Paediatrics & Child Health

Key Facts

Developmental delay is one of the most common reasons for referral to community paediatrics. A systematic approach to investigation, combined with early intervention, improves outcomes for children and families.

Epidemiology

Global developmental delay affects approximately 1-3% of children under 5. Learning disability (intellectual disability) affects approximately 2% of the population. Specific speech and language delay is the most common isolated delay (~5-8%). Autism spectrum disorder affects ~1% of the population.

Aetiology

Prenatal (~50%):

• Genetic: Down syndrome (trisomy 21), fragile X, other chromosomal abnormalities, single gene disorders
• Congenital infections: CMV, toxoplasmosis, rubella, Zika
• Teratogens: Alcohol (FASD), antiepileptics (valproate), recreational drugs
• Structural brain malformations

Perinatal (~15%):

• Birth asphyxia (HIE)
• Prematurity and its complications (IVH, PVL)
• Neonatal sepsis/meningitis

Postnatal (~15%):

• Meningitis/encephalitis, head injury (NAI), metabolic disease
• Environmental deprivation/neglect

Unknown (~20%):

• Despite investigation, cause remains unidentified in ~20-40%

Pathophysiology

Developmental delay reflects impaired or delayed CNS maturation. Genetic causes produce abnormal brain development (neurogenesis, migration, synaptogenesis). Acquired causes (HIE, infection) produce direct neuronal injury. Environmental causes (deprivation) affect synaptogenesis and neural pruning during critical periods.

Patterns of Delay

• Global delay: All/most domains affected - consider genetic, metabolic, structural brain abnormality
• Motor delay: Cerebral palsy, muscular dystrophy, spinal muscular atrophy
• Speech/language delay: Hearing loss, autism, specific language disorder, environmental deprivation
• Social/communication delay: Autism spectrum disorder
• Regression: Loss of previously acquired skills - neurodegenerative disease, Rett syndrome, epileptic encephalopathy

Key History Points

• Pregnancy/birth history (infections, drugs, birth asphyxia, prematurity)
• Family history (consanguinity, learning disability, genetic conditions)
• Developmental milestones in all domains
• Feeding, growth, behaviour
• Parental concerns (always take seriously)

Red Flags

• Developmental regression - urgent investigation
• Dysmorphic features - genetic assessment
• Micro/macrocephaly - measure and plot head circumference
• Seizures with developmental delay - epileptic encephalopathy
• Consanguinity - increased risk of autosomal recessive conditions
• Faltering growth with delay - consider metabolic disease, neglect, FASD

First-Line (All GDD)

• Chromosomal microarray (CMA): First-line genetic test - detects copy number variants; yield ~15-20%
• MRI brain: Structural abnormalities - yield ~30%
• Thyroid function tests: Hypothyroidism (treatable cause)
• Hearing assessment: Formal audiometry
• Vision assessment: Ophthalmology review
• FBC, U&Es, LFTs, ferritin: Baseline

Second-Line (If First-Line Normal)

• Fragile X testing: Especially in boys with GDD + family history
• Whole exome/genome sequencing: Increasingly first-line in NHS; yield ~30-40% when CMA negative
• Metabolic screen: Amino acids, organic acids, acylcarnitine profile, urine mucopolysaccharides, lactate
• CK: If motor delay (muscular dystrophy screening)
• Congenital infection screen: If microcephaly, intracranial calcification, hearing loss

If Regression

• Urgent MRI + MRS: White matter disease, structural change
• EEG: Epileptic encephalopathy
• Metabolic workup: Lactate, ammonia, very long chain fatty acids (VLCFA), white cell enzymes
• Genetic panel: Targeted NGS panels for neurodegeneration

Non-pharmacological

• Early intervention: Begin therapy regardless of whether diagnosis is established
• Physiotherapy: For motor delay - promotes mobility, prevents contractures
• Speech and language therapy: For communication delay
• Occupational therapy: Fine motor skills, ADL support, sensory integration
• Portage: Home-based early learning programme
• Educational support: EHCP (Education, Health and Care Plan) for children with significant needs
• Family support: Parent groups, respite, social care

Pharmacological

• Treat specific underlying conditions (e.g., hypothyroidism, epilepsy)
• Melatonin for sleep disorders in children with developmental delay (common co-morbidity)
• Behavioural management strategies first for challenging behaviour

Referral Criteria

• Not meeting limit-age milestones - community paediatrician
• Developmental regression - urgent paediatric neurology
• Suspected autism - NICE CG128 pathway
• Suspected hearing loss - audiology
• Suspected genetic condition - clinical genetics
• All GDD - multidisciplinary child development centre assessment

• Down syndrome: Mild-moderate learning disability in most; life expectancy ~60 years with modern healthcare
• Cerebral palsy: Varies from minimal disability to severe; GMFCS classification guides prognosis
• FASD: Lifelong neurodevelopmental difficulties; no cure but support improves outcomes
• Autism: Lifelong condition; early intervention improves communication and social skills
• Isolated speech delay: ~50% catch up by age 3; persistent delay may indicate specific language disorder or autism
• Metabolic conditions: Some treatable if identified early (e.g., PKU, hypothyroidism); neurodegenerative conditions have poor prognosis

Developmental Delay Investigation Algorithm

Key Genetic Causes of GDD

Key Facts

The neonatal examination is a key screening opportunity to identify congenital abnormalities and conditions that benefit from early treatment. The NIPE programme is a national NHS screening programme.

Epidemiology

Approximately 650,000 babies are born in England each year. Congenital heart disease affects ~8 per 1,000 live births. DDH affects ~1-3 per 1,000. Undescended testes affect ~3-5% of term boys. Congenital cataracts affect ~3 per 10,000.

Aetiology

The NIPE programme screens for conditions where early detection improves outcome:

• Eyes: Congenital cataracts, retinoblastoma
• Heart: Congenital heart disease (structural)
• Hips: Developmental dysplasia of the hip
• Testes: Undescended testes (cryptorchidism)

Pathophysiology

DDH: Spectrum from acetabular dysplasia to complete dislocation. Risk factors include breech presentation, family history, female sex, first-born, oligohydramnios. Early detection allows Pavlik harness treatment (splintage) which is successful in >90% if started before 6 weeks.

Congenital heart disease: Structural defects in cardiac development. Critical CHD (duct-dependent) may present with cyanosis or collapse as the ductus arteriosus closes in the first days of life.

Systematic Neonatal Examination

• General: Colour, tone, activity, cry, dysmorphic features
• Head: Fontanelles (anterior - closes 12-18 months; posterior - closes 6-8 weeks), moulding, cephalhaematoma, sutures
• Eyes: Red reflex (bilateral), pupil size, discharge
• Mouth: Palate (feel for cleft), tongue-tie, natal teeth
• Cardiovascular: Heart rate, rhythm, murmurs (position, grade), femoral pulses (absent/weak → coarctation), pre- and post-ductal SpO2
• Respiratory: Rate, symmetry, grunting, recession, breath sounds
• Abdomen: Organomegaly, masses, umbilicus
• Genitalia: Testes (palpable, position), hypospadias, ambiguous genitalia
• Hips: Barlow (dislocatable?) and Ortolani (reducible?) tests
• Spine: Midline defects (dimple, tuft of hair, swelling → spinal dysraphism)
• Skin: Birthmarks, rashes (erythema toxicum, milia, Mongolian blue spots)
• Neurology: Tone, posture, primitive reflexes (Moro, grasp, rooting)

Red Flags

• Absent red reflex - retinoblastoma or cataract; urgent ophthalmology
• Central cyanosis - congenital heart disease until proven otherwise
• Absent femoral pulses - coarctation of the aorta
• Ambiguous genitalia - endocrine emergency; do NOT assign sex
• Midline spinal defect - USS spine before 6 weeks (or MRI after)
• Bilious (green) vomiting - malrotation until proven otherwise

Bedside

• Pulse oximetry screening: Pre-ductal (right hand) and post-ductal (either foot) SpO2; difference >3% or SpO2 <95% → echocardiography
• Weight: Birth weight, daily weights if concerns
• Blood glucose: If risk factors (SGA, LGA, maternal diabetes)

Bloods

• Newborn bloodspot screening (day 5): PKU, congenital hypothyroidism, sickle cell, CF, MCADD, and 5 other IEMs
• SBR (serum bilirubin): If jaundice <24h or clinically significant
• Blood group and DAT (Coombs): If early jaundice

Imaging

• USS hips: Indicated if positive Barlow/Ortolani, risk factors (breech, family history, foot deformity)
• Echocardiography: If murmur with features of concern, absent femoral pulses, or abnormal pulse oximetry
• USS spine: Before 6 weeks if midline defect (sacral dimple with concerning features)

Special Tests

• Newborn hearing screening (NHSP): Automated auditory brainstem response (AABR) or otoacoustic emissions (OAE) - within first few weeks

Non-pharmacological

• NIPE examination: Within 72 hours and repeated at 6-8 weeks
• Breast/bottle feeding support: Establish feeding; weight check at day 5
• Skin-to-skin contact: Promotes bonding, thermoregulation, breastfeeding
• Safe sleeping advice: Back to sleep, feet to foot of cot, no co-sleeping with risk factors (SIDS prevention)

Pharmacological

• Vitamin K: IM 1mg (single dose) or oral 2mg at birth, 1 week, 4 weeks - prevents VKDB
• Hepatitis B vaccine: Within 24 hours if mother HBsAg positive + HBIG
• BCG vaccine: For babies at increased risk of TB

Referral Criteria

• Absent red reflex - urgent ophthalmology (within 2 weeks)
• Suspected CHD (murmur + cyanosis/absent femorals/abnormal SpO2) - urgent echo
• Positive Barlow/Ortolani - USS hips at 2 weeks
• Undescended testis - review at 6-8 weeks; surgery referral by 6 months
• Ambiguous genitalia - endocrine/DSD team urgently
• Suspected genetic syndrome - clinical genetics

• DDH: >90% success with Pavlik harness if detected early (<6 weeks); late detection requires surgery and has worse outcomes
• CHD: Depends on lesion; duct-dependent lesions need prostaglandin to maintain ductal patency; surgical outcomes greatly improved
• Undescended testis: Spontaneous descent in ~70% by 3 months; orchidopexy before 12 months reduces infertility and malignancy risk
• Congenital cataracts: Good visual outcome if operated within first 6-8 weeks of life
• Newborn screening: Early treatment of PKU, hypothyroidism, and sickle cell disease dramatically improves long-term outcomes

NIPE Screening - Four Key Areas

Newborn Bloodspot Screening (9 Conditions)

Key Facts

Neonatal jaundice is the most common condition requiring medical attention in the first 2 weeks of life. While usually benign, failure to identify and treat significant hyperbilirubinaemia can result in kernicterus - a devastating but preventable condition.

Epidemiology

Visible jaundice affects approximately 60% of term and 80% of preterm neonates. Approximately 10% of breastfed neonates are still jaundiced at 1 month. Significant hyperbilirubinaemia requiring treatment occurs in approximately 5-10%. Kernicterus has an incidence of approximately 1 per 100,000 live births in the UK.

Aetiology

Unconjugated (most common):

• Physiological (immature hepatic conjugation + high RBC turnover)
• Haemolytic: ABO incompatibility, Rh disease, G6PD deficiency, spherocytosis

• Breast milk jaundice (substance in breast milk inhibits conjugation - benign, prolonged)
• Polycythaemia, bruising (cephalhaematoma), sepsis

Conjugated (always pathological):

• Biliary atresia (MUST exclude - pale stools, dark urine)
• Neonatal hepatitis, choledochal cyst, alpha-1 antitrypsin deficiency
• Infections: UTI, sepsis, TORCH

Pathophysiology

Unconjugated bilirubin is produced from haem breakdown in the reticuloendothelial system. Neonates produce 2-3× more bilirubin per kg than adults (higher RBC mass, shorter RBC lifespan). Hepatic conjugation (UDP-glucuronosyltransferase) is immature in the first 2 weeks. Unconjugated bilirubin is lipid-soluble and can cross the blood-brain barrier at very high levels, depositing in the basal ganglia and brainstem nuclei (kernicterus).

Clinical Assessment

• Visual assessment is unreliable - always measure bilirubin if concerned
• Assess feeding, weight, hydration, urine/stool output

Timing

• <24 hours: PATHOLOGICAL - haemolysis (ABO, Rh), sepsis, G6PD
• Day 2-5: Physiological peak; may also be pathological if high
• >14 days (term) / >21 days (preterm): Prolonged - investigate

Red Flags

• Jaundice <24 hours - urgent investigation
• Pale/chalky stools + dark urine - biliary atresia (surgical emergency if confirmed)
• Lethargy, poor feeding, high-pitched cry - bilirubin encephalopathy (early kernicterus)
• Opisthotonos, seizures, apnoeas - acute bilirubin encephalopathy (emergency)
• SBR rising >8.5 µmol/L/hr - rapid rise suggesting haemolysis

Bedside

• Transcutaneous bilirubinometry (TcB): Screening tool - if above threshold, confirm with SBR
• SBR (serum bilirubin): Plot on NICE CG98 gestation-specific treatment threshold chart
• Weight: Compare with birth weight - dehydration worsens jaundice
• Stool and urine colour: Pale stools + dark urine → biliary atresia

Bloods

• SBR (total and split): Conjugated bilirubin >25 µmol/L is always pathological
• Blood group and DAT (Coombs test): Mother and baby - if early or severe jaundice
• FBC, reticulocyte count, blood film: Haemolysis (spherocytes, reticulocytosis)
• G6PD assay: If at-risk ethnicity or unexplained jaundice

If Prolonged Jaundice

• Split bilirubin: Conjugated vs unconjugated
• TFTs: Congenital hypothyroidism
• Urine MC&S: UTI screen
• Liver USS: Biliary atresia (absent/abnormal gallbladder), choledochal cyst

Special Tests

• HIDA scan: If biliary atresia suspected (no isotope excretion into gut)
• Liver biopsy: Confirms biliary atresia (portal tract fibrosis, bile duct proliferation)
• Kasai portoenterostomy: Definitive investigation/treatment for biliary atresia (ideally <60 days of life)

Phototherapy

• Indication: SBR above treatment threshold on NICE CG98 gestation-specific chart
• Mechanism: Blue-green light (460-490nm) converts unconjugated bilirubin to water-soluble photoisomers
• Delivery: Overhead phototherapy unit; maximise skin exposure; eye protection
• Monitoring: Repeat SBR at 4-6h; continue until SBR is ≥50 µmol/L below treatment threshold
• Rebound SBR: Check 12-18h after stopping phototherapy

Exchange Transfusion

• Indication: SBR at or approaching exchange transfusion threshold; signs of acute bilirubin encephalopathy
• Procedure: Double-volume exchange (2 × 80mL/kg for term); removes bilirubin and antibodies
• Risks: Electrolyte disturbance, thrombocytopenia, NEC, infection, cardiac arrest

Pharmacological

• IV immunoglobulin (IVIG): For immune haemolytic jaundice (Rh disease, ABO) if bilirubin rising despite phototherapy

Non-pharmacological

• Support breastfeeding: Ensure adequate feeding frequency (8-12 feeds/day); supplement with expressed breast milk or formula if inadequate intake
• Hydration: Ensure adequate fluid intake; IV fluids if not feeding adequately

Referral Criteria

• Jaundice <24h - urgent paediatric assessment
• Conjugated hyperbilirubinaemia - urgent referral to paediatric liver centre
• Biliary atresia - Kasai procedure ideally before 60 days of life
• Kernicterus signs - emergency exchange transfusion

• Physiological jaundice: Completely benign; resolves without treatment
• Phototherapy: Effective in >95% of cases; exchange transfusion rarely needed
• Kernicterus: Devastating - chronic bilirubin encephalopathy causes choreoathetoid cerebral palsy, sensorineural hearing loss, gaze palsy, dental enamel dysplasia
• Biliary atresia: Kasai portoenterostomy successful in ~60% if done before 60 days; ~50% eventually require liver transplant
• ABO/Rh haemolytic disease: Excellent prognosis with appropriate monitoring and treatment

NICE CG98 - Key Recommendations

Prolonged Jaundice Investigation

Key Facts

Neonatal sepsis remains a significant cause of neonatal mortality and morbidity. The non-specific nature of neonatal sepsis signs makes a high index of suspicion essential. NICE CG149 provides a structured approach based on risk factors and clinical indicators.

Epidemiology

EONS incidence: ~0.9 per 1,000 live births. GBS-EONS: ~0.5 per 1,000. LONS is more common in preterm infants, especially those with central venous lines. Neonatal sepsis mortality: ~5-10% for term infants, up to ~30% for very preterm.

Aetiology

Early-onset (<72h):

• Group B Streptococcus (GBS) - most common overall
• Escherichia coli - most common Gram-negative; leading cause in preterm EONS
• Listeria monocytogenes - uncommon but important (maternal food-borne)

Late-onset (72h-28 days):

• Coagulase-negative Staphylococci (CoNS) - most common in NICU (line-related)
• Staphylococcus aureus, E. coli, Klebsiella, Candida (very preterm)

Pathophysiology

Neonatal immune system is immature: reduced complement activity, low IgM and IgA (only maternal IgG crosses placenta), impaired neutrophil chemotaxis and opsonisation, and reduced T-cell function. This renders neonates highly susceptible to bacterial infection with rapid progression to septic shock and meningitis.

Clinical Signs (Non-Specific)

• Temperature: Hypothermia (<36°C) or fever (>38°C); hypothermia more common in preterm
• Respiratory: Tachypnoea, grunting, apnoeas, increased O2 requirement
• Cardiovascular: Tachycardia, poor perfusion, hypotension (late sign)
• Neurological: Lethargy, irritability, poor feeding, seizures, bulging fontanelle
• GI: Feeding intolerance, abdominal distension, vomiting
• Skin: Mottling, pallor, petechiae, prolonged CRT

NICE CG149 Risk Factors for EONS

Red flags (treat immediately):

• Clinical signs of neonatal sepsis
• Suspected or confirmed maternal sepsis treated with IV antibiotics
• Maternal GBS colonisation + ONE of: preterm labour, PROM >18h, maternal fever >38°C

Non-red flag risk factors (observe ± treat):

• PROM >18h, maternal GBS colonisation (without additional risk factors), maternal pyrexia, gestational age <37 weeks

Red Flags

• Apnoeas - may be only sign of sepsis in preterm infants
• Seizures - suggests meningitis
• Rapidly deteriorating - fulminant sepsis/meningitis
• Purpura/petechiae - DIC, meningococcal (rare in neonates)

Bedside

• Observations: Temperature, HR, RR, SpO2, BP, capillary refill
• Blood glucose: Hypoglycaemia common in sepsis

Bloods

• Blood culture: GOLD STANDARD - before antibiotics if possible (but do NOT delay antibiotics)
• FBC: WCC may be high, low, or normal; left shift (raised immature:total neutrophil ratio); thrombocytopenia
• CRP: May be normal initially; repeat at 18-24h (serial CRP is useful)
• Procalcitonin: Rising - more specific than CRP for bacterial infection
• Blood gas: Metabolic acidosis in severe sepsis

Imaging

• CXR: If respiratory symptoms - pneumonia, RDS
• AXR: If abdominal signs - NEC

Special Tests

• LP (CSF): For all confirmed sepsis or strong clinical suspicion of meningitis - cell count, protein, glucose, culture, PCR
• Urine MC&S: More relevant in LONS (clean catch or SPA)
• Surface swabs: Ear, umbilicus - may guide therapy but not diagnostic of invasive infection

Pharmacological

EONS (<72h):

• IV benzylpenicillin 25mg/kg 12-hourly (term; 8-hourly if meningitis) + gentamicin 5mg/kg OD
• Start within 1 hour of decision to treat
• Duration: Stop at 36-48h if blood culture negative AND clinically well AND CRP not rising
• If culture positive or meningitis confirmed: Continue for 7-14 days (meningitis: 14-21 days depending on organism)

LONS (>72h):

• Flucloxacillin + gentamicin (or per local protocol if NICU-acquired - may need broader cover)
• Candida: Fluconazole or amphotericin B for very preterm infants

Meningitis:

• Extend to meningitis doses: Benzylpenicillin or cefotaxime 50mg/kg + gentamicin
• Duration: GBS meningitis 14 days; Gram-negative meningitis 21 days

Non-pharmacological

• IV access: Ensure reliable venous access
• Fluid resuscitation: 10-20mL/kg 0.9% NaCl bolus if signs of shock; may need inotropes
• Respiratory support: CPAP, IPPV as needed
• Monitoring: Continuous cardiorespiratory monitoring, fluid balance, blood glucose
• Nutritional support: May need parenteral nutrition if feeds withheld

Referral Criteria

• All unwell neonates - neonatal team assessment
• Meningitis - neonatal unit; may need neurosurgical input if complications
• Not responding to first-line antibiotics - microbiology/infectious disease input
• Preterm LONS - neonatal unit

• EONS (GBS) mortality: ~5-10% in term infants; higher in preterm
• Gram-negative EONS mortality: ~15-20%
• GBS meningitis: Mortality ~10%; long-term neurodevelopmental sequelae in ~50% of survivors
• LONS (CoNS): Low mortality (<5%) but associated with prolonged NICU stay
• CRP as guide: Serial CRP helps guide antibiotic duration; falling CRP supports stopping at 36-48h if culture negative

NICE CG149 - Decision Framework

Antibiotic Doses (Neonatal)

Key Facts

Neonatal resuscitation is fundamentally different from adult resuscitation because the primary pathology is respiratory rather than cardiac. Establishing effective ventilation (lung inflation) is the single most important intervention and resolves the majority of cases.

Epidemiology

Approximately 10% of newborns require some form of resuscitation at birth (stimulation, airway positioning, inflation breaths). Approximately 3-6% require ventilation breaths. Approximately 1% require intubation, chest compressions, or drugs. Neonatal mortality in the UK is approximately 2.7 per 1,000 live births.

Aetiology

Causes of need for resuscitation at birth:

• Respiratory: Failure to establish breathing, meconium aspiration, diaphragmatic hernia, pneumothorax
• Cardiovascular: Congenital heart disease (duct-dependent), hypovolaemia
• Neurological: Birth asphyxia, maternal sedation (opioids), neuromuscular disease
• Prematurity: Surfactant deficiency, immature respiratory drive
• Other: Sepsis, congenital anomalies

Pathophysiology

Assessment at Birth

• Colour: Pink/blue
• Tone: Good/floppy
• Breathing: Regular/irregular/absent
• Heart rate: >100 (good), 60-100 (concerning), <60 (critical)

Apgar Score (1 and 5 minutes)

Red Flags Requiring Resuscitation

• Not breathing or gasping at 30 seconds despite drying and stimulation
• Heart rate <100 after inflation breaths
• Heart rate <60 despite effective ventilation - start compressions
• Meconium-stained liquor with depressed baby - consider airway suctioning
• Hydrops fetalis - may need immediate drainage and ventilation

Bedside

• Heart rate: Best assessed by auscultation or pulse oximetry (right hand - pre-ductal)
• SpO2: Pre-ductal (right hand); normal SpO2 at 2 min = 60%, at 5 min = 80%, at 10 min = 90%
• Colour and tone: Clinical assessment

Bloods

• Cord blood gas: Arterial pH, base excess - assess degree of birth asphyxia
• FBC, blood glucose: After initial stabilisation
• Blood gas (capillary/arterial): Guide ongoing ventilation

Imaging

• CXR: If intubated (confirm ETT position), respiratory distress, suspected pneumothorax or diaphragmatic hernia

Special Tests

• aEEG: If suspected HIE - guide therapeutic hypothermia decision

NLS Algorithm (Resuscitation Council UK)

1. Birth: Dry, wrap, hat, start clock
2. Assess: Tone, breathing, heart rate at 60 seconds
3. If not breathing/gasping: Open airway (neutral position); 5 inflation breaths (30 cmH2O, 2-3 sec each)
4. Reassess: If HR not increasing - check chest movement; reposition; consider 2-person jaw thrust, oropharyngeal airway
5. If HR <60 after effective ventilation: Chest compressions 3:1 ratio at 120 events/min (90 compressions, 30 ventilations)
6. If HR still <60: IV/IO adrenaline 10mcg/kg (0.1mL/kg of 1:10,000); consider IV fluids 10mL/kg
7. Consider: Intubation, UVC access, volume replacement, treatable causes

Key Equipment and Sizes

• ETT: Term = 3.5mm; 34-38 weeks = 3.0mm; <34 weeks = 2.5mm
• Laryngoscope: Straight blade size 1 (term), size 0 (preterm)
• Suction: Max pressure 100 mmHg (150 for meconium)

Pharmacological

• Adrenaline: 10mcg/kg (0.1mL/kg of 1:10,000) via UVC or IO; 50-100mcg/kg via ETT if no IV
• Volume: 10mL/kg 0.9% NaCl or O-negative blood if hypovolaemia suspected
• Glucose 10%: 2.5mL/kg if hypoglycaemic

Post-Resuscitation Care

• Therapeutic hypothermia: For moderate-severe HIE - cool to 33.5°C for 72h; start within 6h of birth
• Monitoring: Continuous SpO2, HR; blood gas, glucose
• NICU admission: All babies requiring prolonged resuscitation
• Debrief: Family communication; documentation; team debrief

• Response to inflation breaths alone: ~90% of babies requiring resuscitation respond to effective ventilation
• Need for chest compressions: ~0.03% of deliveries; prognosis depends on cause and duration
• Therapeutic hypothermia for HIE: NNT 7 to prevent death or disability (TOBY trial)
• Severe HIE (Sarnat 3): ~30% mortality; ~70% of survivors have disability
• Moderate HIE (Sarnat 2) with cooling: ~50% have normal outcome; significantly improved with therapeutic hypothermia

NLS Algorithm Summary

Expected SpO2 After Birth (Pre-Ductal)

Key Facts

Prematurity is the single most important cause of neonatal mortality and long-term morbidity. Advances in neonatal intensive care have dramatically improved survival, but the burden of prematurity-related complications remains significant.

Epidemiology

Approximately 8% of births in the UK are preterm (~60,000/year). Prematurity accounts for approximately 75% of neonatal deaths and 50% of long-term neurological disability in childhood. Survival rates: 23 weeks ~30-40%; 24 weeks ~50-60%; 28 weeks ~90%; 32 weeks ~98%.

Aetiology

• Spontaneous preterm labour (~50%): Idiopathic, cervical incompetence, infection, polyhydramnios, multiple pregnancy
• PPROM (Preterm Premature Rupture of Membranes) (~30%): Infection, inflammation
• Medically indicated (~20%): Pre-eclampsia, IUGR, placenta praevia, maternal medical conditions
• Risk factors: Previous preterm birth (strongest risk factor), multiple pregnancy, extremes of maternal age, low BMI, smoking, cervical surgery

Pathophysiology

Preterm infants have immature organ systems:

• Lungs: Surfactant deficiency (produced from ~24 weeks, mature by ~34-36 weeks) → RDS

• Gut: Immature mucosal barrier → NEC
• Eyes: Incomplete retinal vascularisation → ROP
• Immune system: Immature → high infection susceptibility
• Temperature regulation: High BSA:weight ratio, minimal subcutaneous fat → hypothermia

Immediate Complications

• RDS: Tachypnoea, grunting, recession, cyanosis within hours of birth
• Hypothermia: Rapid heat loss
• Hypoglycaemia: Poor glycogen stores
• IVH: Usually within first 72 hours; may be asymptomatic or cause sudden deterioration

Short-Term Complications

• NEC: Abdominal distension, bloody stools, pneumatosis (usually after day 7)
• Sepsis: Early and late-onset; non-specific signs
• Patent ductus arteriosus (PDA): Failure to close; heart murmur, wide pulse pressure
• Apnoea of prematurity: Cessation of breathing >20 sec; due to immature respiratory control

Long-Term Complications

• Chronic lung disease (BPD): O2 requirement at 36 weeks corrected; affects ~30% of <28 weeks
• ROP: Screening from 30-31 weeks PMA for babies <32 weeks or <1501g
• Neurodevelopmental delay: Cerebral palsy, learning difficulties, behavioural problems
• Growth: May have poor postnatal growth; later risk of metabolic syndrome

Red Flags

• Sudden deterioration in preterm infant - consider NEC, sepsis, IVH, pneumothorax
• Recurrent apnoeas - may be sign of sepsis, NEC, or worsening lung disease
• Abdominal distension with blood in stool - NEC until proven otherwise
• Desaturations with bradycardia - may need escalation of respiratory support

Bedside

• Continuous monitoring: SpO2, HR, RR, temperature
• Blood glucose: Every 3-6h initially
• Blood gas: Guide respiratory support

Bloods

• FBC, CRP, blood culture: If sepsis suspected
• U&Es, glucose: Fluid and electrolyte management
• Bilirubin: Preterm infants have lower treatment thresholds

Imaging

• CXR: RDS (ground glass, air bronchograms), pneumothorax, PDA
• AXR: NEC (pneumatosis intestinalis, portal venous gas, perforation)
• Cranial USS: Day 1-3 (baseline), day 7, day 28, and 36 weeks CGA - screen for IVH and PVL

Special Tests

• ROP screening: By ophthalmologist from 30-31 weeks PMA for all <32 weeks or <1501g
• Hearing screen: Before discharge
• MRI brain: At term-equivalent age if abnormal USS or high-risk

Antenatal

• Corticosteroids: Betamethasone 12mg IM ×2 doses 24h apart (or dexamethasone) at 24-34 weeks - reduces RDS (NNT 12), IVH, NEC, death
• Magnesium sulphate: If <30 weeks expected - neuroprotection (reduces CP risk ~30%)
• Tocolytics: Nifedipine or atosiban - to delay delivery for steroid administration (48h)
• In-utero transfer: To centre with appropriate level NICU

Delivery Room

• Thermoregulation: Plastic wrap for <28 weeks (do not dry); radiant warmer; hat
• Delayed cord clamping: ≥60 seconds if baby does not require immediate resuscitation
• Respiratory support: CPAP from delivery room for very preterm; intubation and surfactant if needed
• Surfactant: Poractant alfa (Curosurf) 200mg/kg via ETT or LISA (Less Invasive Surfactant Administration)

NICU Care

• Respiratory: CPAP, IPPV, HFOV as needed; caffeine citrate for apnoea of prematurity (20mg/kg loading then 5-10mg/kg OD)
• Nutrition: Breast milk (ideally maternal EBM) via NG tube; TPN if enteral feeds not tolerated
• Infection prevention: Strict hand hygiene, minimal handling, antibiotic stewardship
• Kangaroo care: Skin-to-skin contact - improves thermoregulation, bonding, breastfeeding, and neurodevelopment

Referral Criteria

• All preterm births <34 weeks - neonatal team at delivery
• Extremely preterm (<28 weeks) - delivery at Level 3 NICU
• Surgical NEC - neonatal surgical team
• ROP requiring treatment - ophthalmology (laser/anti-VEGF)

• Survival by gestation: 23wk ~30-40%; 24wk ~50-60%; 26wk ~80%; 28wk ~90%; 32wk ~98%
• Disability-free survival: Improves ~10-15% per additional gestational week
• BPD: Affects ~30% of <28 weeks; most wean off oxygen by 1-2 years
• Neurodevelopmental outcome (<28 weeks): ~20% have significant disability (CP, severe learning difficulty); ~40% have minor difficulties
• ROP: Severe ROP (stage 3+) in ~10% of <28 weeks; treatment prevents blindness in most
• EPICure studies: Landmark UK studies tracking outcomes of extremely preterm infants

Complications of Prematurity by System

IVH Grading (Papile Classification)

Key Facts

RDS remains the most significant respiratory condition of the preterm neonate. The combination of antenatal steroids, postnatal CPAP, and surfactant replacement has transformed survival and outcomes over the past 40 years.

Epidemiology

RDS affects approximately 60-80% of infants born at <28 weeks, 15-30% at 32-36 weeks, and is rare at term. It is the most common cause of neonatal death from prematurity. Before surfactant therapy (introduced early 1990s), RDS mortality was approximately 50% in very preterm infants.

Aetiology

Primary surfactant deficiency:

• Prematurity (most common cause) - type II pneumocytes immature
• Risk increased by: Male sex, Caucasian ethnicity, maternal diabetes, caesarean section (reduced catecholamine surge), perinatal asphyxia

Secondary surfactant dysfunction:

• Meconium aspiration syndrome, pneumonia, pulmonary haemorrhage

Pathophysiology

Surfactant is a mixture of phospholipids (predominantly DPPC - dipalmitoylphosphatidylcholine) and surfactant proteins (SP-A, B, C, D) produced by type II alveolar pneumocytes. It reduces alveolar surface tension, preventing atelectasis at end-expiration and maintaining functional residual capacity (FRC). In surfactant deficiency, alveoli collapse with each expiration, requiring increased effort to re-expand them. This causes progressive atelectasis, intrapulmonary shunting, hypoxia, and acidosis. Protein-rich exudate forms hyaline membranes (hence the historical name 'hyaline membrane disease').

Clinical Features (Within First 4-6 Hours)

• Tachypnoea: RR >60/min
• Grunting: Expiratory - maintains positive end-expiratory pressure
• Nasal flaring: Accessory muscle use
• Intercostal/subcostal recession: Compliance mismatch (compliant chest wall, stiff lungs)
• Cyanosis: Worsening hypoxia
• Apnoeas: Especially in extremely preterm infants

Natural History

• Onset within 4-6 hours of birth
• Peaks at 48-72 hours
• Begins to improve after 72 hours (endogenous surfactant production increases)
• Without treatment: Progressive respiratory failure → death

Red Flags

• Worsening respiratory distress despite surfactant - consider complications (pneumothorax, pulmonary haemorrhage, sepsis)
• Sudden deterioration - tension pneumothorax until proven otherwise
• High O2 requirement (FiO2 >0.6) - consider repeat surfactant or intubation if on CPAP
• Persistent hypotension - may indicate PDA or sepsis

Bedside

• Continuous monitoring: SpO2 (pre-ductal), HR, RR, temperature
• Blood gas (capillary/arterial): pH, PaCO2, PaO2, base excess - guide respiratory support
• Blood glucose: Hypoglycaemia screening

Bloods

• FBC, CRP, blood culture: Rule out sepsis (clinical presentation overlaps)
• Blood gas: Serial - guide ventilation strategy

Imaging

• CXR: Ground-glass opacification (diffuse, bilateral), air bronchograms, reduced lung volumes, ETT position if intubated
• Serial CXR: If clinical deterioration - exclude pneumothorax, pulmonary haemorrhage

Special Tests

• Echocardiography: If persistent high O2 requirement - exclude CHD, assess PDA
• Transillumination: Rapid bedside test for pneumothorax (bright light through chest wall)

Antenatal Prevention

• Corticosteroids: Betamethasone 12mg IM ×2 doses 24h apart at 24-34 weeks - reduces RDS by ~50%, mortality, IVH, NEC

Delivery Room

• CPAP from birth: 5-8 cmH2O via T-piece or CPAP driver - establishes FRC, reduces need for intubation
• Surfactant: If FiO2 >0.3 on CPAP (or >0.4 - varies by unit); poractant alfa (Curosurf) 200mg/kg
• LISA technique: Thin catheter surfactant administration during CPAP - avoids intubation; reduced BPD risk

NICU Respiratory Support (Escalating)

1. CPAP (5-8 cmH2O): First-line; non-invasive; maintains FRC
2. NIPPV/BiPAP: Non-invasive; higher support than CPAP
3. Conventional mechanical ventilation (IPPV): If CPAP fails (persistent high FiO2, recurrent apnoeas, respiratory acidosis)
4. High-frequency oscillatory ventilation (HFOV): For refractory hypoxia or air leak

Pharmacological

• Surfactant: Poractant alfa 200mg/kg initial dose; can repeat 100mg/kg ×2 if ongoing high FiO2 requirement
• Caffeine citrate: Loading 20mg/kg IV; maintenance 5-10mg/kg OD - reduces apnoea of prematurity AND BPD (CAP trial)
• Analgesia/sedation: Morphine 10-20mcg/kg/hr if ventilated and distressed; minimise where possible

Supportive Care

• Thermoregulation: Incubator, plastic wrap (<28 weeks)
• Fluid management: Start at 60-80mL/kg/day; increase gradually; avoid fluid overload (worsens PDA, pulmonary oedema)
• Nutrition: Trophic feeds (breast milk) from day 1 if possible; TPN until full enteral feeds established
• Minimal handling: Clustered cares to reduce stress and energy expenditure

Referral Criteria

• All preterm infants with respiratory distress - neonatal team at delivery
• Persistent high FiO2 or respiratory failure despite surfactant - tertiary NICU if not already
• Air leak (pneumothorax) - chest drain; surgical team if needed
• Suspected CHD - cardiology/echocardiography

• With surfactant + CPAP: Survival >90% for infants >28 weeks with RDS
• Without surfactant: Mortality ~50% in very preterm RDS (historical)
• Surfactant NNT: ~6 to prevent 1 death in preterm RDS
• BPD (chronic lung disease): Defined as O2 requirement at 36 weeks CGA; affects ~30% of <28 weeks; most wean off O2 by 1-2 years
• CAP trial (caffeine): Reduced BPD from 47% to 36% AND improved 18-month neurodevelopmental outcome
• Long-term respiratory: Increased risk of asthma, reduced exercise tolerance, and abnormal lung function in childhood and adulthood

RDS vs TTN Comparison

Surfactant Preparations (UK)

Caffeine for Prematurity (CAP Trial)

Key Facts

NEC is the most common surgical emergency in neonatal intensive care. It has high mortality and significant long-term morbidity. Prevention through breast milk feeding and probiotic supplementation is increasingly emphasised.

Epidemiology

NEC affects 5-10% of VLBW infants (<1500g). Overall incidence in NICU admissions is ~2-5%. Mortality is 20-30% overall and up to 50% in those requiring surgery. NEC is more common in formula-fed infants and those with PDA.

Aetiology

NEC is multifactorial:

• Prematurity: Immature gut mucosal barrier, motility, and immune function
• Enteral feeding: Formula feeding increases risk; breast milk is protective
• Ischaemia: Perinatal asphyxia, low-flow states (PDA, cardiac defects)
• Infection: Gut colonisation with pathogenic bacteria
• Iatrogenic: Umbilical catheters, indometacin/ibuprofen for PDA

Pathophysiology

NEC results from a combination of gut mucosal immaturity, abnormal bacterial colonisation, and an exaggerated inflammatory response. The immature gut barrier allows bacterial translocation, triggering a pro-inflammatory cascade (TNF-α, IL-6, PAF) leading to mucosal necrosis. This progresses from superficial mucosal injury to full-thickness bowel wall necrosis, perforation, peritonitis, and septic shock.

Clinical Features

• Feeding intolerance: Increased gastric aspirates, vomiting (bile-stained)
• Abdominal distension: Progressive; tender; erythema of abdominal wall (late)
• Bloody stools: Frank blood or occult blood positive
• Systemic signs: Apnoeas, bradycardias, temperature instability, lethargy, metabolic acidosis, hypotension, DIC

Bell Staging

• Stage I (Suspected): Feeding intolerance, mild distension, occult blood in stool; non-specific systemic signs
• Stage II (Definite): Gross blood in stool, marked distension, absent bowel sounds; pneumatosis on AXR
• Stage III (Advanced): Perforation, peritonitis, shock, DIC; pneumoperitoneum on AXR

Red Flags

• Fixed dilated bowel loop on serial AXR - suggests full-thickness necrosis
• Portal venous gas - severe NEC
• Pneumoperitoneum - perforation; needs urgent surgery
• Rapidly worsening metabolic acidosis and thrombocytopenia - fulminant NEC
• Abdominal wall erythema - transmural necrosis

Bedside

• Observations: HR, RR, SpO2, BP, temperature - sepsis screen
• Abdominal examination: Distension, tenderness, erythema, absent bowel sounds

Bloods

• FBC: Thrombocytopenia (poor prognosis indicator), neutropenia or neutrophilia
• CRP: Elevated (serial monitoring)
• Blood gas: Metabolic acidosis (raised lactate, low pH, low bicarbonate)
• Blood culture: Before starting antibiotics
• Coagulation: DIC screen (PT, APTT, fibrinogen, D-dimer)
• U&Es, glucose: Metabolic derangement

Imaging

• AXR (supine and lateral decubitus): Key investigation
  • Dilated bowel loops
  • Pneumatosis intestinalis (intramural gas - pathognomonic)
  • Portal venous gas (branching lucencies over liver)
  • Pneumoperitoneum (free air = perforation)
  • Fixed dilated loop on serial films (failed to change position)
• USS abdomen: May show free fluid, thickened bowel wall, absent peristalsis, portal venous gas

Special Tests

• Serial AXR: 6-12 hourly in acute phase - track progression
• Stool culture: If infective cause suspected

Medical (Stage I-II)

• Nil by mouth: For 7-14 days depending on severity
• NGT on free drainage: Decompress stomach
• IV antibiotics: Triple therapy - per local protocol (e.g., amoxicillin/ampicillin + gentamicin + metronidazole) for 7-14 days
• TPN: Nutritional support during bowel rest
• IV fluids: Resuscitation with 0.9% NaCl boluses (10-20mL/kg)
• Blood products: Platelets if <50, FFP if DIC, pRBC if anaemic
• Inotropes: If cardiovascular compromise despite fluid resuscitation
• Serial AXR: 6-12 hourly during acute phase

Surgical (Stage III or Failed Medical)

• Indications: Pneumoperitoneum (perforation), clinical deterioration despite medical treatment, fixed dilated bowel loop, abdominal wall cellulitis
• Procedures: Laparotomy with resection of necrotic bowel ± stoma formation; or primary peritoneal drainage (PD) in very small/unstable infants
• Stoma reversal: Usually 6-12 weeks later when recovered

Prevention

• Breast milk feeding: Reduces NEC by ~50% - maternal EBM or donor human milk
• Probiotics: Growing evidence for NEC prevention in preterm infants (Lactobacillus, Bifidobacterium); used in many UK NICUs
• Cautious enteral feeding advancement: Standardised feeding protocols reduce NEC incidence
• Avoid unnecessary antibiotics: Prolonged empirical antibiotics increase NEC risk

Referral Criteria

• All suspected NEC - neonatal surgical team consultation
• Pneumoperitoneum - emergency surgery
• Clinical deterioration despite medical management - surgical review

• Overall mortality: 20-30%; higher in surgical NEC (~50%)
• Short bowel syndrome: Affects ~10-25% of surgical survivors; may need long-term TPN
• Strictures: ~20-30% post-NEC - may present weeks later with obstruction
• Neurodevelopmental impairment: Increased risk compared with gestational age-matched controls without NEC
• Recurrence: ~5-10% in medically managed NEC

Bell Staging of NEC

AXR Findings in NEC

Key Facts

The UK immunisation programme is one of the most comprehensive in the world. It is coordinated by the UK Health Security Agency (UKHSA) and follows the Green Book (Immunisation Against Infectious Disease). High uptake is essential for individual protection and herd immunity.

Epidemiology

UK childhood vaccination uptake for primary immunisations is approximately 91-93% (below the 95% WHO target for herd immunity). MMR uptake has been recovering since the Wakefield fraud but remains below 95% in some areas. Vaccine-preventable disease outbreaks (measles, pertussis) occur when uptake drops.

Aetiology

The schedule is designed to protect against the most serious childhood infections at the age of greatest vulnerability. Vaccines stimulate adaptive immunity (humoral and cellular) without causing the disease.

Pathophysiology

Vaccine types:

• Inactivated/killed: DTaP, IPV, HepB - cannot cause infection; need multiple doses
• Live attenuated: MMR, rotavirus, BCG, nasal flu - weakened pathogen; contraindicated in immunosuppression
• Conjugate: PCV13, MenB, MenACWY, Hib - polysaccharide linked to protein carrier for T-cell response
• Toxoid: Diphtheria, tetanus - inactivated toxin
• mRNA/viral vector: COVID-19 vaccines (not part of routine childhood schedule but relevant historically)

Common Post-Vaccination Reactions

• Injection site: Pain, redness, swelling (50%+)
• Systemic: Fever, irritability, poor feeding (especially after MenB - paracetamol recommended prophylactically)
• MMR: Fever and rash at 7-10 days ('mini measles') - not infectious
• Rotavirus: Mild diarrhoea

Red Flags

• Anaphylaxis post-vaccination - 1 in 1,000,000; treat per resuscitation protocol
• High fever >40°C - unusual; investigate for intercurrent illness
• Hypotonic-hyporesponsive episode - rare reaction to pertussis component; usually self-limiting
• Intussusception post-rotavirus - very rare (~1-6 per 100,000); present within 7 days

Bedside

• Post-vaccination monitoring: Observe for 15 minutes after vaccination
• Temperature: If febrile post-vaccination

Bloods

• Not routinely needed
• Antibody levels: Check in immunocompromised patients to assess vaccine response
• Immunoglobulin levels: If primary immunodeficiency suspected (poor vaccine response, recurrent infections)

Special Tests

• COVER (Cover of Vaccination Evaluated Rapidly): PHE programme monitoring vaccine uptake

Non-pharmacological

• Informed consent: Parents should receive information leaflet before each vaccination
• Correct technique: IM injection (anterolateral thigh in infants, deltoid in older children)
• Cold chain management: Vaccines stored at 2-8°C; do not freeze

Pharmacological

• Paracetamol prophylaxis: Recommended for MenB vaccination (120mg at vaccination, then at 4-6h and 4-6h later for infants 2-3 months)
• Catch-up schedules: For missed or delayed vaccinations - Green Book provides detailed guidance
• Immunocompromised children: Live vaccines contraindicated; may need additional doses of inactivated vaccines; specialist advice from immunology

Referral Criteria

• Suspected immunodeficiency (recurrent infections, poor vaccine response) - immunology
• Severe adverse reaction (anaphylaxis) - allergy assessment; report via Yellow Card
• Parents with vaccine concerns - address with evidence-based information; do not refuse registration

• Vaccine efficacy: DTaP ~85-90% after primary course; MMR ~93% after 1 dose, ~97% after 2 doses; MenB ~88% against MenB strains
• Herd immunity threshold: Measles ~95%; pertussis ~92-94%
• Eradication successes: Smallpox eradicated globally; polio eliminated from most countries
• UK measles outbreaks: Occur when MMR uptake drops below 95% - emphasises importance of maintaining high uptake
• Adverse events: Serious reactions extremely rare; benefits far outweigh risks for all routine vaccines

UK Childhood Immunisation Schedule (2024/25)

Live vs Inactivated Vaccines

Key Facts

Childhood asthma is extremely common and carries significant morbidity. Good asthma control is achievable for most children with appropriate education, inhaler technique, and stepwise pharmacological management.

Epidemiology

Asthma affects approximately 1.1 million children in the UK. Prevalence peaks at age 5-9. Boys are more commonly affected before puberty; girls after puberty. Approximately 25,000 children are hospitalised for asthma annually. UK childhood asthma mortality has improved but remains higher than comparable European countries.

Aetiology

• Genetic: Strong genetic component; polygenic; family history of atopy/asthma
• Environmental: Allergens (house dust mite, pets, mould), viral infections, air pollution, tobacco smoke exposure, exercise, cold air

Pathophysiology

Asthma is a chronic inflammatory airway disease characterised by:

• Airway inflammation: Eosinophilic (type 2), Th2-mediated; mast cell activation
• Bronchial hyperreactivity: Exaggerated bronchoconstriction to triggers
• Reversible airflow obstruction: Smooth muscle contraction, mucosal oedema, mucus hypersecretion
• Airway remodelling: Chronic inflammation → subepithelial fibrosis, smooth muscle hypertrophy (can become irreversible)

Typical Features

• Recurrent episodic wheeze (expiratory)
• Cough (especially nocturnal, exercise-induced)
• Breathlessness, chest tightness
• Symptom variability - worse at night/early morning, with exercise, in cold air, with viral infections
• Symptom reversibility with bronchodilators
• Atopic history (eczema, allergic rhinitis, food allergy) or family history

Severity Assessment (Acute Exacerbation)

• Moderate: SpO2 ≥92%, able to talk, PEF >50%
• Severe: SpO2 <92%, too breathless to talk/feed, PEF 33-50%, HR >140 (<5yr) or >125 (>5yr)
• Life-threatening: SpO2 <92%, silent chest, cyanosis, exhaustion, confusion, PEF <33%

Red Flags

• Previous PICU admission - high-risk patient
• Frequent oral steroid courses (>2/year) - poorly controlled; step up treatment
• Night-time waking with wheeze/cough - indicates inadequate control
• Not growing - consider alternate diagnosis or steroid side effects

Bedside

• PEF diary: Home PEF monitoring - >20% variability supports asthma diagnosis
• SpO2: In acute exacerbation

Objective Tests (>5 years - NICE NG80)

• Spirometry: FEV1/FVC ratio; obstructive pattern (ratio <0.7 or below LLN)
• Bronchodilator reversibility: ≥12% AND 200mL improvement in FEV1 post-SABA
• FeNO: ≥35ppb in children supports eosinophilic airway inflammation
• PEF variability: >20% on twice-daily readings over 2-4 weeks

Bloods

• Total IgE, specific IgE/skin prick tests: Identify atopic triggers
• FBC: Eosinophilia supports atopic inflammation

Imaging

• CXR: If diagnostic uncertainty (exclude foreign body, infection, structural abnormality)

Special Tests

• Challenge tests: Exercise, methacholine - specialist use
• Sweat test: If CF suspected

Non-pharmacological

• Written personalised asthma action plan: Every child - recognise worsening, when to step up, when to seek help
• Inhaler technique: Review at every visit; spacer device (Volumatic/AeroChamber) mandatory for MDI
• Trigger avoidance: Tobacco smoke, allergens; weight management
• Annual influenza vaccination: All children with asthma

Pharmacological (BTS/SIGN Stepwise - Children 5-12)

• Step 1: SABA PRN (salbutamol 100mcg 2 puffs via spacer)
• Step 2: Add low-dose ICS (beclometasone 100-200mcg/day or equivalent)
• Step 3: Add LABA (salmeterol) or LTRA (montelukast 5mg OD); review and stop LTRA if no benefit at 4-8 weeks
• Step 4: Increase ICS to medium dose (200-400mcg beclometasone equivalent)
• Step 5: Refer to specialist; high-dose ICS, oral steroids, biologic therapy (omalizumab, mepolizumab for severe eosinophilic asthma)

Children <5:

Acute Exacerbation

• Salbutamol 10 puffs via spacer (2 puffs at a time); repeat every 20 min if needed
• Oxygen: If SpO2 <94%
• Prednisolone: 1-2mg/kg (max 40mg) for 3 days
• Nebulised salbutamol + ipratropium: If severe/life-threatening
• IV MgSO4, IV aminophylline: Life-threatening asthma

Referral Criteria

• Diagnostic uncertainty - respiratory paediatrician
• Poorly controlled despite Step 3-4 - specialist review
• Previous PICU admission - specialist follow-up
• Considering biologic therapy - tertiary severe asthma centre

• ~50% of childhood asthma resolves by adulthood (especially mild, non-atopic)
• Persistent asthma: More likely if severe, atopic, female, early onset with atopy
• Well-controlled asthma: Children should have normal lung function, normal activity, and no limitations
• Mortality: ~10-20 children die from asthma each year in the UK - most deaths are preventable with better management
• NRAD findings: Most childhood asthma deaths involved modifiable factors (poor adherence, no action plan, under-treatment)

BTS/SIGN Stepwise Management (Children 5-12)

ICS Dose Equivalents (Children)

Key Facts

Viral-induced wheeze is the most common form of wheeze in preschool children. It is pathophysiologically distinct from atopic asthma and generally has an excellent prognosis with most children outgrowing symptoms by school age.

Epidemiology

Approximately 30% of children wheeze in the first 3 years of life. ~60% of these are 'transient early wheezers' who stop wheezing by age 6. Boys are more commonly affected. Peak incidence is in the winter viral season. Only ~30-40% of wheezy preschool children go on to develop persistent asthma.

Aetiology

• Viral triggers: Rhinovirus (most common), RSV (especially bronchiolitis), parainfluenza, adenovirus, human metapneumovirus
• Risk factors: Small airway calibre (male, preterm), parental smoking, older siblings (viral exposure), nursery attendance
• NOT driven by atopy, allergens, or exercise (unlike atopic asthma)

Pathophysiology

Viral infection causes airway inflammation, epithelial damage, and mucosal oedema in small-calibre airways. In young children, the airways are relatively narrow (resistance proportional to radius⁴), so even small amounts of mucosal oedema and mucus cause significant airflow obstruction. This is a predominantly neutrophilic (not eosinophilic) inflammatory response, explaining the relatively poor response to corticosteroids compared with atopic asthma.

Typical Features

• Wheeze only during viral URTI episodes
• Cough, rhinorrhoea, low-grade fever preceding wheeze
• No symptoms between episodes (no interval wheeze)
• Usually <3 years of age
• No atopic features (no eczema, no food allergy, normal IgE)
• Family history of atopy often ABSENT

Red Flags

• Wheeze between viral episodes - consider asthma
• Persistent wheeze not responding to bronchodilators - consider foreign body, CF, anatomical abnormality
• Failure to thrive with wheeze - consider CF, immunodeficiency
• Fixed wheeze or stridor - structural airway abnormality (tracheomalacia, vascular ring)

Bedside

• SpO2: In acute episode
• Clinical assessment: Work of breathing, feeding ability, hydration
• Objective tests (spirometry, FeNO) NOT possible in <5 years - diagnosis is clinical

Bloods

• Not routinely needed
• Total IgE, specific IgE: If atopic asthma suspected (interval symptoms, atopic features)

Imaging

• CXR: Only if diagnostic uncertainty (exclude foreign body, congenital anomaly)

Special Tests

• NPA (nasopharyngeal aspirate): If viral aetiology needs confirmation (e.g., RSV in bronchiolitis)
• Sweat test: If recurrent wheeze with failure to thrive or recurrent chest infections

Acute Episode

• Salbutamol via spacer: 2-10 puffs (trial of treatment); response may be less marked than in asthma, especially <1 year
• Oxygen: If SpO2 <92%
• Prednisolone: 1-2mg/kg for 3 days - less effective than in asthma; consider only for moderate-severe episodes
• Supportive: Adequate hydration, antipyretics

Prevention/Maintenance

• Intermittent montelukast: At onset of viral symptoms - may reduce severity and duration
• Regular low-dose ICS: Consider trial if frequent episodes (>3/year) or severe episodes; assess response at 8 weeks
• Parental smoking cessation: Single most important modifiable risk factor

Non-pharmacological

• Parental education: Explain difference from asthma; provide reassurance about prognosis
• Written management plan: When to use inhaler, when to seek medical help
• Follow-up: Reassess at 8 weeks if ICS trial started; discontinue if no benefit

Referral Criteria

• Recurrent severe episodes requiring hospitalisation - respiratory paediatrician
• Symptoms persisting beyond age 5 with atopic features - investigate for asthma
• Failure to thrive, chronic cough, recurrent infections - investigate for CF, immunodeficiency

• ~60% stop wheezing by age 6 (transient early wheezers)
• ~20% develop persistent atopic asthma
• ~20% have episodic wheeze that gradually improves through childhood
• Risk factors for progression to asthma: Atopic eczema, parental asthma, persistent wheeze between episodes, blood eosinophilia, positive aeroallergen sensitisation
• Lung function: Generally normal in adulthood for those with isolated viral-induced wheeze

Viral-Induced Wheeze vs Atopic Asthma

Key Facts

Bronchiolitis is the most common cause of hospitalisation in infants in the UK. It is a self-limiting condition, and evidence consistently shows that pharmacological interventions do not alter the disease course. Supportive care is the cornerstone of management.

Epidemiology

Bronchiolitis affects approximately 33% of infants in their first year. About 2-3% require hospitalisation (~30,000 admissions/year in England). Peak incidence: November to March. Mortality is very low (<0.5%) but higher in those with comorbidities (prematurity, CHD, immunodeficiency).

Aetiology

• RSV: 70-80% of cases
• Other: Rhinovirus, human metapneumovirus, parainfluenza, adenovirus, influenza
• Risk factors for severe disease: Prematurity (<32 weeks), congenital heart disease, chronic lung disease, immunodeficiency, neuromuscular disease, age <6 weeks

Pathophysiology

RSV infects bronchiolar epithelial cells, causing necrosis, peribronchiolar inflammation, oedema, and mucus plugging. This leads to small airway obstruction with air trapping and atelectasis. The small calibre of infant bronchioles means even minor inflammation causes significant obstruction. Viral shedding continues for 1-3 weeks. Immune response (RSV-specific IgE, neutrophilic inflammation) contributes to airway obstruction.

Typical Course

• Day 1-3: Coryzal prodrome - rhinorrhoea, sneezing, mild cough, low-grade fever
• Day 3-5: Wheeze, crackles, tachypnoea, increased work of breathing, feeding difficulty
• Peak severity: Day 3-5 of respiratory symptoms
• Recovery: 7-10 days (cough may persist 2-3 weeks)

Examination Findings

• Bilateral wheeze AND crackles (characteristic)
• Tachypnoea, subcostal/intercostal recession
• Nasal flaring, head bobbing (infants)
• Hyperinflated chest

Red Flags

• SpO2 <92% - requires supplemental oxygen
• Apnoeas - especially in <6 weeks or ex-preterm; may need monitoring
• Inability to maintain adequate oral intake (<50-75%) - NG or IV fluids
• Exhaustion, reduced consciousness - escalate to HDU/PICU
• Cyanosis - severe disease

NICE NG9 - Investigations NOT Routinely Recommended

• CXR, blood tests, blood gas - not routine
• NPA for RSV - only for infection control (cohorting in hospital)

Bedside

• SpO2: Key monitoring parameter
• Respiratory assessment: Work of breathing, feeding ability
• Weight: Monitor hydration

Bloods

• Only if diagnostic uncertainty or suspected secondary bacterial infection
• Blood gas: If severe respiratory distress or impending respiratory failure

Imaging

• CXR: Only if diagnostic uncertainty or atypical features (do NOT do routinely - overdiagnoses pneumonia and leads to unnecessary antibiotics)

Non-pharmacological (NICE NG9)

• Oxygen: If SpO2 persistently <92% - nasal prongs or high-flow nasal cannula (HFNC)
• Feeding support: NG feeding if oral intake <50-75% of usual; IV fluids (maintenance rate with 0.9% NaCl + 5% glucose) if NG not tolerated
• Minimal handling: Reduce stimulation; cluster cares
• Nasal suctioning: Only if nasal secretions are causing respiratory distress or feeding difficulty - not routine
• Positioning: Head elevated 30°

Pharmacological

• NONE routinely recommended (NICE NG9):
  • Bronchodilators (salbutamol, ipratropium) - NOT effective
  • Corticosteroids - NOT effective
  • Antibiotics - NOT indicated unless secondary bacterial infection confirmed
  • Nebulised hypertonic saline - NOT recommended routinely
  • Adrenaline - NOT recommended

Escalation

• HFNC (High-Flow Nasal Cannula): Increasingly used for moderate-severe bronchiolitis; provides heated humidified oxygen with low-level CPAP effect
• CPAP/BIPAP: If HFNC insufficient
• Intubation and ventilation: Rare; for respiratory failure or frequent apnoeas

Prevention

• Palivizumab: Monthly IM injection during RSV season for high-risk infants (ex-preterm with CLD, haemodynamically significant CHD)
• Hand hygiene: Key infection control measure
• Nirsevimab: Newer long-acting anti-RSV monoclonal antibody - single dose; increasingly available for broader neonatal population

Referral Criteria

• Admission: SpO2 <92%, inadequate feeding, apnoeas, severe distress, high-risk infant
• PICU: Respiratory failure, recurrent apnoeas, exhaustion

• Self-limiting: Most recover within 7-10 days; cough may persist 2-3 weeks
• Hospital stay: Median 2-3 days for admitted infants
• Mortality: <0.5% overall; <0.1% in previously healthy term infants
• Post-bronchiolitis wheeze: ~40-50% have recurrent wheeze in first 2-3 years; most resolve by school age
• Association with asthma: RSV bronchiolitis associated with increased risk of wheezing in later childhood (not definitively causative)

NICE NG9 - When to Admit

NICE NG9 - When to Seek Urgent Care (Parent Advice)

Key Facts

Croup is one of the most common causes of acute upper airway obstruction in children. It is almost always a benign, self-limiting condition. Oral dexamethasone has revolutionised management, significantly reducing the need for hospitalisation.

Epidemiology

Croup affects approximately 3-5% of children in the first 2 years of life. ~5% of affected children attend ED. Only ~1-5% of those seen in ED require admission. Peak incidence: Autumn/winter. Male:female ratio ~1.5:1.

Aetiology

• Parainfluenza (types 1 and 3): ~75% of cases
• RSV, influenza A and B, adenovirus, rhinovirus
• Spasmodic croup: Recurrent episodes without viral prodrome; possible allergic/atopic component

Pathophysiology

Viral infection causes inflammation, oedema, and mucus production in the subglottic region (narrowest part of the paediatric airway). The subglottic region is surrounded by the rigid cricoid cartilage, so even mild oedema causes significant narrowing. Airflow through the narrowed airway produces the characteristic inspiratory stridor. The barking cough results from vibration of the inflamed vocal cords.

Typical Course

• Day 1-2: Coryzal prodrome - rhinorrhoea, mild fever, sore throat
• Day 2-3: Onset of barking cough, hoarse voice, inspiratory stridor
• Worse at night: Symptoms characteristically worsen in the evening/overnight
• Duration: 2-7 days; peak severity usually on night 2-3

Severity Assessment (Westley Score)

• Mild: Barking cough, no stridor at rest, no/mild recession
• Moderate: Stridor at rest, visible recession, no agitation
• Severe: Stridor at rest, marked recession, agitation/lethargy
• Impending failure: Reduced consciousness, minimal stridor (indicates failing ventilation), cyanosis

Red Flags

• Stridor at rest with agitation - severe croup, needs nebulised adrenaline
• Drooling, toxic appearance, no barking cough - think epiglottitis
• Not responding to dexamethasone and adrenaline - consider alternative diagnosis (bacterial tracheitis, foreign body, retropharyngeal abscess)
• Recurrent croup (>2 episodes/year) - consider underlying subglottic stenosis or haemangioma

Bedside

• Clinical diagnosis: Do NOT distress the child with investigations
• SpO2: Monitor - desaturation is a late sign
• Westley croup score: Grade severity

Bloods

• NOT routinely needed - clinical diagnosis

Imaging

• AP neck X-ray: 'Steeple sign' (subglottic narrowing) - rarely needed; do NOT delay treatment for imaging
• Lateral neck X-ray: If epiglottitis suspected ('thumb sign')

Special Tests

• NPA: Only if atypical or influenza season (guide antiviral therapy)
• Direct laryngoscopy: If recurrent croup or diagnostic uncertainty - subglottic stenosis, haemangioma

All Severities

• Oral dexamethasone 0.15mg/kg (single dose; max 12mg) - effective within 2-4 hours; lasts 24-48 hours
• Alternative: Prednisolone 1-2mg/kg if dexamethasone unavailable

Mild Croup

• Dexamethasone and discharge with safety-net advice
• Parent education: Sit upright, comfort child (crying worsens symptoms), cool night air may help

Moderate Croup

• Dexamethasone
• Observe for 2-4 hours after treatment
• Discharge if improving; admit if not

Severe Croup

• Nebulised adrenaline 5mL 1:1000 - rapid effect (within 10-30 min); wears off in ~2 hours; observe for minimum 2 hours for rebound
• Oxygen if SpO2 <92%
• Dexamethasone (if not already given)
• Admit: Monitor in resuscitation area/HDU
• Repeat adrenaline if symptoms recur
• Intubation: Rarely needed; by experienced anaesthetist; use ETT 0.5-1mm smaller than normal

Referral Criteria

• Severe croup not responding to adrenaline - anaesthetics/PICU
• Recurrent croup (>2 episodes/year) - ENT for airway assessment
• Suspected bacterial tracheitis or epiglottitis - emergency management

• Self-limiting: Most children recover within 2-7 days
• Hospitalisation: Only 1-5% of ED attenders
• Intubation: <1% of hospitalised children
• Mortality: Extremely rare in developed countries (<0.5 per 100,000)
• Recurrent croup: ~5% have recurrent episodes; may be associated with atopy or underlying airway abnormality

Westley Croup Score

Mild 0-2; Moderate 3-5; Severe 6-11; Impending failure ≥12.

Croup vs Epiglottitis

Key Facts

Pneumonia is common in children and a significant cause of hospitalisation. Most cases are viral and self-limiting, but bacterial pneumonia requires prompt antibiotic treatment. The BTS guidelines provide a structured evidence-based approach.

Epidemiology

Community-acquired pneumonia (CAP) affects approximately 14-33 per 10,000 children per year in the UK. It is the most common serious bacterial infection in children. Hospitalisation rate is approximately 1-4 per 1,000 per year. PCV13 vaccination has significantly reduced invasive pneumococcal disease.

Aetiology

<5 years: Predominantly viral (RSV, influenza, parainfluenza, adenovirus, rhinovirus) >5 years: Increasing bacterial (S. pneumoniae, Mycoplasma pneumoniae) All ages: S. pneumoniae is the most common bacterial cause; Staphylococcus aureus causes severe necrotising pneumonia (rare)

Pathophysiology

Clinical Features

• Cough (dry → productive), fever, tachypnoea, breathlessness
• Reduced air entry, dullness to percussion, bronchial breathing, crackles (focal)
• Abdominal pain (referred from lower lobe pneumonia - common in children)
• Grunting (in infants - sign of significant respiratory distress)

Tachypnoea (WHO Definition)

• <2 months: RR >60
• 2-12 months: RR >50
• 1-5 years: RR >40

• 5 years: RR >20

Red Flags

• SpO2 <92% - admit, supplemental O2
• Grunting - significant respiratory distress
• Not responding to 48h oral antibiotics - consider empyema, resistant organism, alternative diagnosis
• Staphylococcal pneumonia - rapidly progressive, cavitation, pneumatoceles
• Recurrent pneumonia (≥2 in same lobe) - investigate for underlying cause (FB, bronchiectasis, immunodeficiency)

Bedside

• SpO2: Essential in all children with suspected pneumonia
• Temperature: Fever assessment
• Respiratory assessment: RR, work of breathing, auscultation

Bloods

• FBC, CRP: If admitted; CRP >80 more suggestive of bacterial (not diagnostic)
• Blood culture: If admitted or severe
• Mycoplasma serology/PCR: If atypical pneumonia suspected

Imaging

• CXR: NOT routinely required for non-severe community-managed cases; indicated if: severe, not responding to treatment, recurrent, suspected complication
• USS chest: If effusion suspected - more sensitive than CXR for small effusions
• CT chest: Rarely needed; for complex empyema or abscess

Special Tests

• NPA/throat swab: Viral PCR if viral aetiology needs confirmation
• Sputum culture: In older children who can expectorate
• Pleural fluid MC&S: If effusion drained

Community Management (Non-Severe)

• Amoxicillin 25mg/kg TDS (or 50mg/kg BD) for 5 days - first-line for all ages
• Clarithromycin/erythromycin: If penicillin allergy or atypical pneumonia suspected (Mycoplasma)
• Safety-net: Review if not improving at 48h; return if worsening

Hospital Management (Severe)

• Oxygen: If SpO2 <92%
• IV antibiotics: Amoxicillin 25mg/kg TDS IV ± clarithromycin 7.5mg/kg BD; co-amoxiclav 30mg/kg TDS if severe or <2 years
• IV fluids: If not feeding adequately
• Switch to oral: When afebrile for 24h and improving; total course 5-7 days (longer for complications)

Complications Management

• Lung abscess: Prolonged IV antibiotics (4-6 weeks); rarely needs drainage

Referral Criteria

• SpO2 <92% - admit
• Not responding to 48h oral antibiotics - investigate (CXR, bloods, USS)
• Empyema - paediatric surgery/respiratory for drainage
• Recurrent pneumonia - investigate for underlying cause (immunodeficiency, CF, structural, FB)

• Uncomplicated CAP: Full recovery in 1-2 weeks
• Empyema: Prolongs illness to 2-6 weeks; excellent long-term lung recovery in children
• Mortality: <1% in previously healthy children in UK; higher in immunocompromised, neonates
• Post-PCV13: Significant reduction in pneumococcal pneumonia and invasive disease
• Mycoplasma: Self-limiting but slow recovery; macrolide treatment shortens duration of symptoms

BTS Paediatric Pneumonia Antibiotic Choice

When to CXR in Childhood Pneumonia

Key Facts

Pertussis remains an important cause of morbidity and mortality in young infants despite vaccination. The UK maternal vaccination programme, introduced in 2012 following a major outbreak, has been highly effective in reducing neonatal disease.

Epidemiology

Pertussis notifications in England: typically 5,000-10,000 per year with cyclical peaks every 3-4 years. Incidence is highest in infants <3 months (too young to be vaccinated). ~90% of pertussis deaths occur in infants <3 months. Adolescents and adults are an important reservoir of infection (waning immunity).

Aetiology

• Bordetella pertussis: Primary pathogen
• Bordetella parapertussis: Milder illness; not prevented by pertussis vaccine
• Transmission: Respiratory droplets; highly contagious; incubation 7-21 days

Pathophysiology

B. pertussis attaches to ciliated respiratory epithelium via filamentous haemagglutinin and pertactin. It produces pertussis toxin (ADP-ribosylating exotoxin) which impairs immune cell function, increases insulin secretion (causing lymphocytosis), and sensitises the cough reflex. Tracheal cytotoxin causes ciliated cell damage and mucus hypersecretion. The paroxysmal cough is mediated by sensitisation of cough receptors and persists for weeks after the organism is cleared.

Three Phases

Catarrhal phase (1-2 weeks):

• Coryzal symptoms, mild cough, low-grade fever
• MOST INFECTIOUS period

Paroxysmal phase (2-8 weeks):

• Paroxysms of intense cough (10-30 coughs in a row)
• Inspiratory 'whoop' between paroxysms
• Post-tussive vomiting
• Facial congestion/cyanosis during paroxysms
• May be well between paroxysms

Convalescent phase (weeks to months):

• Gradual resolution; cough may persist for months ('100-day cough')
• Paroxysms may recur with subsequent viral infections

Infants <3 Months

• May NOT whoop - too small to generate whoop
• Apnoea may be the ONLY feature
• Cyanotic episodes, feeding difficulty
• High risk of complications: Pneumonia, seizures, encephalopathy, death

Red Flags

• Apnoea in young infant - admit for monitoring
• Marked lymphocytosis (>20 × 10⁹/L) with leucocytosis - correlates with severe disease in infants
• Pulmonary hypertension - rare but can be fatal in infants with hyperleucocytosis

Bedside

• SpO2: During paroxysms
• Video of paroxysm: Helpful if not witnessed by clinician

Bloods

• FBC: Marked lymphocytosis (>10 × 10⁹/L in >50% of cases; >20 correlates with severity)
• Anti-pertussis toxin IgG: Oral fluid or serum - diagnostic after 2 weeks of symptoms

Microbiology

• Nasal swab PCR: Best in first 2-3 weeks; specificity >95%
• Culture: Gold standard but slow (takes 7 days); sensitivity lower than PCR

Imaging

• CXR: If pneumonia suspected; may show perihilar infiltrates or consolidation

Pharmacological

• Azithromycin: 10mg/kg OD for 3 days (first-line) - reduces infectivity; does NOT shorten paroxysmal phase if already established
• Clarithromycin: Alternative (7.5mg/kg BD for 7 days)
• Most effective if given during catarrhal phase
• Antibiotics for household contacts (post-exposure prophylaxis) - especially if vulnerable contacts (pregnant women, young infants)

Non-pharmacological

• Admission: Infants <6 months (especially <3 months); apnoeas; severe paroxysms; feeding difficulty
• Monitoring: Continuous SpO2 and apnoea monitoring for young infants
• Supportive care: Small frequent feeds, minimal stimulation, oxygen if needed
• Suction: Gentle nasal suction if needed

Prevention

• Childhood vaccination: 6-in-1 (DTaP) at 8, 12, 16 weeks + pre-school booster at 3yr 4mo
• Maternal vaccination: Pertussis-containing vaccine (dTaP/IPV) from 16 weeks gestation - ~90% effective at preventing infant disease
• Post-exposure prophylaxis: Azithromycin for household contacts
• School exclusion: 48 hours after starting antibiotics or 21 days from symptom onset if untreated

Referral Criteria

• All infants <3 months with suspected pertussis - admission
• Apnoeas or cyanotic episodes - PICU assessment
• Severe lymphocytosis in infant - haematology/PICU (exchange transfusion in extreme cases)

• Overall mortality: <0.5% in developed countries; higher in unvaccinated infants <3 months
• Infant mortality: ~1% in hospitalised infants <3 months; most deaths from pulmonary hypertension/hyperleucocytosis
• Duration: Paroxysmal cough lasts 2-8 weeks; total illness 6-12 weeks ('100-day cough')
• Post-pertussis bronchiectasis: Rare with modern management
• Maternal vaccination: Has reduced infant pertussis deaths by ~90% since introduction

Pertussis Phases

When to Notify and Test

Key Facts

Febrile seizures are benign convulsions triggered by fever in developmentally normal children. They are one of the most frightening experiences for parents but carry an excellent prognosis. The key role of the clinician is to identify and treat the source of fever, exclude meningitis, and provide parental reassurance.

Epidemiology

Febrile seizures affect 2-5% of children between 6 months and 5 years. Peak incidence: 18 months. Boys are slightly more commonly affected. ~30% recur. Family history is common (polygenic inheritance).

Aetiology

• Febrile seizures occur during a rapid rise in temperature, usually >38°C
• Most common triggers: Viral URTIs, otitis media, UTI, roseola (HHV-6 - classic cause)
• NOT caused by: Intracranial infection (by definition - must exclude meningitis/encephalitis)
• Genetic predisposition: Polygenic; family history in ~25-40%

Pathophysiology

The immature brain has a lower seizure threshold. Fever increases neuronal excitability through temperature-dependent ion channel changes and inflammatory cytokines (IL-1β, TNF-α). The mechanism is NOT simply the height of fever but likely relates to the rate of temperature rise and individual genetic susceptibility.

Simple Febrile Seizure (70-80%)

• Generalised tonic-clonic
• Duration <15 minutes (usually <5 min)
• Single episode in 24-hour period
• Complete recovery within 1 hour
• Age 6 months to 5 years
• Developmentally normal child

Complex Febrile Seizure (20-30%)

• Focal features (one-sided movements, eye deviation)
• Duration >15 minutes
• Recurs within 24 hours
• Incomplete recovery (prolonged drowsiness, focal weakness - Todd's paresis)

Red Flags

• Signs of meningitis (neck stiffness, bulging fontanelle, non-blanching rash, photophobia)
• Prolonged seizure >5 min - treat with buccal midazolam as per status epilepticus protocol
• Developmental concerns - may indicate underlying neurological condition
• Very young infant (<6 months) - lower threshold for investigation including LP
• Repeated complex febrile seizures - consider underlying epilepsy syndrome

Simple Febrile Seizure

• No routine investigations required - clinical diagnosis
• Identify and treat source of fever (examine ears, throat, chest, urine)
• Urine MC&S: Recommended (UTI is a common occult source in young children)

Complex Febrile Seizure

• Consider LP: If <12 months, signs of meningitis, or not fully recovered
• Blood glucose: Post-seizure
• U&Es, blood culture, FBC: If systemically unwell
• EEG: Not routine; consider if recurrent complex febrile seizures
• MRI brain: Not routine; consider if focal or recurrent complex seizures

When to LP

• Signs of meningitis
• Age <12 months (meningism may be absent)
• Complex febrile seizure with incomplete recovery
• Not immunised against Hib or meningococcus

Acute Seizure (>5 Minutes)

• Buccal midazolam 0.5mg/kg (max 10mg) - parents should have emergency medication if recurrent
• Rectal diazepam 0.5mg/kg: Alternative if buccal midazolam not available
• Follow APLS status epilepticus protocol if not terminating

Post-Seizure

• Recovery position: Once seizure stops
• Identify and treat fever source: Antibiotics if bacterial infection identified
• Paracetamol/ibuprofen: For comfort - but do NOT prevent febrile seizures

Non-pharmacological

• Parental education: Reassure about excellent prognosis; explain what to do if seizure recurs (place on side, time seizure, call ambulance if >5 min)
• No prophylactic anticonvulsants: NOT recommended for simple febrile seizures
• Emergency medication plan: Consider buccal midazolam for parents of children with recurrent prolonged febrile seizures

Referral Criteria

• Complex febrile seizure - paediatric assessment
• Recurrent febrile seizures (>3) - paediatric neurology consideration
• Developmental concerns - community paediatrics
• Febrile status epilepticus (>30 min) - neurology follow-up

• Recurrence: ~30% after first; ~50% after second; highest risk if <18 months at first seizure

• Neurodevelopmental outcome: NO adverse effect on cognition, behaviour, or academic performance
• Duration: Most last <5 minutes; self-limiting
• Mortality: Essentially zero from the seizure itself

Simple vs Complex Febrile Seizure

Recurrence Risk Factors

Key Facts

Childhood epilepsy is a heterogeneous group of conditions with varying prognoses. Accurate classification is essential as it determines treatment choice and prognosis. Many childhood epilepsy syndromes have excellent outcomes with appropriate treatment.

Epidemiology

Epilepsy affects approximately 0.5-1% of children. Incidence is highest in the first year of life. ~60% of childhood epilepsy is well controlled with monotherapy. ~30% have drug-resistant epilepsy. ~50% of childhood epilepsy achieves sustained remission by adulthood.

Aetiology

• Genetic: Idiopathic generalised epilepsies (CAE, JME, JAE), SCN1A mutations (Dravet syndrome)
• Structural: Cortical dysplasia, hippocampal sclerosis, tuberous sclerosis, brain tumour, previous stroke/injury
• Metabolic: Pyridoxine deficiency, glucose transporter deficiency (GLUT-1)
• Immune: Autoimmune encephalitis (NMDA-receptor)
• Infectious: Post-meningitis/encephalitis

Pathophysiology

Seizures result from abnormal, excessive, synchronous neuronal activity. In focal epilepsy, this arises from a localised cortical region. In generalised epilepsy, corticothalamic circuits generate bilateral synchronous discharges. The balance between excitatory (glutamate) and inhibitory (GABA) neurotransmission is disrupted.

Common Childhood Epilepsy Syndromes

Childhood Absence Epilepsy (5-7yr):

• Brief (5-15 sec) blank spells with eye fluttering
• EEG: 3Hz generalised spike-wave
• Excellent prognosis; ~70% remit by adolescence

Juvenile Myoclonic Epilepsy (12-18yr):

• Morning myoclonic jerks + absence seizures + GTC seizures
• EEG: 4-6Hz polyspike-wave
• Lifelong treatment usually required

Benign Epilepsy with Centrotemporal Spikes (BECTS/Rolandic, 3-13yr):

• Nocturnal focal seizures with face/tongue involvement, drooling
• EEG: Centrotemporal spikes
• Almost all remit by mid-adolescence; may not need treatment

West Syndrome (3-12 months):

• Infantile spasms (clusters of flexor spasms on waking)
• EEG: Hypsarrhythmia
• Treatment: Vigabatrin (if tuberous sclerosis) or prednisolone/tetracosactide

Red Flags

• Onset <1 year - higher likelihood of structural/genetic/metabolic cause
• Developmental regression with seizures - neurodegenerative disease
• Refractory seizures - consider surgical evaluation
• Infantile spasms - urgent treatment to preserve neurodevelopment

Bedside

• Detailed seizure history: Description from witness, video if available, precipitants, duration, postictal state
• Developmental assessment: Regression or delay

Bloods

• Blood glucose, calcium, magnesium, U&Es: First seizure workup
• FBC, LFTs: Baseline before starting AEDs
• Genetic testing: If epilepsy syndrome suggests genetic cause

Imaging

• MRI brain: Indicated for focal seizures, onset <2 years, developmental concerns, abnormal neurology; NOT routinely needed for typical generalised epilepsy syndromes

Special Tests

• EEG: Supports diagnosis and syndrome classification - interictal EEG may be normal (normal EEG does not exclude epilepsy)
• Sleep-deprived EEG: Increases sensitivity
• Video-EEG: For diagnostic uncertainty or presurgical evaluation
• Genetic testing: Epilepsy gene panel, chromosomal microarray - increasingly important for diagnosis and treatment selection

Non-pharmacological

• Seizure first aid education: Recovery position, timing, when to call ambulance (>5 min)
• Emergency medication plan: Buccal midazolam prescription and training for parents/school
• Lifestyle: Regular sleep, avoid known triggers; swimming with supervision; driving regulations (DVLA - 1 year seizure-free for driving)
• EHCP: Educational support if needed

Pharmacological (NICE NG217)

Focal seizures:

• First-line: Carbamazepine or lamotrigine
• Second-line: Levetiracetam, oxcarbazepine, sodium valproate

Generalised tonic-clonic:

• First-line: Sodium valproate (males) or lamotrigine (females of childbearing potential)
• Second-line: Levetiracetam, clobazam

Absence seizures:

• First-line: Ethosuximide or sodium valproate
• Second-line: Lamotrigine

Myoclonic seizures:

• First-line: Sodium valproate (males) or levetiracetam
• AVOID carbamazepine (worsens myoclonic/absence seizures)

Infantile spasms:

• Vigabatrin (if tuberous sclerosis) or prednisolone/tetracosactide

Surgical/Interventional

• Epilepsy surgery: For drug-resistant focal epilepsy with identifiable lesion - resective surgery; ~60-70% seizure-free
• Vagus nerve stimulation (VNS): Adjunctive for drug-resistant epilepsy
• Ketogenic diet: Evidence-based for drug-resistant epilepsy; particularly effective in GLUT-1 deficiency and some genetic epilepsies

Referral Criteria

• All first seizures - paediatric assessment
• Drug-resistant epilepsy (failure of 2 appropriate AEDs) - tertiary epilepsy centre
• Infantile spasms - urgent paediatric neurology
• Consideration of epilepsy surgery - specialist epilepsy centre

• ~60% achieve seizure freedom with first AED
• ~50% achieve sustained remission by adulthood
• BECTS: Almost 100% remission by mid-adolescence
• CAE: ~70% remit by adolescence
• JME: Lifelong treatment usually required; well controlled in most
• Drug-resistant epilepsy: ~30%; some benefit from surgery
• SUDEP: Risk ~1 per 1,000 patient-years; higher with uncontrolled GTC seizures

AED Choice by Seizure Type (NICE NG217)

Status Epilepticus Protocol (APLS)

Key Facts

Bacterial meningitis remains one of the most important emergencies in paediatrics. Despite declining incidence due to vaccination, it carries significant mortality and morbidity. Rapid recognition and treatment are the key determinants of outcome.

Epidemiology

Bacterial meningitis incidence in UK children has declined significantly since MenC, Hib, PCV, and MenB vaccination. Current incidence: ~5-10 per 100,000 children. MenB remains the most common cause of meningococcal disease in the UK (despite MenB vaccine). Peak incidence: <1 year and 15-19 years.

Aetiology

By age:

• Neonates: GBS, E. coli, Listeria
• 1 month-5 years: N. meningitidis, S. pneumoniae, Hib (rare post-vaccine)
• >5 years: N. meningitidis, S. pneumoniae

Viral meningitis (much more common than bacterial): Enterovirus (most common), HSV-2, mumps, parechovirus.

Pathophysiology

Bacteria reach the meninges via haematogenous spread (most common), direct extension (skull fracture, sinusitis), or ascending infection. In the subarachnoid space, bacteria multiply rapidly due to low levels of complement and immunoglobulins in CSF. The inflammatory response (cytokines, leukocyte infiltration) causes cerebral oedema, increased ICP, reduced cerebral blood flow, and neuronal injury.

Infants (<1 year)

• Non-specific: Irritability, lethargy, poor feeding, vomiting, fever or hypothermia
• Bulging fontanelle (late sign)
• High-pitched cry
• Neck stiffness often ABSENT
• Seizures

Older Children

• Fever, headache, photophobia
• Neck stiffness (Kernig's sign, Brudzinski's sign)
• Altered consciousness
• Vomiting, irritability
• Non-blanching rash (meningococcal - petechial → purpuric)

Meningococcal Septicaemia

• Petechial/purpuric rash - non-blanching
• Rapid deterioration - septic shock
• DIC - bleeding, purpura fulminans
• May occur WITH or WITHOUT meningitis

Red Flags

• Non-blanching rash with fever - give antibiotics IMMEDIATELY (do NOT wait for LP or results)
• Altered consciousness (GCS <12) - raised ICP; CT before LP
• Seizures - increased morbidity
• Rapid pulse rate, prolonged CRT, cool peripheries - septic shock
• Signs of raised ICP - papilloedema, altered consciousness, Cushing's response

Bedside

• Blood glucose: Before LP (compare with CSF glucose)
• Observations: Sepsis screening - HR, RR, BP, CRT, temperature, GCS

Bloods

• Blood culture: Before antibiotics (but do NOT delay antibiotics)
• FBC, CRP, procalcitonin: Inflammatory markers
• Coagulation: DIC screen
• U&Es: SIADH (hyponatraemia) common in meningitis
• Blood gas: Lactate, pH
• Meningococcal PCR: If blood culture negative

LP (CSF Analysis)

• Contraindications to LP: Signs of raised ICP (CT first), haemodynamic instability, coagulopathy, local infection
• CSF findings:

Imaging

• CT head: Before LP if signs of raised ICP, focal neurology, or GCS <9
• MRI brain: If complications suspected (abscess, subdural empyema, venous sinus thrombosis)

Immediate

• Do NOT delay antibiotics for LP or imaging if bacterial meningitis suspected
• IV ceftriaxone 80mg/kg (max 4g) - start immediately
• Add IV amoxicillin if <3 months (Listeria cover)
• IV dexamethasone 0.15mg/kg QDS: Give before or with first antibiotic dose; continue for 4 days if bacterial meningitis confirmed

Supportive

• Fluid management: Avoid fluid overload (SIADH risk); maintenance fluids initially; reassess frequently
• Seizure management: Buccal midazolam or IV lorazepam per APLS protocol
• Monitoring: Neuro obs, fluid balance, electrolytes, GCS
• PICU: For septic shock, raised ICP, respiratory failure

Duration of Antibiotics

• Meningococcal: 7 days
• Pneumococcal: 14 days
• GBS (neonatal): 14-21 days
• Gram-negative (neonatal): 21 days

Public Health

• Notify: All cases of meningococcal disease to PHE
• Chemoprophylaxis: Ciprofloxacin (adults) or rifampicin (children) for household and kissing contacts of meningococcal disease

Referral Criteria

• All suspected bacterial meningitis - admit, antibiotics immediately
• Septic shock - PICU
• Complications (subdural empyema, hydrocephalus, brain abscess) - neurosurgery

• Bacterial meningitis mortality: ~5-10% in children (higher in neonates and meningococcal septicaemia)
• Long-term sequelae: ~25% - hearing loss (most common), neurodevelopmental impairment, epilepsy, hydrocephalus, limb amputation (meningococcal)
• Dexamethasone: Reduces hearing loss (NNT 14 for pneumococcal meningitis)
• Viral meningitis: Excellent prognosis; full recovery expected
• Post-discharge: Audiology within 4 weeks; neurodevelopmental follow-up

CSF Analysis Summary

Contact Prophylaxis for Meningococcal Disease

Key Facts

Encephalitis is a neurological emergency. HSV encephalitis is the most important treatable cause and empirical aciclovir must be started at the earliest suspicion. Autoimmune encephalitis is increasingly recognised and requires immunotherapy.

Epidemiology

Encephalitis incidence: approximately 2-8 per 100,000 children per year in the UK. HSV encephalitis accounts for ~10% of cases but is the most common identifiable cause. Enterovirus is the most common overall viral cause. Autoimmune encephalitis (particularly anti-NMDA receptor) is now recognised as equally or more common than infectious causes.

Aetiology

Infectious:

• HSV-1 (most common treatable cause; temporal lobe predilection)
• Enterovirus, parechovirus (neonates)
• VZV, EBV, CMV, influenza, adenovirus
• Mycoplasma pneumoniae (post-infectious encephalitis)

Autoimmune:

• Anti-NMDA receptor encephalitis (most common autoimmune; may follow HSV encephalitis)
• Acute disseminated encephalomyelitis (ADEM) - post-infectious demyelination

Pathophysiology

In viral encephalitis, the virus directly invades brain parenchyma causing neuronal damage, inflammation, and oedema. HSV-1 has a tropism for the temporal lobes (via olfactory or trigeminal nerve). In autoimmune encephalitis, antibodies target neuronal surface antigens (NMDA, LGI1, CASPR2), causing neuronal dysfunction through receptor internalisation. ADEM involves perivenous demyelination triggered by molecular mimicry.

Clinical Features

• Behavioural change/personality change
• Seizures (often focal/temporal in HSV)
• Fever (may be absent in autoimmune)
• Focal neurological signs (dysphasia, hemiparesis)
• Movement disorder (anti-NMDA receptor encephalitis - orofacial dyskinesia)

HSV Encephalitis

• Fever + altered consciousness + temporal seizures
• Rapid deterioration over days
• Temporal lobe changes on MRI

Anti-NMDA Receptor Encephalitis

• Psychiatric symptoms (psychosis, catatonia)
• Seizures, movement disorder
• Autonomic instability
• More common in females; may be associated with ovarian teratoma (rare in children)

Red Flags

• Rapidly declining consciousness - urgent CT, consider raised ICP
• Refractory seizures - may need PICU and continuous EEG
• Focal neurological signs - structural lesion (abscess, tumour) must be excluded
• Post-HSV autoimmune encephalitis - movement disorder developing 2-6 weeks after HSV encephalitis; treat with immunotherapy

Bedside

• GCS: Serial monitoring
• Blood glucose: Exclude hypoglycaemia

Bloods

• FBC, CRP, U&Es, LFTs: Baseline; SIADH screening
• Blood cultures: Exclude sepsis
• Autoimmune panel: NMDA-R antibodies, VGKC, other neuronal antibodies
• Mycoplasma serology/PCR: If suspected

CSF

• PCR: HSV-1/2, enterovirus, VZV, parechovirus
• Cell count: Lymphocytic pleocytosis (may be normal early)
• Protein/glucose: Mildly raised protein, normal glucose (viral)
• NMDA-R antibodies: In CSF - more sensitive than serum

Imaging

• MRI brain: Investigation of choice - temporal lobe signal change (HSV), multifocal white matter lesions (ADEM), normal or subtle changes (autoimmune)
• CT head: If MRI not immediately available; to exclude raised ICP before LP

Special Tests

• EEG: May show PLEDs in HSV; continuous EEG for refractory seizures
• Pelvic/abdominal imaging: USS/MRI for ovarian teratoma in anti-NMDA receptor encephalitis

Pharmacological

• IV aciclovir: 500mg/m² TDS (children >3 months) or 20mg/kg TDS (neonates) - start immediately; continue for 14-21 days if HSV confirmed; minimum 14 days

• Autoimmune encephalitis: IV methylprednisolone 30mg/kg (max 1g) for 3-5 days; IVIG 2g/kg over 5 days; plasma exchange; rituximab for refractory cases
• ADEM: IV methylprednisolone 30mg/kg for 3-5 days; IVIG if steroid-refractory

Non-pharmacological

• PICU: For GCS ≤8, refractory seizures, respiratory failure
• Raised ICP management: Head elevation, mannitol/hypertonic saline, neurosurgical consultation
• Rehabilitation: Early multidisciplinary input - physiotherapy, OT, SALT, neuropsychology

Referral Criteria

• All suspected encephalitis - urgent paediatric neurology and infectious diseases
• GCS ≤8 - PICU
• Refractory seizures - PICU, continuous EEG
• Suspected autoimmune encephalitis - paediatric neurology, immunology

• HSV encephalitis with treatment: Mortality ~20%; ~50-60% survivors have neurocognitive sequelae
• HSV encephalitis without treatment: Mortality ~70%
• Autoimmune (anti-NMDA receptor): ~80% good outcome with immunotherapy; may relapse
• ADEM: Generally monophasic with good recovery; ~20% have residual deficits
• Viral (non-HSV): Generally good prognosis; enteroviral usually self-limiting
• Post-HSV autoimmune encephalitis: Increasingly recognised; responds to immunotherapy

Encephalitis Investigation Priority

HSV vs Autoimmune Encephalitis

Key Facts

Kawasaki disease is the leading cause of acquired heart disease in children in developed countries. It is a clinical diagnosis with no confirmatory test. Early treatment with IVIG has dramatically reduced the risk of coronary artery complications.

Epidemiology

Incidence in the UK: approximately 8-9 per 100,000 children <5 years. Much higher in Japan (~300 per 100,000 <5 years). Peak age: 6 months to 5 years. Male:female ratio 1.5:1. Slight seasonal variation (winter/spring).

Aetiology

• Unknown: Likely an abnormal immune response to one or more infectious triggers in genetically susceptible children
• NOT directly infectious but has seasonal and geographic clustering
• Genetic susceptibility factors identified (ITPKC, CASP3 polymorphisms - particularly in Japanese populations)

Pathophysiology

Kawasaki disease is a systemic vasculitis predominantly affecting medium-sized arteries (especially coronary arteries). The acute phase involves neutrophilic infiltration of arterial walls, endothelial damage, and disruption of the internal elastic lamina. This leads to aneurysm formation, particularly at bifurcation points of the coronary arteries. The coronary artery inflammation can cause stenosis, thrombosis, and myocardial infarction.

Diagnostic Criteria (Fever + 4/5 Features)

CRASH mnemonic:

• Conjunctivitis: Bilateral, non-purulent, limbal sparing
• Rash: Polymorphous (maculopapular, erythema multiforme-like, diffuse erythema); NOT vesicular
• Adenopathy: Cervical lymphadenopathy >1.5cm (often unilateral - least common feature)
• Strawberry tongue + lip changes: Red, cracked lips; strawberry tongue; oropharyngeal erythema
• Hand/foot changes: Erythema and oedema acutely; periungual desquamation (peeling) in convalescent phase (weeks 2-3)

Other Features

• Extreme irritability (very characteristic)
• Perianal erythema/desquamation
• BCG site inflammation
• Arthritis, aseptic meningitis, hepatitis, hydrops of gallbladder

Red Flags

• Coronary artery dilatation on echo - treat urgently
• Shock/haemodynamic instability - Kawasaki shock syndrome (10%)
• IVIG-resistant (persistent fever >36h post-IVIG) - second dose IVIG or infliximab
• Incomplete Kawasaki - diagnose and treat even with <4 features if supporting evidence

Bloods

• FBC: Leucocytosis, thrombocytosis (peaks week 2-3 - very characteristic), anaemia
• CRP and ESR: Markedly elevated (CRP >100, ESR >40 supports diagnosis)
• LFTs: Raised transaminases, hypoalbuminaemia
• U&Es: Sterile pyuria (urine WCC from urethral inflammation)
• Blood cultures: Negative (exclude bacterial infection)

Imaging

• Echocardiography: At diagnosis, 2 weeks, and 6-8 weeks - assess coronary artery dimensions (Z-scores), LV function, pericardial effusion, valve regurgitation

Special Tests

• Urine microscopy: Sterile pyuria
• ECG: Prolonged PR, ST changes, arrhythmia (rare)
• CT/MR coronary angiography: For follow-up of known aneurysms in older children

Pharmacological

• IVIG 2g/kg IV over 10-12 hours - single infusion; give within 10 days of fever onset
• Aspirin high-dose: 30-50mg/kg/day in 4 divided doses during acute febrile phase
• Aspirin low-dose: 3-5mg/kg/day after defervescence; continue for 6-8 weeks (or lifelong if aneurysm)

IVIG-Resistant (Fever >36h Post-IVIG)

• Second dose IVIG 2g/kg
• IV methylprednisolone: 30mg/kg for 3 days
• Infliximab 5mg/kg: Single dose (anti-TNF-α)

Coronary Aneurysm Management

• Small/medium aneurysm: Low-dose aspirin ± clopidogrel; serial echo
• Giant aneurysm (>8mm or Z-score >10): Aspirin + warfarin/LMWH; cardiology follow-up; may need coronary intervention

Non-pharmacological

• Rest: During acute phase
• Avoid live vaccines: For 11 months after IVIG (immunoglobulin interferes with vaccine response)

Referral Criteria

• All suspected Kawasaki - paediatric assessment and echo within 24h
• Coronary artery abnormalities - paediatric cardiology
• IVIG-resistant - discuss with tertiary centre

• Without treatment: ~25% develop coronary artery aneurysms
• With IVIG within 10 days: <5% develop coronary artery aneurysms
• Giant aneurysms: Risk of thrombosis, stenosis, MI; lifelong cardiology follow-up
• Coronary regression: ~50% of small-medium aneurysms regress within 1-2 years
• Mortality: <0.1% in developed countries with treatment
• Long-term: Children with coronary involvement need lifelong cardiovascular follow-up; may develop premature atherosclerosis

Kawasaki Disease Treatment Timeline

Coronary Artery Classification (Z-Scores)

Key Facts

HSP is the most common childhood vasculitis and is usually a benign, self-limiting condition. The most important complication is renal disease, which requires monitoring for up to 12 months after the acute episode.

Epidemiology

Incidence: approximately 20 per 100,000 children per year. Peak age: 3-10 years. Male:female ratio 1.5:1. More common in autumn/winter. Rare in adults (but when it occurs, renal involvement is more severe).

Aetiology

• Trigger: Preceding URTI in ~50-75% of cases
• Infections: Group A Streptococcus, adenovirus, parvovirus B19, Mycoplasma
• Other triggers: Drugs, insect bites, food allergens
• Pathogenesis: IgA immune complex deposition in small vessel walls

Pathophysiology

HSP is an IgA-mediated small vessel vasculitis (leucocytoclastic vasculitis). IgA1 immune complexes deposit in the walls of small blood vessels (skin, joints, gut, kidneys), causing complement activation, neutrophil infiltration, and vessel wall damage. In the kidneys, IgA deposition in the glomerular mesangium causes a proliferative glomerulonephritis identical to IgA nephropathy.

Classic Tetrad

1. Purpura (100%): Palpable, non-thrombocytopenic purpura on buttocks and lower limbs (extensor surfaces); may also affect arms and trunk
2. Arthralgia/arthritis (75%): Knees and ankles most commonly; periarticular swelling; non-deforming
3. Abdominal pain (65%): Colicky; may be severe; GI bleeding (melaena, haematemesis) in 20-30%
4. Renal (40%): Microscopic haematuria, proteinuria; macroscopic haematuria in ~5%; nephrotic syndrome rarely

Other Features

• Scrotal swelling/pain (~15% of boys - may mimic testicular torsion)
• Subcutaneous oedema (face, hands, feet - especially in young children)
• Rarely: CNS involvement (headache, seizures, altered consciousness)

Red Flags

• Heavy proteinuria (uPCR >200) - risk of nephrotic syndrome/renal impairment
• Hypertension - renal involvement
• Severe abdominal pain with bloody stool - consider intussusception (ileo-ileal, not the ileocolic type seen in typical intussusception)
• Scrotal pain - exclude testicular torsion
• Relapse with worsening renal function - nephrology referral

Bedside

• Urine dipstick: Haematuria, proteinuria - at diagnosis and weekly for 4 weeks, then monthly for 6-12 months
• Blood pressure: Monitor throughout

Bloods

• FBC: Platelets NORMAL (distinguishes from ITP)
• U&Es: Renal function
• CRP/ESR: May be mildly elevated
• IgA: Elevated in ~50% (supports diagnosis but not diagnostic)
• Coagulation: Normal
• ASOT/throat swab: If preceding streptococcal infection suspected

Imaging

• USS abdomen: If severe abdominal pain - exclude intussusception
• Doppler USS testes: If scrotal swelling - exclude torsion

Special Tests

• Urine protein:creatinine ratio (uPCR): Quantify proteinuria if dipstick positive
• Renal biopsy: Only if progressive renal impairment, heavy proteinuria (uPCR >200), or nephrotic syndrome - IgA mesangial deposits
• Skin biopsy: Rarely needed - leucocytoclastic vasculitis with IgA deposits

Non-pharmacological

• Supportive care: Rest, adequate hydration, elevation of affected limbs
• Monitoring: Weekly urine dipstick and BP for 4 weeks; then monthly for 6-12 months

Pharmacological

• Analgesia: Paracetamol 15mg/kg QDS + ibuprofen 10mg/kg TDS (if no renal involvement)
• Prednisolone 1-2mg/kg/day (max 40mg): Consider for severe abdominal pain or scrotal pain; does NOT prevent nephritis
• ACEi (ramipril): If significant proteinuria - reduces proteinuria and slows renal progression

Renal Involvement Management

• Mild (microscopic haematuria/mild proteinuria): Monitor; usually resolves
• Moderate (heavy proteinuria/macroscopic haematuria): Nephrology referral; ACEi
• Severe (nephrotic syndrome/declining renal function): Immunosuppression (cyclophosphamide, mycophenolate) - guided by renal biopsy

Referral Criteria

• Heavy proteinuria (uPCR >200) - paediatric nephrology
• Declining renal function - nephrology urgently
• Hypertension - nephrology
• Severe abdominal pain not responding to steroids - surgical review (intussusception)
• Scrotal swelling - urology if torsion cannot be excluded

• Self-limiting: ~95% resolve within 4-6 weeks
• Relapse: ~30% have at least one relapse (usually milder)
• Renal involvement: ~40% have haematuria/proteinuria; <5% progress to significant renal disease; <1% develop ESRD
• Long-term renal follow-up: Monitor for 12 months; late-onset nephritis can occur weeks to months after initial presentation
• Pregnancy: Women with history of HSP nephritis have increased risk of hypertension and proteinuria in pregnancy

HSP Monitoring Schedule

EULAR/PRINTO/PRES Classification Criteria

Key Facts

UTI in children is important because of the risk of renal scarring, particularly in young children with vesicoureteral reflux. Prompt diagnosis and treatment, followed by appropriate imaging, reduce the risk of long-term renal damage.

Epidemiology

UTI is one of the most common bacterial infections in children. By age 7: ~8% of girls and ~2% of boys have had at least one UTI. Recurrence rate: ~30% in first year. UTI is the most common cause of fever without source in infants <2 years.

Aetiology

• E. coli: ~80% (commonest at all ages)
• Proteus mirabilis: More common in boys (associated with phimosis/foreskin)
• Klebsiella, Enterococcus, Pseudomonas: Less common; consider if atypical or recurrent
• Risk factors: Female sex, constipation, dysfunctional voiding, VUR, posterior urethral valves (boys), neuropathic bladder, immunosuppression

Pathophysiology

Most childhood UTIs result from ascending infection from the perineum via the urethra to the bladder (cystitis) and potentially to the kidneys (pyelonephritis). VUR allows retrograde flow of urine from the bladder into the ureters/renal pelvis, increasing the risk of pyelonephritis and renal scarring. Renal scarring results from the inflammatory response to infection in the renal parenchyma, particularly in young children with immature kidneys.

Infants (<3 Months)

• Non-specific: Fever, irritability, poor feeding, vomiting, jaundice, failure to thrive
• Sepsis presentation possible
• NO localising urinary symptoms

Infants and Toddlers (3 Months-3 Years)

• Fever (may be the only symptom)
• Irritability, vomiting, poor feeding
• Offensive/cloudy urine
• Abdominal pain

Older Children (>3 Years)

• Dysuria, frequency, urgency
• Abdominal or loin pain
• Enuresis (new onset in previously dry child)
• Fever, vomiting (suggests pyelonephritis)

Red Flags

• Infant <3 months with UTI - admit for IV antibiotics
• Sepsis features (poor perfusion, altered consciousness) - treat as sepsis
• Poor urine flow or palpable bladder - consider posterior urethral valves (boys)
• Raised creatinine - investigate for obstructive uropathy
• Non-E. coli organism - suggests structural abnormality

Urine Collection

• Clean catch: Preferred method - first-line for all ages
• Suprapubic aspiration (SPA): Gold standard in infants <3 months
• In-out catheter: Alternative to SPA
• Bag specimen: High contamination rate (~60%) - only use for dipstick screening; NOT for culture

Urine Analysis

• Dipstick: Nitrites (specific) and leucocyte esterase (sensitive); both positive - treat as UTI
• Microscopy: WCC (pyuria) and bacteria
• Culture: Gold standard - >10⁵ CFU/mL single organism (clean catch); any growth from SPA is significant

Bloods

• FBC, CRP, U&Es: If pyelonephritis or sepsis suspected
• Blood culture: If sepsis suspected

Imaging (NICE CG54)

Responds to treatment within 48h, typical UTI, age >6 months:

• No imaging needed for first UTI

Atypical UTI (any age):

• USS within 6 weeks; DMSA at 4-6 months

Recurrent UTI:

• USS within 6 weeks; DMSA at 4-6 months; MCUG if <6 months or dilated urinary tract or poor urine flow

Age <6 months with first UTI:

• USS within 6 weeks; DMSA at 4-6 months (if atypical or recurrent → add MCUG)

Pharmacological

Lower UTI (cystitis):

• Trimethoprim 4mg/kg BD for 3 days (first-line) OR nitrofurantoin 1mg/kg QDS OR cefalexin 12.5mg/kg BD
• Adjust per local antibiogram

Upper UTI (pyelonephritis/febrile UTI):

• Oral: Cefalexin 12.5mg/kg BD for 7-10 days (or co-amoxiclav)
• IV (if <3 months, sepsis, vomiting): Ceftriaxone 80mg/kg OD or co-amoxiclav 30mg/kg TDS for 2-4 days → switch to oral when improving

Prophylactic antibiotics:

• Consider for recurrent UTI or significant VUR: Trimethoprim 2mg/kg ON or nitrofurantoin 1mg/kg ON

Non-pharmacological

• Adequate fluid intake: Encourage regular drinking
• Regular voiding: Timed voiding, double voiding
• Treat constipation: Very commonly contributes to UTI recurrence
• Hygiene: Front-to-back wiping in girls

Referral Criteria

• Atypical UTI - paediatric assessment + imaging
• Recurrent UTI (≥2) - paediatric assessment + imaging
• Abnormal imaging - paediatric nephrology/urology
• Infant <3 months - admission for IV antibiotics
• Suspected structural abnormality (poor flow, palpable bladder) - paediatric urology

• Uncomplicated UTI: Full resolution with appropriate antibiotics
• Recurrence: ~30% in first year after initial UTI
• Renal scarring: ~5-15% of children with febrile UTI; higher with VUR, delayed treatment, recurrent infections
• VUR: Grades I-III often resolve spontaneously; grades IV-V may require surgery
• Long-term: Renal scarring can lead to hypertension, proteinuria, and CKD; important cause of paediatric hypertension

NICE CG54 Imaging Guide

Atypical UTI Definition (NICE)

Key Facts

Vesicoureteral reflux is the most common urological anomaly in children. It predisposes to pyelonephritis through the retrograde transport of infected urine to the kidneys. Early detection and management aim to prevent renal scarring and long-term complications such as hypertension and chronic kidney disease.

Epidemiology

VUR has a prevalence of approximately 1-2% in the general paediatric population. It is identified in 30-40% of children investigated after a UTI. There is a strong familial tendency - 30-50% of siblings and 66% of offspring of affected parents also have VUR. It is more common in Caucasian children and shows a female predominance for clinical presentation (male predominance in antenatally detected cases).

Aetiology

Primary VUR: Congenital deficiency of the submucosal tunnel of the ureterovesical junction. The normal ratio of tunnel length to ureteric diameter is 5:1; this is reduced in VUR.

Secondary VUR: Due to elevated intravesical pressure - posterior urethral valves, neuropathic bladder, bladder outlet obstruction, dysfunctional voiding.

Pathophysiology

The ureterovesical junction normally acts as a one-way valve. During bladder filling and voiding, the intramural ureter is compressed against the detrusor muscle, preventing reflux. When this mechanism is deficient, urine refluxes retrogradely. If urine is infected, bacteria reach the renal parenchyma causing pyelonephritis. Repeated pyelonephritis leads to renal scarring (reflux nephropathy), characterised by cortical thinning and calyceal clubbing, particularly at the renal poles.

Antenatal Detection

• Antenatal hydronephrosis identified on routine ultrasound
• More common in male infants

UTI Presentation

• Fever, vomiting, poor feeding in infants
• Dysuria, frequency, abdominal/loin pain in older children
• Offensive/cloudy urine
• Failure to thrive in recurrent cases

Complications

• Recurrent febrile UTIs
• Renal scarring - may be asymptomatic initially
• Hypertension (detected on routine monitoring)
• Proteinuria (indicating renal damage)

Red Flags

• Recurrent febrile UTIs in any child - investigate for underlying urological abnormality
• Hypertension in a child with known VUR - suggests renal scarring
• Poor growth or chronic kidney disease features
• Bilateral high-grade reflux with impaired renal function

Bedside

• Urine dipstick and MC&S: Confirm UTI diagnosis
• Blood pressure: Screen for hypertension secondary to renal scarring
• Growth assessment: Plot height and weight on centile charts

Bloods

• U&Es, creatinine: Assess renal function
• FBC, CRP: During acute infection episodes
• Urine protein:creatinine ratio: Screen for proteinuria indicating renal damage

Imaging

• Renal ultrasound: First-line - assess kidney size, hydronephrosis, cortical thinning; does not reliably detect VUR
• MCUG (micturating cystourethrogram): Gold standard for diagnosing and grading VUR (grades I-V). NICE CG54 recommends in infants <6 months with atypical/recurrent UTI
• DMSA scan: Static renal scan to detect cortical scarring - perform 4-6 months after acute infection
• MAG3 renogram: Assess differential renal function and drainage

Special Tests

• Indirect radionuclide cystography: Lower radiation alternative to MCUG for follow-up
• Urodynamic studies: If secondary VUR suspected (neuropathic bladder, dysfunctional voiding)

Non-pharmacological

• Ensure adequate fluid intake and regular voiding
• Manage constipation (commonly coexists and worsens VUR)
• Treat bladder-bowel dysfunction with behavioural strategies
• Parental education on UTI recognition and prompt treatment

Pharmacological

• Antibiotic prophylaxis: Trimethoprim 1-2mg/kg nocte or nitrofurantoin 1mg/kg nocte - consider in recurrent UTI or high-grade VUR (RIVUR trial showed 50% reduction in recurrent UTI)
• Prompt treatment of UTIs: Cefalexin 12.5mg/kg BD for 7-10 days (oral) or IV ceftriaxone 80mg/kg OD for pyelonephritis
• Anticholinergics: Oxybutynin 0.2mg/kg BD-TDS if overactive bladder contributes

Surgical/Interventional

• Endoscopic subureteric injection (Deflux/dextranomer-hyaluronic acid): Day case procedure; 70-80% success rate for grade III-IV
• Ureteric reimplantation (Cohen or Politano-Leadbetter): >95% success rate; reserved for high-grade VUR, failed endoscopic treatment, or breakthrough infections on prophylaxis

Referral Criteria

• All children with confirmed VUR - paediatric urology/nephrology
• Recurrent febrile UTIs despite prophylaxis
• Evidence of new renal scarring on DMSA
• Hypertension or declining renal function

• Grades I-II: Spontaneous resolution in approximately 80% by age 5 years
• Grade III: Resolution in approximately 50% by age 5
• Grades IV-V: Spontaneous resolution uncommon (<20%); higher risk of renal scarring
• Renal scarring: Occurs in 10-40% of children with VUR; risk increases with delayed treatment of UTIs
• Reflux nephropathy: Accounts for 5-10% of children reaching end-stage renal disease
• Bilateral high-grade VUR: Greater risk of CKD - long-term renal function monitoring essential

VUR Grading (International Classification)

NICE CG54 Imaging Recommendations for UTI in Children

Key Facts

Childhood nephrotic syndrome results from increased glomerular permeability to protein, leading to massive proteinuria. Minimal change disease is the predominant cause in young children and typically responds well to corticosteroids.

Epidemiology

Annual incidence is approximately 2-7 per 100,000 children under 16 in the UK. Peak age of onset is 2-5 years. Male:female ratio is approximately 2:1 in younger children, equalising in adolescence. Higher incidence in South Asian and Afro-Caribbean populations.

Aetiology

• Minimal change disease (MCD): ~80% in ages 1-10; idiopathic; probable T-cell-mediated circulating permeability factor
• Focal segmental glomerulosclerosis (FSGS): ~10-15%; more common in Afro-Caribbean children; often steroid-resistant
• Membranous nephropathy: Rare in children (~5%); consider secondary causes (hepatitis B, SLE)
• Membranoproliferative GN: Rare; low complement levels
• Congenital nephrotic syndrome: Presents <3 months; Finnish type (NPHS1 mutation) most common

Pathophysiology

In MCD, electron microscopy reveals diffuse podocyte foot process effacement with normal light microscopy. A circulating permeability factor (possibly produced by dysregulated T cells) increases glomerular capillary wall permeability. Massive urinary protein loss leads to hypoalbuminaemia, reduced oncotic pressure, and oedema. The liver increases lipoprotein synthesis in response to low oncotic pressure, causing hyperlipidaemia. Loss of antithrombin III and other anticoagulant proteins creates a hypercoagulable state.

Classic Presentation

• Periorbital oedema (often first sign, worse in morning)
• Generalised oedema (ascites, scrotal/labial oedema, pleural effusions)
• Weight gain
• Frothy urine
• Reduced urine output

Complications

• Infection: Spontaneous bacterial peritonitis (Streptococcus pneumoniae), cellulitis, sepsis - due to urinary immunoglobulin loss
• Thromboembolism: Renal vein thrombosis, PE, cerebral venous sinus thrombosis
• Hypovolaemia/shock: Intravascular volume depletion despite oedema
• Acute kidney injury: Due to hypovolaemia or renal vein thrombosis

Red Flags

• Abdominal pain with fever - exclude spontaneous bacterial peritonitis
• Tachycardia, cool peripheries - hypovolaemic shock despite oedema
• Haematuria, hypertension, low C3 - suggests non-MCD pathology; biopsy indicated
• Age <1 year or >12 years at presentation - lower likelihood of MCD
• Steroid resistance after 4 weeks - refer for renal biopsy

Bedside

• Urine dipstick: 3-4+ protein; check for haematuria
• Blood pressure: Hypertension suggests non-MCD pathology
• Weight: Daily weight monitoring to track fluid balance
• Urine protein:creatinine ratio (uPCR): >200mg/mmol confirms nephrotic-range proteinuria

Bloods

• Serum albumin: <25g/L (often <20g/L in severe cases)
• U&Es: Assess renal function; hyponatraemia common
• FBC: Haemoconcentration; thrombocytosis increases thrombotic risk
• Lipid profile: Hypercholesterolaemia, raised triglycerides
• Complement (C3, C4): Normal in MCD; low in MPGN, lupus nephritis, post-streptococcal GN
• Immunoglobulins: Low IgG (urinary losses); IgM may be raised
• Hepatitis B/C, HIV serology: Exclude secondary causes

Imaging

• Renal ultrasound: Typically normal or enlarged echogenic kidneys; exclude renal vein thrombosis with Doppler
• Chest X-ray: If respiratory compromise - pleural effusions

Special Tests

• Renal biopsy: Not routinely performed in typical steroid-sensitive nephrotic syndrome (age 1-10, no haematuria, normal complement, normal renal function). Indicated for: steroid resistance, atypical features, age <1 or >12 years

Non-pharmacological

• Fluid restriction: Not routinely required unless severe hyponatraemia
• No added salt diet: Helps limit oedema
• Normal protein diet: High protein diets do not help and may worsen proteinuria
• Activity: Avoid immobilisation (thrombotic risk)
• Education: Teach parents home urine dipstick monitoring

Pharmacological

• First episode: Prednisolone 60mg/m²/day (max 80mg) for 4 weeks, then 40mg/m² alternate days (max 60mg) for 4 weeks, then wean (ISKDC regimen; extended courses of 12-16 weeks may reduce relapse - Cochrane evidence)
• Relapse: Prednisolone 60mg/m²/day until remission (3 consecutive days protein-free urine), then 40mg/m² alternate days for 4 weeks
• Frequently relapsing/steroid-dependent: Add steroid-sparing agent - levamisole 2.5mg/kg alternate days, cyclophosphamide 2mg/kg/day for 8-12 weeks, ciclosporin 4-5mg/kg/day in 2 divided doses, mycophenolate mofetil 600mg/m² BD, or rituximab 375mg/m² × 1-2 doses
• Steroid-resistant: Ciclosporin or tacrolimus (KDIGO 2021); consider rituximab
• Albumin infusion: 20% albumin 1g/kg IV over 4-6h for symptomatic hypovolaemia or severe oedema
• Furosemide: 1-2mg/kg IV ONLY with albumin co-infusion (risk of AKI if given alone)
• Penicillin V prophylaxis: 12.5mg/kg BD during oedematous phase (pneumococcal infection risk)

Surgical/Interventional

• Not typically required; paracentesis for tense symptomatic ascites rarely needed

Referral Criteria

• All children with first presentation of nephrotic syndrome - paediatric nephrology
• Steroid resistance - urgent nephrology for biopsy
• Frequently relapsing or steroid-dependent disease - nephrology for steroid-sparing agents

• Steroid-sensitive MCD: Excellent long-term prognosis; rarely progresses to CKD
• Relapse rate: Approximately 70-80% will have at least one relapse; 50% become frequently relapsing
• Frequently relapsing disease: May continue relapsing into adulthood in ~30% of cases
• Steroid-resistant FSGS: Approximately 50% progress to ESRD within 5-10 years
• Mortality: <2% in developed countries; infection and thromboembolism are main causes
• Most children with MCD become relapse-free by late adolescence/early adulthood

Definitions in Childhood Nephrotic Syndrome

Steroid-Sparing Agents Comparison

Key Facts

Congenital heart disease is the most common category of congenital malformation, encompassing a wide spectrum from minor defects requiring no intervention to complex lesions requiring neonatal surgery. Early detection through the NIPE programme and pulse oximetry screening has improved outcomes.

Epidemiology

Incidence is approximately 6-8 per 1,000 live births. Approximately 25% of CHD is critical, requiring intervention in the first year of life. Survival has improved dramatically - over 90% of children with CHD now survive to adulthood. CHD accounts for approximately 3-4% of infant deaths in the UK.

Aetiology

Most CHD is multifactorial (genetic + environmental). Identifiable causes include:

• Chromosomal: Trisomy 21 (AVSD), Turner syndrome (coarctation), trisomy 13/18 (multiple defects)
• Microdeletion: 22q11.2/DiGeorge (interrupted aortic arch, truncus arteriosus, TOF)
• Single gene: Noonan syndrome (pulmonary stenosis), Marfan syndrome (aortic root dilatation)
• Maternal: Diabetes (TGA, VSD), rubella (PDA, pulmonary stenosis), alcohol (VSD, ASD), SLE (heart block), phenytoin, lithium (Ebstein's anomaly)

Pathophysiology

Cyanotic (right-to-left shunts): Deoxygenated blood enters systemic circulation → cyanosis. Two physiological groups:

• Reduced pulmonary blood flow: TOF, pulmonary atresia, tricuspid atresia
• Abnormal mixing/parallel circulations: TGA, TAPVR, truncus arteriosus

Acyanotic Lesions

• Heart failure: Tachypnoea, poor feeding, faltering growth, sweating during feeds, hepatomegaly
• Murmur detected on NIPE or routine examination
• Recurrent lower respiratory tract infections

Cyanotic Lesions

• Central cyanosis not improving with supplemental oxygen (failed hyperoxia test: PaO2 remains <15kPa on 100% O2)
• Tet spells in TOF: Paroxysmal cyanosis, irritability, hyperpnoea - infant draws knees to chest
• Duct-dependent lesions presenting with collapse at day 2-7 when ductus closes

Specific Lesions

• VSD: Pansystolic murmur at left sternal edge; small VSDs louder than large
• ASD: Fixed split S2, ejection systolic murmur at upper left sternal edge
• PDA: Continuous machinery murmur below left clavicle; bounding pulses
• TOF: Ejection systolic murmur (pulmonary stenosis), cyanosis; boot-shaped heart on CXR
• TGA: Cyanosis from birth, single loud S2; egg-on-side cardiac silhouette
• Coarctation: Radio-femoral delay, upper limb hypertension, ejection systolic murmur

Red Flags

• Cyanosis unresponsive to oxygen in a neonate - duct-dependent CHD until proven otherwise
• Collapse at day 2-7 of life - consider duct-dependent lesion
• Absent femoral pulses - coarctation of the aorta
• Hepatomegaly with tachypnoea in infant - heart failure

Bedside

• Pulse oximetry screening: Pre-ductal (right hand) and post-ductal (either foot) saturations; >3% difference suggests duct-dependent lesion or coarctation
• Hyperoxia test: PaO2 after 15 min of 100% O2 - if <15kPa, cyanotic CHD likely
• 4-limb blood pressure: Coarctation causes >20mmHg difference between upper and lower limbs
• ECG: Axis deviation, chamber hypertrophy, rhythm abnormalities

Bloods

• Blood gas: Assess oxygenation, metabolic acidosis (poor cardiac output)
• FBC: Polycythaemia in chronic cyanosis
• Chromosomal analysis/microarray: If dysmorphic features - check for trisomy 21, 22q11.2 deletion

Imaging

• Chest X-ray: Heart size and shape, pulmonary vascularity (plethoric vs oligaemic)
• Echocardiography: Gold standard - defines anatomy, shunt direction, pressure gradients
• Cardiac MRI: For complex anatomy, great vessel assessment, quantification of shunts
• Cardiac catheterisation: Pre-surgical planning, interventional procedures

Special Tests

• CT angiography: Vascular rings, aortic arch anatomy
• Genetic testing: Karyotype, FISH for 22q11.2, microarray

Non-pharmacological

• Feeding support: NG feeds, high-calorie formula for failure to thrive
• Infection prevention: RSV prophylaxis with palivizumab for haemodynamically significant CHD (NICE TA791)
• Endocarditis prophylaxis: Good dental hygiene; antibiotic prophylaxis no longer routinely recommended (NICE CG64) except for high-risk procedures

Pharmacological

• Duct-dependent lesions: Alprostadil (PGE1) 5-20 ng/kg/min IV - maintain ductal patency (side effects: apnoea, fever, hypotension)
• Heart failure: Furosemide 0.5-2mg/kg/dose BD-TDS, captopril 0.1-0.3mg/kg TDS, spironolactone 1-2mg/kg BD
• Tet spells: Knee-to-chest position, oxygen, morphine 0.1mg/kg IV, IV fluids, phenylephrine 5-20mcg/kg IV; propranolol 0.5-1mg/kg QDS for prevention

Surgical/Interventional

• VSD: Surgical patch closure if large/symptomatic; catheter device closure selected cases
• ASD: Catheter device closure (secundum); surgical for primum/sinus venosus
• PDA: Catheter occlusion or surgical ligation; ibuprofen/paracetamol for premature infants
• TOF: Complete repair at 3-6 months (VSD closure + RVOT relief)
• TGA: Arterial switch operation (Jatene) in first 2 weeks; balloon atrial septostomy (Rashkind) as bridge
• Coarctation: Surgical repair or balloon angioplasty ± stenting

Referral Criteria

• Any suspected CHD - urgent paediatric cardiology referral
• Cyanosis unresponsive to O2 - emergency transfer to cardiac centre
• Pathological murmur with symptoms - echocardiography within 2 weeks

• Overall CHD survival: >90% now survive to adulthood in the UK
• VSD: ~80% of small muscular VSDs close spontaneously; large VSDs require surgery with excellent outcomes
• ASD: Life expectancy normal after closure; risk of atrial arrhythmias if repaired late
• TOF: >95% operative survival; most require pulmonary valve replacement in adulthood
• TGA post-arterial switch: >97% operative survival; excellent long-term outcomes
• Eisenmenger syndrome: Once established, 5-year survival approximately 80%; heart-lung transplant only curative option
• Adults with CHD: Growing population - estimated 220,000 adults living with CHD in the UK

Common CHD Lesions and Associations

CXR Appearances