusion necessary or bilirubin >340 micromol/L considered, request development (phototherapy level) follow-up and hearing test G In babies with positive Coombs test Phototherapy who require phototherapy, check haemoglobin at 2 and 4 weeks of age G If bilirubin near exchange threshold or because of risk of continuing still rising: haemolysis and give folate G increase power number of lights G increase area exposed (e.g. biliblanket and overhead) Exchange transfusion

G See Exchange transfusion in Neonatal guidelines IVIG

G Use as an adjunct to multiple phototherapy in rhesus disease when bilirubin continues to rise by >8.5 micromol/L/hr

Issue 5 Issued: May 2013 136 Expires: May 2014 VITAMIN D DEFICIENCY • 1/1 Serum 25-OHD Vitamin D Manifestation Management concentration status

<25 nmol/L (<10 ug/L) Deficient Rickets Treat with high-dose vitamin D Osteomalacia 25–50 nmol/L (10–20 ug/L) Insufficient Associated with Vitamin D supplementation disease risk 50–75 nmol/L (20–30 ug/L) Adequate Healthy Lifestyle advice >75 nmol/L (>30 ug/L) Optimal Healthy None

Treatment for deficiency G Children who have swallowing difficulties (aged <1 yr or disabled) a For 12 weeks liquid preparation may be used but is unpalatable G Aged <6 months: 3000 units daily G Colecalciferol and ergocalciferol liquid G Aged 6 months–12 yr: 6000 units daily preparation doses are equivalent OR 20,000 units weekly G Colecalciferol capsules may be initiated G Aged >12 yr: 10,000 units daily OR by a paediatrician in children aged <6 yr 40,000 units weekly G GP can continue prescribing the OR maintenance Modified Stoss regime Preparations

G Aged >1 yr: as a one off high dose Liquid G <40 kg: 160,000 units oral stat G ≥40 kg: 300,000 units oral stat or G Healthy Start vitamin drops 300 units 40,000 units once/day for 10 days colecalciferol per daily dose G Malabsorption or chronic liver disease G Abidec ® (contains peanut oil) and ® G aged 1–12 yr: 10,000–25,000 units daily Dalivit 0.6 mL dose 400 units G aged >12 yr: 10,000–40,000 units daily ergocalciferol Maintenance treatment or Colecalciferol capsules treatment for insufficiency G Fultium-D3 800 unit capsule (contains peanut oil) For 6 months G Dekristol 20,000 unit capsule (contains G Aged <1 month: 200 units daily peanut oil) unlicensed (400 units if exclusively breast fed G ProD3 10,000, 20,000 and 30,000 unit or maternal vitamin D deficiency) capsules unlicensed G Aged 1 month–2 yr: 400 units daily Other unlicensed preparations G Aged >2 yr: 800 units daily or 20,000 units colecalciferol weekly G May be very much more expensive: G Malabsorption or chronic liver disease check with pharmacy (use brand name G aged <1 yr: 800 units daily to ensure correct formulation) G G aged >1 yr: 800–1600 units daily Zymad 10,000 units/mL (300 units colecalciferol per drop) 5 drops Administration 1500 units unlicensed G Uvesterol 1500 units/mL ergocalciferol G All children who can swallow normal unlicensed food can take the small colecalciferol G capsules (e.g. Dekristol) Sterogyl 20,000 units/mL ergocalciferol unlicensed G Vigantol 20,000 units/mL colecalciferol

Issue 5 Issued: May 2013 Expires: May 2014 137 BLOOD AND PLATELET TRANSFUSIONS • 1/2

Always check front sheet in Rate of infusion patient notes before prescribing G Give total over 3–4 hr. Max rate any blood product 5 mL/kg/hr

G If Hb <60 g/L, give blood over 4–8 hr Before transfusion (each unit must be used within 4 hr once removed from fridge) G Explain indications for blood products to parents G Give furosemide 1 mg/kg oral if tolerated, or IV half-way through G Document indications and verbal consent Use irradiated blood if G If previous reactions to blood products have occurred, pre-medicate with G Allogenic bone marrow transplant chlorphenamine (oral or IV), if severe (BMT) from start of conditioning with hydrocortisone 4 mg/kg IV regimen BLOOD TRANSFUSION G Allogenic BMT donors G If <7 days pre-harvest for autologous When to transfuse BMT and stem cell transplant patients (e.g. stage IV neuroblastoma) Oncology children G Hodgkin’s disease or if patient has received fludarabine G If haemoglobin ≤80 g/L or if >80 g/L G and symptomatic, transfuse Children with severe immunodeficiency (e.g. SCID) G If having radiotherapy, transfuse if Hb <100 g/L G HLA-matched platelets Non-oncology children G For high risk neonates e.g. post intrauterine transfusion G If haemoglobin <60 g/L or >60 g/L Leucodepleted blood and symptomatic Target Hb and volume to be G All packed cells are leucodepleted transfused CMV negative blood G Aim for target haemoglobin of 120 g/L or for 100 g/L if initial haemoglobin G All the packed cells are leucodepleted <60 g/L and therefore CMV negative G In newly diagnosed patients with G For neonates aged <28 days post leukaemia/profound anaemia, aim for expected date of delivery and for target Hb 80–90 g/L intrauterine transfusions CMV G Calculate volume to be given as: serology negative blood requested (round to nearest unit) [Target Hb – actual Hb (g/L)] x weight (kg) x 0.4 mL G Total volume should not exceed 20 mL/kg

Issue 5 Issued: May 2013 138 Expires: May 2014 BLOOD AND PLATELET TRANSFUSIONS • 2/2

PLATELET TRANSFUSION IN ONCOLOGY CHILDREN

Transfuse platelets if platelet level

G <10 x 10 9/L oncology children except brain tumour G <20 x 10 9/L oncology children except brain tumour and unwell G <30 x 10 9/L brain tumour G <50 x 10 9/L brain tumour and unwell G <50 x 10 9/L for lumbar puncture Dosage and rate

G <15 kg: 15 mL/kg round off the nearest unit G ≥15 kg: one pack G Transfuse within 15–30 min

Immune thrombocytopenic purpura (ITP) – transfuse platelets only if bleeding and see Immune thrombocytopenic purpura (ITP) guideline

Issue 5 Issued: May 2013 Expires: May 2014 139 FEBRILE NEUTROPENIA • 1/3

G Pre-dose vancomycin level before Frequent clinical re-assessment third dose, and no post-dose sample of patients is a vital part of required effective management of febrile G Adjust pre-dose concentration (mg/L) neutropenia in children dose as follows: G <10 give 6-hrly and recheck level RECOGNITION AND before dose 4 or 5 ASSESSMENT G 10–15 continue current dose and recheck concentration in 3–5 days Definition G 15–20 reduce frequency of dosing and recheck level before dose 4 or 5 G Temperature: ≥38°C at any time (unless higher levels advised by G Neutrophils <1x10 9 cells/L microbiology) IMMEDIATE TREATMENT G >20 stop vancomycin and recheck level next day to see if therapy can be See Figure 1 (see BNFc for dose restarted reduction in renal impairment) Haemodynamic compromise ALL PATIENTS – with central G venous access Check A, B, C and initiate appropriate resuscitation G Culture both lumens/portacath. Take G Give sodium chloride 0.9% 20 mL/kg FBC, group and save, coagulation bolus screen, U&E, Cr, LFTs, CRP G Start meropenem 20 mg/kg 8-hrly G Urinalysis in all children aged <5 yr LOW RISK PATIENTS G CXR only if respiratory signs G Do not wait for results, administer G No central access and antibiotics G Neutrophils >0.5x10 9 cells/L and G ‘Door to needle time’ must be within G Clinically well 1 hr G consider discharge on oral antibiotics G Follow individual trust antibiotic policy after discussion with oncology team or individual patient plan if resistant organisms SUBSEQUENT TREATMENT

No haemodynamic compromise G Reassess at 24 hr and chase blood cultures G Start piperacillin with tazobactam G Positive cultures: Discuss patients (Tazocin ®) 90 mg/kg 6-hrly (maximum with microbiologist or paediatric single dose 4.5 g) unless penicillin oncology team for advice on allergy or previous Tazocin ® resistant appropriate treatment. Where blood gram negative infection: cultures positive for yeast in presence G then use meropenem 20 mg/kg 8-hrly of suspected line infection, remove (maximum single dose 1 g) suspected lines promptly G If previous documented MRSA G Give culture-positive patients at least infection, add vancomycin 15 mg/kg 7 days treatment 6-hrly (maximum single dose 700 mg) until levels available. Aim 10–15 mg/L

Issue 5 Issued: May 2013 140 Expires: May 2014 FEBRILE NEUTROPENIA • 2/3

G Negative cultures: Do not switch When to discharge initial empiric antibiotics with unresponsive fever unless there is G If clinically well and afebrile for 48 hr, clinical deterioration or a and no growth in blood cultures after microbiological indication 48 hr: G If febrile after 48 hr: G stop antibiotics G repeat blood cultures and discuss G no need for routine in-patient with on-call consultant/paediatric observation after stopping antibiotics oncology team G Initiate investigations for fungal infection e.g. US abdo/CT chest G If febrile after 96 hr or clinically unstable between 48 and 96 hr: G repeat blood cultures G add liposomal amphotericin (AmBisome ®) 3 mg/kg/day (give test dose 100 microgram/kg (max 1 mg)

Issue 5 Issued: May 2013 Expires: May 2014 141

FE BRILE NEUTROPEN IA • 3/3

Fig ure 1: Management of fever in neutropenic/immunoco mpromised child

Figure 1: Management of fever in neutropenic/immunocompromised child

Clinical assessment

x Blood/urine/stool No Haemodynamic x Other cultures as haemodynamic compromise appropriate: FBC, group & compromise save, coagulation screen,

U&E, Cr, LFTs, CRP x Do not wait for results, administer antibiotics Administer first dose of antibiotic within 1 hr of presenting with diagnosis of possible neutropenic fever

x x Commence piperacillin Previous Check A, B, C and with tazobactam initiate appropriate ® documented (Tazocin ) 90 mg/kg MRSA resuscitation x 6-hrly (maximum single infection Give sodium chloride dose 4.5 g) 0.9% 20 mL/kg bolus x If penicillin allergy or x Commence ® previous Tazocin meropenem 20 mg/kg resistant gram negative 8-hrly (maximum infection, use Add vancomycin 15 mg/kg single dose 1 g) meropenem 20 mg/kg 6-hrly (maximum single x Inform senior 8-hrly (maximum single dose 700 mg) then colleague dose 1 g) according levels, target ± x Stop prophylactic 10 15 mg/L antibiotics apart from co-trimoxazole

Reassess at Cultures positive 48 hr Discuss with consultant microbiologist or All cultures negative paediatric oncology Continued fever at 48 hr team for advice on x appropriate treatment Continue current antibiotic x Do not change antibiotic regimen without discussing with consultant Repeat blood cultures x Afebrile for 48 hr Continued fever at 96 hr and well Add AmBisome® 3 mg/kg/day only x Stop antibiotics Initiate investigations after discussion with consultant and discharge for fungal infection x ? oral antibiotics if e.g. USS abdo/CT chest appropriate

Issue 5 Issued: May 2013 142 Expires: May 2014 HENOCH-SCHÖNLEIN PURPURA • 1/2

RECOGNITION AND G raised urea and creatinine ASSESSMENT G hypertension G oliguria Definition G Nephrotic syndrome: proteinuria +/- oedema and hypoalbuminaemia G Vasculitic condition of unknown G aetiology Oedema of hands, feet, sacrum and scrotum G 50% have a preceding upper respiratory tract infection Neurological G Affects skin, gastrointestinal tract, G Headache (common) joints and renal tract G Seizures, paresis, coma (rare) G Typical age group 2–8 yrs old Symptoms and signs Differential diagnosis G Purpuric rash: Rash G meningococcaemia – clinical diagnosis G Purpuric, raised on extensor surfaces of legs, buttocks and arms, with G thrombocytopenia – FBC (rash looks surrounding erythema different, ITP not vasculitic) G Gastrointestinal tract rarer vasculitides – more difficult to exclude; differentiation requires review over a period of time G Abdominal pain mostly idiopathic, typically resolves in 72 hr G pancreatitis – suspect in abdominal pain lasting >3 days G if severe or persistent, exclude intussusception, testicular torsion or Investigations pancreatitis (rare) G Nausea and vomiting All patients G G Intestinal haemorrhage: BP haematemesis, melaena, bloody G Urine dipstick stools (rare) G if proteinuria, send urine for early Joints morning protein:creatinine ratio G if haematuria, send urine for G Arthralgia and swelling of large joints, microscopy especially ankles and knees. Pain typically resolves in 24–48 hr Additional investigations Renal Blood tests if urinalysis abnormal or diagnosis uncertain G Microscopic haematuria (common) G FBC+ film G Proteinuria can present 4–6 weeks G U&E after initial presentation G Albumin G Hypertension G If fever, blood culture and ASO titre G Nephritic syndrome: haematuria with G at least one of following: Coagulation G Throat swab

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IMMEDIATE Refer to nephrologist if TREATMENT/SUBSEQUENT MANAGEMENT G Urinalysis blood or early morning protein >1+ after 6 months G Condition is self-limiting, symptomatic G Macroscopic haematuria or heavy relief only proteinuria at presentation G Mortality <1% usually related to G Hypertension (see Hypertension kidneys guideline) G Long-term morbidity related to renal G Significant proteinuria (early morning disease urine protein:creatinine ratio G 1% of those with renal involvement >100 g/mmol or 3+ proteinuria for progress to end stage renal failure 3 days) G HSP accounts for 5–15% of patients G Impaired renal function with end stage renal failure in children Refer to rheumatologist if Joint pain G Atypical or rapidly evolving rash G NSAIDs (ibuprofen first-line, DISCHARGE AND FOLLOW-UP indometacin or diclofenac second-line. Use with caution if renal involvement) G Inform parents condition may fluctuate Abdominal pain for several months but recurrence rare once settled properly G Give prednisolone 1 mg/kg/day for G Very rare risk of renal failure, hence 2 weeks importance of monitoring urine G Renal involvement not a G Seek medical advice if child develops contraindication headache, PR bleeding or severe G If severe and persists, exclude abdominal pain pancreatitis, intussusception or spontaneous bowel perforation Uncomplicated HSP MONITORING G GP follow-up in 1–2 weeks. Monthly BP for 6 months and weekly urine Uncomplicated HSP (e.g. urine dipsticks at home until urine clear analysis ≤1+ blood and protein, Discharge from GP follow-up and normal BP) G If urine analysis is normal and G No hospital follow-up required but GP G to follow-up as below If BP normal at 6 months of symptoms onset HSP with haematuria or proteinuria >1+ and normal renal function

G GP follow-up in 1–2 weeks. Monthly BP for 6 months and weekly urine dipsticks at home until urine clear G If blood or protein >1+, routine follow- up in children’s out-patients

Issue 5 Issued: May 2013 144 Expires: May 2014 IMMUNE THROMBOCYTOPENIC PURPURA (ITP) • 1/2 RECOGNITION AND IMMEDIATE TREATMENT ASSESSMENT G None regardless of platelet count, Definition unless life-threatening owing to significant bleeding G Platelets <100 x 10 9/L, usually G If significant bleeding (e.g. <20 x 10 9/L uncontrollable epistaxis, GI G Self-limiting disease with shortened haemorrhage, intracranial bleed), give: platelet survival and increased G platelets (see Blood and platelet megakaryocytes transfusions guideline) G Good prognosis G immunoglobulin 1 g/kg (see local G Acute 0–3 months policy) can be repeated once within 3 days if required G Persistent 3–12 months G If moderate bleeding e.g. prolonged G Chronic >12 months mucosal bleeds, give prednisolone Symptoms and signs 4 mg/kg (max for 4 days) G Consider tranexamic acid for small G Acute onset bruising, purpura and bleeds petechiae G Avoid NSAIDs e.g. ibuprofen G serious mucosal bleeding unusual, G look for other causes Reassure parents G G Preceding infection Discuss newly diagnosed ITP with paediatric haematologist G Absence of: G Discuss treatment with platelets with G hepatosplenomegaly paediatric haematologist in event of: G lymphadenopathy G essential operations G evidence of serious cause/chronic G emergency dental extractions underlying illness SUBSEQUENT MANAGEMENT Investigations G 75–80% resolve in 6 months G FBC, blood film and clotting G favourable outcome irrespective of G Blood group treatment G CMV and EBV IgM G Avoid contact sports G Consider HIV, Hepatitis B and C if risk G impossible to prevent fighting/rigorous factors knockabout games at home G If ITP, headache and neurological G Parents can find additional information signs, urgent CT scan of head from ITP support association: G Bone marrow aspiration unnecessary www.itpsupport.org.uk unless: MONITORING TREATMENT G neutropenia or severe anaemia G hepatosplenomegaly G FBC and film monthly until diagnosis G lymphadenopathy clear or recovery G pallor and lassitude G Repeat sooner if bleeding or G pain limb/abdomen increased bruising G limp

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DISCHARGE AND FOLLOW-UP

G Discharge from long-term follow-up when platelets >100 x 10 9/L and asymptomatic G Advise of risk of relapse (20%) G Note that mothers with history of ITP (even if they have normal platelet counts) can give birth to thrombocytopenic babies CHRONIC IMMUNE THROMBOCYTOPENIC PURPURA

G Avoid NSAIDs G Avoid contact sports G Investigate for autoimmune disease (ANA antinuclear antibody; APLA, antiphospholipid antibodies; ACA, anticardiolipin antibody; and LAC, lupus anticoagulant) and immune deficiency (HIV) G Treat only: G profound thrombocytopenia (<10 x 10 9/L) with repeated mucosal bleeding G older girls with menorrhagia G trauma G acute neurological signs G If treatment indicated, give prednisolone 1–2 mg/kg/day until count responds G reduce gradually G must have bone marrow aspirate before treatment G If unresponsive, discuss with paediatric haematologist about treatment with rituximab

Issue 5 Issued: May 2013 146 Expires: May 2014 HAEMOPHILIA • 1/3

INTRODUCTION G Haemarthrosis, especially weight- bearing joints (e.g. hips and knees) G Haemophilia is a serious disease. G Any lesion requiring 12-hrly or more Each child with haemophilia must: frequent replacement therapy G have open access MANAGEMENT OF ACUTE G be treated within 30 min of attending BLEEDING the ward G be registered with designated tertiary G Patients present for treatment, haemophilia unit particularly when developing a haemarthrosis before any physical G be registered locally (if shared care signs are present appropriate) – for local registration, immediate there will be two copies of the G give replacement therapy treatment sheet, one copy at front of as haemarthroses are very painful and patient notes and a second copy on any delay may increase severity of ward bleed and risk of joint damage G if any doubt, contact haemophilia Inform haemophilia nurse of any nurse or haematologist patient attending for treatment Replacement therapy dosage INDICATIONS FOR ADMISSION G When deciding dose, consider: G Bleeding in mouth, neck, respiratory G type of lesion passages or gastro-intestinal tract G time of onset of symptoms G Suspected internal bleeding G factor level required to sustain (intracranial, intra-thoracic or intra- haemostasis abdominal) G half-life of therapy (varies with each G Haemorrhage endangering a nerve concentrate) (e.g. carpal tunnel – median nerve, iliopsoas – femoral nerve) or other vital structure G Requiring surgical treatment, including dental surgery

Type of lesion Level of factor desired G Uncomplicated bleeding into joints and G Non-weight bearing joint 30% muscles G Weight bearing joint 50% (may need twice daily infusion) G Haematoma in potentially serious G 30–50% situations: G bleeding in mouth G neck G respiratory passages G endangering nerves G Pre-dental extraction G 50% G Major surgery G 80–100% G Serious accident G Head injury

Issue 5 Issued: May 2013 Expires: May 2014 147 HAEMOPHILIA • 2/3

Calculation of replacement factor Duration of treatment

G Most boys with haemophilia receive G Decided by local on-call haematologist recombinant factor or designated tertiary haemophilia unit (on-call haematologist). If in doubt, ask G Calculate units of factor needed, X, using following formula: DESMOPRESSIN IN MILD G X = % rise in factor required x wt (kg) HAEMOPHILIA A AND VON K WILLEBRAND’S DISEASE (where K is the recovery constant) G Subcutaneously, intranasally or IV G Recovery constants vary. Common G may be used to raise Factor VIII factors used are: concentration G haemophilia A: Factor VIII G response usually fourfold rise (IV) or concentrates are: Advate, Kogenate, twofold rise (intranasal) in Factor VIII ReFacto, Helixate and Recombinate, and von Willebrand's antigen with recovery constant (K) = 2 concentration G haemophilia B: Factor IX concentrate Patient selection BeneFIX, with recovery constant (K) = 0.8. It often has a short half-life in G Consider only in mild ( NOT severe) children (aim 60% rise) haemophilia A Not G Factor X deficiency: Beriplex blood G appropriate in Factor IX deficiency product, with recovery constant (K) (haemophilia B) = 2.2 G Do not use in child aged <1 yr G von Willebrand’s disease: use G caution in children aged <2 yr Haemate P, with recovery constant (K) G Check notes for outcome of previous = 1.5 desmopressin challenge G For any other Factor concentrate, Administration of desmopressin contact on-call haematologist to discuss treatment and ascertain G Intravenously: 0.3 microgram/kg IV in correct recovery constant sodium chloride 0.9% 50 mL over 20 min. May be repeated after 12 hr Other treatment G Intranasally: 4 microgram/kg once G On advice of consultant haematologist G Side effects include hypertension for those with inhibitors to factors VIII G measure pulse and BP every 5 min or IX during infusion. If either rises G Factor VIIa (recombinant: Novoseven) unacceptably, reduce rate of infusion 90 microgram/kg 2-hrly with frequent G Ensure blood samples taken before review and after infusion to measure Factor VIII level and ensure therapeutic level Administration of factor reached concentrate G tachyphylaxis can occur with depletion of stored Factor VII with consecutive G Give intravenously over about 3 min days. After 3 days there may be an G adverse reactions rare but include inadequate rise of Factor VIII anaphylactic shock G Restrict patient’s fluid intake to 50% of G During prolonged treatment screen for maintenance (max 1 L/day) over the inhibitors every 5 doses following 24 hr

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VON WILLEBRAND’S DISEASE Tranexamic acid

G More common than haemophilia G Anti-fibrinolytic agent G caused by deficiency (qualitative or G Contraindicated in presence of frank quantitative) of vWF protein, which haematuria (>2+ blood) binds to Factor VIII (prolonging half- G Decrease dose in renal failure life) and platelets G For minimal mucosal bleeding, G Can present with acute episodes of tranexamic acid mouth wash may be mucosal bleeding, helping to form sufficient to stop initial bleeding initial clot G Oral tranexamic acid alone can be G Before treatment, consider: used to treat minor problems such as G von Willebrand’s disease (vWD) sub- recurrent epistaxis, but main use is in type combination with desmopressin if G bleeding history, including previous appropriate response to any treatment G oral dose 15–25 mg/kg 8-hrly (max G nature of haemostatic challenge dose 1.5 g) for max 5 days G Treatment is often a combination of G Intravenous tranexamic acid 10 mg/kg tranexamic acid and desmopressin or (max 1 g) 8-hrly over 10 min Haemate P Desmopressin

G Treatment of choice in responsive patients for spontaneous bleeding, trauma and minor surgery G For administration – see Administration of desmopressin

vWD Type Advice Type 1 G Most patients responsive Type 2A G Some patients responsive G ask about previous challenge Type 2B G DO NOT GIVE desmopressin G it causes platelet agglutination and thrombocytopenia Type 3 G Not all responsive and some can be severe G ask about previous challenge

Haemate P (blood product)

G Avoid if at all possible G Use in patients not responsive to, or unsuitable for, desmopressin (e.g. aged <2 yr) G See above for administration of replacement factor. Recovery constant for Haemate P = 1.5

Issue 5 Issued: May 2013 Expires: May 2014 149 ANTIBIOTICS • 1/2 EMPIRICAL ANTIBIOTICS

Once organism identified, change antibiotic to narrowest spectrum appropriate for site of infection Oral unless unavailable or IV stipulated; if not tolerating oral fluids use same antibiotic IV Pneumonia: Mild/Moderate Severe respiratory distress Community acquired Amoxicillin 1st line (vomiting: benzylpenicillin IV) Co-amoxiclav IV + azithromycin 2nd line Amoxicillin and azithromycin Piperacillin/tazobactom + azithromycin Flu Oseltamivir Empyema Co-amoxiclav IV + clindamycin Oseltamivir + co-amoxiclav Hospital acquired Piperacillin/tazobactam Allergy G azithromycin instead of amoxicillin G clindamycin instead of co-amoxiclav or piperacillin/tazobactam Penicillin allergy Meningitis Cefotaxime or ceftriaxone (high dose) Cefotaxime or ceftriaxone IV + amoxicillin IV (aged <3 months) + teicoplanin IV (multiple antibiotics in last If confirmed severe 3 months or recent travel outside UK) anaphylaxis Sepsis from: chloramphenicol IV or Community Cefotaxime or ceftriaxone (high dose) teicoplanin + gentamicin Hospital Piperacillin/tazobactam Encephalitis Aciclovir IV (high dose) UTI aged <3 months Cefotaxime or ceftriaxone Gentamicin aged >3 months:

Cystitis Cefalexin Gentamicin Pyelonephritis Co-amoxiclav Osteomyelitis Cefotaxime or ceftriaxone aged <5 yr Cefotaxime or ceftriaxone and septic arthritis Flucloxacillin (high dose) IV aged >5 yr Clindamycin Endocarditis Flucloxacillin IV Vancomycin + gentamicin (low dose) + gentamicin (low dose) Prosthesis or ?MRSA Vancomycin + rifampicin + gentamicin (low dose) GI surgical Co-amoxiclav IV Gentamicin + metronidazole prophylaxis Peritonitis treatment Piperacillin/tazobactam Tonsillitis Penicillin V (if can swallow tabs) Azithromycin Amoxicillin (suspension) Otitis media Amoxicllin (1 st line) Azithromycin Co-amoxiclav (2 nd line) Otitis externa Flucloxaxillin (if can swallow tabs) Azithromycin Co-amoxiclav (suspension) Impetigo Fusidic acid 2% ointment Erysipelas Co-amoxiclav Azithromycin Cellulitis Co-amoxiclav Clindamycin Flucloxacillin IV (if severe) Periorbital cellulitis Co-amoxiclav Azithromycin Orbital cellulitis Cefotaxime Chloramphenicol IV

Issue 5 Issued: May 2013 150 Expires: May 2014 ANTIBIOTICS • 2/2

LOCAL ANTIBIOTIC POLICY

G Follow your local Trust antibiotic formulary as appropriate

Issue 5 Issued: May 2013 Expires: May 2014 151 BITES • 1/1

Prevention of infection after bites from G antibiotics are not generally needed if humans and other animals wound ≥2 days old and no sign of local Give prophylactic antibiotics to or systemic infection G Advise patient and carers of signs of G All human bite wounds ≤72 hr old, developing infection and to attend even if no sign of infection urgently for review should this happen G Animal bite wounds if wound ≤48 hr Source is known or suspected old and risk of infection high as follows: to be positive for HIV, hepatitis G bites to hand, foot, and face; puncture B or C, or rabies wounds; wounds requiring surgical debridement; crush wounds with G Ask vaccination and HIV/Hep B and C devitalised tissue; wounds in genital status of person bitten areas; wounds with associated G Ask if biter is willing to be tested oedema; wounds involving joints, G Risk of HIV transmission is extremely tendons, ligaments, or suspected small. See HIV and hepatitis B post- fractures exposure prophylaxis (PEP) guideline G wounds that have undergone primary G Seek advice from consultant closure microbiologist or consultant in G patients at risk of serious wound infectious diseases infection (e.g. immunosuppressed) G If significant risk of blood borne virus G asplenic patients, even after trivial transmission, offer to test person bitten: animal bites G patients with prosthetic implants e.g. heart valve, VP shunt Time Hepatitis C Hepatitis B HIV clotted sample for archiving at time of incident 6 weeks PCR HBsAg Antigen/antibody combined test 3 months PCR and antibody HBsAg Antigen/antibody combined test 6 months Antibody HBsAg Antigen/antibody combined test if PEP Anti-HBc antibody was given (anti-HBs antibody)* * Anti-HBs only needed at 6 months if vaccination only started at injury

Antibiotic prophylaxis

Type of bite Human, dog or cat † Specimen If clinical infection present, send tissue, aspirate or swab for bacterial culture If patient systemically unwell, blood cultures Treatment Tetanus vaccine [e.g. combined diphtheria (low dose), tetanus, and poliomyelitis] and immunoglobulin if indicated (see current BNFc) First line Alternative (penicillin allergy) Co-amoxiclav Clindamycin and cotrimoxazole Duration 5 days † For bites from other mammals, contact consultant microbiologist or consultant in infectious diseases

Issue 5 Issued: May 2013 152 Expires: May 2014 CERVICAL LYMPHADENOPATHY • 1/4

Enlargement of cervical lymph nodes G Axillae, supraclavicular and groin for >1 cm other nodes Acute lymphadenitis G Abdomen for hepatosplenomegaly DIFFERENTIAL DIAGNOSIS G Short history (usually <2 weeks) G Neck mass with features of acute Acute unilateral inflammation Subacute lymphadenopathy G Reactive G URTI ( Strep. pneumoniae ) G History variable G skin infection ( Group A Strep, Staph. G Often non-tender but with overlying aureus ) erythema G dental infection (anaerobes) Chronic lymphadenopathy G Kawasaki (see Kawasaki disease guideline) G Longer history (usually >1 month) G Cat scratch disease (Bartonella: G No feature of acute inflammation tender, axillary lymphadenopathy) HISTORY G Kikuchi-Fujimoto disease (histocytic necrotising lymphadenitis) Symptoms Acute bilateral

G Duration G Reactive G Symptoms of URTI G viral URTI G Fever G EBV, CMV (generalised G Weight loss lymphadendopathy, hepatosplenomegaly) G Night sweats G Eczema/skin infection Subacute G Bruising G Non-tuberculous mycobacteria (aged G Pallor <5 yr, unilateral, non-tender, purple, G Bone pain systemically well) G Pruritis G Mycobacterium tuberculosis G Social Toxoplasma gondii (generalised lymphadenopathy, fatigue, myalgia) G Contact with TB or cats Chronic G Travel G Reactive EXAMINATION G Neoplasia G Site of node(s) (see Figure 1 ) G lymphoma G Size of node(s) G leukaemia G ENT examination G soft tissue tumours G Skin – especially eczema G juvenile chronic arthritis G SLE

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INVESTIGATIONS Surgical excision biopsy

G See Flowchart G Atypical mycobacterial infection G Serology for Bartonella, toxoplasma, G Features highly suggestive of CMV and EBV neoplasia: G CXR G lymph nodes >3 cm diameter G Hilar lymphadenopathy significantly G all supraclavicular nodes increases likelihood of neoplastic G constitutional symptoms disease G hepatosplenomegaly G Ultrasound G generalised lymphadenopathy G high sensitivity and specificity for abscess formation in acute Children undergoing surgical lymphadenitis biopsy for suspected neoplastic G value in chronic lymphadenopathy for disease assessing size, site, shape and vascularity G FBC and film G CT only if suspected deep neck space G infection U&E, LDH, uric acid, LFTs G CXR

Figure 1 Node sites

AT: Anterior triangle PT: Posterior triangle SM: Submandibular triangle SC: Supraclavicular triangle LC: Lower cervical chain MC: Mid cervical chain UC: Upper cervical chain P: Parotid

Issue 5 Issued: May 2013 154 Expires: May 2014 CERVICAL LYMPHADENOPATHY • 3/4

Acute cervical lymphadenitis

G Systemically well No treatment, GP to Yes review in 2 weeks G <3 cm

G Single node >1.5 cm Kawasaki G Rash See Yes guideline G Peeling skin G Red eyes G Red lips, tongue

No

Co-amoxiclav oral Yes Hot, red, tender for 48 hr

No

Improved? Fluctuant G FBC, U&E, LFT, CRP G Blood cultures, throat swab No G Serology for Bartonella, EBV, CMV, toxoplasma* Yes USS

Solid

Continue co-amoxiclav ?atypical mycobacteria 10 days Pus Yes No

Refer to ENT

See Chronic cervical lymphadenopathy flowchart

* For storage pending repeat titre in chronic course

Issue 5 Issued: May 2013 Expires: May 2014 155 CERVICAL LYMPHADENOPATHY • 4/4

Chronic cervical lymphadenopathy

Clinical assessment

G All of: Does not meet either G Any of: G <1 cm criteria G >3 cm G mobile G supraclavicular G well child G constitutional symptoms G generalised LN G hepatosplenomegaly G CXR G USS neck G FBC & film G Serology for: G EBV, CMV, HIV G G toxoplasma CXR G G Bartonella FBC G G Co-amoxiclav for U&E 2 weeks

Review with results at 2 weeks

Yes Improved or No Refer to ENT for Discharge positive urgent surgical serology biopsy

Issue 5 Issued: May 2013 156 Expires: May 2014 FEVER • 1/4

This guideline applies until IDENTIFYING RISK OF underlying condition diagnosed, at SERIOUS ILLNESS which point treat child according to the guidance for that condition Three stages of clinical assessment ASSESSMENT AND INITIAL MANAGEMENT 1. Identify life-threatening features (utilising Airway, Breathing, Circulation G Fever, in child aged <5 yr, usually and Disability assessment) indicates underlying infection 2. Assess risk of serious illness (see G Parental perceptions of fever are Traffic light system for assessment ) usually accurate and must be taken 3. Attempt to identify source of seriously infection/features of specific serious conditions Traffic light system for assessment Low risk Intermediate risk High risk

Colour G Skin, lips and G Pallor reported by carer G Pale, mottled, ashen or tongue normal blue Activity G Responds to normal G Not responding normally to G No response to social social cues social cues cues G Is content or smiles G Wakes only with prolonged G Looks ill G Stays awake/wakes stimulation G Unrousable/doesn’t stay quickly G Decreased activity awake after rousing G Strong normal G No smile G Weak, high pitched or cry/settled/smiles continuous cry Respiration G Normal G Nasal flare G Grunting/nasal flare G Tachypnoea G Tachypnoea G respiratory rate ≥50/min G respiratory rate >60/min (aged <1 yr) (any age) G respiratory rate ≥40/min G Chest wall recession (aged >1 yr) (moderate/severe) G Oxygen saturation ≤95% G Crackles on auscultation Circulation G Normal skin and G Dry mucous membranes G Reduced skin turgor and eyes G Poor feeding (infants) Hydration G Moist mucous Age Heart rate (bpm) membranes <1 yr >160 1–2 yr >150 2–5 yr >140 G CRT ≥3 sec G Reduced urine output Other G No amber/red G Temperature ≥39ºC (aged G Temperature ≥38ºC features 3–6 months (aged <3 months) G Fever ≥5 days G Non-blanching rash G New lump >2 cm diameter G Bulging fontanelle G Swelling of joint/limb G Neck stiffness G Not using a limb/weight G Status epilepticus bearing G Focal neurological signs G Focal seizures G Bilious vomiting

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Assess: look for life-threatening, traffic light Child aged and specific diseases symptoms and signs Child aged <3 months – see table Traffic light system for ≥3 months assessment

If any amber If any red features If all green features G Observe and features and no and no diagnosis and no amber or red monitor: diagnosis reached reached G temperature G heart rate G respiratory rate G Perform urine test G Perform: G Perform: for urinary tract G G infection urine test for blood culture urinary tract G G full blood count Assess for infection symptoms and G urine test for G G Perform: signs of full blood count urinary tract G full blood count pneumonia G blood culture infection G C-reactive protein G Do not perform G C-reactive protein G C-reactive protein G blood culture routine blood G if fever >39°C G urine test for urinary tests or chest and white blood G Consider (guided tract infection X-ray cell count by clinical >20 x 10 9/L – assessment): G if respiratory signs chest X-ray present, chest X-ray G lumbar puncture G if child aged in children of all G if diarrhoea present, G If no diagnosis <1 yr, consider ages stool culture reached, manage lumbar puncture G chest X-ray child at home with irrespective of appropriate care white blood cell advice count and body G Advise parents/ G temperature Admit, perform carers when to G serum lumbar puncture and seek further electrolytes start parenteral attention from G antibiotics if child: healthcare blood gas G aged <1 month services G aged 1–3 months, appearing unwell G aged 1–3 months, with white blood cell count of <5 or >15 x 10 9/L Wherever possible, perform lumbar puncture before administration of antibiotics

G Consider admission according to clinical and social circumstances and treat – see Subsequent management G If child does not need admitting but no diagnosis has been reached, provide parent/carer with verbal and/or written information on warning symptoms and how to access further healthcare G e.g. signs of dehydration: sunken fontanelle/eyes, dry mouth, no tears; non-blanching rash G Liaise with healthcare professionals (including out-of-hours) to ensure parent/carer has direct access for further assessment of child

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Observations G Give immediate IV fluid bolus of sodium chloride 0.9% 20 mL/kg. Give G Measure and record in all febrile additional boluses as necessary children: G If signs of shock, SpO 2 <92% or G temperature clinically indicated, give oxygen aged <4 weeks: electronic G Urgent senior support: discuss with thermometer in the axilla PICU aged >4 weeks: infrared tympanic or G Septicaemia electronic thermometer in the axilla See guideline G respiratory rate, heart rate, capillary SUBSEQUENT MANAGEMENT refill time G signs of dehydration: skin turgor, G Serious bacterial infection suspected: respiratory pattern, weak pulse, cool G shock extremities G G travel history unrousable G Re-assess all children with amber or G meningococcal disease red features within 1 –2 hr G aged <1 month IMMEDIATE TREATMENT G aged 1–3 months with a white blood cell count <5 or >15 x 10 9/L Antipyretic treatment G aged 1–3 months appearing unwell G Tepid sponging not recommended G Ceftriaxone: <50 kg body weight or G Do not over or under dress a child aged <12 yr, 50 mg/kg once daily; with fever >50 kg or aged >12 yr, 1 g once daily G If child appears distressed or unwell, G if ceftriaxone contraindicated consider either paracetamol or (<41 weeks postmenstrual age; ibuprofen neonates with jaundice, G Do not routinely administer both drugs hypoalbuminaemia or acidosis; or on at the same time with the sole aim of IVI calcium) give cefotaxime (aged reducing fever or preventing febrile <1 month see BNFc for neonatal convulsions doses) G Alternate if distress persists or recurs G If no evidence of bacterial sepsis, stop before next dose due antibiotics 36 hr after time blood put in Antibiotics culture bottle G Decreased level of consciousness: G Do not prescribe oral antibiotics to consider meningitis and herpes children with fever without apparent simplex encephalitis source G give aciclovir: aged <3 months G if aged >3 months consider admission 20 mg/kg IV 8-hrly; aged >3 months– and observation 12 yr 500 mg/m 2; aged >12 yr 10 mg/kg Signs of shock IV 8-hrly G RSV/flu: assess for serious illness/UTI G Increased respiratory and heart rate, cold peripheries, prolonged capillary G If rates of antibacterial resistance are refill time, pallor/mottled, significant, refer to local policy drowsy/agitated/confused G See Septicaemia and Meningitis guidelines

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Symptoms and signs of Urinary tract infection specific diseases G Vomiting (in children aged >3 months) Meningococcal disease G Poor feeding G Lethargy G Non-blanching rash with one or more of the following: G Irritability G ill-looking child G Abdominal pain or tenderness G lesions >2 mm in diameter (purpura) G Urinary frequency or dysuria G CRT ≥3 seconds G Offensive urine or haematuria G neck stiffness Septic arthritis/osteomyelitis Meningitis G Swelling of a limb or joint G Neck stiffness G Not using an extremity G Bulging fontanelle G Non weight bearing G Decreased level of consciousness Kawasaki disease G Convulsive status epilepticus G Fever lasting >5 days and at least 4 of Herpes simplex encephalitis the following: G bilateral conjunctival injection G Focal neurological signs G change in upper respiratory tract G Focal seizures mucous membranes (e.g. injected G Decreased level of consciousness pharynx, dry cracked lips or strawberry tongue) Pneumonia G change in peripheral extremities (e.g. G Tachypnoea, measured as: oedema, erythema or desquamation) G G aged 0–5 months: respiratory rate polymorphous rash >60 breaths/min G cervical lymphadenopathy G aged 6–12 months: respiratory rate >50 breaths/min G aged >12 months: respiratory rate >40 breaths/min G Crackles in the chest G Nasal flaring G Chest indrawing G Cyanosis G Oxygen saturation ≤95%

Issue 5 Issued: May 2013 160 Expires: May 2014 FEVER OF UNKNOWN ORIGIN • 1/2

RECOGNITION AND G Bites (tick: arbovirus, malaria) ASSESSMENT G Meat: undercooked (brucella, toxoplasma, hepatitis) Fever G Pica (visceral larva migrans, toxoplasmosis) G Type of thermometer used, site, user Medical history (factitious) G Duration, height G Operations G Pattern: Drug history G intermittent [pyogenic, TB, lymphoma, juvenile idiopathic arthritis (JIA)] G All, including any non-prescription G baseline raised (viral, endocarditis, Ethnic group lymphoma) G sustained (typhoid) G Sephardic Jew, Armenian, Turkish, G days between (malaria, lymphoma) Arab (Familial Mediterranean Fever) G weeks between (metabolic, CNS, G Ashkenazi Jew (familial cyclic neutropenia, hyperIgD) dysautonomia) G Circumstances when fever (e.g. Examination exercise) G Appearance G Sinuses G when fever: well (factitious) G Lymph nodes G between fever: ill (serious) G Chest: murmur, crackles G Response to paracetamol and or G Abdominal: hepato/spleno-megally NSAID (no response: dysautonomia) (salmonella, cat scratch, endocarditis, malaria) Symptoms G Genito-urinary: girls – pelvic G Red eyes (Kawasaki) tenderness (child sex abuse – STI) G Nasal discharge (sinusitis) Skin G Recurrent pharyngitis with ulcers (periodic fever) G Rash only during fever (JIA) G GI: salmonella, intra-abdominal G No sweat (familial dysautonomia) abscess, inflammatory bowel disease G Petechiae (endocarditis, rickettsia) (IBD) G Papules (cat scratch) G Limb pain (leukaemia, osteomyelitis) G Eschar (tularaemia) Contact G Erythema migrans (Lyme) G Malar (SLE) G Human illness G Palpable purpura [polyarteritis nodosa G Animals (PAN)] Travel G Erythema nodosum (JIA, SLE, malignancy, IBD, TB) G Years ago (histoplasmosis) G Seborrheic (histiocytosis) G Part of country G Sparse hair (ectodermal dysplasia) G Prophylaxis and immunisations G Scars (dysautonomia) G Contaminated water/food

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Eyes G LFTs: abnormal (EBV, CMV) G Blood cultures: several times G Conjunctivitis: (endocarditis) G palpebral (infectious mononucleosis) G Urine: pyuria (Kawasaki, intra- G bulbar (Kawasaki) abdominal infection, GU, TB) G phlyctenular (TB) Selective G Retinopathy (PAN, miliary TB, toxoplasmosis, vasculitis) G Stool (if loose) G Pupil dilation (hypothalamic or G Bone marrow (leukaemia, histiocytic autonomic dysfunction) haemophagocytosis) G Oropharynx Serology (syphilis, brucella, EBV, CMV, toxoplasma, bartonella) G Red, no exudates (EBV) G Auto-antibodies (rheumatoid arthritis, G Dental abscess SLE) G Conical teeth (ectodermal dysplasia) G IgG, A & M (recurrent infections) G Smooth tongue (dysautonomia) G IgE (allergy, eosinophilia) G Gum hypertrophy, tooth loss G IgD (periodic fever) (leukaemia, histiocytosis) Imaging (selective) Musculoskeletal G Sinuses G Tender: G US/CT/MR abdo (IBD, abscess, G bone (osteomyelitis, malignancy) lymphadenopathy) G muscle (trichinella, arbovirus, G White cell scan (abscess) dermatomyositis, PAN) G Bone scan (osteomyelitis) G Trapezius (subdiaphragmatic abscess) G PET scan (abscess) G Reflexes Other investigations (selective) G brisk (hyperthyroid) G absent (dysautonomia) G Echo (endocarditis) G Ophthalmologist (uveitis, leukaemia) Investigations G Biopsy (lymph node, liver) All EMPIRICAL TREATMENT

G FBC, ESR, CRP, U&E, LFT, blood G Critically ill: no focus – ceftriaxone or culture, HIV antibody, urinalysis, urine cefotaxime (after blood and urine culture, CXR specimens taken) G TB treatment: after induced sputum, G FBC: lymph node biopsy, TB blood culture G low Hb (malaria, endocarditis, IBD, G Otherwise avoid antibiotics until SLE, TB) organism isolated G high platelets (Kawasaki) REFERRAL G blasts (leukaemia) G eosinophils (fungal, parasites, G Rheumatology (JIA, connective tissue neoplastic, allergic, immune disorder) deficiency) G Gastroenterology (IBD) G ESR/CRP: normal (factitious, G Cardiology (endocarditis/Kawasaki) dysautonomia, drug fever)

Issue 5 Issued: May 2013 162 Expires: May 2014 HEPATITIS • 1/1

Discuss all children with hepatitis B or C HEPATITIS C with infectious diseases team or regional liver unit for counselling, information, consideration for anti-viral therapy and Diagnostic tests need for referral (For neonates see Neonatal guidelines ) HEPATITIS B G Hepatitis C Virus (HCV) antibody (ab) aged >18 months old Diagnostic tests G HCV PCR if HCV ab +ve

G HBsAg (Hepatitis B surface antigen) Action and HBcAb (IgM and IgG) G G HBsAb (anti-HBs: antibody) indicates If HCV ab negative not infected. previous immunisation or infection Discharge G G If HBsAg positive then check HBeAg, If HCV ab positive and HCV PCR HBeAb, genotype and refer to regional negative in two samples taken liver unit 6 months apart, not infected (resolved infection or maternal antibody if aged Yearly follow-up <18 months). Discharge G If HCV PCR positive, check genotype G Clinical assessment and yearly bloods below, refer to G Serology (clotted specimen) regional liver unit G HBsAg Yearly follow-up G if previously HBeAg positive, HBeAg G HBeAb G Clinical assessment G Hepatitis B DNA PCR viral load G HCV PCR viral blood (EDTA) G LFT (bilirubin, ALT/AST, GGT, albumin) G LFT (bilirubin, ALT/AST, GGT, albumin) G FBC G FBC G Coagulation (INR, PT, PTT) G Coagulation (INR, PT, PTT) G Alpha-fetoprotein G Alpha-fetoprotein G Abdominal ultrasound (and fibroscan if G Abdominal ultrasound and fibroscan if available) available (yearly if eAg +ve; 5 yearly if eAb +ve or if rise in alpha-fetoprotein) Action

G If LFT or alpha-fetoprotein abnormal, or viral titres are rising, inform regional liver unit

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INDICATIONS FOR PEP

Source HIV status HIV positive HIV positive Unknown high Unknown low Viral load Viral load prevalence prevalence detectable undetectable group/area group/area Receptive anal sex Recommended Recommended Recommended Not recommended Insertive anal sex Recommended Not Consider Not recommended recommended Receptive vaginal sex Recommended Not Consider Not recommended recommended Insertive vaginal sex Recommended Not Consider Not recommended recommended Fellatio with ejaculation Consider Not Not Not recommended recommended recommended Fellatio without ejaculation Not Not Not Not recommended recommended recommended recommended Splash of semen into eye Consider Not Not Not recommended recommended recommended Cunnilingus Not Not Not Not recommended recommended recommended recommended Sharing of injective Recommended Not Consider Not equipment recommended recommended Human bite Not Not Not Not recommended recommended recommended recommended Needle-stick from a Not Not discarded needle in the recommended recommended community

Consider: recommend PEP if additional high risk factor for HIV PEP

G <40 kg: zidovudine, lamivudine and Kaletra ® G ≥40 kg: Truvada ® 1 tab daily and Kaletra ® 2 x (200/50 tab) 12-hrly G If source has drug-resistant virus, seek expert help G If patient known to have HIV do not give PEP G Start as soon as possible up to 72 hr after exposure

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HIV PEP drugs

Drug Dose Formulation Side effects Intake recommendation Zidovudine 180 mg/m 2/dose Caps. Neutropenia +/- Can be given (ZDV or AZT) (max 250 mg) 100, 250 mg anaemia, nausea, with food; 12-hrly Susp. 10 mg/mL headache, capsules can be hepatitis opened and myopathy, dissolved in neuropathy water Lamivudine (3TC) 4 mg/kg/dose Tab. Peripheral Can be given 100, 150 mg; 12-hrly; max neuropathy, with food Susp. 10 mg/mL; nausea, dose 150 mg 5 mg/mL (room 12-hrly diarrhoea, temp) headache Truvada ® >40 kg Tab. 300 mg Headache, Can be given with or without (TDF and FTC) 1 tab daily Tenofovir (TDF) diarrhoea, food 200 mg nausea, renal tubular Emticitabine (FTC) dysfunction Kaletra ® 300 mg LPV/m 2 + Tab 200 mg LPV/ Diarrhoea, Give with or after 2 food [lopinavir (LPV)/ 75 mg RTV/m 50 mg RTV headache, ritonavir (RTV)] 12-hrly Paed tab 100 mg nausea, vomiting 15–25 kg: 2 x LPV/25 mg RTV Caution in liver 100/25 tab 12-hrly disease 25–35 kg: 3 x Liq 5 mL = 400 mg 100/25 tab 12-hrly LPV/100 mg RTV >35 kg: 2 x 200/50 tab 12-hrly

HEPATITIS PEP G letter for GP G After sexual exposure consider If HIV PEP indicated give emergency contraception and screen for other sexually transmitted infections G Hepatitis B vaccine accelerated course G Arrange HBV, HCV and HIV antibody (0, 1, 2 and 12 months) test baseline and 3 months after G Hepatitis B immunoglobulin only if exposure source known to be HBsAg +ve G Check FBC, U&Es and LFTs if starting FOLLOW-UP PEP G Check need for tetanus immunisation G Before discharge, provide families G If source is HCV RNA PCR positive, embarking on HIV PEP with: arrange the following enhanced HCV G appointment to see a paediatrician follow-up: with experience in antiretroviral drugs G at 6 weeks: EDTA blood for HCV PCR or member of ID/GUM team the same G at 12 weeks: EDTA blood for HCV day or next working day PCR and clotted blood for anti-HCV G contact telephone number in case of antibodies concerns about any aspect of HIV G at 24 weeks: clotted blood for PEP anti-HCV antibodies G enough antiretroviral medication to last until clinic appointment

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INTRODUCTION Pre-test discussion with parents and children able to G HIV is a treatable medical condition give consent G The majority of those living with the virus are well G Purpose of pre-test discussion is to establish informed consent and should G Many are unaware of their HIV infection cover: G Late diagnosis is life-threatening G benefits of testing G HIV testing can be done in any medical G setting and health professionals can details of how result will be disclosed obtain informed consent for an HIV test G Lengthy pre-test HIV counselling is in the same way they do for any other not a requirement medical investigation G Document patient’s consent to testing HOW G If patient refuses test, explore why and ensure decision has not resulted from incorrect beliefs about the virus Who can test? or consequences of testing G Doctor, nurse, midwife or trained G Advise that, if negative, testing will not healthcare worker affect patient’s insurance G Who should be offered a test? Some patients, (e.g. those whose first language is not English) may need additional help to reach a decision G First-line investigation for suspected immune deficiency: unusual type, G Document the offer of an HIV test in severity or frequency of infection. See medical notes, together with any Table 1 relevant discussion and reasons for refusal G Take a sexual history in post-pubertal children G Written consent not necessary but record on laboratory request form Primary HIV infection that consent has been obtained G Arrange appointment for result to be G Symptoms typically occur 2–4 weeks disclosed personally by testing clinician after infection: G fever POST-TEST G rash (maculopapular) G myalgia HIV negative result: post-test discussion G pharyngitis G headache/aseptic meningitis G If still within window period after a G Resolve spontaneously within specific exposure, discuss need to 2–3 weeks repeat test G Source patient in a needlestick for definitive exclusion of HIV infection a further test after three months is injury or other HIV risk exposure recommended G Consent must be obtained from G If reported as reactive or equivocal, source patient before testing refer to infectious diseases (may be G The person obtaining consent must be seroconversion) a healthcare worker, other than person who sustained the injury

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HIV positive result: post-test G Testing clinician must give result discussion personally to patient in a confidential environment and in a clear and direct G For all new HIV positive diagnoses, manner carry out appropriate confirmatory G arrange follow-up programme with assays and test a second sample infectious diseases before informing patient of positive result

Table 1: Clinical indicator diseases for HIV infection AIDS-defining conditions Others where testing should be offered Respiratory Pneumocystis pneumonia Bacterial pneumonia Tuberculosis Aspergillosis Neurology Cerebral toxoplasmosis Aseptic meningitis Primary cerebral lymphoma Space occupying lesion of unknown cause Cryptococcal meningitis Guillain-Barré syndrome Progressive multifocal Transverse myelitis leucoencephalopathy Peripheral neuropathy Dementia Leucoencephalopathy Dermatology Kaposi’s sarcoma Severe/recalcitrant seborrhoeic dermatitis/ psoriasis Multidermatomal or recurrent herpes zoster Gastroenterology Persistent cryptosporidiosis Persistent/recurrent oral candidiasis Oral hairy leukoplakia Chronic diarrhoea/weight loss of unknown cause Salmonella, shigella or campylobacter Hepatitis B/C infection Oncology Non-Hodgkin’s lymphoma Hodgkin’s lymphoma Castleman’s disease Haematology Any unexplained blood dyscrasia Ophthalmology Cytomegalovirus retinitis Infective retinal diseases ENT Lymphadenopathy of unknown cause Chronic parotitis Lymphoepithelial parotid cysts Other Mononucleosis-like syndrome Pyrexia of unknown origin Anyone with a mother who is HIV +ve no matter what age Anyone who has a partner who is HIV +ve Men who have sex with other men Female sexual contacts of men who have sex with men Patients reporting use of injecting drugs Anyone from a country of HIV prevalence >1% Anyone who has had sex in a country of HIV prevalence >1% Anyone who has had sex with someone from a country of HIV prevalence >1% All pregnant women

Issue 5 Issued: May 2013 Expires: May 2014 167 IMMUNODEFICIENCY • 1/2 RECOGNITION AND G High risk group for HIV and no ASSESSMENT antenatal HIV test (a –ve antenatal HIV test does not exclude HIV in the child) G SPUR to recognition: Serious, G Autoimmune liver disease, diabetes, Persistent, Unusual, or Recurrent vasculitis, ITP infections G Poor wound healing G The younger the onset, the more life- G Unexplained bronchiectasis or threatening the immune defect likely pneumatoceles to be G >1 unexpected fracture G bacterial infection; early presentation: antibody defect Signs of immune deficiency G viral/fungal infection; later G Congenital abnormalities: dysmorphic presentation: cellular defect features, congenital heart disease, Warning signs of primary situs inversus, white forelock, immunodeficiency: albinism, microcephaly G Children who appear chronically ill G ≥8 new bacterial ear infections G Scarring or perforation of tympanic G ≥2 serious sinus infections membranes from frequent infection G ≥2 months on antibiotics without G Periodontitis resolution of symptoms G Enlargement of liver and spleen G ≥2 episodes of pneumonia G Hypoplastic tonsils and small lymph G Failure to thrive with prolonged or nodes recurrent diarrhoea G Lymphadenopathy G Recurrent, deep skin or organ abscess G Skin: telangiectasia, severe eczema, G Persistent candida in mouth or napkin erythroderma, granuloma, acneiform area rash, molluscum, zoster G Failure of IV antibiotics to clear G Ataxia infections Other investigations suggestive G ≥2 severe infections (e.g. meningitis, osteomyelitis, cellulitis or sepsis) of immune deficiency G Family history of primary G Haemolytic anaemia immunodeficiency G Neutropenia Symptoms of immune G Eosinophilia deficiency G Hypocalcaemia

G Delayed umbilical cord separation of Unusual organisms suggestive ≥3 weeks, omphalitis of immune deficiency G Delayed shedding of primary teeth G Viruses: CMV, EBV, VZV, warts G Severe adverse reaction to G Fungi: candida, aspergillus, immunisation e.g. BCGitis cryptococcus, pneumocystis, nocardia G Unusually severe course of measles G Protozoa: cryptosporidium, toxoplasma or chickenpox G Bacteria: salmonella, giardia, G Family history of any syndrome mycobacterium (inc BCG), serratia associated with immunodeficiency, G (e.g. DiGeorge anomaly or Wiskott- Recurrent infection with common organisms: H. influenzae, Aldrich syndrome); or of death during S. pneumoniae, N. meningitidis, early childhood S. aureus

Issue 5 Issued: May 2013 168 Expires: May 2014 IMMUNODEFICIENCY • 2/2 Table 1: Investigations

Investigations Sample Volume Minimum Ideal Initial tests (complete all tests for any suspected immune deficiency) FBC (note absolute lymphocyte EDTA 1.3 mL 4 mL count) and ESR IgG, IgM, IgA Clotted 0.5 mL 4 mL IgG function (antibody response to Clotted 0.5 mL 4 mL tetanus, Hib +/- pneumococcus) Retest 4 weeks after vaccination HIV antibody Clotted 0.5 mL 4 mL Second-line tests (with immunology advice) Lymphocyte subsets EDTA 1 mL 4 mL Haematology

Lymphocyte proliferation Lithium heparin Discuss with local immunology centre Enzyme assay (ADA, PNP) EDTA 3.5 mL 1 mL Discuss with Guy’s Hospital Neutrophil function test for CGD EDTA or 0.25 mL 4 mL lithium heparin Discuss with local immunology centre Adhesion molecule assay EDTA 0.25 mL 4 mL Discuss with local immunology centre CH50 & Complement components Clotted 1 mL to reach 4 mL to reach (if recurrent or case with family lab within 2 hr lab within 2 hr history of meningococcal disease) or separate and freeze immediately

SUBSEQUENT MANAGEMENT G In patients with B-cell, T-cell or phagocytic defects, request regular G Avoid live vaccines (e.g. BCG, MMR pulmonary function tests and home and varicella) treatment plan of physiotherapy and inhalation therapy similar to that used G Ensure that any blood products given in cystic fibrosis to patients with suspected or proven G In children with significant primary or T-cell immunodeficiency are irradiated secondary cellular (T-cell) and CMV negative immunodeficiency (e.g. aged <1 yr G For specific infections, use same CD4 <25%, aged 1–5 yr CD4 <15% or antibiotics as in immunocompetent aged >5 yr <200 CD4 cells/mm 3), give patients, at higher recommended Pneumocystis jiroveci (PCP) dosage prophylaxis with co-trimoxazole G Obtain throat, blood and other culture specimens before starting treatment G Treat infectious episodes for longer than usually recommended (approximately double)

Issue 5 Issued: May 2013 Expires: May 2014 169 KAWASAKI DISEASE • 1/3

G LFT’s, CRP Early treatment reduces mortality G from coronary artery aneurysms ECG G If full diagnostic criteria found, RECOGNITION AND echocardiogram not required until ASSESSMENT 6 weeks from onset of signs and symptoms Symptoms and signs G If criteria incomplete or presentation atypical, aneurysms on echo are G Fever for at least 5 days and 4 of the diagnostic: discuss with cardiologist following: G Throat swab for Gp A strep G bilateral non-exudative conjunctival G Anti-streptolysin O titre (ASOT) or infection anti-DNase B for evidence of G oral changes (red lips/pharynx/tongue) streptococcal infection G peripheral oedema followed by G Blood culture desquamation 10–15 days after onset G Urinalysis, microscopy and culture of fever G If rash present, serology for G polymorphous rash enterovirus, parvovirus, EBV, CMV; if G acutely enlarged cervical lymph nodes features of measles urine or throat with individual node(s) >1.5 cm swab in viral transport medium for PCR diameter IMMEDIATE TREATMENT G absence of another diagnosis e.g. group A streptococcal infection (GAS), G Aspirin 7.5–12.5 mg/kg oral 6-hrly until measles afebrile or a minimum of 2 weeks G The presence of a coronary artery G Intravenous immunoglobulin (IVIG) aneurysm with any one of the above 2 g/kg features is diagnostic G check concentration (g/mL) for Other features preparation used in your Trust Administration of 100 mg/mL G Most common in children aged <5 yr (e.g. Flebogamma ® DIF) G Atypical cases may not fulfil all the Rate* Duration above criteria 0.6 mL/kg/hr = ...... mL/hr 30 min G Fever usually precedes the other 1.2 mL/kg/hr = ...... mL/hr 30 min signs and is characteristically 2.4 mL/kg/hr = ...... mL/hr 30 min unresponsive to antipyretics 3.6 mL/kg/hr = ...... mL/hr 30 min G Other symptoms include irritability, 4.8 mL/kg/hr = ...... mL/hr To completion aseptic meningitis, uveitis, cough, vomiting, diarrhoea, abdominal pain, * up to a maximum rate of 180 mL/hr urethritis, arthralgia and arthritis Investigations

None is diagnostic G FBC: platelets often high for 10–15 days after onset of fever G ESR

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Product Infusion rates Infusion time of 70 g in minutes Initial Maximum at maximum rate Baxter Kiovig 0.5 mL/kg/hr 6 mL/kg/hr 100 for 30 min (8 mL/kg/hr in PID) BPL Gammaplex 0.01–0.02 mL/kg/min 0.04–0.08 mL/kg/min 250 for 15 min BPL Vigam 0.01–0.02 mL/kg/min 0.04 mL/kg/min 500 for 30 min (max 3 mL/min) Biotest Intratect 1.4 mL/kg/hr 1.9 mL/kg/hr 640 for 30 min CSL Privigen 0.3 mL/kg/hr 4.8 mL/kg/hr 125 (7.2 mL/kg/hr in PID) Grifols 0.01–0.02 mL/kg/min 0.1 mL/kg/min 200 Flebogamma 5 for 30 min Grifols 0.01 mL/kg/min 0.08 mL/kg/min 125 Flebogamma 10 for 30 min Octapharma 1 mL/kg/hr for 5 mL/kg/hr 241 Octagam 5 30 min Octapharma 0.01–0.02 mL/kg/min 0.12 mL/kg/min 83 Octagam 10 for 30 min

SUBSEQUENT MANAGEMENT Start IVIG as soon as possible (delayed treatment increases risk G If fever persists 36 hr after completion of aneurysm) of IVIG, consider a single repeat dose of IVIG MONITORING IVIG INFUSION G If fever persists after second dose IVIG give intravenous pulse G Monitor temperature, heart rate, BP methylprednisolone, 30 mg/kg over and respiratory rate: 2–3 hr, administered once daily for G every 5 min for first 15 min 1–3 days with BP monitoring 4-hrly G then every 15 min for first hour G After 2 weeks, reduce dose of aspirin to 5 mg/kg oral as single daily dose G Anticipate anaphylaxis, flushing, fever, for 6 weeks (until result of headache, shivering echocardiogram known) G If tolerated, increase infusion rate to give total dose over remaining 10 hr and monitor hourly G If mild reaction, stop infusion for 15 min then restart at slower rate

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DISCHARGE AND FOLLOW-UP

G Discharge when fever settles G Echocardiogram at 6 weeks from onset of signs and symptoms G Out-patient appointment 1 week after echocardiogram G Advise to avoid excessive strenuous activity until out-patient appointment after echocardiogram G Advise to avoid all live vaccines (e.g. MMR) for 3 months following IVIG therapy OUT-PATIENT MANAGEMENT

G No aneurysms at 6 weeks echocardiogram G stop aspirin G no restriction on activity G Single aneurysm <7 mm diameter G aspirin 3–5 mg/kg (max 75 mg) once daily until aneurysm disappears G cardiologist will advise on limitation of activity G annual echocardiogram G Multiple or giant aneurysm G avoid strenuous activity G discuss need for anticoagulation, stress test and repeat echocardiogram with cardiologist

Issue 5 Issued: May 2013 172 Expires: May 2014 MALARIA • 1/3

Falciparum is a medical emergency: immediate treatment is essential

G Test for malaria in anyone with fever G who has travelled to a malarial area within last 12 months G or febrile infant whose mother has travelled to a malarial area in pregnancy Clinical features

Non-specific Severe (complicated) malaria G Fever G Persistent vomiting, severe dehydration G Malaise G Shock, renal failure (oliguria <0.5 mL/kg/hr) G Headache G Depressed conscious state, seizures G Sweating G Tachypnoea or increased work of breathing

G Diarrhoea G Hypoxia (SpO 2 <95%) G Vomiting G Metabolic acidosis (base deficit >8) G Abdominal pain G Severe hyperkalaemia (K >5.5 mmol/L) G Splenomegaly G Hypoglycaemia <3 mmol/L G Anaemia G Severe anaemia (<80 g/L) G Thrombocytopenia G Unable to walk G Jaundice G Parasitaemia >2% or schizonts on film

Investigations SEVERE (COMPLICATED) MALARIA G EDTA blood sample sent to haematology for an urgent thick blood Anti-malaria treatment film G 3 blood films 12 hr apart G Quinine dihydrochloride IV diluted to 2 mg/mL with sodium chloride 0.9% or G Negative malaria ICT (stix test) does glucose 5% not exclude malaria G loading dose 20 mg/kg max 1.4 g as G Do not treat unless proven on blood infusion over 4 hr (NEVER as IV bolus) test G omit loading dose if mefloquine or G Admit all patients with falciparum to a quinine used in previous 24 hr unit with experience in managing G glucose stix 2-hrly during IV quinine, severe malaria (e.g. infectious disease cardiac monitor and daily ECG (check unit) QTc) G Opportunistic screen for other G then 8 hr after start of loading dose, imported diseases: hepatitis B, HIV 10 mg/kg infusion (max 700 mg) over 4 hr every 8 hr If malaria is diagnosed on G when able to swallow give Malarone ® blood film, but type unclear, (see Treatment of uncomplicated treat as falciparum malaria falciparum malaria below) G daily FBC, U&E and blood films as in-patient until asexual parasites undetectable

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G If parasitaemia >15% or from area of G Shock: add cefotaxime quinine resistance (Thai/Cambodia G Hypoglycaemia: common, give border, Papua New Guinea) or history glucose 10% 2 mL/kg IV bolus then of arrhythmias, discuss with ID glucose 10% 5 mL/kg/hr with sodium specialist about artesunate instead of chloride 0.45% quinine G Anaemia: common, transfuse if Hb G Artesunate 2.4 mg/kg IV [in 1 mL <80 g/L sodium bicarbonate (vial provided with G Thrombocytopenia: expected, drug), dilute further in 5 mL glucose 5% transfuse only if bleeding and platelets and inject over approximately 2 min] at <20 x 10 9/L 0, 12 and 24 hrs and then daily G When parasitaemia resolving and CEREBRAL MALARIA patient improving, switch to oral agent: Impaired level of consciousness G Malarone ®, or if resistance suspected G Correct hypoglycaemia Riamet ®, or oral quinine (if neither G other agent available) Monitor GCS, reflexes, pupils G Plan for intubation and transfer to Complications PICU if: G signs of raised ICP G Renal failure: discuss early filtration/dialysis with PICU G persisting shock after 40 mL/kg fluid G Hypovolaemia: cautious rehydration G or pulmonary oedema (high risk pulmonary oedema) TREATMENT OF UNCOMPLICATED FALCIPARUM MALARIA (no clinical features of severe malaria) G If child can tolerate oral intake (can be crushed): G Malarone ® (proguanil with atovaquone) once a day for 3 days

Weight (kg) 5–8 9–10 11–20 21–30 31–40 >40 Dose 2 paed 3 paed 1 adult 2 adult 3 adult 4 adult tablets tablets tablet tablets tablets tablets Paediatric tablet contains proguanil 25 mg and adult tablet 100 mg No second agent required Or Riamet ® (artemether with lumefantrine) Weight (kg) Dose 5–15 1 tablet initially followed by 5 further doses of 1 tablet each given at 8, 24, 36, 48 and 60 hrs (total 6 tablets over 60 hr) 16–25 2 tablets initially followed by 5 further doses of 2 tablets each given at 8, 24, 36, 48 and 60 hrs (total 12 tablets over 60 hr) 26–35 3 tablets initially followed by 5 further doses of 3 tablets each given at 8, 24, 36, 48 and 60 hrs (total 18 tablets over 60 hr) >35 4 tablets initially followed by 5 further doses of 4 tablets each given at 8, 24, (12–18 yr) 36, 48 and 60 hrs (total 24 tablets over 60 hr) No second agent required Or

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Quinine sulphate

G 10 mg/kg (max 600 mg) oral 8-hrly G Reduce to a 12-hrly regimen if severe cinchonism (severe tinnitus, deafness, unsteadiness) G Mild tinnitus and feeling of ‘blocked’ ears are expected on quinine and resolve once therapy completed G Continue until blood films negative or for a 7 day course (whichever is the longer). A shorter course may be possible but only at infectious diseases consultant’s discretion Weight (kg) Paediatric dosing of oral quinine sulphate

1 5–7 50 mg ( /4 x 200 mg tablet) 1 8–12 100 mg ( /2 x 200 mg tablet) 3 13–17 150 mg ( /4 x 200 mg tablet) 18–22 200 mg (1 x 200 mg tablet) 1 1 23–27 250 mg ( /2 x 300 mg + /2 x 200 mg tablet) 28–39 300 mg (1 x 300 mg tablet) 40–49 400 mg (2 x 200 mg tablet) 50–60 500 mg (1 x 200 mg tablet and 1 x 300 mg tablet) >60 600 mg (2 x 300 mg tablet)

G With quinine give second agent G Then give primaquine G aged >12 yr doxycycline 200 mg 250 microgram/kg oral (max 15 mg) once/day for 7 days daily for P. ovale and 500 microgram/kg (max 30 mg) daily for P. G aged <12 yr clindamycin 7–13 mg/kg vivax for 14 days (max 450 mg) 8-hrly for 7 days G Liquid nivaquine 68 mg/5 mL is If in doubt treat as severe equivalent to 50 mg/5 mL chloroquine (complicated) malaria base G Itch is common, does not respond to NON-FALCIPARUM MALARIA antihistamines, if severe give quinine G Complications are rare Before giving primaquine, check G Usually sensitive to chloroquine and review G6PD concentration, (chloroquine-resistant P. vivax reported as severe haemolysis can occur in Indonesia, New Guinea and some adjacent islands) if G6PD-deficient Treatment of non-falciparum G6PD-deficient patients malaria

G If chloroquine resistance suspected G In mild G6PD-deficiency, primaquine then refer to non-complicated 750 microgram/kg (max 30 mg) once falciparum management a week for 8 weeks G Chloroquine 10 mg (base)/kg oral G Otherwise contact ID specialist initial dose (max 620 mg) G then 5 mg/kg (max 310 mg) after 6 hr, then once daily for 2 days

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RECOGNITION AND G Other intracranial sepsis ASSESSMENT G Encephalitis G Systemic sepsis If aged <28-days-old see Neonatal G meningitis in Neonatal Guidelines Malaria in travellers G Other causes of confusion or raised Symptoms may be non-specific: intracranial pressure if meningitis considered, LP INVESTIGATIONS Symptoms and signs Lumbar puncture G Pyrexia G Petechial rash If any doubt about need for G Evidence of raised intracranial lumbar puncture (LP) discuss pressure in the older child with consultant disc oedema (often late sign), any G Perform LP before giving antibiotics if localising neurological features, child stable, do not delay antibiotics reduced conscious level by >1 hr G Neck stiffness G Discuss with consultant if any of G Kernig’s sign positive following: G Irritability G signs suggesting raised intracranial G Focal neurological signs including pressure squints - GCS <9 or drop ≥3 G Infants: - relative bradycardia and poor feeding hypertension vomiting - focal neurological signs irritability - abnormal posture or posturing fever - unequal, dilated or poorly responsive fits pupils full fontanelle (unless dehydrated) - papilloedema G Older child may also have: - abnormal ‘doll’s eye’ movements G severe headache shock photophobia G extensive or spreading purpura confusion G after convulsion until stabilised lower backache G coagulopathy: on anticoagulants or if already obtained platelets Differential diagnosis <100 x 10 9/L or INR >1.4 or suspected (e.g. purpuric rash) G If rash or severely ill, see Septicaemia (including G local infection over lumbar spine meningococcal) guideline G respiratory insufficiency G Look for signs of viral meningitis e.g. G If LP initially contraindicated, perform resolving mumps LP as soon as no longer contraindicated to confirm diagnosis G It is not possible to differentiate viral from bacterial meningitis clinically

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G Repeat LP if: Specimens G no clinical response after 48 hr of therapy G One fluoride tube (and 4 CSF bottles) G re-emergent fever G If tap traumatic, may need more G deterioration samples G persistent abnormal inflammatory G If insufficient CSF discuss priorities markers with microbiology Table 1: Collection of specimens (stated volumes represent minimum required) Department Specimens (6 drops = approx 0.2 mL) Biochemistry G 0.2 mL in a fluoride tube for glucose (also send blood glucose) G 0.2 mL in a CSF bottle for protein G 0.2 mL for lactate if metabolic disorder suspected Microbiology G 0.2 mL in a CSF bottle for MC&S G 0.5 mL for meningococcal and pneumococcal PCR G 1 mL for TB culture if high clinical suspicion of TB meningitis Virology If possible viral meningitis or encephalitis: G 0.5 mL for herpes simplex virus, enterovirus and VZV PCR G 0.3 mL for Human Herpes Virus 6 if rash, high temperature or rapid recovery Save G 0.5 mL plain bottle for additional neurology tests (e.g. oligoclonal bands) depending on other results and progress

Interpretation of cerebrospinal G CSF glucose normally about 1 mmol/L fluid results below serum level G CSF glucose likely to be depressed in G White cell count showing bacterial meningitis including TB polymorphonuclear leucocytosis (lymphocytosis) or usually indicative of bacterial G herpes or mumps encephalitis meningitis but can also be seen in G CSF glucose usually normal in viral viral meningitis meningitis G lymphocytosis can occur in partially- treated pyogenic, TB and viral CT scan meningitis and inflammatory G Not routine, does not exclude raised conditions intracranial pressure G in neonates with no specific signs of G indicated if GCS <9 or focal meningitis up to 20 cells/microlitre neurological signs: if no space may be normal (but treat as meningitis occupying lesion or sign of raised if symptoms suggestive of meningitis intracranial pressure then LP if no e.g. fitting if any neutrophils) other contraindication G older children treat as meningitis if >5 G do not delay treatment for CT white cells or >1 neutrophil/microlitre G stabilise before, and monitor closely G Protein usually elevated in bacterial during CT meningitis (upper limit of normal is 20 mg/L in infants and children, and Other up to 60 mg/L in neonates) G FBC and differential WBC G very high levels of CSF protein are G sometimes seen in TB meningitis Blood cultures before start of antibiotics

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G U&E, glucose and CRP G if suggestive of herpes encephalitis G If meningococcal disease suspected, (period of decreased level of refer to Septicaemia (including consciousness with fever or focal meningococcal) guideline; send EDTA signs) add aciclovir IV 8-hrly: aged blood for meningococcal DNA PCR <3 months 20 mg/kg; aged 2 G Viral titres plus mycoplasma IgM if 3 months–12 yr 500 mg/m ; aged indicated or store to assay if other >12 yr 10 mg/kg results negative G If suggestive of TB (contact with TB, G Stool or rectal swab and throat swab other features of TB, long history), for enteroviruses, if viral meningitis discuss with infectious diseases team suspected G If recent travel outside UK, or prolonged or multiple antibiotics in last IMMEDIATE TREATMENT 3 months add vancomycin G Corticosteroids If definite history of anaphylaxis to cephalosporin give chloramphenicol IV G Give in children aged >3 months with: or vancomycin/teicoplanin and gentamicin if chloramphenicol not G frankly purulent CSF immediately available G CSF WBC count >1000/microlitre G If unusual cause suspected, contact G raised CSF WBC count and protein infectious diseases greater than 1 g/L team/microbiologist G bacteria on Gram stain Anticonvulsants G Do not give in septic shock, meningococcal disease, G Drugs of choice if child has seizures immunocompromised patients or if (prophylaxis not recommended): post-operative G phenytoin G If TB meningitis suspected, discuss with infectious diseases team before G lorazepam for acute control giving Other supportive measures G Dexamethasone sodium phosphate 150 microgram/kg (max 10 mg) IV G If child shocked, give human albumin 6-hrly for 4 days 4.5% or if not immediately available G first dose before antibiotics or as soon sodium chloride 0.9%: as possible up to 12 hr afterwards G initial dose 20 mL/kg over 5–10 min Antibiotics and reassess Do not give excessive fluid Start immediately without waiting boluses: risk of cerebral oedema for identification of organisms or sensitivities Intensive care G Ceftriaxone or cefotaxime G Inform PICU if: G infants aged <3 months, add G depressed conscious level amoxicillin to cover listeriosis G G infants aged <3 months, ceftriaxone shock does not respond to initial may be used but not in premature resuscitation babies or in babies with jaundice, hypoalbuminaemia or acidosis

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MONITORING TREATMENT Fluid restriction

G In a semi-conscious patient, monitor G Maintenance fluids: sodium chloride hourly until improvement evident: 0.9% with glucose 5% with potassium G respiratory rate chloride 10 mmol/500 mL if not hyperkalaemic G pulse and BP G Restrict fluid to 80% maintenance if: G level of consciousness and pupils G severe illness G in young infants, measure head circumference daily G hyponatramia G If persistent pyrexia look for other foci G raised intracranial pressure G repeat blood cultures and other G Measure urine and plasma investigations according to signs osmolalities daily whilst severely ill G CT scan at 10 days for microabscess Public health or hypodensity G if CT normal, repeat LP G Inform Public Health consultant of a case of suspected meningitis SUBSEQUENT MANAGEMENT G Public Health Department will arrange prophylaxis for close contacts Length of antibiotic course G Meningococcal meningitis G Meningococcus: 7 days G if ceftriaxone given as treatment, eradication treatment not required for G Haemophilus influenzae : 10 days patient G Pneumococcus or Group B G Close contacts (all ages): ciprofloxacin Streptococcus: 14 days single dose G Gram-negatives: 21 days G Haemophilus influenzae G Listeria: 21 days (with gentamicin for G close contact aged <10 yr, give first 7 days) rifampicin 20 mg/kg oral once daily for G No organism identified: aged 4 days >3 months, 10 days; aged <3 months, 14 days DISCHARGE AND FOLLOW-UP G Other, discuss with microbiologist G Organise formal hearing test 6 weeks Steroids after discharge from hospital G If severely ill during admission, Continue dexamethasone if: discuss with consultant about follow- G CSF WCC >1000/microlitre up to monitor developmental progress G CSF protein >1 g/L G If viral cause unconfirmed but still possible, repeat viral titres 6 weeks G Bacteria on Gram stain or culture after day of admission G If >1 episode of meningococcal disease, not serogroup B, recurrent serious bacterial infections or family history of meningococcal disease or immune deficiency, refer to immunology or infectious diseases

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URGENT NOTIFICATION G Legionnaires’ G Leprosy G Urgent out-of-hours notifications (to be G Malaria followed by normal paper notification G later) Measles* G G meningitis (suspected bacterial) Meningitis (viral, bacterial or fungal) G G meningococcal infection (clinical Meningococcal disease diagnosis) G Mumps G haemolytic uraemic disease G Paratyphoid fever (suspected) G Plague G infectious bloody diarrhoea G Poliomyelitis NOTIFIABLE DISEASES G Rabies G Rubella* Admitting doctor is required to notify G Severe acute respiratory syndrome suspected or confirmed cases of the (SARS) following to Health Protection Unit: G Scarlet fever* G Cluster or outbreak suspected (two or more cases epidemiologically linked) G Smallpox G Any other case where the potential for G Tetanus transmission is significant (e.g. highly G Tuberculosis* infectious) G Typhoid fever G Where contacts are particularly G Typhus susceptible (e.g. healthcare worker, G school) Viral haemorrhagic fever G G Where public health action is known to Whooping cough* be effective (e.g. prophylaxis, G Yellow fever immunisation) G Other infections or contaminations *Definitions (e.g. chemical) not listed below if G Food poisoning or suspected food potential risk of further harm poisoning: inform public health if G Anthrax acquired abroad or if family member is a food handler or healthcare worker G Botulism G Measles: fever, maculopapular rash G Brucellosis for ≥3 days and two or more of G Cholera following: Koplik’s spots, coryza, G Diphtheria conjunctivitis, raised measles IgM, measles encephalitis or pneumonitis. G Diarrhoea, infectious bloody Inform public health of MMR or G Encephalitis measles vaccination history. Do not G Food poisoning* bring children with suspected measles in primary care to hospital for G Group A streptococcal invasive disease diagnosis, only if hospital based treatment required or if G Haemolytic uraemic syndrome immunocompromised: arrange for G Hepatitis (viral) immediate isolation on arrival

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G Rubella: rash and occipital CONTACT DETAILS lymphadenopathy or arthralgia (if not or parvovirus), congenital rubella or G Contact details for your nearest HPU raised IgM to rubella. Inform public can be found on the Health Protection health of MMR vaccine history Agency website G Scarlet fever: tonsillitis, fever, rash (www.hpa.org.uk/ProductsServices/ with either culture of Streptococcus LocalServices) pyogenes from throat or raised ASO G Template for reporting procedures or anti-DNaseB titre under topics/notifiable/reporting G Tuberculosis: diagnosed clinically, procedures not just microbiologically (atypical mycobacterial infection or patients given chemoprophylaxis but not thought to have TB are not notifiable) G Whooping cough: cough with a whoop, with history of contact with similar illness or positive pernasal swabs for Bordetella pertussis or raised IgM to B. pertussis in an adult or child. Inform public health of pertussis immunisation history Non-statutory notifiable diseases

It has been agreed that, although they are not statutorily notifiable, the following diseases will nevertheless be reported to the consultant in communicable disease control: G AIDS/HIV infection G Legionnaires’ disease G Listeriosis G Psittacosis G Cryptosporidiosis G Giardiasis G Creutzfeldt-Jakob disease and other prion diseases

Issue 5 Issued: May 2013 Expires: May 2014 181 ORBITAL CELLULITIS • 1/1 RECOGNITION AND ASSESSMENT

Preseptal Orbital Facial erythema and tenderness Painful eye movements Normal eye movements Orbital pain and tenderness Normal vision Visual impairment (red-green colour differentiation lost early) Preceding superficial trauma Proptosis Eye pain Chemosis Periorbital swelling Ophthalmoplegia Fever Preceding sinusitis Definition G if improving, convert to oral high dose co-amoxiclav G Infection of soft tissues surrounding G if penicillin allergy give clindamycin the eye G Total duration of treatment (including Complications IV) 14 days

G Intracranial abscess Orbital cellulitis G Meningitis G Urgent ophthalmology review within G Cavernous sinus thrombosis 4 hr G Periorbital abscess G ENT review Investigations G IV cefotaxime or ceftriaxone G Eye swab (send pus if present) G If toxaemic add clindamycin G FBC G If history of anaphylaxis to penicillin G Blood culture give ciprofloxacin and clindamycin G CT scan if: G Consider surgical drainage G orbital involvement suspected G if improving, convert to oral high dose G central neurological signs co-amoxiclav G unable to assess eye movements/vision G if penicillin allergy give clindamycin or if eyelid cannot be opened G Total duration of treatment (including G bilateral oedema IV) 21 days (up to 6 weeks if bone G deterioration despite treatment involvement) MANAGEMENT Intracerebral complications

Preseptal peri-orbital cellulitis G Urgent neurosurgical review G Co-amoxiclav oral Sinusitis G Review eye movements and red- green colour vision twice daily G URTI symptoms ≥10 days and ≥1 of: G If no improvement in 48 hr IV G nasal congestion and discharge antibiotics for 48 hr: G persistent cough (often nocturnal) G if aged ≤4 yr or no Hib vaccination, G Treat with co-amoxiclav oral high dose cefuroxime G Severe if: G if aged >4 yr and has received Hib G vaccination, benzylpenicillin and ill, temperature >39ºC, purulent flucloxacillin discharge G Urgent CT, ENT, neurosurgical review

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RECOGNITION AND G Tissue or pus for Gram stain and ASSESSMENT culture if surgically explored or needle aspiration Symptoms and signs Septic arthritis

G Fever G Aspiration of joint for Gram stain and G Loss of function e.g. limp culture G Pain in bone or joint G interventional radiologist or G localised, constant, increasing orthopaedic registrar G G Restricted range of movement for sedation and analgesia contact paediatric registrar or on-call G Soft tissue swelling paediatric anaesthetist G Point tenderness of bone Further investigations G Effusion

The above symptoms and signs Perform as soon as possible are indicative of osteomyelitis or (must be within 36 hr) septic arthritis (in absence of clear G If plain X-ray normal, infection clinically history of obvious trauma) localised and urgent MR is available: irrespective of WBC, CRP, ESR G consultant paediatrician or orthopaedic and fever or radiological surgeon to authorise urgent MR of bone appearance G if deep sedation or general anaesthetic required, contact on-call Previous history paediatric anaesthetist G G Ask about: If plain X-ray normal, and infection clinically localised and MRI not G duration of symptoms available, request ultrasound scan of G injuries bone G fever G If localising signs poor or possible G antibiotics multifocal infection, request isotope bone scan G antipyretics/anti-inflammatories G If cardiac murmur or multifocal Staph. Urgent investigations aureus, request echocardiogram

G FBC IMMEDIATE TREATMENT G ESR G Admit G CRP G Nil-by-mouth and maintenance fluids IV G Blood culture (before antibiotics) G Bed rest G If cause of fever uncertain, collect G Refer immediately to orthopaedic and other specimens (e.g. urine) for paediatric registrar on-call culture before antibiotics G confirm they will assess child within Osteomyelitis 4 hr of admission G Early involvement of on-call consultant G Plain X-ray AP and lateral of the orthopaedic surgeon affected part

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Antibiotics G Emergency theatres to be alerted as soon as possible (must be within 36 hr (see BNFc for neonatal doses) of admission) G Severe sepsis with organ dysfunction G Contact: (e.g. hypotension, oxygen G anaesthetic office to arrange requirement, GCS <12, platelet <80, paediatric anaesthetist creatinine x 2 normal, abnormal LFTs) G orthopaedic RSO to book patient onto G after blood and urine cultures taken, planned emergency list start cefotaxime 50 mg/kg 6-hrly (high G consultant paediatrician and dose; max 12 g/day) IV over 3–4 min orthopaedic surgeon G No organ dysfunction; as soon as G transfer to Trauma Theatre (nurse possible (must be within 4 hr): escort) G if aged <5 yr: cefotaxime SUBSEQUENT MANAGEMENT 50 mg/kg 6-hrly or ceftriaxone 80 mg/kg daily and flucloxacillin Inform paediatric orthopaedic surgeon 50 mg/kg IV (max 2 g/dose) and paediatrician G if aged >5 yr: flucloxacillin 50 mg/kg IV Uncomplicated septic arthritis (max 2 g/dose) (not complicated by associated G Targeted antibiotic therapy osteomyelitis) G If organism identified, use narrowest spectrum possible with good G Aspirate or drain joint in theatre bone/joint penetration G Request long line insertion under GA G Staph aureus sensitive to flucloxacillin and repeat any blood tests required 50 mg/kg 6-hrly IV (high dose max G If discharged for hospital at home IV 2 g/dose) treatment, change to ceftriaxone G Penicillin allergy, substitute G If treatment started within 24 hr of first flucloxacillin for: symptoms and clinically improving, discuss with consultant about changing G history of rash: cefuroxime IV to oral antibiotics after 72 hr if: G history of anaphylaxis: clindamycin G recovery of joint movement Analgesia G absence of pyrexia after 4-hrly monitoring for 48 hr G If necessary initially, to allow G WCC <11, CRP and ESR falling on two splintage, use morphine IV (see successive specimens ≥24 hr apart Analgesia guideline) G If agreed by consultant, give oral G Elevate and splint affected limb antibiotic to complete treatment G plaster backslab for peripheral joints G no organism identified: co-amoxiclav G rest in skin traction on a pillow for (high dose) central joints G organism identified: narrowest spectrum with good bone penetration Surgery - if Staph. aureus sensitive to Ask parent(s) to stay with child until flucloxacillin: flucloxacillin oral (high consent obtained dose) if capsules tolerated; or co- amoxiclav (high dose) if can only G Resuscitate if severe sepsis take suspension G allergic to penicillin: clindamycin oral

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G Stop treatment only if CRP is normal: G Consultant paediatric medical and agree duration of treatment with orthopaedic review orthopaedic consultant depending on G Exclude important differential individual case diagnoses Early-presenting osteomyelitis G systemic inflammatory response as seen in juvenile chronic arthritis G If IV antibiotics started within 24 hr of G transient synovitis, associated with onset of symptoms with a good clinical intercurrent infection response as above, follow Uncomplicated septic arthritis G acute leukaemia, septicaemia, multifocal disease, endocarditis Established osteomyelitis or G Continuing problems with local sepsis complicated septic arthritis G return to theatre for further debridement and insertion of Hickman G Presentation >24 hr after onset of symptoms or partial treatment (e.g. line oral antibiotics) MONITORING TREATMENT G Formal debridement in theatre with insertion of Hickman line G Peripheral colour, warmth, movement of affected limb: hourly for first 4 hr G Antibiotics IV as above. Discuss with then 4-hrly for 24 hr consultant about switch to oral antibiotics after 14 days, if afebrile, G Respiratory rate, pulse, temperature pain free for 48 hr and CRP <20 4-hrly G Continue antibiotics until ESR <20 (minimum 6 weeks) G Continue oral antibiotics until all inflammatory markers are normal and clear evidence of healing established on radiographs G Discuss with orthopaedic consultant duration of antibiotics on individual case Septic arthritis or osteomyelitis (deteriorating condition/failure to improve within 48 hr)

G Inform orthopaedic team for exploration to drain pus G Review culture result G Discuss with consultant microbiologist and paediatrician G Arrange for repeat blood cultures G consider a change of antibiotic therapy or targeted antibiotic therapy G Complete or repeat any investigations listed above

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RECOGNITION AND ASSESSMENT

Non-blanching rash Treat as meningococcal disease (see Septicaemia (including meningococcal) guideline Yes G FBC Purpura (>3 mm) G U&E G Coagulation screen No G Blood culture G CRP Unwell? G G Meningism Meningococcal PCR G Lethargy G IV antibiotics G Irritable G Capillary refill time >5 sec Yes G Respiratory rate >40 breaths/min G Tachycardia

No

Treat Yes Mechanical? underlying G Local trauma illness G Superior vena cava distribution after vomit/cough

No

Yes Rash progressing?

No

G Check FBC G Coagulation screen G Blood culture G CRP

Yes Treat as Abnormal platelets/coagulation necessary screen?

No

G Observe over 4–6 hr G Registrar review G Discharge if: G no purpura G patient remains well with non- progressive rash G WCC 5–15 and CRP <10

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G th Treat IMMEDIATELY (<1 hr) as If aged <1 yr: bradycardia HR <10 centile for age delay increases mortality G Mean RR >2 SD above normal for age RECOGNITION AND Meningococcal septicaemia ASSESSMENT G Assess severity of disease on G Assess Airway, Breathing, Circulation Glasgow Meningococcal and resuscitate as required Septicaemia Prognostic Score: G D isability: be alert to coexisting G on admission and at least hourly for Meningitis meningitis, see guideline first 4 hr then if improving at least G Core Temp >38.5ºC or <36ºC 4-hrly for next 24 hr G Mean HR >2 SD for age or persistent G a score >8 indicates high risk of elevation over 0.5–4 hr mortality: refer to PICU

Glasgow Meningococcal Septicaemia Prognostic Score (GMSPS) Criteria Score Systolic BP (cuff width > 2/3 upper arm length) if <75 mmHg in child aged <4 yr or <85 mmHg in child aged >4 yr 3 Skin/rectal temperature difference (measure for 2 min) if axilla/rectal temperature difference >3ºC 3 Modified coma scale ( see Glasgow coma score guideline ) if initial score <8 or deterioration of ≥3 points at any time 3 Deterioration in last hour (subjective) ask parents or nurse; if yes, score 2 Neck stiffness if no neck stiffness, score 2 Extent of purpura widespread ecchymoses or extending lesions on review 1 Base deficit if deficit >8 mmol/L 1 Maximum score 15

++ ++ – Investigations G U&E, LFT, Ca , Mg , PO 4 , CRP G Save for serum cortisol G FBC and differential If purpuric rash G Blood culture (important to put in maximum amount of blood bottle G See Petechial/purpuric rashes designed to take) guideline G Blood gas and lactate G If septicaemic treat immediately G Blood glucose If macular rash G Meningococcal PCR G Group and save G Be alert for septicaemia in any child G Clotting profile presenting with a purpuric rash

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G In the early stages of meningococcal G if MRSA suspected, add vancomycin septicaemia, a macular rash that G if anaerobic infection suspected, add DOES blanch on pressure is often metronidazole present first. When in doubt, seek an G experienced opinion urgently if pseudomonas suspected give piperacillin with tazobactam (Tazocin) If no rash G if multiple-resistant organisms suspected (e.g. previous culture G Chest X-ray results, hospital acquired) give G Urine culture (in severe sepsis, meropenem catheterise) G If hypotension continues, peripheries G Lumbar puncture if not contraindicated remain cool, rash continues to evolve, and where cardiovascularly and and capillary refill >2 sec, give sodium haematologically stable chloride 0.9% or human albumin 4.5% 20 mL/kg over 5–10 min Differential diagnosis SUBSEQUENT MANAGEMENT G Toxic shock syndrome G Malaria Circulation still compromised IMMEDIATE TREATMENT G Contact on-call paediatric consultant and anaesthetist and inform PICU G Ensure patent airway and adequate breathing G If hypotension continues give human albumin 4.5% or sodium chloride 0.9% G Administer 15 L/min oxygen through a 20 mL/kg boluses and start inotropes reservoir mask G Dopamine 5–20 microgram/kg/min G if airway and breathing remain (7.5 mg/kg in 50 mL sodium chloride compromised despite simple airway 0.9% at 2–8 mL/hr peripherally) manoeuvres and 100% oxygen, contact consultant and on-call anaesthetist G start at 5 microgram/kg/min G Assess circulation, if severe sepsis or G increase in 5 microgram/kg/min extensive rash, insert two large IV increments every 5 min cannulae or establish intraosseous G up to 20 microgram/kg/min as required access and give 20 mL/kg sodium G If still hypotensive, start adrenaline chloride 0.9% over 5–10 min 0.1 microgram/kg/min (0.3 mL/kg of Antibiotics 1:1000 in 50 mL sodium chloride 0.9% at 1 mL/hr) G Give IV cefotaxime 50 mg/kg (max 3 g G If still hypotensive, give hydrocortisone 6-hrly) over 20 min or ceftriaxone 1 mg/kg 6-hrly 80 mg/kg (max 4 g) daily over 30 –60 min (not with calcium IV; not Reassess ABC <41 weeks postmenstrual age or G neonate with jaundice, If still unstable (requiring >40 mL/kg hypoalbuminaemia or acidosis) see fluid resuscitation), arrange immediate BNFc for neonatal doses intubation with senior anaesthetist G G if documented history of definite prepare atropine 20 microgram/kg anaphylaxis to cephalosporin discuss (max 600 microgram) with consultant microbiologist

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G if no neck stiffness, ketamine 1 mg/kg; G Admit to general paediatric ward for if neck stiffness and BP stable, monitoring and continue treatment thiopental sodium 3 mg/kg G Administer oxygen via face mask or G suxamethonium 2 mg/kg aged >1 yr; nasal cannula to maintain continuous 1 mg/kg aged <1 yr oxygen saturation >95% G then morphine 20 microgram/kg/hr G Treat poor perfusion and hypotension (1 mg/kg in 50 mL sodium chloride with aliquots of human albumin 4.5% 0.9% at 1 mL/hr) 20 mL/kg solution G and midazolam 1–2 microgram/kg/min MONITORING (6 mg/kg in 50 mL sodium chloride 0.9% at 0.5–1 mL/hr) G Monitor the following every 30 min G and vecuronium 1 microgram/kg/min for first 2 hr, hourly for next 2 hr, then (1.5 mg/kg in 25 mL sodium chloride 4-hrly: 0.9% at 1 mL/hr) G conscious level G or other muscle relaxant as per local G temperature practice G respiratory rate G Site nasogastric tube and urinary catheter G heart rate G Prepare for central venous line with G BP portable ultrasound G capillary refill time G Monitor blood glucose hourly for first G Monitor urine output hourly 6 hr: if <3 mmol/L give glucose 10% G Monitor blood glucose and electrolytes 2 mL/kg bolus and start maintenance 6-hrly until stable. Treat fluids with glucose hypoglycaemia with bolus IV glucose G If glucose >3 mmol/L give sodium G Monitor clotting screen 12-hrly. Treat chloride 0.9% at 100% maintenance deranged clotting with FFP 10 mL/kg requirement IV G If passing urine, give IV fluids with potassium 10 mmol/L, if hypokalaemic SUBSEQUENT MANAGEMENT give 0.2 mmol/kg over 1 hr (use commercial pre-mixed bags) G Adjust antibiotic treatment once culture results available G If hypocalcaemic, give calcium gluconate 10% (0.22 mmol/mL) G if meningococcal or no organism 0.5 mL/kg (max 20 mL) IV over 5–10 identified give 7 days: cefotaxime min (do not give ceftriaxone in same 50 mg/kg 6-hrly IV line, give cefotaxime until stable) G or ceftriaxone 80 mg/kg IV daily, over G If INR >2, give fresh frozen plasma 30–60 min (for cautions see (FFP) 10 mL/kg Antibiotics ) G After 60 mL/kg, give packed cells G Treat S. aureus sepsis for 2 weeks 20 mL/kg G Give antibiotics to treat carrier states Haemophilus Patient stabilises with in sepsis (see Meningitis guideline) <40 mL/kg bolus fluids G Avoid enteral feeds until acute shock G Inform on-call consultant paediatrician has resolved

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Public health

G Meningococcal: inform Public Health (see Notifiable infectious diseases guideline) G Public Health Department will arrange prophylaxis for close contacts DISCHARGE AND FOLLOW-UP

G Organise formal hearing test after discharge from hospital G Arrange appointment in follow-up clinic in 8–12 weeks to review problems with: G hearing loss G orthopaedic complications G scarring G psychosocial G neurology and development G renal function G Test for complement deficiency if: G >1 episode meningococcal disease G meningococcus other than type B G other recurrent or serious bacterial infections G family history immune deficiency

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RECOGNITION AND Family and social history ASSESSMENT G Ask about recent contact with any family member (specifically History is most important factor grandparent or parent) who has: in diagnosing tuberculosis G chronic cough Symptoms G previous treatment for TB especially multi-drug resistant (MDR) TB, Suspect TB when following symptoms failed/defaulted TB treatment, or persist for weeks: recurrent TB G Persistent, non-remitting cough for G travelled to regions/countries with a 2–4 weeks high prevalence of TB/MDR TB G Weight loss G recently died G Failure to thrive INVESTIGATIONS G Lack of energy G Tuberculin skin test (Mantoux): does G Fever and sweats not exclude TB, can be negative in G Lymph nodes, especially if painless miliary TB and matted G avoid if other tests positive or clinical G Headache or irritability for >1 week diagnosis of TB G Limp, stiff back G Rapid diagnostic test on primary G Joint swelling specimen for rifampicin resistance if contact with multi-drug resistant G Abdominal distension (MDR) TB Signs G IGRA (interferon-gamma release assay, e.g. QuanitFERON ® TB Gold G Delayed growth: plot weight and or T-SPOT ® TB) are not height on growth chart and compare recommended by NICE for diagnosis with earlier records of active TB G Fever Pulmonary TB G Wasting G Lymphadenopathy G Chest X-ray: look for hilar lymphadenopathy, apical G Chest signs consolidation, pleural effusion, miliary G Cardiac tamponade nodules G Ascites G Sputum: send at least 3 (1 early G Meningism morning) for AFB and TB culture in cooperative child, expectoration may G Conjunctivitis require physio +/- nebulised sodium G Limited flexion of spine chloride 0.9% as necessary (with G Kyphosis FFP3 mask and HEPA filtered ventilation if available) G Swollen joint G Cold abscess

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G If unable to provide sputum specimen, Bone/joint pain send gastric aspirate (for TB culture only as microscopy is unreliable) early G Plain X-ray and CT or MR if available morning before feed, daily for G Biopsy/aspiration important for 3 days diagnosis and sensitivities G if no aspirate, rinse stomach with small volumes of sodium chloride Abdominal distension 0.9% (5 mL aliquots maximum 20 mL) G Ultrasound then CT abdomen G do not send saliva G G Discuss broncho-alveolar lavage for Culture ascites/bowel biopsy AFB and TB culture via bronchoscopy Pyuria with respiratory consultant G Pleural effusion Urinalysis: if blood and leucocytes present, send for smear and culture G Pleural tap +/- biopsy for histology and G non-tuberculous acid-fast bacteria microbiology (AFB and TB culture) common in urine G Discuss with cardiothoracic surgeons G Ultrasound kidneys about pleural biopsy G Early morning urine culture Lymphadenopathy Pericardial effusion

G If single node, excision biopsy G Echocardiogram G If large matted nodes, ultrasound scan G Pericardial fluid +/- simultaneous guided aspiration (discuss before scan) Disseminated (inc. miliary) G Lymph node aspirate: fine needle G CT thorax and ultrasound abdomen aspiration biopsy (FNAB; 23 G needle) G LP (CT or MR first if CNS signs or G low risk, high yield with sedation and symptoms) local anaesthetic G G Send aspirate in two separate bottles: Bone marrow biopsy if diagnosis uncertain G one to microbiology for TB culture with no preservative IMMEDIATE MANAGEMENT G one to histology in 10% formalin G If atypical mycobacterial infection Discuss treatment with local suspected, prefer complete excision TB team and lead paediatrician biopsy, if possible, to aspiration biopsy for TB Meningism G Inform Public Health through TB clinic, who will organise chest X-ray and G CSF: request staining for acid-fast Mantoux for all close and visiting AND bacilli (AFB) culture and sensitivity contacts for TB (Note: TB meningitis extremely G Inform infection prevention and control rare in the UK; often AFB negative) team: advise anyone with cough to G PCR: expensive, consider for CSF if avoid visiting ward highly suspicious of TB meningitis, G Admission not mandatory but useful to poor sensitivity ensure adherence with treatment. If G If tuberculoma suspected, CT or MR supervision can be guaranteed, allow brain treatment at home

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G If sputum +ve and hospitalisation G Pyrazinamide (Z): 35 mg/kg once daily necessary, strict nurse in single room up to max 1.5 g if <50 kg; 2 g ≥50 kg for 2 weeks or discharge G (500 mg tablets can be crushed) G Patient should wear a surgical mask if G Ethambutol (E): 15 mg/kg once daily leaves room (100 mg, 400 mg tablets can be G Masks, gowns and barrier nursing crushed) unnecessary unless MDR TB or G check renal function first, do not aerosol generating procedure round dose up G Negative pressure room for aerosol G Round up doses of HRZ to give easily generating procedure if TB considered measured volumes of syrup or (e.g. nebuliser) appropriate strengths of tablet. Drugs Re-calculate doses with weight gain G Use drug combinations if possible G Isoniazid (H): 10 mg/kg once daily up G Rifater ®: H 50 mg; R 120 mg; Z 300 mg to max 300 mg G Rifinah ® 150/100: H 100 mg; R 150 mg G (suspension; 50 mg, 100 mg tab) G Rifinah ® 300/150: H 150 mg; R 300 mg G Rifampicin (R): 15 mg/kg once daily up to max 450 mg if <50 kg; 600 mg ≥50 kg G (suspension; 150 mg, 300 mg capsule) Presentation Treatment Respiratory TB: lungs, pleural cavity, Rifampicin and isoniazid for 6 months mediastinal lymph nodes or larynx Pyrazinamide and ethambutol for first 2 months Meningeal TB Rifampicin and isoniazid for 12 months Pyrazinamide and ethambutol for first 2 months Prednisolone 1–2 mg/kg (max 40 mg), with gradual withdrawal, starting within 2–3 weeks of initiation Other extra pulmonary lesions As for respiratory TB

G Add pyridoxine to prevent isoniazid SUBSEQUENT MANAGEMENT neuropathy in malnourished or breastfed infants, diabetes, HIV or G HIV test renal failure G Other drugs may be necessary once G Pericardial TB: add prednisolone sensitivities available: if resistant, seek 1 mg/kg/day (max 40 mg/day) specialist advice G Inform patient/parents of both common (gastrointestinal upset, rash) and rare MONITORING TREATMENT but important side effects (staining of G secretions, signs of hepatotoxicity) If baseline ALT/AST raised but ≤2x normal, repeat at 2 weeks; if falling G Advise patient/parents and GP of only recheck if fever, malaise, indications for seeking advice: fever, vomiting, jaundice or unexplained malaise, vomiting, jaundice or deterioration unexplained deterioration. Consider G co-existent viral hepatitis. If AST/ALT If ALT/AST >2x, monitor weekly for level rises to 5x normal, stop treatment 2 weeks then 2-weekly until normal, and seek advice re: alternate regimen check viral hepatitis serology

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G Stop treatment only if ≥5x normal G if aged 4 weeks–2 yr and no BCG, G If on ethambutol and unable to report start isoniazid and repeat Mantoux visual problems, check visual evoked and do IGRA after 6 weeks: if both response –ve, stop isoniazid, if either +ve , do CXR and refer to TB team DISCHARGE AND FOLLOW-UP G if aged 4 weeks–2 yr and BCG, repeat Mantoux and do IGRA after 6 weeks: G Discharge if tolerating treatment and if both –ve, discharge, if either +ve , do adherence guaranteed CXR and refer to TB team G If concerns about adherence, will need direct observed therapy, organised through TB team G Review to ensure adherence: G at least monthly for first 2 months G 2-monthly until treatment complete G for 3 months after end of treatment G further as clinically indicated LATENT TB

Close contact with sputum +ve TB or new entrant from high- incidence country

G If symptomatic refer to TB team G If asymptomatic neonate contact with smear +ve case on treatment <2 weeks, treat with isoniazid for 3 months then do Mantoux. If +ve: CXR and refer to TB team; if –ve repeat Mantoux and do IGRA: if both –ve stop isoniazid and give BCG, if either +ve do CXR and refer to TB team G If asymptomatic aged >4 weeks do Mantoux G If Mantoux +ve (≥15 mm with BCG/ ≥6 mm no BCG) request CXR and refer to TB team for chemoprophylaxis G If Mantoux –ve (<15 mm with BCG/<6 mm no BCG): G if index case smear +ve, after 6 weeks do IGRA and if aged 2–5 yr repeat Mantoux: if Mantoux or IGRA +ve refer to TB team G if index case smear –ve, advise to see GP if symptomatic

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RECOGNITION AND INVESTIGATIONS ASSESSMENT G If all history/examination Definition unremarkable, no investigations needed G Bell’s palsy: idiopathic lower motor G If difficulty in closing eye, neurone facial nerve palsy ophthalmology referral G Facial nerve palsy secondary to G Bilateral facial palsy – consider Lyme infection, inflammation, tumour, disease, Guillain-Barré syndrome, trauma, vascular event brain stem pathology: discuss further investigations with senior Symptoms and signs G Recurrent facial palsy: discuss with G Asymmetry of face or smile and loss senior of nasiolabial fold on same side G Recurrent infections: first line immune G Increased or decreased lacrimation deficiency investigations (including HIV) G Hyperacusis G Severe pain associated with VZV G Altered taste G Facial pain IMMEDIATE TREATMENT G Difficulty in closing eye G If difficulty in closing eye, provide eye History patch and hypromellose eye drops G If no other signs, no other treatment G History of prior viral infection may be necessary present G If vesicles suggest HSV, prescribe G Abrupt onset with no progression aciclovir, test for immune deficiency G No history of preceding seizure or G Within 72 hr prednisolone 1 mg/kg/day head injury for 10 days discuss with a senior Examination DISHCHARGE AND FOLLOW-UP

G Full neurological examination, G 4 weekly GP follow-up until symptoms including other cranial nerves, and and signs have resolved (95% by 1 yr) fundoscopy G If facial palsy does not improve G demonstrable weakness in lower considerably within 4 weeks discuss motor neurone distribution (includes imaging loss of wrinkles on forehead) G Ears, nose and throat to exclude cholesteatoma, mastoiditis or herpes infection G Blood pressure to exclude hypertension G Check for lymphadenopathy, hepatosplenomegaly, pallor or bruising to exclude malignancy

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DEFINITIONS Seizure types

G Seizures/convulsions: paroxysmal Generalised disturbance of consciousness, behaviour, motor function, sensation – G Tonic-clonic/tonic singly or in combination G Clonic G Epilepsy: recurrent seizures without G any provoking factor and happening in Atonic different situations G Absence G Seizure type: (focal, generalised or G Myoclonic any other type) based on history and Focal EEG G Try to categorise into one of epilepsy G Without impairment of consciousness syndromes (focal motor, sensory or other types) RECOGNITION AND G With impairment of consciousness ASSESSMENT (previously known as complex partial) G Focal with secondary generalisation G Detailed and accurate history from an (clinically look like generalised eyewitness (beginning, middle and seizures) – history of aura, Todd’s end of the episode) paralysis, focal features on EEG G When history unclear, recording the episode with a camcorder/mobile Underlying cause phone can be very useful G In most cases epilepsy is idiopathic G Episodes occurring only in certain but a few cases have an underlying situations with certain provoking cause factors (such as fall, emotions, certain posture etc., except photosensitive G actively look for the cause to guide stimuli) are likely to be non-epileptic prognosis, other treatment and recommendation for epilepsy surgery G Any underlying problem: learning difficulties, cerebral palsy, HIE, head EPILEPSY SYNDROMES injury or other CNS insult G Look for any co-morbidity Identification G Family history may be positive in certain idiopathic generalised G Based on: epilepsies, some symptomatic G seizure type epilepsies (tuberous sclerosis), G age of onset autosomal dominant frontal epilepsies G neurodevelopmental status G Genetic conditions (e.g. Angelman’s syndrome) G appearance of EEG (ictal and interictal) G Neurocutaneous syndromes, café-au- Childhood absence epilepsy lait spots/depigmented patches, use Woods Light G Usually presents aged 3–8 yr G Neurological examination G More common in girls G If in doubt about diagnosis, do not G Several (up to 100) brief episodes in label as epilepsy but watch and wait a day or refer to specialist G Very quick recovery Diagnosis of epilepsy is clinical G Typical EEG 3 per sec spike and wave

Issue 5 Issued: May 2013 196 Expires: May 2014 EPILEPSY • 2/5 G 10–30% of children have generalised G Usually starts with vomiting and child seizures at some stage, usually in initially conscious teenage years G Child continues to vomit repeatedly Juvenile absence epilepsy and becomes unresponsive G Subsequent deviation of eyes to one G Usually presents after age 9–10 yr side or may end in hemiclonic seizure G Absence frequency is less than in or (rarely) generalised seizure childhood absence epilepsy G Other autonomic features very G Cluster after awakening common (e.g. dilated pupils, pale skin or flushing, incontinence) G 90% of children have generalised seizures in the same period while they G Usually lasts for a few to 30 min, have absences occasionally for several hours G EEG generalised spike and wave Temporal lobe epilepsy (TLE) Juvenile myoclonic epilepsy G Focal seizures with impaired (JME) consciousness and complex automatism G Usually presents between ages 12–18 yr G Aura is common before the seizure, which could be a sense of fear, G Myoclonic jerks are hallmark of this abnormal abdominal sensation or any syndrome other G Jerks after awakening (myoclonic jerks), G Children are very tired and sleepy common and often go unrecognised after episode G 90% of children have generalised G Children with history of prolonged seizures at some stage febrile seizure in the early years of life G 15–30% of children will have absences may have mesial temporal sclerosis Benign epilepsy of childhood as a cause of their seizures with rolandic spike G Other known causes: cortical dysplasia, gliomas, disembryonic G Usually nocturnal seizures neuroectodermal tumour G Unilateral focal motor seizures of face G Some patients can be a candidate for and arm with gurgling and salivation epilepsy surgery G May become secondary generalised Frontal lobe epilepsy G May present with nocturnal generalised seizures G Usually focal motor seizures G Spikes in one or the other centro G Either tonic or clonic seizures – may temporal areas have speech arrest and head rotation or complex partial seizures or focal G Awake interictal EEG could be normal and sleep EEG would usually show with secondary generalisation the abnormality G Multiple brief seizures in the night Panayiotopoulos syndrome G Repeated/multiple brief nocturnal seizures are a characteristic feature of frontal lobe epilepsy G Younger children (peak age 5 yr) G G usually nocturnal and happens in sleep Ictal EEG can be normal G Can mimic pseudo seizures

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Other epilepsy syndromes G Epilepsy in children aged <2 yr G Myoclonic epilepsy G Epileptic encephalopathy in newborns G Intractable seizures (myoclonic or Ohtohara syndrome) G Loss of previous good control G West’s syndrome (infantile spasms) G Seizures continuing in spite of first line G Severe myoclonic epilepsy of infancy medication (Dravet’s syndrome) G Associated neurological deficits or G Lennox-Gastaut syndrome appearance of new neurological signs G Laundau-Kleffner syndrome G Developmental regression in children G For other epilepsy syndromes see with epilepsy International League against Epilepsy G Infantile spasms (West’s syndrome) website (www.ilae-epilepsy.org) Other investigations INVESTIGATIONS G Sleep or sleep-deprived EEG useful in Indications for EEG all children in whom there is a high clinical suspicion but awake EEG G Clinically diagnosed epilepsy normal G After an episode of status epilepticus G sleep EEG useful to pick up some focal/generalised epilepsies and G Unexplained coma or encephalopathy sleep-deprived EEG useful in G Suspicion of non-convulsive status in generalised epilepsies in young adults children with learning difficulties and including JME. Perform sleep EEG epilepsy with melatonin G Acquired regression of speech or G Video telemetry useful if diagnostic language function dilemma, pseudo seizures or before G Developmental regression suspected surgery to have neurodegenerative condition G Drug levels: phenytoin, phenobarbitone G To monitor progress in West’s (other anticonvulsants only if concerns syndrome and non-convulsive status about compliance and overdose) EEG not indicated G Biochemistry: glucose, calcium, LFT, lactate, ammonia; metabolic and G Funny turns, apnoeic attacks, dizzy genetic investigations where suspicion spells, strange behaviour of metabolic disorder (e.g. progressive developmental delay) G Non-convulsive episodes [e.g. syncope, reflex anoxic seizures, breath-holding G Epileptic encephalopathies, such as episodes (ECG more appropriate)] West's Syndrome, need a series of investigations (discuss with paediatric G Febrile seizures neurologist) G Single uncomplicated generalised tonic-clonic seizures TREATMENT G To monitor progress in well-controlled epilepsy General guidelines G Before stopping treatment G Discuss treatment with a consultant Indications for MRI of brain before starting G Start anti-epileptic only if diagnosis G Focal epilepsy (including TLE) except certain (two or more unprovoked rolandic seizures seizures)

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G Preferably after initial EEG results G Give liquids as sugar-free preparations obtained G Make sure you discuss potential G Start with small dose and build up to adverse effects with parents and half maintenance. If seizures continue, document these in notes increase to full maintenance G If child develops adverse effects, G Increase dose stepwise every 2–3 discuss and reduce dose weeks Discussion with child and First line drugs parents

G See Table for choice of anti-epileptic G Provide additional advice regarding drug safety (e.g. supervision when swimming) G Carbamazepine: start with and document discussion in notes 2.5–5 mg/kg/day in two divided doses G Discuss and prescribe rescue gradually increasing to 20 mg/kg/day treatment, especially in generalised OR epilepsy, with training for parents G Sodium valproate: start with G Provide written information, including 5–10 mg/kg/day in two divided doses information about national or local gradually increasing to 40 mg/kg/day epilepsy associations and website for Epilepsy Action (www.epilepsy.org.uk) G Avoid polypharmacy; do not add a second medication unless the full or G Explain how to gain access to maximum tolerated dose of the first epilepsy specialist nurse medication has been reached (discuss G Allow parents and children to ask with a paediatrician with special questions, especially about sensitive interest or paediatric neurologist issues such as sudden death before adding second drug) G Aim to switch to monotherapy after a period of overlap

Table 1: Drugs of first, second and third choice in treatment of seizure types Seizure type First Second Third Generalised epilepsy Sodium valproate Carbamazepine † Lamotrigine* OR OR Levetiracetam Carbamazepine † Sodium valproate Topiramate Childhood absence Sodium valproate Lamotrigine* Levetiracetam epilepsy Ethosuximide Topiramate Focal epilepsy Carbamazepine Sodium valproate Clobazam including TLE Lamotrigine* Phenytoin Topiramate Levetiracetam Infantile spasms Prednisolone/tetracosactide Sodium valproate Trial of pyridoxine OR Nitrazepam Vigabatrin * Lamotrigine can increase myoclonic seizures in some myoclonic epilepsy syndromes † Carbamazepine should be avoided in childhood absences, juvenile absences and juvenile myoclonic epilepsy and can increase seizures in some epileptic encephalopathies and primary generalised epilepsies

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Epilepsy in adolescence – G risk of unacceptable side effects of additional factors to be medication considered G unilateral structural lesion G psychological or psychiatric co- G Compliance morbidity G Career choices G diagnostic doubt about seizure type G Driving and/or syndrome G Contraception and pregnancy, Refer including pre-pregnancy counselling G Alcohol and drugs G Refer specific syndromes such as: SUBSEQUENT MANAGEMENT G Sturge-Weber syndrome G Rasmussen’s encephalitis G Increase dose of anti-epileptic gradually G hypothalamic hamartoma towards full dose or maximum tolerated dose until control good WITHDRAWAL OF G If control suboptimal with one drug or ANTI-EPILEPTIC DRUGS unacceptable side effects, start G second-line drug Consider when child has been seizure free for 2 yrs OUT-PATIENT MANAGEMENT G Discuss the risks of recurrence (25–30%), if this occurs, recommence G Initial follow-up at 6–8 weeks treatment G Subsequent follow-up/structured G Recurrence is very high in some review every 3–12 months based on syndromes (e.g. juvenile myoclonic clinical need epilepsy, 70–80% usually requires FURTHER OPINION/REFERRAL lifelong treatment) TO SPECIALIST SERVICE OR G Postpone withdrawing anti-epileptic TERTIARY CENTRE medication if important events such as (NICE GUIDELINES) GCSEs are looming G Gradual withdrawal over 2–3 months Refer immediately usual G Some drugs (phenobarbital or G Behavioural or developmental benzodiazepines) need very slow regression withdrawal over 6–12 months G Epilepsy syndrome cannot be identified Refer soon

G When one or more of the following are present: G child aged <2 yr G seizures continuing despite being on anti-epileptic drug (AED) for 2 yrs G 2 AEDs have been tried and are unsuccessful

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G Follow each step until fits resolve, but do not treat post-ictal posturing as seizure G Prepare next step in algorithm immediately after previous one administered G Do not give more than 2 doses of benzodiazepine, including any pre-hospital doses

Airway High flow oxygen Glucose strips

Yes Vascular access? No

† Step Lorazepam* Midazolam buccal 1 0.1 mg/kg IV/IO (max 4 mg) over 6 min 0.5 mg/kg (dilute 1:1 with sodium chloride 0.9%) (see Table below for dose)

If seizure continuing 10 min after start of step 1

Step 2 Lorazepam* 0.1 mg/kg IV/IO ‡

Pre-prepare phenytoin. Call for senior help

If seizure continuing 10 min after start of step 2

Step 3 Phenytoin** 20 mg/kg IV/IO over 20 min with cardiac monitoring If seizure continuing 10 min after start of step 3

Call anaesthetist/PICU RSI with thiopental Do not start sedation, aim for early extubation unless seizure starts again CT scan not routine: only if history of head injury or new focal seizure

* If lorazepam not available give IV diazepam † If buccal midazolam not available give rectal diazepam ‡ If vascular access and intraosseous still not obtained give paraldehyde PR: 0.8 mL/kg ready mixed solution or 0.4 mL/kg diluted with equal volume of olive oil ** If already taking phenytoin, give phenobarbital 20 mg/kg IV/IO over 20 min diluted 1:1 with water for injection

Diazepam (IV) Diazepam (rectal) Midazolam (buccal) Paraldehyde (rectal) volume of 50:50 diluted Aged 1 month–12 yr Aged 1 month–2 yr: Aged <6 months: Aged 1–3 months: 300 microgram/kg 5 mg 300 microgram/kg 0.5 mL (max 10 mg) (max 2.5 mg) Aged >12 yr: 10 mg Aged 2–12 yr: Aged 6 months–1 yr: Aged 3–6 months: 1 mL 5–10 mg 2.5 mg Aged >12 yr: 10 mg Aged 1–5 yr: 5 mg Aged 6 months–1 yr: 1.5 mL Aged 5–10 yr: 7.5 mg Aged 1–2 yr: 2 mL Aged >10 yr: 10 mg Aged 2–5 yr: 3–4 mL Age 5–18 yr: 5–10 mL

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ON ADMISSION G ECG G Blood gas for cardiac status G Ask parents if they have a copy of a G Chest X-ray: clinical signs can fail to care plan detect G Inform child’s long-term consultant collapse/consolidation/cardiomegaly CLINICAL HISTORY G Consider skeletal/spinal X-rays for possible fractures G Adequacy of cough and swallowing Medical problems commonly G Previous sleep difficulties, found in children with myopathy wakefulness at night (nocturnal hypoventilation) G Respiratory failure (hypoxaemia and G Difficulty waking in morning, early hypercapnia) without signs of morning headache (nocturnal respiratory distress. Susceptibility to hypoventilation) respiratory failure due to: G Poor appetite, weight loss (chronic G muscle weakness (upper airway, respiratory failure) intercostals, diaphragm) G Learning or behavioural problems, G scoliosis school attendance (chronic respiratory G poor secretion clearance failure) G aspiration, chest infections G Palpitations, breathlessness, chest G sleep disordered breathing pain (cardiomyopathy) G cardiac failure G Muscle cramps, skeletal pain, back G pain (for fractures) Lower respiratory infection, aspiration pneumonitis G Abdominal pain, distension, melaena G (GI perforation) Cardiomyopathy and cardiac decompensation ASSESSMENT G Gastro-oesophageal reflux, gastritis and gastric ulceration (especially if on G May not show overt signs of respiratory corticosteroids) distress such as tachypnoea, G recessions and use of accessory Adrenal insufficiency (if on muscles even in respiratory failure corticosteroids) G G Assess adequacy of chest wall Fractures, especially vertebral, if on excursion and cough long-term corticosteroids G G Look for pallor, tachycardia, signs of Malignant hyperthermia following circulatory compromise anaesthesia in certain muscular dystrophies and myopathies G Assess for abdominal signs (GI bleed, perforation, gastritis) MANAGEMENT G Measure: G If unwell, on long-term G SpO 2 in air corticosteroids , double usual daily

G CO 2 by blood gas, transcutaneous dose of steroids for 2–3 days. If unable to tolerate oral steroids, use IV CO 2 or end-tidal CO 2, especially if on hydrocortisone oxygen G spirometry: FVC most useful if previous readings available

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Respiratory failure GI tract bleed: prevention and treatment G Carefully titrated administration of oxygen by mask/nasal cannulae to G Nil-by-mouth and IV fluids achieve SpO 2 between 94–98%. G Ranitidine (omeprazole if reflux) Monitor CO and respiratory effort as 2 G Senior advice risk of rising CO 2 and respiratory failure (despite normal oxygen Fractures saturations) if hypoxic respiratory drive overcome by oxygen therapy G Analgesia G G Mask ventilation (bi-level positive Orthopaedic consultation airway pressure, BIPAP) G IV biphosphonates for vertebral G Chest physiotherapy and postural fractures, discuss with metabolic bone drainage expert G Use in/ex-sufflator (e.g. Cough Assist) Malignant hyperthermia if patient has one G Suction Malignant hyperthermia is a G if copious loose secretions use medical emergency glycopyrrolate 40–100 microgram/kg oral max 2 mg 6-hrly (use G Occurs following general anaesthesia 200 microgram/mL IV solution if and may be first presentation of a specials manufacturer solution not neuromuscular disorder available) G Check creatine kinase, calcium, renal G Antibiotics function, urine output and for myoglobinuria: dialysis may be G obtain cough swab or sputum needed specimen, ideally before starting treatment G In addition to temperature control and general life support measures, use IV G check previous culture results dantrolene to control excessive G choice same as for community muscle contraction acquired pneumonia G Obtain senior anaesthetic advice and G if bronchiectasis use broad spectrum liaise with PICU for 14 days to cover pseudomonas (discuss with senior) G if not improving on 1 st line antibiotics add macrolide for atypical pneumonia G Consult senior to discuss need for ITU care, escalation of respiratory support Cardiac failure

G Fluid restriction G Diuretics G Oxygen and respiratory support G Cardiology consultation

Issue 5 Issued: May 2013 Expires: May 2014 203 GLASGOW COMA SCORE • 1/1

Response aged ≥4 yr Response aged <4 yr

Eye opening Score Eye opening Score Spontaneously 4 Spontaneously 4 To speech 3 To speech 3 To pain 2 To pain 2 Never 1 Never 1

Best motor response Score Best motor response Score Obeys commands 6 Obeys commands (or 6 Localises pain 5 spontaneous, purposeful <2 yr) Withdrawal 4 Localises pain or withdraws to 5 Flexion to pain 3 touch Extension to pain 2 Withdrawal 4 None 1 Flexion to pain 3 Extension to pain 2 Best verbal response Score None 1 Orientated and converses 5 Disorientated and converses 4 Best verbal response Score Inappropriate words 3 Alert and babbles, words to usual 5 Incomprehensible sounds 2 ability None 1 Less than usual words, 4 spontaneous irritable cry Cries only to pain 3 Moans to pain 2 None 1

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RECOGNITION AND G FBC: low haemoglobin (dilutional ASSESSMENT effect) G Immunology: complement C3, anti- Definition nuclear antibodies (ANA) and IgG, A and M G Acute inflammatory process affecting G Serology: hepatitis B the glomeruli leading to haematuria, G Antistreptolysin O titres (ASOT) and proteinuria, oedema, hypertension and Anti-DNAase B renal insufficiency G Throat swab for Group A streptococcus Symptoms and signs G Renal ultrasound scan for evidence of pre-existing disease G Reduced urine output Differential diagnosis G Macroscopic haematuria, coca-cola coloured urine G Sequelae of other bacterial/viral G Headache/breathlessness, indicative infections of severe disease G Chronic renal failure with acute G History of sore throat in preceding 2–3 exacerbation weeks G Henoch-Schönlein purpura G Oedema variable, periorbital/pedal G IgA nephropathy G check weight, trend is useful G Alport hereditary nephritis G check jugular venous pressure (JVP), G if raised, indicates volume overload ANCA positive vasculitis, anti GBM (cardiac failure) disease G Oliguria (urine output: infant/child IMMEDIATE TREATMENT <0.5 mL/kg/hr, neonate <0.6 mL/kg/hr) G G Hypertension +/- features of Admit encephalopathy (headache, nausea, G Strict fluid balance monitoring and vomiting, visual disturbance, management restlessness, confusion) G see Renal failure guideline G Signs of cardiac failure (tachypnoea, G Treatment of volume raised JVP, gallop rhythm, basal overload/hypertension crackles, enlarged liver) G furosemide Investigations G see Hypertension guideline G severe cases of fluid overload will G Urine dipstick: >3+ blood and protein require dialysis G Urine microscopy: red cell casts G Treatment of abnormal chemistry G U&E consequent to renal failure G sodium may be low (dilutional effect) G see Renal failure guideline G potassium, urea and creatinine G Oral antibiotics: phenoxymethyl G bicarbonate may be low penicillin if able to take tablets or amoxicillin suspension (if penicillin G phosphate, uric acid allergy azithromycin) for 10 days G albumin usually normal G Nutrition: encourage high carbohydrate intake

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DISCHARGE FROM HOSPITAL DISCHARGE FROM FOLLOW-UP

G BP under good control G Normal BP (when not receiving G Passing urine normally on free fluids antihypertensive treatment) G Renal function improving G Normal renal function G Normal serum potassium G Normal urinalysis SUBSEQUENT MANAGEMENT

Follow-up/progress

G Gross haematuria, oliguria and abnormal chemistry usually resolves by 2–3 weeks G BP usually normal by 3–4 weeks G Serum C3 usually normal by 4–6 weeks G Proteinuria resolves by 6 months G Microscopic haematuria usually resolves by 12 months Tertiary referral

Refer to nearest paediatric renal centre if: G Atypical presentation G Evidence of serious degree of renal failure requiring dialysis G Poorly-controlled hypertension/cardiac failure/encephalopathy G Heavy or persistent proteinuria leading to hypo-albuminaemia G Normal serum C3 at presentation (i.e. not post-streptococcal) G Failure of normalisation of C3 by 6 weeks, positive ANA, ANCA or anti GBM G Associated vasculitis G Delay in recovery as indicated by timescales above G Recurrent episodes

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RECOGNITION AND Investigations ASSESSMENT G FBC and blood film (look for Definition fragmented red cells) G low Hb and platelets G Triad of features G Clotting studies G haemolytic anaemia G U&E, creatinine G thrombocytopenia G Bicarbonate G renal insufficiency G Calcium, phosphate, uric acid Symptoms and signs G Glucose G Liver function tests G Diarrhoea with blood and mucus (HUS G E. coli can occur in absence of diarrhoea) 0157 serology acute and convalescent (10 days after onset of G Vomiting symptoms) G Abdominal pain G Urine stick test for significant blood G Pallor, lethargy and protein (indicating glomerular G Bleeding tendency damage) and leucocytes E. coli G Reduced urine output/facial puffiness G Stool culture for (and typing for 0157 strain) G Pallor G Mucocutaneous bleeding IMMEDIATE TREATMENT G Tachycardia G Admit, discuss with regional paediatric G Reduced consciousness: consider nephrology team in all cases cerebral oedema, intracranial G Strict fluid balance monitoring and haemorrhage management G Convulsions: consider hyponatraemia, G see Renal failure guideline cerebral oedema, intracranial haemorrhage G Dehydration G Paralysis: consider intracranial G if signs of hypovolaemic shock give haemorrhage circulatory support (sodium chloride 0.9% 20 mL/kg IV immediately) G Over-hydration G correct dehydration, see Diarrhoea G oedema (periorbital/pedal) variable and vomiting guideline G weight gain, observe trend G Over-hydration G raised jugular venous pressure (JVP) G if signs of overload/cardiac failure, indicates volume overload (cardiac furosemide IV 2–4 mg/kg (max rate failure) 4 mg/min), repeated 6-hrly if response G oliguria (urine output <1 mL/kg/hr) obtained G tachypnoea G if furosemide ineffective, discuss G liver enlargement dialysis with regional paediatric renal centre G dehydration if diarrhoea has been severe, see Diarrhoea and vomiting G Hypertension – see Hypertension guideline guideline G check BP: hypotension

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G Anaemia DISCHARGE FROM G daily FBC: only transfuse after FOLLOW-UP discussion with regional paediatric nephrology team as may require G Renal function normal dialysis. If asymptomatic, Hb can drop G No proteinuria as low as 60 g/L G Renal growth and function satisfactory G Thrombocytopenia at 5-yrly review for 15 yr G do not transfuse platelets unless there are life-threatening bleeds G AVOID antibiotics G Observe for non-renal complications e.g. encephalopathy and seizures, cardiomyopathy DISCHARGE FROM HOSPITAL

G Patient may be discharged when: G diarrhoea/abdominal pain resolved G Hb stable (haemolysis ceased) G drinking fluids freely and passing normal amounts of urine G urea and electrolytes improving with serum potassium normal SUBSEQUENT MANAGEMENT

Tertiary referral

G If significant renal impairment (anuria, rising creatinine) dialysis required (see Renal failure guideline), refer to regional paediatric renal centre Follow-up

G Weekly until renal function stable G if impaired renal function or proteinuria persists, arrange paediatric renal follow-up G Once renal function normal, arrange GP or general paediatric follow-up every year to check BP and early morning urine (protein:creatinine ratio) with a detailed renal specialist review every 5 yrs G Advise that women with history of haemolytic uraemic syndrome require close monitoring during pregnancy

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RECOGNITION AND G polydipsia, polyuria ASSESSMENT G visual problems G tiredness, irritability Diagnosis is difficult because symptoms G can be minimal and often go cardiac failure unrecognised G facial palsy G Severe hypertension can cause: G epistaxis G loss of consciousness G poor growth, weight loss G convulsion G cardiac murmur G hemiplegia G abdominal pain Definition G enuresis Hypertensive encephalopathy G Depends on age, sex and height of (accelerated hypertension) child G Measure on at least 3 separate G Any neurological sign associated with occasions with ausculatory method grossly elevated blood pressure, most commonly: G Normal: systolic and diastolic BP <90 th centile for age, sex and height G severe generalised headache G High normal: systolic and diastolic BP G visual disturbance (+/- retinal between 90 th and 95 th centile for age, changes) sex and height (>120/80 even if below G seizure 90 th centile in adolescents) Do not delay initiation of treatment G Stage 1 hypertension: 95 th –99 th centile PLUS 5 mmHg pending investigations once diagnosis has been made G Stage 2 hypertension: >99 th centile PLUS 5 mmHg History Symptoms and signs G Family history of hypertension, diabetes, cardiovascular and Hypertension cerebrovascular disease, obesity, hereditary renal and endocrine Listed in order of frequency with disease common presenting features first: G Past history of renal, cardiac, G Infants endocrine or neurological problems G congestive cardiac failure G Presenting complaints as listed above G respiratory distress G Drug intake such as corticosteroids, G failure to thrive, vomiting ciclosporins, tacrolimus, G irritability antidepressants G convulsions Examination G Older children G Detailed clinical examination of all G headaches systems G nausea, vomiting G Do not forget fundoscopy G hypertensive encephalopathy (see below)

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Investigations IMMEDIATE TREATMENT

G Check for evidence of renal disease Hypertensive encephalopathy G serum creatinine, urea and (accelerated hypertension) electrolytes, calcium G urinalysis for blood and protein Urgent treatment necessary but G if urine dipstick positive for protein bring BP under control slowly send early morning urine for protein:creatinine ratio Abrupt BP reduction can result in cerebral ischaemia with the risk of G renal ultrasound scan permanent neurological sequelae, owing G DMSA scan may be required to to failure of cerebral auto-regulation after exclude scarring sustained elevation of BP G Check for cardiovascular causes G Excess BP = actual BP – acceptable G check femoral pulses BP ( Table 1 and 2 ) G right arm and leg blood pressure G ‘acceptable BP’ given by the 90 th percentile according to height G ECG for left ventricular hypertrophy (LVH) G Reduce BP gradually. Aim to reduce ‘excess BP’ by 1/3 in first 8 hr, another G echocardiogram 1/3 in next 12 hr, and final 1/3 in next G Check for endocrine causes 48 hr G fasting plasma glucose G Mark target BP ranges on chart so G lipid profile nurses know when to ask a doctor to G plasma renin and aldosterone review concentration G Monitor perfusion: may need volume G urine catecholamines (contact expansion in first 12 hr if rapid BP drop biochemistry department for details of G Discuss choice of drug treatment with how to perform test) consultant G urine metadrenalines (performed at G Options comprise in following order: Manchester Children’s Hospital) (Table 3 ) G 24 hr urinary free cortisol and/or G sodium nitroprusside infusion discuss with endocrinologist for further - give in high dependency or intensive investigations care unit as close monitoring required Differential diagnosis - starting dose 500 nanogram/kg/min - increase in increments of G Incorrectly sized (too small) or placed 200 nanogram/kg/min BP cuff - maximum 8 microgram/kg/min for G Transient hypertension secondary to first 24 hr, reducing to pain, anxiety, distress 4 microgram/kg/min thereafter - only effective whilst infused as short half-life - stop infusion slowly over 15–30 min to avoid any rebound effects

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G labetalol infusion Renal hypertension - starting dose 0.5–1 mg/kg/hr G - increase by 1 mg/kg/hr every In children with impaired renal 15–30 min until effective function, keep BP within same target range as for children with normal renal - maximum dose 3 mg/kg/hr function (max 120 mg/hr) - stop infusion when effective - restart as BP starts to rise again - normally lasts 4–6 hr G nifedipine oral - quick acting: use modified release to prevent large drop in BP - can be crushed but may have more rapid onset - may be used to clip peaks of BP - dose varies with product: check with pharmacy SUBSEQUENT MANAGEMENT

Essential hypertension

G High normal BP G non pharmacological measures such as weight loss, dietary modification, exercise G medication ( Table 3 ) only if compelling indications such as if symptomatic, diabetes mellitus, heart failure, left ventricular hypertrophy G Stage 1 hypertension G non pharmacological measures G give medications ( Table 3 ) if symptomatic, presence of end organ damage, diabetes, persistent hypertension despite non pharmacological measures G Stage 2 hypertension G non pharmacological measures G start medications ( Table 3 ) G add drug therapy only after discussion with a consultant

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Hypertension 2012-14 OUT-PATIENT MANAGEMENT

Table 1: Blood pressure (BP) for boys by age and height percentiles

Systolic (mmHg) percentile of height Diastolic (mmHg) percentile of height Age BP 5th 10th 25th 50th 75th 90th 95th 5th 10th 25th 50th 75th 90th 95th (years) percentile 1 90th 94 95 97 99 100 102 103 49 50 51 52 53 53 54 95th 98 99 101 103 104 106 106 54 54 55 56 57 58 58 99th 105 106 108 110 112 113 114 61 62 63 64 65 66 66 2 90th 97 99 100 102 104 105 106 54 55 56 57 58 58 59 95th 101 102 104 106 108 109 110 59 59 60 61 62 63 63 99th 109 110 111 113 115 117 117 66 67 68 69 70 71 71 3 90th 100 101 103 105 107 108 109 59 59 60 61 62 63 63 95th 104 105 107 109 110 112 113 63 63 64 65 66 67 67 99th 111 112 114 116 118 119 120 71 71 72 73 74 75 75 4 90th 102 103 105 107 109 110 111 62 63 64 65 66 66 67 95th 106 107 109 111 112 114 115 66 67 68 69 70 71 71 99th 113 114 116 118 120 121 122 74 75 76 77 78 78 79 5 90th 104 105 106 108 110 111 112 65 66 67 68 69 69 70 95th 108 109 110 112 114 115 116 69 70 71 72 73 74 74 99th 115 116 118 120 121 123 123 77 78 79 80 81 81 82 6 90th 105 106 108 110 111 113 113 68 68 69 70 71 72 72 95th 109 110 112 114 115 117 117 72 72 73 74 75 76 76 99th 116 117 119 121 123 124 125 80 80 81 82 83 84 84 7 90th 106 107 109 111 113 114 115 70 70 71 72 73 74 74 95th 110 111 113 115 117 118 119 74 74 75 76 77 78 78 99th 117 118 120 122 124 125 126 82 82 83 84 85 86 86 8 90th 107 109 110 112 114 115 116 71 72 72 73 74 75 76 95th 111 112 114 116 118 119 120 75 76 77 78 79 79 80 99th 119 120 122 123 125 127 127 83 84 85 86 87 87 88 9 90th 109 110 112 114 115 117 118 72 73 74 75 76 76 77 95th 113 114 116 118 119 121 121 76 77 78 79 80 81 81 99th 120 121 123 125 127 128 129 84 85 86 87 88 88 89 10 90th 111 112 114 115 117 119 119 73 73 74 75 76 77 78 95th 115 116 117 119 121 122 123 77 78 79 80 81 81 82 99th 122 123 125 127 128 130 130 85 86 86 88 88 89 90 11 90th 113 114 115 117 119 120 121 74 74 75 76 77 78 78 95th 117 118 119 121 123 124 125 78 78 79 80 81 82 82 99th 124 125 127 129 130 132 132 86 86 87 88 89 90 90 12 90th 115 116 118 120 121 123 123 74 75 75 76 77 78 79 95th 119 120 122 123 125 127 127 78 79 80 81 82 82 83 99th 126 127 129 131 133 134 135 86 87 88 89 90 90 91 13 90th 117 118 120 122 124 125 126 75 75 76 77 78 79 79 95th 121 122 124 126 128 129 130 79 79 80 81 82 83 83 99th 128 130 131 133 135 136 137 87 87 88 89 90 94 94 14 90th 120 121 123 125 126 128 128 75 76 77 78 79 79 80 95th 124 125 127 128 130 132 132 80 80 81 82 83 84 84 99th 131 132 134 136 138 139 140 87 88 89 90 91 95 92 15 90th 122 124 125 127 129 130 131 76 77 78 79 80 80 81 th 95 126 127 129 131 133 134 135 81 81 82 83 8474 85 85 99th 134 135 136 138 140 142 142 88 89 90 91 92 93 93 16 90th 125 126 128 130 131 133 134 78 78 79 80 81 85 82 95th 129 130 132 134 135 137 137 82 83 83 84 85 86 87 99th 136 137 139 141 143 144 145 90 90 91 92 93 94 94 17 90th 127 128 130 132 134 135 136 80 80 81 82 83 84 84 95th 131 132 134 136 138 139 140 84 85 86 87 87 88 89 99th 139 140 141 143 145 146 147 92 93 93 94 95 96 97

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Table 2: Blood pressure (BP) for girls by age and height percentiles

Systolic (mmHg) percentile of height Diastolic (mmHg) percentile of height Age BP 5th 10th 25th 50th 75th 90th 95th 5th 10th 25th 50th 75th 90th 95th (years) percentile 1 90th 97 97 98 100 101 102 103 52 53 53 54 55 55 56 95th 100 101 102 104 105 106 107 56 57 57 58 59 59 60 99th 108 108 109 111 112 113 114 64 64 65 65 66 67 67 2 90th 98 99 100 101 103 104 105 57 58 58 59 60 61 61 95th 102 103 104 105 107 108 109 61 62 62 63 64 65 65 99th 109 110 111 112 114 115 116 69 69 70 70 71 72 72 3 90th 100 100 105 103 104 106 106 61 62 62 63 64 64 65 95th 104 104 105 107 108 109 110 65 66 66 67 68 68 69 99th 111 111 113 114 115 116 117 73 73 74 74 75 76 76 4 90th 101 102 103 104 106 107 108 64 64 65 66 67 67 68 95th 105 106 107 108 110 111 112 68 68 69 70 71 71 72 99th 112 113 114 115 117 118 119 76 76 76 77 78 79 79 5 90th 103 103 105 106 107 109 109 66 67 67 68 69 69 70 95th 107 107 108 110 111 112 113 70 71 71 72 73 73 74 99th 114 114 116 117 118 120 120 78 78 79 79 80 81 81 6 90th 108 105 106 108 109 110 111 68 68 69 70 70 71 72 95th 104 109 110 111 113 114 115 72 72 73 74 74 75 76 99th 115 116 117 119 120 121 122 80 80 80 81 82 83 83 7 90th 106 107 108 109 111 112 113 69 70 70 71 72 72 73 95th 110 111 119 113 115 116 116 73 74 74 75 76 76 77 99th 117 118 119 120 122 123 124 81 81 82 82 83 84 84 8 90th 108 109 110 111 113 114 114 71 71 71 72 73 74 74 95th 112 112 114 115 116 118 118 75 75 75 76 77 78 78 99th 119 120 121 122 123 125 125 82 82 83 83 84 85 86 9 90th 110 110 112 113 114 116 116 72 72 72 73 74 75 75 95th 114 114 115 117 118 119 120 76 76 76 77 78 79 79 99th 121 121 123 124 125 127 127 83 83 84 84 85 86 87 10 90th 112 112 114 115 116 118 118 73 73 73 74 75 76 76 95th 116 116 117 119 120 121 122 77 77 77 78 79 80 80 99th 123 123 125 126 127 129 129 84 84 85 86 86 87 88 11 90th 114 114 116 117 118 119 120 74 74 74 75 76 77 77 95th 118 118 119 121 122 123 124 78 78 78 79 80 81 81 99th 125 125 126 128 129 130 131 85 85 86 87 87 88 89 12 90th 116 116 117 119 120 121 122 75 75 75 76 77 78 78 95th 119 120 121 123 124 125 126 79 79 79 80 81 82 82 99th 127 127 128 130 131 132 133 86 86 87 88 88 89 90 13 90th 117 118 119 121 122 123 124 76 76 76 77 78 79 79 95th 121 122 123 124 126 127 128 80 80 80 81 82 83 83 99th 128 129 130 132 133 134 135 87 87 88 89 89 90 91 14 90th 119 120 121 122 124 125 125 77 77 77 78 79 80 80 95th 123 123 125 126 127 129 129 81 81 81 82 83 84 84 99th 130 131 132 133 135 136 136 88 88 89 90 90 91 92 15 90th 120 121 122 123 125 126 127 78 78 78 79 80 81 81 95th 124 125 126 127 129 130 131 82 82 82 83 84 82 85 99th 131 132 133 134 136 137 138 89 89 90 91 91 92 93 16 90th 121 122 123 124 126 127 128 78 78 79 80 81 81 82 95th 125 126 127 128 130 131 132 82 82 83 84 85 85 86 99th 132 133 134 135 137 138 139 90 90 90 91 92 93 93 17 90th 122 122 123 125 126 127 128 78 79 79 80 81 81 82 95th 125 126 127 129 130 131 132 82 83 83 84 85 85 86 99th 133 133 134 136 137 138 139 90 90 91 91 92 93 93

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Table 3: Drugs commonly used for management of hypertension in children

Drug Mechanism of action Advice

Atenolol Beta-adrenoceptor G Reduces heart contractility – contraindicated in blocker early stages of hypertensive heart failure G Avoid in confirmed asthmatics Labetalol Non-cardioselective G Combining alpha- and beta-blockade reduces beta-blocker with tachycardia that can be a problem without beta- additional alpha-blocking blockade properties G Contraindicated in asthmatics and in heart failure Nifedipine Calcium channel blocker G Can be used in heart failure as any negative inotropic effect offset by a reduction in left ventricular work Enalapril Angiotensin-converting G Recommended in children with renal enzyme (ACE) inhibitor hypertension. First dose should be given at night to prevent transient hypotension G In children with impaired renal function, check serum creatinine and potassium 2–3 days after starting treatment and consider withdrawal if they have risen G Contraindicated in bilateral renal artery stenosis Losartan Angiotensin II receptor G Second line if enalapril contraindicated or not blocker tolerated G In children with impaired renal function, check serum creatinine and potassium 2–3 days after starting treatment and consider withdrawal if they have risen G Contraindicated in bilateral renal artery stenosis Sodium Vasodilator G Use for hypertensive emergencies nitro-prusside G Avoid in hepatic or renal impairment G Monitor blood cyanide if used >3 days

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RECOGNITION AND Abdomen ASSESSMENT G Swelling and shifting dullness: Definition suggest ascites G Tenderness with fever, umbilical flare: A triad of features: suggest peritonitis G Oedema G Scrotal oedema: stretching can cause G Hypoalbuminaemia: plasma albumin ulceration or infection <25 g/L Investigations G Heavy proteinuria, defined as: G dipstick 3+ or more, or Femoral blood sampling is G 2 urinary protein >40 mg/m /hr, or contraindicated because of risk G early morning protein:creatinine ratio of thrombosis >200 mg/mmol Symptoms and signs Urine

Oedema G Urinalysis G Early morning urine protein:creatinine G Peri-orbital, pedal, sacral, scrotal ratio first morning after admission G Also ascites or pleural effusion G normal value <20 mg/mmol; nephrotic Cardiovascular >200 mg/mmol, usually >600 mg/mmol

Difficult to assess due to oedema Baseline bloods Assess for hypovolaemia carefully G U&E and creatinine G Child with diarrhoea and vomiting and G Albumin looks unwell G G Abdominal pain: strongly suggestive FBC G G Poor peripheral perfusion and capillary Immunoglobulins G, A and M refill >2 sec G Complement C3 and C4 G Pulse character: thready, low volume, G Zoster immune status: as a baseline difficult to palpate G Hepatitis B and C serology G Tachycardia or upward trend in pulse rate Second-line tests G Hypotension: a late sign Request only if features suggestive of G Jugular venous pressure (JVP) low more aggressive nephritis (hypertension, macroscopic haematuria, high creatinine, Muffled heart sounds suggest no response to corticosteroids) pericardial effusion G Anti-streptolysin O titre and Respiratory anti-DNase B G Antinuclear antibodies G Tachypnoea and recession: suggest G Anti-ds DNA antibodies pleural effusion

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Interpretation Medication

G High haematocrit suggests G Prednisolone 60 mg/m 2 oral once hypovolaemia daily (maximum 80 mg), in the G Raised creatinine or urea suggests morning (see BNFc for surface area) hypovolaemia, tubular plugging or G Phenoxymethylpenicillin (Penicillin V) other nephritis for pneumococcal prophylaxis G Serum cholesterol and triglycerides: G If oedema upsetting to patient or often elevated causing breathlessness, add G IgG usually low furosemide 1–2 mg/kg oral or 1 mg/kg IV over 5–10 min G C3 normal G may intensify hypovolaemia, in which Differential diagnosis case use 20% albumin: discuss with consultant G Minimal change disease (95%) G If disease severe, especially with G Focal segmental glomerular sclerosis hypovolaemia, as judged by poor (FSGS) perfusion, high haemoglobin, G Multisystem disorders (e.g. HSP, thrombophilia, or abdominal pain or if diabetes mellitus, SLE, and malaria) relapse for >2 weeks, treat with G Congenital nephrotic syndrome very dipyridamole to reduce risk of rare thrombotic complications. Discuss need for heparin/warfarin with IMMEDIATE TREATMENT specialist COMPLICATIONS General

G Admit Acute hypovolaemia G Strict fluid balance monitoring G Abdominal pain, looks unwell, G daily weight: mandatory tachycardia, poor perfusion, high Hb G Avoid added salt, but a low salt diet G Seek senior advice before volume not indicated resuscitation, as a risk of volume G Manage hypovolaemia – see overload Complications G give human albumin 4.5% (if G seek senior advice before volume available) 10 mL/kg immediately or resuscitation, as a risk of volume sodium chloride 0.9% 10 mL/kg overload immediately Fluid restriction Do not confuse 4.5% albumin with 20% as the latter is G Restrict to usual maintenance intake hyperosmolar and can easily (insensible losses plus output) cause fluid overload G If not tolerated, aim for: G 600 mL/day in children aged <5 yr G Start dipyridamole G 800 mL/day in children aged 5–10 yr G 1000 mL/day in children aged >10 yr

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Chronic hypovolaemia G Pulmonary vein G Femoral vein: femoral blood sampling G More common in corticosteroid- contraindicated resistant disease G A fall in platelets, rise in D-Dimers and G Looks unwell, abdominal pain and reduced PTT are suggestive vomiting G USS with Doppler study to look at G Low JVP, rising urea and creatinine, perfusion and to image renal vein and and poor response to diuretics IVC can be helpful. If in any doubt, G Treatment: check with consultant first seek advice from nephrologist G salt-poor hyperosmolar albumin 20% regarding investigation/management 0.5–1.0 g/kg (2.5–5.0 mL/kg) over DISCHARGE POLICY AND 2–4 hr with furosemide 1–2 mg/kg IV SUBSEQUENT MANAGEMENT midway through infusion G regular observations for signs of G Discharge once in remission circulatory overload (e.g. raised JVP, G defined as trace/negative urine protein tachycardia, gallop rhythm, for 3 days breathlessness) G patients with normal BP and stable G often required daily or twice daily: weight who are well may be allowed liaise with a specialist centre home on ward leave with consultant G Start dipyridamole approval. Normally twice weekly Peritonitis review will be required until in remission G Difficult to recognise G Arrange plan of care with patient and below G steroids may mask signs, including carers – see fever, or cause leucocytosis G Out-patient review in 4 weeks G Abdominal pain New patients G consider hypovolaemia and appendicitis: request an early surgical G Prednisolone 60 mg/m 2 (maximum opinion 80 mg) once daily for 4 weeks G Obtain blood culture and peritoneal G Then 40 mg/m 2 (maximum 60 mg) fluid (for gram stain and culture) if alternate days for 4 weeks possible, then start piperacillin with tazobactam (Tazocin ®) IV pending G gradually reduce dose culture results G Response usually apparent in 7–10 days Cellulitis G No response after 4 weeks suggests G Commonly caused by haemolytic corticosteroid resistance streptococci and pneumococci – treat promptly Thrombosis

G Renal vein: an important differential in abdominal pain G Cerebral vasculature

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Relapsing patients G Continue penicillin prophylaxis until oedema has resolved G Three consecutive days of 3+ or more G If zoster non-immune (VZV IgG early morning proteinuria, having negative) and on high-dose previously been in remission corticosteroids, give intramuscular zoster immunoglobulin: G Start prednisolone 60 mg/m 2 (maximum 80 mg) once daily G after definite zoster contact, a contact will be infectious 2 days before onset G continue until nil or trace proteinuria of rash, and cease when all lesions for 3 days are crusted over G then 40 mg/m 2 (maximum 60 mg) G can be given up to 10 days after alternate days for a further 4 weeks exposure. Contact consultant G If relapses frequent despite alternate- microbiologist on duty for release of day prednisolone, discuss with a VZIG paediatric nephrologist G at first sign of illness give aciclovir IV Oral prednisolone G varicella vaccine (live vaccine) available and should be given if a G While on prednisolone 60 mg/m 2 once suitable opportunity arises between daily advise to: relapses G carry a corticosteroid card G If child has not received G seek prompt medical attention for pneumococcal conjugate vaccine – illness, especially zoster contacts see BNFc for schedule Other management Refer for specialist advice if:

G Urine testing G Corticosteroid-resistant disease G teach technique and provide G non-responsive after 4 weeks of daily appropriate dipsticks prednisolone, but start discussions with specialist centre in third week G test only first daily urine sample G Corticosteroid-dependent disease G keep a proteinuria diary G two consecutive relapses during G Corticosteroid diary with instructions corticosteroid treatment or within regarding corticosteroid dosage 14 days of cessation Infectious precautions G Significant corticosteroid toxicity G Aged <1 yr or >12 yr at first G Avoid live immunisations for 3 months presentation after period of treatment with high- dose corticosteroids G Mixed nephritic/nephrotic picture: macroscopic (not microscopic) G Benefit of inactivated vaccines can be haematuria, renal insufficiency or impaired by high-dose corticosteroids hypertension and so a similar delay advisable where possible G Low complement C3/C4 G where not possible because of frequent relapse, give INACTIVATED vaccines after a shorter delay and check for an antibody response

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RECOGNITION AND OUT-PATIENT MANAGEMENT ASSESSMENT Investigations in patients with Definition proven renal calculi

G Presence of crystalline material within G Fasting (before breakfast) blood urinary tract sample for: Symptoms and signs G creatinine G calcium G Non-specific recurrent abdominal pain G phosphate G Dysuria or painful micturition G uric acid G Classical renal colic G venous bicarbonate Proteus G Urinary infection (particularly G pH (warm arterialised capillary sample spp) to coincide with urine pH) G Persistent pyuria G Random mid-stream urine G Macroscopic or microscopic G microscopy, culture and sensitivity haematuria G Early morning urine (first voided G Passage of stones specimen) and 24 hr collection G Renal failure (request ‘urinary stone screen’ and record height and weight on request Initial investigations form) for: G Renal ultrasound scan G calcium G Urine microscopy and culture G oxalate G citrate Further investigations G uric acid G DMSA scan G cystine G to determine function when calculi G creatinine multiple or large G pH (to coincide with blood pH) G Repeat renal ultrasound scan Stone analysis G to see if stones have been passed G to monitor progress of stones G May give useful information about G six weeks after treatment (see below) aetiology, discuss with biochemistry department first IMMEDIATE TREATMENT G If stone passage is frequent or associated with symptoms, ask G Analgesia for severe pain parents to strain urine G If obstruction is present, urgent referral to urology at renal specialist centre G Cefalexin oral if symptomatic for urinary tract infection, adjusted once sensitivities available G antibiotic treatment unlikely to eradicate organism in presence of stones

Issue 5 Issued: May 2013 Expires: May 2014 219 RENAL CALCULI • 2/4 Table 1: Characteristics of urinary stones Type Appearance Causes Radio- opaque* Magnesium Very soft, white, G Infection with urea-splitting No ammonium toothpaste consistency or organisms, especially in children phosphate gravel fragments with urinary stasis Calcium oxalate Hard grey-brown rough G Hypercalciuria (any cause) Yes surface G Hyperoxaluria Calcium Large, smooth, pale, G Infection Yes phosphate friable G Renal tubular acidosis G Vitamin D toxicity G Idiopathic hypercalciuria G Immobilisation G Hyperparathyroidism G Sarcoidosis Cystine Pale-yellow, crystalline G Cystinuria Yes Maple syrup Uric acid Hard, yellow G Lesch-Nyhan syndrome No G Dietary G Induction in haematological malignancies Xanthine Smooth, soft, G Xanthinuria No brown yellow Dihydroxyadenine Friable, grey-blue G Adenine phosphoribosyl No transferase deficiency * Radiolucency depends on amount of calcium in the stone and individual patient can have more than one type of stone, each with different radiolucencies

Interpretation of results G aged <6 months: 0.35 G aged 6–11 months: 0.2 G Urinary pH G aged 1–2 yr: 0.18 G pH <5.3 in presence of normal G aged 3–6 yr: 0.11 capillary pH and bicarbonate excludes G distal renal tubular acidosis aged 7–14 yr: 0.08 G G when above criteria not met, a more aged >14 yr: 0.065 formal test of renal acidification G Uric acid/creatinine (mmol/mmol) ratio required in those with nephrocalcinosis is age-dependent, and suggestive of or in recurrent stone formers hyperuricaemia if it exceeds following thresholds: G pH >6 with capillary bicarbonate <18 mmol/L is seen in mild distal G aged <1 yr: 1.5 tubular acidosis G aged 1–2 yr: 1.26 G Calcium:creatinine (mmol/mmol) ratio G aged 3–6 yr: 0.83 consistently >0.2 indicates G aged 7–10 yr: 0.67 hypercalciuria G aged 11–14 yr: 0.45 G Oxalate:creatinine (mmol/mmol) ratio is age-dependent, and suggestive of G aged >14 yr: 0.4 hyperoxaluria if it exceeds following G Magnesium:creatinine ratio <0.2 may thresholds: increase stone formation

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G Calcium:citrate ratio <0.6 may G pyridoxine can be used in increase stone formation hyperoxaluria G Cystine, if present, is indicative of G alpha-mercaptopropionylglycine or cystinuria penicillamine may be useful for G Overall solubility index (RS value) cystine stones under specialist recommendation as can cause bone G negative value: stable urine marrow suppression and nephrotic G value 0–1: metastable (liable to syndrome precipitate if seeded) G For large stones that are unlikely to G value >1: spontaneous precipitation pass, surgical removal or lithotripsy TREATMENT may be required G modality of treatment determined by G Treat any metabolic disorder identified location and size of stone by above investigations, seek advice G generally, stones <2 cm suitable for from regional nephrology service lithotripsy G Keep urine free from infection, G larger stones treated by percutaneous particularly in those with history of nephrolithotomy (PCNL) or by open Proteus infection by prompt treatment operation if symptomatic G nephrectomy may be advised where G Advise liberal fluid intake kidney function poor G adolescent 3 L/day G pre-puberty (school age) 1.5 L/day G Additional measures for recurrent stone formation or idiopathic hypercalciuria (in order): G dietary assessment to optimise oxalate, vitamin C, calcium, and vitamin D intake G reduced sodium intake in idiopathic hypercalciuria, if sodium excretion >3 mmol/kg/day G high fibre diet with cellulose or whole wheat flour to reduce calcium and oxalate absorption G potassium citrate at a starting dose of 0.5 mEq/kg 12-hrly in patients with low urinary citrate G bendroflumethiazide 1–2 mg/kg/day to reduce calcium and oxalate excretion (unlicensed). Usual dose 50–100 microgram/kg/day if aged <2 yr, 50–400 microgram/kg/day if aged >2 yr; then maintenance of 50–100 microgram/kg up to max 10 mg, aged 12–18 yr 5–10 mg/day

Issue 5 Issued: May 2013 Expires: May 2014 221 RENAL CALCULI • 4/4 Algorithm for metabolic investigations

Paediatric stone patient

Elimination of stones by spontaneous passage or active removal [extracorporeal shockwave lithotripsy (SWL), surgery]

Stone analysis

Mg Ammonium Uric acid Calcium stones Cystine phosphate (struvite) stone CaOX-CaPO

Urine culture Urine pH Urine pH Urine and serum Urine cystine Uric acid levels level Possibly urease producing bacteria Acidic urine Hyperuricosuria Cystinuria Hyperuricaemia

Total elimination of Alkali replacement High fluid intake stone (surgery/SWL) – potassium citrate Potassium citrate antibiotics Allopurinol Alpha-mercaptopropionylglycine Low purine diet Penicillamine

Urine-blood pH

Serum parathyroid Hypercalcaemia hormone (PTH) Urine – blood Ca – uric acid levels, Mg, phosphate urine Ca – oxalate – citrate – Mg, uric acid – phosphate

Urine pH >5.5 Urine pH <5.5

Hyperparathyroidism Further investigation for renal tubular acidosis

Hypercalciuria Hyperoxaluria Hyperuricosiuria Hypocitraturia

K-citrate Diet low in Alkali Citrate Diet (normal calcium low oxalate replacement replacement sodium intake) K-citrate (k-citrate) K-citrate Bendroflumethiazide diuretic Pyridoxine Allopurinol

Issue 5 Issued: May 2013 222 Expires: May 2014 RENAL FAILURE • 1/3 RECOGNITION AND G Ascites ASSESSMENT G BP/capillary refill G Jugular venous pressure (JVP) Definition G Urine output Immediate investigations G Acute renal failure: sudden deterioration in renal function associated with G See separate guidelines for specific retention of nitrogenous waste and causes acute disturbance of water and G Blood electrolyte balance G U&E, creatinine, calcium, phosphate, Presentation uric acid, magnesium, LFT’s and bicarbonate G Poor/absent urine output (oliguria) G FBC with puffiness/oedema: G venous blood gas G neonates <0.6 mL/kg/hr G Urine G infant/child <0.5 mL/kg/hr G urinalysis for blood, protein, nitrites Differential diagnosis and leucocytes G osmolality Pre-renal G electrolytes G Renal ultrasound scan G Secondary to hypotension (e.g. G size and appearance of kidneys hypovolaemia from gastroenteritis or G septicaemia) inflammation and swelling G G Urine osmolality >300 mOsm/kg evidence of obstruction G Urine:plasma urea ratio >5 IMMEDIATE TREATMENT G Urine sodium <20 mmol/L G Correct volume status and maintain G Good response to diuretics after fluid and electrolyte balance correction of hypovolaemia G Prevent hyperkalaemia Renal G Treat underlying cause where appropriate G Haemolytic uraemic syndrome – see Haemolytic uraemic syndrome G Maintain adequate nutrition guideline Fluid and sodium balance G Acute nephritis – see Glomerulonephritis guideline Initial correction G Acute tubular necrosis or renal vein G thrombosis Dehydration G G Unrecognised chronic renal failure for shock, give sodium chloride 0.9% (oliguria usually not a feature) 20 mL/kg immediately G G Acute-on-chronic renal failure (e.g. for correction of dehydration – see Diarrhoea and vomiting dehydration or infection) guideline G Volume overload/hypertension Post-renal G low serum sodium usually indicates G Urinary tract obstruction (rare) fluid overload G furosemide 1 mg/kg IV immediately Assessment (max rate: 500 microgram/kg/min up G Hydration (under/over) to 4 mg/min): if no urine output after 30 min, give a further 1 mg/kg and if still G Weight no urine a third 1 mg/kg after 30 min G Skin (turgor/oedema)

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Metabolic acidosis G Once toxicity develops, the following (see Table 1 ) are holding measures G Sodium bicarbonate may be required whilst dialysis is set up – discuss with on-call consultant G give salbutamol IV or by nebuliser if Potassium no IV access as first-line emergency treatment, followed by oral/rectal G Hyperkalaemia can lead to cardiac calcium polystyrene sulphonate (even arrest or serious arrhythmias if salbutamol effective) to start to G severely restrict potassium intake by reduce potassium load introducing low potassium diet and G if ECG still unstable, give calcium avoiding potassium in IV fluids unless gluconate by slow IV injection serum potassium <3.5 mmol/L or G if patient acidotic pH <7.30, give there are ongoing losses sodium bicarbonate G If potassium >6.0 mmol/L, ECG G if further reduction required after other monitoring essential measures implemented, use insulin G watch for development of prolonged and glucose P-R interval and/or peaked T wave G After starting treatment discuss with G as toxicity worsens, P wave is lost, on-call consultant QRS widens and S-T depression develops Table 1: Emergency treatment of hyperkalaemia Treatment Dose Onset Mode of action Salbutamol 2.5 mg (<25 kg) 5 min. Lasts up to 2 hr; Shifts potassium into nebuliser 5 mg (>25 kg) repeat as necessary cells Salbutamol 4 microgram/kg over 5 min Immediate. Effect Shifts potassium into infusion repeat as necessary. maximal at 60 min cells Limited by tachycardia Calcium 0.5 mL/kg IV (max 20 mL) 1 min Antagonises effect of gluconate 10% over 5–10 min. Monitor ECG Repeat after 5 min if high potassium Do NOT administer through ECG changes persist same line as bicarbonate Sodium 1 mmol/kg IV over 15 min 1 hr Shifts potassium into bicarbonate (2 mL/kg of 4.2% diluted 1 in Effect may last 2 hr cells 4.2% infusion 5 with sodium chloride 0.9%) (only if patient Do NOT administer through acidotic) same line as calcium Glucose/insulin Glucose 10% 0.5 g/kg/hr 15 min. Effect may Shifts potassium into infusion (5 mL/kg/hr) and when blood last several hours cells glucose >10 mmol/L infuse Frequent glucose stick insulin 0.1 units/kg/hr stop checks when glucose stops when K+ falls by 0.5 mmol/L Furosemide 1 mg/kg IV over 5 min May not be effective Potassium excreted in in chronic renal failure urine Polystyrene Calcium polystyrene Oral 2 hr Removes potassium sulphonate sulphonate Rectal 30 min (irrigate from body resins 250 mg/kg 6-hrly (max to remove residue 15 g/dose) oral/rectal 6–8 hrly before next dose)

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G Hypokalaemia is also dangerous Indications for dialysis G if patient becomes potassium depleted from heavy ongoing losses (fistula or G Fluid overload diuretic phase), it is most important G Uncontrolled hypertension (for height- that replacement is given related 97 th centiles – see G amount and rate of replacement Hypertension guideline) depend on estimation of losses and G Potassium toxicity (as indicated by response to initial supplementation. If in features listed previously) doubt, discuss with on-call consultant G Metabolic acidosis (pH <7.2) SUBSEQUENT MANAGEMENT unresponsive to base supplementation G Convulsions Fluid and sodium balance G Loss of general well being +/- alteration in conscious level – see G Once normal hydration restored, aim Glasgow coma score guideline to replace insensible loss G (300–400 mL/m 2/day) + urine output + Spontaneous resumption of renal other losses function likely to be delayed G G In anuric patients (as opposed to acute-on-chronic renal failure oliguric), give fluids that are free of G haemolytic uraemic syndrome electrolytes to compensate for MONITORING TREATMENT insensible loss; in patients having IV fluids, glucose 5% is most appropriate G Accurate fluid balance – maintain initially, although glucose 4%/sodium strict input-output chart chloride 0.18% may be required later to compensate for sodium loss from G Re-assess fluid intake at least 12-hrly sweat G Record weights, plasma sodium and G Replace sodium losses in urine and in PCV as indicators of hydration other fluids (diarrhoea, gastric G Check K + hourly if >6 or <3 mmol/L aspirate, fistula) G Check U&E 12-hrly if K + 3–6 mmol/L G in most patients, dietary sodium will in renal failure suffice G Respond promptly to increase in urine G in those with large fluid losses, volume, fall in serum creatinine and consider IV sodium to match losses increase in urine osmolality by Nutrition increasing fluid intake to prevent prolonged oliguria G Involve a paediatric dietitian G Once diuresis begins, increase electrolyte replacement, including G A low-protein high-energy diet is ideal potassium (aim for energy intake of 400 kcal/m 2) G once stable, reduce fluid intake G Avoid high potassium foods gradually to avoid prolonged diuretic G Be realistic about what a child will take phase

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PROTEIN EXCRETION Tubular proteinuria

G As a diagnostic indicator in any child G Request retinol binding protein thought to have an underlying renal (RBP):creatinine ratio, elevation disorder confirms tubular proteinuria G To monitor progress in renal disorders OSMOLALITY G Normally glomerular, rarely tubular in origin G Used to exclude urinary concentrating G Investigate as below in patients with disorders persistent proteinuria where cause is G patients with polyuria (may present as unknown wetting or excessive drinking) G Request protein:creatinine ratio ( must G Test early morning urine after be first urine specimen voided in the overnight fast >870 mOsm/kg virtually morning); elevation confirms excludes a concentrating defect glomerular proteinuria G if concern re diabetes insipidus, do Protein:creatinine ratio water deprivation tests during the day SODIUM EXCRETION G Best performed on first urine specimen voided in the morning G Fractional sodium excretion (FE Na ) G Upper limit of normal <20 mg/mmol assesses capacity to retain sodium G Significant proteinuria >100 mg/mmol G ensure normal sodium intake (dietitian G Heavy proteinuria (nephrotic) to advise) >200 mg/mmol G stop any existing supplements 6 hr Timed urine collection before taking samples G document weight loss after G Only appropriate for older patients supplements stopped, may provide (out of nappies) useful supporting evidence G Night-time collection to rule out G random urine sample for urinary orthostatic proteinuria sodium (U Na ) and creatinine (U cr ) G empty bladder at bedtime and discard G blood sample immediately after sample voiding for plasma sodium (P Na ) and G collect all urine passed during the creatinine (P cr ) night G enter results into equation (using G empty bladder on rising in morning same units for U and P; and collect urine 1000 micromol = 1 mmol) G record time from bladder emptying at night to bladder emptying in morning G FE Na = U Na .P Cr x 100 G Calculate protein output as mg/m 2/hr PNa .U Cr (see BNFc for surface area) G normal values for FE G Upper limit of normal = 2.5 mg/m 2/hr Na aged 0 to 3 months <3 G Heavy proteinuria >40 mg/m 2/hr aged >3 months <1

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PLASMA CREATININE

G Mean and upper limit dependent on height but can be determined roughly from child’s age if height not available Table 1 Age Height (cm) Mean ( µmol/L) Upper limit Up to 2 weeks 66 87 2 weeks to 6 months 50 44 58 6 months to 1 yr 60 34 49 2 yr 87 28 39 4 yr 101 33 43 6 yr 114 38 52 8 yr 126 42 57 10 yr 137 46 62 12 yr 147 52 69 Adult female 163 68 89 Adult male 174 86 108

GLOMERULAR FILTRATION RATE (GFR)

G Serial measurements of glomerular filtration rate (in mL/min/1.73 m 2) predict rate of deterioration when renal function impaired Table 2 Age Mean GFR Range (2 SD) (mL/min/1.73 m 2) Up to 1 month 48 28–68 1–6 months 77 41–103 6–12 months 103 49–157 1–2 yr 127 63–191 2–12 yr 127 89–165

Plasma creatinine method 51 Cr-EDTA slope clearance

G Estimates GFR in children with G Use only when GFR needs to be reasonable accuracy from P Cr and determined very accurately height, using following formula: G Request via nuclear medicine 2 GFR (mL/min 1.73 m ) = G Provide height and weight of child 40 x height (cm) G ‘correct’ result for surface area and P (µmol/L) Cr expressed as per 1.73 m 2 G Not suitable for children: G if result expressed as mL/min ‘correct’ G aged <3 yr for surface area G with muscle disease/wasting

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ULTRASOUND

Indications

G To indentify structural abnormalities of urinary tract Table 3: Normal values for renal ultrasound measurement Age Length (mm) Range (mm) Up to 3 months 45 35–60 3–6 months 50 50–60 6–9 months 55 52–60 9–12 months 58 54–64 1–3 yr 65 54–72 3–6 yr 75 64–88 6–9 yr 80 73–86 9–12 yr 86 73–100

G Measurements of pelvicalyceal size at G When assessing obstruction in dilated hilum of kidney (during 3 rd trimester): system or outcome of pyeloplasty, give G <9 mm: mild (do not need any furosemide 0.5 mg/kg slow IV bolus intervention/follow-up) over 3–10 min (max rate 4 mg/min) 15 min before giving isotope. Helps to G 9–15 mm: moderate differentiate genuine obstruction from G >15 mm: severe isotope pooling, provided function of affected kidney not severely impaired ISOTOPE SCANS G Do not use furosemide for indirect cystography Dynamic imaging (MAG3) Static imaging ( 99m Tc-DMSA) Indications Indications G To assess obstruction in dilated system G To assess differential function between G To assess drainage 6 months after kidneys and within duplex kidneys pyeloplasty G To locate an ectopic kidney G Indirect cystography in older children G To identify renal scars after recovery before and/or after surgical correction from acute infection of reflux G atypical UTI aged <3 yr or recurrent Operational notes UTI any age Operational notes G Request via nuclear medicine G SHO or nurse required to insert G Request via nuclear medicine venous cannula in young children G Scan kidney 2–6 hr after injection G Consider sedation if child has had G Sedation rarely required previous problems lying still during G examinations Delay DMSA for 3–6 months after infection to avoid false positive G Maintain good hydration

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X-RAY IMAGING

Micturating cystourethrogram (MCUG)

To assess bladder for vesicoureteric reflux Indications

G Atypical or recurrent UTI aged <6 months G Recurrent or atypical UTI in children aged >6 months, but <3 yr if: G dilatation on ultrasound G poor urine flow G non- E. coli infection G family history of VUR Operational notes

G Give prophylactic antibiotics oral for 3 days with MCUG taking place on the second day G Urethral catheter will need to be passed in X-ray dept

Issue 5 Issued: May 2013 Expires: May 2014 229 URINARY TRACT INFECTION • 1/5 RECOGNITION AND ASSESSMENT

Treat symptomatic urinary tract infection (UTI) in infants promptly to reduce risk of renal scarring Symptoms and signs

Age group Most common Intermediate Least common Infants G Fever G Poor feeding G Abdominal pain aged G Vomiting G Failure to thrive G Jaundice <3 months G Lethargy G Haematuria G Irritability G Offensive urine G Fever G Abdominal pain G Lethargy Infants G Loin tenderness G Irritability and Pre- G Vomiting G Haematuria children verbal G G aged Poor feeding Offensive urine G ≥3 months Failure to thrive G Frequency G Dysfunctional voiding G Fever G Dysuria G Changes to continence G Malaise Verbal G Abdominal pain G Vomiting G Loin tenderness G Haematuria G Offensive urine G Cloudy urine

Risk factors for UTI and serious Investigations underlying pathology G Dipstick test fresh urine for leukocytes G The following should always be and nitrites in: recorded in suspected cases of UTI: G all symptomatic children (see Table G poor urine flow in males above) G history suggesting recurrent UTI G all unexplained febrile admissions with G recurrent fever of uncertain origin temp >38ºC G antenatally diagnosed renal or urinary G with an alternate site of infection but tract abnormality who remain unwell G family history of vesico-ureteric reflux G Culture urine if: (VUR) or renal disease G aged <3 yr G constipation G a single positive result for leukocyte G dysfunctional voiding esterase or nitrite G enlarged bladder G recurrent UTI G abdominal mass G infection that does not respond to treatment within 24–48 hr G evidence of spinal lesion G clinical symptoms and dipstick tests G poor growth do not correlate G high blood pressure G suspected pyelonephritis G If child seriously unwell, measure serum electrolytes, take blood cultures and insert cannula

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Collection of specimens G Use borate (red top) only when child large enough to fill bottle G Do not delay treatment if a sample G Keep specimen in fridge at 4ºC until cannot be obtained and child at high transfer to laboratory risk of serious illness G During working hours, transfer G Clean catch in sterile container is specimens to laboratory within 2 hr recommended method: G out-of-hours, keep specimen in fridge G in babies too young to co-operate, until laboratory open eliciting lateral abdominal reflex may G provoke micturition State date and time of collection on specimen bottle G Collect mid-stream urine in those old enough to co-operate Interpretation of results G Pad urine specimens can be used in babies and young children (only useful Always take clinical symptoms into if negative) account when interpreting results Children aged 3 yr: G make sure napkin area thoroughly G ≥ use dipstick to cleaned before applying pad diagnose UTI G urine extracted from specially G Both leukocyte esterase and nitrite designed pads with a syringe positive: start antibiotic treatment for UTI G always follow manufacturer’s instructions G Leukocyte esterase negative and nitrite positive: G do not use cotton wool balls or ‘home start antibiotic made’ equipment treatment, if fresh sample was tested. Send urine sample for culture G for urinalysis (do not send for culture: Leukocyte esterase positive and if +ve nitrites and +ve leukocytes G nitrite negative: collect another urine sample by clean only start antibiotic method) treatment for UTI if there is good clinical evidence of UTI. Send urine G Suprapubic aspiration only required to sample for microscopy and culture obtain urgent specimens in very ill child or where there is continuing G Both leukocyte esterase and nitrite diagnostic uncertainty negative: do not send urine sample for culture unless recommended in G always check there is urine in bladder by ultrasound first indications for culture. Do not start treatment for UTI G lie patient supine with legs held in frog position by assistant Microscopy of fresh sample G cleanse suprapubic skin with alcohol G Indications: G use 21G 3.5 cm needle G G insert midline 1–2 cm above aged <3 yr with fever symphysis pubis, with needle G aged >3 yr, fever with: perpendicular to skin - specific urinary symptoms G advance needle whilst applying gentle - history of recurrent UTI suction, urine aspirated on insertion - seriously ill Handling specimens - leukocyte esterase or nitrite on urinalysis (see Interpretation of G Use plain white-topped sterile bottles results ) for hospital-collected samples

Issue 5 Issued: May 2013 Expires: May 2014 231 URINARY TRACT INFECTION • 3/5 G Very useful method of confirming G when child on prophylaxis already, acute infection always give an alternative antibiotic G bacteria and leukocytes (UTI) for acute infection G bacteria only (UTI or contaminant) G Imaging: urgent ultrasound imaging is G leukocytes only (treat if symptomatic) only indicated in ‘atypical’ cases with: G no bacteria or leukocytes (no UTI) G seriously ill child G Pyuria G poor urine flow G normal <10 x 10 6/L G abdominal or bladder mass G vulvitis, vaginitis or balanitis can also G raised creatinine give rise to high counts G septicaemia G viruses (echovirus, adenovirus and G failure to respond to treatment within CMV) can cause sterile pyuria 48 hr G Colony counts G infection with organisms other than G organism count >10 5 organisms/mL E. coli pure growth confirms infection in properly collected and stored mid- SUBSEQUENT MANAGEMENT stream sample G certainty reduced to 80% with pad urine Imaging G low counts do not exclude infection Dependent of age and type of IMMEDIATE TREATMENT infection G Simple UTI: responds within 48 hr If child systemically unwell, do not delay G treatment while trying to obtain urine Atypical UTI: specimen G seriously ill child G Ensure good hydration with G poor urine flow maintenance fluids G abdominal or bladder mass G Empiric antibiotics (narrow spectrum G raised creatinine as soon as organism and sensitivities known) G septicaemia G If pyelophephritis: systemic illness G failure to respond to treatment within (fever >38ºC or loin pain/tenderness) 48 hr G aged <3 months: cefotaxime, aged G infection with organisms other than >3 months: co-amoxiclav oral if E. Coli tolerated or IV for 7 days G Recurrent UTI: - if penicillin allergy give gentamicin IV G 2 or more episodes of UTI with acute (once daily dosage regimen) over pyelonephritis/upper urinary tract 30 min for 48 hr minimum infection Septicaemia - if shocked refer to G one episode of UTI with acute guideline pyelonephritis/upper urinary tract - ongoing treatment depends on infection plus one or more episode or response UTI with cystitis/lower urinary tract G if cystitis: minor systemic disturbance, infection give cefalexin oral for 3 days G 3 or more episodes or UTI with G high rates of trimethoprim resistance cystitis/lower urinary tract infection (no longer first line)

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Test Simple UTI Atypical UTI Recurrent UTI Aged 0–6 months US during acute infection No Yes Yes US within 6 weeks Yes No No DMSA No Yes Yes MCUG No Yes Yes Aged 6 months–3 yr US during acute infection No Yes No US within 6 weeks No No Yes DMSA No Yes Yes MCUG No No No Aged >3 yr US during acute infection No Yes No US within 6 weeks No No Yes DMSA No No Yes MCUG No No No

G US 6 weeks after infection when not DISCHARGE AND FOLLOW-UP indicated urgently (see above) G Bladder scan pre/post micturition G Home when: helpful to exclude incomplete bladder G symptoms mild, or severe symptoms emptying controlled G DMSA (dimercaptosuccinic acid) scan G taking oral antibiotics and tolerating 6 months after infection them G If child has subsequent UTI while G discuss and advise to avoid risk awaiting DMSA, review timing of test factors at discharge: and consider doing it sooner - constipation G MCUG (micturating cysto- urethrography) 6 weeks after infection - poor perineal hygiene G also required where there are voiding - low fluid intake problems or abnormalities on US scan - infrequent bladder emptying requiring further investigation (discuss G Repeat urine test not required on with consultant) asymptomatic children G requires 3 days of prophylactic G Prompt treatment of recurrences with antibiotics, usually nitrofurantoin co-amoxiclav aged >3 months or cefalexin aged <3 months at night (max 100 mg) G Out-patient review according to previous culture G not required for simple UTI sensitivities, with test on middle day; G MCUG for neonates with in 8–10 weeks where ultrasound hydronephrosis give a single dose of imaging has been indicated IV gentamicin 5 mg/kg over 3–5 min G Prophylactic antibiotics just before MCUG (avoid MCUG in G not required following first simple UTI neonates with UTI)

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G Required for: Management of children with G proven grade 3+ reflux until aged 2 yr renal scars (provided infections well controlled) G No follow-up for minor unilateral G urinary tract obstruction pending parenchymal defect unless recurrent surgical management UTI or family history or lifestyle risk G any child with frequent symptomatic factors for hypertension infections (>3 urinary tract infections G per year) In cases of significant scarring: G annual BP measurement G aged >3 months: nitrofurantoin 1 mg/kg oral at night (max 100 mg) G females must book early when pregnant and inform obstetric team G Surgical management G Where scarring bilateral: G antireflux surgery not routinely indicated in VUR G annual BP measurement G refer for antireflux surgery for G assessment of urinary protein obstructive mega-ureters with reflux excretion and renal function every 3–4 yr G refer for antireflux surgery if failure to control infections with prophylaxis in G long-term follow-up in the renal clinic grade 3+ reflux G transfer to adult service G refer all neuropathic bladder patients G Circumcision may be considered for recurrent UTI in children with structurally abnormal urinary tracts

Issue 5 Issued: May 2013 234 Expires: May 2014 ARTHRITIS • 1/2

RECOGNITION AND G Arthritis associated with inflammatory ASSESSMENT bowel disease G monoarthritis in large joint or Definition peripheral arthritis associated with bowel disease activity G Acute, chronic or recurrent G Malignancy, especially leukaemia or inflammation of a one or more joints neuroblastoma Symptoms and signs G bone pain, lymphadenopathy, hepatosplenomegaly G Pain G Rickets and other endocrine disease G Stiffness (e.g. type 1 DM, thyroid disease) G Refusal to participate in usual G Acute septic arthritis (if fever even if activities history of chronic arthritis see Osteomyelitis and septic arthritis G Swollen, hot, red and/or tender joint guideline) G Reduced range of movement G Infectious causes (e.g. TB, rheumatic Differential diagnosis fever, Lyme disease) G Serum sickness following drug G Juvenile idiopathic arthritis (JIA): ingestion associated with urticarial G arthritis of unknown aetiology before rash age 16 yr (peak aged 1–5 yr) Rarer causes G persisting for ≥6 weeks G morning irritability, stiffness, gradual G Inherited metabolic diseases and refusal to participate in usual activities other genetic disorders G relatively little pain G Chronic recurrent multifocal osteomyelitis G any or multiple joint (rarely hip initially) G Auto-inflammatory diseases, including G Reactive arthritis chronic infantile neurological G history of diarrhoea (salmonella, cutaneous and arthritis syndrome shigella, campylobacter) G Haemophilia G viral illness (parvo, EBV, mumps, rubella) INVESTIGATIONS G monoarthritis of large joint G X-rays of joints most affected if child G 7–14 days after acute illness has features of other differential G self-limiting in response to an infection diagnoses that have radiological changes and, if severe, as a baseline G Reiters syndrome: conjunctivitis, sterile urethritis assessment to look for erosions G G Rheumatic fever (migratory arthritis, FBC, ESR, CRP, ASOT, rheumatoid history of tonsillitis) factor, ANA and if SLE suspected, ds- DNA auto-antibodies to exclude G Non-accidental injury (NAI) differential diagnoses or for JIA G Systemic rheumatic diseases, such as classification purposes (not useful to SLE, dermatomyositis, vasculitis confirm the diagnosis of JIA) (including HSP and Kawasaki disease)

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ACUTE MANAGEMENT DISCHARGE AND FOLLOW-UP

G Telephone local paediatric G Refer all children with suspected JIA rheumatology team for advice for and autoinflammatory connective management of musculoskeletal tissue diseases (e.g. SLE, conditions and assessment of pyrexia dermatomyositis, scleroderma and of unknown origin sarcoidosis) to paediatric G Analgesia /anti-inflammatory rheumatology service for urgent medications vary in individual side appointment effects and clinical effectiveness G Management will involve: G Use with caution in asthma, G exploring differential diagnoses angioedema, urticaria, rhinitis, G disease education coagulation defect, cardiac, hepatic or G renal impairment physiotherapy and rehabilitation G G Contraindicated in gastro-intestinal optimising medical treatment including ulceration or bleeding corticosteroid joint injections (nearly always under general anaesthetic), G Give a proton pump inhibitor if taking methotrexate, and the institution of other medicines that increase the risk shared care monitoring of upper GI side-effects or with serious co-morbidity

If JIA is a possible diagnosis, arrange early referral to local ophthalmologist to start screening program for uveitis, chronic anterior uveitis can be asymptomatic initially and can progress to irreversible loss of vision if referral delayed

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INTRODUCTION

Differential diagnosis varies with age (see also Arthritis guideline) Common/important diagnoses

Any age G Trauma (including NAI) G Septic arthritis G Reactive arthritis G Juvenile idiopathic arthritis (JIA) G Malignancy G Referred pain (e.g. from hip to knee) Aged 0 –4 yr G Developmental dysplasia of hip (DDH) G Transient synovitis G Non-accidental injury (NAI) Aged 4 –10 yr G Perthe’s G Transient synovitis Aged 11 –16 yr G Slipped upper femoral epiphysis (SUFE) G Gonococcal septicaemia

Irritable hip (transient synovitis) Slipped capital femoral epiphysis G Commonest reason for a limp in the pre-school age group G Late childhood/early adolescence G Usually occurs aged 3–8 yr G Weight often >90 th centile G History of recent viral URTI (1–2 weeks) G Presents with pain in hip or knee and associated limp G Child usually able to walk but with pain G G Child otherwise afebrile and well The hip appears externally rotated and shortened G Mild-moderate decrease in range of G hip movement, especially internal Decreased hip movement, especially rotation internal rotation G G Severe limitation of hip movement May be bilateral suggests septic arthritis HISTORY G Exclude septic arthritis: discuss with orthopaedics Ask about: Perthes disease G Trauma G Fever: shivering/sweating G Avascular necrosis of the capital G Recent viral illness femoral epiphysis G Swollen joints G Age range 2–12 yr (majority 4–8 yr) G Stiff joints G 20% bilateral G Sickle cell G Present with pain and limp G Delayed presentation G Restricted hip motion on examination

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EXAMINATION MANAGEMENT

General G If there are any features consistent with septic arthritis: Look for: G severe pain or local tenderness G Fever G range of movement <75% normal G Rash G temperature >37.5ºC G 9 G Pallor WBC >13 x 10 /L G G Bruising ESR >20 mm/first hr G CRP >10 mg/L G Impaired growth G effusion on USS Musculoskeletal OR G Check: X-ray abnormal or suggests orthopaedic problem (e.g. Perthe’s, G Gait SUFE) G Joint swelling G Refer to orthopaedics for diagnostic G Range of movement aspiration/washout – before starting antibiotics (see Osteomyelitis and G Leg lengths septic arthritis guideline) G Weakness DISCHARGE AND FOLLOW-UP G Spinal configuration/movement INITIAL INVESTIGATIONS G If blood tests and X-ray normal, irritable hip (reactive arthritis) likely G FBC and film G discharge with analgesia and reassurance G ESR G advise return if fever occurs or G CRP problem becomes worse G If febrile, blood cultures Review after 5 days G X-ray symptomatic joint (and ‘normal’ side) and, if origin of pain uncertain, G If worse, refer for orthopaedic opinion request X-rays of adjacent joints G If no worse, review after a further G where SUFE a possibility, request AP 5 days and frog views of hips G If still no better, arrange joint G if effusion suspected, confirm with orthopaedic/paediatric review, and ultrasound scan consider referral for paediatric G Other investigations (e.g. muscle rheumatology opinion enzymes, bone scan) may be useful – G If normal at 5 or 10 days, discharge dependent on clinical assessment

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Thorough history and examination

G FBC and film G ESR G CRP G Blood cultures if fever G Plain films of affected joints (bilateral, consider adjacent joints) G USS if indicated and available

Any abnormality?

G Abnormal X-ray G Severe pain G Local tenderness G Range of movement <75% of normal G Temp >37.5 °C G WCC > 13 x 10 9/L G ESR >20 mm/hr G CRP >10 mg/L G Effusion on USS

NO YES

Discharge home with To return if much worse Orthopaedic opinion analgesia or develops fever (withholding antibiotics)

WORSE worse Not worse Review at 5 days Review at 10 days

Normal Abnormal Normal DISCHARGE Joint orthopaedic/paediatric review Consider paediatric rheumatology referral

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G Odd or aggressive parental behaviour Always follow the Child Safeguarding Policy and G Any fracture in an infant without a Procedures in your Trust. satisfactory explanation It is everyone’s responsibility G Any bruise on a child aged <6 months old or pre-mobile G 4 recognised categories of abuse G (rarely seen in isolation) Patterns of bruising, injury or explanation not compatible with child’s G physical abuse (non-accidental injury) development G emotional abuse G Recurrent injuries G neglect G Evidence of other forms of abuse (e.g. G sexual abuse failure to thrive, neglect) NON-ACCIDENTAL INJURY G Previous evidence of injury or neglect (NAI) (check if child known to local authority children’s social care or is the subject of a child protection plan) Definition Referrals Physical abuse may involve hitting, shaking, throwing, poisoning, burning or G Discuss referrals by GP with scalding, drowning, suffocating or consultant before arranging medical otherwise causing physical harm to a assessment by on-call team child. Physical harm may also be caused G consultant will decide whether referral when a parent fabricates the symptoms should be made to the child protection of, or deliberately induces, illness in a agencies first child G Referrals from A&E or surgical wards Recognition and assessment should be taken by a doctor SpR grade or above Unless there is an appropriate G discuss with a consultant first to explanation, discuss injuries with determine who should carry out initial a senior doctor. All child protection examination and whether social care cases must be dealt with by an or police should be present SpR (minimum ST4) or above Always discuss referrals with the There may be direct information from the on-call consultant for child child or carer. The following protection duties presentations need to be considered Immediate action G Delay in seeking medical attention following an injury G If there is an urgent or life-threatening G History incompatible with injury seen situation, start necessary emergency G Numerous explanations suggested for treatment injury G Refer to your Trust on-call child G Changes in the history protection arrangements G Parents ‘shopping around’ for medical G if you suspect harm, refer to social help (e.g. from GP, A&E, different care, and police if they are not already hospitals) involved G History of domestic violence

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G if recommended by police, examine Examination child jointly with the Forensic Medical Examiner (FME) G There should be only one G Keep any social worker or police examination. Repeated examinations officer present informed are not in the child’s interest G Always consider potential risks to G Keep your immediate senior informed siblings or other children If this is a planned medical History assessment at the request of child protection agencies, carers with G Where a referral is made from social parental responsibility and the care and/or the police, the child may have given a full history, often a visual child (depending on age and recording understanding) must give their consent (usually written) for G ask for this information from social worker or police officer at beginning of examination to take place. If examination. It may not be necessary consent not forthcoming, social to repeat this information unless care may obtain a legal order further detail is required giving permission for the child to G If child first presents in a health setting, be examined. This does not apply particularly if story unclear, SpR (ST4 where a child needs urgent or above) or consultant should take assessment and treatment history and examine child before discussing with social care or police G Must include: How G state of child: cleanliness, appropriate clothing, etc G Record findings accurately during or G all body areas immediately after examination, using a G accurate description of all injuries dedicated child protection proforma (size, colour, position and pattern) on with body charts if available body charts G Complete and sign each page and G mouth (torn frenulum of lip and tongue include: especially) G full family history G fundi: look particularly for G persons present at interview haemorrhages. With small children, especially where head injuries are G source of your information (including suspected, this is usually the role of the child) the paediatric ophthalmologist G person giving consent G a note of any birth marks, etc G date and time of start and finish G a full paediatric systemic examination G plotting height and weight and head Take care when talking to the circumference on growth charts child not to ask leading questions G child’s emotional state, demeanour or make suggestions that could and degree of co-operation contaminate evidence in a G a comment on the developmental subsequent trial, document clearly state (or school progress) what is said in child’s own words G observations on relationships or behaviour between parents and child

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Investigations G Interpret all test results with age appropriate reference values A selection of the following tests will G If significant bruises, before further usually be necessary; seek advice from investigations, discuss with a consultant as to which are appropriate: paediatric haematologist: G If personal history of abnormal G von Willebrand Factor antigen and bleeding or concerning family history, activity discuss with a paediatric G Factor 8, 9 and 13 assay haematologist first as other tests may G be indicated blood group G G Bone biochemistry (including vitamin child aged <2 yr: platelet function D) if there are unexplained fractures assay G G Investigations into other suspected child aged <3 months, delayed cord abuse (e.g. failure to thrive) separation, slow healing, bleeding after surgery or after cord separation: G Skeletal survey in children aged <2 yr Factor 13 assay with unexplained injuries, ask radiology if repeat views after 11–14 days EMOTIONAL ABUSE required. Head CT scan in children aged <12 months and in older children Recognition and assessment if focal encephalopathic features, focal neurology or haemorrhagic retinopathy Definition G Further neuroimaging according to RCR/RCPCH guidelines G Habitual harassment of a child by G Document in notes if decision made disparagement, criticism, threat and not to proceed with imaging ridicule G Photographs (often a police G Present in most cases of physical and photographer is used) sexual abuse, and neglect Haematological investigations G presents difficulties in definition, recognition and management When a bleeding diathesis suspected or G long-term consequences upon social, needs to be ruled out, perform following: emotional and cognitive development can be more harmful than other forms G Initial baseline investigations of abuse G FBC and film (EDTA up to 1 mL) Presentation G APTT and PT (not INR) G thrombin time G Part of the differential diagnosis if a G fibrinogen levels child presents with the following non- G if thrombocytopenic, mean platelet specific behaviours: volume G unhappy G send 2 or 3 sodium citrate bottles, G disturbed filled to appropriate fill line level G poor concentration leading to learning Subsequent investigations difficulties/school failure G poor social interactions G Identify all requests as NAI G unable to play investigations

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G problems with attachment to parents G Development or caretakers G gross motor skills, fine motor skills, G over-friendly or craving affection from vision, hearing, language, behaviour, strangers play Assessment SEXUAL ABUSE

G Assessment is complex and requires Recognition and assessment a multidisciplinary approach G Social care take the investigative lead Definition NEGLECT G Forcing or enticing a child or young person to participate in sexual Neglect may not always be activities, whether or not the child is intentional (e.g. parental mental aware of what is happening health problems) G may involve physical contact, including penetrative (e.g. rape or Recognition and assessment buggery) or non-penetrative acts G Definition may include non-contact activities (e.g. involving children in looking at, or in production of, pornographic G Neglect is persistent failure to meet a material, watching sexual activities, or child’s physical and/or psychological encouraging them to behave in needs sexually inappropriate ways) G Lack of care of physical needs that can result in failure to thrive Presentation G important to eliminate organic causes G Information given by child G neglect of physical care most likely to G Symptoms resulting from local trauma come to Child Health attention along or infection (e.g. bruises, bleeding, with developmental delay discharge) Presentation G Symptoms resulting from emotional effects (e.g. behavioural changes, G Child’s appearance enuresis, encopresis, self-harming, G note condition of clothing, hair, skin eating disorders or psychosomatic G Growth symptoms) G height, weight, serial measurements to G Sexualized behaviour or sexual check growth rate knowledge inappropriate to age G head circumference G Under-age pregnancy G mid-upper arm circumference G Sexually transmitted infections G Non attendance (or repeat alterations) Referrals of appointments Physical examination G Referrals usually come from local authority children’s social care or the G Signs of medical problem not police appropriately treated G refer to your departmental child G Evidence of other forms of abuse protection rota

Issue 5 Issued: May 2013 Expires: May 2014 243 CHILD PROTECTION • 5/6 G Contact genito-urinary medicine If a child presents in a medical department setting and there are concerns about sexual abuse, call the G Post exposure prophylaxis should be on-call consultant for child started within 1 hr of assault if indicated (can be given up to 72 hr protection immediately. HIV PEP Depending on any urgent after assault). See guideline medical needs e.g. bleeding; Investigations child protection agencies may need to be contacted before G Mid-stream urine medical assessment G Forensic tests (FME to determine) IMMEDIATE ACTION – HISTORY G Photos/video recordings obtained with AND EXAMINATION a colposcope, stored in accordance with local policy Preparation Always follow the Child Safeguarding Policy and G Where sexual abuse suspected, whoever examines the child MUST Procedures in your Trust have training and experience in this SUBSEQUENT MANAGEMENT field G In exceptional cases, particularly G Majority of children seen will be where there is acute trauma and allowed home if it is safe and after bleeding that may require surgical discussion with social care management, it may be appropriate G for the examination to be carried out some children who have been abused under anaesthetic by a gynaecologist will be admitted while problems are after discussion with the FME investigated G Always keep parents and children Examination informed of concerns and what next actions will be G Purpose of medical examination is to: G Be open and honest with parents G detect traumatic or infective conditions where possible unless this could put that may require treatment child (or others) at risk of further harm G evaluate the nature of any abuse G secure forensic evidence Keeping children safe G reassure the child G If there is clear evidence of child G start process of recovery abuse and parents attempt to remove Initial management child there are two courses of action: G in an emergency, dial 999, the police G If penetration and/or passage of bodily can use police protection powers to fluids are suspected consider sexually keep child safe transmitted diseases and pregnancy G if there is time, a social worker can G pregnancy test obtain an Emergency Protection Order G if assault within 72 hr, offer post-coital from Court (Section 44, Children Act contraception (ideally <12 hr) – usually 1989) levonorgestrel 1.5 mg stat dose G Put the child’s safety first

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G Communicate with other staff involved (e.g. nursing staff) so that situation can be supervised G Consider the safety of siblings G usual for siblings to be examined at same time as index child DISCHARGE AND FOLLOW-UP

Only a consultant may allow child to go home

G Consultant should make decision regarding discharge, usually after discussion with the police and social care

Communication is vital

G Send written report to GP without delay, with a copy for social care and the police G If child referred from A&E, send copy of report to them for feedback G Ensure notes and dictation is available to secretary, marked ‘for urgent attention’ G Ensure report is signed in a timely manner G Complete ward discharge forms G Check with consultant if follow-up is required Child protection conference

G May be convened following a child protection investigation to consider: G whether child needs to be the subject of a child protection plan G Medical and nursing staff will be invited if child has been admitted G expected to contribute, usually in person, or via a written report G ensure reports are available for future reference

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G Self harm can take a number of forms, G Assess risk/need for ongoing including: psychological treatment or support. G cutting or burning The Connect Child and Adolescent Mental Health Services (CAMHS) and G self poisoning with medicines or First Steps Priority Referral Team tablets (PRT) provides service for patients G punching aged up to 18 yr. Obtain valid consent G self strangulation for a referral to CAMHS from parent/other adult with parental G pulling out hair or eyelashes responsibility or the young person if G scratching or picking at skin they are deemed to have capacity G inhaling or sniffing harmful substances (Gillick competence). Clearly G swallowing non-food substances document in medical records who obtained consent, who consent was G inserting objects into the body either taken from and when it was obtained through orifices or the skin i.e. date and time G head banging Documentation ASSESSMENT G Clearly document assessment in notes G Identifying behaviour, intended with any decisions made and reasons behaviour or self-harming thoughts REFERRALS G Who knows about the behaviour G How often this occurred Criteria for referral to PRT G If at risk from others G Stressors e.g. bullying, bereavement, G Deliberate self harm (e.g. overdose, relationships self strangulation, serious cuts) G Difficulties, abuse, sexuality issues G Deliberate harm from substance misuse (e.g. poisoning from excessive G General health alcohol and/or illicit drugs if intention G Use of drugs and alcohol was to self harm) G Education G Mental health symptoms: G Family and social issues G depression/low mood with active G Support network available suicidality G Child protection issues G psychotic symptoms. G MANAGEMENT low weight anorexia nervosa i.e. BMI <15 or accompanied by rapid weight loss G Patients who have self harmed, admit overnight G Referrals must be phoned through to PRT before 1000 hr to be seen that G See Poisoning guidelines day. Where there are exceptional G Advise carers to remove all medications circumstances (as determined by the or other means of self harm ward), referrals will be accepted up to G Manage child protection issues 1230 hr according to local policy and procedures. On-call consultant available 24 hr for child protection advice

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DISCHARGE AND FOLLOW-UP

G Discharge when medically fit and have been assessed by PRT G Discuss with CAMHS to ensure child has an agreed plan in place G If there are safety concerns, refer to children’s social care G Ensure health professionals i.e. GP and school nurse are aware of admission and management plan

Local contact: UHNS: PRT are based at the Ashlands and can be contacted via 0300 7900 235

Issue 5 Issued: May 2013 Expires: May 2014 247 INDEX • 1/3 A Codeine 23, 25 Abdominal pain 114 Coma score 204 Congenital heart disease 14, 64, 74, 168 Abuse 240 –245 Constipation 117 Adrenaline 9, 20, 22, 45, 188 Croup 44 Alcohol poisoning 78 Cyanotic congenital heart disease 61 ALTE 18 Cystic fibrosis Amiodarone 10, 71, 72, 73 Admission 46 Anaesthesia 28, 35 Distal intestinal obstruction 52 Analgesia 24 Exacerbation 48 Anaphylaxis 20 Microbiology 50 Antibiotics 150 Antipyretics 45, 88, 170, 183 APLS D Cardiorespiratory arrest 9 Desferrioxamine 80, 81 Recognition and assessment of Diabetes and fasting 92 the sick child 12 Diabetes new (non-ketotic) 103 Apparent life threatening events (ALTE) 18 Diabetic ketoacidosis 96 Arthritis 235 Diarrhoea and vomiting 122 Arthritis – septic 183, 235 Diclofenac 25 Arthritis – JIA 235, 237 Asthma – acute management 37 Dietitian 46, 104, 128, 225 Distal intestinal obstruction 52 Asystole 9, 10 DKA 96, 111 AVPU 15 Duct dependant congenital heart disease 62 B E Bacterial infection 53, 159, 168, 179, 190 ECG interpretation 65 Bites 152 EEG 29, 196 –199 Blood pressure 12, 123, 209, 212, 213 Blood and platelet transfusions 138 Effusion – pleural 56 Electromechanical dissociation (EMD) 9 Bradycardia 69, 176, 187 Bronchiolitis 41 Empyema 53, 114, 150 Encephalitis 150, 159, 176 C Encopresis 117, 243 Endocarditis prophylaxis 74 Calculi – renal 219 Epiglottitis 44 Cardiac arrest 16, 90, 224 Epilepsy 196 Cardiogenic shock 63, 64 Epilepticus, status 201 Cardiopulmonary resuscitation (CPR) 19, 63 Cellulitis – orbital 150, 182 F Cervical lymphadenopathy 153 Facial palsy 195 Chickenpox 50 –51, 168 Child protection 240 Faecal impaction 117, 119, 121 Failure to thrive 131 Coarctation 61, 63, 64, 68 Fallot’s tetralogy 61, 62, 64, 67, 68

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Fasting and diabetes 92 J Fasting pre-operatively 35 Jaundice 134 Febrile neutropenia 140 Juvenile idiopathic arthritis (JIA) 235, 237 Fever 157 Fever of unknown origin 161 K Fluid therapy 31 Kawasaki disease 170 Food poisoning 180 Ketone monitoring 111 G L General anaesthesia (GA) 35, 203 Limping child 237 Glasgow coma score (GCS) 204 Long line insertion 32 Glomerulonephritis 205 Lumbar puncture 123, 139, 158, 176 Lymphadenopathy 153 H Haematuria 143, 144, 149, 205, 219 M Haemolytic uraemic syndrome 207 Malaria 173 Haemophilia 147 Malignant hyperthermia 202 Heart failure and weak pulses 63 Meningitis 176 Henoch-Schönlein purpura 143 Meningococcal septicaemia 187, 188 Hepatitis 163 Morphine 26, 27, 62, 64, 189 Herpes simplex encephalitis 159, 160, 177 HIV and hepatitis B post-exposure prophylaxis (PEP) 164 N HIV testing 166 Nephrotic syndrome 215 Hydrocortisone 22, 38, 110, 112 Neuromuscular disorders 202 Hyperkalaemia 10, 65, 173, 223 Neutropenia, febrile 140 Hypernatraemic dehydration 31, 123, 125 Nocturnal hypoventilation 202 Hypertension 209 Non accidental injury 240 Hypoglycaemia 105 Notifiable infectious diseases and food poisoning 180 Hyponatraemia 31, 53, 106, 207 Nutritional first line advice 128 I O Ibuprofen 25, 144, 159 Oncology 138, 139, 167 Imaging – renal 228 –229 Orbital cellulitis 182 Immune thrombocytopenic purpura (ITP) 145 Osteomyelitis and septic arthritis 183 Immunodeficiency 168 Overdose 75, 246 Insulin 92, 97, 100, 103, 224 Intraosseous infusion 16 Ipratropium bromide 38, 40, 42 P Iron poisoning 80 Pain assessment 23 IV fluid therapy 31 Paracetamol 23, 24, 75, 82 Paracetamol poisoning 82

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Patient controlled analgesia (PCA) 23, 26 T Petechial/purpuric rashes 186 Tachycardia and bradycardia 69 Phenothiazine poisoning/side effects 87 Thrombocytopenic purpura (ITP) 145 PICC line 32, 33 Transfusion 138 Pituitary-adrenal axis impairment 112 Tricyclic poisoning 90 Platelet transfusion 138 Tuberculosis 191 Pleural effusion 56 Pneumonia 53 U Pneumothorax 59 Urinary tract infection (UTI) 230 Poisoning and drug overdose 75 Post-exposure prophylaxis (PEP) 164 Post GA monitoring V ex-premature infants 36 Varicella (VZV) 50, 168, 218 Prednisolone 38, 144, 193, 216 Ventricular fibrillation (VF) 9, 70 Pre-op fasting 35 Ventricular tachycardia (VT) 9, 70 Pulseless electrical activity (PEA) 9 Vitamin D deficiency 137 Purpura 143, 145 W R Wheezing (acute) 40 Rash 186 Renal calculi 219 Renal failure 223 Renal investigations 226 Respiratory syncytial virus (RSV) 44, 159 Resuscitation 9

S Safeguarding 240 Salbutamol 22, 38, 48, 224 Salicylate poisoning 88 Sedation 28 Seizure 196 Self harm 246 Septicaemia (including meningococcal) 187 Septic arthritis 183 Sexual abuse 243 Shock – IO access 16 Sinusitis 182 Status epilepticus 201 Steroid dependence 112 Stones - renal 219 –222 Supra ventricular tachycardia (SVT) 69, 72 Surgery and diabetes 92

Issue 5 Issued: May 2013 250 Expires: May 2014 Bedside Clinical Guidelines Partnership In association with

Bedside Clinical Guidelines Partnership Paediatric Guidelines In association with 2006

Paediatric Guidelines 2006 Paediatric Guidelines 2013–14 Paediatric These guidelinesISBN: ar 978-0-9567736-1-6e advisory, not mandatory. Every effort has been made to ensure accuracy. The authors cannot accept any responsibility for These guidelinesadverse outcomes.are advisory, not mandatory. Guidelines Every effort has been made to ensure accuracy. SuggestionsThe authors for cannotimprovement accept andany responsibilityadditional for Theseguidelines guidelines would ar adverseebe advisory most outcomes. welcome, not mandatory by the . Every efPartnersfort has in been Paediatrics made toCoor ensurdinatore accuracy, . Suggestions for improvement and additional guidelines The authors cannot accept any responsibility for Tel. 01782would 552002 be most or welcomeEmail nicky by.smith [email protected] in Paediatrics, please contact viaadverse http://www.networks.nhs.uk/nhs-networks/ outcomes. 2013-14 partners-in-paediatrics/guidelines Suggestions for improvement and additional Paediatric Guidelines 2013–14 guidelines would be most welcome by the Partners in Paediatrics Coordinator, Tel. 01782 552002 or Email [email protected] ISSUE 5

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