Paeds Flashcards

Question

Rheumatic fever - how to diagnose and treat

Answer 1

Jones criteria for diagnosis Evidence of strep infection (strep throat, rapid throat swab, scarlet fever, raised step antibody titre) + 2 of carditis, polyarthritis, chorea, erythema Marginatum, subcut nodules or 1 of above plus 2 of polyarthralgia, prolonged PR, fever, inflam markers, hx of rheumatic fever Ix: - throat swab, Rapid streptococcal antigen test (RAST), Anti-streptococcal antibodies, bloods. - ECG/ CXR/ Echo Mx: - IM benzylpenicillin - strict bed rest until CRP normalises - NSAID for arthritis - treat HF - ace inhibitors - corticosteroids may be needed for carditis - Benzos for chorea Prophylaxis with benzathine penicillin injections or oral penicillin should be carried out for 5 years if no carditis, 10 if carditis, life if carditis and valve issues. Mitral stenosis is a complication

Answer 2

Weight (quick estimation method) o <12 months: (age in months/2) + 4 o 1–5yrs—Weight (kg) = (2 x Age years) + 8 o 6–12yrs—Weight (kg) = (3 x Age years) + 7 Energy o 4 joules x kg for defibrillation Tube o Diameter: (age/4) + 4 o Length: (age/2) + 12 Fluids o Resuscitation: 10ml/kg o Maintenance: As above Lorazepam o 0.1mg/kg Adrenaline o 0.1ml/kg of 1:10,000 (10mcg) Glucose o 2ml x kg of 10% dextrose

Answer 3

Hypoxia - due to obstruction or respiratory depression - asthma, inhalation of foreign body - raised ICP, opiate poisoning, convulsions Fluid distribution - hypovolaemia due to vomiting, dehydration, blood loss, burns - maldistribution due to anaphylaxis or sepsis

Answer 4

Assess for global and local danger 1. Assess responsiveness - clap, call, elicit pain 2. Call for help 3. Assess airway - look feel move for 10s - clear any visible obstruction from airway and secure with guedel 4. Rescue breath x5 - only suction if visible meconium 5. Assess for signs of life - brachial pulse if <1, carotid if >1. Start chest compressions if no signs of life, no pulse or pulse <60bpm 6. Start chest compressions at 15:2 rescue breaths at rate of 100bpm 7. Call resus team after 1 min if alone

Answer 5

Innocent childhood murmur - soft, systolic, changes with position Ejection systolic - aortic or pulmonary stenosis, teratology of fallow, truncus arteriosus, ASD Pan-systolic - VSD, mitral regurg Diastolic - mitral stenosis/ aortic regurg, ASD/ VSD Continuous - venous hum, patent ductus arteriosus (mechanical), co-arctation of aorta

Answer 6

Diagnosed if fever >39 for > 5days + 4 or more of: C - conjunctivitis - bilateral, dry R - Rash, maculopapular E - edema, erythema, desquamation A - lymphadenopathy M - mucosal inflammation - strawberry tongue Ix: - FBC - leukocytosis/neutrophilia/ platelets high then low - LFT - raised transaminase/ bilirubin - urinalysis shows sterile pyuria - abdo US shows gallbladder enlargement - ECG - abnormalities due to carditis - Echo - look for coronary artery aneurysms Management - admit to hosp and bed rest - Aspirin + PPI high dose until fever subsides then low dose until repeat Echo at 6-8w - IVIg is mainstay of treatment - 2g/kg over 10-12 hrs - If resistant (persistent fever) repeat IVIg, give steroids, anti-TNF, plasma exchange, ciclosporin etc For aneurysm - refer to Paeds cardiologist - percutaneous coronary intervention e.g. Intracoronary thrombolysis, balloon angioplasty, stent, ablation

Answer 7

Congenital heart defect - non responsive to O2 Resp failure - meconium aspiration, surfactant deficiency Shock, metabolic acidosis Persistent pulmonary hypertension Infection

Answer 8

tetralogy of fallot Ventricular septal defect (VSD) Pulmonary stenosis (PS) Right ventricular hypertrophy (RVH) Overriding aorta Presents in 3 severities - mild - just pulm stenosis/ RVH - often asymptomatic and present with cyanosis aged 1-3 - moderate to severe - present if first few weeks of life with cardio/resp distress - extreme - identified on antenatal scan Present with cyanosis, clubbing, signs of HF (oedema, tachycardia, sweating, pallor, basal crackles). Also have 'tet' spells of deep rapid inspiration, hypoxia, cyanosis, collapse and irritability/crying. On exam: - single S2 due to only aortic valve closure - long systolic murmur due to VSD - ejection systolic murmur - Aortic click due to dilatation of aorta - heave if RVH, thrill Investigations: - Obs e.g. O2 sats, BP etc - ECG - RVH, R axis deviation - Microarray/ karyotyping - CXR - boot shaped heart - Echo - gold standard - MRI pre and post surgery Management - Conservative - squating to imrove venous return - medical - Prostaglandin infusion (keep PDA), - morphine (reduce hyperpnoea), beta blocker and saline bolus for tet spells - surgical - palliative procedures can stent RVOT, definitive surgery at 3m - 4yrs old - lifelong cardiac follow up

Answer 9

Presents with early cyanosis often triggered by crying - typically within 24hrs to 1w if no VSD. If VSD present then baby has mild cyanosis, signs of congestive cardiac failure e.g. tachycardia, tachypnoea, diaphoresis On exam RV heave, loud single second heart sound, systolic murmur if VSD Ix: - O2 sats performed as screening, will be low - Capillary blood gas - low PaO2 and high metabolic acidosis - egg-on-a-string appearance on CXR - ECG - Echo gold standard Mx: - start prostaglandin infusion asap - correct metabolic acidosis - if needed do balloon atrial septostomy - Cure with arterial switch operation at 3d old

Answer 10

When VSD/ASD/ Patent ductus arteriosum mean that LtoR shunt becomes RtoL as a result of a rise in pulmonary vascular resistance

Answer 11

Presents with cyanosis in first few weeks to month and poor feeding On exam have loud S2, pan-systolic murmur of VSD Ix: - ECG - superior axis - Echo - LVH, tricuspid atresia, small RV - CXR - LVH, increased or decreased pulm vascular markings Mx: - emergency give prostaglandin infusion and balloon septostomy - Definitive surgery - multistage - relies on passive venous return to lungs

Answer 12

VSD Presentation depends on size Small - often asymptomatic, discovered due to audible systolic murmur Moderate - sweating, easily fatigued, tachypnoea Severe - sx of heart failure - SOB, chest pain, cough, failure to thrive, freq chest infections Ix: - bloods to look for non cardiac causes e.g. infection - Microarray if suspect genetic cause - CXR - cardiomegaly, pulm oedema, pleural effusion - ECG - LVH - Echo gold standard Mx: - adequate calorie intake and furosemide to reduce pulm vascular resistance first line - 2nd line is ACEi to reduce size of shunt or digoxin to improve contraction - surgery done at 3-4m if signs of congestive cardiac failure or pulm hypertension - place patch over defect from left ventricle. Open or trans catheter.

Answer 13

Majority asymptomatic, can present in young with tachypnoea, failure to gain weight or recurrent chest infections. Majority of cases presents after a stroke or with arrhythmia/ HF in older patients. Associated with smoking, foetal alcohol syndrome, maternal diabetes, rubella, genetics, DS On exam hear soft systolic murmur, wide fixed second heart sound, diastolic rumble of increased blood through tricuspid ECG - Normal or R axis deviation, tall P waves, RBBB CXR - Normal in kids, cardiomegaly in adults Echo - gold standard CT/MRI look for structural defects around heart Mx: Kids - do not exercise limit, if small <5mm will close on own, if symptomatic repair with open or transcatheter surgery Adults - manager arrhythmia/ HF, perform surgery once stable

Answer 14

Most asymptomatic. Large shunt can cause poor weight gain, feeding and recurrent LRTI Continuous murmur beneath left clavicle/ upper left sternal boarder. Wide pulse pressure and bounding pulse Echo gold standard, may also do CXR/ECG If asymptomatic regular echo until 1yo to monitor, if still patent close via end-vascular occlusion Indomethacin, ibuprofen can be given to close the PDA, more effective in premature neonates If symptomatic - diuretics for HF sx, close earlier If raised pulmonary resistance due to another CHD then keep open with prostaglandin E1 infusion

Answer 15

If severe can present with tachypnoea, shock, collapse, HF on 2nd day of life If ductus arteriosus is patent - often less severe - can present later or in childhood with neuro or musculoskeletal signs e.g. headache, nosebleed, leg cramps, lower limb weakness, cold feet On exam; Distal pulses reduced and delayed - weak or absent femoral pulse Upper limb BP higher than lower limb Late systolic murmur over infraclavicular region, radiates to back over scapula Ix: Bloods if shock to rule out other causes e.g. sepsis CXR may show notching of ribs (dilation of collateral vessels), indentation of aorta, HF ECG Echo - allows assessment of severity MRI CT angiogram Mx: Medical - treat HF with diuretic, ACEi + BB, Digoxin as in VSD and AVSD - prostaglandin E1 to maintain PDA Surgical - catheter balloon angioplasty or stenting - more complex procedures if needed e.g. surgical resection

Answer 16

Usually diagnosed prenatally via US scan - if not is a paediatric emergency at birth Causes cyanosis, tachypnoea, tachycardia, asymmetrical chest wall, absent breath sounds on one side and bowel sounds in the chest Ix: - do CXR/ US to confirm - Do ABG to check pH and PaO2/CO2 - general bloods - Screen cardiac, neuro, urinary systems for associated defects Mx: - intubation, avoid bag and mask as can inflate bowels - blood pressure support with fluids/ ionotropes - insert orogastric tube for decompression of stomach - surfactant may be needed - monitor blood glucose and calcium Definitive treatment is surgery to replace bowel in abdomen and close defect in diaphragm

Answer 17

Presents with irritability, crying, fussing that begin/ end for no reason and last > or equal to 3 hours, on 3 or more days for greater or equal to a week Baby has inconsolable crying, red face, clenched fists, brings knees to chest, flatus Clinical diagnosis - do general exam and hx Baby should be normal weight, if failure to thrive consider alternative diagnosis Management: - general advice on feeding regimes, temperature of house, babies clothing and course of condition (usually resolves by 4m) - shared care for baby to prevent mental exhaustion - hold upright after feeding, wind, rocking - hypoallergenic diet beneficial in some - simethicone can reduce flactulance but no proven efficacy

Answer 18

Allergy can be IgE mediated causing urticaria, angioedema, anaphylaxis or non IgE mediated causing delayed diarrhoea, vomiting, abdominal pain Investigate with skin prick test, ELISA allergen test, exclusion and re-introduction diet Treat allergy with exclusion from diet, extensively hydrolysed/ amino-acid based milks, sodium cromogilcate/ antihistamines/ corticosteroids if 1st degree relative has food allergy exclusively breastfeed for 1yr Majority resolve by 2yrs old

Answer 19

Post viral gastroenteritis lactase deficiency - causes diarrhoea, vomiting, abdo pain after milk consumption. Lasts 4-6w after viral infection.

Answer 20

Often presents in first 3 months of life, more common in formula fed babies Either IgE or non-IgE mediated IgE presents with urticaria, angioedema, pruritus, erythema, N+V, diarrhoea, colicky abdo pain and upper/lower resp tract symptoms Non- IgE does not present with urticaria/ angioedema or upper resp tract sx of allergy e.g. itching/ sneezing/ congestion. Does present with more abdominal sx including GORD, infantile colic, blood/ mucus in stool, food aversion, faltering growth etc. Clinical diagnosis - may do RAST if diagnosis unclear or associated asthma/ atopic disease Management - Exclusion diet until 9-12mo then try reintroducing every 6-12m - If breast fed mum has to become dairy free - If bottle fed - hydrolysed 1st line, amino acid 2nd line

Answer 21

``` a- look and relieve obstruction/ allergen b- treat bronchospasm/ resp distress c- pulse, colour, bp d- consciousness, blood glucose e - expose but avoid heat loss ``` If mild give oral antihistamines and observe for 2hrs if severe: - high flow o2 via reservoir, intubate as needed - lie flat, raise legs - IM adrenaline, dose adjusted to age. repeat after 5 mins as needed. >12 0.5mg, 6-12 0.3mg, <6 0.15mg, up to 6m 0.1-0.15mg 1in1000 - Try IV if 2x doses, no improvement - Nebulised adrenaline if stridor/ laryngeal oedema - iv fluid challenge 20ml/kg warmed colloid - after acute management give chlorphenamine/ hydrocortisone admit for 6-12 hours, measure tryptase after emergency treatment to confirm then 1-2 hours later

Answer 22

Presents commonly in babies/ young children with regurgitation following feeding, irritability, distressed behaviour, hoarse/ chronic cough, faltering growth, pneumonia, feeding difficulties (gagging, choking). Key features - occurs after feeds, no bile/ blood in vomit, no projectile vomiting Generally is a clinical diagnosis - may use endoscopy, 24hr pH probe, radioisotope milk scan, oesophageal manometry, CXR if needed Mx: - hold baby upright after feeds, do not feed just before bed - formula fed: 1st ensure baby not fed too much (no greater than 150ml/kg/day), 2nd reduce quantity and increase frequency of meals, 3rd add thickener, 4th remove thickener and add alginate - breast fed: give alginate mixed with water after feed If no improvement with alginate after 2w of use then consider ranitidine or omeprazole Consider pads referral/ further investigations/ surgery if still no improvement

Answer 23

Differentials - Pyloric stenosis - young child in first few weeks of life - GI obstruction - Appendicitis - Gastroenteritis - GORD - Raised ICP, head trauma - Urinary tract issues: UTI, stones - Hirschprungs - DKA - Testicular torsion - ptussis

Answer 24

Presents with vomiting in 2-8w old. - non bilious - 30-60 mins post meal - increases in severity over time as stomach contracts to try and empty - becomes projectile - may be some haematemesis - baby still hungry - is thin, failure to thrive Also have reduced bowel movements, dehydration/ weight loss, abdominal 'olive sized' pyloric mass in epigastrium Ix: - US gold standard - hypertrophy of pylorus muscles - U&E/ Blood gas may show low K+/Cl- and metabolic alkalosis - Test feed to feel pyloric mass/ observe peristalsis is sometimes performed Mx: - correct electrolyte and fluid imbalances - NG tube used to aspirate stomach - regular blood gas/U&E - surgery - Ramstedt pyloromyotomy (open or laparoscopic)

Answer 25

Most commonly cause is viral gastroenteritis Other causes - bloody diarrhoea think bacterial infection - systemic infections e.g. pneumonia, UTI - NEC - Intussusception/ appendicitis - Abx associated colitis, lactase deficiency - hirschsprungs, meckels, volvulus, short bowel syndrome - haemolytic uraemic syndrome - acute presentation of crohns, UC, coeliac, CF Ix: - stool sample - culture and sensitivity, calprotectin if suspect IBD - Bloods - signs of infection, U&E for electrolytes and dehydration, IgA/ tTg, inflam markers - Abdo xray - NEC, volvulus, intussusception, short bowel, hirschsprungs - Breath test for h pylori, biopsy, endoscopy, sweat test Management: - Treat cause - If viral - supportive care, bed rest, oral rehydration - If bacterial/ protozoal - Abx based on stool culture - If supportive not sufficient consider racecadotril for symptomatic relief and rehydration - cont breast feeding Admit to hospital if - Diagnosis unclear, not just viral - systemically unwell - signs of dehydration esp if <6m old - cant orally rehydrate - pre-existing medical condition e.g. diabetes

Answer 26

Present with multiple wet loose stools per day. Often more pale and smelly than normal, is presence of undigested food and rarely abdo pain Examination commonly normal, child 1-5yo, no problems with growth, happy and otherwise well Clinical diagnosis - most grow out of it by 5 Management - monitor fluid intake, make sure not too much - limit fruit juices and squash - increase fat in diet - change fibre in diet (too high or too low is bad)

Answer 27

Usually viral (rotavirus or Norovirus), can be bacterial (campylobacter or Ecoli) or protozoal Presents with: - acute onset watery diarrhoea w/without vomiting - generalised abdominal pain - fever - dehydration - hx of recent contact/ recent travel - bacterial may have bloody diarrhoea Clinical diagnosis, do stool sample if: - suspect septicaemia - blood/ mucus - last >7 days - immunocompromised - recently been abroad - diagnostic uncertainty Assess hydration status - if clinically dehydrated or in shock refer to hospital Mx: - continue with normal feeds - encourage fluids, add ORS - stop fruit juices and carbonated drinks If dehydrated - Give ORS 50ml/kg over 4hrs alongside normal fluid intake If in shock, red flags or cont dehydration start IV fluids Racecadotril can be used to reduce sx and improve rehydration if >3mo Abx if bacterial and suspect septicaemia After rehydration - start full fat milk immediately - slowly reintroduce solid food - no carbonated or fruit juice until diarrhoea stops - no school until 48hrs after last episode - no swimming until 2w after last episode

Answer 28

Characteristically low birth weight preterm babies in first few weeks of life - presents with not tolerating feed, N+V, change in bowel habit (bloody stools), abdo distention and abdo wall erythema. May also see fever, signs of shock (bradycardia, tachypnoea), on exam On exam see visible intestinal loops, erythema of abdomen, reduced bowel sounds, ascites/ abdo mass Ix: - AXR - pneumatosis intestinalis (gas cysts), dilated bowel loops, perforation, thickened bowel wall - bloods - FBC (low platelets/neutrophils), raised CRP, U&E as baseline, blood gas (lactic acidosis) - US may be helpful - new biomarkers can help with early diagnosis - blood cultures Mx: stop feed, decompress, correct electrolytes, abx - Admit to hosp, NBM - nasogastric tube for intermitted bowel decompression - IV fluids, TPN and IV abx for 10-14 days - amoxicillin/cefotaxime/gent + metro/clindamycin - ventilatory support/ circulatory support (dopamine/naloxone) if shock - consider antifungals if not improving with abx or recent course of abx - paracentesis for ascites Surgery may be indicated if perforation or section of necrotic bowel complications: DIC, sepsis, acquired short bowel (malabsorption), perforation, strictures/ abscess

Answer 29

2 or more of following in last 8w - <3 stools per week - pain on passing stool - >1 incontinence per week - palpable stool in abdomen - passing stools that block toilet - withholding behaviours Red flags: - sx commence from birth - delayed passage of meconium >48hrs - Ribbon stools - leg weakness - abdo distension w. vomiting - abnormal exam findings Treatment of idiopathic constipation: Conservative - diet - increase fluids and fibre - address anxiety - encourage using toilet with regular toilet breaks, stars/ rewards, Medical - disimpaction - 1st line - movicol - 2nd line if not effective in 2w - Senna Treat anal fissure with lignocaine ointment Maintenance = long term movicol

Answer 30

Failure to pass meconium within 48 hrs, abdo distension/ vomiting in neonates Treatment resistant constipation, abdo distension/pain and poor weight gain in older kids Can present as enterocolitis - fever, abdo pain, foul smelling and bloody diarrhoea, vomiting Ix: - gold standard is rectal biopsy - also consider AXR (see fluid levels), anal manometry, barium enema - Bloods (FBC for raised WCC in enterocolitis) Mx: - manage obstruction with nasal or rectal decompression/ washout and fluids and NBM - manage enterocolitis with NBM, IV fluids, IV Abx Long term treatment for hirschprungs is surgery

Answer 31

Bouts of abdominal pain (poorly localised around umbilicus), N+V, but no headache, followed by sleep and recovery, well between bouts - last 2-72 hours - may have associated features of migraine e.g. light sensitivity, nausea, sensitivity to movement At least twice in the preceding 12 months, all of the following: - Paroxysmal episodes of intense, acute periumbilical pain lasting at least an hour. - Intervening periods of normal health, lasting weeks to months. - Pain interferes with normal activities. - Pain associated with two or more: anorexia, nausea, vomiting, headache, photophobia, pallor. - Symptoms cannot be attributed to another medical condition. Important to exclude other causes e.g. - Intususseption - US - Volvulus - AXR - Pyloric stenosis - olive sized mass, repeated projectile - apendicitis - fever, raised CRP/ WCC - mesenteric adenitis - fever - constipation - abnormally hard stools Conservative - lie down in a dark room, rest, regular sleeping and eating routine Acute attack - paracetamol, sumitriptan, antiemetics Preventative - pizotifen Antiemetic - domperidone

Answer 32

Inflammed abdo LN due to viral illness hx of cold/ sore throat, pain in umbilicus/RIF, high fever, nausea/ diarrhoea differentiating from appendicitis: - rosvings sign negative - upon observation pain stays steady or improves, not get worse - higher fever than to be expected from appendicitis - appetite retained unlike appendicitis - WCC, CRP often normal Ix: - often clinical diagnosis - if unsure have period of observation, do US - blood tests Tx: - rest, painkillers - safety net - Abx if suspected bacterial cause

Answer 33

Often 5m - 3yo Present with acute onset colicky abdominal pain every 10-20 mins associated with crying, irritability, pallor, raising knees up to chest. Child feels better or tired between episodes. As symptoms progress develop bilious vomiting and red current jelly stool. In late cases get signs of dehydration and shock On exam may feel sausage shaped mass in RUQ or R flank Ix: - gold standard is US - see target sign - Bloods for signs of infection/ dehydration/ DIC/ shock - AXR - may show gas filed proximal bowel, no gas in distal bowel, may show perforation - Barium enema shows proximal contrast, closure of bowel and minimal distal contrast Mx: - hospital admission - IV fluids and NG tube drainage - pain killers 1st line = enema to reverse invagination - air or barium - try 3x for 3 mins - not do if perforated or peritonitis 2nd line = surgery - do if peritonitis/ perforation - pathological lead point - enema not effective - symptoms>24hrs

Answer 34

Can occur in adults or children, sigmoid most common across all ages, midgut specifically in babies and children Presents with sudden onset bilious vomiting, abdominal discomfort, constipation, bloating. if develop ischaemia get intense abdominal pain, bloody stool and signs of septic shock/ peritonitis Ix - first line is emergency contrast imaging using barium via milk or NG tube. See bird beak sign and corkskrew duodenum. - AXR shows double bubble sign - US can exclude intussusception - do bloods (FBC, U&E, CRP) and screen for sepsis Mx - admit to hospital - drip and suck - IV fluids and NG tube aspiration - emergency Ladd's procedure to rotate obstructed bowel - do secondary procedure 36 hours later if suspect bowel ischaemia and remove as needed

Answer 35

Meckels diverticulum commonly also presents with obstruction, may be pathological lead point for intussusception Ix: Scintigraphy for bleed AXR or US for obstruction Mx Manage shock/ obstruction surgical resection

Answer 36

Cause of prolonged jaundice in newborn - have associated light stool and dark urine - failure to thrive due to malabsorption Hepato/ splenomegaly on exam Ix: - raised bilirubin levels (serum or transcutaneous) - conjugated hyperbilirubinaemia - hepatic biopsy confirmative - scintigraphy, US also useful - abnormal LFTs - raised GGT - exclude alpha-1 antitrypsin deficiency and cystic fibrosis (sweat chloride test) Mx: Medical - give Abx to avoid cholangitis, UDCA to improve bile flow, fat soluble vitamin supplements Surgery - portoenterostomy - attach small bowel to portal triad of liver. Liver transplant as last resort

Answer 37

Normal = haemolytic uraemic syndrome | Abnormal clotting = DIC

Answer 38

Can present in neonates with cholestasis, jaundice and hepatitis Typically presents in 30-40s with early onset emphysema/ COPD Can also present in adulthood with hepatitis, liver failure, cirrhosis

Answer 39

In kids <5 not able to diagnose as they cannot before objective tests - treat based on clinical sx and needs step 1 - SABA step 2 - 8w trial of moderate dose paeds ICS and reviews. Continue on low dose paeds ICS if sx return in <4w step 3 - add in LTRA step 4 - stop LTRA and refer to secondary care In kids >5 can do objective tests: 1st line investigation is spirometry. If FEV1/FVC <0.7 suggests obstruction and consider confirming with bronchodilator reversibility test. If >12% + 200ml is positive for diagnosis of asthma If ambiguity 2nd line test is FENO - >35ppb is +ve for asthma If diagnosis still uncertain consider 2-4w peak flow diary to look for variability >20% ``` step 1 - Saba step 2 - low dose ICS Step 3 - add LTRA Step 4 - swap LTRA for LABA Step 5 - Mart Step 6 - moderate dose ICS + LABA (mart or not) Step 7 - refer ```

Answer 40

For children and young people aged 16 and under: ``` Low = 200 mpg or less budesonide Moderate = 200-400 High = >400 ```

Answer 41

- Age at onset of symptoms - Frequency of symptoms - Severity of symptoms (how many days of school missed? Can the child do PE at school? Can they play with their friends without getting symptoms? Night time symptoms?) - Previous treatments tried - Any hospital attendances (A+E or admissions – including HDU/ITU admission – ventilated?) - Presence of food allergies - Triggers for symptoms: Exercise, cold air, smoke, allergens, pets, damp housing - Disease history: Viral infections, eczema, hay fever - Family history of atopy

Answer 42

Moderate - sats >92 - 4 puffs of salbutamol followed by 2 puffs every 2 mins up to max of 10 - oral prednisolone - review, if worsening admit, if better discharge with 3d oral steroids and tapering SABA, review in 48hrs Severe - sats <92, peak flow 33-50% predicted - consider admission - nebulised salbutamol +/- ipratropium - oral prednisolone - oxygen - aim sats 94-98% Life threatening - sats <92, cyanosed, silent chest, unconscious, peak flow <33% - oxygen - nebulised salbutamol + ipratropium - IV hydrocortisone - aminophylline - magnesium sulphate - escalate When discharge put on wheeze discharge plan - look at

Answer 43

Occurs in infants up to 2 years of age, caused by RSV Presents with coryza for 1-3 days followed by all of - persistent cough (often wet sounding) - tachypnoea/ chest recession - fine crackles/ end expiratory wheeze can also have fever, tachycardia, poor feeding, rhinorrhoea ix - pulse oximetry - consider throat swab for RSV in secondary care - CBG - CXR if temp>39 or focal resp signs indicative of pneumonia - cultures/ urine dip if pyrexic mx - primary care = supportive. encourage fluids, feeding, give antipyretics only if child distressed - immediately refer if sats <92, apnoea, signs of resp distress, cyanosis, RR >70, consider referral if dehydrated, less than 50% normal feeding, Comorbs, RR>60, <3mo or premature in secondary care = supportive - fluids if needed - high flow nasal oxygen if sats <92%, CPAP if resp failure - NG tube for feeding - Upper airway suction if issues feeding or apnoea Discharge once - clinically stable - taking oral fluids - O2 sats > 92 for 4hrs Palivizumab can be used for prophylaxis treatment in vulnerable

Answer 44

Presents with SOB, productive cough, fever, raised RR, cyanosis, focal coarse crackles, absent breath sounds/dull percussion if effusion May do CXR if diagnosis uncertain nasopharyngeal aspirate/ sputum culture can help identify cause blood cultures necessary if suspected sepsis Treatment is supportive if critically unwell with signs of resp distress, low O2 sats, cyanosis, dehydration admit to hospital Give antipyretics, IV fluids, first line abx is amoxicillin for 7-14 days, second line is co-amoxiclav

Answer 45

Obstruction due to inhaled foreign body or tumour

Answer 46

Occurs in children aged 6m to 5yr Involves onset of SOB, resp distress, cough and generalised expiratory wheeze after symptoms of viral infection (sore throat, coryza etc) Due to inflammation, oedema and swelling of airway. No other triggers other than viral infection. Often no hx of atopy. No crackles differentiates from bronchiolitis Management: - identify life threatening attack - cyanosis, sats <92, unresponsive etc and admit to hosp - treat as acute asthma with oxygen, salbutamol puffs/ nebs, steroids, ipratropium - discharge with discharge wheeze plan

Answer 47

Presents differently at different ages - in neonate presents with meconium ileus - in infant presents with failure to thrive, recurrent chest infections and steatorrhoea - in childhood presents with rectal prolapse, nasal polyp and sinusitis - in adolescence presents with DM, chronic lung disease, gallstones, liver cirrhosis Ix: Most detected via screening in neonates - raised immunoreactive trypsinogen on heel prick - confirm with sweat test - Cl >60 with Na lower on 2 occasions - CXR - hyperinflation, inflam, infiltrates, bronchial dilation - Spirometry - Sputum culture - LFT, FBC, U&E, fasting glucose, vitamin lvls - Faecal elastase for pancreatic issues Management - for resp do chest physio, give oral abx for prophylaxis (fluclox from diagnosis to age 3 or azithromycin), IV abx in exacerbation, give dornase alfa + hypertonic saline to help reduce and clear secretions. May need transplant - start on high calorie diet, supplementary fat soluble vitamins - monitor growth - CREON may be needed if pancreatic insufficiency - take immediately before or just after each meal - Insulin for diabetes - nasal polyp - nasal steroids or polypectomy - fertility treatment with IVF for azoospermia - liver transplant for liver failure, UDCA can improve bile flow but not alter disease - protect against osteoporosis with vit D, ca, bisphosphonate

Answer 48

oligoarthritis - <4 joint involvement in first 6m Polyarthritis: >4 joints in first 6m, can be RF +ve or -ve Systemic arthritis: peaking temperature >39, lymphadenopathy, salmon pink rash, serositis, hepatic/splenomegaly psoriatic: 2 of: dactylitis, nail pitting/ onycholysis, 1st degree relative with psoriasis All present with morning stiffness, joint swelling, pain Mx Involve MDT - physio, OT, social care, child psychologist etc Nsaids for pain relief Steroid joint injections If steroid injections not effective, same joint needs >3 then start on methotrexate. Alternatives to methotrexate include Etanercept or tocilizumab joint replacement, synovectomy may be needed

Answer 49

Distinguish from mild cerebral palsy or tightness of the Achilles tendons or inflammatory arthritis in the foot/ankle In boys exclude DMD If unilateral consider DDH

Answer 50

Most common cause of hip pain in children aged 2-12 Presentation - Hip pain - Difficulty weight bearing - fever - irritability Acute onset followed by spont recovery with no systemic upset. May be preceded by a viral illness ``` Important to differentiate from septic arthritis Use kosher criteria: - Pain causing inability to weight bear - ESR >40 - WCC >12 - Temperature >38.5 degrees ``` +ve leg roll, no pain at rest and pain only at extremes of ROM is indicative USS and US guided joint aspiration can give definitive differentiation Management - rest, analgesics e.g. NSAIDS/ paracetamol - Mobilise once pain improved - Safety net to go to A&E if pain gets worse or develop a fever - Review in a week to check for resolution

Answer 51

Most common cause is staph A | Do US, bloods, joint aspiration

Answer 52

subluxation/ dislocation of hip due to poor growth/form of the acetabulum/femur Commonly presents in 0-5yo More common in females RF are family hx, breech birth, multiple pregnancy, first born, swaddling, large size, prematurity, oligohydramnios Screen all babies for DDH with Barlow and Ortolani If first relative hx, breech at birth before 36w or breech presentation after 36w then screen with US instead Commonly presents on exam with asymmetrical skin folds, leg length (Galeazzi sign) and limitation/asymmetry of the hip on abduction when flexed to 90 degrees. In older children presents with delayed walking, painless limp, toe walking on affected side, sensation of instability, increased ROM, trendelenburg gait or waddling gait if bilateral. Diagnose with dynamic US, in older kids xray can be used Treatment - if <6mo first line is bracing using a pavlik harness - if >6mo consider surgery with closed or open reduction and casting, osteotomy may be needed in older kids

Answer 53

Avascular necrosis of the femoral epiphysis leading to subchondral collapse and fracture. Most common in boys age 3-10, predisposes to developing OA Presents with several week onset of hip pain, limp and poor hip rotation On exam see stiffness of all movements in particular of internal rotation and abduction, +ve leg roll, antalgic gait if early, Trendelenburg if late Ix: - xray in early disease may show increased joint space, later shows compressed, patchy femoral head, then shows collapse, deformity and new bone formation - MRI/ technetium bone scan can show avascular pathology - FBC/ ESR to exclude inflam causes Mx: If <6yo - conservative mx - rest, not weight bear, painkillers , casting/ traction and physiotherapy until ossification complete If >6yo - consider surgery with pelvic/femoral osteotomy Regular X-rays to monitor healing Often need early hip replacement later in life due to OA

Answer 54

Occurs in obese adolescents aged 10-15 Can be acute or chronic, stable (can weight bear) or unstable (cant weight bear, high risk of osteonecrosis) Acute presents within 3w of sx onset with sudden onset severe hip pain so cant walk or stand, external rotation of the hip, limp, reduce internal rotation Chronic presents after 3w of onset, sx milder, able to walk but antalgic gait, external rotation of leg while walking, shortening of leg, atrophy of thigh may be noted Both present with Drehmann's sign - on flexion of hip, get passive external rotation Ix: - xray - shows slipped capital femoral epiphysis, line of Klein fails intersect the epiphysis during acute phase (Trethowen sign) - US, CT, MRI may be used - Bloods to rule out inflam cause - Technetium bone scan Mx: - stop weight bearing immediately, prevent movement, rest and admit to hospital - give analgesia for pain - insertion of hip screw is first line surgical treatment - open reduction +/- osteotomy is alternative

Answer 55

Most common in adolescents - intravaginal - just testes rotates. Can also occur in neonates - extravaginal - testes and tunica vaginalis rotates Presents with acute onset severe testicular and lower abdominal pain associated with nausea and vomiting often after a period of exercise. Often had a brief episode of previous pain with spont resolution On examination the scrotum on the affected side is red, swollen and the testicle is sitting higher than normal. Lifting the testicle does not ease pain and the cremasteric reflex is absent Doppler US can be performed to confirm diagnosis but should not delay treatment Treatment involves NBM, analgesics and immediate referral to surgery for de-torsion and bilateral orchidopexy

Answer 56

Undescended testes Differentials are retractile testes that elevate out of scrotum when triggered by cremasteric reflex (can become ascending testes), or ectopic testis Identified on routine neonatal examination Not uncommon at birth - if one, review at 6-8w - if bilateral do abdo US to locate testes and perform tests to identify congenital adrenal hyperplasia - β-hCG, α-FP, and LDH to check for embryonal cell carcinoma If needed diagnostic US, MRI can be used Management: Observe until 6m - most resolve on own If still undescended at 4-5m refer to paeds surgery hormone treatment can be used: beta hCG or GnRH First line treatment is orchidopexy Increased risk of testicular cancer in later years so educate need for self examination. Also risk of infertility if not corrected timely. Increased risk of torsion

Answer 57

BXO - balanitis xerotica obliterans Treat with circumcision

Answer 58

Suspect posterior urethral valves, do US within 48hrs, emergency surgery

Answer 59

``` Reassure parents mostly resolves on own Punishment does not help Reduce drinking/ caffeine before bed Manage constipation Use toilet before bed Wake up in night to go to toilet ``` If consistent enuresis over age of 6 consider: - enuresis alarm - desmopressin for short term relief If daytime symptoms need to do urinalysis to exclude infection, DM + do US/ MRI to look for neuropathic bladder, ectopic ureter etc

Answer 60

Henoch-Schonlein purpura - IgA mediated vasculitis If abdo pain - do US as associated with intussusception Rest, rehydration, analgesia Monitor urinalysis and bp for signs of renal involvement Steroids/ plasma exchange may be considered in severe renal failure Usually self resolves after 4-6w

Answer 61

strep pyogenes and EBV Feverpain - absence of cough - onset in last 3d - fever in last 24hrs - purulent tonsils - sever inflammation 2or3 - delayed abx 4or5 - 5-10d phenoxymethylpenicillin Do not give amoxicillin to glandular fever - risk of rash tonsillectomy if >7 in one year, 5 per year for 2 year, 3 per year over 3yr

Answer 62

Presents with signs of URTI e.g. coryza, cough, fever, sore throat that then develops into characteristic barking cough, hoarse voice/cry (both worse at night), inspiratory stridor, increased work of breathing. Affects children 6m-12yr Most often parainfluenza (viral) Assess severity - westly clinical scoring system - mild - just barking cough - moderate - barking cough, stridor, sternal recession - severe/ resp failure - above + agitation/ lethargy, cyanosis If mild can treat at home If moderate to resp failure refer to hospital Often clinical diagnosis - not want to do ix if not needed as will distress child and worsen breathing - sats - CXR see steeple sign - laryngoscopy if suspect obstruction Mx: - supportive care with rest, antipyretics if fever causing distress and fluids - one off oral dexamethasone 0.15mg/kg (alternative is nebulised dobutamine or IM dex) If severe: - nebulised adrenaline to dilate airways - O2 therapy if sats low

Answer 63

Presents with severe one sided thoat pain, difficulty swallowing, drooling, trismus, hot potato voice On exam see swelling superior to tonsil on one side with shift of uvula away from the midline Can do US to differentiate from peritonsillitis and locate optimal location for drainage Treatment is a combination of rest, fluids, analgesia, IV or oral Abx (amoxicillin with clindamycin or metro), IV steroids, and surgical treatment with aspiration, incision and drainage or therapeutic tonsillectomy

Answer 64

Caused by strep e.g. pyogenes or HIB Presents with dysphagia, odynophagia, drooling, hot potato voice, fever, sore throat, tenderness over anterior neck, inspiratory stridor, dyspnoea Often have tripod sign - sit forward with hands on knees Do not use tongue depressor - refer for laryngoscopy (gold standard, perform in theatre incase intubation/ tracheostomy needed). Alternative is neck xray. Once stabilised do throat swabs. - blood cultures if systemically unwell - CT/MRI if suspect abscess Mx: - conservative with fluids, IV/ oral Abx - O2 therapy - nebulised adrenaline can act as bridging therapy until airway control - If acute resp failure may need to intubate or perform tracheostomy - drain abscess if present

Answer 65

Present with feeling generally unwell and lethargic, nausea and vomiting and abdominal pain. On exam see deep sighing (kussmauls breathing), tachypnoea and intercostal recession. Also see shock - tachycardia, low BP and increased CRT, May be change in consciousness pH <7.3 or bicarb <15 Glucose >11 Ketones >3 AtoE Airway - intubate if reduced consciousness Breathing - high flow O2 via face mask Circulation - If in shock give 20ml/kg fluid over 15mins - If not in shock give 10ml/kg fluid over 1 hour - ECG for potassium, take bloods, blood gas Disability - if reduced consciousness or vomiting insert NG tube Exposure - examine for signs of sepsis - fever Start on IV insulin infusion 1-2hrs after fluids - 0.05 – 0.1 units/kg/hour of a soluble insulin e.g. actrapid Retest blood glucose levels, if drop below 11 give 10% dextrose Treat high/low K+ DKA is considered to be resolved once the child is clinically well, drinking and tolerating food and the blood ketones are less than 1mmol/litre or pH is normal. Subcutaneous insulin can then be started and IV insulin stopped one hour later.

Answer 66

cavernous haemangioma, pre/orbital cellulitis

Answer 67

Mongolian blue spots

Answer 68

Small, round macule = capillary haemangioma, often self resolve Large patch/ discolouration = port wine stain, remain present throughout life, improve appearance with laser therapy

Answer 69

First line is wet combing or use of dimeticone 4% lotion. Alternative is malathion 0.5% Repeat treatment after 7 days to treat any lice hatching from eggs.

Answer 70

Lymphoblasts take over bone marrow, reduce: Hb (anaemia): lethargy, fatigue and pallor neutropaenia: frequent or severe infections Platelets (thrombocytopenia): easy bruising, petechiae Also get organomegaly (splenic), testicular enlargement, lymphadenopathy, gum hypertrophy, headaches, bone pain, pain over sternum Ix: - Tdt, PAS +ve - common in trisomy 21 - do karyotyping - do FBC - altered WCC, neutropenia, LFT/U&E prior to tx - Blood film - identifies blast cells - CXR to identify mediastinal mass - bone marrow biopsy and aspirate to confirm diagnosis - LP for CNS involvement Mx: - supportive measures including abx/ anti fungal for infection, G-CSF for myelosuppression, transfusion, allopurinol/rasburicase for tumour lysis syndrome - induce remission with vincristine, steroid, anthracycline - CNS protection - Maintenance with methotrexate and 6-mercaptopurine for 2.5-3 years - stem cell transplant

Answer 71

Neuroblastoma Diagnose by combination of bone marrow biopsy and urinary catecholamines Do US, MRI and MIBG scan (metastasic disease) Treat with monitoring if low risk or combination of surgery/radiotherapy/ chemotherapy if higher risk

Answer 72

Presents with asymptomatic abdominal mass - may be UTI, haematuria, anorexia, abdo pain Ix: - FBC, U&E - US or IV pyelogram can be used to visualise malformation of renal pelvis and hydronephrosis - Renal angiogram - CT can be used to define tumour, look for metastasis - Do not take a biopsy Managment - most treated with nephrectomy and chemo - If stage 3 might need postop radiotherapy - neoadjuvant chemo if unrescectable, bilateral or causing vena cava thrombus

Answer 73

Can be hereditary or sporadic Presents with leukocoria (white pupil reflex), new onset squint or in later presentations visual disturbance/ red irritated eye/ enlargement of eye Refer immediately to ophthalmology if suspected - Exploration under anaesthesia with dilated pupil - US - MRI - Genetic counselling Refer to bham children's or royal London - cryotherapy, phototherapy, chemo - enucleation as last resort

Answer 74

Hashimotos - presents initially with slowing of growth, sleepiness, weight gain, skin changes, cold intolerance Can also cause delayed puberty

Answer 75

Commonly caused by 21-hydroxylase deficiency Can present as salt wasting or non salt wasting Boys - might be hyperpigmentation/ slight enlargement of genitals otherwise normally present at 7-14 days with a salt wasting crisis: lethargy, vomiting, weight loss, dehydration, shock, low Na, high K+ Females - typically present with ambiguous genitalia, enlarged clitoris and common urogenital sinus. Precedes salt wasting crisis Can also present later in milder forms with irregular or absent periods, severe acne, rapid growth during childhood, early appearance of pubic hair Ix: - raised 17-hydroxyprogesterone is diagnostic of 21- OHD - check U&E for low sodium, high K+ - do blood glucose - corticotrophin stimulation test - see if rise in cortisol - pelvic US for ambiguous genitalia - Genetics/ karyotyping Mx - Hydrocortisone to replace glucocorticoids - Fludrocortisone to replace mineralocorticoids - 2-3x dose if unwell, may also need fluids, NaCl supplementation - medical alert card/ bracelet - surgery for ambiguous genitalia

Answer 76

Occurs due to immature brain injury - many causes including hypoxia, intracranial bleed, foetal alcohol syndrome, cocaine, congenital torch infections, asphyxia, birth trauma 4 main types - Spastic - increased tone and pathological hyper-reflexia (cortex issue) - Athetoid - Hyperkinesia (chorea, dyskinesia, athetosis), normal reflexes (basal ganglia issue) - Ataxic (loss of muscular co-ordination) - Mixed As well as above sx typically presents with: - delayed milestones e.g. not sitting by 8m, not walking by 18m - learning difficulties, epilepsy, feeding problems, failure to thrive, sensory impairment, toe walking Ix: - Brain US can identify major intracranial lesions, if not MRI can be used to investigate cause of known or suspected CP - TFTs, CSF, pyruvate, lactate and protein, chromosomal - EEG can help detect damage from hypoxia and vascular insult - evoked potentials can identify sensory issues e.g. hearing Mx: Involves MDT for physio stretching, Orthotics splints, SALT for feeding, speech and language, OT for wheelchair, hoists, social care for schools etc Medication - Baclofen for long term spasticity - Diazepam for short term spasticity relief in pain crisis - Botulinum A for focal spasticity - Levodopa or baclofen for dystonia - treat pneumonia/ epilepsy Surgery - Tendon releases - Bone osteotomy and realignment - correction of scoliosis - Dorsal rhizotomy

Answer 77

Defined as >6 symptoms of inattention or hyperactivity lasting >6m with significant impairment on functional well being, affecting >2 settings e.g. home/work and present before the age of 12 If suspected in primary care do a 10w watch and wait, if sx still present then refer to secondary care specialist for full psychosocial assessment - can use strengths and difficulties questionnaire or conners rating scale Exclude differentials e.g. caffeine, hyperthyroid, steroids, beta agonists, bipolar, personality disorder Mx: - education, school involvement, environment modification, diet (no sweetners) and exercise - can do a food diary and refer to dietician - Psychosocial support: group parent training programme for preschool age, group parent and child programme for school age. CBT can also be helpful - Medication - first line methylphenidate (only if above not worked, >5yrs old), monitor weight, height, pulse and BP 6monthly. Do weight, height, BP, pulse, CV exam and ECG before starting meds SE include stunted growth, hypertension, palpitations, disturbed sleep, appetite suppression

Answer 78

Presents with proximal muscle weakness/wasting and loss of function. Commonly children have delayed walking (later than 18m), waddling gait on running, gowers manoeuvre when standing from sitting, enlarged calf muscles Also commonly have speech and language delay, failure to thrive Due to absence of dystrophin Diagnosis - Raised CK may be first indication - Diagnosis based on combination of genetic testing, muscle biopsy and clinical exam Management - important to do early genetic testing and counselling before have another baby in early years: - physio for contracture - orthoses to prolong walking - may need serial leg casting - wheel chair, adaptations - prednisolone can help improve muscle strength and function - Vit D/ Ca supplements - Bisphosphonate if vertebral fractures In older years: - management of respiratory complications such as hypoventilation (NIV, oxygen), absent cough (cough assist, physio) and recurrent infections (prompt treatment) - management of cardio complications such as dilated cardiomyopathy (regular monitoring with ECG/ Echo and treatment with ACEi, beta blockers, diuretics, anticoag)

Answer 79

Present at any age, more commonly bilateral and may be associated with auras including hemiparesis, ophthalmoplegia and dizziness/syncope Criteria for diagnosis - with aura: 2 or more attacks with 3 of: aura present that comes on gradually, is fully reversible, lasts <60mins or is followed by headache within 60mins - without aura: 5 or more attacks lasting 2-72hrs with 2 of: uni/bilateral, throbbing, moderate-severe, aggravated by physical activity + 1 of N/V or photo/phonophobia RF - family hx, past hx of abdo migraine, Management - reassurance, headache diary to detect triggers, sleep/diet - acute attacks - paracetamol/ibuprofen first line, triptans second line. Domperidone/ prochlorperazine (>12yo) if nauseous - prophylactic - if significant affect on life - topiramate, propanalol, pizotifen first line

Answer 80

Can be communicating or noncommunicating/ obstructive Obstructive = aqueduct stenosis, mass, malformation, haematoma communicating = infection, SAH, intraventricular haemorrhage Presents with irritability, vomiting, headache (in older kids), large head size, tense fontanelles, impaired consciousness, papilloedema, 6th nerve palsy. Sunsetting sign of eyes, cracked pot sound on head percussion may be present Investigate with CT/US/MRI Managment - often just monitor - If needed acetazolamide and furosemide can help inhibit CSF secretion - Isosorbide can increase reasorbtion - Serial LP can precent need for neurosurgery in communicating - Mannitol can be used in an emergency - May need insertion of drain/shunt

Answer 81

``` macro = head circumference >98th centile Micro = <2nd centile ```

Answer 82

Differential for epilepsy Triggered by fear/anxiety/ pain Increased vagal responsiveness leads to asystole Child goes pale, looses consciousness, then limbs go stiff and exhibit clonic jerking Lasts 30-60s with quick recovery (shorter than epilepsy) Some fatigue after No tongue biting Ix: No EEG changes Do ECG to look for cardiac differentials May consider vagal stress test Mx: Treat with reassurance and education on seizure management e.g. recovery position If severe, frequent attacks consider pacemaker insertion

Answer 83

Marcus gunn

Answer 84

Associated with brain damage e.g. hydrocephalus, hypoxia, Down syndrome or can be idiopathic Presents with contractile spasms (sudden contractions followed by gradual relaxation) on waking or falling asleep. Tend to last several seconds and repeat over a period To investigate - do EEG - shows characteristic sign of hypsarrhythmia - MRI to look for structural cause - blood/ urine cultures if suspect infection - blood tests Management - first line is vabigatran, prednisolone or ACTH - second line is benzodiazepines, lamotrigine, valproate - pyridoxine and ketogenic diet can also help

Answer 85

Seizure in presence of temperature >38 without CNS infection from age of 6m to 6yrs Simple - generalised tonic clonic, last <15mins, not recur within 24hrs or illness, complete recovery within 1 hour Complex - focal partial seizure often affecting one side of body/ limb or not matching above Cause can be bacterial or viral Assess for signs of infection to decide cause, as well as signs of meningitis/ septicaemia (pale, mottled, non blanching rash, abnormal behaviour, stiff/floppy, tense fontanelles, meningism) Do traffic light signals Ix: - FBC, u&e, coag, glucose, CRP/ESR - Blood cultures - Urine mc&s - LP Mx: - reassure parents and teach first aid - recommend to call ambulance or try benzodiazepine if not resolve within 5mins - admit to hosp if systemically unwell, unsure of diagnosis, neuro deficit, recurrent, first presentation - Treat infection according to locus - antipyretics, fluids, remove excessive clothing - treat status as per normal Most stop by 6 years, prognosis good, some risk of epilepsy development

Answer 86

Can be generalised or focal Generalised = - absence seizures - vacant and unresponsive with small motor movements, occur regularly and last several seconds - tonic - stiff contraction - clonic - rhythmic jerking - myoclonic - quick shock like movements - juvenile myoclonic epilepsy - adolescents, seizure in morning, jerks of shoulders and arms, triggered by light or sleep deprivation (genetic) - generalised tonic clonic - spasm Focal = - simple - consciousness maintained or complex - lost - frontal - motor sx, sound production - parietal - sensory sx (numbness) - ophthalmic - visual disturbance - temporal - fear, auditory/ visual hallucinations

Answer 87

Commonly do EEG after 2nd seizure (photic stimulation and hyperventilation). Do sleep or long term EEG if no findings. Also consider MRI if present <2yo, focal onset, resistant to first line meds Education - first aid, safety including not having baths, avoid cooking near flames, heights etc Regular 3-12monthly reviews Drug treatment - dependant on type - tonic clonic - valproate or lamotrigine - absence - ethosuximide or valproate - tonic/atonic - valproate or levetiracetam - myoclonic - valproate or levetiracetam - infantile spasm - prednisolone or vigabatrin Stick to mono therapy, same formula and brand, stress adherence. Consider slow withdrawal after 2yrs seizure free Ketogenic diet can help as can vagal nerve stimulation in those resistant to first line AEDs Consider surgery if drug resistant - involves resection

Answer 88

Commonest causes are s pneumonia, Hib and Neisseria meningitides In neonates most common causes are group b strep, listeria and e.coli Present with general signs in neonates e.g. fever, irritability, change in colour, refusing food, N+V, joint pain, bulging fontanelle, non blanching rash In older children present with neck stiffness, photophobia, headache, N+V, joint pain, cold extremities, shock, altered mental status, seizure Ix: - Immediate LP unless suspect raised ICP or severe sepsis/shock. Send for WCC, glucose, lactate, culture, PCR, protein - Bloods including FBC, coag - Blood culture - Blood gas Management - immediate referral to secondary care - if meningococcal septicaemia, non blanching rash give immediate IM/IV benzylpenicillin - Supportive measures e.g. antipyretic, analgesic, nutritional, fluids - if >3mo start dexamethasone asap 0.15mg/kg 4x daily for 4d - Start on IV ceftriaxone if >3m or IV cefuroxime + amoxicillin or ampicillin if <3mo - give O2 as needed If raised ICP treat with fluid restriction, acetazolamide, mannitol If shock give 20ml/kg 0.9% saline fluid boluses Family prophylaxis with rifampicin or ciprofloxacin

Answer 89

Blood tests: FBC, clotting screen o Alternative cause for bruising Skeletal survey or bone scan o If physical abuse suspected, <2 years Brain imaging: o Needed if suspected head injury o If physical abuse suspected, <1 year o Looking for signs of skull fractures or bleed Retinal examination o If head injury o Retinal haemorrhages are a sign of intracranial injury o Also check for rib fractures Spinal imaging: o A full skeletal survey as part of bone scan - X-rays of the spine, including lateral views. o If any fracture is seen or spinal cord injury is suspected, do an MRI scan of the spine. Sexual health tests: o If suspected sexual abuse Forensic dentistry: o Interpret bite marks, differentiating animal from human ones, and sometimes identifying the abuser.

Answer 90

partial or total removal of the external genitalia or other injury to the genital organs for non-medical reasons Abdominal pain, new onset urinary sx or bleeding from genitalia, signs of infection, trauma or change in behaviour

Answer 91

Onset of secondary sexual characteristics in girls before age of 8 and boys before age of 9 Can be central or precocious pseudopuberty (peripheral) Central is due to raised GnRH and LH/FSH e.g. in intracranial tumours, intracranial infection, injury, hydrocephalus, can also be idiopathic Peripheral is due to raised sex steroid hormones, LH/FSH low. Causes include: CAH, mccune Albright, adrenal tumours, ovarian/testicular tumours, testotoxicosis Testes size helps with cause: bilateral enlargement = gonadotrophin release from intracranial lesion unilateral enlargement = gonadal tumour small testes = adrenal cause (tumour or adrenal hyperplasia) Ix: - assess sex hormone levels - Random LH - if high indicates central - GnRH stimulation test - if LH/FSH rise indicates central - TFT - 17-hydroxylase for CAH - US / MRI for adrenal/ ovarian tumours - Brain MRI for intracranial masses - Xray of wrist to assess bone age - bone age typically ahead of actual Mx: - may allow to continue as normal if idiopathic, not likely to affect height - treat cause, remove tumour via surgery - if early puberty likely to limit final height give GnRH analogues and delay puberty until appropriate age. Can give GH if needed - Aromatase inhibit e.g. testolactone used in mccune Albright and testotoxicosis

Answer 92

Absent testicular growth <4ml at age 14 Absent breast development by 13, primary amenorrhea by 15 Can be due to: - normal HPG axis: constitutional delay, chronic illness, malnutrition, excessive exercise, steroid therapy - abnormal HPG axis: tumour, irradiation, congenital, surgery -peripheral: primary gonadal failure, syndromes e.g. kleinfelters, turners, cyclophosphamide, CAH, PCOS, testicular torsion, undescended testes Ix: - Investigate for chronic disease - Karyotyping, genetic screen - FSH/LH and sex hormone levels (low in constitutional delay, high in gonadal failure) - Pelvic US - Brain MRI - bone age scan Mx - can reassure if constitutional delay - induce puberty with • Low dose testosterone therapy for 3-6m – Boys • Gradually increasing oestrogen with eventual cyclical progesterone – Girls If other cause - treat, induce puberty with testosterone injections, oestrogen/ progesterone as above

Answer 93

Home life: relationships, support Education: school, exams, future career/ further education Activities: exercise, leisure, social relationships Affect: body image, self-harm, depression Drugs: cigarettes, alcohol, illicit substances. How much and how often is key Diet: weight, caffeine, eating disorder Sex: concerns, periods, contraception. Use gender neutral terms Sleep

Answer 94

Can be caused by error in meiotic division leading to an extra chromosome, can be caused by mosaicism or can be caused by robertsonian translocation with chromosome 14 or 22 Features: hypotonia, epicanthic folds, Brushfield spots in iris, low set ears, high arched palate, loose skin on neck, single palmar crease, short little finger, wide gap between big and 2nd toe, heart defect (AVSD), duodenal atresia (double bubble sign) Ix: - Karyotyping to confirm diagnosis - Bloods to identify myeloproliferative disorder, polycythaemia - echo to look for structural heart defects - Assess hearing using brainstem audiometry provoked response - Do TFTs - Assess tone, do swallowing assessment if issues feeding - Assess red reflex for congenital cataract

Answer 95

Turners - one X chromosome Growth hormone and oestrogen replacement therapy Ejection systolic murmur due to bicuspid aortic valve

Answer 96

Klinefelters XXY Give testosterone injections on start of puberty Plastic surgery for gynaecomastia ICSI if trying to have child

Answer 97

Skin - striae, especially thoracolumbar and sacral. Cardiovascular - thoracic aortic dilatation/rupture/dissection (usually asymptomatic), aortic regurgitation, mitral valve prolapse, mitral regurgitation, abdominal aortic aneurysm, cardiac dysrhythmia. Lungs - bullae, pleural rupture causing pneumothorax Eyes - lens dislocation, closed-angle glaucoma, high myopia. Skeleton - arachnodactyly, hypermobility, arthralgia, joint instability, finger contractures, pectus excavatum or carinatum deformities, misshapen chest, kyphoscoliosis, protrusio acetabuli and hindfoot deformity. Nervous system - dural ectasia hernias presenting with low back pain and symptoms akin to cauda equina syndrome or chronic postural headache due to CSF leakage Facial characteristics - maxillary/mandibular retrognathia, long face (dolichocephaly) and high, arched palate, enophthalmos, downslanting palpebral fissures and malar hypoplasia

Answer 98

Tuberous sclerosis Autosomal dominant due to TSC1 or TSC2 - hamartin or tuberin deficiency

Answer 99

Phenylketonuria - Autosomal recessive - Tested for in newborn heel prick - treat with diet low in phenylalanine, some medication available to decrease phenylalanine levels

Answer 100

>Two males are affected, either siblings or in successive generations No affected females Affected males are linked through unaffected females DMD is x linked

Answer 101

Suspect in child presenting with hx of infection/ fever and signs of shock e.g. tachycardia, hypotension, prolonged CRT or altered consciousness Ix - As part of sepsis 6 do Blood cultures and blood gas for lactate (also may see metabolic acidosis) - Also want to locate infection site: Urine culture, CXR, LP, Stool culture, PCR for Neisseria meningitides - FBC, CRP and baseline bloods - Blood glucose (often low in children with infection) Treatment - measure urine output - give high flow O2 - Give fluids 10ml/kg if CRT >3s - IV broad spectrum e.g. ceftriaxone or cefuroxime - If blood sugar <3 give 2ml/kg 10% dextrose Treat cause - meningitis - IV ceftriaxone - neonatal sepsis - IV benpen and gent - Abdo sepsis - metro + gent - Cellulitis - IV fluclox

Answer 102

Vesicoureteric reflux - can be primary or secondary due to obstruction in the urethral tract diagnose with US or voiding cystourethrogram - Often improves on own - Treat UTI with abx, if recurrent consider low dose prophylactic abx - surgery may be indicated if no improvement or secondary cause

Answer 103

- Familial short stature - Achondroplasia - Turners syndrome - Downs - Prader- willi - Failure to thrive - malnutrition, anorexia, crohns, UC, coeliac

Answer 104

In a boy: Mid-parental height (cm) = (Father's height + (Mother's height + 13)) divided by 2. In a girl: Mid-parental height (cm) = ((Father's height - 13) + Mother's height) divided by 2.

Answer 105

``` o Skin turgor o Moist membrane o Fontanelle o Sunken eyes o Capillary refil time ```

Answer 106

Staph aureus or strep pyogenes Risk factors include poor hygiene and skin conditions that lead to a break in the protective layers. Atopic eczema is a common risk factor; others include bites, trauma to the skin, scabies, chickenpox, burns and contact dermatitis. Non-bullous lesions usually start as tiny pustules or vesicles that evolve rapidly into honey-coloured crusted plaques that tend to be under 2 cm in diameter. Diagnosis is clinical, skin swab if unsure Mx: Keep the affected area(s) clean. Wash hands after touching affected area(s). Avoid sharing of towels and bathwater. Avoid scratching the affected area. Keep fingernails short and clean. Localised: - 1st line - Hydrogen peroxide 1% cream - If unsuitable - offer: fusidic acid 2%, alternative = mupirocin 2%. If widespread, bullous or resistant to above try oral fluclox

Answer 107

4 things to assess - gross motor - fine motor - speech - social Gross motor Newborn - Limbs flexed, symmetrical pattern, head lag on pulling up 6-8 weeks - Raise head when prone (tummy-time) 6-8 months - Sits without support (limit 9m) 8-9 months - crawl 10 months - stand independently, cruises around furniture 12 months - walks unsteadily with a broad gait, hands apart (limit 18m) 15 months - walks steadily 2.5 years - runs and jumps ``` Fine motor 6w - Follows moving object or face by turning the head (limit 3m) 4m - Reaches out for toys (limit 6m) 4-6m - Palmar grasp 7m - Transfers toys from one hand to another (limit 9m) 10m - Mature pincer grip (limit 12m) 16-18m - Makes marks with crayons 14m-4y - Brick building - Tower of three – 18 months - Tower of six – 2 years - Tower of eight or a train with four bricks – 2.5 years - Bridge (from a model) – 3 years 2-5ys - pencil skills - Line - 2yrs - Circle - 3 years - Cross - 3.5 years - Square - 4 years - Triangle - 5 years ``` Speech, hearing and language Newborn - Startles to loud noises 3-4m - Vocalises alone or when spoken to, coos and laughs “aa, aa” 7m - Turns to soft sounds out of sight, polysyllabic babble (“babababa, lalalalala”) 7-10m - Sounds used indiscriminately then discriminately to parents at 10 months e.g. “Dada, Mama” 12m - Two to three words other than ‘Dada’ or ‘Mama’, understands name 18m - 6-10 words, Is able to show two parts of the body “Where is your nose?” – Baby will point 20-24m - Joins two or more words to make simple phrases “Give me teddy” 2.5-3 yrs - Talks constantly in 3 – 4-word sentences, understands 2 joined commands “Push me fast Daddy” Social environmental and developmental 6w - Smiles (limit 8w) 6-8m - Puts food in their mouth 10-12m - Waves bye-bye, plays peek-a-boo 12m - Drinks from a cup with two hands 18m - Holds spoon and gets food safely to mouth 18-24m - Symbolic play (limit age 2-2.5yrs) 2yrs - toilet trained, pulls off some clothing 2.5-3yrs - Parallel play, interactive play evolving, takes turns

Answer 108

Height - Measure supine using board if <2yo - Measure standing up against backboard if >2yo - Ensure corner of eyes in line with middle of ear - measure to last complete mm Weight - Measure naked until 2 years old, then light clothing - record to last gram for neonates, <4kg - Record to last 100g if older or >4kg Head circumference - over ears - midway between hairline and eyebrows to occipital protuberance - measure to nearest mm 99% of children between the 0.4th – 99.6th centiles A sustained drop of 2 or more centiles for height or weight < 0.4th centile should be investigated Calculate mid parental centile - plot both heights, join with a line and that gives centile - >3 centiles below - investigate (9/10 within +/- 2) Gestational correction - do for babies <36+6 If 37 or greater treat as term and dont use premature section of growth chart At 42w switch to main section of growth chart - plot age in weeks with a dot and then do arrow back the number of weeks before 40 (term) - Continue with this for one year 32-36+6 - Continue for 2 years once <32w

Answer 109

Pityriasis Rosea

Answer 110

up to 10kg = 100ml/kg/day next 10kg = 50ml/kg/day per Kg after this = 20ml/kg/day

Answer 111

o Diuretics (furosemide) – reduce pulm congestion o ACE inhib (captopril) – reduce afterload o Digoxin – increases systolic contraction • Added later If needed o Adequate calorie intake

Answer 112

4 or below - oral glucose | 2.5 or below - IV dextrose

Answer 113

Otherwise normal platelets are destroyed by antibodies Can be associated with viral infection Low platelets lead to bleeding and bruising Ix - FBC - Platelet count - Blood film Mx - In majority of cases it improves on its own - most have complete recovery within 3-6m - Avoid contact sports - Avoid aspirin or ibuprofen - Avoid IM injections - Look out for spread of rash, large bruising or bleeding that won't stop In severe cases may need - Prednisolone - IV immunoglobulins - Blood transfusions if required - Platelet transfusions only work temporarily

Answer 114

Meningitis, HSP, ITP, non accidental injury, viral infection, leukaemia, von-Willebrands

Answer 115

Reduced urine output, blood in urine, Abdo pain, bruising Urgent referral to the paediatric renal unit for renal dialysis if required Antihypertensives if required Careful maintenance of fluid balance Blood transfusions if required

Answer 116

Therapeutic hypothermia

Answer 117

Consider bloods, ECG, echo, EEG Avoid dehydration Avoid missing meals Avoid standing up for long periods If feel hot/dizzy/clammy - sit or lie down and have some water

Answer 118

Is caused by rhesus incompatibility Occurs in second pregnancy where rhesus negative mother has previously been exposed to rhesus positive baby Autoantibodies attack the foetus' red blood cells leading to a haemolytic anaemia Use direct Coombs test to diagnose ``` Tests for differentials: Full blood count for haemoglobin and MCV Blood film Reticulocyte count Ferritin (low iron deficiency) B12 and folate Bilirubin (raised in haemolysis) Direct Coombs test (autoimmune haemolytic anaemia) Haemoglobin electrophoresis (haemoglobinopathies) ```

Answer 119

Think hereditary spherocytosis, beta or alpha thalassaemia,

Answer 120

Due to a short lingual frenulum - prevents extension of tongue and latching onto breast May not need treatment - monitor feeding In some need to do frenotomy

Answer 121

Occurs commonly after an episode of otitis media Present with reduced hearing, inattention, developmental delay Fullness and popping Hx of ear infections Loss of balance, clumsiness Due to Eustachian tube dysfunction Ix: Otoscopy - effusion of glue-like fluid behind an intact tympanic membrane in the absence of signs and symptoms of acute inflammation. Opacity of drum, loss of light reflex, retracted drum, fluid level Audiometry - hearing test - mild conductive hearing loss Assess development Mx Often resolves on own within several months - year Facing their child when speaking to them. Slowing their speech. Keeping speech clear. Increasing speech volume slightly. Turning off competing stimuli such as radio or TV. Encouraging daily reading, which helps language development. Autoinflation kits can be bought online to help open the eustachian tube Gromits if - persistent bilateral OME lasting three or more months. - hearing loss in the best ear of 25-30 dB or worse, averaged at 0.5, 1, 2 and 4 kHz. - social, educational or developmental difficulties

Answer 122

``` Hx of viral infection? Growth spurt? Fever Waking at night with pain Weight loss Anorexia Night sweats Fatigue Persistent pain Stiffness in the morning Swollen or red joint Recent trauma? Any rashes ``` Any other illness Any growth concerns

Answer 123

Caused by vitamin D or calcium deficiency Can be caused by reduced vit D intake, reduced sun exposure, IBD, liver disease and CKD Vitamin D is present in leafy green veg and meat Bone pain, proximal muscle wasting, increased risk of fragility fractures Thin soft skull bones, delayed closure of fontanelles, prominent frontal bone, protruding abdomen Bloods: low vit d, low ca, high ALP Treat with vitamin D supplements

Answer 124

Anterior knee pain worse on exercise | Treat with rest, NSAID, ICE