Computer generated transcript

Warning!
The following transcript was generated automatically from the content and has not been checked or corrected manually.

Rapid Review Paediatrics Emma Jackson, Shehani Kumarasiri & Ayesha Holderness Presentation adapted from original by Isabelle Williams and Natasha Thomas Appendix with a LOT of extra information at the end • Emergency Paeds See our shared google drive • Cardiology for our deep-dives into each • Respiratory subspecialty throughout the year! • Gastro • Neuro What we will • MSK cover • Infectious diseases We are covering a LOT so won’t • Endocrinology • Neonatology have time to discuss in too much detail! ● Good resources for paediatrics revision: ○ 100 cases in paediatrics ○ Clinical Cases Uncovered ○ Clinical knowledge summariesPart I Emergencies Cardiology Respiratory I Emergency paeds1-year-old Zane presents to the ED with swollen lips and noisy breathing for the last 2 hours. What do you want to rule out? Management? https://dftbskindeep.com/ Anaphylaxis •What? Severe type of allergic • Management: reaction • Acutely •Presentation: • A to E • Lie patient flat (with or without legs elevated)on) • sudden onset and rapid progression • IM Adrenaline at 1 mg/mL (1:1000) concentration of generalized pruritis, widespread • Establish airway, give high flow oxygen erythema/ urticarial rash, • Apply monitoring: pulse oximetry, ECG, blood • hoarse voice/ stridor, wheeze, SoB, • If no response repeat IM adrenaline after 5 minutes hypotension, tachycardia + IV fluid bolus Note chlorphenamine and hydrocortisone no longer part of the anaphylaxis algorithm •Investigations: • Admit for at least 6-12 hours (biphasic reaction) • Bloods for mast cell serum tryptase (3x) • Refer to allergy clinic • Education (Epipen, allergen avoidance)Burns Burn Appearance Referral? • A to E assessment classification • Burns first aid: Always consider NAI Superficial Red and painful Primary care Epidermal (1st • Cool area with running water (20 min) degree) • Plastic wraps after cooling • Pain relief Partial thickness Pale, pink, >2% TBSA in a (2 degree) painful child → burns • Estimate burn size: Superficial facility • Lund and Browder chart dermal • All burns to face, ears, eyes, hands, feet, genitalia, perineum Parndal thickness White, reduced All deep dermal or major joint need to be referred to burn’s facility (2 degree) sensation burns in a child Deep → burns facility • Fluid resus formula…? dermal • Parkland formula Fulrdthickness White / Burns facility • Volume of fluid = total body surface (3 degree) necrotic, area of the burn % x weight (kg) x4 painless st • Give half in 1 8hLund and BrowderCommon poisonings •General Management: • A&E: TOXBASE • Within 1h? Activated charcoal • Antidote? • Circumstance? •Paracetamol: • Presentation: • Early: abdo pain, vomiting • Later: acute liver failure • Mechanism: • Accumulation of NAPQI (toxic metabolite) w/ saturation of liver metabolism • Management: • Measure levels at 4h • Treat with NAC if levels above treatment line • Staggered OD/ ingestion time unknown = treat • Urticaria common with NAC infusions: stop, re-start at slower rate; chlorphenamineHead injury – NICE CT head guidelines What features require a CT within 1 hr? • Are any of the following present? • If no → are any of the following present? • Suspicion of NAI • Witnessed LOC >5 min • Post-traumatic seizure • Abnormal drowsiness • GCS <14 initially or <15 2h post injury • 3 or more discrete eps of vomiting • Suspected open/depressed skull • Dangerous mechanism of injury – e.g., fracture high-speed road traffic collision, or fall from • Sign of basal skull fracture – panda height >3m eyes, battle sign, CSF leak from • Amnesia >5 min nose/ears • If yes for more than 1 → urgent CT within an hour • Focal neurological signs • Less than 1 year old with • If only 1 → admit and observe for at least 4h; CT if bruise/swelling >5cm on head concerned • No symptoms/ signs and benign mechanism of • If yes → urgent CT within an hour injury → discharge home with written adviceRIF pain in children? Differentials Appendicitis, torsion, inflamed meckel's diverticulum, strangulated inguinal hernia, intestinal obstruction, DKA, pyelonephritis, Henoch-schonlein purpura… Acute appendicitis • Most common cause of acute surgical abdomen in • Bloods: children • Anatomy: McBurney’s point • Raised WCC • Urine dip: mild leukocytosis • Pathology • Obstruction of appendix lumen by faecolith, • Imaging: lymphoid hyperplasia • 1 line = USS • Symptoms: • eCXR if ?perforation • Periumbilical pain → localized to RIF • Boys → examine scrotum (differential = torsion) • Anorexia • Low-grade fever • has started her periods)if of child-bearing age; ask if she • Vomiting (once) • Management • Signs: • Laparoscopic appendicectomy w/ prior abxs • Rigidity/ guarding • Rovsing’s sign • Non-operative management is only recommended • Peritonism if rupture in the absence of an appendicolith Rashes IM BenPen → Blue light to hospital Courtesy of Skin Deep https://dftbskindeep.com/ Blue = Gram positive Red = Gram negative Bacterial meningitis: Causes • Can be part of a bacteraemia or occur via direct spread e.g., from ear infections, skull or sinus fractures • Use dexamethasone to prevent sensorineural hearing loss Newborn (Baby BEL) • IV cefotaxime + amoxicillin • unless septicaemia or <3mo • Group B strep, E. (covers Listeria) E.coli coli, Listeria No LP if: <6yrs (NHS) • IV ceftriaxone (good BBB listeria • ?meningoccocal septicaemia (DIC) • N. meningitidis penetration; not in (diplococcus) neonates as increased • ?raised ICP (do CT head if any focal • Strep pneumoniae bilirubin - use cefotaxime neurology, papilloedema) • HiB instead) • Consider TB in • IV dexamethasone for >3mo Neisseria • If will significantly delay giving abxs <5yrs >6yrs • N. meningitidis • IV ceftriaxone + IV dex • Strep pneumoniaeMeningococcal sepsis Mx •Management: • Community → blue light, IM ben-pen • Secondary care → • <3 months = IV cefotaxime + amoxicillin • >3 months = IV ceftriaxone • Prophylactic abxs for contacts (ciprofloxacin) amoxicillin = listeria cover Why? cefotaxime = some babies • Vaccination → MEN-B, MEN-ACWY can cause jaundice, acidosis • Bacterial meningitis notifiable diseases •Complications: 50-75% neonates have some developmental disorder post-meningitis • Septic shock, DIC • Multi-organ failure and death • Hearing loss - damage cochlea • Hydrocephalus, cerebral palsy • Waterhouse-Frederickson syndrome - bilateral adrenal haemorrhageCardiologyA 6-year-old boy presents to his GP with a cold. On examination the GP notices a soft systolic murmur at the left sternal edge. Are you worried?Innocent murmur: S • Sensitive to position • Short duration • Sternal edge (left) • Soft, blowing murmur • Sweet • Systolic • No Symptoms • Increased CO during illness or anaemia • Reassure and monitor • Refer for echo if unsure A 5-week-old baby was referred to hospital because of wheezing, poor feeding and poor weight gain. On examination there is a thrill and a pansystolic murmur at the lower left sternal edge. What is the diagnosis? VSD •Most common congenital heart defect (30% of defects) •murmurs)o a pan-systolic murmur loudest at the left sternal edge (smaller holes have larger Features: •Small VSDs can be asymptomatic •large VSDs can cause: • Breathlessness • Failure to thrive • Recurrent chest infections • Enlarged liver (heart failure) Management: • Conservative - monitor small VSD, may close spontaneously • Medical - diuretics and ACE-i for heart failure • Surgical - at 3-6 months what are the other acyanotic heart defects?Acyanotic defects L → R shunts Causes: • ASD • VSD • PDA • Coarctation of the aorta • are picked up antenatallyheart defects • factor for infective endocarditiss a riskASD Presentation • Usually only noticed in adulthood • May be no murmurrcise tolerance • May be ejection systolic at upper left sternal border due to increased flow across pulmonary valve Management: • Meadults- anticoagulation to reduce risk of stroke in • Surgical - 3-5 years if symptomatic/ RV overload Complications • Paradoxical emboli → stroke I PDA Failure of DA closure at 1 month, causes pulmonary hypertension Presentation: • Can cause feeding difficulties, failure to thrive, LRTIs • Continuous “machinery” murmur beneath the left clavicle • Collapsing pulse and wide pulse pressure • Seen commonly in premature babies Management: • If associated with another CHD, give PGE to maintain ductus • Medical - NSAIDs (ibuprofen, indomethacin) 1 • Surgical - ligation, coils 16-year-old girl presents with dizziness. On examination she has a systolic murmur heard loudest at the back between her scapulae. She is short for her age with a broad chest and wide neck. Which syndrome do you suspect? Which heart condition?Coarctation of the aorta • May occur due to the remnant of the ductus arteriosus acting as descending aortaictive band of the aorta = narrowing of • More common in boys • Associated with Turner’s syndrome Features: • Can be asymptomatic • Hypertension • Ejection systolic murmur best in interscapular area • Radiofemoral delay & absent or weak femoral pulses Investigations: • CXR – rib notching (late) • CT angiography Management: • Early - prostaglandin infusion to keep ductus arteriosus open • Corrective surgery/ stenting Complications • Congestive heart failure (high afterload), aortic dissection,What is the syndrome where there is a reversal of L-> R shunting? Eisenmenger’s syndrome •Large left to right shunt (e.g., VSD, ASD, PDA) •Over time, right ventricular overload leads to pulmonary artery hypertension, increased right heart pressures → reversal of shunt (right to left) → cyanosis •At around 10-15 yrs Management: • Prevention! • Treat right heart failure – what are some signs of right heart failure? • Transplant Parents bring their 4-week-old baby to the GP as they have noticed his lips getting blue when he cries, and he has not been breastfeeding well. On examination the GP can hear an ejection systolic murmur at the left sternal edge. What is the diagnosis? A) Tetralogy of Fallot B) VSD C) Transposition of the Great Vessels D) Eisenmenger’s E) Flow murmur secondary to sepsis See more in the Cyanotic heart defects – all the T’s appendix • Examples: (5 T’s) • Tetralogy of fallot -commonest • Truncus arteriosus • Tricuspid atresiaulmonary venous connection • Pulmonary aTresia • +Transposition of great arteries • ToF • 4 weeks, murmur due to pulmonary stenosis • mo• (VSD, pulmonary stenosis, RVH, overriding aorta); • can present in first weeks of life with cyanosis, respiratory distress and failure to thrive. • CXR - may show boot shaped heart due to VH; need echo to diagnoseRespiratoryUpper/ lower respiratory tract infectionURTI vs LRTI • Upper respiratory tract = the airway above the glottis (vocal chords) → larynx, pharynx, sinuses and nose • cough, fever, a fluctuating level of unwellness but absence of respiratory distress • Lower respiratory tract = the airway below the glottis → trachea, bronchi, bronchioles and alveoli • Pneumonia = LRTI specifically involving the alveoli Pneumonia ● Pneumonia should be considered if the child has: ○ A high fever (over 39°C) and/or ■ Cyanosis. ■ Raised respiratory rate ● (greater than 60 breaths per minute, age 0–5 months; greater than 50 breaths per minute, age 6–12 months; greater than 40 breaths per minute, age older than 12 months). ■ Signs of increased work of breathing, such as chest indrawing and nasal flaring. ■ Persistently focal crackles. ■ Oxygen saturation of 95% or less in air. ■ Absent breath sounds with a dull percussion note (possibility of pneumonia complicated by an effusion). ● Pneumonia is a clinical diagnosis ○ CXR is not recommended as a way of diagnosing uncomplicated community acquired pneumonia ● All children with a clinical diagnosis of pneumonia should receive antibiotics ○ As bacterial and viral pneumonia cannot reliably be distinguished from each otherRespiratory distress signs in children • tachypnoea • nasal flaring • grunting = expiration across a partially closed glottis • intercostal recession, subcostal recession, suprasternal • cyanosis if severe Stridor URT obstruction Inspiratory Differentials in a child?Croup • URTI - Parainfluenza virus • Management: • Presentation: 6mo to 6yo in autumn • For ALL: single dose of oral Dex • Stridor • Mild: manage at home, parental • Increased WoB obs • Barking cough, worse at night • Severe: hospital treatment: • Low-grade fever • Coryza ■ high-flow O 2 • Grading: CKS ■ nebulised adrenaline • Mild → seal-like barking cough, no stridor or ■ close obs sternal/intercostal recession at rest • sternal recession at rest; no agitation/lethargyand • Severe → seal-like barking cough with stridor and sternal/intercostal recession associated with agitation or lethargyAcute epiglottitis •Intense swelling of the epiglottis and •Management surrounding tissues associated with • DO NOT examine child septicaemia • Urgent hospital admission •Life threatening due to high risk of – ITU airway obstruction • Ask for senior help – ENT •Cause: HiB infection • Check vaccination history • secure, cultures, IV abxs •The 4 D’s • Dysphagia • Prophylactic rifampicin of • Dysphonia – ‘hot potato’ voice household contacts • Drooling • Distress I Bacterial tracheitis •= pseudomembranous croup •Rare but dangerous •Usually follows a viral URTI •Similar to severe epiglottitis - acute airway obstruction with thick airway secretions •High-grade fever •Cause: S. aureus •Management: IV abx ± intubation and ventilation I Other causes of stridor •Acute stridor • FB inhalation • Anaphylaxis •Chronic stridor – usually due to a structural problem • Laryngomalacia – floppy larynx • Subglottic stenosis (hx of neonatal ventilation) • Papillomatosis (HSV infection in mother) • Subglottic haemangioma Part II Respiratory Gastroenterology Neurology 3 min breakThe wheezy childBronchiolitis •Most common serious respiratory •Features: infection of infancy • Coryza •2-3% of all infants are admitted to • Feeding difficulties with hospital with the disease each year dyspnoea during winter epidemics • Dry, wheezy cough •90% are 1-9 months • Cyanosis or pallor •Cause: RSV (80%), parainfluenza virus, • Hyperinflation of the chest rhinovirus, adenovirus, influenza virus • Subcostal and intercostal and human metapneumovirus recession •‘Gets worse before it gets better’ • Fine end-inspiratory cracklesCont … •Investigations: Pulse oximetry •Management is supportive • Sats determine when admission is needed • Humidified oxygen/ CPAP • CXR & blood gases only if atypical • IV fluids or NG feeding • Infection control, as RSV is very •Hospital admission if any of: contagious • Apnoea (observed or reported) •Discharge: when infant is stable, • Persistent oxygen sats <90% on air maintaining sats o/a >92% for 4h, • Inadequate oral fluid intake incl period of sleep for 12hrsormal) or no wet nappies • Severe respiratory distress •Prevention: monoclonal RSV • NICE traffic light antibody is given to high-risk preterm infants (pavilizumab) I Viral-induced wheeze •Consider in those 6 months - 5 years •Episodic – only wheeze when viral URTI and is symptom free between episodes • Infective exacerbations of asthma and viral-induced wheeze are often a respiratory tract infection, unless there is a history of wheeze in the absence of infection (children in this age group cannot perform lung function tests reliably for definitive asthma diagnosis) •Multiple trigger wheeze – other factors appear to trigger wheeze • Exercise, allergens, smoke •Acute management: • Determine severity: as acute asthma • Severe: nebulised salbutamol / Oxygen • Mild-to-mod: SABA via MDI w/ large volume spacer •Long-term management: • Episodic: SABA via spacer • MTW: inhaled steroids or trial of montelukast • Increased risk of asthma Asthma • Reversible (lower) airway • Signs: obstruction • Raised HR/RR • Aetiology: • Widespread, • Airway (type I) polyphonic wheeze hypersensitivity • Ask about FHx of • Prolonged exp phase atopy, parental • Chronic: Hyperinflated smoking, sleep/ school chest, Harrison’s sulcus • Presentation: • Atopy: Polyps/eczema • Dry cough, worse at • Diagnosis: night • Noisy breathing: • <5: clinical dx wheeze • 5-16: spirometry w/ • Hx of precipitants, e.g., BDR, FeNO cold, exercise Asthma Management • Management (NICE): • 0-2: MDI + spacer + face mask • Step 2: SABA + ICS • Step 3: SABA + ICS + LTRA • 3-5: MDI + spacer • Step 4: SABA + ICS + LABAAcute asthma management • Severe/ life-threatening asthma attack → immediate transfer to hospital Severe Life-threatening • Mild-to-mod: SpO2 < 92% SpO2 < 92% •SABA via spacer PEF 33-50% best or PEF < 33% best or •Oral pred predicted predicted • Severe / Life-threat: Can't complete full Silent chest sentences Poor respiratory effort •High-flow oxygen (sats <92%) HR > 125/min Altered consciousness •SABA via spacer/ neb (back-to-back) RR > 30/min Cyanosis •Oral pred Accessory muscle use Hypotension •Consider: ip brom, aminophylline, Mg2+ •Discuss w/ PICUDischarge • Criteria for discharge • Been stable on their discharge medication (i.e. no nebulisers or oxygen) for 12–24 hours • Inhaler technique checked and recorded • PEF >75% of best or predicted • Also need to arrange • Weaning plan for steroids/ inhalers • Review previous meds/ triggers and adapt doses: NICE/BTS guidelines • Review by GP in 48h • Review in asthma clinic in 2 weeks What investigation is likely A 10-year-old child presents with to be diagnostic? enlarged tonsils that meet in the midline, first noticed 4 days ago. A. Throat swab B. Bone marrow aspirate Throat examination confirms this + trephine finding, and you also notice C. FBC petechial haemorrhages affecting the oropharynx. On systemic D. testm heterophile ab examination he is noted to have E. ASOT titre splenomegaly. • Acute tonsillitis = inflammation of the tonsils ± inflammation of pharynx (pharyngitis) • Terms used interchangeable • Cause: mostly viral or GABHS • Strep pyogenes (predominant GABHS species) Sore throat • Px: fever, sore throat, painful swallowing • Use the Centor criteria to determine likelihood that sore throat is due to GABHS Investigations •DO NOT routinely take throat swabs •ASOT - no role in dx but helps confirm hx of GABHS in kids with GN / rheumatic fever Management Sore throat •Self-limiting cont … •Abx of choice - Pen V or erythromycin for 7-10 days •AVOID AMOXICILLIN – in context of acute glandular fever can lead to generalised rash •If on DMARDs/ carbimazole - do FBC urgently • ?neutropenic sepsis Scarlet fever • by GABHSfever: reaction to erythrogenic toxins produced • Strawberry tongue • Sandpaper rash • Circumoral pallor • Desquamation occurs later • Management: • Pen-V for 10 days • Can return to school 24h after commencing abxs (Droplet spread) • Complications: • AOM = most common • Rh fever • Acute GN • Invasive infection – bacteremia, meningitis, nec fasc https://dontforgetthebubbles.com/scarlet-f ever/Acute otitis media •Middle ear inflammation • Abxs should be prescribed if •Common GP presentation • Symptoms lasting more than 4d, or worsening • Systemically unwell (<3 mo with T 38º or •Presentation: more, or 3-6 mo with T 39º or more) • Otalgia, fever ± discharge • Follows viral URTI • Immunocompromised • Child may be crying/ pulling at affected • <2yo with bilateral OM ear • OM with perforation ± discharge in canal •otorrhoea (if perforation)um ± purulent • 1 line = amoxicillin •Management: • Complications •(return if symptoms worsen/do not net advice • C(S)OM improve after 3 days) • Mastoiditis • MeningitisOther resp disorders to • Cystic fibrosis • Pertussis look upGastroDifferentials to know ● Abdominal pain ● Vomiting ● Constipation ● Diarrhoea ● Abdominal mass • Inadequate intake → poor feeding • Malabsorption → coeliac disease, CF • Excess loss → vomiting (GORD), pyloric stenosis, protein-losing enteropathy Failure to (CMPA), IBD • Increased demand → CCF, recurrent thrive infection (UTI) chronic infection (HIV), malignancy, inborn error of metabolism • Metabolic/endocrine → diabetes mellitus, thryoid disease • Social → child abuse/ neglect I Constipation Very common Red flags: • Failure to pass meconium in 1 48h Differentials: • FTT ● Non-pathological (dehydration, • Abdominal distension low-fibre diet) • Abnormal lower limb neurology/ deformity, sacral dimple ● Hirschsprung’s disease above natal cleft, hairy patch • Perianal bruising/ fissures/ fistulae ● Cystic fibrosis Should not routinely perform DRE in children ● Medications - opioids, antacids containing aluminium and calcium, anticonvulsants Can result in overflow diarrhoea ● Hypercalcaemia ● Hypothyroidism Management: • Conservative: Fluids, behavioural, dietary fibre ● CMPA • Disimpaction regimen with Movicol Paediatric Plain (polyethylene glycol 3350 + electrolytes) • Followed by maintenance treatment Investigations: Hirschsprung’s disease • AXR +/- contrast • Suction rectal biopsy 3x >2.5cm above dentate line Pathophysiology: Management: • Failure of NCC migration from top → • Initially: rectal washouts and antibiotics to reduce bottom of GIT → developmental risk of enterocolitis failure of parasympathetic Auerbach/ • For enterocolitis: stoma to decompress distal Meissner’s plexuses → aganglionic bowel distal bowel → no peristalsis • Surgery: pull-through procedure, Soave procedure • a/w Down’s syndrome, MEN-2 Complications: • Bowel perforation • HD enterocolitis Presentation: • Failure to pass meconium in 1 48h • Abdominal distension, explosive diarrhoea, bile-stained vomit • FTT CMPA • Very common - affects 3-6% of infants in 1 3 months of Management life • Systemic symptoms w/ angioedema? Suspect • More commonly seen in bottle-fed infants anaphylaxis • Consider referral to • Types: • Allergy clinic if FTT • Immediate (IgE-mediated) • Delayed (non-IgE-mediated) • Paediatric dietician Presentation: • Cow’s milk protein elimination/ home • Irritability (colic) reintroduction (milk ladder) • Reflux & vomiting • Formula: eHF/ AAF • Refusing/ disliking being fed • Loose/ frequent stool • iMAP guidelines for parents • Abdominal painstraining to pass soft stool) Note: CMPA usually resolves in most children • Blood/ mucus in stool (non-IgE > IgE) • Iching/ redness of skin, eczema Investigations: • Diagnosis is often clinical (improvement with cow’s milk protein elimination) • Skin prick/ patch testing • Total IgE/ specific IgE (RAST) for cow’s milk proteinGastroenteritis – acute diarrhoea • Most common cause in kids: rotavirus (l ive Investigations: vaccine available) • Clinical diagnosis usually • Bacterial causes less common • Stool culture if septic, blood/ mucus in stool • Bloody → campylobacter, shigella, salmonella, • Consider culture if recent travel, not improving EHEC • U&Es, Cr, glucose • Non-bloody → cholera, ETEC Management: • Infants are at risk of severe dehydration/ shock • No evidence of dehydration: encourage fluid due to increased SVR, higher basal fluid intake, discourage fruit juices/ fizzy drinks requirements and immature renal anatomy • Dehydration: oral fluid challenge / oral rehydration solution • Red flags: • Unwell/ deteriorating • Red flags or child is vomiting ORS: IV fluids • Irritable, lethargy • DO NOT use anti-diarrhoeal medications • Eyes sunken (loperamide) • SAFETY NET • Tachycardia/ tachypnoea • Reduced skin turgor Investigations: Coeliac disease ● Serum tTG IgA levels ○ Check total IgA alongside ○ Abs become negative on strict gluten-free Gluten-sensitive enteropathy diet ● Genotyping ● Small bowel biopsy Clinical features ○ Must be eating gluten for 6 weeks prior • FTT • Abdominal distension • Buttock wasting Management: • Anaemia ● Life-long adherence to gluten-free diet • Dermatitis herpetiformis (older adults) ● Correct anaemia ● Vaccinations due to hyposplenism ○ Annual influenza Screen those at risk: ○ One-off pneumococcal • T1DM ● Assess bone mineral density with DEXA if • Autoimmune thyroid disease indicated (osteoporosis/ osteomalacia) • Downs syndrome Rare complication = EATL (night sweats, fever)Duodenal biopsy • Villous atrophy (reduced absorptive surface) • Crypt hyperplasia (increased cellular proliferation) • Inflammatory infiltration (increased intraepithelial lymphocytes, influx of immune cells into lamina propria)Vomiting Differ ntialsls Red flags • Pyloric stenosis • Bile-stained vomit • GORD • Projectile vomiting in first few Vomiting • Gastroenteritis weeks of life • Obstruction • Vomiting at the end of a (intussusception, paroxysmal cough atesia) • Abdominal tenderness/ pain • Whooping cough • Abdominal distension • Strangulated hernia • Hepatosplenomegaly • DKA • Blood in stool • NEC • Severe dehydration, shock • Raised ICP • Bulging fontanelle or seizures • Faltering growth Complications: GORD • Faltering growth • Oesophagitis • Recurrent pneumonia • Very common in infants • Sandifer syndrome • Inappropriate relaxation of the Investigations: LOS bc of functional immaturity • Exacerbating factors: • Clinical diagnosis • Fluid-based diet • 24h oesophageal pH/ impedance monitoring • Horizontal feeding posture Management: • Anatomy • Reassure parents • Nearly all resolve by 12 mo of age • Feed position → 30 degree head-up • Feed thickeners • Acid suppression • Failure to response to treatment, consider alternative diagnosis, e.g., CMPA Pyloric stenosis “olive-shaped mass” • Hypertrophy of pyloric muscle causing gastric outlet obstruction • Presents at 2-8 weeks of life • M > F • 5-10% have a positive family history Presentation: Management: • Hungry baby (early) • Projectile non-bilious vomiting • NBM and NG tube for decompression + IV • Failure to gain weight fluids (rehydration and replacement of Investigations: electrolytes) • Surgical – Ramstedt’s pyloromyotomy • Bloods - Hypochloraemic, hypokalaemic metabolic alkalosis • Abdominal USS • Typically affects children 5 mo to 2 years • In children, most common cause is hypertrophic lymphoid tissue following an infection • Lead points (more common in adults) include • GI malignancy – colorectal cancer, mets, small bowel lymphoma/ Burkitt lymphoma • Congenital – Meckel’s • Inflammatory – appendicitis Presentation: • Lethargy is early sign • Redcurrant jelly stool – late signn Intussusception • Sausage-shaped mass in RUQ and emptiness in RLQ (Dance’s sign) Investigations: • USS – target sign Management: • Air enema reduction • Surgical reduction/ resection is usually required Red flags • Older child (over 2yrs) • Enlarged liver • Atypical presentationTarget signNeurology • Definition = umbrella term for permanent disorder of movement and/ or posture and of motor function due to a non-progressive abnormality in the developing brain • Affects 2 in 1000 live births • 80% antenatal due to haemorrhage or ischaemia, cortical migration disorders or structural malformations • 10% perinatal due to HIE • 10% postnatal due to acquired brain injury, e.g. • Meningitis Cerebral palsy • Head trauma • NAI • Kernicterus • Consider CP… • Hand dominance before 1 yo age in boys • Abnormal gait • Delayed motor milestones • Feeding difficultiesMDT Management: Paediatrician, PT/OT, SALT, Specialist nurse, Dietician, Psychologist, Social services Spasticity treatment: Baclofen Analgesia Anticonvulsants if neededHeadache Red Flags •Developmental regression in a child •Raised ICP •Headache worse when lying down / in the morning •a/w confusion, vomiting •Papilloedema • Vi• Also growth failurehink craniopharyngioma = pituitary tumour) • Neck stiffness, signs of infection • MeningitisMigraine •Migraine w/o aura is most common cause of primary headache in children •Management: • 1 line = ibuprofen •To diagnose, must have 5 or more episodes with: • Headache lasting 4-72h, and • If >12 yo age: sumatriptan nasal • 2/4 key features: spray (oral not licensed if <18) •Prophylaxis: • bilateral or unilateral (frontal/temporal) location • Pulsatile • Pizotifen, propranolol • Moderate/severe intensity • Worsened by routine physical activity • And at least one of: • Nausea +/- vomiting • Photophobia and phonophobia Most paediatric brain tumours are primary (in contrast to secondary in adults) Most common benign brain tumour in children: pilocytic astrocytoma Most common malignant brain tumour in Brain tumour children: medulloblastoma As a general rule … in children, most primary brain tumours arise infratentorially Important exception – craniopharyngioma (see appendix)Neurofibromatosis NF-1 NF-2 • AD inheritance • AD inheritance • Café au lait • 18-24yrs, tumours by 30yrs • Axillary freckling • Bilateral vestibular schwannomas • Fibromas → SNHL • Iris harmatomas (Lisch nodules) • Meningiomas • Skeletal anomalies (scoliosis, sphenoid dysplasia, leg bowing) • Ependymomas • Pheochromocytomas • Optic glioma - may cause visual impairment • Astrocytomas • Other tumours e.g. malignant peripheral nerve sheath tumourNF-1 Febrile seizures Breath-holding spells Epileptic Anoxic seizures Reflex anoxic seizure Seizure Non-epileptic Infection Seizure mimics Head trauma Toxins Other Metabolic Febrile convulsion • 6 months to 6 years • Generalised tonic-clonic seizure associated with fever Simple Complex Status • 25% have FHx <15mins 15-30mins 30+mins • 30% have further episodes • Must exclude underlying causes such as infections e.g., Generalised: tonic Focal meningitis clonic Complete recovery May have second • Advice to parents: within 1hr within 24hrs • Short lasting seizures are not harmful • Reducing the fever does not prevent recurrence • Normal intellect and behaviour Increased risk of • Usually don’t develop epilepsy (2% risk) epilepsyAnoxic seizures Breath holding attack Reflex anoxic seizure • Usually having a tantrum/crying and •Unexpected pain before convulsions they hold their breath (not seen intentionally) •Child went “deadly white” then • Child went “blue” then “collapsed” “collapsed” • Holds breath → cyanosis and cerebral •Pain → vagus nerve stimulation → anoxia bradycardia/brief asystole → cerebral • Grow out of this anoxia I Absence seizure Myoclonic Generalised Tonic-clonic Tonic Atonic Frontal Epileptic Temporal Focal Seizure Non-epileptic Parietal Seizure mimics Occipital West syndrome Epilepsy syndromes LGS Janz syndrome I Generalised epilepsy •Abs• ‘petit-mal’ • During typical absence, person becomes blank or unresponsive for a few seconds • ‘daydreaming’, look blank/ stare or eyelids begin to blink or flutter • They do NOT response to what is happening around them •Tonic clonic • ‘grand-mal’ • Stiff, then jerking • LoC, tongue biting, incontinence Investigations • EEG (3Hz) • Video telemetry • MRI Management • Advice Epilepsy • Avoid baths and swimming in the sea • Alcohol, drugs and sleep deprivation may trigger an episode work-up • Medical • Generalised: Valproate (teratogenic) or Levetiracetam first line • Absence: Ethosuximide • Atonic: Valproate or Lamotrigine first line • Focal: Carbamazepine or lamotrigine • Infantile spasms: Vigabatrin Regular follow upWest syndrome • Triad of • “Jackknife” Flexion of head, trunk, limbs → extension of 1-2 secs, repeat up to 50t times • Developmental regression • EEG: hypsarrhythmia • 90% begin before 1st birthday • Manage with Vigabatrin • Can use pred if not sclerosistuberous https://www.youtube.com/watch?v=yAY P8r-MBcg Part III MSK Infectious diseases Endocrine Neonatology 3 min breakMSK Broad list of differentials: • Infection •Septic arthritis •Osteomyelitis •Post-strep (reactive) • Malignancy •ALL Painful limbs •Bone tumour (osteosarcoma, Ewing’s sarcoma) •Mets • Trauma •Bone fracture / NAI • Kawasaki disease (CRASH and BURN) • JIA Septic arthritis Organisms Most common pathogen causing septic arthritis Staph aureus Most common pathogen causing septic arthritis in Salmonellae an individual with sickle cell Gram negative diplococcus in knee aspirate N. gonorrhea Bone tumours Ewing’s Osteosarcoma Lytic Osteoid producing 10-20yrs 15-30yrs • Uncommon before puberty Diaphysis of long bones, Metaphysis of long bones • Male predominance pelvis, patella and spine • Px: localized bone pain, severe Sx Tumour of neuroectoderm Mesenchymal cells with following minor injury (small round blue cell) osteoblastic differentiation • Investigations: Stain for CD99 • XR/ MRI/ bone scan Radiology: onion skin Radiology: Codman’s • OS → destruction, new bone appearance triangle (periosteal formation elevation), sunburst • ES → soft tissue involvement appearance • Management: surgery + chemo-RT 95% have Translocation Associated with Rb involving EWS gene on Chr mutations 22Ewing’s sarcoma Osteosarcoma Definition: arthritis in 1 or more joint(s) for more than 6 weeks in under 16-year-olds in the absence of infection or other defined cause. Presentation: •5x more common in girls •Stiffness in morning, ‘gelling’ after periods of rest •Limp •Anterior uveitis – BUT WHITE EYE NOT RED Types: •Systemic (high daily spiking fever, salmon pink rash, lymphadenopathy, serositis) JIA •Polyarticular (RhF+/-) - 5 or more joints •Oligoarticular (most common) - 1-4 joints Investigations: •FBC, blood film, ESR/CRP/ferritin, Antibodies (ANA/RhF/HLA-B27), slit lamp examination, NAAT for gonococcus, MRIs to identify extent of disease Management: •1 line = NSAIDs •2 line = DMARDs •Physio to maintain joint function and ophthalmology reviews •Aim to avoid systemic steroids as much as possible Age Causes 1-3yrs Transient synovitis DDH Neuromuscular (CP) 3-10yrs Transient synovitis Perthes disease The limping JIA NM disorders (muscular dystrophy) 11-16yr Slipped upper femoral epiphysis child Reactive arthritis JIA DDx depends on age of child Complex regional pain syndrome Osgood-Schlatter disease All ages JIA Trauma Infection: septic arthritis, osteomyelitis Malignancy: leukaemia, neuroblastoma, bone tumour • Acute joint pain associated with viral infection • Most commonly affects the hip and is the most common cause of hip pain in children • Age 2-10 • May have low grade fever Transient • If high, suspect septic arthritis • Investigations: synovitis • Normal XR, FBC, CRP, ESR • USS – may see effusion • Management: • Self-limiting • Rest + analgesia • Should recover in 6 weeksPerthes’ disease • Avascular necrosis of the femoral head • Typically affects boys 4-8 Presentation: • Progressively worsening hip pain + limp • Reduced range of movement - especially abduction • Antalgic gait, leg length discrepancy Investigations • Bilateral hip XR - flattening of femoral head • MRI Management • Conservative - casts and braces to keep femoral head within the acetabulum, physiotherapy • Surgical - THR if severe Complications • Osteoarthritis • Premature fusion of growth plates Early diagnosis key to prognosisSUFE • Pheadrior inferior displacement of femoral Right Left • Typically affects obese boys aged 10-15 Presentation: • Loss of internal rotation • Externally rotated, shortened leg • 20% bilateral Investigations: • Bilateral hip XR: AP and frog-leg views (Klein’s line) Management: • Admit and refer to orthopods • Surgery – internal fixation to stabilize growth plate • Screening: • For ALL → NIPE involves Barlow, Ortolani DDH • USS within 6 weeks if • 1 degree affected relative • Affects up to 3% of newborns, 20% • Breech bilateral • Multiple pregnancy • What happens? • If >4.5mo, bilateral hip XR • Femoral head dislocates • Management: • Ball and socket grow out of • Pavlik harness if <4-5mo proportion to each other • If >6mo, surgical correction • Risk factors: the F’s → feet first, female, fluid low, fat (maternal T2DM), first-born, FHx • Signs: leg length discrepancy, asymmetric skin folds • If not detected at birth → waddling gait, OA in adultsIA 6-year-old boy is brought to surgery by his mother. For the past 2 months he has been complaining of pain in his shins and ankles at night-time. His symptoms are bilateral; he is otherwise well. There is no family history of note. Clinical examination is unremarkable. I Pain in the legs, no obvious cause M=F The rules of growing pains: Growing pains • Age 3-12 • Symmetrical, lower limb • Never at start of day after waking • No limp • Examination normal, incl pGALSInfectious diseases KOPLIK SPOTS Case 1 Measles • 17-month-old previously healthy but unimmunised child develops fever, malaise, cough, runny nose, and conjunctivitis, that worsen over several days. • Fever increases gradually up to 39-40°C over several days and the cough worsens. Photophobia and whitish lesions on erythematous buccal mucosa. • Two days later, an erythematous, maculopapular rash appears on the head and spreads from head to toe over the next 3 days.Measles • Highly infectious disease caused by measles virus • Characterised by a maculopapular rash, cough, coryza, conjunctivitis, and a pathognomonic exanthem (Koplik's spots) • Incubation period ~10 days. • Preventable by immunisation but high levels of coverage are required to prevent outbreaks of disease from occurring → increasing levels in community • No specific treatment for measles is available except for supportive care. • Notifiable disease. • School exclusion for 4 days from onset of rash. • Complications include pneumonia, laryngotracheitis, otitis media, and encephalitis → more common in immunocompromised and malnourished individualsMumps • Acute systemic infectious disease caused by an RNA paramyxovirus • Spread by droplets. Infects respiratory tract epithelial cells → parotid glands → other tissues • Infective 7 days before and 9 days after parotid swelling starts. Incubation period = 14-21 days • Clinical features = fever, malaise, muscular pain, parotitis ('earache', 'pain on eating') → unilateral initially then becomes bilateral in 70% • Prevention through vaccination (MMR) • Management is supportive. Notifiable disease. • Complications • Orchitis - more common post-puberty • Hearing loss - usually unilateral and transient • Meningoencephalitis • PancreatitisParvovirus B19 •Causes erythema infectiosum (also known as fifth disease or 'slapped-cheek syndrome') •Features: mild feverish illness, rose-red rash over cheeks (may spread across trunk and limbs) •Mx: supportive care •Complications: • Aplastic crisis if child has haemolytic anaemia (sickle cell, HS, G6PD) • Disaster in pregnancy • PV infects erythrocyte precursors → severe fetal anaemia → high output cardiac failure → non-immune fetal hydrops • Mx: intrauterine blood transfusionHand, foot and mouth disease Causes: Enterovirus 71/Coxsackie A16 O/E: • Mild systemic upset: sore throat, fever • Oral ulcers • Followed later by vesicles on the palms and soles of the feet Very contagious: nursery outbreaks are common Management: symptomatic treatment only, no school exclusion required Roseola infantum Hand, foot and mouth disease Causes HHV6 Enterovirus 71/Coxsackie A16 Features • Initial high fever lasting a few • Mild systemic upset: sore days throat, fever • Maculopapular rash after fever • Oral ulcers • Nagayama spots → papular • Followed later by vesicles on enanthem on the uvula and soft the palms and soles of the palate feet • Febrile convulsions occur in around 10-15% Very contagious: nursery outbreaks • Diarrhoea and cough are also are common commonly seen Mx? Symptomatic treatment only, no school exclusion requiredEndocrine Presentation: weight loss, FTT, polyuria/ polydipsia, secondary nocturnal enuresis, candida infection/balanitis, DKA Diagnosis: •Symptoms + RG ≥ 11.1 mmol/L or FG ≥7.0 mmol/L, •OR asymptomatic with 2 RGs/FGs of ≥11.1/ 7.0, respectively Management: T1DM •Subcut insulin: 0.5 units/kg/day •MDII as basal bolus regimen •Admit for education/ stabilization •Discharge when able to check BM with telephone follow-up Review: eyes, renal function, BMI, HbA1c, thyroid/coeliac abs, feet, BP and imms NOTE T2DM is rare in children, but becoming more common as obesity increases •Triad of: • Hyperglycaemia (>11) • Acidosis (pH <7.30 or HCO 315 mmol/L) • Ketonaemia (>3.0 mmol/L) Quick note on •Cerebral oedema is most common cause of death in children DKA! DKA • Headache, behavioural change, low GCS • Too rapid lowering of serum Na+ • Neuro obs every 30 mins Puberty (too early, too late) Other things to Growth failure (craniopharyngioma) cover CAH (neonatal px) Congenital hypothyroidismNeonatology Neonatal jaundice Time Jaundice in the first 24hrs Physiological jaundice Prolonged jaundice: of life is always Apparent on day 2-3 → peaks on >2 weeks term, >3 weeks preterm pathological day 5-7 → usually fades by day • Conjugated bilirubin levels (biliary atresia) Unconjugated 10 • LFTs • FBC Causes: ● Rhesus haemolytic Not all cases need treatment • Urine culture disease • Metabolic screen ● ABO haemolytic Dehydration is the major cause Causes: disease (often seen in exclusively ● Biliary atresia ● Hereditary breastfed babies) ● Neonatal hepatitis spherocytosis ● Intrahepatic biliary hypoplasia (Alagille’s) ● G6PD ● Prolonged parenteral nutrition Mx: educate and encourage ● Perinatal asphyxia parents RE regular feeds and ● Bacterial sepsis top-ups if needed ● IU infections ● CF Physiological Neonatal jaundice • More common in breastfed infants • Reduced RBC lifespan • Immature bilirubin metabolism / enterohepatic circulation Differentials: Unconjugated Jaundice in the first 24 hrs is always pathological ● Haemolytic disorders ○ Blood group incompatibility - Causes of jaundice in the first 24h rhesus haemolytic disease, ABO haemolytic disease) • Rhesus haemolytic disease ○ Red cell enzyme deficiency - • ABO haemolytic disease G6PD deficiency • Hereditary spherocytosis ● Meconium retention • G6PD Conjugated After 14d = prolonged jaundice screen ● Biliary atresia • Features of obstruction (pale stool, dark urine) ● breast milk jaundice • Conjugated bilirubin levels (biliary atresia) ● Infections • LFTs ● Hypothyroidism • FBC • Urine culture • Metabolic screenNeonatal jaundice • Unconjugated bilirubin is toxic to neural tissue (crosses BBB), greater risk with increasing prematurity • Kernicterus: yellow staining of cerebral tissue (basal ganglia) • Bilirubin encephalopathy • Acute: lethargy, irritability, abnormal muscle tone/ posture, apnoea, convulsions • Chronic: CP (diplegic), sensorineural hearing loss, gaze palsy, dental dysplasia • Investigations: confirm jaundice by measuring serum bilirubin levels • Management: • Depends on tx threshold graph/table • Includes • Phototherapy • Exchange transfusionRespiratory distress in the newborn 1. Premature or term infant?Premature infant Respiratory Distress Syndrome Bronchopulmonary dysplasia Features: Features: ● Grunting on expiration (helps maintain ● Persistent oxygen requirement airways) >36 weeks ● Nasal flaring and chest wall recession Ix: Ix: ● CXR: widespread opacification ● CXR: diffuse ground glass appearance, Risk of pulmonary HTN and loss of heart border pneumonia Mx: Mx: ● O2, CPAP, ventilation + surfactant ● Antenatal steroids reduce risk ● LTOT and gradual weaning Premature Term RDS: Respiratory Distress Syndrome TTN - Transient Tachypnoea of the Newborn • Presentation: tachypnoea >60 shortly after birth • Delayed lung liquid resorption at birth (fluid seen in horizontal • Grunting on expiration to help maintain airways open fissure on CXR) • Nasal flaring and chest wall recession • More common after C-section • CXR: diffuse ground glass appearance (may lose Heart border) • Usually resolves within a day but must exclude infection • Mx: oxygen / CPAP / ventilation + surfactant • Diagnosis made after exclusion of other causes • Reduce risk with antenatal steroids (which also reduce the risk of BPD and IVH) • May occur in term infants from diabetic mothers BPD: Bronchopulmonary Dysplasia PPHN - persistent pulmonary hypertension of the newborn • Persistent oxygen requirement after 36 weeks • Associated with birth asphyxia, meconium aspiration, • Attributed to delay in lung maturation but also likely related to septicaemia and RDS mechanical trauma etc. • High resistance through pulmonary system leads to reversal • CXR: widespread opacification of shunts and leads to cyanosis • Risk of pulmonary HTN and pneumonia • ECHO to rule out CHD • Often requires long term oxygen therapy and gradual weaning • Life-threatening may lead to cyanosis after birth • Likely to have more severe disease course with subsequent chest • infections T erm infant Transient Tachypnoea of the Newborn Persistant Pulmonary Hypertension of the Newborn • Delayed lung liquid resorption at birth (fluid seen • Associated with birth asphyxia, meconium in horizontal fissure on CXR) aspiration, septicaemia and RDS • More common after C-section • High resistance through pulmonary system leads • Usually resolves within a day but must exclude to reversal of shunts and leads to cyanosis infection • ECHO to rule out CHD • Diagnosis made after exclusion of other causes • Life-threatening may lead to cyanosis after birth Other differentials to consider (see appendix for more notes): ● Meconium aspiration ● Pneumothorax ● Congenital diaphragmatic hernia ● Pneumonia Other causes of respiratory distress in term infants Meconium aspiration Meconium is passed before birth in 8-20% of babies - at birth this may be inhaled Lung irritant → chemical pneumonitis, mechanical obstruction, increased risk of infection May require artificial ventilation Usually, post-term deliveries RFs: Maternal HTN, Pre-eclampsia, Chorioamnionitis, smoking, substance abuse CXR: over-inflated, patches of collapse and consolidation, air leak → pneumothorax Risk of developing permanent pulmonary hypertension of the newborn, associated with severe morbidity and mortality Pneumothorax - spontaneous in 2% or as a result of ventilation Congenital Diaphragmatic Hernia - usually on left side - bowel displacing heart Consider in infants who fail to respond to resus Confirmed with XR of chest and abdo Mx: NG tube to stop overdistension of the bowel and urgent repair of defect Long term → hypoplastic lung due to impaired development of the lung in utero Pneumonia What is the definitive A 6-day-old baby was born prematurely management? at 33 weeks. He has been suffering from A. IV ceftriaxone and respiratory distress syndrome and has vancomycin been receiving ventilatory support on NICU. He has developed abdominal B. IV fluids distension and is increasingly septic. C. Repeat AXR Ultrasound of the abdomen shows free D. Urgent laparotomy fluid and evidence of small bowel E. Increase target oxygen dilatation. His blood pressure has sats remained labile despite inotropic support. NEC • The premature gut is at risk of ischaemia/ bacterial invasion bc of immature immunity • Breast milk protective (IgA) • Signs: abdominal distension, passage of bloody stools • Investigations: • AXR: pneumatosis intestinalis • Treatment: • Total gut rest and TPN • IV abxs • Perforation → laparotomy and resection of necrotic bowel • Complications: Crescents of intramural gas = pathognomonic • Short gut syndrome • Strictures I … and breathe that was A LOTTIPS and TRICKS SAQ • Give a full differential • Bullet point answers & diagrams • Always consider NAI and safeguarding • Don’t forget the basics SBA • Don’t forget General Medicine • Common is common • Read the question! (immediate vs definitive vs rule out)We would be grateful for your feedback! Feedback: Link to our shared google drive:CUPS COMMITTEE APPLICA TIONS! We’ll be opening applications for committee positions at the end of April, so keep an eye out for how to apply and the deadline! I Appendix I Dermatology I A 2-month-old baby comes into the GP with a dry, scaly rash on his face and chest. Baby is alert and smiling. FHx of asthma. https://dftbskindeep.com/Eczema • Inflammatory skin condition characterised by dry, pruritic skin with a chronic relapsing course • Most commonly diagnosed before 5 years of age and affects 10% to 20% of children • Presentation: • Infants: on cheeks/ face, dry, scaly • Toddlers: extensors (wrists, ankles, knees) • School-age: flexors (joint creases), lichenification • Management: • Educate parents • Avoid irritants (synthetic clothing) • Use soap substitutes • Keep nails short • Emollients - large amounts needed (250g/week) • Mild topical steroid e.g., 1% hydrocortisone for acute flare of eczema I Very potent • Clobetasol propionate 0.05% (Dermovate) Potent • Fluticasone propionate 0.05% (Cutivate) Moderate • Betamethasone • Betamethasone valerate 0.1% valerate 0.025% (Betnovate) Mild (Betnovate RD) • Hydrocortisone • Clobetasone butyrate 0.5-2.5% 0.05% (Eumovate) A 6-month-old boy presents with a severe exacerbation of eczema. His skin is markedly inflamed all over. He has been breast-fed from birth. His mother has a sore on her lip. His temperature is 37.8°C. Courtesy of Skin Deep https://dftbskindeep.com/ Ask: has anyone in the family currently got cold sores? What? HSV infection of eczematous skin Presentation: • Grouped vesicles and punched-out erosions Eczema • Lesions can become widespread and coalesce • 2º bacterial infection (golden crust) herpeticum • Unwell looking child Management • Admit child for urgent dermatology review, swabs and IV aciclovir • May also give concurrent Abst if cannot distinguish between viral vs bacterial infection (1 line = flucloxacillin) Kawasaki disease • Vasculitis • Presentation: • Fever >5 days & resistant to paracetamol • Conjunctival ‘injection’ • Rash • Cervical lymphAdenopathy • Strawberry tongue & Red cracked lips • Hands and feet - Red palms and soles of feet – later, peeling • Investigations: • High CRP and ESR • Delayed rise in platelets (week 2) • Management: aspirin + IVIg • Follow up: echo (risk of coronary artery aneurysms) I Oncology I • 2mo presents to ED with widespread purpuric rash and abdominal distension • Urgent referral to paeds made and BM aspirate + trephine Neuroblastoma • Origin: neural crest of SNS (adrenal medulla) • Prognosis: • Median age of onset: 20 mo • Age and stage are important • Presentation: • Better differentiation = better prognosis • 60% present w/ metastatic disease → LNs, BM, liver, skin (blueberry muffin baby), orbits, • Poor if dura • >1 • Abdominal mass • Amplification of MYC-N • Hepatosplenomegaly • Management: • Pallor, weight loss • Bone pain, limp • Some resolve spontaneously • Localised disease: surgery alone • Investigations: FBC, urinary catecholamines • Metastatic disease: chemo chest, MRI heaf, MIBG, BM trephine primary site/ • Many patients with high-risk disease will relapse or be refractory to primary treatment Cont … Neuroblasts: small round Neuropil: eosinophilic Ganglion cells: mature Schwannian stroma: blue cells fibrillary acellular material neuroblasts, with abundant cellular wavy stroma pink cytoplasm and a large formed from Schwann nucleolus with a prominent cells nucleolus May be arranged in Homer-Wright Neuroblastoma = most immature, pseudo-rosettes with a centre of <50% Schwannian stroma neuropil Undifferentiated form: no neuropil Poorly differentiated form: some neuropil, <5% ganglion cells Differentiated form: neuropil, >5% ganglion cells Neuroblastoma 🡪 ganglioneuroblastoma 🡪 ganglioneuroma Which feature of the history led to A 4 yo girl, Jane, is seen in the urgent her urgent referral? paediatrics clinic after being referred by A. Abdominal mass her GP yday who felt a mass in her R side. She is complaining of pain on the B. High BP same side. She has no other GI/ urinary C. Abdominal pain Sx, but GP did find her BP higher than D. Absence of the normal range for her age on 3 urinary readings. symptoms E. <5yrs • Origin: undifferentiated mesodermal tumour of intermediate cell mass • Classic triad of painless haematuria, loin pain, abdominal pass • Less common cause of renal mass mesoblastic nephroma (benign) in infancy (reversed incidence after infancy) • Normally affects <6 yo • Commonly unilateral, can be bilateral Wilms’ tumour = • Mutated WT1 gene on Chr 11 → nephrogenic rests (precursor lesion) nephroblastoma • a/w BWS, WAGR syndrome • Histology: • SRBCT • Triphasic: metanephric blastema, stromal cells, epithelial cells • Anaplastic Wilms’: nuclei 3x normal size, hyperchromatic and abnormal mitosesCont … Investigations: • Check BP • Basic bloods • Imaging: USS, CT+- MRI abdo, CXR/ CT chest for staging Management: • Chemo & total nephrectomy • RT if advanced • Prognosis is goodHydrocephalus •Sutures have not yet fused → disproportionate increase in head circumference •Secondary to either • Obstruction within ventricles (Chiari malformation, posterior fossa tumour), or • Obstruction at arachnoid villi (meningitis) •Setting sun sign •Management: VP shunt YUM! These rosettes are Medulloblastoma delicious. •Precursor: primitive neuroectodermal tissue •Arises from cerebellar vermis •Presentation: • Cerebellar syndrome: clumsiness (ataxia), poor coordination, difficulty walking • Features of raised ICP: morning headaches, bulging anterior fontanelle, N&V, diplopia … from which CN defect? •Diagnosis: cranial MRI w/ contrast Small round blue cells •Treatment: resection; exquisitely Homer-Wright rosettes radiosensitiveCraniopharyngioma • Origin: remnants of Rathke’s pouch (ectoderm) • Supratentorial (suprasellar space) • Bimodal age distribution: 5-14 yo age, 50-70 yo age • Clinical features: • Bitemporal hemianopia • Disconnection hyperprolactinemia • Hyperphagia, obesity • Hypopituitarism Adamantinomatous type: • Workup: • Visual field testing cysts/ nodules of lamellated, calcified • Labs: pituitary/ endocrine profile wet keratin • Brain imaging (MRI, CT) Cholesterol crystals • Biopsy I Cardiology Tetralogy of Fallot arteriesition of the great Cyanotic Persistent truncus arteriosus heart disease Total anomalous pulmonary venous drainage Tricuspid atresia •Defects: • VSD • Pulm stenosis (RVOT obstruction) • RVH • Overriding aorta •Presentation: • At 1-2 months • ‘tet spells’ T etralogy of •Examination: • Clubbing Fallot • murmur?n systolic murmur at left sternal edge - what defect causes the •Investigations: • CXR - “boot-shaped” heart • Echo •Management: • Surgical - intermediary shunts (Blalock-Taussig shunt), balloon dilatation, definitive surgery at 6 monthsTGA • Two parallel circulations • Most occur with VSD, ASD or PDA • Usually presents around day 2 of life when DA closes • Management: • Medical - prostaglandins • SurgeryHaematology • IDA • Haemoglobinopathy • HS • Thalassaemia Pallor • Haemolytic anaemia • G6DP • Sickle cell • ALL ALL • Accounts for 80% of leukaemia in children • Management: chemo • 2-5 years • Poor prognostic indicators: • a/w T21, Klinefelter’s syndrome • WCC >100 x 109/L • General features: malaise, anorexia • <2 or >10 • Bone marrow infiltration:- pancytopenia • Male • Anaemia: pallor, lethargy • T-cell origin • Neutropenia: infection • Philadelphia chromosome • Thrombocytopenia: bruising, petechiae, nose bleeds • Slow response to Rx: more than 5% • Bone pain blasts persisting on d28 • Reticulo-endothelial infiltration: • Investigations: • FBC, BM aspirate + trephine, coag screen, LP, CXR • Low platelets • ITP • Abnormal clotting • Haemophilia Excessive • DIC • Meningococcal bruising • Vasculitis • HSP • Bone marrow disorder • ALL • NAI • Concerning features: • Injury to immobile child • Mechanism inconsistent with injury • Vague hx • Delayed px to ED • Parent/ carer aggressive/ unconcerned • Recurrent px to ED/ previous injury (different coloured bruises) NAI • Background of RFs – low BW, mother <30, unwanted pregnancy, stress, poverty • Concerning injuries: • Triangle of safety • Bruising in shape of hand, teeth, implement Full history and examination Investigate both for medical cause of bruising and NAI • Height and weight + head circumference in infants (increased size -> bleed?) • Bloods: FBC, blood film, clotting screen • If bony tenderness or strong suspicion of NAI 🡪 skeletal survey • CT head if head injury <1yr Document, document & document again • Use a body map to document all bruising / injuries and get consent from parents to take photos if needed. • Fully document any concerns and discussions with parents / NAI actions child Escalate if concerned • Keep parents informed – be open and honest about the process (this is usually done by a senior paediatrician) • Talk to senior / child safeguarding lead • intervention form) or call directlylling out a SIF (statutory • Admit to paediatric ward as a place of safety if needed. • If parents try to leave against medical advice, then contact police (in preference to security) • Consider siblings / other children potentially at riskRenal A 4-year-old boy presented to the GP with a 7-day history of swollen eyelids. He is generally well. Urine dip: WCC -, blood-, nitrites-, protein++. Podocyte pathology Clinical features • Proteinuria (>3.5g/ 24 hrs) • Oedema: periorbital, scrotal, leg and ankle • Hypoalbuminaemia (<30g/L) • +/- hyperlipidaemia, thrombophilia, clotting risk Nephrotic Most common cause in children: minimal change disease • Steroid sensitive → give pred po for 8 weeks • If no response → renal biopsy necessary syndrome • Biopsy •Normal glomeruli on light microscopy •EM → fusion of podocytes/ effacement of foot processes Prognosis: rule of 3 • 1/3 never recur • 1/3 have infrequent recurrence • 1/3 have frequent relapsesAcute nephritis •Acute GN: immune-mediated damage to glomerulus •Urinalysis: active urinary sediment (blood++, protein++, red cell casts) •Post-infectious: GABHS • 1-2 weeks after URTI • ASOT titre • Mx: 10d course of Pen-V •IgA nephropathy • 2-3 days after URTI •HSP (systemic form of IgA nephropathy) Most common cause of acute paediatric renal failure Triad : 1. Microangiopathic haemolytic anaemia Haemolytic 3. AKI w/ haematuria and proteinuria uraemic Cause: E. coli O157:H7 → haemorrhagic colitis syndrome DO NOT give abxsChild psychiatry M > F monthsbefore age of 3 and can occur in first few Triad of •Difficulties with social relationships •Problems with communication •Absorbing and narrowing interests Autistic Presentation •Attachment to unusual objects spectrum •Insistence on sameness (only eats white things) •Restricted range of interests/ activities disorder •Sensory hypersensitivity Management •Parental support and education •MDT: GP, paediatrician, CAMHS, charities and support groups, SEN coordinator, applied behavioural analysis, environmental modifications Asperger’s → relatively unimpaired language & intelligence A 6-year-old boy is brought to see you by his mother who is concerned with his school performance. She reports he is unable to pay attention in his classes for more than a few minutes, as a result of which he is falling behind his peers and getting in trouble a lot due to getting distracted and making impulsive decisions. Furthermore, in the last 6 months he has also been having similar problems at home, switching rapidly between activities. • DSM-5 = ADHD is a condition incorporating features relating to inattention and/or hyperactivity/impulsivity that are persistent • Triad of • Inattention • Hyperactivity • Impulsivity • Symptoms normally present by age 7, and in at least 2 ADHD settings (home AND school) • Management • NICE – 10 week watch and wait period • Classroom intervention/ parental education • Drug therapy • If 5 yo or older → methylphenidate • Give on 6-week trial basis • SEs: abdo pain, nausea → monitor height/ weightMiscellaneous I Abdominal wall defects Omphalocele Gastroschisis • Central defect of anterior abdominal wall •allowing extrusion of abdominal content cord • abdomen of midgut rotation and return to • Abdominal cavity may be too small • RFs: young parents, smoking, maternal infection (= socioeconomic deprivation) • Wharton’s jelly + peritoneum) • Bowel is not covered • Baby at risk of fluid, electrolytes and heat • Associated anomalies loss • Management: •Management • Staged closure • Surgery immediately following delivery •Rule of 3 (Wessel criteria) • Resolves by 3 (to 4) months of age • Lasts more than 3 hours per day Infantile colic • Occurs more than 3d/week • Persists longer than 3 weeks •Infant is otherwise wellOligoarthritis (persistent) F: 1-6yrs 1-4 joints (knee, ankle, wrist common) Chronic anterior uveitis, leg length discrepancy Oligoarthritis (extended) F: 1-6yrs >4 joints after 6mo Chronic anterior uveitis Polyarthritis (RF negative) F: 1-6yrs Symmetrical large and small joints often with finger Low-grade fever involvement Polyarthritis (RF positive) F: Symmetrical large and small joints often with finger Rheumatoid nodules 10-16yrs involvement Systemic arthritis 1-10yrs Oligo or polyarthritis Acute illness, malaise, high daily fever May have muscle aches and pains but initially no Salmon pink macular rash arthritis Lymphadenopathy Anaemia Raised ESR, neutrophils etc. Psoriatic arthritis 1-16yrs Usually asymmetrical large and small joints, dactylitis Psoriasis, nail pitting, chronic anterior uveitis Enthesitis-related arthritis M: Lower limb, large joints initially, spinal / sacroiliac Enthesitis (localised inflammation of insertion of tendon / 6-16yrs involvement later ligaments e.g., achilles tendonitis) HLA-B27 Undifferentiated M: Overlapping 1-16yrs Inguinal hernia Hydrocele Patent processus vaginalis Fluid collecting in a patent processus vaginalis (young boys) Neonates → risk of strangulation R/O testicular ca in older men Cannot get above it CAN get above it Does not transilluminate Transilluminates 6/2 rule Reassure (resolves by 2 yo age) Lump in the groinLump in groin cont. • Testicular torsion • Urological emergency! •Prehn’s sign testicle, N&V, negative • Surgery → bilateral fixation • Consent for orchidectomy • Lymph nodes → lymphadenopathy • Malignancy– TB • Systemic inflammatory conditions – JIABedwetting (nocturnal enuresis) •Dry by day + night at 3-4 years •Plot height and weight on •Primary or secondary to chart, perform urinalysis, • Constipation inspect genitalia/ spine, do • Diabetes lower limb neurology • UTI • Psychosocial •Management •2ry if dry for 6 mo previously • Advice on fluid intake, diet, •Assess fluid intake/ toileting habits toileting •Keep diary for 2 weeks • Reward systems •Ask about constipation/ soiling, • Enuresis alarm if <7 yo development/ behaviour, weight, polydipsia • trial for 4 weeks.smopressin-