Paeds Flashcards
What is child abuse?
Action by another person that causes significant harm to a child in a given culture in a given time
What are the different categories of abuse?
Physical
Emotional
Neglect
Sexual
Who has responsibility for child protection?
Everyone
What is a serious case review?
When child either dies or is seriously harmed after previous involvement with social services/police
If you find that a child under 13 is having sex what should you do?
Sexual intercourse under age of 13 - statutory rape and must be referred to social services
What is a CSE screening tool?
Child sexual exploitation screening tool
Enable professionals to assess child’s (under 18) level of risk of CSE in a quick and consistent manner
What are deemed to be high risk behaviours when using the child sexual exploitation screening tool?
Internet use Financial rewards Adult mobile phone apps Episodes of going missing Socially adverse circumstances High risk locations New friends Older relationships Drug and alcohol misuse Gum clinic attendances Pregnancies STIs
Which medical condition could be confused for a cigarette burn in a child?
Bullous impetigo
How should you escalate things if you are worried about a child protection issue?
Discuss with senior Discuss with Paeds Discuss with social care Documentation Communication Photographs Body maps Re-examination at later date Consider differences of opinion
What is a common assessment framework?
Structured approach to supporting the family of a child
Allows input from a variety of services
May avoid need for more formal child protection procedures
What are the 3 sides of the CAF triangle?
Child’s developmental needs: health, education, identity
Parenting capacity: stimulation, ensuring safety, guidance and boundaries
Family and environmental factors: housing, employment, family’s social integration
A male neonate is reviewed on the postnatal ward eight hours after an uncomplicated vaginal delivery at 38 weeks gestation. The paediatrician notices that his sclerae and skin appear yellow. Give causes of neonatal jaundice relevant to this patient
Rhesus haemolytic disease ABO incompatibility G6PD deficiency Congenital spherocytosis Sepsis - TORCH infections Gilbert's syndrome Crigler-Nijar syndrome
What is the most important immediate blood test which will help determine management in a case of neonatal jaundice?
Bilirubin levels - conjugated/unconjugated
A male neonate is reviewed on the postnatal ward eight hours after an uncomplicated vaginal delivery at 38 weeks gestation. The paediatrician notices that his sclerae and skin appear yellow. List investigations that should be requested in this patient to help determine the cause of the jaundice?
Bilirubin FBC Blood film Blood group - ABO Rhesus status Direct antiglobulin/Coombs test LFTs G6PD enzyme test Blood culture Urine dipstick/MC and S Viral serology - TORCH/hepatitis TORCH screen
Give interventions for managing neonatal jaundice. How would you decide which of these to use?
Phototherapy
Exchange transfusion
Plot bilirubin levels and look at relationship to nomogram/treatment lines
What serious complication of neonatal jaundice may develop if left untreated?
Kernicterus (bilirubin encephalopathy)
What do you look for on observation of an infant in a neuro examination?
Posture: flexed? Frog-like? Extended? Asymmetrical?
Supine: moving all 4 limbs against gravity, tremors, hand clenching, thumb adduction, rhythmic mouthing, cycling or swimming movements of the limbs?
Ventral Suspension and Pull to Sit/Stand
Hand regard and playing with feet. Visually active?
Attempting to roll? Any abnormal movement / muscle wasting etc?
What do you need to examine in an infant top to toe exam of the head in a neuro exam?
Palpate head (Fontanelles, Sutures) and consider shape
Plot head circumference on a centile chart, Compare with previous measures- micro or macrocephaly?
Signs of Meningism (often subtle in younger children/infants)
Eyes: visually alert, fixes and follows, any nystagmus/squint/cataract? Reactive to light? Ptosis?
Face: Dysmorphism, facial asymmetry. Any reciprocal smile? Cleft
palate?
Vocalising and cooing? Turning towards noise?
What neuro cutaneous stigmata might you look for in an infant neuro exam?
Depigmented patches
Café-au-lait spots
Trigeminal port-wine stain
By when should the Moro reflex integrate?
2 to 4 months
By when should the rooting reflex integrate?
3 to 4 months
By when should the palmer reflex integrate?
5 to 6 months
By when should the ATNR reflex integrate?
6 months
By when should the spinal gallant reflex integrate?
3 to 9 months
By when should the TNR reflex integrate?
3 and a half years
By when should the landau reflex integrate?
1 year
By when should the STNR reflex integrate?
9 to 11 months
What is the palmer reflex?
Palmar grasp - primitive
When object placed in infants hand and strokes palm, fingers close and they grasp it
What is the ATNR reflex?
Asymmetrical tonic neck reflex
Fencing reflex - face turned to one side when lying on back - arm and leg on same side extend, opposite arm and leg flex
What is the spinal galant reflex?
Baby curves hip outward if lower back stroked next to spine
What is the TLR reflex?
Tonic labyrinthine reflex
Supine- Titling head back while lying on back causes back to stiffen and arch backwards, legs straighten stiffen and push together, toes point, arms bend at elbows and wrists and hands become fisted
Prone - hip knee flexion with shoulder protraction and further flexion
What is the landau reflex?
Hold baby in air in prone position
Maintain convex arc with head raised and legs slightly flexed
What is the STNR reflex?
Symmetrical tonic neck reflex
Important in transition from lying on floor
When head flexes, arms flex, legs extend
When head extends, arms extend and legs flex
What do you assess in the limbs in a neuro exam of an infant?
Look for asymmetry in tone, bulk and power
Assess by degree of elbow and knee flexion during arm and leg traction. Note the speed of recoil
Look for coordination and function during play and use of objects
Examine hips for increased adductor tone
Look for clonus: sudden stretching on hypertonic muscle produces reflex contraction- a measure of reflex excitability
What do you assess in the neck and trunk in a neuro exam of an infant?
Head control is assessed by the ability to return the head to
the vertical after allowing it to fall forwards and then back in asitting position. Assess head control
Assess flexor and extensor muscles when pulling to sit and in ventral suspension
Is posture developmentally appropriate for age?
What primitive and postural reflexes are assessed for in an infant neuro exam?
Suck Rooting Grasp Moro Asymmetrical tonic neck Plantar Stepping Parachute
What are key areas of a paediatric history?
Antenatal
Birth history
Developmental history
Family history
What are important parts of a general examination in a child?
Head circumference - parents and child Distinctive features - dysmorphism, special needs Assess growth - height and weight Skin - neurocutaneous markers, scars Eye - movements, asymmetry, red reflex, squint, vascular lesions Spine - scoliosis, scars Abdomen - scars, organomegaly Equipment/aids
What are important examination assessments in a newborn?
Head circumference and shape Posture Movement Limb and truncal tone Reflexes and neuro behaviour - sleep/cry/feeding/interaction
What further assessment should be done for a neuro exam in an infant?
Any hand dominance?
Can the infant support weight when prone?
Check spine for kyphoscoliosis, meningomyelocoele, sacral pit, tuft of hair
Can the infant weight bear? Crawl, Cruise? Walk with support?
Do the findings match with the child’s chronological and expected developmental age in all areas?
What are common CNS causes of hypotonia in a child?
Chromosome disorders i.e. Prader-Willi Metabolic diseases Spinal cord injuries Cerebral dysgenesis Hypoxic ischaemia injuries
What are common nerve causes of hypotonia in a child?
Congenital hypomyelinating neuropathy
Familial dysautonomia
Infantile neuraxonal degeneration
What are common neuromuscular junction causes of hypotonia?
Congenital and transient myasthenia gravis
Infantile botulism
What are common muscle causes of hypotonia?
Muscular dystrophies
Metabolic myopathies
Central core disease/ fibre myopathies
If a baby is floppy and strong, where is the problem?
Central nervous system
If a baby is floppy and weak where is the problem?
Peripheral nervous system
What are important aspects in a history of a floppy baby?
Reduced foetal movements Polyhydramnios Needing prolonged resuscitation/artificial ventilation Poor feeding/choking/aspiration Poor cry Poor movements Alert/not alert Fasciculations
With a central nervous system problem causing a floppy baby, what signs might you find?
Increased tendon reflexes Extensor plantar response Sustained ankle clonus Global developmental delay Microcephaly/dysmorphism Prolonged seizures Axial weakness
What could be some causes of a central nervous system problem leading to a floppy baby?
Hypoxic ishaemic encephalopathy Hypoglycaemia Down's syndrome Prader-Willi syndrome Cerebral malformations
With a peripheral nervous system problem causing a floppy baby, what signs might you find?
Hypo-areflexia Selective motor delay Normal head circumference and growth Preserved social interaction Weakness of limbs Low pitched weak cry Tongue fasciculations
What could be some causes of a peripheral nervous system problem leading to a floppy baby?
Congenital myopathy Myotonic dystrophy Myasthenia gravis Motor sensory neuropathy Spinal muscular atrophy
What additional assessments need to be done in a neuro exam of an older child?
Cerebellar function Gowers test Test sensation Cognitive functioning Cranial nerves
What is gowers sign?
Weakness of proximal muscles
Patient has to use arms to walk up their body from squatting position due to lack of hip and thigh strength
What might you look for around the room when assessing a child’s neuro status?
Mobility aids Splints Oxygen Suction Feed pump Medication Drips Orthotic boots Wheelchair Glasses
If a child is sitting in a wheelchair what should you assess on general inspection?
Posture - flexed or extended Central/truncal tone Head position/support Type of wheelchair Attachments Communication aids
What do you look for in an examination of lower limb neuro in a child?
Posture - adducted and extended, frog leg, short leg adducted Scars - tendon releases Muscle bulk - wasting, inverted champagne bottle, high foot arch, calf hypertrophy Skin - neuro cutaneous markers Symmetry - limb hypertrophy Spine Tone Clonus Power Reflexes Sensation Pain and temp Light touch and proprioception Gait
What is cerebral palsy?
Primary abnormality of movement and posture secondary to non progressive lesion of developing brain
What are the different types of cerebral palsy? Where is the problem?
Ataxic - cerebellum
Spastic - pyramidal system
Dyskinetic - separated into athetoid and dystonic - basal ganglia
What is the GMFCS?
Gross motor function classification system
Get an idea of how self sufficient a child can be at home, school etc
Head control, movement transition, walking, gross motor skills
Levels 1 (normal) to 5 (transported in manual wheelchair in all settings)
What problems are associated with cerebral palsy?
Motor - gross and fine Vision and hearing Speech Epilepsy Feeding Gastro oesophageal reflux Constipation/incontinence Secondary disability - hips, spine, arthritis Learning disabilities
What are treatment aims for cerebral palsy?
Treat spasticity - physio, systemic local and intrathecal drugs, orthopaedic surgery, nerve and spinal root surgery
Treat associated problems
Enhance quality of life
What is an epileptic seizure?
Transient occurrence of signs/symptoms, result of a primary change to electrical activity, abnormally excessive or synchronous, in the brain
What is epilepsy?
Recurrent unprovoked seizures due to abnormal hyper-synchronous discharge of cortical neurons
What are differential diagnoses for epilepsy?
Dystopia Movement disorder Febrile seizure Non epileptic event Vaso vagal syncope Reflex anoxic seizures Cardiac arrhythmia Sleep associated disorders
What are different classifications of generalised seizures?
Myoclonic Atonic Tonic Tonic clonic Infantile spasms
What are causes of epilepsy?
Genetic Structural Metabolic Immune Infectious Unknown
What factors are important in an epilepsy history?
DESSCRIBE
Description
Epileptic or non epileptic
Seizure type - focal or generalised, behaviour change, sensory
Syndrome - age of onset, clinical features, EEG
Cause
Relevant - impairment, behaviour, education
What is important in a history when you are getting a patient to describe their epilepsy episodes?
Witnessed When, where, how long Before, during, after LOC Limb jerking movements, which muscle groups Have they happened before, were they the same Triggers Prodromal period hours or days before Aura immediately preceding Residual muscle weakness Tongue biting Bladder/bowel incontinence
What are other key questions in a child with epilepsy to evaluate other differentials?
Family history of early adult death Palpitations Headache Vomiting Paraesthesia Regression of skills
What is childhood absence epilepsy? What are the features?
Vacant episodes sometimes associated with eye flickering Abrupt start and stop No post event drowsiness Brief - 5 to 15 secs Occur in clusters Precipitated by hyperventilation
In which age of child is childhood absence epilepsy least prevalent?
Uncommon before 5 years old
What will an EEG show in childhood absence epilepsy?
3/sec spike and wave
What are differentials for absence seizures?
Day dreaming
Behavioural
What is treatment for childhood absence epilepsy?
Sodium valproate
Ethosuximide
What is prognosis for childhood absence epilepsy?
Rapid remission with treatment in 80% children
What is west syndrome?
Severe epilepsy syndrome composed of triad of infantile spasms, an interictal electroencephalogram (EEG) pattern of hypsarrhythmia (chaotic background), and mental retardation
What is the age of onset of west syndrome?
3 to 12 months
What is an infantile spasm?
Sudden generalised symmetrical contractions of muscles of limb (extensor), trunk (flexor) and neck occurring in clusters lasting 0.2-2 secs
What are causes of west syndrome?
Hypoxic ishaemic encephalopathy Infections Acquired brain injury Cortical malformations Tuberous sclerosis
What is the treatment for west syndrome?
Steroid
Vigabatrin
Multiple anti epileptic medications
Ketogenic diet
What is the prognosis for west syndrome?
Poor with developmental delay/neurological impairment
Spasms resolve with treatment but other seizure types develop
What is juvenile absence epilepsy?
Absence seizures with age of onset of 9-13 years
3-4Hz spike wave on EEG
Generalised tonic clonic seizures may occur
What is juvenile myoclonic epilepsy? What does EEG show?
Characterised by myoclonic seizures that occur most commonly after waking, age of onset 5-20+ years
Absence and generalised tonic clonic seizures may occur in between 50-80% of people with JME
EEG shows 3-6Hz generalised polyspike and wave activity
What is benign epilepsy with centrotemporal spikes?
Rolandic epilepsy
5-14 years onset
Characterised by focal motor and secondary generalised seizures, majority from sleep in otherwise normal individual with centrotemportal spikes on EEG
What investigations can be done to evaluate epilepsy?
Witnessed history Video Diary of episodes Investigations of other causes Bloods: baseline, genetics, metabolic ECG EEG MRI
What are treatments for epilepsy?
Anti epileptic medication
Ketogenic diet
Vagal nerve stimulator
Epilepsy surgery
What is a focal seizure?
Originates in networks limited to one hemisphere
What is a generalised seizure?
Originates in and rapidly engages bilaterally distributed networks
What is a generalised tonic clonic seizure?
Sudden onset involving generalised stiffening and subsequent rhythmic jerking of the limbs as a result of widespread engagement of bilateral cortical and subcritical networks
What is a myoclonic seizure?
Sudden brief and almost shock like involuntary single or multiple jerks due to abnormal excessive or synchronous neuronal activity associated with poly spikes on EEG
What is a tonic seizure?
Abrupt generalised muscle stiffening possibly causing a fall
Usually lasts 2-10 secs
What is an atonic seizure?
Generalised seizure characterised by sudden onset of loss of muscle tone
What is a febrile seizure?
Seizure precipitated by fever without evidence of cns infection or another defined cause
Onset 6 months to 6 years
Usually brief, generalised, tonic clonic
What is todds paresis associated with in febrile seizures?
Higher risk of epilepsy
What are differentials for a febrile seizure?
Meningitis
Meningo encephalitis
What are treatments for febrile seizures?
Treat the focus
Reassurance
Parent education
Control temp with anti pyretics and cooling measures
What is status epilepticus?
Any seizure lasting duration of 30 mins or repeated seizures lasting 30 mins or longer from which the patient doesn’t regain consciousness
In which patients are convulsive status episodes most common?
Children and those over 60
Why does injury to the brain occur in status epilepticus?
Underlying disorder
Systemic complications of the convulsions - hypoxia from airway obstruction and acidosis when hypotension occurs
Direct injury from repetitive neuronal discharge
What areas of the brain can be damaged by status epilepticus?
Hippocampus Amygdala Cerebellum Thalamus Cerebral cortex
What is management of status epilepticus?
ABC - check BM and treat if less than 3
Oxygen
Vascular access: midazolam/diazepam/lorazepam. Repeat after 5 mins. If no response after 5 mins - phenytoin or phenobarbital. Wait 20 mins if no response, rapid sequence induction with thiopentone or propofol
No vascular access: midazolam IM, buccal or intranasal. Repeat after 10 mins. If no response after 10 mins, paraldehyde PR. Then rapid sequence induction
Call outreach team for PICU
Reassess ABC
Monitor for respiratory depression post benzo
Regular neuro obs and monitor glucose
Restrict fluids to 60% of maintenance and monitor urine output
Consider NG to aspirate stomach contents
Consider CT head
What are complications of status epilepticus?
Airway obstruction Hypoxia Aspiration Respiratory depression secondary to excess benzodiazepines Cardiac arrhythmias Pulmonary oedema Hyperthermia Hypertension Disseminated intravascular coagulation
What factors effect choice of medication in epilepsy?
Seizure type Epilepsy syndrome Co medication and co morbidity Lifestyle Child and family preferences Monotherapy wherever possible Formulations
When should anticonvulsants be initiated? And how?
If diagnosis of epilepsy established
After discussion of potential side effects and management plan
Discuss SUDEP - sudden unexpected death in epilepsy
Start at subtherapeutic dose and increase slowly in 1-2 week intervals until therapeutic window
Monitor response 3-12 monthly
When can you withdraw epilepsy medication?
Wean treatment after 2-3 years seizure freedom
Drugs gradually withdrawn over last 2-3 months by reducing daily dose by 10-25% at intervals of 1-2 weeks
Benzos may take longer
What are side effects of anti epileptic drugs?
GI side effects Allergic rash Severe immune reactions - SJS Weight gain /loss Behavioural Liver/renal toxicity Tremors
The 4 month old baby daughter of a HIV positive mother is admitted with seizures. She has neonatal jaundice and microcephaly. What has caused this?
Cytomegalovirus
What might be some features of cytomegalovirus inclusion disease?
Microcephaly Seizures Neonatal jaundice Hepatosplenomegaly Deafness Mental retardation
What is Ebsteins anomaly? How does it present?
Congenital heart defect where septal and posterior leaflets of tricuspid valve displaced towards apex of right ventricle
Large right atrium, small right ventricle
S3 and S4 heart sounds
Systolic murmur along left lower sternal border
Wolff Parkinson white syndrome often accompanies
Which drug taken by a mother whilst pregnant may contribute to the baby developing Ebsteins anomaly?
Lithium
If spontaneous closure of a patent ductus arteriosus does not occur, when is surgical closure recommended?
Between 6 months to 1 year
Up to what size of ASD may spontaneously close without intervention?
8mm
If a child with a congenital heart defect is described as squatting, what do they likely have?
Tetralogy of fallot
Why do children with tetralogy of fallot squat?
Squatting increases peripheral vascular resistance so decreases magnitude of right to left shunt across VSD and therefore increase pulmonary blood flow
What are the four abnormalities in tetralogy of fallot?
VSD
Overriding aorta
Right ventricular outflow obstuction - pulmonary stenosis
Right ventricular hypertrophy
What causes right ventricular outflow tract obstruction in tetralogy of fallot?
Failure of rotation by pulmonary artery during its development
Causes infundibular obstruction - entrance to pulmonary artery just before valve
Why is dyspnoea and cyanosis much worse on exertion in tetralogy of fallot?
Right ventricular outflow tract obstruction is variable because of its infundibular nature
Tissue below pulmonary valve is muscular so responds to sympathetic stimulation
This can cause it to contact during exertion and so worsen the obstruction
This increases the flow of deoxygenated blood across the VSD and up the left ventricular outflow tract
What happens to the murmur in tetralogy of fallot during cyanotic episodes (tet spells)?
Murmur becomes quieter or disappear as there is reduced flow across the stenosed valve as more blood is shunted through the VSD
What is a boot shaped heart sign (coeur en sabot) indicative of?
Tetralogy of fallot
Product of small pulmonary tree and enlarged right ventricle
What is Eisenmengers syndrome?
Process in which a long standing left to right cardiac shunt caused by congenital heart defect (VSD, ASD or PDA) causes pulmonary HTN and eventually a reversal of the shunt into a cyanotic right to left shunt
What factors imply significant shunting across a VSD?
Heart failure
Pulmonary plethora
Mid diastolic murmur due to increased flow across the mitral valve
Loud second heart sound
Give some causes of neonatal goitre
Maternal drugs: carbimazole, iodine containing compounds
Pendreds syndrome: bilateral hearing loss and goitre
Maternal Graves: passage of TSH antibodies across placenta
Which is the most common congenital obstruction of the stomach and intestines?
Pyloric stenosis
What is the incidence of pyloric stenosis?
1 in 400
Is pyloric stenosis more common in males or females?
Males - 4/5x more common
What is the main presenting complaint with pyloric stenosis?
Projectile vomiting starting in 2/3rd week of life
What causes pyloric stenosis?
Hypertrophy and hyperplasia of antrum of stomach
What electrolyte abnormality might present in pyloric stenosis?
Hyponatraemia
Hypokalaemia
Hypochloraemia
Metabolic acidosis
A 4 day old boy has failed to pass meconium and has a distended abdomen. What would be on your differential list?
Hirschsprung’s disease
Anorectal malformations: imperforate anus, colonic atresia, colonic stenosis
Meconium plug/Small left colon syndrome
Hypoganglionosis
Neuronal intestinal dysplasia
Megacystis-microcolon-intestinal hypoperistalsis syndrome
What is Hirschsprung’s disease?
Congential aganglionic section of colon that starts at anus and progresses upwards
Children present with bowel obstruction in first few weeks of life
What is meconium plug syndrome?
Functional colonic obstruction in a newborn due to an obstructing meconium plug
What is Hypoganglionsosis?
Reduced number of nerves in the intestinal wall
May present like Hirschsprung’s disease
What is Neuroal intestinal dysplasia?
Problem with the motor neurons that lead to the intestine, inhibiting this process and thus preventing digestion
May present like Hirschsprung’s disease
What is Berdon syndrome?
Megacystis-microcolon-intestinal hypoperistalsis syndrome
Constipation and urinary retention, microcolon, giant bladder (megacystis), intestinal hypoperistalis, hydronephrosis, and dilated small bowel
Abundance of ganglion cells in both dilated and narrow areas of the intestine
A 1 year old girl has presented with severe cramping abdominal pain. She has passed a stool mixed with blood and mucus. On examination there is a sausage shaped mass in the upper abdomen. What is the likely diagnosis?
Intussusception
What is intussusception?
Telescoping of one portion of the bowel into an immediately adjacent segment
What is the commonest cause of intestinal obstruction in children aged 3 months to 6 years?
Intussusception
A 15 year old boy has presented with weight loss and increasing bowel frequency and diarrhoea. On examination he has apthous ulcers and perianal fistulae. What is the likely diagnosis?
Crohn’s disease
What are characteristics of achondroplasia?
Macrocephaly Frontal bossing Depressed nasal bridge Rhizomelic dwarfism Unusually prominent abdomen and buttocks Short hands with trident fingers during extension
What is McArdles disease?
Glycogen storage disease that results in mild lactic acidosis with exercise
A newborn boy presents with mild abdominal distension and failure to pass meconium after 24 hours. X-ray shows dilated bowel loops and fluid levels, the anus is normally located. What is it?
Hirschprungs disease
What is a Wilms tumour? When does it present?
Nephroblastoma
Usually in first 4 years of life
A 6 day old baby was born prematurely at 33 weeks. He has been suffering from respiratory distress syndrome and has been receiving ventilatory support on NICU. He has developed abdominal distension and is increasingly septic. Ultrasound of the abdomen shows free fluid and evidence of small bowel dilatation. His blood pressure has remained labile despite inotropic support. What is the diagnosis and how is it managed?
Necrotising enterocolitis and whilst this is often initially managed medically a laparotomy is required if the situation deteriorates
A 5-year-old child has been unwell with a sore throat and fever for several days. He progresses to develop periumbilical abdominal discomfort and passes diarrhoea. This becomes blood stained. The paediatricians call you because the ultrasound has shown a ‘target sign’. What is the diagnosis and how is it managed?
Intussusception. The lymphadenopathy will have initiated it. A target sign is seen on ultrasound and is the side on view of multiple layers of bowel wall. Reduction using fluoroscopy with air is the usual first line management. Ileo-colic intussceceptions are generally most reliably reduced using this method, long ileo-ileal intussceceptions usually result in surgery
What is the treatment for pyloric stenosis?
Ramstedt pyloromyotomy (open or laparoscopic)
What are red flags for speech development?
Loss of developmental skills at any age
No vocalising by 3 months
No babbling by 10 months
Not responding to name by 12 months
What is the mode of inheritance of peutz jeghers syndrome?
Autosomal dominant
An 18 month old boy is referred with loss of consciousness on 6 occasions. Each was preceded by a tantrum. What is the diagnosis?
Blue breath holding episodes
What are the different types of breath holding spells?
Simple: no major alteration in circulation or oxygenation, spontaneous recovery
Cyanotic/blue: precipitated by anger, cries, forced expiration, loss of muscle tone, loss of consciousness
Pallid/pale: painful event, pale, loses consciousness with little or no crying
Complicated: begins as blue or pale but then associated with seizure activity
How does congenital adrenal hyperplasia present?
Vomiting
Weight loss
Dehydration
Girls with virilised genitalia
How does hirschsprungs disease present?
Constipation
Generalised abdominal distension
Rectum empty
Palpable faecal mass
How’s does meckels diverticulum present?
Rectal bleeding
Intussusception
Like appendicitis
How does nephroblastoma present?
Wilms tumour Abdominal pain Vomiting Palpable abdominal lump Hypertensive
How does neuroblastoma present?
Asymptomatic mass
Pallor and hypotension if it bleeds
What is the maximum age by which intervention should ideally be in place if a pre lingually deaf child is to acquire language in a manner as close as possible to a hearing child?
12 months
What intervention is required to correct a congenital hearing loss in a child?
Hearing aid fitted initially to allow available sound to be delivered to child’s developing auditory system
For children with severe-profound hearing loss for whom hearing aids are insufficient, cochlear implantation should be considered
Why is tetracycline contraindicated in children?
Side effects affecting bones and teeth discoloration and growth
How would salt losing congenital adrenal hyperplasia present?
Failure to thrive Vomiting Dehydration Shocked Low sodium
What are risk factors for neonatal hearing loss?
Family history of hearing loss Prematurity Low birth weight Neonatal jaundice Rubella Non bacterial intrauterine infection Anoxia Craniofacial deformity Bacterial meningitis Apgar score of 0-3
What proportion of children with born with a hearing impairment are detected by high risk screening programmes?
Half
What is the most common drug responsible for fatal poisonings in children?
Tricyclic antidepressants
What causes a barking cough in children?
Croup
What is the treatment for croup?
Humidified oxygen
If more severe - dexamethasone
What are treatments for bronchiolitis?
Supportive: humidified oxygen and supporting feeding
What is McArdles disease?
Painful muscle cramps Myoglobinuria After intense exercise Autosomal recessive condition Myophosphorylase deficiency which leads to inability to utilise glucose
What is chondromalacia patellae?
Softening of cartilage of patella
Common in teenage girls
Anterior knee pain on walking up and down stairs and rising from prolonged sitting
What is osgood schlatter disease?
Tibial apophysitis
Seen in sporty teenagers
Pain, tenderness and swelling over tibial tubercle
An 18 day old breast fed boy who has gained 300g since birth has no other problems but jaundice with yellow stools. What is the likely diagnosis?
Breastfeeding jaundice
A 10 day old baby has shown no weight gain since birth and has jaundice associated with clay coloured stools, what is the likely diagnosis?
Biliary atresia
What is osteochondritis dissecans?
Pain after exercise
Intermittent swelling and locking
A 1 day baby born at term is noted to be developing increasing jaundice. What is the likely cause?
Rhesus incompatibility
An 8 year old boy presents with puffy eyes, ascites and scrotal swelling following an URTI. What is the likely diagnosis?
Minimal change glomerulonephritis
A 4 year old child was seen in a refugee camp. He has angular stomatitis, hyperkeratosis with desquamation rash, sparse hair and ascites. What is the likely diagnosis?
Kwashiorkor - protein energy malnutrition
What is Dubin Johnson syndrome?
Autosomal recessive disorder
Increase in conjugated bilirubin
Black liver
Defect in ability of hepatocytes to secrete conjugated bilirubin into bile due to mutation in multiple drug resistance protein 2 in canalicular membrane
What is rotor syndrome?
Autosomal recessive bilirubin disorder causing increased conjugated bilirubin
Liver appears normal (in contrast to dubin Johnson)
Mutations in transport proteins so liver cannot remove bilirubin
What is Gilbert’s syndrome?
Reduced activity of glucuronyltransferase which conjugates bilirubin so causes unconjugated hyperbilirubinaemia
What is G6PD deficiency?
X linked recessive inborn error of metabolism predisposing to haemolysis and jaundice
Triggered by food, illness or medication
Low levels of glucose 6 phosphate dehydrogenase which is involved in pentose phosphate pathway
How can G6PD deficiency manifest/be complicated?
Prolonged neonatal jaundice Kernicterus Haemolytic crises Diabetic ketoacidosis Acute kidney failure
What is favism?
Haemolytic response to consumption of fava beans associated with G6PD deficiency
What are some triggers of haemolytic crises in G6PD deficiency?
Fava beans
Drugs: antimalarials, sulfonamides, nitrofurantoin
Stress: bacterial or viral infection
What are features of Klinefelters syndrome?
Tall Reduced muscle control and coordination Reduced secondary sexual characteristics Gynaecomastia Infertility Microorchidism
What is the karyotype of klinefelters?
47 XXY
What are features of fragile x syndrome?
Behavioural problems Intellectual disability/learning difficulties Developmental delay Low muscle tone Long narrow face, prominent ears High palate Large testicles Mitral valve prolapse
What are features of Kallmanns syndrome?
Anosmia Cleft palate Renal agenesis/aplasia Failure to start or complete puberty Lack of testicle development Primary amenorrhoea Infertility
What is Kallmanns syndrome?
Failure of hypothalamus to release GnRH at appropriate time due to GnRH releasing neurons not migrating into correct location during embryonic development
Hypogonadotropic hypogonadism
What is the most common extracutanous site for chickenpox virus in children?
Cerebellar ataxia
What is Rett syndrome?
Cerebroatrophic hyperammonaemia Post natal neurological disorder of grey matter seen in females Small hands and feet Deceleration of rate of head growth Repetitive stereotyped hand movements Associated with autism
What is the management for a child under 3 months with a temperature of over 38?
Urgent referral to paediatrician
What is Hirschsprung’s disease? How does it present?
Absence of ganglion cells in neural plexus of intestinal wall
More common in boys than girls
Delayed passage of meconium and abdo distension
In who is necrotising enterocolitis more common?
Premature infants
Why does necrotising enterocolitis cause sepsis?
Mesenteric ischaemia causes bacterial invasion of mucosa leading to sepsis
Which regions of the bowel are commonly affected in necrotising enterocolitis?
Terminal ileum
Caecum
Distal colon
How does necrotising enterocolitis present?
Distended tense abdomen
Passage of blood and mucus per rectum
Signs of sepsis
Which condition is associated with meconium ileus?
Cystic fibrosis
Why does cystic fibrosis cause meconium ileus?
Deficient intestinal secretions leading to abnormal bulky and viscid meconium
How is a diagnosis of pyloric stenosis made?
Test feed or USS
What is the treatment for pyloric stenosis?
Ramstedt pyloromyotomy
When and how does pyloric stenosis usually present?
4-6 weeks of life
Projectile non bile stained vomiting
How does intussusception usually present?
Child age 6-9 months Colicky pain Diarrhoea and vomiting Sausage shaped mass Red jelly stool
What is the treatment for intussusception?
Reduction with air insufflation
What is oesophageal atresia associated with?
Tracheo-oesophageal fistula
Polyhydramnios
How does biliary atresia usually present?
Jaundice >14 days after birth
Increased conjugated bilirubin
How is biliary atresia treated?
Urgent Kasai procedure - small intestine pulled up and used to create bile duct
Roux en Y connection of duodenum to newly inserted small bowel
What are causes of constipation in children?
Idiopathic Dehydration Low fibre diet Medications: opiates Anal fissure Over enthusiastic potty training Hypothyroidism Hirschsprung's disease Hypercalcaemia Learning disabilities
What are some red flags in terms of stools and constipation in children?
Reported from birth or first few weeks of life
Meconium passed after 48 hours
Ribbon stools
Faltering growth
Previously unknown weakness in legs, locomotor delay
Abdomen distension
What factors in a child suggest faecal impaction?
Symptoms of severe constipation
Overflow soiling
Faecal mass palpable in abdomen
What is the management if faecal impaction is present in a child?
Polyethylene glycol 3350 and electrolytes using escalating dose regimen (movicol paediatric plain)
Add stimulant laxative if not disimpacted after 2 weeks
Substitute stimulant laxative +/- osmotic laxative if MPP not tolerated
Inform family that disimpaction can initially increase symptoms of soiling and abdominal pain
What does WETFLAG stand for in paediatric life support?
Weight: 0-12 months (0.5 x age in months) +4 1-5 years (2x age) +8 6-12 years (3x age) +7 Energy: 4 joules/kg Tube: age/4 +4 Fluids: illness 20ml/kg, trauma 10ml/kg Lorazepam: 0.1ml/kg IV or IO Adrenaline: 0.1ml/kg IV or IO 1:10000 Glucose: 2ml/kg 10% dextrose
How does step 3 of asthma management differ in children?
5 or over: add LABA
Under 5: add leukotriene antagonist
What are risk factors for neonatal meningitis?
Low birth weight Prematurity Traumatic delivery Foetal hypoxia Maternal peripartum infection
Which causative organisms are neonates at risk of developing meningitis from?
Group B strep
E. coli
Listeria monocytogenes
At what point should a child with an undescended testis be referred?
3 months of age
Should be seen before 6 months
What is the management for nocturnal enuresis?
Look for underlying causes: constipation, diabetes, UTI
Advise on fluid intake, diet and toiletting before bedtime
Reward systems: stars for using the toilet before sleep
Child under 7: enuresis alarm
7 or over: desmopressin
At what age should children be offered an influenza vaccine?
Intranasally at 2-3 years
Annually after that
What are contraindications to a child receiving an influenza vaccine?
Immunocompromised
Aged <2
Current febrile illness/blocked nose
Current wheeze or history of severe asthma
Egg allergy
Pregnancy/breastfeeding
If child is taking aspirin - Kawasaki disease, due to risk of Reye’s syndrome
What vaccines should a child have at birth?
BCG/hep B if risk factors
What vaccines should a child have at 2 months?
DTaP/IPV/Hib
PCV
Oral rotavirus
Men B
What vaccines should a child have at 3 months?
DTaP/IPV/Hib
Oral rotavirus
What vaccines should a child have at 4 months?
DTaP/IPV/Hib
PCV
Men B
What vaccines should a child have at 12-13 months?
Hib/Men C
MMR
PCV
Men B
What vaccines should a child have at 2- 7 years?
annual flu vaccine
What vaccines should a child have at 3-4 years?
MMR and DTaP/IPV
What vaccines should a child have at 12-13 years?
HPV vaccine for girls
What vaccines should a child have at 13 - 18 years?
DT/IPV
Men ACWY
What is the management for croup?
Oral dexamethasone 0.15mg/kg single dose
High flow oxygen
Nebulised adrenaline
What are features of patau syndrome?
Microcephaly Small eyes Cleft lip/palate Polydactyly Scalp lesions
What is patau syndrome?
Trisomy 13
What are features of Edwards syndrome?
Micrognathia
Low set ears
Rocker bottom feet
Overlapping of fingers
What is Edwards syndrome?
Trisomy 18
What are features of fragile x?
Learning difficulties Macrocephaly Long face Large ears Macro orchidism
What are features of noonan syndrome?
Webbed neck
Pectus excavatum
Short stature
Pulmonary stenosis
What are features of Pierre robin syndrome?
Micrognathia
Posterior displacement of tongue
Cleft palate
What are features of prader Willi syndrome?
Hypotonia
Hypogonadism
Obesity
What are features of Williams syndrome?
Short stature Learning difficulties Friendly extrovert personality Transient neonatal hypercalcaemia Supravalvular aortic stenosis
What causes headlice?
Pediculosis capitis
How are head lice diagnosed?
Fine toothed combing of wet or dry hair
What is the management of head lice?
Treatment only indicated if living lice found
Choice should be offered: malathion, wet combing, dimeticone, isopropyl myrisate and cyclomethicone
Which patients typically get Wilms tumours?
Under 5 years, median age 3
How does a wilms tumour usually present?
Abdo mass Painless haematuria Flank pain Anorexia Fever Mets: usually lung
What is the management of a wilms tumour?
Nephrectomy
Chemotherapy
Radiotherapy if advanced
Under what age can a child not consent to sex?
Under 13
What is talipes equinovarus?
Club foot
Inverted and plantar flexed foot
What are associations with club foot?
Spina bifida Cerebral palsy Edwards syndrome Oligohydramnios Arthrogryposis
What is the ponseti method?
Manipulative technique that corrects congenital club foot without invasive surgery
Manipulation and progressive casting starting soon after birth
Usually corrected after 6-10 weeks
Achilles tenotomy required in 85%
Night time braces required until age 4
What type of diet can be useful in patients with difficult to treat epilepsy?
Ketogenic diet: high fat, low carb, controlled protein
What is wests syndrome?
Infantile spasms
Childhood epilepsy presents in first 4-8 months of life
More common in males
Characteristic salaam attacks: flexion of head, trunk and arms followed by extension of arms
Progressive mental handicap
What would an EEG show in an infant with west syndrome?
Hypsarrhythmia - high amplitude irregular waves and spikes in background of chaotic and disorganised activity
What are clinical features of tuberous sclerosis?
Ash leaf spots Adenoma sebaceum Shagreen patches Subungual fibromata Epilepsy Developmental problems Retinal hamartomas
What are risk factors for developmental dysplasia of the hip?
Female Breech presentation Positive family history Firstborn Oligohydramnios Birth weight >5kg Congenital calcaneovalgus foot deformity
What is the management of developmental dysplasia of the hip?
Most will spontaneously stabilise by 3-6 weeks
Pavlik harness in children younger than 4-5 months
Older children may require surgery
Which hearing test is used in newborn babies?
Automated otoacoustic emissions test
What is the classic triad of problems in haemolytic uraemic syndrome?
Haemolytic anaemia
Renal failure
Thrombocytopenia
Which bug usually causes haemolytic anaemic syndrome?
E. coli 0157
What needs to be done about a suspected case of female genital mutilation?
Inform medical team and police - child protection and safeguarding issues
What is a cephalohaematoma?
Swelling on newborns head
Develops hours after delivery
Due to bleeding between periosteum and skull
Most commonly in parietal region
What is caput succedaneum?
Serosanguinous subcutaneous extraperiosteal fluid collection with poorly defined margins caused by pressure of presenting part of scalp against dilating cervix
What is a chignon?
Temporary swelling left on infants head after a ventouse suction cup has been used for delivery
What is a subaponeurotic haemorrhage?
Bleeding in potential space between periosteum and subgaleal aponeurosis
Boggy swelling that grows insidiously and is not confined to skull sutures
May present as haemorrhagic shock
How should a child aged less than 3 months be managed if they have a UTI?
Refer immediately to paediatrician
What is the management for cradle cap (seborrhoeic dermatitis)?
Mild to moderate: baby shampoo and baby oils
Severe: mild topical steroids e.g. 1% hydrocortisone
What is bardet Biedl syndrome?
Polydactyly
Obesity
Retinitis pigmentosa
What are causes of neonatal jaundice in the first 24 hours of life?
Rhesus haemolytic disease
ABO haemolytic disease
Hereditary spherocytosis
G6PD deficiency
What is done if jaundice is still present after 14 days in a neonate?
Measure conjugated and unconjugated bilirubin Direct antiglobulin test TFTs FBC and blood film Urine for MC and S and reducing sugars U and Es LFTs
What are causes of prolonged jaundice in a newborn?
Biliary atresia Hypothyroidism Galactosaemia Urinary tract infection Breast milk jaundice Congenital infection: CMV, toxoplasmosis
What is the management for severe croup?
Steroids
Nebulised adrenaline
What is Kawasaki disease?
Small vessel vasculitis Prolonged fever Children under 5 Mucocutaneous lymph node syndrome Rash, lymphadenopathy, red eyes, dry cracked lips, red fingers and toes
What is the management for viral induced wheeze?
Salbutamol inhaler
If this isn’t helping, add oral montelukast or inhaled corticosteroid
What are features of Kawasaki disease?
High grade fever >5 days Conjunctival injection Bright red, cracked lips Strawberry tongue Cervical lymphadenopathy Red palms and soles which later peel
What is the management of Kawasaki disease?
High dose aspirin
IV immunoglobulin
Echo to screen for coronary artery aneurysms
What is G6PD?
X linked disorder affecting red cell enzymes
Reduced ability of red cells to respond to oxidative stress so have shorter life span and are more susceptible to haemolysis particularly in response to drugs, infection, acidosis and fava beans
Red cell fragments: Heinz bodies
What are features of G6PD deficiency?
Neonatal jaundice Intravascular haemolysis Gallstones Splenomegaly Heinz bodies on blood film
What is eneuresis?
Involuntary discharge of urine day or night or both in a child aged 5 or over in absence of congenital or acquired defects of nervous system or urinary tract
What is the management of whooping cough?
Oral macrolide: clarithromycin, azithromycin, erythromycin if onset of cough is within previous 21 days
What are complications of whooping cough?
Subconjunctival haemorrhage
Pneumonia
Bronchiectasis
Seizures
What are features of Bartter syndrome?
Neonatal: polyhydramnios, polyuria, polydipsia, hypocalcaemia, nephrocalcinosis
Classic: polyuria, polydipsia, vomiting, growth retardation, hypokalaemia, alkalosis, low BP
Until what age are up going plantars normal?
1 year
At how many months do infant IgM levels reach that of adult?
2-5 months
Which immunoglobulin crosses the placenta?
IgG
What is Kallmanns syndrome?
Failure to start puberty and anosmia
Hypogonadotrophic hypogonadism
GnRH releasing neurones prevented from reaching hypothalamus or cribriform plate
What is Noonan syndrome?
Pulmonary stenosis ASD Hypertrophic cardiomyopathy Short stature Learning difficulty Pectus excavatum Webbed neck Flat nose bridge
What is a congenital undescended testis?
One that has failed to reach the bottom of the scrotum by 3 months of age
Which congenital defects may be associated with undescended testis?
Patent processus vaginalis Abnormal epididymis Cerebral palsy Mental retardation Wilms tumour Abdominal wall defect
What are reasons for correcting cryptorchidism?
Reduce risk of infertility
Allows testis to be examined for cancer
Avoid torsion
Cosmetic appearance
What is the treatment for cryptorchidism?
Orchidopexy at 6-18 months of age
After age of 2 if untreated, Sertoli cells degrade and so may be better to do orchidectomy
Which congenital abnormality is associated with holoprosencephaly and cyclops?
Trisomy 13: patau syndrome
Which congenital abnormality is associated with dysplastic heart valves and oesophageal atresia?
Trisomy 18: Edwards syndrome
Which conditions are screened for on a neonatal blood spot screening?
Congenital hypothyroidism Cystic fibrosis Sickle cell Phenylketonuria Medium chain acyl coA dehydrogenase deficiency Maple syrup urine disease Isovolaemic acidaemia Glutaric aciduria type 1 Homocystinuria
When is neonatal blood spot screening performed?
Between 5th and 9th day of life
What is a Wilms tumour?
Nephroblastoma seen in children
Usually presents as painless palpable abdominal mass, anorexia, haematuria, HTN
What are some associations of a Wilms tumour?
Beckwith wiedemann syndrome
WAGR syndrome
Hemihypertrophy
Loss of function in WT1 gene
How does listeriosis present in a neonate?
Multi organ disease
Granulomata on skin
How does toxoplasmosis present in a neonate?
Hydrocephaly
Seizures
Chorioretinitis
What advice can be given to a mother during pregnancy to try and avoid toxoplasmosis in a baby?
Wear gloves when gardening or handling cat litter
Cook meat thoroughly
How does cytomegalovirus present in a neonate?
Jaundice
Hepatosplenomegaly
Microcephaly
How does neonatal varicella infection present?
Cerebral cortical and cerebellar hypoplasia Microcephaly Convulsions Limb hypoplasia Rudimentary digits
How does neonatal rubella present?
Cataracts Cardiac abnormalities Thrombocytopenia Cerebral calcification Deafness
What is the antibiotic of choice for whooping cough?
Erythromycin
What is gnathopathy?
Protrusion of upper teeth
Feature of sickle cell
What order do features of male puberty develop in?
Testicular enlargement
Secondary sexual characteristics: public hair, axillary hair, body odour, deepening of voice
Growth spurt 18 months after onset
What order do features of female puberty develop in?
Breast development
Axillary and pubic hair
Growth spurt
Menarche
In which groups does puberty usually occur earlier?
Afro American children
Obese
Due to raised oestrogen levels
What is pendred syndrome?
Congenital bilateral sensorineural hearing loss and goitre with euthyroid or mild hypothyroidism
The presence of what features alongside meconium would warrant assessment by the neonatal team?
Resp rate above 60 Grunting Heart rate below 100 or over 160 Cap refil over 3 Temp 38 or above or 37.5 on 2 occasions 30 mins apart Sats below 95% Central cyanosis
What is the most common cause of stridor in infants?
Laryngomalacia
What is laryngomalacia?
Floppy epiglottis which folds into airway on inspiration
Which are cyanotic heart defects?
Tetralogy of Fallot Transposition of great vessels Hypoplastic left heart Tricuspid atresia Eisenmengers complex
What is eisenmengers syndrome?
Reversal of a shunt due to pulmonary hypertension
What are causes of neonatal hypoglycaemia?
Maternal diabetes mellitus Prematurity IUGR Hypothermia Neonatal sepsis Inborn errors of metabolism Nesidioblastosis Beckwith Wiedemann syndrome
What is DiGeorge syndrome?
Thymic aplasia with agammaglobulinaemia Congenital heart problems Frequent infections Developmental delay 22q11.2 deletion syndrome
What is Wiskott Aldrich syndrome?
X linked immunodeficiency disorder characterised by lymphopenia and recurrent bacterial infections
What are some predisposing conditions for intussusception?
Enlarged peyers patches
Meckels diverticula
Tumour
Haematoma complicating HSP
In which age group does intussusception tend to present?
3 months to 3 years
What are key features of the children’s flu vaccine?
Intranasal
First dose at 2-3 years then annually after that
Live vaccine
What are contraindications to the children’s flu vaccine?
Immunocompromised
Aged <2 years
Current febrile illness or blocked nose rhinorrhoea
Current wheeze or hx severe asthma
Egg allergy
Pregnancy/breastfeeding
If child is taking asthma due to risk of Reyes
What are side effects of flu vaccine?
Blocked nose/rhinorrhoea
Headache
Anorexia
What are features of Lesch-Nyhan syndrome?
Hyperuricaemia Gout Choreoathetosis Spasticity Mental deficiency Behavioural disturbance (particularly self mutilation)
What is the epidemiology of autism?
75% male
Usually develops before age 3
What features must be present for a diagnosis of autism to be made?
Global impairment of language and communication
Impairment of social relationships
Ritualistic and compulsive behaviour
Which conditions are particularly associated with autism?
Fragile x syndrome
Retts syndrome
What is the most appropriate treatment for a neonate with otitis media?
IV cefotaxime
How will a child with foetal alcohol syndrome present?
Short stature Short palpebral fissures Flat philtrum Thin upper lip vermillion Neurocognitive problems: hyperactivity, delinquent behaviour
Which criteria are used to give a diagnosis of marfans syndrome?
Ghent criteria
Two cardinal features: dilated aortic root and displacement of optic lens
How often would you expect a 2 month old to feed and how many wet nappies should usually go with this?
Feed 8-10 times a day for 5-10 mins at a time
5-6 wet nappies with 3-4 stools
Why do patients with kallmans syndrome get anosmia and primary amenorrhoea?
Failure of development of olfactory bulb
Failure of production of gonadotrophin releasing hormone
Which drug can be used to close a patent ductus arteriosus?
Indomethacin
Which immune cells are deficient in DiGeorge syndrome?
T cells due to failure of thymic development
What are features of Prader-Willi syndrome?
Obesity due to compulsive food consumption
Mental retardation
What are features of Kawasaki disease?
High grade fever which lasts >5 days, resistant to antipyretics Conjunctival injection Bright red, cracked lips Strawberry tongue Cervical lymphadenopathy Red palms and soles which later peel
What is the management of Kawasaki disease?
High dose aspirin
IV immunoglobulin
Echo to screen for coronary artery aneurysm
At what point should a baby be referred if they have an undescended testicle?
Consider from 3 months
Ideally should see surgeon before 6 months
Usually procedure at around 1 year
What is the emergency management of a child with severe croup?
Oxygen and nebulised adrenaline
How is a diagnosis of pertussis confirmed?
Nasal secretions - culture
In what age are breath holding attacks most common?
6 months to 5 years
What are clinical features of turners syndrome?
Delayed secondary sexual characteristics Webbed neck Systolic murmur Broad shield chest with widely spaced nipples Cubitus valgus Short fourth metacarpal Low set ears Low hairline Hypoplastic nails Short stature
What treatments are used in turners syndrome?
Growth hormone
Anabolic steroids
Oestrogen/progesterone
What are possible features of galactosaemia?
Vomiting Diarrhoea Jaundice Lethargy Hypotonia Failure to thrive Cataracts Mental retardation Seizures Complement deficiency
What is galactosaemia?
Rare metabolic genetic disorder that affects ability to metabolise galactose properly
Autosomal recessive deficiency in enzyme needed to break down galactose
What is a cephalohaematoma?
Haemorrhage of blood between skull and periosteum due to rupture of vessels crossing periosteum
What are causes of cephalohaematoma?
Prolonged second stage labour
Instrumental delivery, particularly ventouse
What are symptoms of cephalohaematoma?
Jaundice Anaemia Hypotension Infection Unnatural bulges
When does a cephalohaematoma usually manifest?
Several hours after delivery
Over how long will a cephalohaematoma take to resolve?
3 months
Which disorders can cause hair on end appearance on skull X-ray?
SHITE Sickle cell anemia Hereditary spherocytosis Iron deficiency anemia Thalassemia Enzyme deficiency (G6PD)
Why do patients with thalassemia get cirrhosis and iron overload?
Multiple transfusions
When does jaundice start in babies with beta thalassemia?
3 months because haemoglobin f is still predominant in neonates
In a child that has been dry for some time, what may be causes of new onset bed wetting?
Psychological
UTI
Diabetes
What are features of patau syndrome (trisomy 13)?
Midline defects Hypotelorism Cleft palate Microphthalmia Cyclopia
How is a patent ductus arteriosus closed?
COX inhibitor infusion to block synthesis of prostaglandins which keep it open
What are some causes of polyhydramnios?
Maternal diabetes Rhesus incompatibility Duodenal atresia GI obstruction Tracheo-oesophageal fistula Anencephaly
How is hereditary spherocytosis inherited?
Autosomal dominant
Which treatment can lead to a normal life for patients with hereditary spherocytosis?
Splenectomy
Done after 5 years when chances of life threatening encapsulated bacterial infection reduce
When is the peak age for sudden infant death syndrome?
Under 6 months
Particularly second month
In which babies is sudden infant death syndrome most likely?
Low birth weight Premature Twins Boys Babies born to young mothers Low socio economic class Smokers in household Sleeping prone Co sleeping with parents History of drug or alcohol misuse in family
What are treatment options for minimal change disease?
Diuretics
Salt restriction
Steroids
What are complications of minimal change disease?
Venous thrombosis
Sepsis
Acute renal failure
Renal vein thrombosis
Why does congenital adrenal hyperplasia lead to accelerated bone age?
21 alpha hydroxylase deficiency results in increased production of androgens which drive bone proliferation, particularly at puberty
What does small for gestational age mean?
Birth weight below the 10th centile for gestational age and sex
What is intrauterine growth retardation?
Decrease in foetal growth rate that prevents infant obtaining genetic growth potential
What are potential complications of intrauterine growth retardation?
Perinatal asphyxia Meconium aspiration Electrolyte imbalance Metabolic acidosis Polycythemia Hypoglycaemia
At what age would you refer a child if they were unable to speak in intelligible short sentences?
3 years
At what age would you refer a child if they were not reliably dry at night whilst continent of urine and faeces?
6 years
At what age would you refer a child who was unable to sit unsupported?
10 months
What is Gaucher’s disease?
Genetic disorder where glucocerebroside accumulates in cells and organs
Characterised by bruising, fatigue, anaemia, hepatosplenomegaly, skeletal disorder, neuropathy, pingueculae in cornea
At what age would you refer a child if they were unable to walk independently?
18 months
At what age would you refer a child if they were unable to smile?
2 months
What is the name of the eye infection caused by vertical transmission of neisseria gonorrhoea to a neonate?
Ophthalmia neonatorum
What are features of HSP?
Palpable purpuric rash over buttocks and extensor surfaces of arms and legs Abdominal pain Polyarthritis Haematuria Renal failure
What proportion of patients will have a relapse of their HSP?
1/3
What is the management for mild croup?
Oral dexamethasone 0.15mg/kg single dose and review
What is the management for severe croup?
Systemic dexamethasone and nebulised adrenaline 5ml of 1:1000
Oxygen
How long do children need to be kept out of school if they have whooping cough?
Five days from commencing antibiotics
How long does a child need to be excluded from school if they have roseola inantum?
No exclusion
How long does a child need to be excluded from school if they have diarrhoea and vomiting?
Until symptoms have settled for 48 hours
Which protein is defected in Marfans syndrome?
Fibrillin
What is the immediate management of necrotising enterocolitis?
Broad spectrum antibiotics
What is the treatment for impetigo?
Topical fusidic acid
How does diphtheria present?
Grey membrane on tonsils Recent visitors to Eastern Europe/Russia/Asia Sore throat Bulky cervical lymphadenopathy Neuritis Heart block
What are features of congenital toxoplasmosis?
Microcephaly
Hydrocephalus
Cerebral calcification
Choroidoretinitis
How might a baby who was exposed to cytomegalovirus during pregnancy present?
Neonatal jaundice
Deafness
Microcephaly
What is wiskott Aldrich syndrome?
Rare X-linked recessive disease characterized by eczema, thrombocytopenia, immune deficiency, and bloody diarrhoea secondary to the thrombocytopenia
When are doses of MMR vaccine given?
12-15 months
3-4 years
What are contraindications to MMR vaccine?
Severe immunosuppression
Allergy to neomycin
Children who have received another live vaccine within 4 weeks
Pregnancy should be avoided for 1 month after
Immunoglobulin therapy within past 3 months
What are the criteria for immediate head CT in a child after head injury?
Loss of consciousness more than 5 mins
Amnesia lasting more than 5 mins
Abnormal drowsiness
Three or more episodes of vomiting
Clinical suspicion of NAI
Post traumatic seizure with no Hx epilepsy
GCS <14 or for baby under 1 GCS <15 in ED
Suspicion of open or depressed skull injury or tense fontanelle
Basal skull fracture: haemotympanum, panda eyes, CSF rhinorrhoea, battles sign
Focal neurological deficit
If under 1: bruise, swelling or laceration more than 5cm on head
Dangerous mechanism of injury: high speed road accident, fall from height greater than 3cm, high speed injury from projectile
What are cutaneous features of tuberous sclerosis?
Depigmented ash leaf spots
Roughened patches of skin over lumbar spine (shagreen patches)
Adenoma sebaceum (angiofibromas over nose)
Subungual fibromata
Cafe au lait spots
What is the most common cause of a persistent watery eye in an infant?
Nasolacrimal duct obstruction
What is Reye’s syndrome?
Severe inflammatory progressive encephalitic illness of children often accompanied by fatty infiltration
What is McArdles disease?
Inherited disorder resulting in rhabdomyolysis with minor illness or mild/moderate exertion
Inherited defect in gene for muscle phosphorylase enzyme
What are causes of jaundice in first 24 hours of life?
Rhesus haemolytic disease
ABO haemolytic disease
Hereditary spherocytosis
G6PD deficiency
What are some causes of prolonged jaundice in a newborn? (Beyond 14 days)
Biliary atresia Hypothyroidism Galactosaemia UTI Breast milk jaundice Congenital infection e.g. CMV, toxoplasmosis
When is the rotavirus vaccine given?
2 months and 3 months
Why should the rotavirus vaccine not be given after 23 weeks and 6 days?
Theoretical risk of intussusception
Which babies are most likely to have a patent ductus arteriosus?
Premature
Born at high altitude
Maternal rubella infection in first trimester
What are features of a PDA?
Left subclavicular thrill Continuous machinery murmur Large volume, bounding, collapsing pulse Wide pulse pressure Heaving apex beat
What is the management of PDA?
Indomethacin
What does a combination of scaphoid abdomen and bilious vomiting suggest?
Intestinal malrotation and volvulus
What should be given to a baby who has a high risk of vertical transmission of hep b?
Hep b vaccine and 0.5ml HBIG within 12 hours of birth
Hepatitis vaccine at 1-2 months
Vaccine at 6 months
What is a neonatal death? What is the difference between early and late neonatal death?
Death within 28 days of delivery of a live born foetus regardless of gestation
Early: 0-6 days
Late: 7-27 days
When should the fontanelles close?
Posterior: 8 weeks
Anterior: 12 and 18 months
How is a diagnosis of Kawasaki disease made?
Prolonged fever more than 5 days
Typical mucocutaneous changes: desquamation of fingers and toes including palms and soles
Lymphadenopathy
What is a NIPE? When should it be done?
Newborn and infant physical examination
Within 72 hours of birth unless sick baby then within 24 hours
What is choanal atresia?
Congenital disorder
Posterior nasal airway occluded by soft tissue or bone
Episodes of cyanosis worst during feeding made better by crying
What are signs of respiratory distress syndrome in a neonate?
Cyanosis Tachypnoea Nasal flaring Grunting Intercostal/subcostal recession Apnoea
What causes tracheal tug?
Aneurysm of aortic arch
What is Rett syndrome?
Genetic disorder which affects brain development primarily in females
Development proceeds in normal fashion for 6-18 months then change in behaviour with regression or loss of abilities including gross motor, speech, reasoning and hand use
They typically have repetition of gestures e.g. Hand ringing or hand washing
What are features of prader Willi syndrome?
Compulsive eating
Childhood obesity
Developmental delay
Chromosome 15 abnormalities
What is Harrison’s sulcus?
Horizontal groove along lower border of thorax corresponding to costal insertion of diaphragm
Usually caused by chronic asthma or COPD, can also appear in rickets due to defective mineralisation of bones so diaphragm pulls softened bone inwards
Why are newborns vitamin k deficient?
Immature hepatic metabolism
Low placental transfer
No oral intake of vitamin k or precursors
Lack of gastrointestinal bacterial colonisation
Why is intramuscular vitamin k given to newborns at birth?
Reduce vitamin k deficiency bleeding - haemorrhagic disease of newborn
What are features of digeorge syndrome?
CATCH 22
Cardiac abnormalities
Abnormal facies (high broad nose, low set ears, small teeth, narrow eyes)
Thymic hypoplasia
Hypocalcaemia (poor development of parathyroid)
Deletion on chromosome 22
What are features of fragile x syndrome?
Tall High arched palate Long ears Long face Macro orchidism Learning difficulties
How is otitis media in a neonate managed?
IV cefotaxime
What is the leading cause of death in ITP?
Intracranial haemorrhage
What is the treatment of choice for a patient with massive haemorrhage in ITP?
Platelet transfusion
Intravenous methylprednisolone
IV immune globulin
Splenectomy
Why can pyloric stenosis lead to hypocalcaemia?
Worsening alkalosis means increasing bicarbonate levels combine with calcium and so reduce serum ionised calcium
What are sequelae of mumps?
Meningoencephalitis Arthritis Transverse myelitis Cerebellar ataxia Deafness
What causes degranulation of mast cells?
Crosslinking of IgE
What causes hereditary angio oedema?
Autosomal dominant condition associated with deficiency of C1 esterase inhibitor
What is distal intestinal obstruction syndrome?
Occurs in CF patients
Accumulation of viscous mucus and faecal material in terminal ileum, caecum and ascending colon
What are features of cows milk protein intolerance/allergy?
Regurgitation and vomiting Diarrhoea Urticaria Atopic eczema Colic: irritability, crying Wheeze Chronic cough Rarely angioedema and anaphylaxis
How do you investigate cows milk protein intolerance?
Improvement with cows milk protein elimination
Skin prick/patch testing
Total IgE and specific IgE for cows milk protein (RAST)
How is cows milk protein intolerance managed?
Refer to paeds if failure to thrive
Formula fed: extensive hydrolysed formula, amino acid based formula
Breast fed: continue breast feeding, eliminate cows milk protein from maternal diet, use extensive hydrolysed milk when breast feeding stops until 12 months
Hand preference before what age may indicate cerebral palsy?
12 months
What are symptoms of threadworm infection?
Perianal itching particularly at night
Girls may have vulval symptoms
What are risk factors for developmental dysplasia of the hip?
Female sex Breech presentation Positive family history Firstborn Oligohydramnios Birth weight >5kg Congenital calcaneovalgus foot deformity
What is management for developmental dysplasia of the hip?
Most unstable hips spontaneously stabilise by 3-6 weeks
Pavlik harness (flexion abduction orthosis) in children younger than 4-5 months
Older children may need surgery
What are poor prognostic factors for ALL?
Age <2 or >10 WBC >20 at diagnosis T or B cell surface markers Non Caucasian Male sex
What test is used for newborn hearing screening?
Otoacoustic emission test
Computer generated click played through small ear piece
Presence of soft echo indicates healthy cochlea
What test is done if otoacoustic emission test has an abnormal result in a newborn?
Auditory brainstem response test
What is the problem in prader willi syndrome?
Deletion of chromosome 15 paternal gene not received
What is the first sign of puberty in males?
Testicular growth at around 12 years
Volume >4ml indicates puberty
What is the first sign of puberty in females?
Breast development at around 11.5 years
What are features of bartters syndrome?
Failure to thrive Polyuria Polydipsia Hypokalaemia Normotension Weakness
What are features of peutz jeghers syndrome?
Hamartomatous polyps in GI tract
Pigmented lesions on lips, oral mucosa, face, palms and soles
Intestinal obstruction: intussusception
GI bleeding
What are some causes of minimal change nephropathy?
Idiopathic
Drugs: NSAIDs, rifampicin
Hodgkin’s lymphoma
Infectious mononucleosis
What is the pathophysiology of minimal change nephropathy?
T cell and cytokine mediated damage to glomerular basement membrane - polyanion loss
Reduction of electrostatic charge - increased glomerular permeability to serum albumin
What are features of minimal change nephropathy?
Nephrotic syndrome
Normotension
Highly selective proteinuria
Electron microscopy shows fusion of podocytes
What is the most useful investigation to screen for complication of Kawasaki disease?
Echo to look for coronary artery aneurysm
What are features of Kawasaki disease?
High grade fever lasting over 5 days, resistant to antipyretics
Conjunctival injection
Bright red, cracked lips
Strawberry tongue
Cervical lymphadenopathy
Red palms of hands and soles which later peel
What is the management of kawasaki disease?
High dose aspirin
IV immunoglobulin
Echo
What is a suitable screening test for childhood squints?
Corneal light reflection test
What is the difference between a concomitant and paralytic squint?
Concomitant: imbalance in extraocular muscles, convergent more common
Paralytic: paralysis of extraocular muscles
What test is used to identify the nature of a squint in a child?
Cover test
What is the management of squint in children?
Eye patches to prevent amblyopia
Referral to secondary care
What is the most common cause of death in the first year of life?
Sudden infant death syndrome
What are risk factors for sudden infant death syndrome?
Prematurity Parental smoking Hyperthermia Putting baby to sleep prone Male Multiple births Bottle feeding Social class IV and V Maternal drug use Winter
What are risk factors for surfactant deficient lung disease?
Prematurity Male Diabetic mother C section Second born of premature twins
What is management of surfactant deficient lung disease?
Maternal steroids to induce lung maturation
Oxygen
Assisted ventilation
Exogenous surfactant given via endotracheal tube
What are the different classifications of squint?
Esotropia: towards nose
Exotropia: temporally
Hypertropia: superior
Hypotropia: inferior
What are features of fragile x in males?
Learning difficulties Large low set ears, long thin face, high arched palate Macroorchidism Hypotonia Autism Mitral valve prolapse
What is palivizumab and what is it used for?
Monoclonal antibody used to prevent respiratory syncytial virus in children at increased risk of severe disease: premature, lung or heart abnormality, immunocompromise
What is precocious puberty?
Development of secondary sexual characteristics before 8 years in females and 9 years in males
What are features of HSP?
Palpable purpuric rash over buttocks and extensor surfaces of arms and legs
Abdominal pain
Polyarthritis
Features of IgA nephropathy: haematuria, renal failure
What is the management for HSP?
Analgesia for arthralgia
Supportive treatment for nephropathy
What is an umbilical granuloma?
Overgrowth of tissue which occurs during the healing process of the umbilicus
Common in first few weeks of life
Small red growth, usually wet and leaks clear or yellow fluid
What are features of acute epiglottis?
Rapid onset
High temperature
Stridor
Drooling of saliva
How are paediatric maintenance fluids calculated?
100ml/kg/day for 0-10kg
50ml/kg/day for 11-20kg
20ml/kg/day beyond that
What is the triad of symptoms seen in haemolytic uraemic syndrome?
Acute renal failure
Microangiopathic haemolytic anaemia
Thrombocytopenia
What are causes of haemolytic uraemic syndrome?
Post dysentery - E. coli Tumours Pregnancy Ciclosporin COCP SLE HIV
What is the management of haemolytic uraemic syndrome?
Fluids
Blood transfusion
Dialysis
What is toddlers diarrhoea?
Stools containing undigested food
Chronic non specific diarrhoea
Should remit as child grows up
More common in boys age 1-5
What are causes of neonatal hypoglycaemia?
Maternal diabetes mellitus Prematurity IUGR Hypothermia Neonatal sepsis Inborn errors of metabolism Beckwith wiedemann syndrome
What is beckwith wiedemann syndrome?
Overgrowth disorder present at birth Increased risk of childhood cancer Macroglossia Macrosomia Microcephaly Neonatal hypoglycaemia Hepatoblastoma
What dysmorphic features are associated with Down’s syndrome?
Hypertelorism (wide spaced eyes) Downward slanting palpebral fissures Epicanthal folds Short broad nose Deeply grooved philtrum Small chin and short neck Full lips with high wide peaks to vermillion border of upper lip Low set ears Single palmer crease Sandal gap - wide space between first and second toe
What infections do infants with SCID usually present with?
Secondary to lack of T cell function: pneumocystis jirovecii, systemic candidiasis, generalised herpetic infections
What is the most common cause of vomiting in infancy?
GORD
What are risk factors for GORD in infants?
Preterm delivery
Neurological disorders
How should GORD in infants be managed?
Advise regarding head position during feeds - 30 degree head up
Sleep on backs
Ensure not being over fed, consider trial of smaller more frequent meals
Trial thickened formula
Trial of alginate therapy (gaviscon)
PPI or H2 antagonist if unexplained feeding difficulty, distressed, faltering growth
What are complications of GORD in infants?
Distress Failure to thrive Aspiration Frequent otitis media Dental erosion
What is ebsteins anomaly?
Posterior leaflets of tricuspid valve displaced anteriorly towards apex of right ventricle
Tricuspid regurg (pan systolic murmur)
Tricuspid stenosis (mid diastolic murmur)
Enlargement of right atrium
With which drug used during pregnancy is ebsteins anomaly associated?
Lithium
When does the Moro reflex usually disappear?
3-4 months
What are brushfield spots and in which condition are they seen?
White/grey spots on periphery of iris due to aggregation of connective tissue
Seen in Down’s syndrome
What are the different types of JIA? How common are they?
Oligoarticular JIA (50% of JIA) Polyarticular JIA - RF negative (25%) Polyarticular JIA - RF positive (5%) Systemic-onset JIA (5-10%) Juvenile psoriatic arthritis (2-15%) Enthesitis-related arthritis (2-10%) Undifferentiated arthritis (1-10%)
What is Quebec scoring for congenital hypothyroidism?
Feeding problems Constipation Lethargy Hypotonia Coarse facies (3) Macroglossia Open posterior fontanel (1.5) Dry skin (1.5) Mottling of skin Umbilical hernia If score is >4/13 hypothyroidism is suspected
How does oligoarticular JIA present?
Arthritis affecting 1-4 joints in the first six months. 70% patients are ANA positive
Extended oligoarthritis: more than four joints affected after six months
Persistent oligoarthritis: no more than four joints affected after six months
It usually presents in those under 6 years old and is more common in females.
It typically presents with one or two swollen joints causing stiffness and reduced movement but often not much pain. The child usually feels well.
The knee and ankle are most commonly affected.
What features of a murmur in a newborn suggest that it is innocent?
Sensitive: changes with position or respiration Short duration: not holosystolic Single: no associated clicks or gallops Small: small area or non radiating Soft: low amplitude Sweet: not harsh sounding Systolic
What complications can children with Down’s syndrome get?
Heart defects Coeliac disease Imperforate anus Hirschsprung's disease Cataracts Nystagmus Hearing loss Hypothyroidism Alzheimer's Acute leukaemia: mainly ALL Epilepsy
What is langerhans cell histiocytosis?
Clonal proliferation of langerhans cells capable of migrating from skin to lymph nodes, bones, pituitary and thyroid
What chest xray findings might you expect to see with coarctation of the aorta?
Cardiomegaly
Increased pulmonary vascular markings
What chest xray findings might you expect to see with tetralogy of fallot?
Concavity of left heart border - boot shape
Decreased pulmonary vascular markings
What are features of kawasaki disease?
High grade fever lasting >5 days which is resistant to antipyretics Conjunctival injection Bright red cracked lips Strawberry tongue Cervical lymphadenopathy Red palms and soles which later peel
What is the management of kawasaki disease?
High dose aspirin
IV immunoglobulin
Echo to screen for coronary aneurysms
When does newborn blood spot screening occur?
Day 5
Which disorders are tested for on a newborn blood spot test?
PKU Congenital hypothyroidism Sickle cell disease Cystic fibrosis MCADD (medium chain acyl CoA dehydrogenase deficiency Maple syrup urine disease Isovlaeric acidaemia Glutaric aciduria type 1 Homocystinuria
What test is done for newborn hearing screening?
Automated otoacoustic emissions
Which test is used if an automated otoacoustic emissions screening test shows no clear response?
Automated auditory brain stem response
What is a Morgagni hernia?
Congenital diaphragmatic hernia
Usually right sided containing transverse colon
What is a bochdalek hernia?
Congenital diaphragmatic hernia
Tend to be left sided containing stomach
What are protective factors for successful development in a child?
Good parent-child relationship Easy, outgoing temperament Positive peer influence Successful school experiences Caring adult role models Participation in pro-social groups Access to needed services, e.g. healthcare, mental health, crisis intervention
What is the diagnostic criteria for intellectual disability?
IQ 70 or less on an individually administered IQ test
Onset before age 18 years
Concurrent deficits or impairments in adaptive functioning in at least two of these areas: communication, self care, home living, social and interpersonal skills, use of community resources, self direction, functional
academic skills, work, leisure, health, or safety
What is the definition of mild, moderate and severe intellectual disability?
Mild ID: IQ 50/55 to 70. School may acquire skills up to 6th grade level. Social and Communication Skills develop spontaneously. May first be detected in school. May acquire vocational skills and be self-supportive
Moderate ID: IQ 35/40 to 50/55. Social and Communication Skills develop, but impaired. Early detection (before entering school). School unlikely to progress past 2nd grade level. May work under close supervision (sheltered workshop)
Severe ID: IQ 20/25 to 35/40. School May learn to sight-read (survival
words). Social/Communication Skills little or no communicative speech. Often display poor motor development. May acquire elementary hygiene skills and perform simple tasks; unable to benefit from vocational training
Profound ID: IQ Below 20/25, Social and Communication Skills rarely have communicative speech efforts; minimal sensorimotor abilities. Require constant aid and supervision; nursing care
What are examples of pervasive developmental disorders?
Autism Spectrum disorder
Rett’s Disorder
Childhood Integrative Disorder
PDD, not otherwise specified
What are some organic associations with autism?
Congenital Rubella
PKU
Tuberous Sclerosis
Fragile X Syndrome
What is Aspergers disorder?
High functioning autism
Impaired use of non-verbal communication (gaze, posture, gestures regulating to social interaction)
Lack of interactive play, impaired peer relations
Stereotypic, repetitive mannerisms
No delays in language and cognitive development
What is the management of autism?
Mainstay: Early intervention; speech and language services; structured behavioral and educational programs; OT, PT
Medications: To control seizures, hyperactivity, severe aggression, SIB, repetitive behaviors or mood disorders
What is ADHD?
Persistent pattern of inattention and/or hyperactivity more frequent and severe than istypical of children at a similar level of development
Onset before age 7
Impairment in at least two settings: social, academic, or work
Duration at least six months
Inattention, Hyperactivity, Impulsivity
What are management options for ADHD?
Stimulants: Methylphenidate, Dextroamphetamine
Non-Stimulants: Atomoxetine, Clonidine, Guanfacine, Bupropion, TCAs (atypical antipsychotics for treatment unresponsive cases)
Psychotherapy: Behavioral modifications, environmental structuring, parental Education and training, social skills training
What is a tic?
Sudden, rapid, recurrent, nonrhythmic, stereotyped motor movements or vocalizations
What are different DSM diagnoses for tic disorders?
Tourette’s Syndrome
Chronic Motor Tic DisorderChronic Vocal Tic Disorder
Transient Tic Disorder
Tic Disorder NOS
What is oppositional defiant disorder?
Recurrent pattern of negativistic, defiant, disobedient and hostile behavior towards authority figures
Duration > 6 Months
Impairment in social, academic and work settings
Symptoms not part of the mood or thought disorder
What is treatment for oppositional defiant disorder?
Parent training (PCIT)
Individual psychotherapy
Family Therapy
What is conduct disorder?
Aggression to people and animals
Destruction of property
Deceitfulness or theft
Serious violation of rules
What are treatment options for conduct disorder?
Multimodality treatment programs Environmental structuring Family Therapy Group Therapy Ind. Therapy – problem solving skills Medications as adjuncts
What is separation anxiety disorder?
Developmentally inappropriate and excessive anxiety about separation from caretakers or home, of at least 4 weeks duration with onset before 18 years
Can lead to school refusal (school phobia)
Associated with physical complaints, fear of sleeping alone, worries about parent’s safety
How does childhood depression present?
Irritability Sleep cycle disturbance Oppositional behavior Social isolation Crying spells
What treatment options are used for childhood mental health problems?
Psychotherapy: Individual Therapies (play, behavioral, cognitive, supportive, dynamic), Family Therapy and Parent Training, Group Therapy - especially important for adolescents
Pharmacological: SSRIs, Ritalin, valproate, lithium, TCAs, antipsychotics
Which professionals are involved in CAMHs services?
Psychiatrists Psychologists Social workers Nurses Psychological therapists – this may include child psychotherapists, family psychotherapists, play therapists and creative art therapists Support workers Occupational therapists Primary mental health link workers Specialist substance misuse workers Peads
Who can refer to CAMHS?
GP
School or college – for example, a teacher, pastoral lead, school nurse or special educational needs co-ordinator (SENCO)
Health visitors
General hospitals
What are criteria for urgent admission in a child with pneumonia symptoms?
Tachypnoea, measured as: <6 months RR >55/60. <2 years RR >40. <6 years RR >34 Nasal flaring In-drawing chest Cyanosis Significant crepitations Oxygen saturation 95% or less
What are worrying features for meningococcal meningitis?
Non-blanching rash, particularly with one or more of the following: An ill-looking child CRT 3 or more seconds Lesions larger than 2 mm in diameter (purpura) Neck stiffness Decreased level of consciousness Bulging fontanelle Convulsive status epilepticus Photophobia
What features might make you think of herpes simplex encephalitis?
Focal neurological signs Focal seizures Decreased level of consciousness Recent herpes infection in household / nursery / school Immunosuppression
What proportion of children who have a febrile convulsion will go on to have further episodes?
1/3
By what age do febrile convulsions usually stop?
By age 6
Under what circumstances does a febrile convulsion warrant a 999 call?
First fit
Signs of underlying meningitis or encephalitis
Seizure not terminating within 5 mins, risk of status epilepticus
Respiratory involvement call 999
Specialist care will assess, take urinalysis and may do EEG / LP at first fit
When should a complicated UTI be considered in a child?
children >3 months if fever plus: Persistent vomiting Lethargy Irritability Abdominal pain or tenderness Urinary frequency or dysuria Offensive urine or haematuria
What is Kawasaki disease?
Medium vessel vasculitis
Fever lasting longer than 5 days and at least four of following:
Bilateral conjunctival injection
Change in peripheral extremities e.g oedema, erythema or desquamation
Change in upper respiratory tract mucous membranes e.g injected pharynx, dry cracked lips or strawberry tongue
Polymorphous rash
Cervical lymphadenopathy
What is the management of measles exposure?
Notifiable disease, swab
MMR vaccination (over 6 months old), ideally within 72 hours of exposure
Human normal immunoglobulin, within five days of exposure for children and adults with compromised immune systems
Pregnant women who are exposed to measles may also be considered for IM normal immunoglobulin
What is the management for rubella?
Keep child away from school for seven days after rash appears
Use antipyretics for fever - avoid aspirin in children, due to danger of Reye’s syndrome
Ask about any contact with pregnant women
Notifiable
What is the management for scarlet fever?
Penicillin or azithromycin if penicillin-allergic for 10 days
Notifiable
What factors lead to spread of scabies?
Poverty and overcrowding
Institutional care, such as rest homes, hospitals, prisons
Refugee camps
Individuals with immune deficiency
How do you catch scabies?
Nearly always acquired by skin-to-skin contact with someone else with scabies
Contact may be quite brief such as holding hands with an infested child
Sometimes sexually transmitted
Occasionally scabies is acquired via bedding or furnishings
Why does the scabies rash appear 4-6 weeks after infestation?
Hypersensitivity reaction
What is the management of scabies?
Treat whole family simultaneously
Warn patients that itch persists for few weeks after mites killed
Use parasitacide: 1st line Permethrin liquid or cream, apply once weekly for 2 doses to whole body, then wash off after 8hours
Or Malathion
What are causative organisms of cellulitis?
Streptococcus or Staphylococcus spp
What are risk factors for cellulitis?
Immunosuppression, wounds, toe web intertrigo, minor skin injury, Diabetes, venous insufficiency, lymphoedema
What is the management of cellulitis?
Elevation of any affected limbs
Prescribe analgesia as necessary (paracetamol or ibuprofen)
Flucloxacillin 500 mg four times daily (in adults) is usually given as first-line in uncomplicated infection. In sufficient doses, this covers both beta-haemolytic streptococci and penicillinase-resistant staphylococci
Erythromycin 500 mg four times daily can be used if the patient is penicillin-allergic and clarithromycin (500 mg twice daily) if patient is intolerant to erythromycin
What is staphylococcal scalded skin syndrome?
Production of epidermolytic toxin from benzylpenicillin-resistant (coagulase positive) staphylococci infection
Develops within a few hours to a few days
Develop fever, irritability, widespread skin erythema, then fluid-
filled blisters which rupture to leave area that looks like a burn
Perioral crusting is typical
Lesions are very painful
What is the management of staphylococcal scalded skin syndrome?
Admission
IV Antibiotics – flucloxacillin, clindamycin
Analgesia
Skin care
How can a correct diagnosis of fungal infection be confirmed?
Skin scrapings, hair or nail clippings, skin swabs
What is pityriasis versicolor?
Non contagious, superficial yeast infection
Can lead to temporary hypopigmentation
Pityrosporumovale is normal commensal - becomes a pathogen if increased humidity at skin surface and/or increased sebum production
What is management of pityriasis versicolor?
Ketoconazole or Selenium Sulphide shampoo overnight then wash off, apply twice weekly for 3 weeks
Topical imidazole antifungals an alternative
What are exacerbating factors for atopic eczema?
Infections Allergens Sweating Heat Stress
What is the management of eczema?
General measures: Avoid known exacerbating factors, Regular emollients, Soap substitutes
Topical therapies: Steroids, Immunomodulators eg tacrolimus, pimecrolimus
Oral therapies: Antihistamine for symptomatic relief, Antibiotics/fungal for secondary infection
Phototherapy
Immunosuppressants: Oral prednisolone, Ciclosporin, Azathioprine
Which heart abnormalities are associated with Down’s syndrome?
AVSD
VSD
Tetralogy of fallot
Which heart abnormalities are associated with Edwards and patau syndromes?
VSD
Other various defects
Which heart defects are associated with turners syndrome?
Coarctation of aorta
Aortic stenosis
Which heart defects are associated with diGeorge syndrome?
Truncus arteriosus
Interrupted aortic arch
Tetralogy of fallot
Which heart defect is associated with Williams syndrome?
Supravalvar aortic stenosis
Which congenital heart defects are associated with maternal diabetes?
Transposition of great arteries
VSD
HOCM
With which foetal heart problem is maternal SLE associated?
Heart block
With which heart defects is teratogenic exposure to rubella associated?
Coarctation of aorta
VSD
Patent ductus arteriosus
With which heart defect is teratogenic exposure to lithium associated?
Ebsteins anomaly
What are clinical manifestations of congenital heart disease?
Cardiac failure: Lt to Rt shunt – first few months
LV outflow obstruction – few days/weeks
Functional failure-cardiomyopathy– tachypnoea, tachycardia, poor feeding, vomiting, sweating, failure to thrive, hepatomegaly
Central Cyanosis: poor lung perfusion Rt to Lt shunt, duct dependant -acutely unwell neonate– cyanotic spells - Tetralogy of fallot
Which congenital heart diseases cause cyanosis?
5 Ts: Tetralogy of fallot Transposition great arteries Tricuspid atresia Total anomalous pulmonary venous drainage Truncus Arteriosus Pulmonary atresia
What is a stills murmur?
Functional heart murmur Commonest age group 3-7yr Vibratory/musical in quality At apex, radiation to carotids May only be audible in supine position
What are features of innocent murmurs?
Change in intensity with posture Always systolic (except venous hum –continuous) ASYMPTOMATIC
What investigations should be done if a baby is found to have a murmur?
Chest X-ray – cardiac size, lung vascularity (left to right shunt, pulmonary plethora)
ECG – chamber enlargement
Hyperoxia test - to differentiate between cardiac and pulmonary cause of cyanosis in neonate
Echocardiography - definitive diagnosis
Consider chromosomal analysis (T21, 22q11)
Which acyanotic heart defects cause pulmonary plethora?
VSD
ASD
PDA
Severe LV outflow obstruction/ hypoplastic left heart
Which cyanotic heart defects cause pulmonary oligaemia?
Severe PS/atresia
TOF
How should a duct be kept open in a neonate with a duct dependent lesion?
PGE1 infusion
Which heart defects are duct dependent?
Coarctation of aorta Interrupted aortic arch Critical aortic stenosis Hypoplastic left heart syndrome TGA
Why does squatting help cyanotic spells in tetralogy of fallot?
Increase systemic resistance, increase return to right heart so increase pulmonary blood flow
What are components of tetralogy of fallot?
Pulmonary stenosis
Large VSD
Overriding aorta
RVH
What are neonatal fluid requirements from birth?
Day 1: 60mls/kg/day
Day 2: 90mls/kg/day
Day 3: 120mls/kg/day
Day 4: 150mls/kg/day
Day they are born is day 0 age but day 1 fluids
Use birth weight for calculation until regained birth weight
What size fluid bolus should be given to a shocked child?
20mls/kg 0.9% saline
Unless DKA/Trauma/cardiac – 10mls/kg
How do you calculate maintenance fluids for a child?
0.9% saline with 5% dextrose +/- 10 mmol KCl per 500ml bag
100mls/kg for first 10kg
50mls/kg for second 10kg
20mls/kg after this
How do you calculate rehydration fluids in a child?
If shocked add 100mls/kg to maintenance requirements. If not shocked add 50mls/kg or
Weight x 10 x percentage dehydration
What can be used as oral rehydration fluids in a child?
Normal milk
Oral rehydration salts
Avoid fruit juices. fizzy drinks, plain water
How much loss of birth weight is acceptable in a newborn?
Loss of up to 8% birth weight is acceptable. Any more is too much, are they dehydrated? Failing to thrive
What is cerebral palsy?
Primary abnormality of movement and posture secondary to a non-progressive lesion of a developing brain
Which brain regions can be affected in cerebral palsy and what defects does this lead to?
Injury to extrapyramidal system causes- dystonic CP (basal ganglia) or ataxic CP (cerebellum)
Injury to Pyramidal system (corticospinal tract) causes Spastic CP-Diplegia, Quadriplegia
What are some causes of cerebral palsy?
Prematurity: 20% at 28/40 Vs 4% at 32/40 LBW Infection/Inflammation Coagulopathy Antenatal bleeding Pregnancy induced hypertension/Pre-Eclampsia Multiple pregnancy Foetal distress Sepsis Kernicterus (Severe unconjugated jaundice) Severe and prolonged hypoglycaemia
What problems are associated with cerebral palsy?
Motor –gross, fine motor Vision and Hearing Speech Epilepsy Feeding Gastro Oesophageal Reflux Constipation/Incontinence Secondary disabilities – hips, spine, arthritis Learning disabilities
What are management options for cerebral palsy?
Treatment of Spasticity- Physiotherapy, Pharmacology- Systemic, Local, Intrathecal, Surgery- Orthopaedic, Peripheral Nerves, Spinal Roots
Treatment of Associated Problems
Enhancing quality of life
What is a febrile seizure?
Seizures precipitated by fever without evidence of CNS infection or another defined cause
What are atypical features of a febrile seizure?
Prolonged, Focal or associated with Todd’s paresis
What is epilepsy?
Recurrent, Unprovoked Seizures due to abnormal hyper-synchronous discharge of cortical neurons
What are different forms of generalised epilepsy?
Myoclonic Atonic Tonic Tonic clonic Infantile Spasms
At what age goes childhood absence epilepsy usually occur?
Uncommon before age 5
What are features of childhood absence epilepsy?
Vacant episodes, absence, sometimes associated with eye flickering
Abrupt start and stop, No post event drowsiness
Very brief lasting 5 to 15 seconds
Occurs in clusters (multiple)
Precipitated by hyperventilation
EEG-3 /seconds spike and wave
What is west syndrome?
Epileptic Encephalopathy
Age of onset: 3 to 12 months (90% before 1 yr of age)
Infantile Spasm: Sudden, Generalised, Symmetrical Contractions of muscles of limb, trunk and neck occuring in clusters
EEG- Hypsarrhythmia-Chaotic Background
What are some causes of West syndrome?
Hypoxic ischaemic encephalopathy, Infections, Acquired Brain Injury, Cortical malformations, Tuberous Sclerosis
Which antiepileptics are used in absence seizures?
Sodium Valproate, Ethosuximide
What treatments are used for west syndrome?
Steroid (ACTH), Vigabatrin, Multiple Antiepileptic medications
What features are important to ask about in a floppy baby?
Hypotonia Reduced foetal movements Polyhydramnios Needing prolonged resuscitation/Artificial Ventilation Poor feeding/Choking/Aspiration Poor cry Poor movements Alert/ not alert Fasciculation
What might be features of a floppy but strong baby?
UMN lesion Increased tendon reflexes Extensor plantar response Sustained ankle clonus Global developmental delay Microcephaly +/-Dysmorphism Prolonged Seizures Axial weakness a significant feature
What may be causes of a floppy but strong baby?
Hypoxic ischaemic encephalopathy, Hypoglycaemia, Chronic Encephalopathies, Down’s Syndrome, Prader Willi Syndrome, Cerebral Malformations
What might be features of a floppy and weak baby?
Lower motor neuron disorder Hypo- to areflexia Selective motor delay Normal head circumference and growth Preserved social interaction Weakness of limb muscles Low pitched weak cry Tongue fasciculations
What are some causes of a floppy and weak baby?
Neuro-muscular-Congenital Myopathy, Myotonic dystrophy, Myaesthenia, Motor Sensory Neuropathy, Spinal muscular atrophy
What investigations would you do for a floppy baby?
Bloods: Creatine Kinase, TFT, Congenital Infections (TORCH), Genetics, Metabolic EMG Nerve Conduction Studies ECG MRI Brain Nerve/Muscle Biopsy
What are management options for a floppy baby?
Physio to prevent contractures
OT to facilitate activities of daily living
Prevention/Treatment of Scoliosis
Evaluation and treatment of associated cardiac dysfunction
Feeding
Management of Gastro-Oesophageal Reflux
Orthopaedic Interventions
Prevention and prompt treatment of respiratory infections
What is the algorithm for paediatric basic life support?
Safe, Stimulate, Shout Airway opening manoeuvres Check for breaths (Look/Listen/Feel) 5 rescue breaths Check signs of life – if absent 15:2 COMPRESSIONS:BREATHS
What equations can be used to estimate a child’s weight?
For Infants <12 months: Weight (kg) = (age in months + 9)/2
For Children aged 1-5 years: Weight (kg) = 2 x (age in years +5)
For Children aged 5-14 years: Weight (kg) = 4 x age in years
What is life threatening asthma?
Sats <92% Silent chest Poor respiratory effort Altered consciousness Cyanosis PEFR <33% best/predicted
What is acute severe asthma?
PEFR 33-50% best or predicted Sats 92% or above Can't complete sentences Resp rate 25 or more Pulse 110 or more
What is asthma emergency management?
High Flow Oxygen Nebulised Salbutamol Nebulised Ipratropium Bromide Prednisolone/Hydrocortisone IV Magnesium IV Salbutamol bolus/infusion IV Aminophylline Nurse in HDU Continuous monitoring – Sats/Pulse Repeated nebulisation depending on response Consider CXR and blood gas Daily prednisolone for 3-5 days May need to discuss with ICU
What are signs of respiratory distress in an infant?
Tachypnoea Subcostal/intercostal recessions Tracheal tug Head bobbing Nasal flaring Grunting
What is the management of bronchiolitis?
High flow O2 – humidified
Hydration – NG feeding or IV fluids
May need to consider high-flow or CPAP
Worst on day 4
What is the management of shock in children?
High flow oxygen
Venous access – glucose, blood gas
Fluids 20 ml/Kg 0.9% NaCl (except in trauma - 10ml/kg)
Treat low glucose
Involve senior help early
May need inotropic support - central lines
Specific treatment: Iv abx/antivirals if sepsis, IM adrenalin if anaphylaxis
Why should you have a higher index of suspicion for sepsis in a child less than 8 weeks old with a temperature?
Not yet immunised
What septic screen should be performed on a child?
Blood – culture, GLUCOSE, gas, FBC/CRP
CXR
Urine
LP if stable enough and no purpuric rash
What is the most important and possibly fatal problem associated with DKA?
Cerebral oedema from correcting fluids too quickly
What can be done to manage cerebral oedema?
Mannitol or hypertonic saline
Head up
Intubate and ventilate, keep CO2 low normal
ITU
What is round pneumonia?
Defined well rounded opacities representing regions of infective consolidation usually only seen in children due to immature airways - lack of development of interalveolar communication and collateral airways
Repeat xray in 4-6 weeks, if it is pneumonia it should have resolved. If not resolved, explore other options
What are normal chest X-ray features in a child?
Cardiothoracic ratio 60%
Kink of trachea to the right due to prominent thymus
Diaphragm lies at the level of 6th anterior rib
Thymus
What is the sail sign on a child’s chest X-ray?
Thymus shadow - normal
What are radiographic findings of acute respiratory distress syndrome in an infant?
Diffuse symmetric reticulogranular densities
Prominent central air bronchograms
Generalised hypoventilation
What are complications of respiratory distress syndrome?
Compliance of lungs is too low, or mean airway pressure is too high, barotrauma will result
Signs of barotrauma should be identified on neonatal CXR: Pneumothorax, Pulmonary interstitial emphysema (PIE)
Results from rupture of alveoli with air accumulating in peribronchial and perivascular spaces
What is the deep sulcus sign?
Deep lucent ipsilateral costophrenic angle within nondependent portions of pleural space
Indicator of pneumothorax on a supine chest X-ray
What might be seen on an abdominal film in a neonate with necrotising enterocolitis?
Dilated loops of bowel Intramural gas Portal venous gas Pneumoperitoneum - football sign Riglers sign
Which injuries can be picked up on imaging that may suggest NAI?
Metaphyseal corner fractures
Fractures and history not consistent with development
Rib fractures – especially posterior and lateral ribs
Subdural haematoma and skull fractures
Other neurological injury – hypoxic ischaemic brain injury, vertebral fractures, spinal haematoma
Fractures healing at different stages
What are chest manifestations of cystic fibrosis?
Bronchiectasis
Pneumothorax
Infection (consolidation)
Collapse secondary to mucus plugging
What does a double bubble sign suggest?
Prominent stomach and first part of duodenum
Duodenal atresia
What does rickets look like on an X-ray?
Cupped and frayed metaphyses
What are signs of clinical dehydration in a child?
Altered responsiveness Skin colour unchanged Warm extremities Sunken eyes Dry mucous membranes Tachycardia Tachypnoea Normal peripheral pulses Normal capillary refill time Reduced skin turgor Normal blood pressure
What are signs of clinical shock in a child?
Decreased level of consciousness Pale or mottled skin Cold extremities Sunken eyes Dry mucous membranes Tachycardia Tachypnoea Weak peripheral pulses Prolonged capillary refill time Reduced skin turgor Hypotension
What is the management for a child with acute gastroenteritis with no signs of clinical dehydration?
Rehydrate orally using ORS
Frequent small volumes of ORS (5-10 mL every 5 min)
Continue breast feeding
Discourage Fruit juices and carbonated drinks
Continue to supplement with ORS for each watery stool/vomit (5-10 mL/kg per watery stool)
Do not withhold food unless vomiting
Full feeding appropriate for age, well tolerated with no adverse effects
What is the management for a child with acute gastroenteritis with signs of clinical dehydration?
ORS 50 mL/kg oral over 4 hours for deficit as well as maintenance fluids
If not tolerating oral rehydration (refuses, vomits, takes insufficient
volume), use NG tube
Review after 4 hr when rehydrated start a normal diet, and continue maintenance fluids and supplementary ORS for each watery stool
Consider IV rehydration if not improving
Consider Oral Ondansetron
What are causes of chronic diarrhoea (>4 weeks) in a child?
Infections
Endocrine: Hyperthyroidism, Adrenal insufficiency
Chronic nonspecific diarrhoea
Coeliac disease
IBS
IBD
Infections
Carbohydrates malabsorption: Lactase deficiency, Glucose-glactose
malabsorption
Dietary: Cow’s Milk/soy protein intolerance
Immune defects: Agammaglobulinemia, IgA deficiency, AIDS
Metabolic: Familial chloride diarrhoea
Cystic fibrosis
What proportion of children with constipation will develop chronic symptoms requiring referral?
1/3
What are normal bowel habits for babies?
Significant variation in babies : stool with each feed or a stool every few days
Breast fed: lots of variability in bowel habit, more than formula fed
What are causes of constipation in children?
Idiopathic: Diet, Stool holding, Emotional problems/phobia (autism)
Due to underlying disease: Neurological conditions (Spina bifida, cerebral palsy), Cystic Fibrosis, Hirsprungs or abnormal bowel development (Meconium >48 hours - delayed, Ribbon stools)
Side effects of medications
Maltreatment or abuse
What features in a child under 1 would indicate constipation?
<3 type 3 or type 4 stools/week (excl. Breast fed babies after 6wk) Passage of hard large stools Type 1 ‘rabbit droppings’ Distress on stooling Bleeding with hard stool Straining Anal fissures
What findings would indicate constipation in a child over 1?
<3 type 3/4 stools/week Overflow/soiling/smelly Type 1 rabbit droppings Large infrequent stools that block the toilet Poor appetite Waxing and waning abdo pain Retentive posturing Straining /anal pain Anal fissures Blood with bowel movements
What are red and amber flags for constipation in a child?
Red Flags: Present at birth /first few weeks of life, Failure to pass meconium within 48hrs of birth, Ribbon stools, Previously unknown/undiagnosed leg weakness or motor delay, Abdo distension and vomiting, Abnormal appearance/position/patency of anus, Abnormal spinal examination, Lower limb deformities, Abnormal reflexes
Amber Flags: Faltering growth, Disclosure /evidence raising concerns over maltreatment
What tips can be given for early management of constipation in a child?
Drinks: Ensure adequate fluid intake, Additional water between feeds, Diluted fruit juice or pureed fruit/veg, Avoid fizzy/sugary drinks/milk to quench thirst, Fruit juices containing fructose/sorbitol have a laxative action
Diet: High fibre diet, Offer fruit with meals, Add powered bran to foods Regular toileting: A set time, not rushed, Reward system when stool passed in toilet/potty, Remain relaxed when accidents happen
What is the management of impacted constipation in a child?
Movicol Paediatric Plain, Escalating dose regime mixed with a cold drink
If no disimpaction after 2 weeks add stimulant laxative
If unable to tolerate Movicol, Substitute stimulant laxative +/- osmotic laxative
Once disimpacted, maintenance doses approx half disimpaction dose
Continue at maintenance dose for several weeks after regular bowel habit established
Gradual reduction thereafter, over months
Rare occasions where laxatives may be required for years
What investigations should be done for a child presenting with faltering growth?
FBC U+E LFT TFT ESR, CRP Ig G,A,M FAB (functional antibody test) Anti TTG Urine CXR, T-spot, Mantoux
How does coeliac usually present in children?
Classically presents at 6-18 m of age once gluten is introduced Most common gastrointestinal manifestations Diarrhoea Vomiting FTT or weight loss Distended abdomen Abdominal pain Constipation
What pathological changes are seen in coeliac disease?
Villous atrophy Crypt hyperplasia Loss of enterocyte height Lamina propria infiltration Increased intra-epithelial lymphocytes
How is diagnosis of coeliac disease made?
Gliadin IgA/IgG Endomysial IgA Tissue Transglutaminase IgA (Anti body test of choice) IgA deficiency: False-negatives HLA typing Duodenal biopsy (Gold standard test)
What conditions are associated with coeliac disease?
Dermatitis herpetiformis
IgA deficiency (3-5%)
Autoimmune conditions: Thyroid disease, Type 1 diabetes, Addison’s, Sjogrens syndrome
What investigations should be done in a child suspected of having IBD?
FBC ESR LFTs including Albumin CRP pANCA (CD) GI endoscopy Radiology: Strictures, toxic megacolon
What is the treatment of pyloric stenosis?
Fluid resuscitation
Correct electrolyte imbalances
Pyloromyotomy
When does laryngomalacia usually resolve by?
Usually before 2 years
What are reasons that childhood deaths from infections have decreased in recent years?
Improved nutrition
Vaccination
Improved housing / hygiene
Antibiotics
What may be presenting symptoms of UTI in a child?
Fever Vomiting Anorexia Rigors Abdominal pain
What are causative organisms of pneumonia in children?
Other than neonatal period, viruses (eg RSV) are most common cause
Streptococcus pneumoniae (~70%)
Haemophilus influenzae
Atypical bacteria : Mycoplasma, Chlamydophila pneumoniae
Which antibiotics are used to manage pneumonia in children?
First line – Amoxicillin
In penicillin allergy or fail to respond – Macrolides
Atypical pneumonia - Macrolide
What is di George syndrome?
Microdeletion Chromosome 22
Hypocalcemia, recurrent infection (due to problems with T cell mediated response due to hypoplastic thymus), Cleft palate, low set ears, learning problems, Aortic arch anomalies (Rt sided aortic arch, inturrepted AA), Trucal arteriosis, VSD, PDA, TOF
What does TGA look like on a chest X-ray?
Egg on side shaped
Increased pulmonary marking
What are the 3 types of total anomalous pulmonary venous drainage?
Supracardiac
Cardiac
Infracardiac
What sign on chest X-ray suggests total anomalous pulmonary venous drainage?
Snowman sign/figure of 8
What are causes of cardiomegaly in a neonate?
Heart failure HOCM/DCM Pericardial effusion Significant Lt to Rt shunts Ebstein Anomaly
What are differentials for a widespread rash in a child?
Viral Rash Drug Rash Allergic Rash Scabies Erythema multiforme ITP Meningococcal Scarlet fever Urticaria Measles Rubella Chicken pox
What is heat rash/miliaria?
Occurs secondary to heat (warm climates)
Due to obstruction of sweat ducts with leakage of sweat into dermis/epidermis
Common in infant due to underdeveloped sweat glands
What is milia?
Epidermal inclusion cysts Pearly, yellow, 1-3mm diameter papules Face, chin, forehead 50% newborns Usually resolve in first month without treatment, but may persist for several months
What is a Mongolian blue spot?
Blue/black macular discolouration at base of spine and on buttocks
Occasionally on legs or other parts
Usually in dark skin, Afro-Caribbean or Asians
Fades slowly over few days to 1 yr
What is Vernix Caseosa?
Whitish greasy coat produced by epithelial cell breakdown in newborns
Protect skin from amniotic fluid in utero
What is erythema toxicum?
Benign Common after 2-3 days of life
Also called neonatal urticaria
White pinpoint papule with erythematous base
Fluid contain eosinophil
Comes and goes
Resolve without any treatment self limiting in 2-4 days
What is the most common benign tumour of infancy?
Hemangioma (Strawberry Naevus)
What is the natural history of a strawberry naevus?
Begin as barely visible telangiectasia or red macules and grow in size until 9 month, then regress
60% occur on head and neck area
All gone by age 5yrs
How can large strawberry naevi be managed?
steriods or interferon-alpha
What is a salmon patch?
Naevus simplex
Common (40% newborns)
Small flat patches pink or red, poorly defined borders
Nape of neck (stork mark), forehead (angel kiss), eyelids and sacrum
Worse with crying
Not associated with extracutaneous findings
Fade during first month of life
What is a port wine stain?
Naevus flammeus
Less common than salmon patch, but permanent
Present at birth and darken with age
Large flat patch of purple or dark red skin with well defined borders
Persist in childhood then darkens and thickens
Extracutaneous defects Association: Glaucoma
Along Trigeminal (sturge-weber syndrome) may associate
with intracranial vascular anomalies/epilepsy
Laser Therapy for Cosmetic benefit
What are 2 different types of nappy rash?
Irritant rash(contact): most common occur when nappies not changed frequently (urine). Skin Flexures (Intertriginous areas) usually spared. Treat with emollient (aquous cream), barrier
cream (Zinc oxide), Nappy free period, Hydrocartisone cream
Candidal Rash involve skin flexures and associated satellite lesions. Treat with topical antifungal agents +/- hydrocortisone
What are the presenting features of HSP?
Rash: buttocks, extensor surfaces of legs and arms
Joint pain/swelling: knees and ankles
Abdominal pain: haematemesis, melaena, intussusception
Renal: Haematuria, nephrotic syndrome
What is the most common vasculitis in childhood?
HSP
What is the natural history of chickenpox?
Begins as itchy red papules progressing to vesicles on bright red base (dew drops on a rose petal) on stomach, back and face, and then spreading to other parts of body
Central umbilication of blisters follows rapidly, crusting and desquamation within 10 days
What are complications of chickenpox?
Bacterial superinfection Encephalitis Aseptic meningitis Pneumonitis DIC
What are the different types of impetigo?
Impetigo contagiosa and Bullous Impetigo
What causes impetigo?
Strep pyogenes or staph aureus
What is management of impetigo?
Advice about avoiding spread
Localised lesion- topical antibiotic eg Fucidin, mupircin cream
Remove crust with gentle washing
Widespread infection - oral flucloxacillin or erythromycin/augmentin
What are prodromal features of measles?
Fever Malasie Dry cough Coryza Conjunctivitis Photophobia
When are patients with measles infectious?
Contagious 4 days before rash and 4 days after
What are complications of measles?
Pneumonia Otitis media Tracheitis Febrile convulsions Encephalitis Subacute sclerosing panencephalitis Diarrhoea Hepatitis Appendicitis Corneal ulceration Myocarditis
What causes roseola infantum?
Human Herpes virus 6
What causes slapped cheek?
parvovirus B19
What causes molluscum contagiosum?
DNA pox virus
What are common features of turners?
Lymphoedema of hands and feet Short Stature Webbing of Neck Wide carrying Angle (cubitus valgus) Coarctation of Aorta Delayed Puberty-Amenorrhea Ovarian dysgenesis (infertility) Hypothyroidism Renal Anomalies-horseshoe kidney
What feature distinguishes noonan syndrome in a male from turners in a female?
Noonan: Rt sided PS
What are features of achondroplasia?
Short stature Macrocephaly Flat midface with prominent forehead Associated hydrocephalus Dental malocclusion Hearing loss Normal intelligence and normal lifespans
What are features of Williams syndrome?
Moderate Developmental Delay Typical personality (Cocktail) Characteristic facial features: Periorbital fullness, Stellate iris, Prominent lips/open mouth, Depressed nasal bridge Supravalvular aortic stenosis Hypercalcemia Renal anomalies Hoarse voice Microdeletion 7q11.3
What are features of DiGeorge syndrome?
Physical Birth Defects: congenital heart disease, cleft lip and palate
Medical Complications: endocrine, immune, skeletal, neurologic, GI
Communication Disorders: Velopharyngeal inadequacy, language delays
Developmental Delays / LD
Social/Behavioural Difficulties
Increased risk for psychiatric illness (25% lifetime)
What are features of foetal alcohol syndrome?
Saddle shaped nose Short palpebral fissures Epicanthal folds Indistinct philtrum Thin upper lip Mid facial hypoplasia/microcephaly Maxillary hypoplasia Developmental delay
What are markers of asthma from a peak flow chart?
Diurnal variation peak flow greater than 20% for at least 3 days in a week
Or improvement 10 mins after bronchodilator
What are the 3 pathological features of asthma?
Bronchospasm
Mucosal oedema
Mucous plugging
Which cause obstruction of expiration. Can breathe in but not breathe out which leads to hyperinflation
What are side effects of salbutamol?
tachycardia, tremor, hyperglycaemia, hypokalaemia
What is the asthma ladder for 5-12 year olds?
Inhaled SABA as required
Add inhaled steroid 200-400micrograms/day
Add LABA, if control not adequate can increase steroid to 400. If control not adequate, stop LABA, increase dose, add other agent - leukotriene or theophylline
Increase steroid to 800 micrograms
Refer to paeds, use steroid tablets
What is the asthma ladder for under 5s?
Inhaled SABA as required
Add inhaled steroid 200-400micrograms or leukotriene antagonist
In those taking steroid, add leukotriene. In those on leukotriene add steroid. If under 2 proceed to referral
Refer to paeds
What is cystic fibrosis?
Autosomal recessive
Mutation in CTFR gene: chloride channel
Absence / defect in channel leads to thicker mucous
Most common mutation is delta F508, part of Newborn Blood Spot screening
How can cystic fibrosis be diagnosed?
Sweat test (>60mmol/l Cl-suggests CF) Genetic testing
What are important management steps for cystic fibrosis?
Nebulised saline / antibiotics /mucolytics
Supplementation: pancreatic enzymes, fat-soluble vitamins, nutritional support
Chest physio
Aggressive treatment of chest infections with IV antibiotics
What are clinical signs of dehydration in a neonate?
Mild (5%): restlessness, slightly dry mucous membranes, normal skin elasticity, oliguria
Moderate (5-9%): sunken eyes, depressed fontanelle, decreased skin turgor, dry mucous membranes
Severe (10%): drowsy, irritable with signs of circulatory collapse (rapid weak pulses, delayed cap refil, low BP), sunken eyes, decreased skin turgor
What are risk factors for surfactant deficient lung disease/acute respiratory distress syndrome?
Prematurity Male sex Diabetic mother C section Second born of premature twins
What is a chest X-ray appearance in ARDS?
Ground glass appearance
Indistinct heart border
Low volumes
Bell shaped thorax
What is management of ARDS?
Prevention during pregnancy: maternal corticosteroids
Oxygen
Assisted ventilation
Exogenous surfactant given via endotracheal tube
How is persistent pulmonary HTN treated?
Nitric oxide - pulmonary vasodilation
What is persistent pulmonary HTN of the newborn?
Problem at birth meaning that lungs don’t properly fill with air and pressure remains high in vessels so blood can’t get in to pick up oxygen
What factors are associated with persistent pulmonary HTN of the newborn?
Meconium aspiration Infection - PROM, GBS Congenital abnormalities of heart and lungs Hyaline membrane disease Oligohydramnios
What are risk factors for meconium aspiration?
Pre eclampsia Maternal HTN Oligohydramnios Maternal infections Maternal drug use Placental insufficiency IUGR Post dates
What are causes of oligohydramios?
Foetal chromosomal abnormalities
Intrauterine infections
Drugs: PG inhibitors, ACE inhibitors
Renal agenesis or obstruction to urinary tract
IUGR
Post dates
Amnion nodosum: failure of secretion by cells of amnion
What are causes of polyhydramnios?
Intrauterine infections Rhesus isoimmunisation Chorioangioma of placenta Gut atresia Hydrops fetalis Twin to twin transfusion syndrome Maternal cardiac/renal problems Diabetes
In which babies is PDA more common?
Premature
Born at high altitude
Maternal rubella infection in first trimester
What are features of PDA?
Left subclavicular thrill Continuous machinery murmur Large volume, bounding, collapsing pulse Wide pulse pressure Heaving apex beat
What is the management of PDA?
Indomethacin closes connection in majority of cases
If associated with another heart defect amenable to surgery, PGE1 useful to keep duct open until after surgical repair
What are features of congenital CMV infection?
Growth retardation Pinpoint petechial “blueberry muffin” skin lesions Microcephaly Sensorineural deafness Encephalitis Hepatosplenomegaly
What are cutaneous features of tuberous Sclerosis?
Ash leaf spots which fluoresce under UV light
Shagreen patches (rough patches over lumbar spine)
Adenoma sebaceum (angiofibromas)
Subungual fibromata
Cafe au lait spots
What are neurological features of tuberous Sclerosis?
Developmental delay
Epilepsy (infantile spasms or partial)
Intellectual impairment
What is cyclical vomiting syndrome?
Commonly associated migraines
Severe nausea and sudden vomiting lasting hours to days
Prodromal intense sweating and nausea
Well between episodes
What are the most common heart problems in Down’s syndrome?
AVSD VSD PDA TOF ASD
What is an indication to start steroids in ITP?
If child develops bleeding complications
What is PKU?
Autosomal recessive disorder of phenylalanine metabolism due to defect in phenylalanine hydroxylase
High levels of phenylalanine lead to learning difficulties, seizures (infantile spasms)
Other features fair hair, blue eyes, eczema, musty odour to urine and sweat
