Paediatrics Flashcards

Question

Evaluation of poor weight gain

Answer 1

Hx - age of onset - medical hx - diet and feeding - >vomiting/diarrhoea/rumination - >picky eating, anorexia - >food preferences/avoidance - >dietary restrictions or beliefs - >anatomical abnormalities - psychosocial - >stressors (poverty) - >access to resources - >feeding skills and knowledge - >maternal health Exam - measurements - >consider velocity and proportionality - appearance - >lethargic - >wasted - >dehydration - caregiver-child interaction - behaviour and development

Answer 2

Breastfeeding - recommended as exclusive source for 6 months - by 1 year most infants predominately on solids - benefits - >antimicrobial effects (IgA, HMO) - >promotes GI growth and function - >reduces risk of acute illnesses - >protects against chronic disease (T1DM, IBD) - >reduces morbidity and mortality Compliment feeding - introduce compliment foods at 6 months - by then breast milk deficient in - >energy - >protein - >iron (plus vitamin C for absorption) - >zinc - >fat soluble vitamins (vitamin D) - cereals recommended first - > high in iron

Answer 3

Benign - familial short stature - >length/height velocity normal - >weight/length normal - >bone age matches chronological age - constitutional delay of growth and puberty - >normal birth size - >at 3-6 months growth rate declines below normal - >by 3-4 years growth rate is normal, but remain below 3rd gentile for height - >puberty and pubertal growth spurt is delayed - >growth spurt prolonged so that adult height within normal range - >bone age matches expected age for height - idiopathic short stature - >growth velocity normal - >no obvious cause - >thought to be polygenic or epigenetic cause - small for GA with catch up growth - >reach normal range within 2 years Pathological (SMUDGE) - steroids - metabolic disorders - >diabetes - undernutrition - disease (any) - genetic disorder - >turners - >SHOX syndrome - >noonans - endocrine disease - >hypothyroidism - >precocious puberty - >cushings - >growth hormone deficiency

Answer 4

Activity 0 = limp 1 = some flexion 2 = active Pulse 0 = absent 1 = <100 2 = >100 Grimace 0 = none 1 = grimace 2 = sneeze/cough Appearance 0 = blue/pale 1 = acrocyanosis 2 = pink Respiratory 0 = absent 1 = irregular/slow 2 = crying/good performed at 1 and 5 mins <3 = concerning >7 = reassuring

Answer 5

Viral - supportive treatment only - >fluids - >paracetamol - >NSAIDs - >lozenges/honey/rest Strep - supportive treatment for everyone - >return if symptoms >7 days or new symptoms develop - shared decision making - >symptoms usually last 7 days - >antibiotics only shorten symptoms by 1 day - >antibiotics reduce risk of complications - >antibiotics have their own risks - antibiotics recommended for high risk groups - >ATSI - >scarlett fever - >rheumatic heart disease - >severe strep pharyngitis - antibiotic therapy - >oral phenoxymethylpenicilin for 10 days

Answer 6

GRASP FATSO Post streptococcal glomerulonephritis - more common in kids and developing world - due to immune complex disease - >complement activation and inflammation - pathology - >light microscopy = proliferative glomerulonephritis - >IF = granular IgG and C3 deposition (starry sky) - >electron microscopy = sub epithelial humps - clinical - >asymptomatic to nephritic syndrome Rheumatic fever - developing world/low SES - latent period of approx 3 weeks Scarlet fever - scarlatiniform rash - >delayed hypersensitivity to strep exotoxin - >strawberry tongue - >circumoral pallor - >treatment as usual for pharyngitis Post streptococcal reactive arthritis - unsure if it is seperate from polyarthritis of ARF - >occurs earlier - >less responsive to NSAIDs - >less association with carditis PANDAS - paediatric autoimmune neuropsychiatric disorder associated with group A strep - controversial existence/autoimmune basis - temporal association between GAS infection and - >tic disorder - >OCD Fasciitis - pathogenesis - >usually when predisposing trauma has occurred - >due to haematogenous spread - >spreads along fascia plane (poor blood supply) - >surrounding muscle spared (good blood supply) - presentation - >abrupt onset pain - >erythema of overlying skin (may also be unaffected) - >bullae - >systemically unwell (can become septic) - diagnosis - >surgical exploration with gram stain is definitive - >CT best imaging (shows gas in soft tissue plane) Abscess -usually polymicrobial, including GAS Streptococcal toxic shock syndrome - rare complication - shock with multi-organ failure - due to inflam cytokines and increased cap permeability Sinusitis - common complication - direct extension from nasopharynx along ostiomeatal complex Otitis media - common complication - due to direct extension along eustachian tube

Answer 7

Epidemiology - both under school age - can occur in endemics Aetiology - virology - >multiple serotypes of enterovirus species - >most common enterovirus species is enterovirus A - >most common group is coxsackie A and enterovirus Pathophys - transmission - >as usual for enterovirus - >can be from ingestion of vesicle secretions HFMD presentation - hx - >sore throat/poor feeding - >fever - >usually no systemic features - exam - oral endathem and/or exanthem - oral endanthem - >anywhere in anterior half of mouth including tongue - >begins as erythematous macules/papules - >becomes small vesicles with erythematous halo - >vesicles rupture forming small ulcers - exanthem - >can occur anywhere (face and trunk uncommon) - >same appearance as endanthem - >non pruitic - >may or may not be painful Herpangina presentation - hx - >abrupt onset - >high fevers - >systemic features more likely (malaise/headache etc) - >sore throat/poor feeding - exam - >oral endanthem - >similar appearance to HFMD but posterior half of mouth - >no exanthem Investigations - usually not necessary - >PCR - >viral culture Treatment - prevention - >hand hygiene after blisters, cough/sneeze, toileting - >avoid sharing items (cutlery, toothbrush etc) - >avoid school/day care until blisters dry - >note virus shed in stools for weeks/months after - >safety net (blisters last for about a week) - public health - >not notifiable - >consider informing school/day care - supportive - >fluids/electrolytes - >analgesia (NSAIDs/paracetamol) - >don't pop blisters

Answer 8

Circulation - highly oxygenated blood passes from placenta through umbilical vein - >largely by passes liver due to ductus venosus - joins poorly oxygenated blood in IVC - enters right atrium - >shunted across FO more than poorly oxygenated blood due to streaming effects - majority of CO is from right ventricle - >only 10% of total CO goes to lungs - >majority of RVO goes through ductus arteriosus - ductus joins aorta at isthmus (after left subclavian) - >means that coronary/brain Pa02 is high - placenta is very low resistance circulation - >majority of descending aorta flows through umbilical arteries (from internal iliacs) - >makes whole systemic circulation low resistance - lungs filled with fluid - >making pulmonary circulation high resistance Oxygenation - very low PO2 in-utero - >approx 55mmHg in umbilical vein - >approx 15mmHg in umbilical arteries - adequate tissue oxygenation due to - >high O2 affinity of fetal Hb - >decreased O2 consumption (thermoregulation, respiratory effort, digestion decreased) - >preferential delivery of high P02 blood to vital organs (ductus venosus, carotids/coronarys before isthmus) - low PO2 maintains fetal circulation dynamics - >causes pulmonary vasoconstriction - >causing high pulmonary resistance - >promotes shunting through PO and DA

Answer 9

Alveolar fluid clearance - eNAC - >allows secretion of water during gestation (promoting lung growth and development) - >increase in catecholamines late in gestation causes eNAC to absorb Na and draw water out of alveoli - >this increases at birth due to high PO2 - Initial breaths - >inspiratory and expiratory pressures during initial breaths are far higher than normal - >drives alveolar fluid into interstitial - thoracic squeeze - >compression of chest during birth squeezes fluid out - >minimal contribution Lung expansion - air movement begins with decreasing intrathoracic pressure during first breaths - lung expansion promotes surfactant secretion - >decreases surface tension (P=2T/R) Circulatory pressure gradient - placenta is clamped - >increases systemic resistance - lung expands - >decreasing pulmonary vascular resistance - result - >DA begins to shunt left to right - >increases pulmonary blood flow - result - >increased oxygen saturation as some blood double-dips - >increased stroke volume increases cerebral perfusion Change in shunts - close FO - >high systemic/low pulmonary resistance - >high left atrial/low right atrial pressures - close DA - >high oxygen saturation - >decrease in prostaglandins

Answer 10

BICEP Blockage of airways - congenital airway malformation - >bilateral choanal atresia - >robin sequence (glossoptosis) - mucus - meconium Impaired lung function - external - >pneumothorax - >hydros fetalis - intrinsic - >RDS - >TTN - >neonatal pneumonia - pulmonary hypoplasia - >congenital diaphragmatic hernia - >oligohydramnios - >congenital anomaly of kidney and ureters Congenital heart disease poor respiratory Effort - CNS depression - >neonatal encephalopathy - >drug exposure - Neuromuscular disorder Persistant pulmonary hypertension of newborn - pulmonary hypoplasia - >cross sectional area of pulmonary vasculature reduced - pulmonary dysplasia - >abnormal pulmonary vasculature muscle and ECM - >due to meconium aspiration syndrome - maladaptation - >vasoconstriction prevents decrease in PVR - >due to RDS, pneumonia, asphyxia

Answer 11

When - healthy baby - >at term - >good tone - >breathing or crying - within 48 hours - >always before discharge Patient centred - seek parental consent - consider cultural needs - discuss - >purpose - >process - >limitations - ask about concerns Hx - review delivery - >gestational age - >mode of delivery - >complications - >APGARs - >need for resuscitation - review current pregnancy - >complications - >screening tests (imaging and bloods) - >risk factors for sepsis - review past pregnancies - >congenital anomalies - >still births and SUDI - >genetic/syndromic conditions - maternal health - >blood group - >illnesses prior to and during pregnancy - >medications, alcohol, drugs - family hx - >genetic and congenital conditions - >still births and SIDS - >psychosocial dynamics - since birth - >vitals - >measurements - >medications - >feeding Exam -top to toe

Answer 12

Smile Focus and track with eyes Startle to loud noise

Answer 13

Vitamin K - recommended for all shortly after birth - >prophylaxis for hemorrhagic disease of newborn - approx 1mg IM Hep B - vaccine offered to all within 7 days - IgG offered when mum HBVsAg+ - refusal when mum HBVsAg+ - >counselling - >report to child services Umbilicus - standard infection control only - >usual hand hygiene - >clamp 2cm from skin - >wash with soap and water - >expose to air (above nappy) - detachment - >usually at 1 week Review feeding Glucose - not routinely measured - indication for measurement - >pre term - >LGA or SGA - >diabetic mother - >family hx genetic hypoglycaemia Newborn screening - blood spot - SWISH Pulse oximetry for CHD - approx 30% with critical CHD missed on baby check - positive screen - >any limb <90% - >any limb <95% on three occasions - >pre-ductal 3% higher than post ductal - followed up with usual cyanosis evaluation Jaundice - all babies - >review risk factors - >visual or transcutaneous (every 8-12 hrs) Patient education - complete blue book (my personal health record) - normal newborn care - >sleep - >feeding - >urine and stools (frequency, colour, meconium passing) - >growth - >umbilical cord care - >detection of jaundice - health promotion - >injury prevention - >illness warning signs - >written information of SUDI - >breast feeding advocacy - >immunisation schedule - information on support agencies - arrange follow up - >one week

Answer 14

What - 25 medical conditions screened for - >Harry Potter MAGIC - biochemical test for screening - DNA testing - >only performed if positive screen - >for CF and fatty acid oxidation disorder Why - About 1/1000 lead to diagnosis - False negative rate is 1/100,000 - characteristic of diseases - >can be detected early - >result in serious illness or developmental delay - >early intervention is effective Offered - to everyone - after 2-3 days - requires signed consent - refusal requires signature Collection - prick heel with lancet - discard first drop - fill three circles on screening card - send to central laboratory Results - after approx 2 days - not contacted if results are normal - if results abnormal - >contacted - >more blood collected for re-testing - if re-testing is positive - >referred to specialist Storage - in a secure facility - 2 years minimum (auditing and quality) - after 2 years - >request card to be returned/destroyed - >if not, stored for 18 years (when content lapses) - access to card - >lab for further testing - >anonymised research - >court order or coroner

Answer 15

Harry Potter MAGIC hypothyroidism (primary congenital) - epidemiology - >40 births/yr in NSW - aetiology - >dysgenesis (absence/abnormal thyroid gland) - pathophys - >growth retardation - >intellectual disability - management - >daily thyroxine phenylketonuria (PKU) - epidemiology - >10 births/yr in NSW - aetiology - >recessively inherited - >deficiency in phenylalanine hydroxylase - pathophys - >cannot break down amino acid phenylalanine - >severe intellectual disability - management - >low protein diet medium chain acylCoa dehydrogenase deficiency - epidemiology - >6 births/yr in NSW - aetiology - >inability to break down fat - pathophys - >coma and liver failure when seriously ill or fasted - >results in intellectual disability or death - management - >avoid fasting - >IV glucose when unwell adrenal hyperplasia (congenital) - epidemiology - >6 births/yr in NSW - aetiology - >genetic defect - >deficient in enzyme involved in cortisol biosynthesis - pathophys - >low cortisol - >increased ACTH - >adrenal gland hyperplasia - >high androgens and mineralocorticoids - >disordered regulation of metabolism, salt, response to infection, sex characteristics - management - >hormone replacement - >salt supplementation galactocaemia - epidemiology - >3 births/yr in NSW - aetiology - >deficiency in Gal-1-PUT - pathophys - >build up in galactose in blood - >liver failure and sepsis (potentially lethal) - >cirrhosis, renal tubular acidosis, cataracts, ID - management - >low galactose diet inborn errors of metabolism (other rare) -collectively account for approximately 20 births/year in NSW cystic fibrosis

Answer 16

What -audiology screening for all newborns Why - incidence of permanent severe bilateral hearing loss - >80 births/year in NSW - intervention before 6 months - >prevents poor health, social and cognitive impairement Process - when - >ideally first few days of life - >up to 3 months - requires consent - >refusal documented in blue book - screening test - >automated auditory brainstem response (AABR) - >baby asleep or resting - >electrodes on head - >sound introduced through earphones - >waveform detected and compared to template - results available immediately - >parents informed of results - if negative - >routine surveillance - if positive - >second screen conducted to confirm result - still positive - >audiology test at john hunter, westmead or SCH - if diagnosed, referral to australia hearing - >different commonwealth funded interventions offered - document screening in blue book

Answer 17

epidemiology -incidence increases with decreasing GA pathogenesis - vertical transmission (early onset) - >maternal genital tract - >contaminated amniotic fluid - horizontal transmission (late onset) - >contact with care provider and environment - >forceps and electrodes - >disruption of skin/mucosa (eg. canula) aetiology - microbio - >GBS - >E. coli - >S. aureus (late onset sepsis) - >coagulase negative staph (premature infants) - >listeria monocytogenes (rare) - >herpes - risk factors - >maternal GBS infection - >chorioamnionitis - >intrapartum maternal temp >38 - >premature - >membrane rupture >18hrs - >metabolic disturbance (reduces immune function)

Answer 18

GA - 34-37 = late pre-term - 32-34 = moderate pre-term - 28-32 = early pre-term - <28 = very early pre-term Weight - normal = 2.5-4 - low = <2.5 - very low = <1.5 - extremely low = <1

Answer 19

Mortality and morbidity rates increase with decreasing GA and birth weight Short-Term =GRINCHES - glucose - respiratory - >RDS - >apnea of prematurity - intraventricular haemorrhage - NEC - cardiovascular - >PDA - >BP - hypothermia - eyes (retinopathy of prematurity) - sepsis Medium-Term (infancy/early childhood) = BANGERS - bronchopulmonary dysplasia - asthma hospitalisations - neurodevelopmental - >developmental delay - >cognitive and social impairment - >psychiatric illness - >cerebral palsy - growth impairment - enteritis - respiratory infections - SIDS Long-term = KIILO - kidney disease - insulin resistance - IHD - lung disease (chronic of prematurity) - obesity

Answer 20

Epidemiology - over 90% of incidences RDS occur in extreme pre-term - still significant risk for late pre-term Aetiology - surfactant production begins GA 20 - alveolar budding (saccular stage) begins GA 24 Pathophys - surfactant - >phospholipids (detergent) and proteins (reabsorption) - >produced by type II alveolar cells - >production begins GA 20 - >reduces surface tension (P=2T/R) - decrease in quantity/quality of surfactant - >atelectasis->decreased compliance/ventilation - pulmonary oedema and inflammation - >airway damage due to high pressures - >reduced lung expansion/eNAC expression = reduces alveolar fluid clearance - >worsens compliance/ventilation - >inactivates surfactant - shunting - >atelectasis/vasoconstriction = high pulmonary pressures - >right to left shunting across FO/DA - hypoxaemia - >due to poor ventilation/shunting Clinical manifestations - almost always preterm infant - presents in first minutes to hrs of life - respiratory distress - cyanosis (due shunting/hypoxaemia) - decreased urine output - peripheral oedema - often improves over 48-72hrs with surfactant production Investigations - ABG - >hypoxaemia - >hypercarbia and respiratory acidosis - >hyponatraemia (fluid retention) - CXR - >ground glass (atelectasis) - >air bronchograms (pulmonary oedema)

Answer 21

Antenatal corticosteroids - MOA - >accelerate development of type I and II alveolar cells - >surfactant production - >architectural maturation - >up regulation of eNAC = fluid resorption - >up regulation B1 receptors = surfactant release - Indication - >risk of at least moderate pre-term birth in next 7 days - >diabetes/gestational diabetes at risk of pre-term birth in next 7 days - >multiple pregnancies at risk of pre-term birth in next 7 days - Dose - >2x12mg betamethazone IM 24hrs apart - >4x6mg dexamethasone IM 12hrs apart - Timing - >greatest effect 2-7 days prior to birth - >still indicated if delivery expected within 24hrs Management - CPAP - >preferred initial intervention - >at risk or confirmed RDS - >associated with long term morbidity - Consider caffeine therapy - >indicated extremely low birth weight - >prevents apnea of prematurity - Intubation - >indications = pH <7.2/FiO2>0.4/apnea - >associated with higher mortality and BPD - >complications = placement in right main/air leak - Exogenous surfactant - >given with intubation - >reduces mortality and morbidity - Supportive care (reduces caloric needs/O2 consumption) - >maintain thermonneutral temp - >monitor BP - >suspect PDA - >maintain slight negative fluid balance - >avoid diuretics - >adequate nutrition (may require enteral)

Answer 22

Epidemiology -5/1000 births Aetiology - >prematurity - >caesarian birth Pathophys - >inadequate loss of alveolar fluid - >decreased compliance/ventilation/air trapping Clinical manifestations - >onset usually immediately after birth - >respiratory distress - >chest clear to auscultation - >large chest Investigations - rarely needed - ABG - >mild hypoxaemia - >hypercarbia with respiratory acidosis - CXR - >increased lung volumes - >cardiomegaly - >increased vascular markings - >fluid in interlobar fissures - >pleural effusion Management - >usually resolves within 48hrs - >CPAP - >O2 supplementation - >consider ddx's

Answer 23

Peripheral cyanosis - high O2 extraction due to slow capillary flow - >acrocyanosis (cold and vasoconstriction) - >sepsis - >shock - >venous thrombosis - >polycythaemia Normal A-a gradient (hypoventilation) - Upper airway obstruction - >congenital (laryngomalacia/choanal atresia/micrognathia) - >acquired (croup/epiglotitis/bacterial tracheitis/anaphylaxis) - Neurological - >neonatal encephalopathy - >intraventricular haemorrhage - >seizures - >drug exposure - >neuromuscular disorder - Apnea - >prematurity - >metabolic disorders High A-a gradient hypoxic hypoxia - Impaired alveolar diffusion (pulmonary oedema) - >pulmonary parenchymal disease - >sepsis (ARDS) - >heart failure (CCHD/AVM) - V/Q mismatch - >RDS - >TTN - >pneumonia - >pneumothorax - >pleural effusion - >meconium aspiration syndrome - Right to left shunting - >CCHD - >PPHN Anaemic hypoxia - Haemoglobinopathies - >methaemoglobinaemia (ferric haem iron) most common - Polycythemia - Carbon monoxide poisoning (smoke inhalation) Histotoxic hypoxia -Cyanide poisening (burning plastics/wool/rubber) Stagnant hypoxia -obstructive left CHD

Answer 24

Epidemiology - CHD = approx 10/1,000 births - CCH = 15% of CHD - critical CHD = 25% of CHD Aetiology - risk factors - >prematurity - >family hx - >genetic disorders - >maternal illness (diabetes/HTN/obesity/thyroid disorder) - >maternal exposure (drugs/alcohol/smoking/phenytoin) - >assisted reproductive technology - >in utero infection (influenza/TORCH) Pathogenesis (5 T's and heart failure) - Transposition of great arteries - TOF - Tricuspid valve abnormalities - >tricuspid atresia - >tricuspid stenosis - >epstein anomaly - Truncus arteriosus - Total anomalous pulmonary venous connection - Heart failure (CHIC) - >coarctation of aorta - >hypoplastic left heart syndrome - >interrupted aortic arch - >critical aortic valve stenosis

Answer 25

TOF - VSD - >systemic/pulmonary mixing - over-riding aorta - >mixed CO - RV hypertrophy - stenotic pulmonary trunk - >decreased flow Tricuspid atresia - no connect between RA/RV - right to left shunt through PFO - may have VSD/transposition of arteries Tricuspid stenosis - hypoplastic RV - right to left shunting through PFO Epstein anomaly - displacement tricuspid leaflets into RV - >RV outflow tract obstruction - tricuspid incompetence - >large RA - right to left shunt across PFO TGA - aorta from RV/pulmonary trunk from LV - >two parallel circuits - >deoxy blood to systemic/RV & oxy blood to pulmonary/LV - survival due to mixing - >PFO/VSD/PDA Truncus arteriosus - single outflow veseels - >gives rise to aorta/pulmonary/coronary arteries - always VSD - >mixing Total anomalous pulmonary venous return - 4 pulmonary veins don't drain to RA - >connection to vena cava/coronary sinus/portal vein - >mixing - right to left shunt across PFO Hypoplastic left heart syndrome - varying degrees of - >aortic valve stenosis/atresia - >mitral valve stenosis/atresia - >ascending aorta hypoplasia - >LV hypertrophy/hypoplasia - PDA - >supplies systemic circulation - >subclavian/carotids/coronary via retrograde flow Coarctation of the aorta - marked stenosis of aorta - >distal to left subclavian - >proximal to isthmus - PDA supplies lower systemic circulation Interrupted arch aorta - complete interruption - most commonly between LSub and LCar - ductus continues as descending aorta - >mixed Critical aortic valve stenosis - bicuspid aortic valve - in utero - >RVCO low and LVCO through DA - DA closes - >pulmonary flow/LA return increased - >if LV can't fill or empty = failure/cyanosis

Answer 26

Hx - review risk factors - family hx - >CHD - >arrhythmias (long GT syndrome) - >SIDs/childhood death - infants - >failure to thrive - >poor feeding - >lethargy and stopping feeds early - >resp distress during feeds - >irritability especially during feeds - >sweating during feeds - children - >exercise intolerance - >chest pain - > exertional syncope/syncope with chest pain - >tachypnoea/dyspnoea - >fever (cardiac path due to systemic disease) Exam - pulse - >tachycardia/bradycardia - praecordium - >hyperkinetic/forceful apical impulse - >parasternal heave - >thrill - pathologic added sounds - >single or fixed splitting of S2 - >clicks - >S3 gallop - >friction rub - pathologic murmurs - >holosytolic or diastolic - >high grade - >harsh or blowing quality - >louder when seated upright - additional - >tachypnoea - >crackles - >hepatomegaly - >syndromic features

Answer 27

Shock - >obstructive left heart aetiologies - >worsens with ductus closure Ductal dependent cyanosis - >right heart obstruction (lose retrograde flow to lungs) - >left heart obstruction (stagnant hypoxia) Non ductal dependent cyanosis - >TOF - >truncus arteriosus - >total anomalous pulmonary venous return Differential cyanosis - >coarctation of the aorta - >interrupted aortic arch Tachypnoea - drop in PVR over first days = pulmonary oedema - >truncus arterious - >total anomalous venous return - >PDA - >VSD

Answer 28

``` Pulse oximetry -confirm cyanosis -pre and post ductal ->difference >3% is sig. ECG -may not be atypical -normal ->right axis deviation ->RV hypertrophy Glucose ->apnea ``` ``` ABG -PaO2 -PaCO2 ->high suggest pulmonary aetiology -low pH suggests shock FBC -high haematocrit = polycythemia -abnormal WCC = sepsis ``` CXR - evidence of lung pathology - >TTN - >RDS - >congenital lung abnormality - heart size - >cardiomegaly with left sided obstruction - heart shape - >boot shape = TOF - >egg on a string = TGA - pulmonary vascular markings - >reduced in CCHD/PPHN - >increased in left sided obstruction - right sided arch of aorta - >TOF - >truncus arteriosus Echo - definitive diagnosis - >abnormal anatomy or flow Consider hyperoxia test if no echo - measure before O2 administration - >PaO2 in right radial or - >SpO2 - 10 mins of 100% O2 - pulmonary disease if - >more than 150mmHg PaO2 - >greater than 10% increase SpO2 Sepsis screen - important and common ddx for cyanosis - blood cultures - urinalysis and urine culture

Answer 29

Support airway and breathing Specific pathway determined by ddx - undifferentiated shock - sepsis - cardiac specific - >PGE2 (alprostadil) - >maintains patent PDA - >can cause apnea/NEC/hypotension/tachycardia - >cardiac catheterisation (ballooning of obstructions)

Answer 30

Initial stabilisations - level of consciousness - airway patency - breathing movements - circulation - >heart rate, pulses and perfusion - >gain IV access Hx - Description of event - >duration - >choking/gagging - >breathing or attempting to breathe - >level of consciousness - >level of tone - >movements - Description of prior events - >awake or asleep and positioning - >relation to feeding - >availability of choking hazards - >illness in prior days - >sick contacts Exam - general appearance - >level of consciousness - >tone - >movements - >crying/noises - >breathing efforts - resp exam - >resp rate - >distress non specific - >wheeze/stridor more specific - >asymmetrical auscultation findings - cardio exam - >pulses in all four limbs - >radio-radial/radio-femoral delay - >brachial and popliteal BP (coarctation) - >added sounds or pathological murmur

Answer 31

- Review labour - >GA - >complications/APGARs/resuscitation - >risk factors for sepsis - >meconium staining (PPHN) - >anaesthetic exposure (resp depression) - >caesarian (TTN) - Review pregnancy - >screening results - >oligohydramnios (BPD) - >polyhydramnios (tracheo-oesophageal fistula) - Maternal risk factors - >diabetes (CCHD/polycythaemia/TTN/RDS/hypoglycaemia) - >asthma (TTN) - >opioid use (respiratory depression - >HTN (IGR/polycythaemia/hypoglycaemia) - CHD risk factors - Family hx - >congenital diseases - >haemoglobinopathies - >RDS - >SIDS or serious childhood diseases

Answer 32

Epidemiology - incidence - >over half of all new borns - >approx 80% of pre term - incidence of severe - >less than 1/10,000 - risk factors - >male - >asian - >maternal diabetes - >premature - >low birth weight - >decreased caloric intake/weight loss - >breast feeding Aetiology - causes - >physiologic - >unconjugated (PREM PEACHES) - >conjugated/cholestasis (HABIT MAP) Pathophys (physiological) - haem -> iron + biliverdin - biliverdin -> bilirubin (bound to albumin) - taken up by hepatocyte - UGT1A1 conjugates bilirubin + glucuronic acid - >water insoluble - active secretion into bile - unable to be absorbed across intestinal epithelium - in adult - >reduced to urobilin by colonic bacteria - >urobilinogen (urine) - >stercobilinogen (faeces) - in newborn - >increased enterohepatic circulation - >sterile gut - >more beta glucuronidase (un-conjugates) Clinical manifestions - physiological jaundice - >appears after 2-4 days (later in asians) - >peaks lower than 150micromol/L - >formula feed = lasts 1 week - >breast feed = lasts 2 weeks - pathological jaundice - >within 24hrs - >lasting more than 2 weeks - >TB/TcB > 95th centile - >ABE/CBE

Answer 33

Physiological - >more RBCs - >shorter RBC life span (approx 80 days) - >UGT1A1 activity very low until about 2 weeks - >sterile gut Unconjugated (SPERM BEACH) - Sepsis - Polycythemia - Enterohepatic circulation - >meconium ileus - >hirschprungs - >pyloric stenosis - >intestinal stenosis/atresia - Rh or ABO incompatibility - Metabolic - >hypothyroidism - >galactocaemia - >maternal diabetes - Breast feeding - >breast milk jaundice - >lactation failure jaundice - Extravasation - >cephalohaematoma - >internal haemorrhage - >large haemangiomas - Albumin binding - >antibiotics - >asphyxia - >acidosis - Conjugation - >crigler Najjar 1 and 2 - >gilberts - Haemolytic anaemia - >haemoglobinopathies - >G6PD - >spherocytosis Conjugated (HABIT MAP) - Hepatocellular infections - >Hep A/B - >CMV - >rubella - Alagile syndrome - Biliary atresia - Idiopathic neonatal hepatitis - Total parenteral nutrition - Metabolic - >galactosaemiia - Alpha 1 anti trypsin deficiency - Progressive familial intrahepatic cholestasis

Answer 34

Epidemiology - acute bilirubin encephalopathy - >up to 10% with >30mg/dL - chronic bilirubin encephalopathy - >up to 25% with >30mg/dL - >almost all with >35mg/dL Aetiology -SPERM BEACH Pathophys - high levels of unbound unconjugated bilirubin - >cross BBB - taken up by basal ganglia and sub cortical nuclei - neurological injury - >impairs mitochondrial function - spectrum of disease affecting - >visuocortical pathways - >sensorineural hearing - >proprioception - >speech and language ABE - early - >lethargy - >poor suck - >hypotonia/expressionless facies - >high pitched cry - intermediate - >febrile - >irritable/jittery - >variable tone - >high pitched cry - advanced - >apnoea - >seizure - >stupor - >retrocollis/opisthotonos CBE - kernicterus - >pathological hallmark - >yellowing of basal ganglia/hippocampus/cerebellum - choreoathetoid CP - >chorea - >ballismus - >tremor - >dystonia - sensorineural hearing loss - upward gaze paralysis - enamel dysplasia

Answer 35

Screening in neonate -transcutaneous >95th centile Serum bilirubin -total >95th centile Direct <1mg/dL or <20% of total - FBC - >WCC derangement = sepsis - >RCC = anaemia - >haematocrit = polycythaemia - >reticulocyte count = haemolysis - >smear = abnormal RBC morphology - Coombs test +ive - >check maternal/newborn blood compatibility - >mother O and newborn A or B - >newborn Rh+ and mother Rh- - >consider minor blood groups - G6PD screening - LFTs - >normal = criglar bajar/gilberts - >abnormal = hepatocellular infection ``` Direct >1mg/dL or >20% of total -FBC ->WCC derangement = sepsis ->low WCC/low platelets = portal HTN -Glucose, bicarb, electrolytes ->deranged in metabolic disorders -LFTs -Albumin Consider -Ammonia/plasma/urine amino acids ->metabolic screen -TSH -Alpha 1 antitrypsin -urinalysis and urine culture ->source of infection Imaging -abdo ultrasound ->abnormal organs/hepatbiliary tract Still undifferentiated -Liver biopsy for biliary atresia ```

Answer 36

Presenting complain - Age of onset - Appetite - Vomiting - >BO/pyloric stenosis/metabolic disorder - Stools - >clay colour = cholestasis - >delayed = cystic fibrosis/hypothyroidism - >diarrhoea= infection/PFIC/metabolic disorder - Dark urine - Triggers - >illness - >medications Antenatal - ultrasound - >choledochal cysts - maternal infections - >hepatocellular infection - intrahepatic cholestasis of pregnancy - >PFIC - fatty liver of pregnancy - >fatty acid metabolism error Perinatal - Measurements - >indicator of severity Antenatal -Newborn screening results Family hx - newborn jaundice - haemolytic disease - liver disease Dietary hx - exposure to milk (galactosaemia) - flava beans (G6PD) Immunization status - Hepatitis - TORCH - Sepsis Exam - General appearance - >septic - >pallor/plethora - >jaundice - >scleral icterus (absent in carotenaemia) - Skin - >bruising/petechiae/haemorrhage/haematoma - Facies - >syndromic (allagile) - Cardiac - >CHD = biliary atresia/allagile - GI - >abdominal wall veins/ascites = portal HTN - >hepatomegaly = cholestasis - >splenomegaly = portal HTN/haemolysis

Answer 37

Unconjugated - feeding/hydration - phototherapy - >above 95th centile - >keep hydrated and cover eyes - >increased risk of epilepsy in males - exchange transfusion - >bilirubin >25mg/dL - >signs of ABE - >refractory to phototherapy - >serious underlying aetiology - albumin infusion - >consider during exchange transfusion - IVIg - >isoimmune haemolytic disease Conjugated - phototherapy contraindicated (bronze baby syndrome) - exchange transfusions not indicated - treatment aetiology specific Physiological - no treatment indicated - consider withdrawing from breastfeeding for 24-48hrs

Answer 38

Epidemiology - almost all cases occur in - >early preterm or lower GA - >very low birth weight - mortality rate is approx 1/3 - >higher in lower GA Pathophys - immature gut and immune system - >ineffective mucosal barrier allows bacterial invasion - >slow transit allows bacterial overgrowth - >high gastric pH - >lower levels protective enzymes and IgA - microbial dysbiosis and disruption of mucosal barrier - >antibiotics - >non-human milk and formula - >hyperosmolar medications - >H2 antagonists - >circulatory compromise - >severe anaemia - exaggerated innate immune response to stimuli - >inflammation - >apoptosis, infarction and necrosis of bowel Clinical manifestations - approx 2-3 weeks - >presents earlier with later GA - abdo - >distension - >tenderness - >bilious vomiting/gastric aspirate - >decreased feeding tolerance - >diarrhoea/haematochezia - non specific - >lethargy - >apnea - >resp distress - >temperature instability - >bacteraemia

Answer 39

Investigations - FBC - >neutropenia - >thrombocytopenia - lactate and pH - >metabolic acidosis - electrolytes - >hyponatraemia - sepsis screen - >blood cultures - >CF cultures - abdo xray (insensitive) - >dilated loops of bowel = ileus - >pneumatosus intestinalis = gas in bowel wall - >pneumoperitoneum = perforation - >portal venous gas = bacterial gas Medical management - supportive care - >bowel rest for 2 weeks - >gastric decompression - >TPN and fluid replacement - broad spectrum antibiotics - >amoxicillin, gentamycin, metronidazole - >2 week course - serial monitoring - >physical exam - >lab investigations - >abdo xrays Surgical management - indications - >perforation - >high risk of perforation - procedures - >bedside primary peritoneal drainage - >laparotomy w bowel resection and stoma

Answer 40

Epidemiology - incidence increases with maternal age - >1/300 @35 - >1/100 @40 - risk factors - >maternal age Aetiology - genetic abnormality - >almost of all have extra chromosome 21 - >some have balanced translocations - >rarely mosaic - heretability - >usually occurs as denovo error - >risk is increased in subsequent pregnancies Pathophys -majority non dysfunction error during meiosis of oocyte

Answer 41

Newborn exam (FIFTH SUNBEAM) - >fifth middle phalanx shortened - >iliac wings hypoplastic - >flexible joints - >transverse palmar crease - >sandal gap - >up slanting palbebral fissures - >neck skin - >brachycephaly - >epicanthal folds - >abnormal auricles - >moro absent Other manifestations and complications - fetal demise in approx 30% after definitive testing - ID - >almost all, to varying degrees - >most expressive language deficit - Development - >gross motor takes approximately twice as long - >delay in other domains - Growth - >low weight, height, HC - >early and blunted pubertal growth spurt - >obesity common - Psychiatric - >depression and anxiety - >ADHD - >alzheimers - Neck - >atlantoaxial instability - Eyes - >congenital cataracts - >strabismus/nystagmus - Ears - >hearing loss due to otitis media - Cardioresp - >congenital heart disease (mainly septal defects) - >pulmonary HTN - >sleep apnea - GI - >duodenal atresia - >hirschprungs - Endocrine - >diabetes - >thyroid disease - Haematological - >newborn polycythaemia - >transient myeloproliferative disorder - >ALL - Fertility - >most women fertile - >most males infertile

Answer 42

Epidemiology -1/5,000 Aetiology - almost always sporadic - 45, X (most common) - >entire X chromosome missing (monosomy X) - >sex chromosome non disjunction during meiosis - >oocyte combines with sperm missing x chromosome - 45, X mosaicism - >error following conception (non disjunction in mitosis) - >45 X + 46 XX (47 cell line dies off) - X chromosome abnormalities (+/- mosaicism) - >various deletions and anomalies Pathophys - genes on tips of X chromosome account for syndrome - >single copy SHOX = short stature - >haploinsufficiency of X genes = ovarian failure - >qX abnormalities = cardiac disease Clinical manifestations - Neonate (10%) - >severe CCD - >congenital abnormality of kidney (horseshoe) - Childhood (20%) - >lymphoedema of hands and feet - >neck webbing - >shield chest (wide spaced nipples) - >short stature - Puberty (majority) - >primary amenorrhoea - >ovarian failure with delayed pubarche - >madelung deformity of wrist - >cubitus valgus - >cardiac disease (aortic valve/dissection) - >thyroid/liver/HTN/hearing Investigations - Diagnosis - >peripheral white cell karyotype - Additional - >xray for bone age - >FSH and AMH (low levels support ovarian failure) - >ultrasound (ovarian streak morphology) - >conductive/sensorineural hearing loss - >renal ultrasound + EUCs - >cardiac MRI/MRA - Complications screen - >TSH = autoimmune thyroid disease - >LFTs = turners hepatitis - >glucose = diabetes - >lipids = dyslipidaemia - >coeliac screen Management - investigate and manage cardiac risk - recombinant growth hormone for short stature - low dose estrogen therapy at 11-12yrs for hypogonadism

Answer 43

Epidemiology - edward = 1/5000 - patau = 1/15,000 Aetiology - edward = trisomy 18 - patau = trisomy 13 Pathophys for both - meiosis non dysfunction - unblanaced robertsonian translocation - mosaicism Key manifestations - edward - >heart defects - >oesophageal atresia - >polyhydramnios - >horseshoe kidney - >bronchopulmonary dysplasia - >ID - patau - >holoprosencephaly - >heart defects - >polycystic kidneys - >meningomyelocele Prognosis for both - >majority die in utero - >majority of infants die in first 2 weeks - >5-10% live to first year - >severe ID and failure to thrive

Paediatrics Flashcards

(67 cards)