Phase 3a

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Paediatrics

• Phase 3a
• Emma Faragher Jenny Hosty

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Contents

• Respiratory
• Asthma
• Infections
• Prematurity
• Cystic Fibrosis
• Cardiology
• Congenital heart disease Rheumatic fever
  Infective endocarditis
• Renal

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Wheeze

• A polyphonic expiratory sound
• Due to airway restriction and narrowing
• Children with wheeze can be very panicked
• As can their parents!
• Especially if it is the first time
• 50 of children will wheeze at some point in the
  first 3 years of life

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Asthma

• Usually occurs in atopic individuals
• Affects 15-20 of children and causes 20 deaths
  per year in the UK
• Narrowing of airways due to swelling and
  bronchiole constriction
• Made worse by URTIs

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Asthma clinical features

• Wheeze on more than 1 occasion
• Symptoms worse at night and early morning
• Symptoms have triggers (exercise, pets, dust,
  cold air, emotion, etc)
• Interval symptoms between acute exacerbations
• Hx of atopy (patient or family)
• Symptoms are reversible
• Normal examination between exacerbations
• Hyperinflation, generalised polyphonic expiratory
  wheeze and prolonged expiratory phase found in
  long standing asthma

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Asthma Management

• Acute severe and life threatening asthma
• Steroids
• Nebulised salbutamol (short acting beta 2
  agonist)
• Chronic asthma
• Short acting beta-2 agonists (last 2-4 hours)
  salbutamol, terbutaline
• Long acting beta-2 agonists (last 12 hrs)
  salmeterol, formoterol always used with inhaled
  corticosteroids
• Anticholinergic bronchodilators ipratroprium
  bromide young infants when other
  bronchodilators ineffective and in the treatment
  of severe acute asthma
• Inhaled corticosteroids

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Asthma management

• Add on therapies in children under 5
• Leukotriene receptor antagonist montelukast
• Slow release oral theophylines (high side effect
  profile)
• Other therapies
• Oral prednisolone in severe persistent asthma
  when other treatment has failed
• Anti-IgE therapy (omalizumab) injectable
  monoclonal antibody used in sever atopic asthma
  under specialist care
• Avoid allergens!
• Stop smoking in the house and car (new rules make
  the latter illegal)

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Signs of severe asthma

• Wheeze and tachpnoea
• gt50 aged 2-5, or gt30 aged gt5
• Increasing tachycardia
• gt130bmp aged 2-5 or gt120bpm aged gt5
• Use of accessory muscles and chest wall recession
• Marked pulsus paradoxus (difficult to measure)
• Breathlessness interferes with talking
• Cyanosis, fatigue and drowsiness are late signs!
• Silent chest on auscultation is an emergency!
• O2 sats lt92 on air means severe/ life
  threatening asthma

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Bronchiolitis

• Commonest resp infection of infancy
• Rare in children over 1yr old
• 80 are due to Respiratory syncytial virus (RSV)
• Other causes
• Human metapneumovirus, parainfluenza virus,
  rhinovirus, adenovirus, influenza virus and
  mycoplasma pneumoniae
• Dry cough (sounds sharp)
• Increasing breathlessness with feeding difficulty
• Tachypnoea
• Subcostal and intercostal recession
• Hyperinflation of the chest
• Cyanosis or pallor
• Fine end-inspiratory crackles
• High-pitched wheeze
• tachycardia

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Bronchiolitis

• Supportive
• Humidified O2 via nasal cannulae
• Monitor for apnoea
• Fluids IV or NG
• Assisted ventilation with CPAP/ full ventilation
• Infection control measures
• Most recover in 2 weeks
• Half have recurrent cough and wheeze
• Rarely results in permanent airway damage
• Monoclonal RSV antibody in high risk pre-term
  infants palivizumab in monthly IM injections

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Pneumonia

• Peak incidence in infancy and old age but common
  in children
• No causative pathogen found in 50
• Viruses more common in younger children, bacteria
  in older
• Newborn organisms from mothers genital tract,
  group B strep, gram ve enterococci
• Infants and young children RSV, strep
  pneumoniae, H influenzae, Bordetella pertussis
  and chlamydia trachomatis (staph aureus is
  uncommon but severe)
• Children gt5 years mycoplasma pneumoniae, strep
  pneumoniae, chlamydia pneumoniae
• All ages think of TB!

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Pneumonia

• Fever
• Difficulty breathing
• Cough
• Lethargy
• Poor feeding
• Unwell child
• Tachypnoea
• Nasal flaring
• Chest indrawing
• End-inspiratory course crackles over affected
  area
• Dullness to percussion
• Reduced breath sounds
• Bronchial breathing

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Pneumonia

• Localised chest abdo or neck pain in pleural
  irritation
• CXR shows consolodation if strep pneumoniae
• Nasopharangeal aspirate to identify viral causes

• Most managed at home
• Hospital if
• O2 sats lt93
• Severe tachypnoea and difficulty breathing
• Apnoea
• Not feeding
• Beware sepsis

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Respiratory Distress Syndrome

• Surfactant deficiency usually excreted by type
  2 pneumocytes
• Common in infants born before 28 weeks gestation
• Surfactant therapy given via tracheal tube
• Symptoms
• Tachypnoea gt60 breaths per minute
• Signs of laboured breathing chest wall recession
  and nasal flaring
• Expiratory grunting (creates positive pressure
  during expiration to maintain functional residual
  capacity)
• Cyanosis if severe
• Characteristic CXR with bilateral pneumothoraces
• Give oxygen and either CPAP or artificial
  ventilation as needed

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Cystic Fibrosis

• Genetic condition
• 1/25 Caucasians are carriers
• Live birth rate of 1/2500
• Defective cystic fibrosis transmembrane
  conductance regulator (cyclic AMP dependent
  chloride channel in cell membranes)
• Gene located on chromosome 7
• Genotype and phenotype links are (relatively)
  weak for lung disease
• Screen done as part of the heel-prick or Guthrie
  test in newborns
• Other presentation chronic infection, poor
  growth, malabsorption
• Diagnosis via sweat test (chloride elevated to
  60-125mmol/L)
• Manage symptoms lung function tests in older
  children, treat infections (remember likelihood
  of atypicals) and prophylaxis with
  flucloxacillin, physiotherapy twice daily.
  Several other medications.
• Bilateral lung transplantation in end stage
  disease.

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The normal anatomy and physiology
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Congenital Heart Disease

• Which is a right to left shunt? ? i.e. cyanotic
• Which is left to right shunt?
• Outflow obstruction

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Ventricular Septal Defect

• COMMONEST congenital heart condition
• The smaller the defect, the louder the murmur
• Small VSD (lt3mm)
• Loud pansystolic murmur at lower left sternal
  edge
• Normal Ix
• Close spontaneously, no tx needed
• Large VSD (gt3mm)
• Presents with HF breathlessness, FTT, recurrent
  chest infections, tachycardia, hepatomegaly
• Soft pansystolic murmur, or no murmur
• CXR cardiomegaly, enlarge pulmonary arteries,
  pulmonary oedema, increased pulm vascular marking
• Management
• Diuretics and captopril for HF
• Additional calorie input
• Surgery at 3-6m to prevent long term HF and
  pulmonary HTN

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Atrial Septal Defect

• Left to Right shunt
• 80 defect in ostium secundum
• Symptoms
• Usually asymptomatic
• ? Or mild fatigue, recurrent chest
  infections
• Signs
• Ejection systolic murmur at upper left sternal
  edge
• (caused by increased flow across pulmonary
  valve)
• Fixed and widely split S2
• Ix Echo is diagnostic
• Management
• Large lesions surgery at 3-5 years (cardiac
  catheterisation with insertion of occlusion
  device) to prevent RHF and Eisenmengers

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Atrioventricular Septal Defect

• Most often seen in Downs
• Often detected antenatally
• No murmur
• Pulmonary hypertension
• Surgical repair at 3-6m

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An F1 notes that a neonate has a pink torso
and upper extremities with blue lower
extremities. It is also noted that the newborn
has bounding pulses and a continuous rumbling
murmur.
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Patent Ductus Arteriosus
Failure of the DA to close by 1 month after
expected date of delivery Blood flows from aorta
to pulmonary artery Signs Continuous machinery
murmur beneath L clavicle Widened pulse pressure
causing collapsing/bounding pulse Normally
asymptomatic Normal ECG and CXR unless
hypertrophy occurs Echo is diagnostic Management
Indomethacin/Ibuprofen (NSAID) - inhibits
prostaglandin production If still open - closure
with a coil or occlusion device by cardiac
catheterisation by 1 year of age RF Prematurity R
ubella infection in 1st trimester
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A 2-week-old newborn is brought to the GP because
his lips have turned blue on three occasions
during feeding. BP is 75/45, Pulse 170, and RR
44. A harsh systolic ejection murmur is heard
at the left upper sternal border. CXR shows a
small boot shaped heart and decreased pulmonary
vascular markings.
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Less commonly

• A four-year-old boy is brought to the GP by his
  concerned mother who states that he
  intermittently turns blue. She reports that he
  has been growing and developing normally, but
  recently he has been having crying fits where he
  turns blue and then squats down into a ball.
  Vital signs are stable. Physical examination
  reveals a pansystolic murmur at the left lower
  sternal border.

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Tetralogy of Fallot
pulmonary stenosisright ventricular hypertrophy
(RVH)overriding aorta VSD

• Symptoms
• Hypercyanotic spells irritability,
  breathlessness, pallor
• (if older child squatting)
• FTT
• Signs
• Loud harsh ejection systolic murmur at L upper
  sternal border
• Clubbing in older children
• Investigations
• - CXR boot-shaped heart, decreased pulmonary
  vascular markings
• - Echocardiography for definitive diagnosis

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Tetralogy of Fallot
Management If infant is very cyanosed, a shunt
is put in to increase pulmonary blood flow
(modified Blalock Taussig shunt) Surgery at 6
months to close VSD and relieve RV outflow
obstruction Cyanotic spells usually self
limiting if prolonged can give O2, IV fluids,
morphine
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• A 2 day old infant is found to have blueish
  decolouration of the skin and mucous membranes,
  and has tachypnoea and tachycardia. O2 saturation
  is 75. No murmur can be heard. It was a normal
  delivery and the neonate was well yesterday.
• A CXR shows mild cardiomegaly and an egg on
  side appearance of the cardiac shadow.

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TGA
Aorta from right ventricle and pulmonary artery
from LV Incompatible with life UNLESS mixing
allowed e.g. PDA Presentation Profound cyanosis
presents on day 2 following DA closure Usually no
murmur Loud S2 CXR narrow upper mediastinum with
egg on side cardiac shadow, increased
pulmonary vascular marking Echo is
diagnostic Treatment Maintain DA with IV
prostaglandin 20 require urgent balloon atrial
septostomy Arterial switch procedure in neonatal
period i.e. asap
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Outflow Obstruction

• Sick child
• present with shock and HF in the neonatal period
  (unless diagnosed antenatally) manage is ABC and
  commence prostaglandins until surgical repair can
  be done
• Coarctation of the aorta
• Hypoplastic left heart syndrome
• Interruption of the aortic arch
• Well child
• asymptomatic or only mild symptoms, present
  later or incidentally
• Aortic Stenosis
• Pulmonary Stenosis
• Adult-type Coarctation

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Aortic Stenosis

• Asymptomatic or SOBOE, decreased exercise
  tolerance
• Small volume, slow rising pulse
• Ejection systolic murmur radiating to the neck,
  apical ejection click
• Treatment depends on severity may need balloon
  valvotomy and later aortic valve replacement

Pulmonary Stenosis

• Mostly asymptomatic
• Ejection systolic murmur upper LSE, ejection
  click
• May develop RV hypertrophy
• Intervention only required when there is a
  significant pressure gradient
• (trans-catheter balloon dilatation )

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A 7-year-old-boy is seen for the first time by
his GP. His parents report that he tires easily
and often complains of weakness in his
legs. Physical exam shows a healthy boy with a
blood pressure of 141/91. His lower extremities
are slightly atrophic with a mottling appearance
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Coarctation of the Aorta

• Narrowing of the lumen of the aorta just distal
  to the subclavian artery
• Causes severe obstruction to LV
• Associated with Turners syndrome and bicuspid
  aortic valve
• Presentation
• Neonates present on 2nd day of life with acute
  circulatory collapse
• severe HF dyspnoea (especially on exertion),
  poor feeding, sweating, tachycardia/pneoa, cool
  peripheries, gallop rhythm.
• Absent femoral pulses
• Severe metabolic acidosis
• OR
• Presents in childhood with secondary
  hypertension, dyspnoea on exertion, syncope

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Coarctation of the Aorta
O/E Blood pressure higher in upper limbs than
lower limbs may be higher in right
arm Weak femoral/distal pulses, lower leg skin
mottling Continuous murmur over collateral
vessel in the back CXR Reverse 3 sign or rib
notching due to collateral circulation
through intercostals Diagnosis made by CT,
echo gives further evidence Management Surgical
correction or balloon angioplasty /- stent
insertion ASAP Endocarditis prophylaxis 25
develop secondary hypertension regardless
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Causes of Congenital Heart Disease?

• Maternal Infection Rubella (PDA)
• Chromosonal abnormalities
• Downs Syndrome (AVSD, VSD, ToF)
• Turners Syndrome (AS, CoA)
• Fetal alcohol syndrome (AVSD, VSD, ToF)
• Maternal warfarin therapy (PS, PDA)
• Maternal SLE anti-Ro/anti-La antibody can cause
  heart block

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Rheumatic Fever

• Group A ßhemolytic strep i.e. strep pyogenes
• Mostly aged 5-15 years low incidence in
  developed countries
• Clinically 1) Pharyngeal infection
• 2) Latent period of 2-6 weeks
• 3) Polyarthritis, mild fever and malaise
  develop
• Management
• Bed rest
• Penicillin (or erythromycin)
• NSAIDs and/or high dose aspirin
• Corticosteroids if not resolving, and
  antibiotics if persisting infection
• If HF develops, treat with diuretics,
  ACE-inhibitors, digoxin
• Complications
• Pericardial effusions
• Chronic Rheumatic heart disease most commonly
  mitral stenosis
• Congestive cardiac failure

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Jones Criteria

• 2 major or 1 major and 2 minor plus evidence of
  preceding group A strep infection e.g. raised ASO
  titre, group A strep on throat culture
• Major
• Pancarditis 50
• Polyarthritis (flitting ankles, knees,
  wrists) 80
• Syndenham chorea
• Erythema marginatum
• Subcut nodules
• Minor
• Fever
• Polyarthralgia
• Hx rheumatic fever
• Raised acute phase reactants
• Prolonged PR interval

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Infective Endocarditis

• Cause?
• a-hemolytic streptococcus e.g. strep viridans
• Note - Staph aureus is more common in adults, esp
  IVDU

• Symptoms and signs?
• Fever and malaise are the commonest symptoms
• New/changing heart murmur
• Weight loss, cough, anaemia
• Vascular phenomena splinter haemorrhages,
  Janeway lesions, conjunctival haemorrhage,
  haematuria, neuro signs from cerebral infarction
• Oslers nodes (painful)
• Clubbing (late sign)

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Infective Endocarditis

• Diagnosis?
• Duke Criteria - 1 major and 1 minor, or 3 minor
• Major
• Positive blood culture for IE (2 separate
  cultures needed)
• Evidence on echo
• Minor
• Prediposing factors e.g. congenital heart
  disease, IVDU
• Fever gt38
• Positive blood culture or echo not meeting
  criteria for major
• Vascular phenomena (infarcts, Janeway lesions
  etc)
• Immunological (glomerulonephritis, Oslers nodes,
  Roth spots)

• Therefore investigations
• Blood cultures x2
• Echocardiogram
• Bloods anaemia, raised ESR

• Treatment
• 6 weeks IV abx penicillin and aminoglycoside
  (e.g. streptomycin)
• Surgical removal of any infected prosthetic
  materal

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RENAL DISORDERS

• UTI
• Nephrotic syndrome most often minimal change
  disease
• Acute nephritis post strep, HSP
• Haemolytic Uraemic Syndrome
• Not covered AKI/CKD similar to in adults,
  cause more likely to be structural malformations

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Urinary Tract Infections

• Present as very general symptoms in children!
• Commonly caused by E. Coli, then Klebsiella,
  Proteus, Pseudomonas and Strep. faecalis
• More common with structural abnormalities
• Management
• If lt3months old refer to hospital for IV abx
  until afebrile
• If gt3months old and acute pyelonephritis/ upper
  UTI give oral abx (co-amoxiclav) 7-10 days OR IV
  abx (cefotaxime/ ceftriaxone) 2-4 days followed
  by 7-10 days of oral
• If cystitis/ lower UTI in children give oral abx
  3 days

Prevention High fluid intake Regular
voiding Complete emptying Prevent
constipation Good hygiene Prophylactic abx
(usually trimethoprim)
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Nephrotic Syndrome

• Proteinuria leads to low plasma albumin and
  oedema
• Clinical signs
• Periorbital oedema (especially on waking)
• Scrotal/ vulval, leg and ankle oedema
• Ascities
• SOB (pleural effusions and abdominal distension)
• Investigations
• Urine dipstick
• Bloods (FBC, ESR, UEs, Creat, Albumin,
  Complement)
• Antistreptolysin O/ anti-DNAse B titres and
  throat swab
• MSU (microscopy and culture)
• Hep B and C screen
• Malaria screen if been abroad

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Nephrotic syndrome

• 85-90 resolve with corticosteriods
• 60mg/m2 per day of prednisolone for 4 weeks
• Then 40mg/m2 alternate days for 4 weeks
• Complications
• Hypovolaemia
• Thrombosis
• Infection
• Hypercholesterolaemia
• Steroid-resistant nephrotic syndrome
• Diuretics, salt restriction, ACE
  inhibitors,NSAIDS
• Specialist care!

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Acute nephritis

• Decreased urine output
• Hypertension (may cause seizures)
• Oedema (around eyes characteristic)
• Haematuria and proteinuria
• Manage with attention to water and electrolyte
  balance, occasionally diuretics
• Post Strep culture/ raised ASO/anti-DNAse B
  titres and low compliment C3 (normalise after 3-4
  weeks)

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Henoch-Schonlein Purpura

• Most common vasculitis in children
• IgA mediated
• Features
• Usually winter months
• Mildly ill with low-grade fever
• Symmetrical, erythematous macular rash especially
  on backs of legs, buttocks and ulnar sides of
  arms
• Macules to purpuric lesions in 24 hrs (resemble
  bruises if coalesce)
• Abdo pain and bloody diarrhoea (precedes rash)
• Joint pain especially in knees and ankles
• Usually self limiting
• NSAIDS (caution in renal insufficiency) and
  corticosteroids

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Haemolytic Uraemic Syndrome

• Triad Acute renal failure, microangiopathic
  haemolytic anaemia and thrombocytopenia
• Usually secondary to GI infection with
  verocytotoxin producing E. Coli or Shigella
• Bloody diarhoea
• Then toxin localises to kidney causing
  intravascular thrombogenesis (coagulation cascade
  activated but clotting normal!). Platelets used
  up. Occluded microcirculation damages red blood
  cells.
• Dialysis in typical HUS with diarrhoea good
  outcome
• If no diarrhoea has high risk of hypertension and
  chronic renal failure with a high mortality
  (familial and relapsing)

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Questions?Comments?Declarations of impending
doom?No time to go home then.
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And remember youll be fine!A few more months
and youll be enjoying Christmas holidays
(unlike some of us)