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
