Paeds Flashcards
What is the definition of sepsis?
Generalised inflammatory response, defined by the presence of 2 or greater criteria (abnormal temperature or WCC mist be one of the criteria):
- abnormal core temperature (<36 or >38.5)
- abnormal HR (>2 SD above normal for age, or less than 10th centile for age if child aged <1 years)
- Raised RR (>2 SD above normal for age, or mechanical ventilation for acute lung disease)
- Abnormal WCC in circulating blood (above or below normal range for age, or >10% immature cells)
What are the red-flag signs for sepsis?
Clinically based criteria to diagnose high-risk sepsis –> immediate sepsis 6 pathway
- hypotension
- prolonged cap refil >5 seconds
- O2 needed to maintain sats >92%
- AVPU = V, P, U
- Blood lactate >2mmol/L
- Pale/mottled or non-blanching rash
- RR>60 or >5 below normal or grunting
- Abnormal behaviours (dry nappies, lack of response to social cues, significantly decreased activity, weak, high pitched)
What is sepsis?
SIRS in the presence of infection
What is severe sepsis?
Sepsis in presence of CV dysfunction, resp distress syndrome or dysfunction of >/= 2 organs
What is septic shock?
Sepsis with CV dysfunction persisting after at least 40 mL/kg of fluid restriction in 1 hour
What are the common organisms that cause sepsis?
- GBS and E.Coli, L. monocytogenes (early onset neonatal sepsis)
- Coagulase-negative Staph (CoNS) - staph epidermidis - (late onset neonatal sepsis)
Other orgs:
- Staph Aureus (Coagulase +ve) (Non-pyogenic strep)
- Strep pneumoniae
What is the investigations for sepsis?
- Clinical suspicion (diagnosis cannot be delayed)
- Bloods
What are the risk factors for neonatal sepsis?
- PROM/PPROM
- Chorioamnionitis (ie fever during labour)
What is the management of sepsis?
- Paediatric sepsis 6 within 1 hour and transfer to acute setting (+ continuously monitoring, review hourly)
- IV access (If failed after 2 attempts give IO access)
- Review by ST4 or above (<30 minute) and then consultation (<1 hour)
- LP in the following = <1 month, 1-3m who appear unwell, 1-3m with WCC <5 or >15x10^9
- IV fluid restriction + 20mL/kg 0.9% NaCl bolus over 5-10 mins
- CXR, urine dipstick on MSU
Bloods:
- Clotting (as DIC can feature in sepsis)
- Blood culture
- CRP (takes 12-24 huors to rise)
- VBG (including glucose and lactate) - ASAP
- FBC
- U&Es and creatinine
What antibiotics are given in sepsis?
Give within 1 hour and follow local guidelines:
If meningococcal sepsis:
- IM benzylpenicillin (in the community)
- IV cefotaxime (in hospital)
If other type of infection .. GOSH child ABx treatment:
- early onset <72 hours –> GBS, L. Monocytogenes, E. Coli –> IV cefotaxine, amikacin+ampicillin
- late onset >72 hours –> CoNS –> IV meropenem, amikacin+ampicillin
What are the signs and symptoms of CNS infection in a child?
- Bulging fontanelle, hyperextension of neck and back (opisthotonons)
- Headache, photophobia, neck stiffness, fever
- Kernig sign - pain on straightening the leg
- Brudzinzkis sign - supine neck flexion –> knee/hip flexion
- Lethargy, drowsiness, non-blanching rash (80% of meningococcal)
- HR starts high to compensate ischaemia in the brain
- HR then drops as baroreceptors in heart sense high BP (from HR)
- Raised ICP symptoms (late signs) = Cushings triad
- High BP, low HR and irregular RR
What are the investigations for CNS infection in a child?
- LP (CT head before Lp if concerns of raised ICP)
- Blood culture (an LP would be done before this)
- FBC, CRP, U&Es and glucose
- Coagulation profile
- Further immunological analysis (complement deficiency) if >1 episode of meningococcal meningitis
What is the management of CNS infection in a child? (bacterial meningitis, child >3 months)
Admit to hosp and follow sepsis 6 pathway 1) Antibiotics: Child <3 m old: - IV cefotaxine - IV amox/ampicillin
Child >3 m old:
- IM benzylpen, STAT (if penicillin allergy –> moxifloxacin & vancomycin)
- IV ceftriaxone:
•Neisseria meningitidis (meningococcal meningitis) – 7 days
•Neisseria meningitidis (meningococcal meningitis) – 7 days
•Haemophilus influenzae type b – 10 days
•Streptococcus pneumoniae – 14 days
2) Steroids (dexamethasone), if CSF shows:
- Purulent CSF
- WBC >1000/uL
- Raised CSF WCC + protein >1g/L
- Bacteria gram stain
- >1m & H. influenzae
- NOT MENINGOCOCCAL
3) Mannitol (reduce ICP)
4) IV Saline NaCL 0.9% (4-2-1 maintenance)
Notify HPU, review patient 4-6 weeks after discharge, discuss long term complications
What are the long term complications of CNS infection in a child?
- Hearing loss (audiological assessment)
- Neurological/development problems
- Orthopaedic, skin, psychosocial complications
- Renal failure
- Purpura fulminans = the haemorrhagic skin necrosis from DIC = acute/fatal, thrombotic disorder, manifest as blood spots/bruising/discolouration of skin (needs FFP, debridement or amputation)
What do you treat the contacts of a CNS infection in a child?
Ciproflaxacin and offer support www.meningitisnow.org
What is the most common org of viral meningitis?
- Coxsackie Group B
- echovirus
What is the management of viral meningitis?
- Discharge home (after tests to exclude bacterial causes) with supportive therapy (i.e. fluids)
- Safety net
What is encephalitis?
Inflammation of the brain parenchyma
What is the aetiology of encephalitis?
- Direct invasion of cerebellum by neurotoxic virus (e.g. HSV)
- Post-infectious encephalopathy of delayed brain swelling following neuroimmunological response to antigen
- Slow virus infection (e.g. HIV or subacute sclerosing pan-encephalitis following measles); in UK:
Most common UK:
• Enterovirus
• Respiratory viruses (influenza)
• Herpes (HSV (rare cause of childhood encephalitis), VZV, HHV-6) (>70% mortality from untreated HSV encephalitis)
Other = chickenpox, bacteria & fungus (very rare), mosquitos (Japanese encephalitis), ticks (tick-borne encephalitis), mammals (rabies encephalitis)
What are the signs and symptoms of encephalitis?
- Same as per meningitis (may not be able to differentiate clinically - begin treatment for both)
Main:
- fever,
- altered consciousness, seizures (differentiate potentially by behavioural change)
What are the investigations for encephalitis?
Same as meningitis.
LP contraindications:
- Cardiorespiratory instability - Focal neurological signs
- Signs of raised ICP (coma, high BP, low HR)
- Coagulopathy
- Thrombocytopenia - Local infection at LP site
- Causes undue delay in starting ABx
- Meningococcal meningitis
MRI: hyperintense lesion, oedema, BBB breakdown
PCR for virus
What is the management of encephalitis?
IV acyclovir (high-dose): - 3 weeks – HSV is a rare cause but complications are major, so treat empirically
IV administration:
- 500mg/m^2 every 8 hours for 21 days (use body surface area calculation)
- Reconstitute to: 25mg/ml with water and 5mg/ML with NaCL
Other:
- CMV –> add in ganciclovir and Foscarnet
- VZV –> acyclovir/ganiclovir
- EBV - acyclovir
Supportive care:
- fluids, ventilation etc.
What is anaphylaxis?
A Type 1 hypersensitivity reaction = antigen cross-linking with IgE membrane-bound antibody of mast cell or basophil
How often does anaphylaxis occur?
- 1;20,000 persons a year and 1;1000 are fatal
- 85% due to food allergy
What are the signs and symptoms of anaphylaxis?
- Airway (swelling, hoarseness, stridor)
- Breathing (high RR, wheeze, cyanosis, O2<92%)
- Circulation (pale, clammy, low BP, coma)
- Skin (urticaria/angioedema)
What is the management of anaphylaxis?
- ABCDE approach
- Call for help
- BLS if unresponsive/not breathing
IM Adrenaline (1: 1,000) – dose as per guidelines/weight:
- Given in thigh
- Assess response after 5 minutes and repeat if needed
Monitoring and additional treatment:
- Establish airway + high-flow O2
- IV fluids (20mL/kg crystalloids)
- IV Chlorpheniramine, 10mg (IM or slow IV)
- IV Hydrocortisone, 200mg (IM or slow IV)
- Salbutamol (if wheeze)
What is hereditary angioedema?
- C1 esterase deficiency
- recurrent facial swelling and abdo pain
What do babies with foetal alcohol syndrome present with?
- Microcephaly,
- absent philtrum,
- cardiac abnormalities,
- reduced IQ,
- IUGR,
- small upper lip
What does cigarette smoking in pregnancy do to a foetus?
- IUGR,
- miscarriage,
- stillbirth
What does rubella in pregnancy do to a foetus?
TRIAD: Cataracts, deafness, cardiac abnormalities
What does varicella in pregnancy do to a foetus?
- Skin scarring,
- eye defects (small eyes),
- neurological defects (low IQ, microcephaly)
What does syphilis infection do in pregnancy?
- Rhinitis,
- saddle-nose,
- deafness (sensorineural),
- hepatosplenomegaly,
- jaundice
What is Pataus syndrome and how prevalent is it?
- Trisomy 13
- 1 in 14, 000 births
- 80% die within the first month of life
What are the symptoms of pataus syndrome?
- Microcephaly (and brain defects)
- Microphthalmia (small eyes)
- Other eye defects
- Cleft lip/palate
- Polydactyl
- Omphalocele / Gastroschisis
What are the investigations for Pataus syndrome?
- USS analysis in 2nd trimester
- Chromosomal analysis from amniocentesis/cffDNA (NIPT)
What is Edwards syndrome and how prevalent is it?
- Trisomy 18
- 1 in 14,000 births
- Many will die in infancy, but prolonged survival is possible
- Associated with Exomphalos / Omphalocele
What are the symptoms of Edwards syndrome?
- LBW
- Small mouth/chin
- Low-set ears
- ‘Rocker-bottom’ feet
- Overlapping fingers
- Intellectual disability
- Cardiac, renal and GI abnormalities
- Omphalocele / Gastroschisis
What are the investigations for Edwards syndrome?
- USS analysis in 2nd trimester
- Chromosomal analysis from amniocentesis/cffDNA (NIPT)
What is Noonans syndrome?
- Autosomal dominant with a normal karyotype (affects both males and females)
- Penetrance varies greatly (from lethal prenatally to minimal morbidity)
- Mutated RAS/Mitogen Activated Protein Kinase
What are the signs and symptoms of noonans?
- Webbed neck
- Trident hairline
- Pectus excavatum
- Short stature
- Pulmonary stenosis – ES murmur
What is Kleinfelter’s syndrome?
- Male with 47 XXY
- 1-2 per 1,000 males live-born
- Normal appearance and normal IQ
What is Turners syndrome?
- Woman with 45X
- 1 in 2,500
What are the signs and symptoms of Turners syndrome in neonates?
- Pyloric stenosis
- Cardiac problems (coarctation of aorta, bicuspid aortic valve)
• AS murmur = ESM over aortic valve - Renal anomalies
- Cystic hygroma (may resolve early skin on the back of the neck)
- Ovarian dysgenesis
What are the signs and symptoms of Turners syndrome?
- Lymphoedema of hands/feet in neonate
- Short stature, spoon-shaped nails
- Wide carrying angle
- Thick or webbed neck
- Infertility
- Bicuspid aortic valve > Aortic coarctation (ESM over aortic valve)
- Delayed puberty
- Hypothyroidism
- Omphalocele / Gastroschisis
What is the management of turners syndrome?
- Growth hormone therapy (plot height on syndrome specific growth charts)
- Oestrogen replacement (at time of puberty for development of secondary sexual characteristics)
What is Fragile X syndrome?
- 1 in 4,000 births; although X-linked, some males can be unaffected and assume the role of ‘genetic carriers’ and transmit the condition through daughters to their grandsons (potentially due to variable penetrance due to genetic anticipation)
- A trinucleotide repeat-expansion mutation
What are other trinucleotide repeat-expansion mutations?
- Fragile X syndrome
- Myotonic dystrophy
- Huntington disease (occurs in a coding region of the genes abnormal protein production)
What are the signs and symptoms of Fragile X?
- IQ 20-80 (mean 50) – 2nd most common cause of low IQ after Down’s Syndrome
- Macrocephaly, macroorchidism
- Large, low-set ears
- Long, thin face
- Other – autism, joint laxity, scoliosis
- Complication: Mitral valve prolapse
What is the management of Fragile X?
Genetic counselling to all fragile-x families
What is PWS?
- Lack of paternal PWS region on CHr 15
What is the alternate name for PWS?
- Angelmans syndrome
- Lack of maternal PWS region on CHr 15
What are the signs of Angelmans syndrome?
- cognitive impairment,
- ataxia,
- epilepsy,
- abnormal facial appearance
What are the signs of PWS?
Fat, floppy, flaccid
- Hypotonia
- Hyperphagia
- Almond-shaped eyes
- Hypogonadism
- Obesity (in later childhood)
- Epicanthal folds
- Flat nasal bridge + upturned nose
- Learning disability
How does the PWS and Angelmans deletion come about?
- De novo deletion in the child
- Uniparental disomy (two copies from one parents, none from the other)
What is the management of PWS and angelmans?
- Growth hormone if clinical evidence of growth failure
- Management of feeding and obesity )ie. lock cupboards)
What is the pathogenesis of Down syndrome?
- Meiotic non-disjunction (94%)
- Error at meiosis, pair of chromosomes 21 fail to separate (one gamete has 2 x 21 and one has none)
- No need to examine parental chromosome - Translocation
- Extra chromosome 21 is joined to another chromosome (usually 14), Robertsonian translocation, parental Chr analysis is recommended, risk of recurrence 10-15% if mother is translocation carrier - Mosaicism
- Milder phenotype, some cells are normal, and some have trisomy 21, formation of chromosomally normal zygote but non-disjunction at mitosis
What are the craniofacial signs of down syndrome?
Craniofacial appearance:
- round face,
- flat nasal bridge,
- epicanthic folds,
- brushfield spots in iris,
- small mouth,
- small ears,
- flat occiput,
- 3rd fontanelle,
- short neck,
- single palmar crease,
- sandal-gap,
- hypotonia
What are the birth medical problems present with down syndrome?
- Congenital heart defects
- duodenal atresia
- hirschprungs disease
- omphalocele +/- umbilical hernia
What are the associated congenital conditions of down syndrome?
- Kostmanns syndrome
- Blood syndrome
- Fanconi syndrome; Diamond Black-fan
- NF 1
- Li Fraumeni syndrome
What are the later medical problems associated with down syndrome?
- Delayed motor milestone
- LD
- short stature
- OSA
- Visual impairment
- Sensory otitis media
Increased chance of:
- TAM
- Hypothyroid and coeliacs
- Early onset alzheimers
- Epilepsy
- Jpint laxity (screen for atlantoaxial instability in those doing sports –> risk of neck dislocation)
What is the immediate management of down syndrome?
- Echocardiogram (AVSD) and evaluation by paediatricians (duodenal atresia)
- Genetic counselling (review chromosome results, discuss risk of recurrence)
- Early intervention programmes if developmental delay is present
• Physiotherapy → prevent abnormal compensatory movements for physical limitations
• OT → fine motor and self-care
• SALT → speech intelligibility and to manage language delay
What is the later management of down syndrome?
- Annual hearing test, thyroid levels, ophthalmic evaluation (up to 5 years then every 2 years)
- Appropriate education placement with an individualised educational plan
- Haemoglobin level for IDA
- Monitor for symptoms of sleep apnoea (OSA)
- Monitor growth using updated Down’s syndrome growth charts
What support can you offer a child with down syndrome?
- Contact local DS clinic, access to local parent support groups
- Down syndrome association → helpline with lists of local groups, new parents pack, info for families and carers (www.downsyndrome.org.uk / National down syndrome society → events and support)
What is hypoxic ischaemic encephalopathy (HIE)?
Occurs if perinatal asphyxia has occurred ( cardiorespiratory depression)
- HIE is the neonatal condition (0.5-1 in 1,000 live births)
- Cerebral palsy is the post-neonatal condition (i.e. Severe HIE that is not treated)
What are the causes of HIE?
- Failure of gas exchange across placenta (i.e. placental abruption)
- Interruption of umbilical blood flow (i.e. shoulder dystocia –> cord compression)
- Inadequate maternal placental perfusion (i.e. maternal hypotension)
- Compromised foetus (i.e. - Failure to breathe at birth)
What determines the grade of HIE?
Response within the first 48 hours
What is mild HIE?
Mild = infant irritable, responds excessively to stimulation, staring eyes, hyperventilation, hypertonia
- Complete recovery can be expected
What is moderate HIE?
Moderate = abnormalities of movement, hypotonic, cannot feed, has seizures
- If fully resolved by 2 weeks of age, there is a good long-term prognosis
- If persistent past 2 weeks, bad prognosis
What is severe HIE?
Severe = no normal movements or response to pain, tone in limbs fluctuates hypo- to hyper-tonic, seizures prolonged and refractory to treatment, multi-organ failure may be present
- 30-40% mortality
- Over 80% have neurodisability (if not cooled) –> cerebral palsy
What is the management of HIE?
Supportive:
- Respiratory support
- Anticonvulsants (treat seizures)
- Fluid restriction (transient renal impairment)
- Inotropes (treatment of hypotension)
- Electrolytes and glucose (treat hypoglycaemia and electrolyte imbalances)
Therapeutic Hypothermia (>36w; mild hypothermia can reduce the morbidity from HIE –> requires NICU)
What is cerebral palsy?
Abnormality of movement and posture, causing activity limitation attributed to non-progressive disturbances that occurred in the developing foetal or infant brain 1 in 400 live births – most common cause of motor impairment
- If brain injury occurs after 2yo, it is diagnosed at acquired brain injury (and not CP)
What are the risk factors for cerebral palsy?
Antenatal:
- preterm birth,
- chorioamnionitis,
- maternal infection
Perinatal:
- LBW,
- HIE,
- neonatal sepsis
Postnatal:
-meningitis
What are the causes of cerebral palsy?
HIE during delivery - ie. during cord compression –> dyskinetic CP
Postnatal PVL (preiventricular leukomalacia) 2nd to ischaemia +/- severe intraventricular haemorrhage
What are the signs and symptoms of cerebral palsy?
- Delayed milestones (it is a progressive disease, it should not result in the loss of previously attained milestones)
- Feeding difficulties (slow, gagging, vomit), oro-motor miscoordination
- Abnormal gait once walking
- Hand preference before 1 year old (esp. spastic unilateral CP)
- Abnormal limb or trunk posture and tone in infancy
Stiff legs, scissoring of legs Unable to lift head
Unable to weight bear Rounded back when sitting
Hypotonia (floppy), spasticity (stiff)
Fisted hands
What is spastic cerebral palsy?
90% of CP
- Damage to UMN pathway —> ↑ tone (spasticity), brisk reflexes, extensor plantar, ‘clasp knife’ rigidity
• Damage to pyramidal (also known as corticospinal) tracts
• “Clasp knife” = increased tone suddenly gives under pressure
• Dynamic catch → faster the muscle stretched, greater the resistance, “velocity dependent” - Presents early, even neonatally as hypotonia
3 main types of Spastic CP:
(1) Unilateral / Hemiplegia → unilateral arm and leg, face spared
- Presents 4-12 months with [think of an Egyptian]
Fisting of affected hand and asymmetric hand function
Flexed pronated arm
With a tiptoe walk on affected side
- Initially flaccid but then ↑↑ tone
- Likely normal PMHx and unremarkable birth Hx (i.e. unknown antenatal cause)
(2) Bilateral / Quadriplegia – all 4 limbs, often severe
- Involving trunk, opisthotonos (extensor positioning)
- Poor head control
- Low central tone → associated seizures, microencephaly, moderate to severe learning disability, history of hypoxic-ischemic encephalopathy (HIE)
(3) Diplegia – legs affected to a greater degree (but all 4 limbs affected)
- Abnormal walking, difficulties with functional use of hands
- Associated with preterm birth damage and PVL
What is ataxic (hypotonic) cerebral palsy?
4% of CP
- Damage to cerebellum
- Hypotonic CP, most genetically determined
- Hypotonia, ataxia, mal-coordination, delayed motor development ± intention tremor
What are the investigations for cerebral palsy?
- MRI: offer MRI if the cause is not clear from history, developmental progress, clinical examination and cranial ultrasound (however, MRI cannot establish timing of injury) – GA or sedation is usually needed
Follow-up MDT - if child has risk factors for CP, offer MDT follow-up for 2 years
MDT Members:
• Main Members: paediatrician, nurse, physiotherapist, OT, SALT, dietetics, psychology
• Supplementary Members: orthopaedics, orthotics, visual and hearing
- Possible early motor features of CP
• Unusual fidgety movements or abnormal movement (asymmetry or paucity of movement)
• Abnormalities of tone (includes hypotonia, spasticity or dystonia (fluctuating tone))
• Abnormal motor developing (including late head control, rolling and crawling)
•Feeding difficulties - Delayed motor milestones (correct for gestational age)
• Not sitting by 8 months
• Not walking by 18 months
• Hand preference before 1 year - Refer all children with persistent toe walking
What are the investigations for cerebral palsy?
- MRI: offer MRI if the cause is not clear from history, developmental progress, clinical examination and cranial ultrasound (however, MRI cannot establish timing of injury) – GA or sedation is usually needed
Follow-up MDT - if child has risk factors for CP, offer MDT follow-up for 2 years
MDT Members:
• Main Members: paediatrician, nurse, physiotherapist, OT, SALT, dietetics, psychology
• Supplementary Members: orthopaedics, orthotics, visual and hearing
- Possible early motor features of CP
• Unusual fidgety movements or abnormal movement (asymmetry or paucity of movement)
• Abnormalities of tone (includes hypotonia, spasticity or dystonia (fluctuating tone))
• Abnormal motor developing (including late head control, rolling and crawling)
•Feeding difficulties - Delayed motor milestones (correct for gestational age)
• Not sitting by 8 months
• Not walking by 18 months
• Hand preference before 1 year - Refer all children with persistent toe walking
What are the red flags for other neurological conditions?
- Absence of risk factors
- Family history of progressive neurological disorder
- Loss of already attained cognitive or developmental abilities
- Development of unexpected focal neurological signs
- MRI findings suggestive of progressive (CP is classically non-progressive) neurological disorder
- MRI findings not in keeping with CP
What is the advice given to parents of children with cerebral palsy?
Information about prognosis to parents (and parental education):
- Walking: children who can sit by age 2 years are likely to be able to walk unaided by 6 years old
- Speech: 50% have difficulties with communication, 33% have difficulties with speech and language
- Life Expectancy: the more severe the CP the greater the likelihood of reduced life expectancy
- Support:
• SCOPE disability charity
• www.cerebralpalsy.org.uk
Paediatrician – medical problems
- 33% have epilepsy
Physiotherapy – encourage movement, improve strength and stop muscles from losing range of motion
- Speech therapy – improve language abilities
- 50% have communication difficulties
- 33% have speech and language
Occupation therapy – identify everyday tasks that may be difficult and help make these tasks more accessible
What are the medications used to treat cerebral palsy?
Stiffness – 1st: diazepam, 2nd: baclofen
Sleeping – melatonin
Constipation – Movicol
Drooling – anticholinergic
What is necrotising enterocolitis (NEC)?
Most common surgical emergency in newborn babies
- Leads to serious intestinal injury after combination vascular, mucosal, toxic and other insults to an immature gut
- Occurs due to immature/fragile bowels; RFs: PDA, pre-term
- Main cause is unknown and thought to be a combination of many factors – thought to lead to a combination of poor blood flow and infection of the intestines – often begins after starting enteral feeding
Who does NEC commonly affect?
Affects premature babies and LBW
What are the signs and symptoms of NEC?
- Early signs = biliary vomiting, feed intolerance
- Abdomen distension
- Blood-stained stool
- Rapid deterioration and shock
What are the investigations for NEC?
- AXR – ‘gas cysts’ in bowel wall
- Blood cultures
What is the management of NEC?
Use ‘Bell staging’ to decide the management:
- Bowel rest (NPO (stop oral feed) and switch to parenteral nutrition)
- Broad-spectrum antibiotics (cefotaxime/tazocin and vancomycin)
- Stage IA/IB (3 days), stage IIA (7-10 days), stage IIB, III (14 days)
- Laparotomy (if perforated as seen on AXR)
What are the long term consequences of NEC?
- 20% mortality/morbidity in the acute scenario
- Development of strictures
- Malabsorption (if extensive bowel resection is necessary)
What are the reasons for neonatal jaundice at birth?
- Physiological Hb release from RBC (high [Hb] at birth) - Breast milk jaundice (only >24hrs)
- RBC lifespan of 70 days rather than 120 days in adults
- BR metabolism less efficient in 1st few days of
How does kernicterus happen?
uBR –> deposit in basal ganglia –> kernicterus (a form of encephalopathy):
- Once albumin saturated and free uBR circulates (fat-soluble)
- crosses BBB and accumulates
- Causes spectrum from severe damage –> death; signs & symptoms:
• Lethargy, poor feeding • Irritability
• Increased muscle tone (arched back)
• Seizures and coma - May develop into cerebral palsy (dyskinetic), learning difficulties and sensorineural deafness
- Was a major problem with Rhesus disease of the newborn (not so much now with anti-D prophylaxis)
- Prevented with phototherapy ± IVIG, and exchange transfusion if required
Visible jaundice seen at 80umol/L
- uBR (fat soluble) –> kernicterus
- cBR (water soluble) –> dark urine, pale stools
How does phototherapy work?
- Phototherapy only works on uBR (converts uBR to lumirubin and photobilirubin) and not cBR. - If you give phototherapy and ‘bronzing’ occurs, then the child has cBR and not uBR and the phototherapy should be stopped.
What are the investigations of jaundice in a newborn <24 hours?
This is abnormal:
- direct (total) BR/SBR (as total BR is UBR)
- blood film analysis
- transcutaneous not appropriate
What are the investigations of jaundice in a newborn 2-14 days?
This can be normal:
- transcutaneous levels of BR (interpret at UBR as not likely CBR)
- blood film analysis
What are the investigations of jaundice in a newborn after 14 days?
This can be normal:
- check direct and indirect serum BR (check if it is UBR or CBR)
What is the cut off for prolonged bilirubin?
- > 14 days if term
- >21 days if preterm
What is the assessment of a child with jaundice?
- Visually inspect the child
Measure bilirubin:
• <24 hours –> serum BR (direct = total BR and indirect = uBR) so can assume tBR = uBR
• 24 hours to 2 weeks –> transcutaneous BR, can assume TC-BR = uBR
- Non-invasive – uses spectroscopy to measure light reflection from the skin
- If the result is >250 μmol/L, check the result by measuring serum bilirubin
• >2 weeks –> split serum BR (direct = total BR, indirect = uBR)
What are the measurement that assess the risk of developing kernicterus?
Increased risk if:
• Serum bilirubin >340 μmol/L in babies >37 weeks’ gestation
• Rapidly rising bilirubin of >8.5 μmol/L per hour
• Clinical features of kernicterus (poor feeding, extreme lethargy, hypotonia, high-pitched cry)
What investigations do you carry out to find out the underlying cause of jaundice in a newborn?
TC or serum BR must be taken within 6 hours of presentation (admit if <24hrs or >7 days):
- Haematocrit
- Blood group of mother and baby
- DAT test (Coombs; if <24hrs)
- FBC / blood film (e.g. hereditary spherocytosis)
- G6PD levels (ethnic origin) - MC&S of blood, urine and/or CSF (if suspected infection)
- TSH, LFTs
- Osmotic fragility (hereditary spherocytosis)
What is the management of jaundice in a newborn?
- No treatment needed if high cBR
(1) Phototherapy (converts uBR into water-soluble pigment excreted in urine) ± IVIG (ABO/Rh haemolysis)
- If 350 total serum BR in term baby
- If 120 total serum BR in premature baby
- Repeat BR measurement every 4-6 hours (until drops below threshold or stable)
- Ensure short breaks for breastfeeding
- Protect eyes
- Monitor temperature
- Stop once BR >50umol/L below threshold for treatment
- Check for rebound hyperbilirubinaemia with serum BR measurement 12-18 hours after
(2) IVIG used alongside in cases of:
- Rhesus disease
- Lower albumin –> lower ability to bind BR
- ABO incompatibility
- Leaky BBB
When is intensive phototherapy given in a neonate with jaundice?
- Rapidly rising serum BR
- Serum BR within 50umol/L of exchange transfusion threshold (after 72hrs of life)
- BR level fails to respond after 6 hours of therapy
When is exchange transfusion + phototherapy + IVIG given to a neonate with jaundice?
- If 450 total serum BR in term baby
- If 230 total serum BR in premature baby
- If signs of kernicterus
Make sure to give folic acid to prevent anaemia
What are resp signs of distress in a neonate?
- High RR (>60)
- Laboured breathing
- Chest wall recessions
- Nasal flaring
- Expiratory grunting (PAP)
- Cyanosis (if severe)
What is transient tachypnoea of the neonate?
- Commonest cause of resp distress in infants (most common in C-section babies)
- delayed resorption of lung fluid
What are the investigations of transient tachypnoea of the neonate?
- Cyanosis, high RR (i.e. >60)
- CXR → fluid in horizontal fissure
- Diagnosis made after exclusion of other causes
What is the management of transient tachypnoea of the neonate?
- Usually settles within first day of life
- Additional O2 if required
What is persistent pulmonary hypertension?
- Life threatening and usually associated with birth asphyxia, meconium aspiration, septicaemia, RDS (may also be primary)
- A result of the high pulmonary vascular resistance –> right to left shunting within lungs and at atrial and ductal levels
What are the signs and symptoms of PPH?
- Cyanosis after birth
- Absent heart murmurs and signs of HF
What are the investigations for PPH?
- CXR = normal sized heart but some pulmonary oligaemia
- Echocardiogram (urgent) = ensure no cardiac defect
What is the management of PPH?
Medications:
- O2
- NO (inhaled)
- Sildenafil
Ventilation:
- Mechanical ventilation / circulatory support
- High-frequency (oscillatory) ventilation
- (SEVERE) Extracorporeal membrane oxygenation (ECMO) ± heart and lung bypass
What is CLD?
Chronic lung disease of prematurity
- CLD occurs when infection, barotrauma or iatrogenic injury causes chronic lung problems
- Lung damage due to pressure and volume trauma from artificial ventilation, O2 toxicity, and infection
- Often defined by an O2 dependence ≤36w GA
When is CLD more common?
- Premature infants, increased risk in LBW or low GA
What are the signs and symptoms of CLD?
- Classically 23-26w progresses from ventilation to CPAP to supplementary O2
- Initially positive response which increases in O2 and ventilatory requirements in first 2 weeks of life
- Signs of respiratory distress, poor feeding, poor weight gain
What are the investigations of CLD?
- CXR –> widespread opacification
- CBG or VBG –> acidosis, hypercapnia, hypoxia
What is the management of CLD?
- Respiratory support = prolonged artificial ventilation –> wean to CPAP –> wean to additional O2
- (±) Corticosteroid therapy – dexamethasone for short term clinical improvement (limit use due to concern about abnormal neuro development and other adverse effects)
How to you prevent ventilation associated lung injury in a neonate with CLD?
- if baby does not need intubation or ventilation, it is important to minimise ventilation-associated lung injury using strict monitoring and maintaining of tidal volume
What is RDS?
Respiratory distress syndrome
- Deficiency of surfactant (phospholipids and proteins produced by type II pneumocytes)
What are the RFs of RDS?
Male, DM mothers (due to delayed lung maturation), CS, 2nd born of premature twins
Common if born <28 weeks gestation
What are the signs and symptoms of RDS?
At delivery or within 4 hours of birth:
- High RR (>60)
- Laboured breathing with recessions and nasal flaring
- Expiratory grunting (baby trying to make +ve airway pressure)
- Cyanosis (if severe)
What are the investigations of RDS?
- Clinical diagnosis
- Pulse oximetry
- CXR –> pneumothorax (from ventilation), ‘ground-glass’ appearance, indistinct heart border
What is the antenatal management of RDS?
- Steroid therapy (delivery <34w)
- Tocolytic therapy so steroids have at least 24 hours to work
What is the postnatal management of RDS?
- O2 and ventilation
- CPAP or artificial ventilation (via a tracheal tube may be necessary ± exogenous surfactant)
- Other options: mechanical ventilation, high-flow humidified oxygen therapy
When can a pneumothorax occur in a neonate?
- Can occur from RDS (or from the ventilation used to treat RDS) –> pulmonary interstitial emphysema
Ventilation-Associated Pneumothorax: to prevent pneumothoraces, infants should be ventilated with the lowest pressures that provide adequate chest movement and blood gasses
What is the management of a pneumothorax in a neonate?
- Immediate decompression
- O2 therapy
- Chest drain if tension pneumothorax
What does meconium aspiration cause in a neonate?
- respiratory distress in the newborn due to presence of meconium in trachea (causing mechanical obstruction and/or chemical pneumonitis –> pneumonia/infection) → occurs exclusively in immediate neonatal period
When is meconium aspiration more likely to occur?
Increased risk with gestational age
What are the RFs of meconium aspiration?
- GA >42 weeks - Maternal history of HTN/PET/smoking/substance abuse
- Fetal distress/hypoxia - Oligohydramnios
- Meconium stained amniotic fluid
- Chorioamnionitis
What are the signs and symptoms of meconium aspiration?
Respiratory distress – increased RR, chest retraction and hypoxia
What are the investigations for meconium aspiration?
- CXR (diagnostic) –> overinflated lungs, patches of collapse and consolidation
- Pneumothorax (from air leak)
- Pneumomediastinum (from air leak)
- FBC/CRP/Culture
What is the management of meconium aspiration?
- Observation = normal term infant with meconium-stained amniotic fluid but no history of GBS
- IV ampicillin and IV gentamicin = features of infection
- O2 and NIV (i.e. CPAP) = severe cases
What is a meconium ileus?
- thick, sticky meconium that has a prolonged passing time
- Meconium usually passes within 24hrs of delivery, if not, there may be an ileus
- The child may vomit the meconium instead of passing it as stool
- Associated with Cystic Fibrosis (90%) and biliary atresia
- 1 in 25,000 babies get an ileus
What is the meconium ileus management?
- 1st line = gastrograffin enema (N-acetylcysteine can also be used)
- 2nd line = surgery
What are the causes of billous vomitting in a child?
- NEC
- Duodenal atresia
- Jejunal/ileal atresia
- meconium Ileus
- Malrotation volvulus
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What us a cleft palate?
- Results from failure of fusion of the frontonasal and maxillary processes; polygenic inheritance
- Types: combined cleft lip and palate (45%) > isolated cleft palate (40%) > isolated cleft lip (15%)
- 1 in 1000 babies
What are the RFs of a cleft palate?
- Maternal antiepileptic use
- Maternal BDZ use
What are the investigations for cleft palate?
- 75% are detected 20w anomaly scan
- Increased risk of secretory otitis media
- Ask about feeding difficulties, weight gain (lower), hearing (otitis media)
What is the management of cleft palate?
o Pre-surgical concerns:
- Specialised feeding
- Watch out for airway problems (e.g. Pierre-Robin sequence)
- Pre-surgical lip tapping, oral appliances or pre-surgical nasal alveolar moulding (PNAM) to narrow cleft
o Surgery (definitive) – 3m for lip; 6-12m for palate
What is a diaphragmatic hernia?
- Part of intestine moves through the left chest area – stops lungs from developing properly (Bochdalek hernia)
- 85% = left-sided Bochdalek hernia –> prognosis depends on… (1) liver position, (2) lung-to-head ratio
- 1 in 2000 birthda occur at 6-8 weeks of pregnancy
What are the signs and symptoms of diaphragmatic hernia?
- Diagnose on routine USS or after resp distress at delivery
- Concave chest at birth
- Respiratory distress in the neonate (RR >60, absent breath sounds, cyanosis, etc.)
What are the complications of a diaphragmatic hernia?
- intestinal obstruction,
- volvulus of stomach,
- acute respiratory distress –> collapse/consolidation
What are the investigations of a diagphragmatic hernia?
- CXR (displaced mediastinum to left, collapsed left lung, bowel loops in thorax)
- Blood gas, U&Es, SpO2
What is the management of a diaphragmatic hernia?
- 1st –> NG tube and suction to prevent distension of intrathoracic bowel and allow breathing to occur
- 2nd –> surgery reduction and repair –> re-expansion of lung –> TPN/ventilatory support during recover
What is a TOF/OA?
- OA = malformation of oesophagus so it does not connect to stomach
- TOF = part of oesophagus joined to trachea; often occurs alongside OA
What type of TOF is most common?
Type C (90%) - Stomach acid can regurge and go into the lungs --> CLD/BPD
What are the associations of TOF/OA?
- polyhydramnios (no swallow),
- other developmental issues
What are the investigations fo TOF/OA?
- NG tube to aspirate stomach contents can quickly confirm/exclude
- Gold-standard –> Gastrograffin swallow
What is the management of TOF/OA?
- 1st = Replogle tube (drain saliva from oesophagus)
- 2nd = Surgical repair (few days of birth/neonatal) –> NICU and ventilator support
What are the complications of TOF/OA?
- Take longer to adjust to solids
- Respiratory complications - GORD
- Tracheomalacia - Feeding issues – stricture formation
What is billiary atresia?
Progressive fibrosis and obliteration of extra- and intra-hepatic trees –> chronic liver failure in 2 years
What are the 3 types of billiary atresia?
- T1 = common bile duct atresia
- T2 = cystic duct atresia
- T3 = full atresia (>90%)
What are the signs and symptoms of billiary atresia?
Obstructive jaundice picture:
- mild jaundice, pale stools, dark urine
- no vomiting
- Normal BW –> faltering growth
- Hepatosplenomegaly
What are the investigations of billiary atresia?
(raised cBR >14 days):
1st = USS –> triangular cord sign
- PT / INR
- LFTs (AST, ALT, ALP, GGT raised – biliary)
- FBC
- GOLD-STANDARD = TIBIDA isotope scan
- CONFIRMATION = ERCP ± biopsy
What is the management of billiary atresia?
- 1st line = Kasai hepatoportoenterostomy (involves ligating the fibrous ducts above the join with the duodenum and joining an end of the duodenum directly to the porta hepatis of the liver)
- transplant if unsuccessful
What are the management complications of billiary atresia?
- growth failure,
- portal hypertension,
- cholangitis,
- ascites
What is done/monitored in first year of biliary atresia?
- F = Fat-soluble vitamins (levels monitored)
- U = Ursodeoxycholic acid promotes bile flow
- P = Prophylactic ABx to prevent cholangitis (co-trimoxazole)
What is small bowel atresia?
- Congenital malformation of the small bowel –> absence or complete closure of a part of its lumen
What is duodenal atresia associated with?
- polyhydramnios (impaired swallow),
- Down’s (33%),
- congenital cardiac abnormalities
What are the signs and symptoms duodenal stenosis?
- no vomiting,
- potential for obstruction
- ‘double bubble’ on AXR
What are the signs and symptoms of small bowel atresia?
- Bile-stained vomiting (jejunal or ileal atresia) – tx as intestinal obstruction until proven otherwise
- Non-bilious or bilious vomiting (duodenal atresia; before or after sphincter of Oddi)
- Abdominal distension
What are the investigations for small bowel atresia?
Bloods – LFT, total serum BR (interpret as uBR), INR, serum amino acids
Urinary – organic acids, succinyl acetone, bile acids, lactate: pyruvate ratio
Imaging – AXR, CXR, USS and cholangiogram, Tc-99m scan
What is the management of small bowel atresia?
ABCDE to stabilise neonate ± NG tube decompression
Surgical:
- Primary anastomosis or Ladd procedure if malrotation present
- Need to examine entire bowel to exclude other multiple atretic segments
What are the complications of small bowel atresia?
- Pulmonary aspiration
- Anastomotic complications (stenosis or leak)
- Proximal bowel may have abnormal peristalsis – may need prolonged post-op TPN
When does small bowel atresia present?
Duodenal:
- <6 horus of after birth
- AXR double bubble sign
- Duodenoduodenostomy
Jejunal/illeal:
- <24 hours after birth
- AXR air fluid filled
- laparotomy
What are urinary tract anomalies in neonates?
Congenital structural abnormalities of kidneys, bladder or urethra
- associated with diff chromosome loci
- PAX 2 gene mutation
What are renal urinary malformations in neonates?
- Multicystic kidneys (AR PKD)
- Medullary spongy kidney
- Renal agenesis - Horseshoe kidney
What are non-renal urinary malformations in neonates?
- Pelvoureteric junction (PUJ) obstruction (2nd to stenosis, atresia of proximal ureter)
- Vesicoureteral reflux (VUR) – in 30% of children presenting with UTIs
- Bladder outlet obstruction
What is potters sequence?
Congenital abnormality in a neonate:
- now set ears
- beaked nose
- prominent epicanthis folds and downward slant to eyes
- pulmonary hypoplasia causing resp failure
- limb deformaties
What does potters sequence have oligohydramnios in pregnancy?
- Bilateral renal angesis or bilateral multicystic dysplastic kidneys
- leads to reduced foetal urine
- oligohydramnios leading to foetal compression
What are the signs and symptoms of urinary tract malformations antenatally?
- oligohydramnios
- decreased foetal UO
What are the signs and symptoms of urinary tract malformations postnatally?
- irritability,
- infections
- Decreased foetal urine output
- Intra- abdominal mass
- Haematuria
- Renal calculi and failure
- HTN
- Hepatosplenomegaly
What are the investigations for urinary tract malformations?
- Renal USS
- DMSA scan (Tc-99m) – detect scarring & functional defects, sensitive so only >2m after last UTI (gives false +ve)
- MCUG (Micturating Cystourethrogram) – visualise anatomy, see VUR and urethral obstruction, not past infancy
- MAG3 renogram (Tc-99m) – dynamic scan to see MAG3 excreted from blood into urine – use furosemide
- Genetic karyotyping –> Potter sequence (a sequence – renal problems —> oligohydramnios –> problems seen)
What is the management of a neonate with urinary malformation?
Treat underlying cause ie. surgery
What is the presentation of an atypical UTI in a child?
- Poor UO
- Abdominal/bladder mass - Raised creatinine
- Sepsis
- Failure to respond <48 hours
- Infection with non-E. coli organisms
What is the management of a neonate with urinary malformation with a UTI?
1st –> renal USS for…
- All children with an atypical UTI (USS during acute infection)
- Recurrent UTI; <6m (USS during acute infection)
- Recurrent UTI; >6m (USS urgent USS (<6w))
First UTI; <6m, responds to treatment (<48 hours) (USS urgent USS (<6w))
2nd –> DMSA scan for… (cannot be <2m after last UTI)
- All children with a recurrent UTI (DMSA routine (4-6m))
- Atypical UTI; <3yo (DMSA routine (4-6m))
3rd or VUR –> MCUG/VCUG scan for…
- VUR suspected on USS (Male, recurrent UTI Female, <3yo, 1st UTI)
- Obstruction, trauma (Female, pyelonephritis, recurrent UTI Female, <5yo, febrile UTI)
What is VUR?
Vesico-ureteric Reflux
- 30% of children presenting with UTIs
What is the pathophysiology of VUR?
Ureters enter bladder perpendicularly –> shorter intramural course –> VUR
What are the investigations for VUR?
- MCUG to diagnose
- DMSA to check for scarring (35% of VUR develop scarring)
What are the classifications of VUR?
- I = reflux into ureter only, no dilatation
- II = reflux into renal pelvis on micturition, no dilatation
- III = mild/moderate dilation of ureter, renal pelvis and calyces
- IV = dilatation of renal pelvis & calyces + moderate ureteral tortuosity
- V = gross dilatation of renal pelvis & calyces + ureteral tortuosity
What are the complications of urinary anomalies?
- HTN, renal osteodystrophy, UTI and calculi
- Renal causes –> prognosis bad (end-stage renal failure)
- Non-renal causes –> prognosis good (if treated)
What is a low anorectal anomaly?
anus closed over, in a different position or narrower than usual + fistula to skin
What is a high anorectal anomaly?
bowel has closed end at high level, does not connect with anus, usually associated with bladder/urethral/vaginal fistula
What are the signs and symptoms of anorectal anomalies?
- No/delayed meconium –> swollen abdomen, vomiting
- If fistula – may pass stool from abnormal area
What are the investigations of anorectal anomalies?
- Clinical
- Checked on neonatal check
What is the management of anorectal anomalies?
- Surgical correction by 9m (depending on specific anomaly) –> i.e. anorectoplasty + loop stoma
What is cryptorchidism?
Undescended testes
What is the RF for cryptorchidism?
- Prematurity
- Normal descent by 3m of age, however, it can take up to 6m to full descend
What is the management of cryptorchidism?
Unilateral (at birth) = commonly idiopathic and will resolve spontaneously
- Arrange review for 6-8
- At 6-8 weeks if still undescended, review at 3 months
- At 3 months, if undescended → refer to paediatric surgeon, to be seen before 6 months of age
Bilateral (at birth) = pituitary causes –> refer to paediatricians/endocrinologists for further tests
- Testes descent is controlled by testosterone
- If lack of testosterone, they will not descend (i.e. pituitary apoplexy)
- If suggestion of a disorder of sexual development at any point (e.g. ambiguous genitalia or hypospadias) then refer to senior paediatrician for endocrinological and genetic investigation
‘Retractile’ (i.e. testes have descended but come in and out of scrotal sac)
- Detect at 3m and advise annual follow-up throughout childhood
- Surgical = orchidopexy (palpable testes = inguinal approach; non-palpable = laparoscopy)
- Medical = ±b-hCG (analogue to LH and FSH and thus, stimulates testosterone release to stimulate descent)
What is possetting?
bringing up small amounts of milk along with swallowed air, occurs in nearly all babies
What is regurgitation?
larger, more frequent losses of food
What is vomiting?
forceful ejection of gastric contents
What are the causes of vomiting in an infant?
- GO reflux
- Feeding problems - Infection
- Dietary protein intolerance - Intestinal obstruction - Inborn errors of metabolism
- Congenital adrenal hyperplasia
- Renal failure
If chronic:
- gastro-oesophageal reflux or feeding problem (overfeeding, force feeding)
If transient:
- gastroenteritis, URTI
- Exclude meningitis or UTI
- If bile stained, exclude intestinal obstruction – intussusception, malrotation, strangulated inguinal hernia
What are the causes of vomiting in a pre-schooler?
- Gastroenteritis
- Appendicitis
- Intestinal obstruction
- Increased ICP
- Coeliac
- Renal failure
- Testicular torsion
What are the causes of vomiting in school age kids?
- Gastroenteritis
- Infection
- Peptic ulcer
- Appendicitis
- Migraines
- Increased ICP
- Coeliac disease
- Renal failure
- DKA
- Alcohol/drugs
- Cyclical vomiting syndrome - Bulimia/anorexia
- Pregnancy
- Testicular torsion
What are the causes of constipation in a baby?
- Hirschsprung’s,
- anorectal abnormality,
- hypothyroid,
- hypercalcemia,
- idiopathic
What are the causes of constipation in an older child?
- toilet training issues,
- stress,
- following acute febrile illness
What is the complication of long-standing constipation?
- If long standing, rectum becomes overdistended
- Loss of feeling the need to defecate → involuntary soiling with overflow
What is the management of constipation?
- Dis-impact with stool softeners (macrogol laxative → Movicol) with an escalating dose regime over 1-2 weeks
- If unsuccessful, consider stimulant laxative – Senna
- Then use maintenance therapy – Movicol
- Increase fluids and balanced diet with increasing fibre
- Make sure to engage with child – ICE, star reward chart
What are important questions to ask about if presenting with constipation?
- Did they poo in the first 24 hours of life?
- Majority of children who don’t will have an underlying gut problem, but by 48h 90% will - Diet and water – how much do they drink?
- Good hydration needed for meds to work and normal stool to form
What is GOR?
- GOR = due to inappropriate relaxation of LOS (functional immaturity) –> most resolve
- If persistent, can be due to GORD
What are the investigations for GOR?
- Clinical diagnosis
- 24hr LOS pH monitoring (should remain mostly above 4)
- OGD
What is the management of GOR?
Referral:
- same day if haematemesis, malaena or dysphagia
Assessment by paediatrician if:
- Red flag symptoms
- Faltering growth
- Unexplained distress
- Unresponsive to medical therapy
- Feeding aversion
- Unexplained IDA
- No improvement after 1 year of age
- Suspected Sandifer’s syndrome
Refer if there are complications:
- Recurrent aspiration pneumonia
- Unexplained apnoea
- Unexplained epileptic seizure-like events
- Unexplained upper airway inflammation
- Dental erosion with neurodisability
- Recurrent acute otitis media
What is the initial management of GOR?
Reassure (very common condition) –> may be frequent, less frequent with time, resolves by 12m
Review infant or child if:
- Projectile regurgitation - Bile-stained or haematemesis
- New concerns (e.g. faltering growth, feeding difficulties)
- Extend beyond 1 year
Initial Management (no positional management – MUST sleep on back):
- If breast-fed:
1. breastfeeding assessment
2, consider trial of alginate for 1-2 weeks
3. pharmacological
- If formula-fed:
1. review feeding history
2. trial smaller, more frequent feeds (aim for 150-180 mL/kg/day)
3. trial of thickened formula (e.g. containing rice starch –> Enfamil, Carabel)
4. trial of alginate therapy (stop periodically to see if infant has recovered)
5. pharmacological
What is the pharmacological management of GOR?
Pharmacological Management (only done in certain circumstances):
4-week PPI/H2 antagonist trial – in children unable to describe symptoms or ≥1 of:
- Unexplained feeding difficulties (refusing feeds, choking)
- Distressed behaviour
- Faltering growth
- Children complaining of persistent heartburn, retrosternal or epigastric pain
Mnemonic (order):
G - Gaviscon (a form of alginate therapy)
O - Omeprazole
R - Ranitidine
D - Dunno, so refer to get metoclopramide
What is pyloric stenosis?
- hypertrophy of the pyloric muscle causing gastric outlet obstruction
- Presents at 2-8 weeks of age, (boys > girls at 4: 1)
- Aetiology = hypertrophy of circular muscles of the pylorus (Associated with Turner’s syndrome)
Signs and symptoms of pyloric stenosis?
- Projectile Vomiting (increases in frequency and forcefulness over time, ultimately becoming projectile)
- Occurs ~30 minutes after a feed
- NON-BILIOUS
- Palpable ‘olive’ mass in RUQ
- Visible peristalsis in upper abdomen
- Hunger –> dehydration –> loss of interest in feeding –> weight loss + depressed fontanelle
Hypochloraemia hypokalaemic metabolic alkalosis (low [Cl-], low [H+]; low [K+] and [Na+]):
- HCO3- is elevated (metabolic alkalosis; H + HCO CO2 and H2O)
- May progress to a dehydrated lactic acidosis (opposite biochemical picture)
What are the investigations for pyloric stenosis?
- Test feed observe for gastric peristalsis
- USS confirmation – target lesion, >3mm thickness
What is the management of pyloric stenosis?
- IV slow fluid resuscitation + correct any disturbances = 1.5x maintenance rate +5% dextrose + 0.45% saline
- Laparoscopic Ramstedt pyloromyotomy
What is infant colic?
- Describes a common (40% of babies in the first few months of life) symptom complex
- manifests as random inconsolable crying and drawing up on the hands and feet
- resolves by 3-12 months
What is the management of colic?
- Soothe infant – hold with gentle motion, optimal winding technique, white noise
- If persistent → consider cow’s milk protein allergy or reflux, consider:
(1) 2-week trial of whey hydrolysate formula; followed by
(2) 2-week trial of anti-reflux treatment
Support:
- Self-help support group www.cry-sis.org.uk for families with excessive crying or sleepless children
- Get support from health visitor, family, friends and other parents
What is appendicitis?
- inflammation of the appendix
- Feacolith more common in pre-school children (see on AXR) and perforation more common
What are the signs and symptoms of appendicitis?
- Anorexia, vomiting, nausea, umbilicalRIF pain
- Fever, tenderness, etc
What are the investigations of appendicitis?
- FBC, pregnancy test (if female)
- Clinical (watchful waiting observation)
- AXR ± CTAP
What is the management of appendicitis?
G - Group and save
A - ABx, IV
M - MRSE screen
E - eating avoid (NMB)
Appendiectomy
What is intussusception?
Invagination of proximal bowel into distal component; 95% ileum through to caecum through ileocecal valve (ilio-colic)
Stretching and constriction of the mesentery –> venous obstruction –> engorgement and bleeding from the bowel mucosa, fluid loss –> bowel perforation, peritonitis and gut necrosis
What are the causes of intussusception?
- idiopathic,
- enlarged Peyer’s patches after gastroenteritis,
- lead points,
- hypertrophy (CF)
What are the associations of intussusception?
- lymphoma,
- gastroenteritis (viral illness enlarges Peyer’s patches),
- HSP,
- CF
What is the common appearance of intussusception?
- 3m - 2 years
- (rarely < 3m)
- Males 2x more than females
What are the signs and symptoms of intussusception?
- Colic (draw into a ball)
- Vomit (may be bile stained depending on the site of the intussusception)
- Late sign: red-currant jelly stool (bloody mucus)
- Abdominal distension in RUQ (± sausage-shaped mass) and shock
- Dance sign = emptiness on palpitation in RLQ
What are the investigations for intussusception?
- 1st line: Abdominal USS –> “Target Mass”
- 2nd line: Abdominal X-Ray –> paucity (less) of air in RUQ + large bowel, thickened wall (oedema), poorly defined liver edge, dilated small bowel loops
- 3rd line: barium (or gastrograffin) enema
What is the management of intussusception?
EMERGENCY + drip and suck
- 1st line = rectal air insufflation / barium/gastrograffin enema
- Process = light GA, barium trickled in <30cmH2O, assess location + treat
- Broad-spectrum antibiotics –> Clindamycin + gentamicin; OR Tazocin; OR Cefoxitin + vancomycin
- 2nd line (perforation) = surgical reduction with broad-spectrum antibiotics [if perforated]
What is the management for recurrent intussusception?
consider investigating for a lead point (Meckel’s diverticulum, polyps, appendix)
What is Meckel’s diverticulum?
An ileal remnant of the vitello-intestinal duct on anti-mesenteric border containing ectopic gastric mucosa (i.e. can form gastric ulcers that bleed) or pancreatic tissue – as the child was developing, a small pouch formed elsewhere in the body
When do babies present with Meckel’s diverticulum?
Between 1 and 2 years olf
What are the signs and symptoms fo Meckel’s diverticulum?
- PAINLESS MASSIVE PR bleeding (dark red)
- May present in addition to intussusception, volvulus or diverticulitis
- May show bilious vomiting, dehydration, intractable constipation
What are the investigations of Meckel’s diverticulum?
- Technetium scan (Meckel’s scan) indicates increased uptake by gastric mucosa in 70% of cases
- AXR or abdominal USS ± laparoscopy
What is the management of asymptomatic Meckel’s diverticulum?
Incidental imaging finding – NO treatment required)
What is the management of symptomatic Meckel’s diverticulum?
- Bleeding – excision of the diverticulum with blood transfusion (if haemodynamically unstable)
- Obstruction – excision of diverticulum and lysis of adhesions
- Perforation/peritonitis – excision or small bowel segmental resection with perioperative antibiotics
Antibiotics: Cefotaxime, clindamycin/metronidazole
What is the rule of 2 in Meckels Diverticulum?
(1 to) 2-years-old
2% population
2x more common in boys
2 feet from ileocecal valve (2 feet for adult)
2 inches long
2 different mucosae (gastric and pancreatic)
What is malrotation/volvulus?
Predisposition to volvulus if mesentery is not fixed to duodenal flexure or ileo-ceacal region (can occur during rotation of the GI tract during foetal development –> shorter base w/volvulus ± Ladd bands obstruct duodenum –> biliary vomiting)
What are the associations of malrotation/volvulus?
- exomphalos,
- congenital diaphragmagmatic hernia
What are signs and symptoms of malrotation/volvulus?
- Asymptomatic and present at any age with volvulus; OR
- Present in first few days of life with:
- Obstruction
- Obstruction + compromised blood supply
- Symptoms = abdominal pain, bilious vomiting, peritonism, etc.
- Scaphoid abdomen (i.e. concave abdomen)
What are the investigations of malrotation/volvulus?
- Upper GI contrast study (URGENT) to assess patency if bilious vomiting
- USS
What is the management of malrotation/volvulus?
- Urgent laparotomy (Ladd’s procedure if signs of vascular compromise)
- Untwist volvulus, mobilise duodenum, place bowel in non-rotated position and remove necrotic bowel
What is irritable bowel syndrome?
- Altered GI mobility and abnormal sensation ± psychosocial stress and anxiety effect
- Often a FHx component; Coeliac’s must be excluded; symptoms may be precipitated by GI infection
What is the signs and symptoms of irritable bowel syndrome?
- Abdominal pain – often worse before or relieved by defecation
- Explosive loose or mucus stools
- Boating
- Feeling of incomplete defecation – tenesmus
- Constipation
What are the causes of gastroenteritis?
- Rotavirus infection = MOST COMMON CAUSE (60%)
- Campylobacter jejuni = Severe abdominal pain, bloody stool
- Shigella / Salmonella = Blood/pus in stool, pain, tenesmus, fever
- Cholera / E. coli = Dehydrating diarrhoea
- CHESS organisms = bloody diarrhoea (Campylobacter, Haemorrhagic E.coli, Entamoeba histolytica, shigella, salmonella)
What are the signs and symptoms of gastroenteritis?
- Sudden change to loose-stools and accompanied by vomiting (potential travel history)
- Complications → dehydration → shock (increased risk if…: <6m, >2 vomits in <24 hours, cannot tolerate extra fluids or malnourishes, >5 diarrhoeal stools in <24 hours)
What are the investigations for gastroenteritis?
- AXR and exclude other causes
- Stool sample analysis –> young/viral cause = stool electron microscopy; older/bacterial cause = stool culture
What is the management of gastroenteritis?
Give rehydration advice:
Maintenance fluid volumes:
• 0-10 kg = 100ml/kg
• 10-20kg = 1000ml + 50ml/kg for each kg over 10kg
• 20+ kg = 1500ml + 20ml/kg for each kg over 20kg
Modes of rehydration:
• <5yo = 50 ml/kg IV fluids over 4 hours as well as maintenance with oral rehydration solution
• 5+ years = 200 mL after each loose stool
Do not drink sugary or carbonated drinks (sugar in the gut can draw excess water into the gut)
Do not give anti-diarrhoeals to <5yo
What gastroenteritis organisms need to be notified to the Health protection unit:
- Campylobacter
- Listeria
- E. Coli )157
- Shigella
- Salmonella
What is the safety net for gastroenteritis?
- Diarrhoea: usually 5-7d, most stop within 2 weeks
- Vomiting: usually 1-2d, most stop within 3 days
How do you assess dehydration in a child?
o Weight loss is the most accurate marker
- No clinically detectable dehydration (<5% loss of body weight)
- Clinical dehydration (5-10%)
- Shock (>10%)
o Can use clinical signs to estimate degree of dehydration
What are the signs of hypernatraemia?
Full OF SALT
Flushing Oedema Fever Seizures Agitation / jittery movements Low urine output Thirst
What are the signs of hyponatraemia?
SALT LOSS
Stupor Anorexia (+ N&V) Limp tone Tendon reflexes reduced Lethargy Orthostatic hypotension Seizures Stomach cramps
What are the investigations for dehydration?
Clinical Examination (likely viral cause):
- U&E and FBC
- Stool M&C (only if BLOODY diarrhoea – CHESS organisms)
What is the management of dehydration?
Over 24 hours… = maintenance + dehydration:
- Oral rehydration solution –> clinical dehydration
(Oral rehydration solution contains glucose –> absorption in gut –> water follows glucose absorption) - IV fluids –> shock / deterioration / persistent vomiting / sick child
What is the management of shock?
Bolus fluids (these don’t need to be subtracted from the dehydration corrections):
- 20mL/kg 0.9% NaCl over 15 minutes (most situations, new DKA guidelines)
- 10mL/kg 0.9% NaCl over 60 minutes (trauma, fluid overload, heart failure, old DKA guidelines)
How do you calculate dehydration corrections?
Over 24 hours:
Add to maintenance fluids:
1st - weight child (or estimate weight)
2nd - calculate weight lost from fluids
How do you calculate normal maintenance fluids for dehydration?
- 5% dextrose (do not use in boluses; do not use in immediate maintenance in DKA)
- 0.9% sodium chloride (as per below)
“4:2:1” approach
- 4 x = 10kg +
- 2 x = 10kg +
- 1 x = 10kg = TOTAL ml/hour
- E.G. 70kg (10, 10, 50) = “4 x 10” + “2 x 10” + “1 x 50” = 110mL/hour (2,640mL/day
so 2,500mL/day as max for boys and 2,000mL/day for girls)
What is neonatal fluid restriction?
- Day 0: 60 ml/kg/day
- Day 1: 90 ml/kg/day
- Day 2: 120 ml/kg/day
- For term neonates use isotonic crystalloids with 10% dextrose
I.E. 2.5kg neonate at day 2 fed 3 times a day
I.E. 2.5*120 = 300 over 24 hours –> 300/8 = 37.5mL per 8-hourly feed
How to treat hypernatraemic dehydration?
- Oral rehydration solution
- If IV fluids required, take care with cerebral oedema (rapid reduction in plasma sodium concentration and osmolality will lead to a shift of water into the cerebral cells and may result in seizures and cerebral oedema)
- Fluid deficit replaced over at least 48 hours and the plasma sodium should be measured regularly
Why are antidiarrheal drugs and antiemetics not used in children?
o Ineffective o May prolong the excretion of bacteria in the stools
o Can be associated with side-effects
o Add unnecessarily to cost
o Focus attention away from oral rehydration
Are antibiotics use in gastroenteritis treatment?
o Not routinely required to treat gastroenteritis (even if the cause is bacterial)
o Only indicated for:
- Suspected or confirmed SEPSIS
- Extra-intestinal spread of bacterial infection
- Salmonella gastroenteritis if < 6 months
- Malnourished or immunocompromised children
- Specific bacterial or protozoal infections
• C. difficile associated with pseudomembranous colitis
• Cholera, shigellosis, giardiasis
What is post gastroenteritis syndrome?
o After gastroenteritis –> introduction of a normal diet results in a return of the watery diarrhoea
o Treatment: oral rehydration therapy
What is chrons disease?
- Affects any part of the GI tract mouth to anus
* Transmural and most commonly affects the distal ileum and proximal colon
What are the signs and symptoms of chrons?
o Abdominal pain, diarrhoea, weight loss
o May see growth failure, delayed puberty, fever, lethargy, aphthous ulcers, perianal skin tags
o Uveitis, arthralgia, erythema nodosum
o Complications → inflamed thickened bowel is susceptible to strictures and fistulae
What are the investigations for chrons?
- Biopsy = non-caseating epithelioid cell granulomata
- FBC (including iron, B12 and folate)
- CRP and ESR
- Faecal calprotectin
- Upper GI and small bowel contrast scan
- Colonoscopy and biopsy (cobblestones)
- Assess impact on daily functioning (anxiety, depression)
- Stopping smoking (reduce risk of relapse)
- Assess risk of osteoporosis
What is the management of Chrons?
1st) Induce remission:
- Nutritional management –> effective in 85-100% patients (crohns.org)
• Replace diet with whole protein modular diet – excessively liquid, for 6-8 weeks
• May need NG if the child struggles to drink that much
• Products are easily digested, provide all nutrients needed to replace lost weight
- Pharmacological management –> steroids (prednisolone) may be used to induce remission
2nd) Maintain remission – you can use steroids, but these have long-term consequences…
- Aminosalicylates (e.g. mesalazine)
- Immunosuppressive drugs (azathioprine, methotrexate, mercaptopurine)
• Azathioprine cannot be given to people with a TPMT mutation
• Must not have live vaccines
• Must have pneumococcal and influenza vaccines
- Anti-TNF antibodies in biologic therapies (e.g. infliximab)
- Medical therapies require monitoring of biochemical measures (e.g. ferritin, B12, calcium and vitamin D)
o Surgery for complications – obstruction, fistula, abscess, severe localized disease unresponsive to treatment
o SUPPORT → www.chronsandcolitis.org.uk – has information leaflet and grants available (Educate on features of flare-ups)
What are the signs and symptoms of UC?
Partial thickness, distal to proximal, crypt damage, ulceration
o Classic presentation is rectal bleeding, diarrhoea, abdominal pain
o Weight loss and growth failure
o Erythema nodosum, arthritis
What are the associations and complications of UC?
- PSC
- Toxic megacolon
- Perforation
- Enteric arthritis
- Haemorrhage
- Bowel cancer
What are the investigations of UC?
(Faecal calprotectin screens for IBD in general)
o Endoscopy and histological features:
- Confluent colitis extending from rectum proximally
- Histology = mucosal inflammation/ulceration, crypt damage (abscesses, loss, architectural distortion)
- Small bowel imaging to check extra-colonic inflammation (Crohn’s) is not present
o In children, 90% have pancolitis
o Severity graded using: - Paediatric Ulcerative Colitis Activity Index (PUCAI) (N.B. be aware of coexistent depression) • Severe = >65 points • Mild-Moderate = 10-64 points - Truelove and Witts score
What is the management of UC?
1st line: topical –> oral aminosalicylates – if no improvement 4 weeks after starting, move to oral, then 2nd line
- Often used to maintain remission
- Can use oral azathioprine or mercaptopurine if aminosalicylates insufficient
2nd line: topical –> oral corticosteroid (i.e. if aminosalicylates not tolerated/contraindicated)
- Prednisolone
- Beclomethasone
3rd line: oral tacrolimus
4th line: biological agents (infliximab, adalimumab and golimumab)
5th line (resistant disease) –> surgery (colectomy with ileostomy or ileojejunal pouch)
What medical education/support do you give a parent with a child with UC?
- UC is associated with an increased risk of bowel cancer
- Crohn’s and Colitis UK
- Regular screening performed after 10 years of diagnosis
What is severe fulminating disease?
EMERGENCY: Severe ulcerative colitis is defined by more than eight bloody stools daily and evidence of toxicity, demonstrated by fever, tachycardia, anemia, or an elevated erythrocyte sedimentation rate.
- MDT approach (medics and surgeons)
- IV corticosteroids or ciclosporin and assess likelihood of needing surgery
–> Increased likelihood of needing surgery if:
• Stool frequency > 8 per day
• Pyrexia
• Tachycardia
• AXR showing colonic dilatation
• Low alb, low hb, high platelets or CRP - Consider IV ciclosporin (if IV corticosteroids are contraindicated or ineffective)
What is toddlers diarrhoea/diarrhoea chronic non-specific diarrhoea?
Toddler diarrhoea = chronic and non-specific diarrhoea
- Commonest cause of loose stools in preschool kids
- Underlying maturational delay in intestinal mobility –> increased intestinal hurry (not malabsorption)
What are the signs and symptoms of toddlers diarrhoea/diarrhoea chronic non-specific diarrhoea?
- Varying consistency stools (well-formed to explosive and loose ± presence of undigested vegetables in stool)
- Child is well and thriving (no precipitating dietary factors and normal examination)
What is the management of toddlers diarrhoea/diarrhoea chronic non-specific diarrhoea?
- Increased fibre and fat in diet (whole milk, yoghurts, cheeses) –> relieve symptoms
- Avoid fruit juice and squash
What is the first-line management of constipation?
1) Advise behavioural interventions (scheduled toileting, bowel habit diary, reward system)
• Sit on the toilet after mealtimes to utilise the physiological gastrocolic reflex
• Behavioural interventions (e.g. star chart) may be useful to aid motivation and keep a record
- Advise diet and lifestyle advice (adequate fluid intake)
2) Medications –> disimpaction or mild constipation:
• Step 1: Movicol Paediatric Plain (polyethylene glycol + electrolyte) escalating dose for 2 weeks
o Disimpaction inadequate response –> add a stimulant laxative
o Mild constipation –> maybe add a stimulant laxative
• Step 2: Maintain for 6 months
What are the different types of laxatives?
1st line: Osmotic = Polyethylene Glycol 3350 (Movicol Paediatric Plain), lactulose
2nd line: Stimulant: Bisacodyl, Senna, sodium picosulphate
Bulk-forming: fybogel, methylcellulose
Stool-softener: arachis oil, docusate sodium
( Emphasise that laxative use is safe, even in the long-term, Underuse is the most common cause of treatment failure)
What is Hirschprungs disease?
An absence of ganglion cells from the myenteric (Auerbach) and submucosal (Meissner’s) plexuses; begins at the rectum and spreads proximally for a variable distance (75% rectosigmoid), ending at normally innervated, dilated colon
Complications: meconium plug syndrome, Hirschprung’s enterocolitis (perforated colon)
Risk factor = Down’s, MEN2a, del(chr10), male
What are the signs and symptoms of Hirschprungs disease?
- Failure to pass meconium <24hrs –> abdominal distension, bile-stained vomiting
- Explosive passage of liquid/foul stools
- May present later in first few weeks of life with severe, life-threatening Hirschsprung enterocolitis (C. diff)
What are the investigations for Hirschprungs disease?
Initial –> AXR (if obstruction), contrast (barium) enema (dilated distal segment + narrowed proximal segment)
Definitive –> suction-assisted full-thickness rectal biopsy –> absence of ganglion cells, ACh +ve nerve trunks
What is the management of Hirschprungs disease?
- 1st line (initial management) = bowel irrigation
- 1st line (after) = Endorectal pull-through – colostomy followed by anastomosing normally innervated bowel
- Total colonic agangliosis would require initial ileostomy with later corrective surgery
Other procedures = recto-sigmoidectomy, retro-rectal trans-anal pull-through, and ano-rectal myomectomy
–> I.E. Swenson, Duhamel, Soave procedures
What is an anal fissure?
Tears in skin around the anus, usually as a side effect of constipation → sphincter stretches to allow hard dry stool out
What are the signs and symptoms of anal fissure?
o Painful passing of stool
o Bright red blood on tissue (can examine for fissures)
What is the management of anal fissure?
- Conservative –> ensure stools are soft and easy to pass:
- Increase dietary fibre (include foods containing whole grains, fruits and vegetables)
- Increase fluid intake
- Manage pain – simple analgesia; sit in shallow, warm bath
• Topical anaesthetics can be used (i.e. lidocaine) or GTN ointment - Anal hygiene
- Advise against stool withholding
Consider constipation treatment pathway
SAFETY NET: seek further help if not healed within 2 weeks
What are the signs and symptoms of a threadworm?
- extreme itching around the anus or vagina, particularly at night
- irritability and waking up during the night
- worms seen in poo and backend of anus
What is the management of a threadworm?
Single dose of an anti-helminth (e.g. mebendazole / “Ovex”) for the whole household
- The dose may be repeated in 2 weeks if the infection persists
- Advise rigorous hygiene for 2 weeks if on mebendazole or 6 weeks if using hygiene measures alone:
• Hand washing
• Cut fingernails regularly, avoid biting nails and scratching around anus
• Shower each morning, including the perineal area, to remove eggs from skin
• Change bed linin and nightwear daily for several days after treatment (don’t shake bedsheets)
• Thoroughly dust and vacuum
Exclusion from school/nursery is NOT required
Children <6 months should be treated with hygiene measures alone for 6 weeks (seek advice from ID specialist)
Consider treating all household contacts (threadworm is highly transmissible)
What is mesenteric adenitis?
Mainly in children <15yo; recent viral/bacterial infection –> common cause of abdominal pain
What are the signs and symptoms of mesenteric adenitis?
- Abdominal pain – central or RIF
- Nausea ± diarrhoea, ↓ appetite
- INFECTIOUS picture: high temperature, lymphadenopathy, ↑WCC often preceded by UTI
What are the investigations for mesenteric adenitis?
- Large mesenteric lymph nodes seen at laparoscopy (w/ normal appendix) –> definitive
- Diagnosis of exclusion (as laparoscopy is a bit much…) –> exclude appendicitis (bloods, urine MC&S)
What is the management of mesenteric adenitis?
- Simple analgesia (symptoms usually resolve in a few days, maximum 2 weeks)
- Antibiotics (maybe, but not routine)
- Safety net for increased pain, deterioration, etc.
What is lactose intolerance and the RFs?
Lactase deficiency (lactose → glucose and galactose) –> lactose ferments in gut –> ↑ waste gas –> pain and bloating
o RFs: FHx, ethnicity
o Affects up to 75% of world’s population (less Caucasian, more Asian, African and Hispanic)
What is primary and secondary lactose intolerance?
Primary (70%) / AR → deficient lactase (Asian, African, Hispanics)
Secondary (30%) → damage to gut, temporary lactase deficiency (gastroenteritis, Crohn’s, coeliac, alcoholism)
- I.E. previous bout of gastroenteritis and full resolution with persistent diarrhoea from temporary damage
Exclude: gastroenteritis (stool sample), Crohn’s (faecal calprotectin, colonoscopy) Coeliac’s (anti-tTG/EMA)
What are the signs and symptoms of lactose tolerance?
- wind,
- diarrhoea,
- bloating with lactose ingestion,
- abdominal rumblings
- pain
What are the investigations for lactose intolerance?
- Clinical diagnosis (trial lactose-free diet for 2 weeks and see how symptoms are)
- Breath hydrogen test:
Normal = unabsorbed CHO fermented by large intestine GIT bacteria to produce H2 which is absorbed by the blood and exhaled from the lungs a period of time after initial ingestion
Pathology = GIT bacteria extend to small intestine from large intestine (due to overgrowth) and CHO metabolism occurs earlier in digestion leading to an earlier rise in exhaled H2 following CHO ingestion - Lactose intolerance test (outdated):
Lactose ingested after fasting and a lack of a rise in blood sugar is noted - FBC (rule out secondary disease –> anaemia, ↑WCC)
What is the management of lactose intolerance?
- Dietician referral
- Avoid milk and dairy products –> provide calcium and vitamin-D supplementation
For primary LD:
- Experiment with diet – different with each child, need to discover individual lactose threshold
- Potential foods:
• High-fat dairy (lower lactose)
• Hard cheeses
• Milk substitutes (almond, soya, coconut)
For secondary LD:
- Cut out dairy and allow gut time to heal
- May need Ca2+ and Vitamin D supplements
- Digestive enzymes can be taken in a capsule before eating lactose until gut heals/matures
What is coeliacs disease?
Autoimmunity to gliadin (in gluten, wheat, barley and rye) –> shorter villi and flat mucosa
Damage to proximal small intestinal mucosa –> rate of migration of absorptive cells moving up the villi (enterocytes) from the crypts is massively increased but insufficient to compensate for increased cell loss from the villous tips
o Common; 1% of infants
o HLA DQ2 (95%) and DQ8 (80%) association
What are the signs and symptoms of coeliacs disease?
Malabsorption syndrome (failure to thrive, abdominal distension, bloating, irritability)
- Presents 8-24m after introduction of wheat-foods
- May present later with non-specific GI symptoms
Malnutrition (check weight, height, BMI) –> wasted buttocks and distended abdomen
Pathogenomic = dermatitis herpetiformis (pruritic papulovesicular elbow/knee rash)
What are the investigations for coeliacs disease?
Serological diagnosis:
- Most sensitive = IgA tissue transglutaminase (anti-tTG)
- Less sensitive = IgA anti-endomysial cell antibodies (anti-EMA)
- If IgA deficient –> IgG DGP / Deiminated Gliadin Peptide
FBC and blood smear (iron deficient, vitamin B12/folate deficient, vitamin D deficient)
Confirmation of diagnosis (n.b. grading with the ‘Marsh’ system):
- Older children / adults –> OGD + jejunal biopsy (villous atrophy, crypt hyperplasia, ↑ IELs)
- Very young children –> no histopathological confirmation / biopsy –> EMA and HLA DQ2/DQ8 testing
What is the management of coeliacs disease?
- Remove all products containing wheat, rye and barley FOR LIFE
- MDT – dietician, child psychologist, school involvement, GP, paediatric gastroenterologist
- Dietician referral (if problems with adhering to the diet) and annual (6-12m) review:
Regular checks of height, weight and BMI – check this at home
Review symptoms
Review adherence to diet
Consider blood tests (coeliac serology, FBC, TFT, LFT, vitamin D, B12, folate, calcium, U&E) - Support sources: Coeliac UK
- Explain the importance of keeping to a strict gluten-free diet
Non-adherence to diet –> micronutrient deficiency (vitamin D, iron), osteoporosis, EATL, hyposplenism
What are the types of hernias?
- indirect inguinal,
- umbilical,
- epigastric,
- femoral
What is the pathophysiology of an indirect inguinal hernia?
- During development, the testicles develop inside the abdomen and towards the end of the pregnancy, each testicle creates a passage (process vagialis) as it travels into the scrotum
- Failure of this passage to close → abdominal lining and bowel protrude through defect
- If bowel remains trapped → could become damaged due to increased pressure on the blood supply to the area bowel death serious infection and bowel disorders
What are the risk factors for indirect inguinal hernias?
- male,
- prematurity,
- connective tissue disorders
What are the differentials for indirect inguinal hernias?
- hydrocele (fluid, this transluminates so use a torch to differentiate)
What are the signs and symptoms of an indirect inguinal hernia?
- Scrotal sac enlarged, contains palpable loops of bowel, fluid (does not always transluminate) ± pain
- Swelling or bulge may be intermittent, and can appear on crying or straining
What are the investigations for indirect inguinal hernia?
- Clinical diagnosis
- Determine type of hernia –> examine supine and standing, try to reduce it
• If incarcerated → tender, firm mass + vomiting, obstruction (unable to pass stool), poor feeding, erythematous/discoloured skin overlying
• More commonly on right (60%) due to delayed descent of right testicle
What is the management of indirect inguinal hernia?
Urgent Surgical correction (lap or open) = Elective herniorrhaphy (risk of strangulation/incarceration)
• <6w old - correct within 2 days
• <6m old - correct within 2 weeks
• <6yo - correct within 2 months
What is an umbilical hernia risk factors?
- Afro-Caribbean,
- Down’s,
- Mucopolysaccharide diseases
What is an umbillical hernia?
Common in new-borns and often resolve by 12m (watch and wait are appropriate)
What is the management of umbillical hernias?
- <1yo –> watch and wait
- >1yo –> large or symptomatic = surgical repair 2-3yo; small or asymptomatic = surgical repair 4-5yo
What is an umbillical granuloma?
wet, moist and leaks fluid –> treat with salt initially and can be later cauterised with silver nitrate
What is a femoral hernia?
- Difficult to differentiate from indirect
- Located below inguinal canal (through femoral canal)
- Differentiation often made during operation
- S/S same as for indirect inguinal hernia
What is gastroschisis?
- paraumbilical abdominal wall defect –> abdominal contents outside body, without peritoneal covering
What is the management of gastroschisis?
Manage with immediate surgery (cover with cling-film) “Gastro-ski-sis”
What is an omphalocele/exomphalos?
- bowel protruding out the body with a peritoneal covering / umbilical attached
What is the management of omphalocele/exomphalos?
- Manage with staged closure starting immediately, finishing at 6-12 months
- Chromosomal abnormalities in 15% of cases (Trisomy 13 (Patau’s), 18 (Edward’s), 21 (Down’s); Turner’s
What is encopresis?
- soiling of underwear with stool in children who are past the age of toilet training (>4yo)
- Usually due to constipation with overflow
What is the management of encopresis?
- enquire about psych stressors, changes in medications, food intolerances, etc.
What is acute liver failure?
- Massive hepatic necrosis with loss of liver function ± hepatic encephalopathy
- Majority from paracetamol overdose, infection and metabolic disease
What are the signs and symptoms of acute liver failure?
- Jaundice
- Encephalopathy (alternative irritable –> confusion/drowsiness episodes)
- Coagulopathy
- Hypoglycaemia
- Electrolyte disturbance
- Older children (aggressive, unusually difficult)
What are the investigations for acute liver failure?
- LFTs – early BR normal (esp. in metabolic disease) AST/ALT (very high) ALP (high)
- Liver FUNCTION = INR (using PT)
- Liver INFLAMMATION = AST and ALT
- Clotting – abnormal
- Plasma ammonia raised
- EEG and CT – acute hepatic encephalopathy and cerebral oedema
What is the management of acute liver failure?
Referral to a national paediatric liver centre
Steps to stabilise the child:
- Maintaining blood glucose (> 4 mmol/L) with IV dextrose
- Preventing sepsis with broad-spectrum antibiotics and antifungals
- Preventing haemorrhage with IV vitamin K and H2 antagonists/PPIs
- Prevent cerebral oedema by fluid restriction and mannitol diuresis
- Management is dependent on the suspected cause of acute liver failure
What are the features of poor prognosis in acute liver failure?
- Shrinking liver - Rising bilirubin
- Coma
- Falling transaminases
- Worsening coagulopathy
What are the complications of acute liver failure?
- Hepatic encephalopathy –> supportive, reduce N2 load (lactulose, ABx)
- Cirrhosis, portal HTN –> fluid restrict, diuretics
What antibodies does PBC have?
anti-mitochondrial AB
What antibodies does PSC have?
pANCA, anti-smooth muscle AB
What is the management of autoimmune hepatitis?
- Prednisolone and azathioprine
- Sclerosing cholangitis –> ursodeoxycholic acid (aids bile flow)
- Liver transplants may be considered in severe cases
What is the management of Wilson disease?
- Zinc (blocks intestinal copper resorption)
- Trientine / Penicillamine (increases urinary copper excretion)
- Pyridoxine (vitamin B6; given to prevent peripheral neuropathy – improvement may take up to 12m)
- Symptomatic treatment for tremor, dystonia and speech impediment
- Liver transplantation - considered in children with end-stage liver disease
What is the management of non-alcoholic fatty liver disease?
- Weight loss (and bariatric surgery)
- Treatment of insulin resistance and diabetes
- Statins
- Vitamin E and C
- Ursodeoxycholic acid (improved bile flow)
What is the management of paracetamol overdose?
<1 hour –> activated charcoal –> Ix: paracetamol level ≥4 hours after ingestion –> NAC if indicated
> 1 hour –> Ix: paracetamol level ≥4 hours after ingestion –> NAC if indicated
(NAC = N-acetylcysteine)
What is the management of toxoplasmosis in a child?
- 1st line: Pyrimethamine + Sulfadiazine for 1 year
- Adjunct: Prednisolone
What is the management of syphilis in a child?
- IM benzathine penicillin
What is the management of Pavrovirus in a child?
- Intrauterine –> blood transfusion if foetal hydrops
- Infant –> self-limiting
What is the management of VZV in a child?
- IV aciclovir
- Neonatal ophthalmic examination
- Monitored until 28 days after maternal infection
What is the management of HIV in a child?
- Cord clamped as soon as possible and baby bathed immediately after birth
- Zidovudine monotherapy for 2-4w (low/medium risk) OR 4w PEP combination (high risk)
- Women not to breastfeed
- Give all immunisations including BCG (unless a moderate-high risk of transmission)
- PCR HIV virions at 6 and 12 weeks (at least 2 and 8 weeks after stopping prophylaxis)
• Baby will have passive IgG from the mother up until at least 6 months
What is the management of Rubella in a child?
- Refer to foetal medicine unit and notify HPU
- No effective treatment (rest, adequate fluids, simple analgesia)
- Infant –> cardiac scans, hearing tests
What is the management of CMV in a child?
IV ganciclovir / oral valganciclovir
What is the management of HSV in a child?
Aciclovir (400mg, TDS) –> if neonate exposed on delivery
What is the management of HepB in a child?
Acute HBV infection –> not dangerous
Chronic HBV with cirrhosis –> IUGR and prematurity
- HBV vaccination (for infants of mothers who are HBsAg positive)
- Mother: Tenofovir disoproxil OR lamivudine (mothers should receive antiviral monotherapy)
- No risk of transference through breastfeeding
- Baby: passive immunisation in HBV IG and HBV vaccine [within 12 hours of birth]
What is the management of GroupB Strep in a child?
GOSH child ABx:
- Early onset <72 hours –> GBS and L. monocytogenes –> IV cefotaxime + amikacin + ampicillin
- Late onset >72 hours –> CoNS (S. epidermidis) –> IV meropenem + amikacin + ampicillin
- Mother ABx (during labour):
- -> IV benzylpenicillin
What does listeria monocytogenes cause in a neonate?
An important cause of neonatal sepsis; mother has a mild influenza-like illness and passes to child in placenta
- Can cause spontaneous abortion, PTL, neonatal sepsis
- Characteristics: meconium staining of liquor in pre-term infant, widespread rash, sepsis, pneumonia, meningitis
- Mortality 30%
What is the management of listeria monocytogenes in a child?
- IV amoxicillin/ampicillin OR co-trimoxazole (trimethoprim contraindicated in pregnancy)
- If systemic infection: IV benzylpenicillin + gentamicin
What is kawasaki disease?
Systemic vasculitis; children 6m to 4yo (peak 1yo); Japanese, Black-Caribbean ethnicity
What are the signs and symptoms of kawasaki disease?
FEVER +/- 4/5 other features:
C - Conjunctivitis
R - Rash (polymorphous; begins hands/feet)
A - Adenopathy (cervical lymphadenopathy)
S - Strawberry tongue
H - Hands & feet swollen (and desquamate/peel)
Burn - Fever >5 days (not responsive to antipyretics)
What are the investigations for Kawasaki disease?
- Diagnosis on clinical findings (no test)
- FBC (inc. platelets), CRP, ESR
- Echocardiography
What is the management of kawasaki disease?
ADMISSION
- IVIG (within 10 days) + high-dose aspirin (reduce thrombosis risk; treatment (high) –> prophylaxis (low) dose)
- Other: corticosteroids, infliximab/ciclosporin and plasmapheresis
- I.E. for persistent inflammation and fever
What are the complications of kawasaki disease?
Children with coronary aneurysms may require long-term warfarin and close follow-up
What organism is responsible for malaria?
Protozoa Plasmodium, spread by female Anopheles mosquito
- Falciparum most fatal (ovale, malariae and vivax also exist)
What are the signs and symptoms of malaria?
- Onset 7-10 days after inoculation (<1yr)
- Fever (cyclical / continuous with spikes)
- D&V
- Flu-like symptoms (shaking, chills, night sweats, headache, myalgia)
- Jaundice, anaemia - Thrombocytopaenia
- Particularly susceptible to cerebral malaria, severe anaemia
What are the investigations for malaria?
- 3 thick and thin blood films (thick = parasite; thin = species, parasitaemia)
- Malaria rapid antigen detection tests (plasmodial HRP-II, parasite LDH)
What is the management for malaria?
Prevention:
- Anti-malarial prophylaxis with quinine
- Bite prevention – repellent and nets
Arrange immediate admission –> Medical emergency; notify PHE (can have a RAPID deterioration)
Treatment (very variable, dependant on strain, severity, tolerability, resistance):
1) Non-falciparum:
- 1st line = Chloroquinine
2) Mild falciparum (Mild = if not vomiting, parasitaemia <2% and ambulant):
- 1st line = ACT (Artemisinin Combination Therapy)
- 2nd line = Atovaquone-proguanil (cannot give doxycycline to age <12yo)
3) Severe/complicated falciparum:
- 1st line = IV Artesunate (N.B. anti-malarial drugs may precipitate G6PDD)
- 2nd line = IV Quinine
Primaquine (not G6PDD) for eradication of hypnozoites (dormant parasites in liver in vivax and ovale)
What organism cause typhoid fever?
Salmonella typhi or paratyphoid
What is the transmission of typhoid fever?
Faeco-oral transmission
What are the complications of typhoid fever?
GI perforation, myocarditis, hepatitis, nephritis
What are the signs and symptoms of typhoid fever?
- Fever (+ bradycardia)
- Headache
- Cough (dry)
- Anorexia (WL +++)
- Malaise, myalgia
- GI symptoms (by 2nd week; diarrhoea or constipation)
- Splenomegaly, bradycardia, rose-spots on trunk
What is the buzzword for typhoid fever?
TRAVEL: Pakistan, India, Bangladesh
What are the investigations for typhoid fever?
- Blood culture [diagnostic]
- Other – FBC, LFTs, stool culture
What is the management of typhoid fever?
- 1st line = IV ceftriaxone OR IV cefotaxime
- 2nd line = PO azithromycin
What organism causes dengue?
Caused by dengue arbovirus transmitted by Aedes Aegyptii mosquito
- Flavivirus
- Short incubation period (~5 days)
- Usually imported from SE Asia & South Africa
What are the signs and symptoms of dengue fever?
Low WCC, low plts, low Hb:
o Primary infection:
- Headache (retro-orbital)
- Fine erythematous sunburn-like rash (50%)
- High fever and myalgia
o Other = hepatomegaly, abdominal distension
o Severe = low WCC, low platelets, haemorrhage
What is the investigations for dengue?
- Gold standard – PCR viral antigen, serology IgM
- FBC, LFTs, serum albumin
What is the management of dengue?
- Supportive (fluids and monitoring)
- ITU (if increased deterioration)
What is dengue hemorrhagic fever?
A secondary infection:
- Previously infected child –> subsequent infection (different strain) –> severe capillary leak, hypotension, haemorrhagic manifestations –> fluid resuscitation usually helps a lot
- Due to partially effective host immune response augmenting the severity of the infection
What is mumps?
Mumps paramyxovirus
- Transmission by respiratory secretions
- Long incubation period (15-24 days)
- Infectious 5 days before and 5 days after the parotid swelling (should pass totally in 1-2 weeks)
- Can still get infected if they have had the vaccine but likely to be reduced clinical symptoms
What are the signs and symptoms of mumps?
- Asymptomatic (in 30% of cases)
- Headache, fever, parotid swelling
- Other – pancreatitis, neuritis, arthritis, mastitis, nephritis, thyroiditis, pericarditis
What are the investigations for mumps?
- Oral fluid IgM sample
- Amylase is raised in the blood
What is the management of mumps?
- Notify HPU, isolate for 5 days from time of parotid swelling
- Advise and educate –> supportive care (rest, analgesia and fluids) as this is a ‘viral’ infection
What is the safety net complications for mumps?
- Mumps orchitis –> infertility (very rare)
- Viral meningitis –> encephalitis (very rare)
- Deafness (unilateral and transient)
What is measles?
Paramyxovirus, spread through respiratory secretions
- One of the most highly communicable diseases (>15 mins in direct contact is enough to transmit)
- Incubation = 7-18-days
- Infective period = 4 days before and 4 days after rash
What are the signs and symptoms of measles?
- Prodrome: high fever, irritability, conjunctivitis/coryza
( can cause Febrile convulsions) - Maculopapular rash (face/neck –> hands/feet)
- Koplik spots (small white spots surrounded by red ring in mouth)
- Cough
- No lymphadenopathy
What are the investigations for measles?
- 1st line = Measles serology (IgM and IgG) from Oral Fluid Test – OFT
- 2nd line = PCR of blood or saliva
What is the management of measles?
- Notify HPU, isolate for 4 days after development of rash
- Rest and supportive treatment (fluids, antipyretics, rest)
- -> Children isolated in hospital
- Immunise close contacts and encourage vaccination after acute episode
What is the safety net complications for measles?
- Encephalitis (1 in 5,000) – after 1-2w: • Headaches, lethargy • Irritability seizures • Coma = 15% death - SSPE (1 in 100,000) after 7 years • Sub-acute Sclerosing Panencephalitis • Measles dormant in the CNS • S/S: dementia and death - Otitis media (most common complication) - Pneumonia (most common cause of death) - Keratoconjunctivitis
What is rubella?
German measles’ caused by a Togavirus, spread through sneezing/coughing (SIMILAR TO MEASLES)
- Incubation period = 6-21 days
- Infective period = 1 week before to 5 days after rash onset
- Illness = for 7-10 days
What are the signs and symptoms of rubella?
- Prodrome = mild fever / asymptomatic
- Pink maculopapular rash (face –> whole body), fades in 3-5 days
(20% –> Forchheimer spots red spots on soft palate) - Lymphadenopathy (suboccipital, postauricular)
- No Koplik spots or conjunctivitis
What are the investigations for rubella?
- Rubella serology (IgG and IgM) from oral fluid test
- RT-PCR (2nd line)
What is the management of rubella?
- Notify HPU, isolate for 4 days after development of rash
- Supportive (virus; fluids, analgesia, rest)
What is the safety net complications for rubella?
Haemorrhagic complications due to thrombocytopenia
What is erythema infectiosum?
- “Fifth disease / erythema infectiosum / B19 / Slapped Cheek”
- Transmission via respiratory secretions,
- Infects RBC precursors in BM
- Infectious period = 10 days before to 1 day after rash develops
What are the signs and symptoms of erythema infectiosum?
- 1st: Asymptomatic OR coryzal illness for 2-3 days –> latent for 7-10 days
- 2nd: Erythema infectiosum (most common):
(1) Red ‘slapped cheek’ rash on face (viraemic phase of fever, malaise, headache, myalgia)
(2) Progresses (1 week later) to maculopapular (lace) like rash in trunk and limbs - Aplastic crisis – occurs in children with chronic haemolytic anaemia (sickle cell) or immunodeficient
- Fetal disease – maternal transmission – leads to fetal hydrops, death due to severe anaemia
What is the investigations for erythema infectiosum?
- B19 serology (IgG and IgM) –> n.b. similar to rubella
- RT-PCR (2nd line)
What is the management of erythema infectiosum?
- Supportive (virus; fluids, analgesia, rest) –> will clear in ~3 weeks
- No need to stay off school or avoid pregnant women (as not really infectious once the rash develops)
What is the safety net complications for erythema infectiosum?
Anaemia, lethargy, complications to pregnancy
What is chickenpox?
Varicella zoster virus (HHv-3) – reactivation of dormant virus after chickenpox leads to herpes zoster (shingles)
- Incubation period = 10-21 days
- Infectious period = 48 hours before rash to last crusted over lesion / 5-7 days after rash appears
What are the signs and symptoms of chickenpox?
- Pyrexia, headache, abdominal pain, malaise
- Crops of vesicles appear over 3-5 days:
- Head, neck, trunk (less on limbs) – itchy
- Papule → vesicle → crust – several stages at once
What is the investigation for chickenpox?
Clinical diagnosis
What is the managment of chickenpox?
- Supportive (virus; fluids, analgesia (no ibuprofen), rest)
- Advice – nails short, loose clothing, infectious period = 1-2 days before rash to last crusted over lesion
Isolate from:
- Immunocompromised
- Neonates (<28d old)
- Pregnant women - Keep home from school
What are the special cases of chickenpox?
Admit if serious complications (e.g. pneumonia, encephalitis, dehydration)
- Secondary bacterial superinfection –> sudden high fever: toxic shock, necrotising fasciitis
- Encephalitis (ataxic with cerebellar signs; better prognosis than HSV-encephalitis)
- Purpura fulminans –> large necrotic loss of skin from cross-activation of antiviral ABs –> inhibit the inhibitory coagulation proteins factors C and S –> increased clotting and purpuric skin rash
- Dehydration (severe)
Immunocompetent adolescents/adults –> oral aciclovir 800 mg 5/day for 7 days (if <24hrs of rash)
Immunocompromised children –> IV aciclovir –> oral aciclovir
• Prophylactic prevention = human VZV IVIG
What is the causative organism of hand foot and mouth disease?
- Most commonly due to Coxsackie A16 virus • (SEVERE: Enterovirus 71)
- (Atypical: Coxsackie A6)
- Common under 10yo; very contagious
Do you need to be kept out of school for hand foot and mouth disease?
• Don’t need to be kept from school but HPA recommend they do until they feel better
What are the signs and symptoms of hand foot and mouth disease?
o Painful, itchy, vesicular lesions (hands, foot, mouth, tongue, buttocks)
o Mild systemic features – fever, sore throat, spots in mouth → develop into ulcers
What is the management of hand foot and mouth disease?
o Supportive (virus; fluids, analgesia, rest) --> will clear in 7-10 days o Safety net: dehydration, if it doesn’t clear up in 2 weeks, pregnancy
What is roseola infantum?
o HHV6 and HHV7 can present very similarly (HHV6 is more common = Roseola Infantum)
o Most children infected by age 2 (6m to 2yo) – highly infectious, infective during whole period of disease
o N.B. infected very YOUNG compared to other infections
What are the signs and symptoms of roseola infantum?
o High fever and malaise (3-4 days) –> generalised macular (small pink spots) rash (appears as the fever wanes)
- Rash starts on neck/body and spread to arms, lasting 1-2 days, non-itchy, blanching
- Many have a febrile illness and never develop a rash; commonly misdiagnosed as measles/rubella
- Febrile convulsions in 10-15%
o Sore throat, lymphadenopathy, coryzal symptoms, D&V
o Nagayama spots (spots on the uvula and soft palate)
o N.B. lack of Koplik spots (white spots on buccal mucosa)
What are the investigations for roseola infantum?
o HHV6/7 serology (IgG and IgM)
o Measles & rubella serology (similar presentation)
What is the management of roseola infantum?
o Supportive (virus; fluids, analgesia, rest) –> will clear in ~1 week
o No need to stay off school
o Safety net the complications – high fever –> febrile convulsions (10-15%)
Do you need to stay off school with roseola infantum?
No need to stay off school
What are the investigations for HIV in a child?
o Children >18 months –> antibody detection (ELISA)
o Children <18 months (still have transplacental anti-HIV IgG from mother) –> PCR of virus
- Measured at birth, on discharge, 6 weeks, 12 weeks and finally, at 18 months
What is the management of HIV in a child?
o Cord clamped as soon as possible and baby bathed immediately after birth
o Zidovudine monotherapy for 2-4w (low/medium risk) OR 4w PEP combination (x2 NRTI + x1 INI; high risk)
o Women not to breastfeed
o Give all immunisations including BCG (unless a moderate-high risk of transmission)
o Infant testing for HIV at 6 and 12 weeks (at least 2 and 8 weeks after stopping prophylaxis)
What are the signs and symptoms of Hyper IgM (Job/Buckley) syndrome?
Eczema,
coarse facial features,
recurrent RTIs,
cold abscesses, candidiasis
What is ataxia telangestasia?
o Defective DNA repair
o Increased risk of lymphoma
o S/S: cerebellar ataxia, developmental delay, telangiectasia in the eyes
What is Wiskott-Aldrich syndrome?
X-linked –> impaired Wiskott-Aldrich gene; rare; ~7m old
What are the signs and symptoms Wiskott-Aldrich syndrome?
o S/S: eczema, recurrent infections, thrombocytopenia (petechiae, bloody diarrhoea)
- “WATER” = Wiskott-Aldrich Thrombocytopenia Eczema Recurrent infections
- May look like ITP (however, Wiskott-Aldrich would present much younger; ITP = ~4yo)
- Raised = IgA, IgE –> eczema
- Low = IgG, IgM –> recurrent infections
What is the management of Wiskott-Aldrich syndrome?
IVIG –> HSCT
What is Duncans disease?
o X-linked lymphoproliferative disease
o Inability to generate a normal response to EBV
o S/S: death to initial EBV, or develop a secondary B-cell lymphoma
What is a primary allergy?
- child has failed to develop immune tolerance
- Infants → milk (cow’s milk), egg, peanut
- Older children → peanut, fish, shellfish
What is a secondary allergy?
initially tolerate –> become allergic later
- Cross-reactivity between proteins in fruit/nuts and pollen → “oral allergy syndrome”
What are the signs and symptoms of an IgE mediated allergy?
urticaria, facial swelling (angioedema), rash, erythema, nausea, D&V, colicky abdominal pain, nasal itching, sneezing, rhinorrhoea, congestion, cough, tightness, wheeze –> ANAPHYLAXIS in 10-15 mins
What are the signs and symptoms of an non-IgE mediated allergy?
erythema, atopic eczema, GORD, change in frequency of stools, blood/mucus in stools, abdo pain, FTT, infantile colic, constipation, food aversion, pallor
What are the investigations for food allergies?
o Allergy-focussed clinical history:
- Classify the reaction – speed of onset, age of onset, severity, location, reproducibility, history
- Atopic history (personal or FHx)
- Food diary
- Details of food avoidance and why
- Details of any feeding history (age of weaning, breast/formula)
- Cultural/religious factors surrounding food
- Any previous trial elimination of suspected allergen for 2-6 weeks then reintroduction
o Test 1: Skin prick allergy testing (supports an allergy-focussed history and can confirm diagnosis);
o Test 2: Measurement of specific IgE antibodies (RAST)
When do you refer to a specialist for food allergy?
- Faltering growth with ≥1 GI symptoms of allergy
- ≥1 acute systemic or severe delayed reactions
- Severe atopic eczema - Persisting suspicion
- Multiple allergies
What is the management of food allergy?
o Specialist care (if indicated, as above)
o Avoid relevant foods
o MDT – advice from paediatric dietician to avoid nutritional deficiencies
o Teach family and child how to manage an allergic attack (Allergy Action Plan)
- Written information/leaflet + adequate training
• Explain what an allergy is
• Explain some children grow out of allergies (i.e. cow’s milk protein)
- Mild attacks –> antihistamines (i.e. loratadine)
- Severe attacks –> EpiPen (IM adrenaline)
What are the signs and symptoms of allergic rhinitis?
o Coryza, conjunctivitis, chronically blocked nose
o Sleep disturbance, impaired daytime behaviour/concentration
What are the investigations for allergic rhinitis?
To exclude other causes:
o Identify any co-existent asthma (or other atopy)
o Examine nose for nasal polyps, deviated nasal septum, mucosal swelling or depressed or widened nasal bridge
What is the management of occasional allergic rhinitis?
Occasional symptomatic relief:
- 2-5yo → give oral (or liquid if young) antihistamine (cetirizine, loratadine) as required
- Any age: intranasal Azelastine
What is the management of frequent allergic rhinitis?
Frequent symptomatic relief:
- Advise to avoid causative allergen
- Main issue (nasal blockage / polyps) –> intranasal corticosteroid (beclomethasone)
- Main issue (sneezing / discharge) –> intranasal corticosteroid or oral antihistamine
What is specific allergen immunotherapy?
(SCIT = Sub-Cutaneous Immunotherapy)
Used to treat:
• Allergic rhinitis and conjunctivitis
• Insect stings
• Anaphylaxis • Asthma
- Solutions of an allergic allergen are injected SC or sublingually on a regular basis for 3-5 years
- It can provide protection for many years but has a risk of inducing anaphylaxis (specialist supervision)
What is cow milk protein allergy?
- Common (3-6%)
- Can be immediate (IgE mediated) or delayed – usually presents in first 3m of life in formula-fed children
- Infants that are breast-fed can still get a reaction from proteins that the mother eats passing to breast milk
What is the classical symptom of cow milk protein allergy?
3-month-old baby that vomits and has diarrhoea after every feed
What is the investigation of cow milk protein allergy?
Same as food allergy
What is the management of cow milk protein allergy?
1st: Trial cows’ milk elimination from diet for 2-6 weeks:
- Breastfed Babies: mother to exclude cow’s milk protein from her diet
- Consider prescribing daily supplement of 1g of calcium and 10 mcg of vitamin D
- N.B. it takes 2-3 weeks to fully eliminate cow’s milk from breastmilk
- Formula-fed Babies: replacement of cows’ milk-based formula with hypoallergenic infant formula (e.g. extensively hydrolysed formula or amino acid formula –> if SEVERE: use amino-acid based formula)
- Weaned infants/older children: exclude cows’ milk protein from their diet
2nd: Regularly monitor growth, nutritional counselling with a paediatric dietician
3rd: Re-evaluate tolerance to cows’ milk protein (every 6-12 months) –> re-introduce cows’ milk protein into the diet –> if tolerance is established, greater exposure of less processed milk is advised with ‘Milk Ladder’
What is croup?
“Viral laryngotracheobronchitis”
• Affects 6m to 6yo (peak 2yo), autumn
What are the causes of croup?
o Main cause = Parainfluenza
o Other causes = RSV, rhinovirus, influenza
What are the risk factors of croup?
- FHx
- LBW/prematurity
- Autumn/winter
- M>F
What are the differentials for croup?
- laryngomalacia,
- acute epiglottitis,
- inhaled foreign body
What are the signs and symptoms fo croup?
- Coryal symptoms
- Barking cough (from vocal cord impairment)
- Stridor (from inflamed/oedematous airways)
What are the investigations of croup?
- Clinical
- DO NOT EXAMINE THROAT
What is the scoring chart for croup?
Westley score - helps determine admission, treatment
Mild: 0 –> 2:
- Oral dexamethasone + discharge
(“With oral dexamethasone, the symptoms should resolve within 48 hours”)
Moderate: 3 –> 7:
- ADMIT + dexamethasone
Severe: 8 –> 11:
- ADMIT + dexamethasone ± adrenaline
Impending respiratory failure (RR >70): 12 –> 17:
- ADMIT + dexamethasone ± adrenaline
When do you consider admission for croup?
- RR>60
- Other co-morbid condition
- Toxic looking
- Age <3m
What are the medications for treatment of croup?
- 1st line: Oral dexamethasone (fluid suspension)
- 2nd line: Nebulised budesonide or IM dexamethasone
- Respiratory distress (RR >70) –> Neb adrenaline + O2
Give adrenaline first line if they are SEVERE as they wouldn’t be able to swallow dexamethasone
What is the complications of croup?
- secondary bacterial
- superinfection,
- pulmonary oedema,
- pneumothorax
What is acute epiglottis?
Intense swelling of epiglottis associated with sepsis:
- most common ages 1-6yo (can affect all ages)
What is the causative organism of epiglottitis?
Haemophilus influenza type B (HiB)
- uncommon due to vaccination
What are the signs and symptoms of acute epiglottis?
MEDICAL EMERGENCY: N.B. no cough (like in croup)
- High fever (‘toxic-looking’ child)
- Drooling (child cannot swallow as too sore)
- Stridor (soft inspiratory with high RR)
- Immobile, upright and open mouth “Tripod sign”
What is the investigations and management of epiglottis?
MEDICAL EMERGENCY
- Do not lie the child down (their immobile and upright stance is optimal)
- Do not examine the child’s throat (may precipitate total obstruction)
(1) Immediate referral to ENT, paediatrics and anaesthetics –> transfer to ITU/anaesthetics –> secure airway
(2) Blood cultures and empirical ABx (cefuroxime) ± dexamethasone (reduce inflammation)
o Most children recover in 2-3 days
o Rifampicin given to close household contacts as prophylaxis
What is bronchiolotis?
- 2-3% of all infants admitted each year; peak in winter
- <1yo (90% between 1m and 9m; 3-6m peak)
What is the causative organism for bronchiolitis?
Cause = RSV (80%), parainfluenza, rhinovirus, adenovirus, influenza, human metapneumovirus (rare; but PICU care)
Co-infection –> more severe illness
- Bronchiolitis (0-1yo) –> VIF (1-5yo) –> Asthma (>5yo)
- RSV highly infectious so infection control measures
- RFs: pre-term/BPD, passive smoking, LBW, chronic heart disease, hypotonia, (BREASTFEEDING PROTECTIVE)
- Rarely, the illness may cause permanent damage to the airways (bronchiolitis obliterans)
What are the signs and symptoms of bronchiolotis?
- 1st = Coryzal symptoms (which progresses to…)
- 2nd = ± dry wheezy cough, SoB, grunting, high RR/HR
- Subcostal/intercostal recessions, hyperinflation
- Auscultate – how to differentiate from croup/other ‘-itits’
- Fine, bi-basal, end-inspiratory crackles
- High-pitch wheeze (exp > insp)
- Feeding difficulty (from SoB) –> admission
What are the investigations for bronchiolotis?
- clinical diagnosis with SpO2… but can do NPA to confirm
- If there is significant respiratory distress + fever –> carry out a CXR to help rule out pneumonia
What is the management of bronchiolitis?
Hospital admission if:
- <2m old, lower threshold as deteriorate quick:
- Apnoea / central cyanosis / grunting
- SpO2 <92% on room air
- Poor oral fluid intake (≤50% normal in <24hrs) - Severe respiratory distress (i.e. RR>70)
Supportive (nasal O2 + NG fluids/feeds ± nebulised 3% saline) –> CPAP (if respiratory failure)
- <6m old = no beta receptors in lungs so salbutamol won’t work – would give it if over 1yo
If high-risk preterm infant (BPD, congenital HD, immunodeficiency) –> palivizumab (monoclonal AB against RSV)
