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Paeds Summary Sheet
Paeds Summary Sheet
Term: 37-42 weeks Normal birth length: 50-60 cm Normal birth-weight: 2.5-3.5 kg Caloric requirements
● Tells us about growth potential ● Measurements made a year apart ● Parental heights give indication Puberty (females): 8cm/year peaking
● Greulich-Pyle and Tanner- ● 4-6cm/year in childhood of target height at 13 years, 1 year after breast
Whitehouse systems budding; by menarche growth almost
● If bone age>>chronological age: Height 2-height 1 Interval (decimal complete with 3-5cm more post
o Tall stature: worry years) Girls: (Father’s height + mother’s
about brain tumour height -12.5cm) / 2
o Short stature: worry b/c Puberty (males): testicular volume 10-
less room for growth 15mL in mid-late puberty→reach
● If bone age<<chronological age: maximum height
Boys: (Father’s height + mother’s
o Short stature: promising
height + 12.5 cm) /2
growth still ahead
● Boys start pubertal growth spurt
2 years later, with greater
intensity
Hyperthyroidism
Simple obesity
Homocystinuria **
● Temporary delay in the skeletal growth and thus height of a child with no other <1 year: 110-160 Neonate N=30-50 T>60
physical abnormalities causing the delay
● Variant of normal growth 2-5 years: 95-140 Infants N=20-30 T>50
● Most common cause of short stature and delayed puberty (M>F)
● Psychological difficulties, warranting treatment for some individuals 5-12 years: 80-120 Young Children N=20-30 T>40
Investigations: Delays in growth and sexual development are quantified by bone age →
Growth and development are appropriate for an individual's biologic age (skeletal age)
rather than for their chronologic age.
DEVELOPMENT
Gross Motor Fine Motor (and vision) Speech and Hearing Social
Symmetrical postures
6 weeks Raises head to 45 degrees Follows moving object or face Smiles responsively
6 months Sits without support (round Palmar grasp Turns to soft sounds out of Puts food in mouth
back) sight
1 year Walking unsteadily 2-3 words other than dada, 10-12: Waves bye bye
mama
Broad gait Peek a boo
15 months Walks alone, steadily 16-18: Marks paper with 18: 6-10 words 18: Holds spoon and gets food
crayons to mouth
Shows two parts of the body
18: Tower with 3 blocks 18-24: Symbolic play
20-24: 2 ore more words to
2 yrs: “ 6 blocks, line make simple phrases 2 yrs: dry by day; pulls off
some clothing
3 yrs: ‘ 9 blocks, circle 2.5-3 yrs: Talks constantly in 3 yrs: Parallel play, takes turns
3-4 word sentences
4 yrs: cross
4.5: square
<5: triangle
CHILDHOOD INFECTIONS
Occult Bacteremia High index of suspicion for illness if: Empirical Abx with rectal T > 38 and >15,000 WBC
▪ Fever (>38) rectally that lasts <1 week with >1500 bands
▪ WBC >15, 000
▪ Children <36 months
▪ Blood cultures are positive ▪ Neonates: GBS, E. coli, listeria
▪ Bacteria present in blood but no obvious focus
▪ If temperature is >39→ urine, stool and blood ▪ Infants: S. pneumonia
of infection
culture, CXR
▪ Likely organisms: S. pneumonia, N.
▪ Treat empirically until results come back
meningitides, H. influenza Type B, salmonella
▪ If cultures are (+) for S. pneumonia but afebrile, Any infant or child that appears toxic must be
▪ Non-specific signs: poor feeding, decreased
repeal blood culture and consider LP admitted for IV Abx
activity, fussiness, not toxic looking
Immunizations Killed/Inactivated
Live attenuated
Viral
Viral
▪ Rabies
▪ Measles ▪ Influenza
▪ Mumps ▪ Polio (Salk)
▪ Rubella ▪ Influenza A
▪ Varicella ▪ Hepatitis B
▪ Nasal influenza
Bacterial
▪ Yellow fever
▪ Pertussis
▪ Diphtheria
Bacterial ▪ Tetanus
▪ Pneumococcal
▪ BCG ▪ HiB
▪ Oral typhoid ▪ Meningococcal
▪ Smallpox
MENINGITIS
Suspect this in any ill baby or child Pathogenesis Investigations Meningococcal Disease (Grp B)
Pathogens depend on age: URTI→bacteremia (blood stream)→ FBC, CRP (↑/↓), U/E ▪ If suspect→ give penicillinIM ASAP
seeding and inflammation of meninges ▪ Presents as meningitis or sepsis
Neonatal Period Blood glucose: elevated due to (meningococcemia)
stress o Mgt different
▪ Group B Strep (50%)
▪ E. coli (20%) Symptoms Depend on Age Blood Culture ▪ Endotoxin→vasodilatation→ third
▪ Listeria (7%) spacing →shock
▪ Misc GN (10%) Young Child Blood PCR: of causative ▪ Fluid resuscitation for profound shock
organism
▪ Misc GP (10%) ▪ Subtle—unusual crying
4 weeks-12 weeks ▪ Poor feeding—anorexia LP: definitive investigation Signs & Symptoms of Septicemia
▪ Lethargy, listlessness
▪ CSF analysis and culture ▪ Fever/vomiting
▪ GBS, E. coli, pneumococcus, ▪ Irritable
salmonella, listeria, H. ▪ Drowsy, quiet ▪ Abx treatment decision ▪ Cold hands/shivering
▪ Improves outcome ▪ Rapid breathing
influenzae ▪ High or low temperature
▪ Convulsions ▪ Do not delay in ▪ Stomach, joint, muscle pain
>3 month of age ▪ Nausea/vomiting neonates/infants ▪ Drowsy
▪ In older children, send
▪ Neisseria meningitidis Older Child other investigations first Petechial Rash
▪ Strep pneumonia
▪ H. influenza (rare with HiB) ▪ Fever ▪ Differentiated from other causes by a
▪ Vomiting Contraindications rapidly spreading, non-blanching,
Viral Causes petechial rash which may precede
▪ Photophobia* (late sign)
▪ Chills ▪ Systemically unwell other symptoms.
▪ Enterovirus (commonest in ▪ Signs of cerebral ▪ Trunk, lower extremities, skin folds,
<3mths) ▪ Severe headache herniation or ↑ICP mucous membranes, conjuctiva, and
▪ Herpes, influenza, rubella, ▪ Neck pain and stiffness ▪ GCS <8 palms of the hands or soles of the feet.
echo, coxsackie, EBV, ▪ Seizures* ▪ Abnormal pupils ▪ Petechiae and purpura +/-
adenovirus ▪ Kernig’s—resistance to ▪ Abnormal tone meningitis→ purpura fulminans
extending knee with flexed hip ▪ Papilloedema
Pathogens depend on underlying Complications
▪ Brudsinski’s—hips flex on ▪ Focal neurological signs
conditions: bending head forward ▪ Cardioresp compromise ▪ Waterhouse-Friderichsen
VP Shunt ▪ Obvious signs of syndrome→hemorrhage of adrenal
meningococcaemia glands secondary to sepsis→adrenal
▪ Clinical Signs of Meningitis
S. epidermidis ▪ Thrombocytopenia— crisis
▪ S. aureus worry about bleeding
Infection/sepsis: fever, pallor, cyanosis, ▪ Severe hypotension and septic shock
▪ Coliforms DIC, ↑HR, ↓BP, ↑ capillary refill time, ▪ Infection of skin over ▪ DIC
tachypnoea,↑WCC site ▪ Acidosis
Head Injury
▪ DO NOT DELAY Abx FOR ▪ Adreno-cortical insufficiency
▪ Raised ICP: ↑ BP with ↓HR, CT SCAN
S. pneumoniae if there is ▪ Renal and HF
CSF leak bulging/tense fontanelles, ▪ Normal or low WCC/ESR, BP <70, rash
▪ S. aureus papilloedema(blurring of optic disc onset w/in 12 hrs=poorer prognosis
▪ Pseudomonas margins), high-pitched cry, HA, emesis,
oculomotor or abducens palsies, apnea,
decorticate or decerebrate posturing,
stupor, coma
High vaginal swab to test for GBS—
antepartum penicillin Nuchal rigidity: stiffness
Treatment Complications
Normal Bacterial Viral TB
Initial Empirical Abx: Cefotaxime/ceftriaxone + ▪ ↑ ICP with herniation and seizures
Protein 0.3-2.0 ↑ N/↑ ↑ vancomycin ▪ Circulatory collapse (tachycardia,
hypotension, ↓ cap refill)
Glucose >2/3 of Normal/ N /↓ ↓ Abx depends on pathogen, resistance of local ▪ Septic shock→DIC
blood ↓ pathogens, and penetrance of CSF ▪ Focal neurological abnormalities—CN
palsies, stroke
WCC 5-15 ↑PMN ↑ ↑ <2 months: Triple therapy→cefotaxime, ▪ Hydrocephalus
Lymph Lymph gentamicin, and ampicillin (3/52)
No PMN 1000- ▪ Cerebral oedema
5000 10-100 50-500 >2months: ceftriaxone +/- vancomycin ▪ Brain abscess
▪ Arteritis/venous thrombosis
Gram + - + ZN If suspect ▪ Subdural effusions—esp in infants with
stain HiB
→ S. pnemoniae→vancomycin (7-10 days)
Aseptic/Viral Meningitis ▪ Seizure
→N. meningitides→ penicillin (5-7 days) ▪ Hearing impairment (haemophilus)
▪ HA, hyperesthesia in older children ▪ Highest mortality in neonates and
▪ Irritability and lethargy in infants → HiB→ ampicillin (7-10 days) adults
▪ Fever, nausea, vomiting, photophobia, neck, leg and ▪ Less: MR, developmental delay, visual
Dexamethasone now recommended for all types
back pain impairment
of bacterial causes (esp Hib and pneumococcal)—
▪ Exanthems= echovirus and coxsackie, varicella, measles,
improves neuro outcome (hearing), fever and U
and rubleaa
CSF protein—give before Abx
▪ Complications: Guillain-Barre, transverse myelitis,
hemiplegia, cerebellar ataxia and encephalitis (VZV), Prophylaxis—rifampicin for 2/7 (5mg/kg BD)
focal seizures coma and death (Herpes simplex), 8th indicated for Neisseria and HiB
nerve damage (EBV, mumps)
First Disease: Scarlet fever Scarlet Fever (GAS) Measles (rubeola) RNA Paramyxovirus
Second Disease: Measles ▪ Rare in young infant ▪ ‘9 day measles’ Dx: Clinical, viral C/S; serology (IgM
▪ Pharyngitis with exudate ▪ Prodrome 3-5 days response slow)
Third Disease: Rubella ▪ Sudden onset fever o Cough
Tx: Vit A if deficient, ribavirine in IC
Fifth Disease: Erythema ▪ Headache o Coryza
infectiosum ▪ Rash 2 days after (finely o Conjunctivitis Prevention: MMR vaccination
punctate @ trunk, peripheries, o Koplik’s spots
Sixth Disease: Roseola Infantum blanching) o +/- cervical
▪ Late desquamation lymphadenopathy
Complications
▪ Strawberry tongue ▪ Incubation 8-12 days
▪ Circumoral pallor o Fever Otitis media
o Rash→macular; head,
trunk, extremities Pneumonia
Croup
Diarrhea
Encephalitis
SSPE
Rubella (German measles) ▪ Mild fever + Rash→ similar to Fifth’s Disease Sixth Disease
measles; begins on face and
▪ ‘3 day measles’ spreads down—lasts 3 days ▪ Erythema infectiosum ▪ HHV-6—Roseola, exanthema subitum
▪ Mild, self-limited disease in ▪ Parvovirus B19 (DNA virus) ▪ Peaks in children <5, usually 6-15
▪ DX: Clinical, confirm with
children serology ▪ Mild systemic symptoms months
▪ Incubation: 14-21 days ▪ Slapped cheek ▪ Incubation: 5-15 days
▪ Tx: Supportive
▪ Contagious 2 days before and ▪ Prevention: MMR ▪ Lacy reticular rash over trunk and ▪ Prodrome 3-7 days: High fever and
5-7 days after rash extremities signs of URTI, occipital
▪ Post-auricular and occipital ▪ Rash can last up to 40 days! lymphadenopathy
lymphadenopathy Complications: Congenital rubella ▪ Arthritis ▪ Rash on 3rd day→ maculopapular on
▪ Forscheimer spots on soft syndrome if contracted during trunks, arms, neck, face; rose coloured,
palate pregnancy; polyarthralgia, Dx: Clinical, labs not routine except begins as papules
when Dx hydrops, then viral DNA in
▪ Polyarthritis (F>M) thrombocytopenia, encephalitis ▪ No studies necessary
fetal blood is helpful ▪ Treatment is supportive
Complications Complications
▪ Abortion ▪ Febrile seizures
▪ Stillbirth ▪ Encephalitis
▪ Hydrops fetalis
▪ Aplastic crisis in those with
hemolytic anemia
Mumps Dx: enzyme immunoassays for IgG Scabies DX: microscopic scraping
and IgM, ↑ serum amylase
▪ Paramyxovirus ▪ Due to Sarcoptes scabiei that
▪ Droplet and secretions Tx burrows and releases toxic
▪ Incubation: 14-24 days substances Treatment
Tx: supportive; steroids/NSAIDs for
▪ Contagious 1 day before and ▪ Intense itching at site of infection ▪ >2 months: permetrin 5% or Lindane
3 days after parotid swelling arthritis especially at night 1% (potentially neurotoxic) on entire
appears ▪ 1-2mm red papules with body from neck down
▪ Fever, HA, malaise excoriation, crusting, scaling ▪ <2months: 6% sulfur in petrolatum
▪ Unilateral or bilateral salivary Complications: meningo- ▪ Appears like thread-like burrows ▪ Launder all linens and clothes in hot
gland swelling (+parotids) encephalomyelitis, pancreatitis, under skin water to sterilize
▪ Arthritis thyroiditis, myocarditis, deafness, ▪ Infants→ can involve palms, face, ▪ Treat entire family
▪ Orchitis—can result in dacryoadenitis soles, scalp
sterility if bilateral ▪ Older children→between webs of
fingers, wrist flexors, anterior
axillary folds, ankles, areola,
buttocks, genitals
HF
▪ Bordetella pertussis Catarrhal: 2 wks→ cold-like Sx ▪ BARC mnemonic ▪ Doxycycline (>8yo) or amoxicillin
▪ Very contagious, by droplets ▪ Erythema migrans (>10cm) (<8 yo) for 14-21 days
▪ Re-infection possible Paroxysmal: 2-5 wks→severe ▪ Fever, malaise, HA ▪ If meningitis or carditis: penicillin or
coughing, inspiratory whoop, facial
▪ Whooping cough ▪ Lymphadenopathy ceftriaxone for 14-28 days
▪ Missed immunizations petechiae ▪ Uveitis ▪ Bell’s palsy: doxycycline or
▪ NPA culture Convalescent: 2 wks→ gradual ▪ Bells Palsy amoxicillin for 21 days
▪ PCR resolution of cough ▪ Carditis ▪ Late disease: give for 28 days or 2nd
▪ Lymphocytosis ▪ CNS: meningitis, neuropathy course if required
▪ Erythromycin for 14 days ↓ ▪ Late: arthritis of large joints
infectious period, may shorten
course of illness
▪ Treat all contacts
TORCH INFECTIONS
Toxoplasma gondii Rubella CMV
Respiratory System
NB. If nose, throat affected→ viral If tonsils or ears affected separately → bacterial
Common Cold Nasal discharge, stuffiness Infections viral URTI Nil Antipyretics (paracetamol)
Myalgia
Lethargy
Anorexia
Sore Throat Conjunctivitis Viral Throat Swab: not sensitive or Supportive and Symptomatic
specific
Pharyngitis Rhinitis Uncommon under 1 year Abx only for severe infection or GABHS
Rapid Ag testing
Tonsillitis Cough Peak 4-7 years 10 days of penicillin
Diarrhea
Upper airway obstruction DDx: Bacterial tracheitis, foreign body, inhalation of smoke/hot air, trauma, angioedema, congenital structural abnormality, large
diphtheria pseudomembrane in posterior pharynx
Croup Viral prodrome 3 months-5 years, winter Swelling of glottis and Assess severity: stridor +/- recession +/-
subglottis RD +/- decreased air entry and altered
Laryngotracheo- Runny nose LOC
bronchitis Cellular infiltrate shows
Cough Parainfluenza Type 1-3 histio/lymphocytosis, plasma Admit if: moderate-severe, toxic child,
Congestion Influenza Type A and B cells and PMNs poor oral intake, <6 mths
Foreign Body After witnessed event 3-4 years old commonly Witnessed event Removal by rigid bronchoscope
Acute choking CXR reveals air trapping
Wheezing Coins
Sudden onset RD
Hoarsness
Epiglottitis Dramatic onset: Haemophilus influenza Swelling of epiglottis and Establish patent airway→
supraglottis
Fever >38.5, sore throat, Failure of getting vaccine Endotracheal intubation
SOB, and rapidly progressive Examination: Do nothing to
airway obstruction upset the child
Strep pyogenes ABx to cover staphylococci, HiB and
Toxic appearance
resistant strep
Dysphagia Strep pneumonia Controlled visualization of
▪ IV chloramphenicol
S. Aureus cherry-red swollen epiglottitis
Drooling ▪ Anti-staph
Mycoplasma Thumb sign on lateral neck ▪ 3rd gen cephalosporin
Stridor—late finding Xray
Emergency tracheostomy
Soft whispering, muffled cry
Bacterial Brassy cough <3 years old Mucosal swelling at cricoid Abx: Anti-staph
Tracheitis cartilage→copious, thick,
High fever Usually following viral URTI purulent secretions with May require intubation if severe
NO drooling or dysphagia
Clinical plus laryngoscopy
Bronchiolitis Acute RD—oedema, airway Most common in <2years Mild and self-limiting 10% will be admitted
inflammation
Improves in 3 days (up to 12) 1% will require O2
Viral prodrome
RSV(Syncitum, woven 3-7% of that will require ventilation
Runny nose appearance of tissue)
Decrease/absent air entry
Congestion Parainfluenza
Percussion: Hyper-resonant Supportive: Monitor sats and hydration
Poor feeding Adenovirus
Auscultation: Fine crackles, Humidified O2 (<94%)
Fever (<38.3) Influenza all zones, prolonged
expiratory phase Analgesia and anti-pyretics (Nurofen,
Vomiting Rhinovirus Brufen, paracetamol)
Chest recession Congenital heart disease Occasional collapse Cardioresp monitoring—pulse oximetry
Pneumonia **Tachypnoea Majority are viral: Chest movement: ↓ on both Admit if <3 months, fever >38.5, refusal
sides, ↓ breath sounds to eat/vomiting, rapid breathing and
Rapid shallow breaths RSV, influenza, adeno, parainflu cyanosis, failure of previous Abx,
Percussion: Dull recurrent, underling disorder
<2 months: >60bpm Newborn:
Bronchial breathing (higher
2-12 mths: >50bpm Group B Strep pitched)
12m-5yrs: >40 bpm E. coli Supportive
Crackles, rhonchi
Viral: Klebsiella Abx
CXR:confirms diagnosis
Mild fever, URTI Sx 1-3 months: chlamydia Augmentin
Viral→ Hyperinflation,
trachomatis (sticky eyes)
If severe: cyanosis, resp bilateral interstitial infiltrates Amoxicillin
fatigue Preschool: Pneumococcus→ confluent Flucloxacillin
Bacterial Pneumococcus (lobar), H. lobar consolidation
influenza, S. aureus (multiple Mycoplasma→unilateral
High fever—chills, acute cavities with fluid/air), GAS, IPD: IV cefuroxime +/- Vancomycin (if
onset lower lobe interstitial—looks
morexella, pseudomonas worse then presentation suspect S. aureus)
Non-productive cough School: Chlamydia→ interstitial or
Drowsiness Mycoplasma pneumonia lobar
Blood culture
Serology (mycoplasma—IgM )
Asthma Cannot diagnose before age Consider Good Hx vital Assess asthma control (5)
5 but pay attn to onset of
Frequency, severity, duration, SaO2 <91% Avoid triggers, inhaler technique,
symptoms/history
home treatments, required past compliance, environment
treatments, baseline peak flow, PEFR<50
# of ED visits, hospitalizations, PaC02 >5.3 KPa (40mmHg)
Dry cough (early) ICU admissions, intubations Reliever( blue): Salbutamol (ventolin),
Wheeze (worse at night) terbutaline (bricanyl), ipratropium
Signs: Harrison’s sulcus, AP (atrovent)
SOB Proposed Triggers diameter increased
Noisy breathing Exposure to allergens Reduced FEV1, PEFR Preventer: Cromoglycate,
Hygiene hypothesis variability >20%, exercise methylxanthines, montelukast
induced bronchospasm,
Infants: may not have (singulair)/zafirlukast, oral prednisolone
Viral respiratory infection sputum with eosinophils
abnormal signs on
auscultation Air pollution/tobacco
Mainstay: Inhaled CS (200-400mg/day):
Stress, emotional factors, cold Chest movement: Reduced budesonide (Pulmicort brown),
air, exercise but hyper-inflated; use of
Chronic inflammation beclomethasone (Becotide), fluticasone
accessory muscles, chest wall (orange)
Reversible airway retraction
obstruction Risk Factors Long acting BD: Salmeterol, formetrol
Percussion: Hyperresonant
Bronchial hypersensitivity Hx of infantile eczema, rhinitis, Prolonged expiration often Combo:
prematurity, mother smoking with wheeze
Reversible bronchospasm during pregnancy, RSV, pets Seretide: fluticasone+salmeterol—purple
Cystic Fibrosis AR disease of c’some 7— RESP: Obstruction and infection GIT: Malabsorption and Clear airway secretions and
CFTR gene of airways → maldigestion→ Pancreatic infections
insufficiency, intussusception, fecal
Exam (CXR): Hyperinflation, ↑ ▪ Chest therapy, postural
impactions, reflux, ADEK deficiency,
AP diameter, hyper resonance, ↑DM, acute pancreatitis, rectal drainage
Failure to thrive rales, bronchiectasis, patchy ▪ Aerosol: albuterol/saline
prolapse
Foul-smelling, greasy stools atelectasis, flattening of ▪ Daily human recombinant
diaphragm, sternal bowing, DNAse (mucolytic)
Cough—purulent mucous narrow cardiac shadow, ▪ Abx: P.aeroginosa most
extensive bronchiolitis, clubbing, Meconium ileus – may be first common
Salty taste of skin presentation, dilated loops on XR,
expiratory wheeze, SOB, cor ▪ Aerosol ABX: tobramycin
(>60mEq/L) ground glass material in lower
pulmonale, RF, late cyanosis ▪ IPD: 2 weeks: IV piperacillin +
central abdomen; gastrografin
↑ potential difference tobramycin/ceftazidime
PFTs: <5 yrs—obstructive, then enema may clear ▪ Ibuprofen chronically
across nasal epithelium
progresses to restrictive ▪ Pancreatic enzymes with food
(fibrosis) ▪ ADEK supplementation
Sputum (+) for S. aureus, P. Test: 72 hour fecal fat collection, ▪ Fluid replacement
Antenatal Dx in + FHx
stool for trypsin, serum
aeruginosa, Burkholderia
Newborn screen— cepacia immunoreactive trypsinogen
immunoreactive trypsinogen
in blood spots Opacified paranasal sinuses
RDS ▪ Surfactant deficiency Persistently low O2 tension→ Lecithin to sphingomyelin ratio in ▪ Supplemental O2 at high [ ]
leads to ↑ surface risk of PDA the amniotic fluid (<1.5) ▪ Nasal CPAP and/or mechanical
tension and alveolar ventilation
collapse RF: prematurity, maternal ▪ Prevent with maternal steroids ▪ Intra-tracheal surfactant
diabetes (due to elevated before birth (<36 weeks),
▪ Surfactant usually made
at 35 weeks insulin), cesarean delivery (↓ artificial surfactant for infant
release of fetal glucocorticoids)
Chronic Cough Ddx: recurrent RTI, asthma, allergic rhinitis, sinusitis, infections (pertussis, RSV, mycoplasma), inhaled foreign body, lung disease (CF, PCD), GERD,
TB, cigarette smoking, psychogenic
Recurrent wheeze: asthma, post RSV bronchiolitis, recurrent aspiration of feeds, chronic lung disease, CF, pulmonary/cardiac anomalies, maternal smoking, cow
milk allergy/intolerance, GERD
Cardiovascular System
Splitting: Inspiration leads to drop in intrathoracic pressure which ↑ pulmonary capacity—P2 closes later to accommodate more blood entering lungs; A2 closes
earlier because ↓ return to left heart (inspiration brings A2 and P2 further apart)
Wide splitting: condition that delay RV emptying (pulmonary stenosis, RBBB)→ causes delayed P2 regardless of breath
Paradoxical splitting: condition that delay LV emptying (aortic stenosis, LBBB)→ order of valve closures reversed (P2 before A2; on inspiration brings sounds
closer together)
Venous blood bypasses lung→ ↓ PaO2 (hypoxemia) CNS abscess, intracranial abscess—vegetations from IE
Right to left shunts to brain
↑ A-a gradient
Thromboembolic stroke (due to polycythaemia)
Clubbing
Mucosal cyanosis
Infection—infective endocarditis
Poor growth
Tetralogy of Fallot Most common cause of early cyanosis: 8-10% +/- clubbing
Anterosuperior displacement of infundibular septum Loud ejection systolic murmur along L sternal edge
Entire systemic/VR enters LA from foramen ovale or ASD ↑ left ventricular impulse
↓ pulmonary flow LV blood to RV via VSD is augmented by PDA Holosystolic murmurs along LSB (most have VSD)
Requires both ASD and VSD for viability ECG: LAD + LVH
Transposition of the Most common cyanotic lesion presenting in immediate newborn period Single loud S2 sound (anterior aorta dominates picture)
great arteries
Parallel circulations: separation of sys/pulm circulations Often no murmur (esp if no VSD)
Aorta off of RV (anterior) and pulmonary trunk off of LV (posterior) VSD present: harsh murmur at LLSB, or holosystolic
Due to failure of aorticopulmonary septum to spiral CXR: Mild CM, narrow mediastinum, normal to ↑ pulm
flow, egg on string appearance of heart
Not compatible with life unless a shunt is present to allow mixing
Tx: PGE infusion to keep PDA open
Can mix at 3 levels: PDA, PFO/ASD, +/- VSD if present
Balloon atrioseptostomy
With intact septum (no VSD), as PDA closes→ severe cyanosis and
tacypnea Sx: Arterial switch operation; w/o Sx most die w/in
first few months of life
Life threatening cyanosis as neonate, O2 sats < 50%
Total Anomalous Pulmonary veins drain into right heart circulation (SVC, coronary sinus) CXR: snowman appearance: supracardiac shadow with
pulmonary venous enlarged cardiac shadow
return (TAPVR) Total mixing of systemic venous and pulmonary venous blood
ECG: RVH and tall, spiked P waves (RAE)
Right atrial blood→ RV and PA OR LA (via PFO/ASD)
Tx: PGE1, surgical correction
Enlarged RA, RV and PA
Truncus Arteriosis (1%) Failure of truncus arteriosus to divide into pulmonary trunk and aorta; Initially SEM with loud thrill, single S2 and minimal
incomplete development of aorticopulmonary septum resulting in a single cyanosis b/c total mixing
great vessel leaving heart and supplying all circulations
Eisenmenger syndrome and early death if not corrected
Therefore, aorta, PA’s, coronary vessels originate from single vessel greatly
CXR: heart enlargement with ↑ pulm BF
↑ → HF
ECG: biventricular hypertrophy
Progressive cyanosis as PDA closes CXR: cardiomegaly (if collaterals present) with absent
MPA
Mixed prognosis
Hypoplastic Left Heart Underdeveloped left heart: hypoplastic or atretic mitral and aortic valves, Ashen grey colour (low CO)
Syndrome (HLHS) small LV, small ascending aorta, coarctation (any combination)
Cyanosis
RV maintains both pulmonary and systemic ciculation
Signs of HF, shock
Intracardiac mixing: pulmonary venous blood passes through PFO or ASD
Weak/no brachial and femoral pulses—all reduced
from LA to RA
Ductus arteriosus supplies descending aorta, ascending aorta and coronary Single S2; Often no murmur
arteries from retrograde flow ECG: RVH, RAE with ↓ left-sided forces
May present with CVS collapse when PDA closes (hypoxia, acidosis, death) CXnR: +/- cardiomegaly with right parasternal shift,
pulmonary plethora
Systemic circulation cannot be maintained, and if there is a moderate to
large VSD→ pulmonary overcirculation Tx: Palliativ;3 consecutive surgeries: Norwood
procedure/bidirectional Glenn anastomosis/Fontan
procedure; cardiac transplant
Fetal dx possible >20 weeks gestation
ACYANOTIC ↑ pulmonary resistance due to arteriolar thickening→ progressive pulmonary Eisenmenger’s: Uncorrected VSD, ASD, PDA causes
HEART DISEASE HT→ R to L shunt eventually (Eisenmenger’s syndrome) compensatory vascular hypertrophy due to overload
of R heart→ progressive pulmonary HT; as pulmonary
Step Up in Right side of heart wrt O2 sats resistance ↑shunt reverses to R→L which causes late
Left to Right Arterial blood shunted to venous circulation cyanosis (clubbing, polycythaemia)
Shunts
Does not cause hypoxemia until there is a shunt reversal
VSD Most common congenital cardiac anomaly Symptoms relate to degree of shunt: VSD size, pulm
vascular resistance
2 main types: membranous (perimembranous)—75%; muscular
If small: no symptoms
When PVR>SCR: Can be single or multiple in any combination
Eisenmenger If large: high pulmonary BF; CHF
Dx: Direct measurement of PO2 and O2 sat in RA, RV, PA
Syndrome
Tachypnoea
O2 step up from RA to RV
Dyspnoea and ↑ work of breathing
PDA Delayed closure associated with prematurity, perinatal distress, congenital Small defect: Pink, full volume pulses
rubella, and FAS
Large: HF, wide pulse pressure, bounding arterial
▪ In fetal period, shunt is R→L (pulm A to aorta; normal) pulses
▪ In neonatal period, lung resistance ↓ and shunt becomes L→ R with RVH and
failure (abnormal) Harsh systolic or continuous machinery-type murmur
(sys and dias) @ left clavicle—loudest at S2
▪ F> M (2:1)
▪ Normally closes by 3rd month in response to high Pa02 of blood shunting from ECG: Normal (small)
L→R through ductus at birth
▪ CHF symptoms if large ductus in young infant—but often asymptomatic LVH +/- RVH (large PDA)
▪ PO2 and O2 sats step up from RV to PA; PA only
CXR: +/- cardiomegaly, pulm plethora (↑ pulm A and
▪ Differential cyanosis: “pink on top, blue on bottom”➔ Congenital rubella pulmonary markings and edema)
Result of reduced arterial 02 saturation in distal aorta compared to aorta
proximal to take off of L subclavian A (unoxy blood distal to subclav A) Treatment
Aortic Stenosis Thickened aortic valves, or bicuspid aortic valves Usually asymptomatic in children
Supra-valvular stenosis (uncommon) associated with Williams’s syndrome With ↑ severity→ ↓ pulses, ↑heart size, LV apical
thrust
Critical AS: LVF and ↓ CO
Early systolic ejection click at apex of LSB
ECG: Normal (mild); LVH +/- strain (severe)
Systolic ejection murmur upper right 2 nd intercostal—
ST wave changes radiating to neck and left midsternal border with thrill
CXR: Normal +/- dilated ascending aorta, LVH in suprasternal notch
Treatment: balloon valvuloplasty (majority); surgical valvotomy with valve If severe: no click, ↓ S1 and S2 and maybe a S4
replacement later in life
Acute Rheumatic Most common form of acquired heart disease worldwide Treatment
Fever
▪ Bed rest, close monitoring
Commonly follows Group A strep infection (within several weeks) ▪ Penicillin PO (erythromycin if allergic) for 10
days
5-15 years old who get strep pharyngitis ▪ Arthralgia: Aspirin if no CHF
Antigens are shared b/w strep components and heart tissue ▪ If carditis/CHF: prednisone for 2-3 weeks
▪ Digoxin, salt restrict, diuretics as needed
Complications: valvular heart disease (M>A>T) ▪ Chorea: phenobarbital
Prevention
Hepatomegaly(normally palpable 1-
2cm below costal margin)
Non-active precordium
Sacral oedema
Infective Endocarditis Congenital Cardiac Defect Associations
Ampicillin PO and gentamicin if GIT or GU procedure Maternal SLE Complete heart block (20-40%)
Allergy: Clindamycin 20mg/kg, vancomycin and gentamicin, macrolide Alcohol ASD, VSD (25-30%)
High risk patients for GI/GU procedures: 2 doses amp + gent IV (30 min Iatrogenic Phenytoin: VSD, ASD, PS, AS, coarctation
before procedure and 6 hours later)
Valproate: Coarctation, HLHS, AS, VSD
Gastrointestinal System
Abdominal Distension—five F’s Other signs: Causes of Hepatomegaly Causes of Splenomegaly
Wilm’s: renal, sometimes visible, does Increased: intestinal obstruction, Usually functional, non-organic Dysmorphic facies—Down’s
not cross midline, low grade pain acute diarrhea, G/E (duodenal atresia)
Celiac Disease
Neuroblastoma: irregular and firm, High pitched/tinkling: obstruction Conjunctiva hemorrhage—vomiting
may cross midline, child very unwell Hirschsprung
Reduced/absent: paralytic ileus, Liver failure—CF
Fecal mass: mobile, non-tender, peritonitis
indentable Jaundice/yellow schlera—uremia
Tx: bowel clear out, lifestyle
modification, fiber, diet changes Sacral and pedal oedema—
Intussusception: acutely unwell, mass
may be palpable, RUQ hypoalbuminuria
Vomiting DDx
EXTERNAL SUPPORTS
NG Tube Percutaneous Endoscopic TPN Fluid Management
Gastrostomy (PEG) tube
Absence of suck, palate Unable to take food by mouth Peritoneal drains Maintenance: 0.45% NaCl + 2.5/5% dextrose
Vomiting Infants:
NEC
0-10kg: 100ml/kg/day
CI: base of skull fractures, Hirschsprung’s
severe facial fractures
11-20kg: 1000ml + 50ml/kg/day
(especially to the nose), Imperforate anus
obstructed esophagus,
esophageal varices, and/or Crohn’s and UC in >3yo >20kg: 1500ml +20ml/kg/day
obstructed airway
Malrotation and midgut
volvulus
Fluids + glucose + electrolytes
Na+ : 2-3mmol/kg/day
K+ : 1-2 mmol/kg/day
● Hx and calculate losses Sunken fontanelles ● Na+ >150 ● Na+ < 130
● Check urinary output and ● Too much salt/too little H20 ● Too little salt/too much H2O
Decreased tears
electrolytes ● Greatest loss intracellularly ● Fluid goes from extra to intra-cellular→oedema,
Altered skin turgor ● Correct slowly convulsions
● If child not fitting and >120—correct slowly
3%--mild losses—dry eyes, Dry tongue ● If unstable/seizing and <120 –correct quickly, ensure
mucous membranes seizure is due to Na+ and not glucose
↓ wet nappies
5%--↓urinary output, ● Giver hypertonic saline
tachycardia ↑ thirst
10%--hypotension, altered ↑ HR
mental state
Toe-walkers: often idiopathic, but ▪ Cerebral palsy (2/1000 live births) Ie In adductors and internal rotators Ie. Sitting in a frog-like posture
may suggest pyramidal tract ▪ Congenital myopathy of hip, clonus at ankles→pyramidal
(corticospinal) dysfunction causing ▪ Primary muscle disease dysfunction (also upgoing plantar ▪ In pyramidal tract disorders—
trunk and head arch backwards
spasticity and tight Achilles tendon, ▪ Spinal cord lesions (spina bifida) reflex)
spinal pathology or neuropathy ▪ Global development delay ▪ Central brain disorders
Tight Achilles tendon: myopathy due (syndromes) Spastic quadriplegia: Scissoring of legs ▪ Type I spinal muscle atrophy→
to weakness (excessive adduction of hips), Werdnig-Hoffman; progressive
pronated forearms, fisting, extended ▪ Tay Sach’s
Muscle Wasting legs ▪ Metachromic Leukodystrophy
Broad-based: immature gait, ▪ Down’s syndrome
cerebellar disorder, proximal muscle ▪ Cerebral palsy ▪ Prader-Willi
▪ Meningomyelocele ▪ Hypothyroidism
*Hand dominance not developed until
weakness around hip girdle ▪ Muscle disorder 1-2 years so asymmetry of motor skills ▪ Congenital galactoasemia
▪ Previous poliomyelitis ▪ Marfan syndrome
Gower’s maneuver: Duchenne’s is always abnormal
Septicemia
● Anencephaly is a result of a
failure of closure of the anterior
neural tube prior to 26 days of
fetal life which ultimately results
in the degeneration of the
forebrain.
Tuberous Sclerosis (AD) Physiology behind Birth Disruption results from an extrinsic
▪ Hallmark: CNS tubers found in Defects insult, which destroys normal tissue
convolutions of cerebral A malformation is commonly defined altering the formation of a structure.
hemispheres→calcify→ as a single localized poor formation of Eg. The patterns of findings that
obstructive hydrocephalus tissue that initiates a chain of
result from amniotic bands and limb
▪ Infancy: infantile spasms, Ash-leaf subsequent defects
strangulation (a condition in which
spots, calcified tubers
Eg. Anencephaly torn amniotic tissue strands surround
▪ Childhood: generalized seizures,
a portion of the of body, resulting in
sebaceous adenoma, Shagreen
deep grooves or amputations)
patches in lumbosacral area
▪ Dx: skin, retinal, CNS lesions, A deformation is a result of extrinsic
seizure control, renal US, ECG,CXR mechanical forces on otherwise
▪ Complications: Retinal lesions, normal tissue. If the primary defect is a lack of
50% have rhabdomyoma of the normal organization of cells into
heart, renal lesions (hamartoma Eg. The characteristic pattern of tissue, a dysplasia will result.
or polycystic kidneys) abnormalities including the abnormal
facies, pulmonary hypoplasia, and Eg. This is best illustra ted by the
limb contractures that result from pattern of bonyabnormalities found in
prolonged oligohydramnios, either achondroplasia where a defect in the
secondary to renal agenesis (Potter gene encoding fibroblast growth
syndrome) or premature rupture of factor receptor 3 results in abnormal
membranes (Potter sequence). cartilageformation.
● Fit that is brought on by a rapid ● Often become unconscious ▪ Due to immaturity of the CNS 1. Focal→ face and extremities
rise in body temperature (>39) ● Tonic-clonic stiffening ▪ Subtle seizures—difficult to
● Occur b/w 6 months and 5 years ● Eye staring/flickering recognize 2. Multifocal→ many muscle groups
● Peak: 14-18 months ● Vomiting 3. Tonic→ rigid posturing
● Incidence: 2-5% ● Drowsiness Causes
● Often FHx of febrile seizures ● Fecal or urinary incontinence ▪ Hypoxic ischaemic 4. Myoclonic→ brief focal or
● 1% chance of developing epilepsy ● Lasts < 5min encephalophaty—presents w/in generalized jerks of extermities
after a single episode 12-14 hours after birth 5. Subtle→ chewing, excess salivation,
● Typical: generalized tonic-clonic ▪ CNS infection/hemorrhage apnea, blinking, nystagmus, pedaling
seizure, <10-15 min with brief Work-up ▪ Structural abnormality movements
postictal period ▪ Blood BCHM abnormalities
● Atypical seizure: >15 min, more Determine cause of fever ▪ IEM
than one in a day, focal findings ▪ Drug withdrawal
R/o meningitis Infantile Spasm
● Risk of epilepsy ↑: Atypical
seizure, if he/she is less than 9 No routine labs ▪ Symmetric contractions of neck,
months old, FHx of epilepsy,
No EEG or neuroimaging Testing trunk, extremities due to
abnormal development, or pre-
↑corticotropin-releasing factor
existing neuro disorder ▪ CBC, electrolytes leading to neuronal hyper
● If child is <12 months, there is ▪ HYPO Calcium, magnesium, excitability
50% chance that they will have a Management phosphorus ▪ 4-8 months of age
2nd one; 30% chance if child is >12 ▪ Glucose
Recovery position ▪ Characteristic EEG changes:
months ▪ LP to exclude meningitis or bleed hypsarrhythmia
Control fever ▪ CT scan in term babies ▪ Cause: Tuberous sclerosis
▪ US in preterm to Dx bleed ▪ Tx: ACTH, prednisone if no
If more than 5 min→ ED ▪ Blood and urine culture response
▪ Newborn screen for IEM
▪ Tx. Lorazepam, phenobarbital
Renal System
UTI
Urinalysis: WCC, U/E, CRP, blood culture, LP in Absence of symptoms E. coli (80%)
febrile child <3months
Recent manipulation of urinary tract Klebsiella
Clean catch urine—MSU (↑ contamination)
Presence of epithelial cells Proteus
Suprapubic aspirate is gold standard (invasive,
painful) Absence of WCC Enterobacter
Pyuria >100
● Atypical organism: non-E. coli 1 episode of UTI with upper UTI + >1 episode of UTI Hydronephrosis
● Tx failure within 48 hours with lower UTI OR
● Poor urine flow Bladder palpaple in urinary retention
>3 episodes of UTI with lower UTI
● Ill patient, septicemic Renal bruits: RAS, renal AV malformations
● ↑ creatinine
● Abdo or bladder mass
DM
Amyloidosis
Endocrinology
Hypothyroidism
Congenital ‘CRETINSIM’ Sleepiness Weight and height normal TSH—elevation; unaffected in secondary
and tertiary form
1/3500 Cold skin and ↓ temperature Prolonged jaundice
Free T4 (low or normal)
1) Primary Congenital Poor feeding (slow to feed) Large tongue with normal mouth (open)
Hypothyroidism—dysgenesis TPO: thyroid Abs
(wrongly formed) Constipation Large fontanels—anterior and posterior
XRay knee—bone age to assess severity
2) Ectopic—found along path of
descent (excision of base of Hoarse cry Umbilical hernia of disease (use wrist >2 yr)
tongue)
3) Enzyme defect (10%) FHx of congenital hypothyroidism Hypotonia Technetium scan with US—position of
(enzyme defects) thyroid and location of iodine
4) HPA axis defects—central Coarse facies
hypopituitarism Maternal history of thyroid disease Guthrie testing: tests for congenital
5) Iodine deficiency or endemic Distended abdomen cause; allows early Dx and Tx; do it by
goiter day 5
Slow Reflexes
6) Iatrogenic: radioiodine
Prognosis
exposure, fetal exposure to Edema
excessive iodine, anti-thyroid If delay early treatment—profound
drugs Mental and developmental retardation
irreversible neurological
development (<6 weeks of age; Apnea--sluggish
goal <2 weeks)
-Normal mouth
-Prominent tongue
Delayed reflexes
History of SUFE (pain in knee, hip,
irregular gait), other AI disease Pubertal delay
Hyperthyroidism
Diabetes
Retinopathy
Nephropathy—microalbuminuria
Neuropathy
Lipids—IHD risk
Urgent referral to MDT diabetes team Rapid-acting: Novorapid, lispro Carry Id bracelet, increase awareness
Admission (home care if not in DKA) Short-acting: Actrapid/Humulin S Avoid very low HbA1c
Insulin administration Int-acting: Insultard/Humulin I Tx: Rapid access to CHO and blood sugar monitoring;
glucagon
Regular blood checks—4x daily Long-acting: Glargine/detemir with basal bolus in
older children
Education about disease to all those
involved—parents, teachers Sick Days
MDT: Dietician, endocrinologist, psychology, Regimen: Take more blood sugars, regular snacks, check for
social worker, ophthalmology, chiroprody, ketones
1-3 injections/day of rapid or short acting
GP, diabetic nurse
premixed/self-mixed with intermediate-acting If persistent vomiting bring to hospital
Transitional care
Meter-dose insulin: Rapid or short acting insulin Never omit insulin
Monitor long term growth and before meals with intermediate or long acting insulin
development, pubertal development
Insulin pump therapy: Novorapid with no background
Advice on social—EtOH llll insulin→ ↑ DKA risk
Exercise
Type 2 Diabetes MODY: Monogenic maturity onset diabetes of the Neonatal Diabetes
young
More common with increased obesity ▪ Very rare (1/400,000)
▪ Single gene disorder causing B cell dysfunction ▪ Insulin requiring hyperglycaemia in first 3-6 months
Insulin resistance ▪ AD FHx of life—combo of resistance and infection
● Acanthosis nigricans ▪ Usually require less insulin than classic T1DM ▪ May be associated with IUGR
● High insulin or c-peptide leves ▪ Slim build ▪ 50% cases transient
● Dyslipidemia ▪ Endogenous insulin secretion ▪ Permanency assoc with pancreatic aplasia, genetic
● Polycystic ovarian syndrome ▪ Not prone to ketoacidosis mutations
▪ No signs of insulin resistance ▪ Predisposes to impaired glucose tolerance and T2DM
More common in ethnic minority groups ▪ Mutations found in >80% of patients in later life
Rx: metformin
ADRENAL DISEASE
CAH
▪ Prevalence <1-2/10,000 ➔Disturb synthesis or catabolism of ➔Inborn errors of intermediary ➔IEM causing deficiencies in energy
▪ Majority are AR complex molecules metabolism production
▪ 50% enzyme is sufficient to
▪ Lysosomal disorders ▪ Aminoacidopathies ▪ Lactic academia
protect against disease
▪ Peroxisomal ▪ Organic acidurias—accumulation of ▪ Respiratory chain abnormalities
an acid which does not contain an
X-linked: Hunters and Fabry’s ▪ Carbohydrate deficient amino group
glycoprotein syndrome
▪ Most common: mitochondrial
respiratory chain disorders
▪ Peaks in newborn period when ▪ Consanguinity ▪ Children of any age with ▪ FBC
feeding is started, when ▪ FHx off SIDs, recurrent unexplained encephalopathy ▪ Urea, Na, K, Ca
weeningon new foods, or at 12 spontaneous abortions, acute (dysmorphic features) ▪ Venous blood gases
months (↑ energy with life threatening events ▪ Progressive neuro disease ▪ Lactate
walking), puberty or pregnancy ▪ + FHx ▪ Acid/base imbalance or ▪ Glucose
(↑ E demands), and post- ▪ Acute fatty liver of pregnancy hypoglycaemia ▪ Renal/liver/bone profile
partum with involution of uterus (due to placental transfer of ▪ Lactic acidosis ▪ Calculate anion gap
acycarnitine from fetus) ▪ Unexplained multi organ disease ▪ Ammonia (if metabolic alkalosis
▪ HEELP suspect UCD)
Presentation ▪ Unexplained developmental ▪ CK
▪ Developmental delay delay Examination Clues ▪ TFT
▪ Unexplained hypotonia ▪ Multiple endocrine deficiencies ▪ Urate
▪ Self-injurious (Lysch Nyhan) ▪ Dysmorphology (Hunters, Hurlers) ▪ Serum amino acids
▪ Visual and hearing problms
▪ Cardiomyophaty ▪ Odour (MSUD, PKU) ▪ Urine: glycosaminoglycan, aas,
▪ Organomegaly (Mt disease, GSD, organic acids
▪ ↑liver transaminases and CK
▪ Renal tubular defects LSP)
▪ Opthalmology (cherry red macule,
( phosphate leak)
▪ Contractures, abdo hernias pigment retinopathy)
▪ Cardiomyopathy (Mt and FAOD)
▪ Cyclical vomiting = UCD crisis
▪ Extreme food fadism ▪ Marked scoliosis (skeletal muscle
weakness in Mt disease)
▪ Peripheral neuropathy
▪ >50 genetic disorders More rapid diagnostics Eyes: corneal clouding, glaucoma, ▪ 1/26 000
▪ Cause progressive accumulation ▪ Deficient in iduronate sulfatase
of intermediate metabolic Newborn screening retinal degeneration ▪ Obstructed airway
products such as sphingolipids, ▪ Microcephaly
sulphatides New small molecule treatments eg Skin: thick, coarse facies ▪ Ortho and heart problems
tetrathiomolybdate (Wilson disease),
▪ Usually display progressive Heart: Failure, valvular lesions
disease patterns with wide NTBC (alkapturia)
spectrum of symptoms, signs, Haematopoeitic stem cell transplant Developmental regression Bone marrow transplantation in
and severity children <2 years with IQ >70
Skeletal: Thick skull, broad ribs, claw
Enzyme replacement therapy—
infusions hand, thoracic kyphosis, lumbar lordosis Cord blood transplant
Other: hepatomegaly, carpal tunnel,
conductive deafness, umbilical and
inguinal hernia
Presentation Treatment
HEMATOLOGY
Purpura If ill and platelet normal or ↑: Immune Thrombocytopenic Tests:
● Purple spots/nodules not
▪ Viruses (measles; enterovirus) Purpura ▪ ↓ platelets <20,000/ml
disappearing on pressure ▪ Most common cause of
▪ Vasculitis (Kawasaki) ▪ Lymphocytosis
● Petechiae (<3mm) thrombocytopenia in childhood
▪ SBE ▪ Other cell lines normal
● Ecchymoses (>1cm) ▪ Auto antibodies against platelet
▪ BM: normal/↑ megakaryocytes
● Purpura is in between 0.3 – 1cm surface
If well and platelet count normal:
Presentation
▪ With no Hx of trauma: HSP Treatment
2 questions ▪ Peak: 2-6 years; M=F
▪ May follow viral infection 1-4 ▪ Avoid contact sports
1) Is the child ill?
If well and platelet count ↓: weeks (CMV, EBV, parvovirus B19) ▪ IVIg for 1-2 days
2) What is the platelet count?
▪ Gradual resolution over 3 months ▪ If inadequate response→
▪ Idiopathic thrombocytopenic ▪ Acute bruising, purpura, and prednisone
purpura petechiae ▪ Rituximab and anti-D to reduce
If ill and platelets ↓: ▪ Bleeding from gums, nose, or need for splenectomy
rectum
▪ Meningococcaemia Rarely Wiskott-Aldrich syndrome or Splenectomy for older children with
▪ <1% intracranial hemorrhage
(penicillin) aplastic anemia
▪ 10-20% develop chronic ITP more severe disease
▪ Leukemia
▪ DIC (blood film and WCC)
▪ HUS
Henoch-Schonlein Purpura Presentation Lab: Sturge-Weber
(HSP) ▪ Sporadic congenital vascular
▪ Fever and fatigue ↑ ESR, WBCs and often platelets
▪ Most common childhood systemic disorder affecting capillaries
▪ Symmetrical palpable purpura
vasculitis ↑ IgA and IgM ▪ Port-wine stain (facial naevus) in
(maculopapular) over buttocks,
▪ IgA mediated Vasculitis of small dist of CN V (V1 always involved)
scrotum, extensor surfaces, Anemia
vessels→ IgA and C3 in skin, ▪ Leptomeningeal angiomatosis
ankles +/- urticarial may progress
glomeruli and GIT (intracerebral AVM)
to petechial and purpura Urine: albumin, RBC, WBC, casts
▪ Non-thrombocytopenic purpura ▪ Mental retardation
▪ Arthritis/arthralgia—knees and
▪ Ages 3-10 M>F: 2:1 ▪ Seizures—contralateral to naevus
ankles, periarticular swelling
▪ Peaks during winter months ▪ Hemiplegia
▪ GI: abdominal pain, melaena, TX for HSP nephritis:
▪ Preceded by URTI ▪ Skull radio-opacity
intussusception, diarhhea,
▪ Recovery within 2 months ▪ Tram-track calcifications on skull
haematemesis Symptomatic treatment—self-limited
▪ Recurrence in 35% radiograph—intracranial
▪ Scrotal oedema
▪ Type III hypersensitivity GIT: IV or PO high dose corticosteroids calcifications
▪ Renal: GN, nephrotic syndrome,
Cyclophosphamide ▪ Glaucoma in ipsilateral eye
haematuria
▪ Dx: Skull X-ray shows occipital-
▪ Hepatosplenomegaly,
parietal calcification; intraocular
lymphadenopathy
pressure reading; CT scan to show
Complications:
unilateral cortical atrophy and
Renal insufficiency/failure hydrocephalus ex vacua
Scrotal oedema
Testicular torsion
Ileus
Hemangiomas
Location, size important
Kasabach-Merritt
SYNDROMES
● When a particular set of multiple anomalies occurs repeatedly in a consistent pattern
● Usually associated with moderate to severe learning difficulties
● Syndromes are typically a result of a single genetic abnormality (Down’s)
● Associations are non-random collections of birth defects, which may have resulted from a number ofgenetic factors (VACTERL)
Small penis
Happy puppet
Inappropriate laughter
Commonest cause of mental Short neck, excess nuchal skin Sleep apnea
retardation
Short stature Early onset Alzheimer’s
10% survive past one year Low set dysplastic ears Omphalocoele
Cleft palate
Polydactyly
Shield-shaped chest
Omphalocoele
Hemihypertrophy
Pierre Robin Isolated finding or associated with Mandibular hypoplasia (in utero)
Robin some syndromes/malformations
Posteriorly placed tongue
Sequence FAS
Cleft palate
Edwards
Micrognathia
Glossoptosis
Older paternal age Small foramen magnum (may have hydrocephalus) Small Eustachian tube—otitis media and
hearing loss
FGFR 3 mutation Short cranial base
Prominent forehead
Lumbar lordosis
Miscellaneous
Non- ▪ History inconsistent with physical findings, or history not Shaken Baby Syndrome
Accidental reproducible ▪ Violent shaking of infant resulting in intracranial hemorrhages,
▪ Delay in seeking medical attention retinal hemorrhages, and fractures (posterior rib #)
Injury
▪ Physical findings not consistent with any underlying medical ▪ Dx confirmed by head CT or MRI, ophthalmologic exam, skeletal
condition survey/bone scan
▪ Injuries of varied ages, recurrent, or multiple injuries ▪ Head trauma is leading cause of death in child maltreatment
▪ Distinctive marks: belt buckle, cigarette burns, hand prints
▪ Bruises on face, abdomen, buttocks, genitalia, upper back,
posterior rib fractures, immersion burns
▪ Altered mental status: head injury, poisoning
▪ Physical
➔Most common childhood cancer ➔2nd most common childhood CA) ▪ 1/3 of tumours
▪ Cranoiopharyngioma (benign)
▪ ALL accounts for 80%, AML for 15% ➔Most common solid tumour (25%) o 7-10% of all brain tumours
▪ Acute lymphoblastic leukemia o Minimally invasive
o 2-6 years old ▪ Usually <7 years
▪ Largest number of cancer deaths: Mortality 45% o May present with short stature,
o 80% cure bitemporal hemianopia and signs
o Bone and joint pain of lower extremities of panhypopituitarism
—new limp ▪ CT scan shows calcification of sella turcica
o S&S of BM failure—petechia, bruising, Infratentorial (2/3)
▪ Optic Nerve Glioma
purpura, pallor, epistaxis, mucous o Most frequent tumour of the optic
▪ Benign astrocytoma—most common
membrane bleeding,
o Juvenile pilocytic astrocytoma nerv
o Lymphadenopathy, o Unilateral visual loss
o Located in cerebellum—causes new-onset
hepatosplenomegaly, joint swelling,
seizure, wide based gait (ataxia) o Eye deviation and optic atrophy
mediastinal mass o Strabismus, nyastagus
▪ Malignant astrocytoma
o ↓ Hb, plts, WCC (↑ WCC has worst
o Glioblastoma multiforme—much worst o Increased incidence in
prognosis)
o Chemotherapy: vincristine, prognosis—more rare in children neurofibratomatosis
daunorubicin, predinisone→tumor lysis ▪ Medulloblastoma—2nd most common
syndrome o Located in the posterior fossa at the midline
o CNS Tx with methotrexate cerebellar vermis
o Relapse 15-20% o Can cause 4th ventricular obstruction and
o ↑ ICP or isolated CNN palsies, testicular hydrocephalus
relapse, pneumocystis pneumonia and o Highly malignant
other infections ▪ Brain stem tumour—third most common
▪ o Causes motor weakness, CN defects,
cerebellar defects, signs of ↑ ICP,
personality change