Paeds Flashcards

Question

By when should the TNR reflex integrate?

Answer 1

3 and a half years

Answer 2

9 to 11 months

Answer 3

Palmar grasp - primitive | When object placed in infants hand and strokes palm, fingers close and they grasp it

Answer 4

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

Answer 5

Baby curves hip outward if lower back stroked next to spine

Answer 6

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

Answer 7

Hold baby in air in prone position | Maintain convex arc with head raised and legs slightly flexed

Answer 8

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

Answer 9

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

Answer 10

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?

Answer 11

``` Suck Rooting Grasp Moro Asymmetrical tonic neck Plantar Stepping Parachute ```

Answer 12

Antenatal Birth history Developmental history Family history

Answer 13

``` 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 ```

Answer 14

``` Head circumference and shape Posture Movement Limb and truncal tone Reflexes and neuro behaviour - sleep/cry/feeding/interaction ```

Answer 15

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?

Answer 16

``` Chromosome disorders i.e. Prader-Willi Metabolic diseases Spinal cord injuries Cerebral dysgenesis Hypoxic ischaemia injuries ```

Answer 17

Congenital hypomyelinating neuropathy Familial dysautonomia Infantile neuraxonal degeneration

Answer 18

Congenital and transient myasthenia gravis | Infantile botulism

Answer 19

Muscular dystrophies Metabolic myopathies Central core disease/ fibre myopathies

Answer 20

Central nervous system

Answer 21

Peripheral nervous system

Answer 22

``` Reduced foetal movements Polyhydramnios Needing prolonged resuscitation/artificial ventilation Poor feeding/choking/aspiration Poor cry Poor movements Alert/not alert Fasciculations ```

Answer 23

``` Increased tendon reflexes Extensor plantar response Sustained ankle clonus Global developmental delay Microcephaly/dysmorphism Prolonged seizures Axial weakness ```

Answer 24

``` Hypoxic ishaemic encephalopathy Hypoglycaemia Down's syndrome Prader-Willi syndrome Cerebral malformations ```

Answer 25

``` Hypo-areflexia Selective motor delay Normal head circumference and growth Preserved social interaction Weakness of limbs Low pitched weak cry Tongue fasciculations ```

Answer 26

``` Congenital myopathy Myotonic dystrophy Myasthenia gravis Motor sensory neuropathy Spinal muscular atrophy ```

Answer 27

``` Cerebellar function Gowers test Test sensation Cognitive functioning Cranial nerves ```

Answer 28

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

Answer 29

``` Mobility aids Splints Oxygen Suction Feed pump Medication Drips Orthotic boots Wheelchair Glasses ```

Answer 30

``` Posture - flexed or extended Central/truncal tone Head position/support Type of wheelchair Attachments Communication aids ```

Answer 31

``` 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 ```

Answer 32

Primary abnormality of movement and posture secondary to non progressive lesion of developing brain

Answer 33

Ataxic - cerebellum Spastic - pyramidal system Dyskinetic - separated into athetoid and dystonic - basal ganglia

Answer 34

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)

Answer 35

``` Motor - gross and fine Vision and hearing Speech Epilepsy Feeding Gastro oesophageal reflux Constipation/incontinence Secondary disability - hips, spine, arthritis Learning disabilities ```

Answer 36

Treat spasticity - physio, systemic local and intrathecal drugs, orthopaedic surgery, nerve and spinal root surgery Treat associated problems Enhance quality of life

Answer 37

Transient occurrence of signs/symptoms, result of a primary change to electrical activity, abnormally excessive or synchronous, in the brain

Answer 38

Recurrent unprovoked seizures due to abnormal hyper-synchronous discharge of cortical neurons

Answer 39

``` Dystopia Movement disorder Febrile seizure Non epileptic event Vaso vagal syncope Reflex anoxic seizures Cardiac arrhythmia Sleep associated disorders ```

Answer 40

``` Myoclonic Atonic Tonic Tonic clonic Infantile spasms ```

Answer 41

``` Genetic Structural Metabolic Immune Infectious Unknown ```

Answer 42

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

Answer 43

``` 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 ```

Answer 44

``` Family history of early adult death Palpitations Headache Vomiting Paraesthesia Regression of skills ```

Answer 45

``` 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 ```

Answer 46

Uncommon before 5 years old

Answer 47

3/sec spike and wave

Answer 48

Day dreaming | Behavioural

Answer 49

Sodium valproate | Ethosuximide

Answer 50

Rapid remission with treatment in 80% children

Answer 51

Severe epilepsy syndrome composed of triad of infantile spasms, an interictal electroencephalogram (EEG) pattern of hypsarrhythmia (chaotic background), and mental retardation

Answer 52

3 to 12 months

Answer 53

Sudden generalised symmetrical contractions of muscles of limb (extensor), trunk (flexor) and neck occurring in clusters lasting 0.2-2 secs

Answer 54

``` Hypoxic ishaemic encephalopathy Infections Acquired brain injury Cortical malformations Tuberous sclerosis ```

Answer 55

Steroid Vigabatrin Multiple anti epileptic medications Ketogenic diet

Answer 56

Poor with developmental delay/neurological impairment | Spasms resolve with treatment but other seizure types develop

Answer 57

Absence seizures with age of onset of 9-13 years 3-4Hz spike wave on EEG Generalised tonic clonic seizures may occur

Answer 58

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

Answer 59

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

Answer 60

``` Witnessed history Video Diary of episodes Investigations of other causes Bloods: baseline, genetics, metabolic ECG EEG MRI ```

Answer 61

Anti epileptic medication Ketogenic diet Vagal nerve stimulator Epilepsy surgery

Answer 62

Originates in networks limited to one hemisphere

Answer 63

Originates in and rapidly engages bilaterally distributed networks

Answer 64

Sudden onset involving generalised stiffening and subsequent rhythmic jerking of the limbs as a result of widespread engagement of bilateral cortical and subcritical networks

Answer 65

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

Answer 66

Abrupt generalised muscle stiffening possibly causing a fall Usually lasts 2-10 secs

Answer 67

Generalised seizure characterised by sudden onset of loss of muscle tone

Answer 68

Seizure precipitated by fever without evidence of cns infection or another defined cause Onset 6 months to 6 years Usually brief, generalised, tonic clonic

Answer 69

Higher risk of epilepsy

Answer 70

Meningitis | Meningo encephalitis

Answer 71

Treat the focus Reassurance Parent education Control temp with anti pyretics and cooling measures

Answer 72

Any seizure lasting duration of 30 mins or repeated seizures lasting 30 mins or longer from which the patient doesn't regain consciousness

Answer 73

Children and those over 60

Answer 74

Underlying disorder Systemic complications of the convulsions - hypoxia from airway obstruction and acidosis when hypotension occurs Direct injury from repetitive neuronal discharge

Answer 75

``` Hippocampus Amygdala Cerebellum Thalamus Cerebral cortex ```

Answer 76

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

Answer 77

``` Airway obstruction Hypoxia Aspiration Respiratory depression secondary to excess benzodiazepines Cardiac arrhythmias Pulmonary oedema Hyperthermia Hypertension Disseminated intravascular coagulation ```

Answer 78

``` Seizure type Epilepsy syndrome Co medication and co morbidity Lifestyle Child and family preferences Monotherapy wherever possible Formulations ```

Answer 79

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

Answer 80

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

Answer 81

``` GI side effects Allergic rash Severe immune reactions - SJS Weight gain /loss Behavioural Liver/renal toxicity Tremors ```

Answer 82

Cytomegalovirus

Answer 83

``` Microcephaly Seizures Neonatal jaundice Hepatosplenomegaly Deafness Mental retardation ```

Answer 84

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

Answer 85

Between 6 months to 1 year

Answer 86

Tetralogy of fallot

Answer 87

Squatting increases peripheral vascular resistance so decreases magnitude of right to left shunt across VSD and therefore increase pulmonary blood flow

Answer 88

VSD Overriding aorta Right ventricular outflow obstuction - pulmonary stenosis Right ventricular hypertrophy

Answer 89

Failure of rotation by pulmonary artery during its development Causes infundibular obstruction - entrance to pulmonary artery just before valve

Answer 90

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

Answer 91

Murmur becomes quieter or disappear as there is reduced flow across the stenosed valve as more blood is shunted through the VSD

Answer 92

Tetralogy of fallot | Product of small pulmonary tree and enlarged right ventricle

Answer 93

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

Answer 94

Heart failure Pulmonary plethora Mid diastolic murmur due to increased flow across the mitral valve Loud second heart sound

Answer 95

Maternal drugs: carbimazole, iodine containing compounds Pendreds syndrome: bilateral hearing loss and goitre Maternal Graves: passage of TSH antibodies across placenta

Answer 96

Pyloric stenosis

Answer 97

Males - 4/5x more common

Answer 98

Projectile vomiting starting in 2/3rd week of life

Answer 99

Hypertrophy and hyperplasia of antrum of stomach

Answer 100

Hyponatraemia Hypokalaemia Hypochloraemia Metabolic acidosis

Answer 101

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

Answer 102

Congential aganglionic section of colon that starts at anus and progresses upwards Children present with bowel obstruction in first few weeks of life

Answer 103

Functional colonic obstruction in a newborn due to an obstructing meconium plug

Answer 104

Reduced number of nerves in the intestinal wall | May present like Hirschsprung's disease

Answer 105

Problem with the motor neurons that lead to the intestine, inhibiting this process and thus preventing digestion May present like Hirschsprung's disease

Answer 106

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

Answer 107

Intussusception

Answer 108

Telescoping of one portion of the bowel into an immediately adjacent segment

Answer 109

Intussusception

Answer 110

Crohn's disease

Answer 111

``` Macrocephaly Frontal bossing Depressed nasal bridge Rhizomelic dwarfism Unusually prominent abdomen and buttocks Short hands with trident fingers during extension ```

Answer 112

Glycogen storage disease that results in mild lactic acidosis with exercise

Answer 113

Hirschprungs disease

Answer 114

Nephroblastoma | Usually in first 4 years of life

Answer 115

Necrotising enterocolitis and whilst this is often initially managed medically a laparotomy is required if the situation deteriorates

Answer 116

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

Answer 117

Ramstedt pyloromyotomy (open or laparoscopic)

Answer 118

Loss of developmental skills at any age No vocalising by 3 months No babbling by 10 months Not responding to name by 12 months

Answer 119

Autosomal dominant

Answer 120

Blue breath holding episodes

Answer 121

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

Answer 122

Vomiting Weight loss Dehydration Girls with virilised genitalia

Answer 123

Constipation Generalised abdominal distension Rectum empty Palpable faecal mass

Answer 124

Rectal bleeding Intussusception Like appendicitis

Answer 125

``` Wilms tumour Abdominal pain Vomiting Palpable abdominal lump Hypertensive ```

Answer 126

Asymptomatic mass | Pallor and hypotension if it bleeds

Answer 127

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

Answer 128

Side effects affecting bones and teeth discoloration and growth

Answer 129

``` Failure to thrive Vomiting Dehydration Shocked Low sodium ```

Answer 130

``` 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 ```

Answer 131

Tricyclic antidepressants

Answer 132

Humidified oxygen | If more severe - dexamethasone

Answer 133

Supportive: humidified oxygen and supporting feeding

Answer 134

``` Painful muscle cramps Myoglobinuria After intense exercise Autosomal recessive condition Myophosphorylase deficiency which leads to inability to utilise glucose ```

Answer 135

Softening of cartilage of patella Common in teenage girls Anterior knee pain on walking up and down stairs and rising from prolonged sitting

Answer 136

Tibial apophysitis Seen in sporty teenagers Pain, tenderness and swelling over tibial tubercle

Answer 137

Breastfeeding jaundice

Answer 138

Biliary atresia

Answer 139

Pain after exercise | Intermittent swelling and locking

Answer 140

Rhesus incompatibility

Answer 141

Minimal change glomerulonephritis

Answer 142

Kwashiorkor - protein energy malnutrition

Answer 143

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

Answer 144

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

Answer 145

Reduced activity of glucuronyltransferase which conjugates bilirubin so causes unconjugated hyperbilirubinaemia

Answer 146

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

Answer 147

``` Prolonged neonatal jaundice Kernicterus Haemolytic crises Diabetic ketoacidosis Acute kidney failure ```

Answer 148

Haemolytic response to consumption of fava beans associated with G6PD deficiency

Answer 149

Fava beans Drugs: antimalarials, sulfonamides, nitrofurantoin Stress: bacterial or viral infection

Answer 150

``` Tall Reduced muscle control and coordination Reduced secondary sexual characteristics Gynaecomastia Infertility Microorchidism ```

Answer 151

``` Behavioural problems Intellectual disability/learning difficulties Developmental delay Low muscle tone Long narrow face, prominent ears High palate Large testicles Mitral valve prolapse ```

Answer 152

``` Anosmia Cleft palate Renal agenesis/aplasia Failure to start or complete puberty Lack of testicle development Primary amenorrhoea Infertility ```

Answer 153

Failure of hypothalamus to release GnRH at appropriate time due to GnRH releasing neurons not migrating into correct location during embryonic development Hypogonadotropic hypogonadism

Answer 154

Cerebellar ataxia

Answer 155

``` 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 ```

Answer 156

Urgent referral to paediatrician

Answer 157

Absence of ganglion cells in neural plexus of intestinal wall More common in boys than girls Delayed passage of meconium and abdo distension

Answer 158

Premature infants

Answer 159

Mesenteric ischaemia causes bacterial invasion of mucosa leading to sepsis

Answer 160

Terminal ileum Caecum Distal colon

Answer 161

Distended tense abdomen Passage of blood and mucus per rectum Signs of sepsis

Answer 162

Cystic fibrosis

Answer 163

Deficient intestinal secretions leading to abnormal bulky and viscid meconium

Answer 164

Test feed or USS

Answer 165

Ramstedt pyloromyotomy

Answer 166

4-6 weeks of life | Projectile non bile stained vomiting

Answer 167

``` Child age 6-9 months Colicky pain Diarrhoea and vomiting Sausage shaped mass Red jelly stool ```

Answer 168

Reduction with air insufflation

Answer 169

Tracheo-oesophageal fistula | Polyhydramnios

Answer 170

Jaundice >14 days after birth | Increased conjugated bilirubin

Answer 171

Urgent Kasai procedure - small intestine pulled up and used to create bile duct Roux en Y connection of duodenum to newly inserted small bowel

Answer 172

``` Idiopathic Dehydration Low fibre diet Medications: opiates Anal fissure Over enthusiastic potty training Hypothyroidism Hirschsprung's disease Hypercalcaemia Learning disabilities ```

Answer 173

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

Answer 174

Symptoms of severe constipation Overflow soiling Faecal mass palpable in abdomen

Answer 175

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

Answer 176

``` 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 ```

Answer 177

5 or over: add LABA | Under 5: add leukotriene antagonist

Answer 178

``` Low birth weight Prematurity Traumatic delivery Foetal hypoxia Maternal peripartum infection ```

Answer 179

Group B strep E. coli Listeria monocytogenes

Answer 180

3 months of age | Should be seen before 6 months

Answer 181

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

Answer 182

Intranasally at 2-3 years | Annually after that

Answer 183

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

Answer 184

BCG/hep B if risk factors

Answer 185

DTaP/IPV/Hib PCV Oral rotavirus Men B

Answer 186

DTaP/IPV/Hib | Oral rotavirus

Answer 187

DTaP/IPV/Hib PCV Men B

Answer 188

Hib/Men C MMR PCV Men B

Answer 189

annual flu vaccine

Answer 190

MMR and DTaP/IPV

Answer 191

HPV vaccine for girls

Answer 192

DT/IPV | Men ACWY

Answer 193

Oral dexamethasone 0.15mg/kg single dose High flow oxygen Nebulised adrenaline

Answer 194

``` Microcephaly Small eyes Cleft lip/palate Polydactyly Scalp lesions ```

Answer 195

Trisomy 13

Answer 196

Micrognathia Low set ears Rocker bottom feet Overlapping of fingers

Answer 197

Trisomy 18

Answer 198

``` Learning difficulties Macrocephaly Long face Large ears Macro orchidism ```

Answer 199

Webbed neck Pectus excavatum Short stature Pulmonary stenosis

Answer 200

Micrognathia Posterior displacement of tongue Cleft palate

Answer 201

Hypotonia Hypogonadism Obesity

Answer 202

``` Short stature Learning difficulties Friendly extrovert personality Transient neonatal hypercalcaemia Supravalvular aortic stenosis ```

Answer 203

Pediculosis capitis

Answer 204

Fine toothed combing of wet or dry hair

Answer 205

Treatment only indicated if living lice found | Choice should be offered: malathion, wet combing, dimeticone, isopropyl myrisate and cyclomethicone

Answer 206

Under 5 years, median age 3

Answer 207

``` Abdo mass Painless haematuria Flank pain Anorexia Fever Mets: usually lung ```

Answer 208

Nephrectomy Chemotherapy Radiotherapy if advanced

Answer 209

Club foot | Inverted and plantar flexed foot

Answer 210

``` Spina bifida Cerebral palsy Edwards syndrome Oligohydramnios Arthrogryposis ```

Answer 211

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

Answer 212

Ketogenic diet: high fat, low carb, controlled protein

Answer 213

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

Answer 214

Hypsarrhythmia - high amplitude irregular waves and spikes in background of chaotic and disorganised activity

Answer 215

``` Ash leaf spots Adenoma sebaceum Shagreen patches Subungual fibromata Epilepsy Developmental problems Retinal hamartomas ```

Answer 216

``` Female Breech presentation Positive family history Firstborn Oligohydramnios Birth weight >5kg Congenital calcaneovalgus foot deformity ```

Answer 217

Most will spontaneously stabilise by 3-6 weeks Pavlik harness in children younger than 4-5 months Older children may require surgery

Answer 218

Automated otoacoustic emissions test

Answer 219

Haemolytic anaemia Renal failure Thrombocytopenia

Answer 220

E. coli 0157

Answer 221

Inform medical team and police - child protection and safeguarding issues

Answer 222

Swelling on newborns head Develops hours after delivery Due to bleeding between periosteum and skull Most commonly in parietal region

Answer 223

Serosanguinous subcutaneous extraperiosteal fluid collection with poorly defined margins caused by pressure of presenting part of scalp against dilating cervix

Answer 224

Temporary swelling left on infants head after a ventouse suction cup has been used for delivery

Answer 225

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

Answer 226

Refer immediately to paediatrician

Answer 227

Mild to moderate: baby shampoo and baby oils | Severe: mild topical steroids e.g. 1% hydrocortisone

Answer 228

Polydactyly Obesity Retinitis pigmentosa

Answer 229

Rhesus haemolytic disease ABO haemolytic disease Hereditary spherocytosis G6PD deficiency

Answer 230

``` 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 ```

Answer 231

``` Biliary atresia Hypothyroidism Galactosaemia Urinary tract infection Breast milk jaundice Congenital infection: CMV, toxoplasmosis ```

Answer 232

Steroids | Nebulised adrenaline

Answer 233

``` Small vessel vasculitis Prolonged fever Children under 5 Mucocutaneous lymph node syndrome Rash, lymphadenopathy, red eyes, dry cracked lips, red fingers and toes ```

Answer 234

Salbutamol inhaler | If this isn't helping, add oral montelukast or inhaled corticosteroid

Answer 235

``` High grade fever >5 days Conjunctival injection Bright red, cracked lips Strawberry tongue Cervical lymphadenopathy Red palms and soles which later peel ```

Answer 236

High dose aspirin IV immunoglobulin Echo to screen for coronary artery aneurysms

Answer 237

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

Answer 238

``` Neonatal jaundice Intravascular haemolysis Gallstones Splenomegaly Heinz bodies on blood film ```

Answer 239

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

Answer 240

Oral macrolide: clarithromycin, azithromycin, erythromycin if onset of cough is within previous 21 days

Answer 241

Subconjunctival haemorrhage Pneumonia Bronchiectasis Seizures

Answer 242

Neonatal: polyhydramnios, polyuria, polydipsia, hypocalcaemia, nephrocalcinosis Classic: polyuria, polydipsia, vomiting, growth retardation, hypokalaemia, alkalosis, low BP

Answer 243

2-5 months

Answer 244

Failure to start puberty and anosmia Hypogonadotrophic hypogonadism GnRH releasing neurones prevented from reaching hypothalamus or cribriform plate

Answer 245

``` Pulmonary stenosis ASD Hypertrophic cardiomyopathy Short stature Learning difficulty Pectus excavatum Webbed neck Flat nose bridge ```

Answer 246

One that has failed to reach the bottom of the scrotum by 3 months of age

Answer 247

``` Patent processus vaginalis Abnormal epididymis Cerebral palsy Mental retardation Wilms tumour Abdominal wall defect ```

Answer 248

Reduce risk of infertility Allows testis to be examined for cancer Avoid torsion Cosmetic appearance

Answer 249

Orchidopexy at 6-18 months of age | After age of 2 if untreated, Sertoli cells degrade and so may be better to do orchidectomy

Answer 250

Trisomy 13: patau syndrome

Answer 251

Trisomy 18: Edwards syndrome

Answer 252

``` Congenital hypothyroidism Cystic fibrosis Sickle cell Phenylketonuria Medium chain acyl coA dehydrogenase deficiency Maple syrup urine disease Isovolaemic acidaemia Glutaric aciduria type 1 Homocystinuria ```

Answer 253

Between 5th and 9th day of life

Answer 254

Nephroblastoma seen in children | Usually presents as painless palpable abdominal mass, anorexia, haematuria, HTN

Answer 255

Beckwith wiedemann syndrome WAGR syndrome Hemihypertrophy Loss of function in WT1 gene

Answer 256

Multi organ disease | Granulomata on skin

Answer 257

Hydrocephaly Seizures Chorioretinitis

Answer 258

Wear gloves when gardening or handling cat litter | Cook meat thoroughly

Answer 259

Jaundice Hepatosplenomegaly Microcephaly

Answer 260

``` Cerebral cortical and cerebellar hypoplasia Microcephaly Convulsions Limb hypoplasia Rudimentary digits ```

Answer 261

``` Cataracts Cardiac abnormalities Thrombocytopenia Cerebral calcification Deafness ```

Answer 262

Erythromycin

Answer 263

Protrusion of upper teeth | Feature of sickle cell

Answer 264

Testicular enlargement Secondary sexual characteristics: public hair, axillary hair, body odour, deepening of voice Growth spurt 18 months after onset

Answer 265

Breast development Axillary and pubic hair Growth spurt Menarche

Answer 266

Afro American children Obese Due to raised oestrogen levels

Answer 267

Congenital bilateral sensorineural hearing loss and goitre with euthyroid or mild hypothyroidism

Answer 268

``` 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 ```

Answer 269

Laryngomalacia

Answer 270

Floppy epiglottis which folds into airway on inspiration

Answer 271

``` Tetralogy of Fallot Transposition of great vessels Hypoplastic left heart Tricuspid atresia Eisenmengers complex ```

Answer 272

Reversal of a shunt due to pulmonary hypertension

Answer 273

``` Maternal diabetes mellitus Prematurity IUGR Hypothermia Neonatal sepsis Inborn errors of metabolism Nesidioblastosis Beckwith Wiedemann syndrome ```

Answer 274

``` Thymic aplasia with agammaglobulinaemia Congenital heart problems Frequent infections Developmental delay 22q11.2 deletion syndrome ```

Answer 275

X linked immunodeficiency disorder characterised by lymphopenia and recurrent bacterial infections

Answer 276

Enlarged peyers patches Meckels diverticula Tumour Haematoma complicating HSP

Answer 277

3 months to 3 years

Answer 278

Intranasal First dose at 2-3 years then annually after that Live vaccine

Answer 279

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

Answer 280

Blocked nose/rhinorrhoea Headache Anorexia

Answer 281

``` Hyperuricaemia Gout Choreoathetosis Spasticity Mental deficiency Behavioural disturbance (particularly self mutilation) ```

Answer 282

75% male | Usually develops before age 3

Answer 283

Global impairment of language and communication Impairment of social relationships Ritualistic and compulsive behaviour

Answer 284

Fragile x syndrome | Retts syndrome

Answer 285

IV cefotaxime

Answer 286

``` Short stature Short palpebral fissures Flat philtrum Thin upper lip vermillion Neurocognitive problems: hyperactivity, delinquent behaviour ```

Answer 287

Ghent criteria | Two cardinal features: dilated aortic root and displacement of optic lens

Answer 288

Feed 8-10 times a day for 5-10 mins at a time | 5-6 wet nappies with 3-4 stools

Answer 289

Failure of development of olfactory bulb | Failure of production of gonadotrophin releasing hormone

Answer 290

Indomethacin

Answer 291

T cells due to failure of thymic development

Answer 292

Obesity due to compulsive food consumption | Mental retardation

Answer 293

``` 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 ```

Answer 294

High dose aspirin IV immunoglobulin Echo to screen for coronary artery aneurysm

Answer 295

Consider from 3 months Ideally should see surgeon before 6 months Usually procedure at around 1 year

Answer 296

Oxygen and nebulised adrenaline

Answer 297

Nasal secretions - culture

Answer 298

6 months to 5 years

Answer 299

``` 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 ```

Answer 300

Growth hormone Anabolic steroids Oestrogen/progesterone

Answer 301

``` Vomiting Diarrhoea Jaundice Lethargy Hypotonia Failure to thrive Cataracts Mental retardation Seizures Complement deficiency ```

Answer 302

Rare metabolic genetic disorder that affects ability to metabolise galactose properly Autosomal recessive deficiency in enzyme needed to break down galactose

Answer 303

Haemorrhage of blood between skull and periosteum due to rupture of vessels crossing periosteum

Answer 304

Prolonged second stage labour | Instrumental delivery, particularly ventouse

Answer 305

``` Jaundice Anaemia Hypotension Infection Unnatural bulges ```

Answer 306

Several hours after delivery

Answer 307

``` SHITE Sickle cell anemia Hereditary spherocytosis Iron deficiency anemia Thalassemia Enzyme deficiency (G6PD) ```

Answer 308

Multiple transfusions

Answer 309

3 months because haemoglobin f is still predominant in neonates

Answer 310

Psychological UTI Diabetes

Answer 311

``` Midline defects Hypotelorism Cleft palate Microphthalmia Cyclopia ```

Answer 312

COX inhibitor infusion to block synthesis of prostaglandins which keep it open

Answer 313

``` Maternal diabetes Rhesus incompatibility Duodenal atresia GI obstruction Tracheo-oesophageal fistula Anencephaly ```

Answer 314

Autosomal dominant

Answer 315

Splenectomy | Done after 5 years when chances of life threatening encapsulated bacterial infection reduce

Answer 316

Under 6 months | Particularly second month

Answer 317

``` 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 ```

Answer 318

Diuretics Salt restriction Steroids

Answer 319

Venous thrombosis Sepsis Acute renal failure Renal vein thrombosis

Answer 320

21 alpha hydroxylase deficiency results in increased production of androgens which drive bone proliferation, particularly at puberty

Answer 321

Birth weight below the 10th centile for gestational age and sex

Answer 322

Decrease in foetal growth rate that prevents infant obtaining genetic growth potential

Answer 323

``` Perinatal asphyxia Meconium aspiration Electrolyte imbalance Metabolic acidosis Polycythemia Hypoglycaemia ```

Answer 324

Genetic disorder where glucocerebroside accumulates in cells and organs Characterised by bruising, fatigue, anaemia, hepatosplenomegaly, skeletal disorder, neuropathy, pingueculae in cornea

Answer 325

Ophthalmia neonatorum

Answer 326

``` Palpable purpuric rash over buttocks and extensor surfaces of arms and legs Abdominal pain Polyarthritis Haematuria Renal failure ```

Answer 327

Oral dexamethasone 0.15mg/kg single dose and review

Answer 328

Systemic dexamethasone and nebulised adrenaline 5ml of 1:1000 Oxygen

Answer 329

Five days from commencing antibiotics

Answer 330

No exclusion

Answer 331

Until symptoms have settled for 48 hours

Answer 332

Broad spectrum antibiotics

Answer 333

Topical fusidic acid

Answer 334

``` Grey membrane on tonsils Recent visitors to Eastern Europe/Russia/Asia Sore throat Bulky cervical lymphadenopathy Neuritis Heart block ```

Answer 335

Microcephaly Hydrocephalus Cerebral calcification Choroidoretinitis

Answer 336

Neonatal jaundice Deafness Microcephaly

Answer 337

Rare X-linked recessive disease characterized by eczema, thrombocytopenia, immune deficiency, and bloody diarrhoea secondary to the thrombocytopenia

Answer 338

12-15 months | 3-4 years

Answer 339

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

Answer 340

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

Answer 341

Depigmented ash leaf spots Roughened patches of skin over lumbar spine (shagreen patches) Adenoma sebaceum (angiofibromas over nose) Subungual fibromata Cafe au lait spots

Answer 342

Nasolacrimal duct obstruction

Answer 343

Severe inflammatory progressive encephalitic illness of children often accompanied by fatty infiltration

Answer 344

Inherited disorder resulting in rhabdomyolysis with minor illness or mild/moderate exertion Inherited defect in gene for muscle phosphorylase enzyme

Answer 345

Rhesus haemolytic disease ABO haemolytic disease Hereditary spherocytosis G6PD deficiency

Answer 346

``` Biliary atresia Hypothyroidism Galactosaemia UTI Breast milk jaundice Congenital infection e.g. CMV, toxoplasmosis ```

Answer 347

2 months and 3 months

Answer 348

Theoretical risk of intussusception

Answer 349

Premature Born at high altitude Maternal rubella infection in first trimester

Answer 350

``` Left subclavicular thrill Continuous machinery murmur Large volume, bounding, collapsing pulse Wide pulse pressure Heaving apex beat ```

Answer 351

Indomethacin

Answer 352

Intestinal malrotation and volvulus

Answer 353

Hep b vaccine and 0.5ml HBIG within 12 hours of birth Hepatitis vaccine at 1-2 months Vaccine at 6 months

Answer 354

Death within 28 days of delivery of a live born foetus regardless of gestation Early: 0-6 days Late: 7-27 days

Answer 355

Posterior: 8 weeks Anterior: 12 and 18 months

Answer 356

Prolonged fever more than 5 days Typical mucocutaneous changes: desquamation of fingers and toes including palms and soles Lymphadenopathy

Answer 357

Newborn and infant physical examination | Within 72 hours of birth unless sick baby then within 24 hours

Answer 358

Congenital disorder Posterior nasal airway occluded by soft tissue or bone Episodes of cyanosis worst during feeding made better by crying

Answer 359

``` Cyanosis Tachypnoea Nasal flaring Grunting Intercostal/subcostal recession Apnoea ```

Answer 360

Aneurysm of aortic arch

Answer 361

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

Answer 362

Compulsive eating Childhood obesity Developmental delay Chromosome 15 abnormalities

Answer 363

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

Answer 364

Immature hepatic metabolism Low placental transfer No oral intake of vitamin k or precursors Lack of gastrointestinal bacterial colonisation

Answer 365

Reduce vitamin k deficiency bleeding - haemorrhagic disease of newborn

Answer 366

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

Answer 367

``` Tall High arched palate Long ears Long face Macro orchidism Learning difficulties ```

Answer 368

IV cefotaxime

Answer 369

Intracranial haemorrhage

Answer 370

Platelet transfusion Intravenous methylprednisolone IV immune globulin Splenectomy

Answer 371

Worsening alkalosis means increasing bicarbonate levels combine with calcium and so reduce serum ionised calcium

Answer 372

``` Meningoencephalitis Arthritis Transverse myelitis Cerebellar ataxia Deafness ```

Answer 373

Crosslinking of IgE

Answer 374

Autosomal dominant condition associated with deficiency of C1 esterase inhibitor

Answer 375

Occurs in CF patients | Accumulation of viscous mucus and faecal material in terminal ileum, caecum and ascending colon

Answer 376

``` Regurgitation and vomiting Diarrhoea Urticaria Atopic eczema Colic: irritability, crying Wheeze Chronic cough Rarely angioedema and anaphylaxis ```

Answer 377

Improvement with cows milk protein elimination Skin prick/patch testing Total IgE and specific IgE for cows milk protein (RAST)

Answer 378

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

Answer 379

Perianal itching particularly at night | Girls may have vulval symptoms

Answer 380

``` Female sex Breech presentation Positive family history Firstborn Oligohydramnios Birth weight >5kg Congenital calcaneovalgus foot deformity ```

Answer 381

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

Answer 382

``` Age <2 or >10 WBC >20 at diagnosis T or B cell surface markers Non Caucasian Male sex ```

Answer 383

Otoacoustic emission test Computer generated click played through small ear piece Presence of soft echo indicates healthy cochlea

Answer 384

Auditory brainstem response test

Answer 385

Deletion of chromosome 15 paternal gene not received

Answer 386

Testicular growth at around 12 years | Volume >4ml indicates puberty

Answer 387

Breast development at around 11.5 years

Answer 388

``` Failure to thrive Polyuria Polydipsia Hypokalaemia Normotension Weakness ```

Answer 389

Hamartomatous polyps in GI tract Pigmented lesions on lips, oral mucosa, face, palms and soles Intestinal obstruction: intussusception GI bleeding

Answer 390

Idiopathic Drugs: NSAIDs, rifampicin Hodgkin's lymphoma Infectious mononucleosis

Answer 391

T cell and cytokine mediated damage to glomerular basement membrane - polyanion loss Reduction of electrostatic charge - increased glomerular permeability to serum albumin

Answer 392

Nephrotic syndrome Normotension Highly selective proteinuria Electron microscopy shows fusion of podocytes

Answer 393

Echo to look for coronary artery aneurysm

Answer 394

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

Answer 395

High dose aspirin IV immunoglobulin Echo

Answer 396

Corneal light reflection test

Answer 397

Concomitant: imbalance in extraocular muscles, convergent more common Paralytic: paralysis of extraocular muscles

Answer 398

Cover test

Answer 399

Eye patches to prevent amblyopia | Referral to secondary care

Answer 400

Sudden infant death syndrome

Answer 401

``` Prematurity Parental smoking Hyperthermia Putting baby to sleep prone Male Multiple births Bottle feeding Social class IV and V Maternal drug use Winter ```

Answer 402

``` Prematurity Male Diabetic mother C section Second born of premature twins ```

Answer 403

Maternal steroids to induce lung maturation Oxygen Assisted ventilation Exogenous surfactant given via endotracheal tube

Answer 404

Esotropia: towards nose Exotropia: temporally Hypertropia: superior Hypotropia: inferior

Answer 405

``` Learning difficulties Large low set ears, long thin face, high arched palate Macroorchidism Hypotonia Autism Mitral valve prolapse ```

Answer 406

Monoclonal antibody used to prevent respiratory syncytial virus in children at increased risk of severe disease: premature, lung or heart abnormality, immunocompromise

Answer 407

Development of secondary sexual characteristics before 8 years in females and 9 years in males

Answer 408

Palpable purpuric rash over buttocks and extensor surfaces of arms and legs Abdominal pain Polyarthritis Features of IgA nephropathy: haematuria, renal failure

Answer 409

Analgesia for arthralgia | Supportive treatment for nephropathy

Answer 410

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

Answer 411

Rapid onset High temperature Stridor Drooling of saliva

Answer 412

100ml/kg/day for 0-10kg 50ml/kg/day for 11-20kg 20ml/kg/day beyond that

Answer 413

Acute renal failure Microangiopathic haemolytic anaemia Thrombocytopenia

Answer 414

``` Post dysentery - E. coli Tumours Pregnancy Ciclosporin COCP SLE HIV ```

Answer 415

Fluids Blood transfusion Dialysis

Answer 416

Stools containing undigested food Chronic non specific diarrhoea Should remit as child grows up More common in boys age 1-5

Answer 417

``` Maternal diabetes mellitus Prematurity IUGR Hypothermia Neonatal sepsis Inborn errors of metabolism Beckwith wiedemann syndrome ```

Answer 418

``` Overgrowth disorder present at birth Increased risk of childhood cancer Macroglossia Macrosomia Microcephaly Neonatal hypoglycaemia Hepatoblastoma ```

Answer 419

``` 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 ```

Answer 420

Secondary to lack of T cell function: pneumocystis jirovecii, systemic candidiasis, generalised herpetic infections

Answer 421

Preterm delivery | Neurological disorders

Answer 422

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

Answer 423

``` Distress Failure to thrive Aspiration Frequent otitis media Dental erosion ```

Answer 424

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

Answer 425

3-4 months

Answer 426

White/grey spots on periphery of iris due to aggregation of connective tissue Seen in Down's syndrome

Answer 427

``` 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%) ```

Answer 428

``` 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 ```

Answer 429

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.

Answer 430

``` 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 ```

Answer 431

``` Heart defects Coeliac disease Imperforate anus Hirschsprung's disease Cataracts Nystagmus Hearing loss Hypothyroidism Alzheimer's Acute leukaemia: mainly ALL Epilepsy ```

Answer 432

Clonal proliferation of langerhans cells capable of migrating from skin to lymph nodes, bones, pituitary and thyroid

Answer 433

Cardiomegaly | Increased pulmonary vascular markings

Answer 434

Concavity of left heart border - boot shape | Decreased pulmonary vascular markings

Answer 435

``` 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 ```

Answer 436

High dose aspirin IV immunoglobulin Echo to screen for coronary aneurysms

Answer 437

``` 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 ```

Answer 438

Automated otoacoustic emissions

Answer 439

Automated auditory brain stem response

Answer 440

Congenital diaphragmatic hernia | Usually right sided containing transverse colon

Answer 441

Congenital diaphragmatic hernia | Tend to be left sided containing stomach

Answer 442

``` 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 ```

Answer 443

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

Answer 444

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

Answer 445

Autism Spectrum disorder Rett’s Disorder Childhood Integrative Disorder PDD, not otherwise specified

Answer 446

Congenital Rubella PKU Tuberous Sclerosis Fragile X Syndrome

Answer 447

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

Answer 448

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

Answer 449

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

Answer 450

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

Answer 451

Sudden, rapid, recurrent, nonrhythmic, stereotyped motor movements or vocalizations

Answer 452

Tourette’s Syndrome Chronic Motor Tic DisorderChronic Vocal Tic Disorder Transient Tic Disorder Tic Disorder NOS

Answer 453

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

Answer 454

Parent training (PCIT) Individual psychotherapy Family Therapy

Answer 455

Aggression to people and animals Destruction of property Deceitfulness or theft Serious violation of rules

Answer 456

``` Multimodality treatment programs Environmental structuring Family Therapy Group Therapy Ind. Therapy – problem solving skills Medications as adjuncts ```

Answer 457

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

Answer 458

``` Irritability Sleep cycle disturbance Oppositional behavior Social isolation Crying spells ```

Answer 459

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

Answer 460

``` 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 ```

Answer 461

GP School or college – for example, a teacher, pastoral lead, school nurse or special educational needs co-ordinator (SENCO) Health visitors General hospitals

Answer 462

``` 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 ```

Answer 463

``` 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 ```

Answer 464

``` Focal neurological signs Focal seizures Decreased level of consciousness Recent herpes infection in household / nursery / school Immunosuppression ```

Answer 465

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

Answer 466

``` children >3 months if fever plus: Persistent vomiting Lethargy Irritability Abdominal pain or tenderness Urinary frequency or dysuria Offensive urine or haematuria ```

Answer 467

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

Answer 468

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

Answer 469

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

Answer 470

Penicillin or azithromycin if penicillin-allergic for 10 days Notifiable

Answer 471

Poverty and overcrowding Institutional care, such as rest homes, hospitals, prisons Refugee camps Individuals with immune deficiency

Answer 472

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

Answer 473

Hypersensitivity reaction

Answer 474

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

Answer 475

Streptococcus or Staphylococcus spp

Answer 476

Immunosuppression, wounds, toe web intertrigo, minor skin injury, Diabetes, venous insufficiency, lymphoedema

Answer 477

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

Answer 478

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

Answer 479

Admission IV Antibiotics – flucloxacillin, clindamycin Analgesia Skin care

Answer 480

Skin scrapings, hair or nail clippings, skin swabs

Answer 481

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

Answer 482

Ketoconazole or Selenium Sulphide shampoo overnight then wash off, apply twice weekly for 3 weeks Topical imidazole antifungals an alternative

Answer 483

``` Infections Allergens Sweating Heat Stress ```

Answer 484

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

Answer 485

AVSD VSD Tetralogy of fallot

Answer 486

VSD | Other various defects

Answer 487

Coarctation of aorta | Aortic stenosis

Answer 488

Truncus arteriosus Interrupted aortic arch Tetralogy of fallot

Answer 489

Supravalvar aortic stenosis

Answer 490

Transposition of great arteries VSD HOCM

Answer 491

Heart block

Answer 492

Coarctation of aorta VSD Patent ductus arteriosus

Answer 493

Ebsteins anomaly

Answer 494

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

Answer 495

``` 5 Ts: Tetralogy of fallot Transposition great arteries Tricuspid atresia Total anomalous pulmonary venous drainage Truncus Arteriosus Pulmonary atresia ```

Answer 496

``` Functional heart murmur Commonest age group 3-7yr Vibratory/musical in quality At apex, radiation to carotids May only be audible in supine position ```

Answer 497

``` Change in intensity with posture Always systolic (except venous hum –continuous) ASYMPTOMATIC ```

Answer 498

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)

Answer 499

VSD ASD PDA Severe LV outflow obstruction/ hypoplastic left heart

Answer 500

Severe PS/atresia | TOF

Answer 501

PGE1 infusion

Answer 502

``` Coarctation of aorta Interrupted aortic arch Critical aortic stenosis Hypoplastic left heart syndrome TGA ```

Answer 503

Increase systemic resistance, increase return to right heart so increase pulmonary blood flow

Answer 504

Pulmonary stenosis Large VSD Overriding aorta RVH

Answer 505

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

Answer 506

20mls/kg 0.9% saline | Unless DKA/Trauma/cardiac – 10mls/kg

Answer 507

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

Answer 508

If shocked add 100mls/kg to maintenance requirements. If not shocked add 50mls/kg or Weight x 10 x percentage dehydration

Answer 509

Normal milk Oral rehydration salts Avoid fruit juices. fizzy drinks, plain water

Answer 510

Loss of up to 8% birth weight is acceptable. Any more is too much, are they dehydrated? Failing to thrive

Answer 511

Primary abnormality of movement and posture secondary to a non-progressive lesion of a developing brain

Answer 512

Injury to extrapyramidal system causes- dystonic CP (basal ganglia) or ataxic CP (cerebellum) Injury to Pyramidal system (corticospinal tract) causes Spastic CP-Diplegia, Quadriplegia

Answer 513

``` 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 ```

Answer 514

``` Motor –gross, fine motor Vision and Hearing Speech Epilepsy Feeding Gastro Oesophageal Reflux Constipation/Incontinence Secondary disabilities – hips, spine, arthritis Learning disabilities ```

Answer 515

Treatment of Spasticity- Physiotherapy, Pharmacology- Systemic, Local, Intrathecal, Surgery- Orthopaedic, Peripheral Nerves, Spinal Roots Treatment of Associated Problems Enhancing quality of life

Answer 516

Seizures precipitated by fever without evidence of CNS infection or another defined cause

Answer 517

Prolonged, Focal or associated with Todd’s paresis

Answer 518

Recurrent, Unprovoked Seizures due to abnormal hyper-synchronous discharge of cortical neurons

Answer 519

``` Myoclonic Atonic Tonic Tonic clonic Infantile Spasms ```

Answer 520

Uncommon before age 5

Answer 521

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

Answer 522

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

Answer 523

Hypoxic ischaemic encephalopathy, Infections, Acquired Brain Injury, Cortical malformations, Tuberous Sclerosis

Answer 524

Sodium Valproate, Ethosuximide

Answer 525

Steroid (ACTH), Vigabatrin, Multiple Antiepileptic medications

Answer 526

``` Hypotonia Reduced foetal movements Polyhydramnios Needing prolonged resuscitation/Artificial Ventilation Poor feeding/Choking/Aspiration Poor cry Poor movements Alert/ not alert Fasciculation ```

Answer 527

``` UMN lesion Increased tendon reflexes Extensor plantar response Sustained ankle clonus Global developmental delay Microcephaly +/-Dysmorphism Prolonged Seizures Axial weakness a significant feature ```

Answer 528

Hypoxic ischaemic encephalopathy, Hypoglycaemia, Chronic Encephalopathies, Down’s Syndrome, Prader Willi Syndrome, Cerebral Malformations

Answer 529

``` 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 ```

Answer 530

Neuro-muscular-Congenital Myopathy, Myotonic dystrophy, Myaesthenia, Motor Sensory Neuropathy, Spinal muscular atrophy

Answer 531

``` Bloods: Creatine Kinase, TFT, Congenital Infections (TORCH), Genetics, Metabolic EMG Nerve Conduction Studies ECG MRI Brain Nerve/Muscle Biopsy ```

Answer 532

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

Answer 533

``` 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 ```

Answer 534

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

Answer 535

``` Sats <92% Silent chest Poor respiratory effort Altered consciousness Cyanosis PEFR <33% best/predicted ```

Answer 536

``` PEFR 33-50% best or predicted Sats 92% or above Can't complete sentences Resp rate 25 or more Pulse 110 or more ```

Answer 537

``` 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 ```

Answer 538

``` Tachypnoea Subcostal/intercostal recessions Tracheal tug Head bobbing Nasal flaring Grunting ```

Answer 539

High flow O2 – humidified Hydration – NG feeding or IV fluids May need to consider high-flow or CPAP Worst on day 4

Answer 540

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

Answer 541

Not yet immunised

Answer 542

Blood – culture, GLUCOSE, gas, FBC/CRP CXR Urine LP if stable enough and no purpuric rash

Answer 543

Cerebral oedema from correcting fluids too quickly

Answer 544

Mannitol or hypertonic saline Head up Intubate and ventilate, keep CO2 low normal ITU

Answer 545

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

Answer 546

Cardiothoracic ratio 60% Kink of trachea to the right due to prominent thymus Diaphragm lies at the level of 6th anterior rib Thymus

Answer 547

Thymus shadow - normal

Answer 548

Diffuse symmetric reticulogranular densities Prominent central air bronchograms Generalised hypoventilation

Answer 549

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

Answer 550

Deep lucent ipsilateral costophrenic angle within nondependent portions of pleural space Indicator of pneumothorax on a supine chest X-ray

Answer 551

``` Dilated loops of bowel Intramural gas Portal venous gas Pneumoperitoneum - football sign Riglers sign ```

Answer 552

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

Answer 553

Bronchiectasis Pneumothorax Infection (consolidation) Collapse secondary to mucus plugging

Answer 554

Prominent stomach and first part of duodenum | Duodenal atresia

Answer 555

Cupped and frayed metaphyses

Answer 556

``` 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 ```

Answer 557

``` 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 ```

Answer 558

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

Answer 559

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

Answer 560

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

Answer 561

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

Answer 562

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

Answer 563

``` <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 ```

Answer 564

``` <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 ```

Answer 565

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

Answer 566

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

Answer 567

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

Answer 568

``` FBC U+E LFT TFT ESR, CRP Ig G,A,M FAB (functional antibody test) Anti TTG Urine CXR, T-spot, Mantoux ```

Answer 569

``` 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 ```

Answer 570

``` Villous atrophy Crypt hyperplasia Loss of enterocyte height Lamina propria infiltration Increased intra-epithelial lymphocytes ```

Answer 571

``` Gliadin IgA/IgG Endomysial IgA Tissue Transglutaminase IgA (Anti body test of choice) IgA deficiency: False-negatives HLA typing Duodenal biopsy (Gold standard test) ```

Answer 572

Dermatitis herpetiformis IgA deficiency (3-5%) Autoimmune conditions: Thyroid disease, Type 1 diabetes, Addison’s, Sjogrens syndrome

Answer 573

``` FBC ESR LFTs including Albumin CRP pANCA (CD) GI endoscopy Radiology: Strictures, toxic megacolon ```

Answer 574

Fluid resuscitation Correct electrolyte imbalances Pyloromyotomy

Answer 575

Usually before 2 years

Answer 576

Improved nutrition Vaccination Improved housing / hygiene Antibiotics

Answer 577

``` Fever Vomiting Anorexia Rigors Abdominal pain ```

Answer 578

Other than neonatal period, viruses (eg RSV) are most common cause Streptococcus pneumoniae (~70%) Haemophilus influenzae Atypical bacteria : Mycoplasma, Chlamydophila pneumoniae

Answer 579

First line – Amoxicillin In penicillin allergy or fail to respond – Macrolides Atypical pneumonia - Macrolide

Answer 580

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

Answer 581

Egg on side shaped | Increased pulmonary marking

Answer 582

Supracardiac Cardiac Infracardiac

Answer 583

Snowman sign/figure of 8

Answer 584

``` Heart failure HOCM/DCM Pericardial effusion Significant Lt to Rt shunts Ebstein Anomaly ```

Answer 585

``` Viral Rash Drug Rash Allergic Rash Scabies Erythema multiforme ITP Meningococcal Scarlet fever Urticaria Measles Rubella Chicken pox ```

Answer 586

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

Answer 587

``` 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 ```

Answer 588

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

Answer 589

Whitish greasy coat produced by epithelial cell breakdown in newborns Protect skin from amniotic fluid in utero

Answer 590

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

Answer 591

Hemangioma (Strawberry Naevus)

Answer 592

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

Answer 593

steriods or interferon-alpha

Answer 594

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

Answer 595

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

Answer 596

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

Answer 597

Rash: buttocks, extensor surfaces of legs and arms Joint pain/swelling: knees and ankles Abdominal pain: haematemesis, melaena, intussusception Renal: Haematuria, nephrotic syndrome

Answer 598

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

Answer 599

``` Bacterial superinfection Encephalitis Aseptic meningitis Pneumonitis DIC ```

Answer 600

Impetigo contagiosa and Bullous Impetigo

Answer 601

Strep pyogenes or staph aureus

Answer 602

Advice about avoiding spread Localised lesion- topical antibiotic eg Fucidin, mupircin cream Remove crust with gentle washing Widespread infection - oral flucloxacillin or erythromycin/augmentin

Answer 603

``` Fever Malasie Dry cough Coryza Conjunctivitis Photophobia ```

Answer 604

Contagious 4 days before rash and 4 days after

Answer 605

``` Pneumonia Otitis media Tracheitis Febrile convulsions Encephalitis Subacute sclerosing panencephalitis Diarrhoea Hepatitis Appendicitis Corneal ulceration Myocarditis ```

Answer 606

Human Herpes virus 6

Answer 607

parvovirus B19

Answer 608

DNA pox virus

Answer 609

``` 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 ```

Answer 610

Noonan: Rt sided PS

Answer 611

``` Short stature Macrocephaly Flat midface with prominent forehead Associated hydrocephalus Dental malocclusion Hearing loss Normal intelligence and normal lifespans ```

Answer 612

``` 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 ```

Answer 613

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)

Answer 614

``` Saddle shaped nose Short palpebral fissures Epicanthal folds Indistinct philtrum Thin upper lip Mid facial hypoplasia/microcephaly Maxillary hypoplasia Developmental delay ```

Answer 615

Diurnal variation peak flow greater than 20% for at least 3 days in a week Or improvement 10 mins after bronchodilator

Answer 616

Bronchospasm Mucosal oedema Mucous plugging Which cause obstruction of expiration. Can breathe in but not breathe out which leads to hyperinflation

Answer 617

tachycardia, tremor, hyperglycaemia, hypokalaemia

Answer 618

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

Answer 619

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

Answer 620

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

Answer 621

``` Sweat test (>60mmol/l Cl-suggests CF) Genetic testing ```

Answer 622

Nebulised saline / antibiotics /mucolytics Supplementation: pancreatic enzymes, fat-soluble vitamins, nutritional support Chest physio Aggressive treatment of chest infections with IV antibiotics

Answer 623

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

Answer 624

``` Prematurity Male sex Diabetic mother C section Second born of premature twins ```

Answer 625

Ground glass appearance Indistinct heart border Low volumes Bell shaped thorax

Answer 626

Prevention during pregnancy: maternal corticosteroids Oxygen Assisted ventilation Exogenous surfactant given via endotracheal tube

Answer 627

Nitric oxide - pulmonary vasodilation

Answer 628

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

Answer 629

``` Meconium aspiration Infection - PROM, GBS Congenital abnormalities of heart and lungs Hyaline membrane disease Oligohydramnios ```

Answer 630

``` Pre eclampsia Maternal HTN Oligohydramnios Maternal infections Maternal drug use Placental insufficiency IUGR Post dates ```

Answer 631

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

Answer 632

``` Intrauterine infections Rhesus isoimmunisation Chorioangioma of placenta Gut atresia Hydrops fetalis Twin to twin transfusion syndrome Maternal cardiac/renal problems Diabetes ```

Answer 633

Premature Born at high altitude Maternal rubella infection in first trimester

Answer 634

``` Left subclavicular thrill Continuous machinery murmur Large volume, bounding, collapsing pulse Wide pulse pressure Heaving apex beat ```

Answer 635

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

Answer 636

``` Growth retardation Pinpoint petechial “blueberry muffin” skin lesions Microcephaly Sensorineural deafness Encephalitis Hepatosplenomegaly ```

Answer 637

Ash leaf spots which fluoresce under UV light Shagreen patches (rough patches over lumbar spine) Adenoma sebaceum (angiofibromas) Subungual fibromata Cafe au lait spots

Answer 638

Developmental delay Epilepsy (infantile spasms or partial) Intellectual impairment

Answer 639

Commonly associated migraines Severe nausea and sudden vomiting lasting hours to days Prodromal intense sweating and nausea Well between episodes

Answer 640

``` AVSD VSD PDA TOF ASD ```

Answer 641

If child develops bleeding complications

Answer 642

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