Pediatrics

p
Hedatros
Emily Baker, Eileen Estrabillo and Manisha Verma, chapter editors
Aseem Bishnoi and Grace Yeung, associate editors
Amy Shafey, EBM editor
Dr. Hosanna Au, Dr. Stacey Bernstein, Dr. Rayfel Schneider and Dr. Michael Weinstein, staf editors
with contributions from Dr. Ann Jefferies
Pediatric Quick Reference Values n n n n n n n n n n n 3
Primary Care Pediatrics n n n n n n n n n n n n n n n n n n n 4
Regular Visits
Developmental Milestones
Immunization
Routine Immunization
Other Vaccines
Nutrition
Normal Physical Growth
Dentition
Failure to Thrive (FTT)
Obesity
Colic
Milk Caries
Injury Prevention Counselling
Sudden Infant Death Syndrome (SIDS)
Circumcision
Toilet Training
Approach to the Crying/Fussing Child
Abnormal Child Behaviours .............. 13
Elimination Disorders
Sleep Disturbances
Breath-Holding Spells
Child Abuse and Neglect n n n n n n n n n n n n n n n n n 15
Adolescent Medicine n n n n n n n n n n n n n n n n n n n n 16
Normal Sexual Development
Normal Variation in Puberty
Cardiology ............................ 18
Heart Murmurs
Congenital Heart Disease (CHD)
Acyanotic CHD
Cyanotic CHD
Congestive Heart Failure (CHF)
Infective Endocarditis
Dysrhythmias
Development .......................... 24
Developmental Delay
Intellectual Disability
Language Delay
Learning Disorders
Fetal Alcohol Spectrum Disorder
E ndocrinology ......................... 27
Diabetes Mellitus (DM)
Diabetes Insipidus
Syndrome of Inappropriate Antidiuretic
Hormone (SIADH)
Hypercalemia/Hypocalcemia/Rickets
Hypothyroidism
Hyperthyroidism
Ambiguous Genitalia
Congenital Adrenal Hyperplasia
Precocious Puberty
Toronto Notes 2010
Delayed Pubery
Short Stature
Growth Hormone Deficiency
Tall Stature
Gastroenterology n n n n n n n n n n n n n n n n n n n n n n n 34
Vomiting
Vomiting in the Newborn Period
Vomiting After the Newborn Period
Acute Diarrhea
Chronic Diarrhea
Chronic Diarrhea Without Failure to Thrive
Chronic Diarrhea With Failure to Thrive
Constipation
Acute Abdominal Pain
Chronic Abdominal Pain
Abdominal Mass
Upper Gastrointestinal Bleeding
Lower Gastrointestinal Bleeding
Genetics, Dysmorphisms, and Metabolism . .41
Approach to the Dysmorphic Child
Genetic Syndromes
Muscular Dystrophy (MD)
Associations
VACTERL
CHARGE
Metabolic Disease
Phenylketonuria
Galactosemia
Hematology .......................... .46
Physiologic Anemia
Iron Deficiency Anemia
Anemia of Chronic Disease
Hemoglobinopathies
Bleeding Disorders
Immune Thrombocytopenic Purpura (lTP)
Hemophilia
von Willebrand's Disease
Infectious Diseases .................... .49
Fever
Acute Otitis Media (AOM)
Meningitis
HIV Infection
Pharyngitis and Tonsillitis
Streptococcal (GAS) Pharyngitis
Infectious Mononucleosis
Pertussis
Varicella (Chickenpox)
Roseola
Measles
Mumps
Rubella
Reye Syndrome
Erythema Infectiosum
Urinary Tract Infection (UTI)
Pediatrics
p
Hedatros
Neonatology ........................... 60
Normal Baby at Term
Gestational Age (GA) and Size
Routine Neonatal Care
Approach to the Depressed Newborn
Neonatal Resuscitation
Sepsis in the Neonate
Cyanosis
Persistent Pulmonary Hypertension of the
Newborn (PPHN)
Apnea
Respiratory Distress in the Newborn
Respiratory Distress Syndrome (RDS)
Transient Tachypnea of the Newborn (TTN)
Meconium Aspiration Syndrome (MAS)
Pneumonia
Diaphragmatic Hernia
Chronic Lung Disease (CLD)
Hypoglycemia
Jaundice
Bleeding Disorders in Neonates
Necrotizing Enterocolitis (NEC)
Intraventricular Hemorrhage (lVH)
Retinopathy of Prematurity (ROP)
Common Neonatal Skin Conditions
Nephrology ............................ 7 3
Dehydration
Fluid and Electrolyte Therapy
Hematuria
Proteinuria
Hemolytic Uremic Syndrome (HUS)
Nephritic Syndrome
Nephrotic Syndrome
Hypertension in Childhood
Neurology ............................. 7 9
Seizure Disorders
Benign Febrile Seizures
Recurrent Headache
Hypotonia
Cerebral Palsy (CP)
Neurocutaneous Syndromes
Acute Disseminated Encelphalomyelitis (ADEM)
Neurosurgery ....................... NS34
Neural Tube Defects
Intraventricular Hemorrhage (lVH)
Hydrocephalus
Brain Tumours
Dandy-Walker Cyst
Chiari Malformation
Craniosynostosis
Oncology .............................. 8 5
Leukemia
Lymphoma
Brain Tumours
Wilms' Tumour (Nephroblastoma)
Neuroblastoma
Rhabdomyosarcoma
Generalized Lymphadenopathy
Orhopaedics ....................... OR37
Fractures in Children
Epiphyseal Injury
Pulled Elbow
Developmental Dysplasia of the Hip
P2 Pediatrics
Legg-Calve-Perthes Disease
Slipped Capital Femoral Epiphysis
Congenital Talipes Equinovarus
Scoliosis
Otolaryngology ...................... OT5 8
Otitis Media with Effusion (OME)
Acute Tonsillitis
Tonsillectomy
Airway Problems
Signs of Airway Obstruction
Acute Laryngotracheobronchitis (Croup)
Acute Epiglottitis
Subglottic Stenosis
Laryngomalacia
Foreign Body
Plastic Surgery ....................... PL34
Cleft Lip
Cleft Palate
Syndactyly
Polydactyly
Hemangioma
Psychiatry ........................... PS33
Autism Spectrum
Asperger
Attention Deficit and Hyperactivity Disorder
Schizophrenia
Mood Disorders
Anxiety Disorders
Eating Disorders
Respirology ............................ 8 8
Approach to Dyspnea
Upper Respiratory Tract Diseases
Croup
Bacterial Tracheitis
Epiglottitis
Lower Respiratory Tract Diseases
Pneumonia
Bronchiolitis
Asthma
Cystic Fibrosis (CF)
Rheumatology ......................... 92
Evaluation of Limb Pain
Septic Arthritis
Growing Pains
Transient Synovitis
Juvenile Idiopathic Arthritis (JIA)
Systemic Lupus Erythematosus (SLE)
Reactive Arthritis
Lyme Arthritis
Vasculitides
Henoch-Sch6nlein Purpura (HSP)
Kawasaki Disease
Juvenile Dermatomyositis
Urology ............................... U
Urinary Tract Obstruction
Vesicoureteral Reflux (VUR)
Genital Abnormalities
Common Medications ................... 9 6
References ............................ 9 7
Toronto Notes 2010
Toronto Notes 2010 Pediatric Quick Reference Values
Pediatric Quick Reference Values
Tabl e 1 . Average Vital s at Various Ages
Age Pulse (bpm) Resp. Rate (br/min)
Bi rth 1 20 - 1 60 35 - 50
Preschool 70 - 1 40 20 - 30
Adolescent 60 - 1 20 1 5 - 20
Tabl e 2. Average Blood Chemi stry at Various Ages
Test Birh Preschool
Sodi um (mmoVL) 1 33 - 1 42 1 35 - 1 43
Potassi um (mmoVL) 4.5 - 6. 5 3. 5 - 5. 2
Chloride (mmoVL) 96 - 1 06 99 - 1 1 1
Serum Creati ni ne (moVL) < 1 25 <44
BUN (mmoI/L) 2.9 - 1 0 1 . 8 - 5. 4
Gl ucose (fasting; mmoVL) >2. 5 2. 8 - 6. 1
Gl ucose (fasting; mgldL) >45 50 - 1 1 0
p H (arteri al ) 7. 30 - 7.40 7. 35 - 7. 45
pC02 ( mmHg) 30 - 50 30 - 42
p02 ( mmHg) 50 - 60 80 - 1 00
Bi carbonate (mmol lL) 1 6 - 22 1 8 - 24
ALT (U/L) <60 <40
AST ( UIL) < 1 1 0 <45
Tabl e 3. Average Hematological Labs at Various Ages
Test 2 Days 1 Week
Hemogl obi n (gL) 1 50 - 250
Hemogl obi n (gldL) 1 5 - 20
Hematocrit 0.46 - 0. 74
RBC count (x 1 01
2
/L) 3.5 - 6. 0
Reti cul ocytes (x W/L) <5. 0
MCV (fL) 1 1 0
MCH (pg)
MCHC (gL) 320 - 360 320 - 360
WBC (x 1 09/L) 20 - 40 5 - 21
Polymorphs ,x W/L) 6 - 26 1 . 5 - 1 0
Bands (x W/L) 0 - 4. 5 0 - 4. 5
Lymphocytes (x 1 09/L) 2. 0 - 1 1 .0 2.0 - 1 1 . 0
Platelets ,x W/L) 1 50 - 450 1 50 - 450
I NR 0. 9-2. 7 0. 9-2. 7
P (s) 25 - 60 25 - 60
TSH (mUlL) 1 -38. 9
Pediatric Quick Formula Reference
Mid Parental Height:
sBP (mmHg)
70
80 - 90
90 - 1 20
Adolescent
1 35 - 1 45
3. 5 - 5. 2
98 - 1 06
< 1 06
2. 9 - 7. 1
3 . 3 - 6 . 1
6 0 - 1 1 0
7. 35 - 7. 45
33 - 46
80 - 1 00
20 - 28
<40
<36
1 Month 1 5 VI
1 1 5 - 1 80 1 1 0 - 1 40
1 1 . 5 - 1 8 1 1 - 1 4
0. 35 - 0. 54 0. 33 - 0. 42
3. 5 - 5. 5 4. 0 - 5. 0
<5.0 1 0. 0 - 1 00. 0
90 80 - 94
24 - 34 24 - 31
320 - 360 320 - 360
5 - 1 2
1 . 5 - 8. 5
0
2.0 - 8. 0
1 50 - 450 1 50 - 450
0. 8- 1 . 2 0. 8- 1 . 2
25 - 60 25 - 40
1 .7- 9. 1 0. 7-6. 4
5oy* latherheightcm) motherheightcm) J3cm] / 2
Cirl* latherheigtcm) mother height cm)- J3 cm] / 2
BMI
weigt kg) / height m
)
Energy Requirements:
0-J0kg. J00 cal / kg/ day
0-20kg. J,000 cal+ 50cal / kg/ dayloreachkg>J0
+20kg. J,500cal+ 20cal / kg/ dayloreachkg>20
Pediatrics P3
61 4 VI
1 20 - 1 60
1 2 - 1 6
0. 36 - 0. 48
4. 5 - 5. 5
1 0. 0 - 1 00. 0
80 - 94
24 - 31
320 - 360
4 - 1 0
1 . 5 - 7. 5
0
1 . 5 - 7. 0
1 50 - 450
0. 8- 1 . 2
25 - 40
0. 7-6. 4
P4 Pediatrics

)
,
Pediatric Developmental Milestones
1 year:
- Single words
2years:
2word sentences
- Understands 2step commands
3 years:
- 3 word combos
- Repeats 3 di gi ts
- Ri des tricycl e
4 years:
- Draws square
- Counts 4 objects
p
Developmental Red Flags
Gross motor: Not wal ki ng at 18 mos
Fine motor: Handedness at < 1 0 mos
Speech: <3 words at 18 mos
Social: Not smi l i ng at 3 mos
Cognitive: No peekaboo at 9 mos
Primary Care Pediatrics Toronto Notes 2010
Primary Care Pediatrics
Regular Visits
usualschedule.newLorn, withinJ weekpost-discharge,J,2,4,6, 9, J2, J5, J8, 24months
yearly untilage6, thenevery otheryear
yearlyagainalterageJJ
history
physicalexam
immunizationseeImmunization, P5)
counselling/anticipatoryguidanceseeNutrition P/, Colic PJ0, Sudden Infant Death
Syndrome (SIDS) Pll, andInjury Prevention Counselling Pllsectons)
Developmental Milestones
Tabl e 4. Developmental Mi lestones
Age Gross Motor Fine Motor Speech and Language Adaptive and Social Skills
6 weeks Pronelifts chi n intermittently Social smi le
2 months Pronearms extended forand Pul l s at clothes Coos Recognizes parents
4 months Proneraises head + chest, Reach and grasp, Responds to voice,
rolls over, no head lag objects to mouth l aughs
6 months Pronewei ght on hands, Ulnar grasp, transfers Begi ns to babbl e, Stranger anxi ety
tripod si t objects from hand responds to name begi nni ng of object
to hand permanence
9 months Pulls to stand, crawls Fi ngerthumb grasp "Mama, dada" Plays games, plays peekaboo,
appropriate, i mitates separation/stranger anxi ety
1 wond
1 2 months Wl ks wi th support Pi ncer grasp, throws 2 wonds, follows l step Dri nks wi th cup, waves byebye
command
15 months Wl ks without support Draws a l i ne Jargon Points to needs
1 8 months Up steps wi th hel p Tower of 3 cubes, 1 0 wonds, follows Uses spoon, poi nts to body
scri bbl i ng si mple commands parts
24 months Up 2 feetstep, runs, kicks Tower of 6 cubes, 23 wond phrases, Paral lel play, helps to dress
bal l , wal ks up and down steps undresses uses " I, me, you",
50% i ntel l i gi bl e
3 years Tricycle, up 1 footstep, down Copi es a circle and a Prepositions, pl ural s, Dressundress ful l y except
2 feetstep, stands on one cross, puts on shoes counts to 1 0, 75% buttons, knows sex, age
foot, j umps i ntel l i gi bl e
4 year Hops on 1 foot, down 1 Copi es a square, uses Tel l s stor, knows Cooperative play, toi l et trai ned,
footstep scissors 4 colours, speech buttons clothes, knows names
i ntel l i gi bl e, uses past of body pars
tense
5 years Ski ps, rides bicycle Copi es a tri angle, prints Fl uent speech, future
name, ti es shoelaces tense, al phabet
Primitive Reflexes
rellexes seen innormalnewLorns,mayindicateaLnormalitye.g.cereLralpalsy)ilpersist
alter4-6months
Mororellex
inlantisplacedsemi-upright,headsupportedLyexaminershand,sudden
withdrawalolsupportedheadwithimmediateresupportelicitsrellex
rellexconsistsolaLductionandextensionolthearms,openingolthehands,
lollowedLyllexionandadductionolarms
aLsenceolMorosuggestsCMSinjury,asymmetrysuggestslocalmotorlesions e.g.
Lrachialplexusinjury)
Calantrellex
inlantisheldinventralsuspensionandonesideoltheLackisstrokedalong
paraverteLralline,thepelviswillmoveinthedirectionolstimulatedside
grasprellex.llexionollingerswiththeplacementolalingerintheinlantspalm
tonicneckrellex.turningtheheadresultsinthelencing postureextensionolipsilateral
legandarm)
placingandsteppingrellexprimitivewalking). inlantplaceslootonasurlacewhenit
isLrought into contact withit
Toronto Notes 2010 Primary Care Pediatrics
rootingrellex.inlant pursues tactile stimulinearthemouth
parachute rellex. tiltingtheinlantto the side whileinasittingpositionresultsin
ipsilateralarmextensionappearsLy6-8months)
upgoingplantar rellexes 5aLinskisign)isnormalininlants ie.<2yrs)
Routine Immunization
Tabl e 5. Routine I mmuni zati on Schedul e
Vaccine Schedule Route Reacton Contraindications
DTaPIPV 2, 4, 6, 1 8 mos 1M At 2448 hrs Previous anaphylactic reaction to
46 yrs Mi nor: fever, local redness, swel l i ng, vaccine; evolvi ng unstable neurol ogi c
i rri tabi l i ty di sease; hyporesponsive/hypotoni c
Major: prolonged cri ng ( 1 %) , followi ng previous vaccine;
hypotoni c unresponsi ve state (1 : 1 750), anaphylactic reaction to neomycin
seizure (1 : 1 950) on day of vacci ne or streptomyci n
Prophylaxis: acetami nophen 1 01 5 mg/kg
gi ven 4 hrs prior to i njection and q4h
afterards
Hib 2, 4, 6, 1 8 mos 1M Mi nor: fever, local redness, swel l i ng, irritabi l i ty
Pneu-C 2, 4, 6, 1 5 mos 1 M Mi nor: fever, l ocal redness, swel l i ng, irritabi l i ty
MMR' 1 2, 1 8 mos SC At 7- 1 4 days Pregnancy, i mmunocompromised
Fever, measl e-l i ke rash infants (except heal thy HI V positive
Lymphadenopathy, arthral gi a, arthritis, chi l dren), anaphyl acti c reaction to
parotitis (rare) gel ati n
Men-C 2, 4, 6 mos 1 M Rednessswel l i ng 50%), fever (9%),
OR 1 2 mos i rri tabi l i ty 80%), rash ( 0. 1 %)
Var' 1 5 mos SC Mi l d local reaction (20% but hi gher i n Pregnant or pl anni ng t o get pregnant
i mmunocompromised) wi thi n next 3 months; anaphyl acti c
Mi l d varicel l a- l i ke papul es or vesicles (5%) reaction to gel ati n
Low-grade fever ( 1 5%)
Hep B 3 doses: 1 M Local redness, swel l i ng Anaphyl acti c reaction to Baker's yeast
0, 1 , 6 months;
admi ni stered i n
some provi nces
in grade 7 (can be
given at bi rth or
i n school )
dTap Start at 1 4-1 6 yrs 1M Anaphyl axi s (ver rare) Pregnancy (1 st trimester)
Td Adul t yrs, ql 0 yrs 1M Local erythema and swel l i ng (70%)
Flu" Every autumn 1 M Local tenderness at i njection site Anaphyl acti c reaction to eggs,
6-23 mos Fever, mal ai se, myal gi a, rash, febrile seizures <6 mos of age
Hypersensitivity reacti ons
GardasifM Gi ven in grades 1M Local tenderness, redness, i tchi ng, swel l i ng
7-8 i n some at i njection site.
provi nces. Fever
3 doses at 0, 2, 6
months for femal es
between 9-26
DTaP - IPV - diptheria. tetanus, acellular pertussis, inactivated polio vaccine I for children under 7 yrsl
MMR - measles, mumps, rubella VaccinePneu-C - pneumococcal 7-valent conjugate vaccine
Hib - Hemophi lus inflenzae type b conjugate vaccine Var - varicella vaccine
Men-C - meningococcal C conjugate vaccine dTap - diphtheria, tetanus, acellular perussis vaccine ladolescenVadult formulationl
Hep B - Hepatitis B vaccine T d - tetanus and diphthera adult type formulation
Flu - influenza vaccine
*If varicella vaccine and MMR vaccine not given during the same visit, they must be administered at least 28 days apar.
"For children with severe or chronic disease, e. g. cardiac, pulmonary, or renal disease, sickle cell disease, diabetes, endocrine disorders, HIV, immunosuppressed,
long-term aspirin therapy, or those who visit residents of chronic care facilities
Adapted from: National Advisory Commitee on Immunization. Recommended Immunizaton Schedule for Infants, Children and Yout lupdated March 20051
Administration of Vaccines
injectionsite
inlants <J2 months old). anterolateralthigh
children. deltoid
DTaP+IPV+HiLPentacel`,Pentavax).5vaccinesgivenasoneIMinj ection
twolivevaccinesvaricella,MMR)mustLegivensuLcutaneouslyeitheratthesamevisit
or separatedLy4weeksormore
Contraindications to Any Vaccine
moderateto severe illness levernoneedtodelay vaccinationlormild URTI)
allergyto vaccinecomponent
Pediatrics P5
- ` *
Safet of MMR Vaccine

Accordi ng to the CDC. the wei ght of
currentl y avai l abl e sci enti fi c evi dence
does not support the hypothesi s that
MMR vacci ne causes ei ther auti sm or
I BD.
http1!w .cdc.gr/nip/acsafeconcerns/autisra
uism-mmr.htm#2
Isee website for more details 1
P6 Pediatrics
Safety and Eficacy of an Atenuated Vccine
against Severe Rotavirs Gastoentertis
NEM 2006;354: 1 1 22
Stdy: Randomized, doubleblind, phase 3 tral.
Patients: 63,225 healthy infants from Latin Amerca
and Finland.
Interention: Two oral doses mHRV vaccine vs.
placebo at 2 and 4 mos of age.
Outome: Episodes mgastroentertis and severty
Results: The vaccine is 85% efficacious against
severe rotavirs gastroentertis and hospitaliztions
associated with gastroentertis and 1 00%
effcacious against more severe gastroenteritis.
Possible Adverse Reactions
any vaccine
local.indurationortenderness MMRisespeciallypainlull)
systemic. lever, rash
allergic.urticaria,rhinitis,anaphylaxis
specilic vaccine reactions see TaLle5)
Other Vaccines
Hepatitis A
Toronto Notes 2010
inactivated monovalenthepatitisAvaccine Havrix`,Vaqta,Avaxim,Epaxal5erna)
givenasaseriesol2vaccinations4-6monthsapartcost-$50total)
recommendedaspre-exposureprophylaxislorindividualsatincreasedriskolinlection
e.g. traveltoendemiccountries, residents olcommunities withhighendemicrates, IV
druguse,etc. )
canalsoLegivenasacomLinationvaccinewithHep5 Twinrix`)
immunogloLulincanLeusedlorshort-termprotectionininlantsandimmunocompromised
patients
Hepatitis B
setol3 vaccinations givenininlancy0, J,6 months) ilatincreasedriskolinlection Lorn
to motherwithHep5,Lelongingtoanareawithhighendemicrates),ormid-childhoodto
earlyteens inOntario,giveningrade/)
ilmotherisH5sAgve,giveH5IgatLirthandHep5 vaccineatLirth, J mo,6mos
BeG Vaccine
inlantsolparents withinlectiousT5 attimeoldelivery
groups /communitieswithhighratesoldisease /inlectionolleredto aLoriginalchildren
onreserves),healthcareworkers atrisk
onlygivenilpatienthasanegativeT5skintest
side ellects. erythema,papulelormation3-6weeksalterintradermalinjection,
enlargementolregionallymphnodes
TB Sk in Test (Mantoux)
screenhighriskpopulationsonlylamilyhistory, HIVimmigrantslrom countries with
increasedincidence, suLstanceaLuseinlamily,homeless, aLoriginal)
intradermalinjectionol5TU 0.J ml)oltuLerculousantigen purilied proteinderivative),
readresultat48-/2hrs
T5 testshouldLepostponedlor4-6weeksalteradministrationollive5CCvaccinedueto
riskollalsenegative result
testinterpretation
checkareaolraisedIMDURATIOMnotjustareaolerythema)at48-/2hours
positiveresultil.
>J5mm.children>4yearswithno risklactors
>J0mm.children<4years,oratrisklorenvironmentalexposure
>5mm.childrenwithcloseT5contact,immunosuppressed
5CChistoryirrelevant doesnotusuallygivepositiveresponseunless<6weeks
previously)
positivereactionmeansactivediseaseorpreviouscontactwithT5
Human Papillomavirus Vaccine (GardasWM) seeCnecolo_
giveningrades/-8insomeprovinces
Quadrivalent Meningococcal Vaccine (Menactra
T
M)
given insomeprovincesinCrade9
protectsagainstNeisseria meningitidis strainsA, C,W-J35, andY
inCanada, currentlyrecommendedlor patients with asplenia, travelerstoendemic areas
suchastheHajj inMecca),laLoratoryworkers,and military recruits
Rotavirus Vaccine (RotaTeqTM)
oral vaccine givenin3doseswith lirst atage6-J2weeks
showntoreduceviralgastroenteritisininlants
not currently coveredinCanada
Nutrition
Breast Feeding
colostrumlorlirstlewdays- clear lluidwithnutrients highprotein, lowlat)and
immunogloLulins
lullmilkproductionLy 3-/days,maturemilkLy J5-45 days
supportlor mothers whowantto Lreast leedshouldstartwhileinhospital
signsolinadequateintake.<6wetdiapersperdayalterlirstweek, sleepyorlethargic,
</leedsperday, sleepingthrougoutthenigt, weightloss>J0,olLirthweigt,jaundice
ruleolthumL. - J stool/dayolagelorlirstweek
leedingschedulenewLornLaLyneeds J20 kcal / kg/day)
premature inlants.q2-3hours
terminlants. q3.5-4hours,q5hoursatnightuntilaLout2-3monthsolage
Lreast-ledLaLiesrequirethe lollowingsupplements
vitaminK givenIMatLirth)
vitaminD400IU/dayTri-Vi-Sol`orDi-Vi-Sol`),especiallyduringwintermonths
lluoride alter 6monthsilnotsullicientinwater supply)
iron.lrom4monthsto J2monthsironlortiliedcerealsorlerroussulphatesolution)
contraindicationstoLreastleeding
motherreceivingchemotherapyorradioactivecompounds
motherwithHIV/AIDS,activeuntreatedT5,herpesinLreastregion
motherusing>0.5g/kg/dayalcoholand/orillicitdrugs decrease milkproduction
and/ordirectlytoxicto LaLy)
mothertakingcertainmedicationse.g.antimetaLolites,Lromocriptine,
chloramphenicol,high dose diazepam, ergots, gold,metronidazole,tetracycline,
lithium,cyclophosphamide
Mote.oralcontraceptivepillsOCP)notacontraindicationtoLreastleeding
estrogenmaydecreaselactationLutisnotdangeroustoinlant)
Advantages of Breast Feeding
5reastis5est exclusive Lreastleedingduringthelirst4monthsollileisrecommended
LyHealthCanada,theDietitiansolCanada,andthe CanadianPediatricSociety
Lreastmilkiseasilydigestedandhasalowrenalsoluteload
immunologic
IgA, macrophages,active lymphocytes, lysozymes, lactolerrin lactolerrininhiLits
E. coli growthinintestine)
protection is greatestduringearlymonths,Lutiscumulativewithincreasedduration
olLreastleeding
lowerallergenicitythancows milkprotein decreased cows milkproteinallergy
andeczema)
lowerpHpromotesgrowthollactoLacillusinthegastrointestinaltractprotective
againstpathogenicintestinalLacteria)
parent-childLonding,economical,convenient
Complications of Breast Feeding
mother
sore /crackednipples.treatwithwarmcompresses,massage,lrequentleeds,
soothingLarrier creamsDesitin,Vaseline), properlatchingtechnique
Lreastengorgement usuallyinlirst week).continueLreastleedingand/orpumping
mastitis usuallydueto S. aureus): treat withcold compressesLetweenleeds,
cloxacillinlormother,continuenursing, incisionanddrainage
inlant
Lreastleedingjaundice lirst J-2 weeks). dueto lackolmilkproductionand
suLsequent dehydration see]aundice)
Lreastmilkjaundice.rare0.5,olnewLorns),dueto suLstancesinLreastmilkthat
inhiLitconjugationolLiliruLin persistsupto4-6months)
poor weightgain. considerdehydrationorlailuretothrive
oralcandidiasisthrush).checkLaLys mouthlorwhitecheesymaterialthatdoesnot
scrape oll,treatLaLywithantilungalsuchasnystatin Mycostatin) treatmother
topicallytoprevent transmission)
Alternatives to Breast Feeding
Tabl e 6. I nfant Formulas
Type of Formula
Breast milk
Cow's milk based
( Enfami l '", Si mi l ac'")
Indication(s)
Most babi es
Premature babi es
Transi tional
Contrai ndi cation to breastfeedi ng
Content (compared to breast milk)
70: 30 whey:casei n ratio
Fat from dietar butterfat
Carbohydrate from lactose
Pl ant fats instead of dietar butterfat
Lower whey:casei n ratio
Pediatrics P7
Infant Growth and Healt Outcomes Associated
with 1Monts Compared wit aMonths d
Eclusive Breasteeding
Te Amercan Journal of Cliical Nutr#on 78: 291
295.2003
Purpose: To compare differences in growh and
health in infants exclusively breastfed for 3 versus 6
months.
Study: Obserational cohor study Ith 3483 ter
newborns
Results: The rate of gastrointestinal infections was
sigriicantly reduced in the group of infants who
were exclusively breastfed for 6 months. This
finding was limited to the period between 3 and 6
months of age ladjusted lOR 0.35195% CI: 0.13,
0.961. The breastfed babies were smaller at 6
months but there was no different in growth
beteen the to groups by 12 months. No
sigriicant association was found beteen
breastleeding and the rate of eczema or respiratory
infections.
Conclusions: There is an association between
breasteeding and a lower incidence of
gastrointestinal infections in term infants.
P8 Pediatrics
-`
+F
Scoliosis Screening
Despi te mass school screeni ng
i mplemented i n pars of the USA and
Canada i n the 1 970s-90s. the Canadi an
( 1 994) and Ameri can (2004) Task
Forces on Preventive Health Care do
NOT currently recommend routi ne
screeni ng usi ng the Forward Bend Test
( FBT) . Cohort studi es i ndi cate that the
forward bend test has poor sensitivity
for i dentifyi ng pathol ogi cal curves
(Karachal i os eta|. 1 999. Yawn eta|.
1 999. Prui js eta|. 1 996) . Furthermore.
there i s no evi dence to suggest that
screeni ng and i ncreased braci ng l ead to
better outcomes.
Term newborn shoul d gai n 20-30 g/day
"1 oz. per day except on Sunday"
- `
>F
To esti mate wei ght of chi l d > 1 year
(kg) - Age x 2 + 8
K
Head Ci rcumference
Remember 3, 9,and multiples of 5:
Newborn 35 cm
3 mos 40 cm
9mos 45 cm
3 yrs 50 cm
9yrs 55 cm
Tabl e 6, I nfant Formulas ocnt|nued)
Type of Formula
Forfied formula
Soy protein
(I somi l '", Prosobee |
Indication(s)
Low bi rth wei ght
Premature babi es
Gal actosemi a
Lactose intolerance
Toronto Notes 2010
Content (compared to breast milk)
More calories
Hi gher amounts of vi tami ns A, C, D, K
May onl y be used in hospi tal due to risk of
fat-sol uble vi tami n toxicity
Corn syrup sol i ds or sucrose instead of lactose
Parially hydrolyed proteins
(Good Start '")
Delayed gastric emptyi ng
Ri sk of cow's mi l k al l ergy
Protei n i s 1 00% whey wi th no casei n
Protein hyd rolysate
( Nutrami gen '", Al i mentum '",
Pregesti mi l'", Portagen '"I
Amino acid
(Neocate '")
Mal absorpti on
Food allergy
Food allergy
Short gut
Protei n i s 1 00% casei n wi th no whey
Corn syrup sol ids, sucrose, OR tapi oca starch
i nstead of lactose
No protei ns, j ust free ami no aci ds
Corn syrup sol i ds i nstead of lactose
Ver expensive
Metabolic Inborn errors of metabol i sm Various different composi ti ons for chi ldren wi th
galactosemi a, propi oni c aci demi a, etc.
Most formulas contain 680 calories per litre; "fortified" formulas for premature babies may contain more calories. Formula may also be supplemented with specific
nutrients in babies with malabsorption syndromes. True lactose intolerance is extremely rare in children under age 5
Infant Feeding
Tabl e 7, Dietary Schedul e
Age
Jto 4-6 months
6 to 9 months
9 to 1 2 months
Food
Breast mi l k, formul a
I ron enri ched cereals
Pureed vegetables
Pureed fruits and j uices
Pureed meats, fish, poultr, egg yol k
Fi nger foods, peel ed fruit, cheese
and cooked vegetables, homo mi l k
Comment
Ri ce cereals first because less al l ergeni c
Yel l ow/orange vegetables first and green l ast (more bul k)
Avoi d vegetabl es wi th hi gh nitrite content (beets, spi nach, turni ps)
Introduce vegetables before fruit (alternate yel low and green
vegetables dai l y)
No egg whi te unti l 1 2 months (ri sk of al l ergy)
No honey unti l > 1 2 months (risk of botul i sm)
No peanuts or raw, hard vegetables unti l age 3 to 4 years
No added sugar, salt, fat or seasoni ngs
donotdelayintroductionolsolidloodsLeyond9months
introduce2-3newloodsper week easier to identily adverse reactions)andallowalew
daysLetweeneachintroduction
avoidexcessive milk/juiceintakewhen>Jyear
Normal Physical Growth
newLornsizeinlluencedLymaternallactors placenta,inuteroenvironment)
prematureinlants 3/weeks). usecorrectedgestationalageuntil2years
notlinear.mostrapidgrowthduringlirsttwoyears,growthspurtatpuLerty
dillerenttissuegrowthatdillerenttimes
lirsttwoyears. CMS
mid-childhood.lymphoidtissue
puLerty.gonadalmaturationtestes,Lreasttissue)
Lodyproportions.upper/ lowersegmentratio midpointissymphysispuLis
newLornJ./,adultmale0.9/,adultlemale J.0
poor correlationLetweenLirthweightandadultweight
Tabl e 8. Average Growth Parameters
Normal Growth
Bi rth Wei ght
Length/Hei ght
3. 25 kg
(7 I bs)
50 cm
(20 i n)
Head Ci rcumference 35 cm
( 1 4 i n)
2 x bi rth wt by 4-5 mos
3 x bi rth wt by 1 year
4 x bi rth wt by 2 years
25 cm in 1 st year
1 2 cm in 2nd year
B cm in 3rd year then
4-7 cryear unti l puberty
1/2 adult hei ght at 2 years
2 cm/month for 1 st 3 mos
1 cm/month at 3-6 mos
0.5 crmonth at 6-1 2 mos
Comment
W. loss ( up to 1 0% of bi rth wt) in first 7 days of life i s normal
Neonate should regai n wt by 10 days of age
Measure supi ne length unti l 2 years of age, then measure
standi ng hei ght
Measure around occi pi tal , parietal, and frontal promi nences to
obtai n the greatest circumference
Dentition
primary dentition 20teeth)
lirsttoothat5-9monthslowerincisor),thenJ permonthuntil20teeth
6-8centralteethLyJ year
secondarydentition32teeth)
lirstadulttoothis Jstmolarat6years,thenlowerincisors
2^'molarsatJ2years,3''molarsatJ8years
Failure to Thrive (FTT)
Tabl e 9. Fai l ure to Thrive Paterns
Growth Parameter
decreased W Normal Ht Normal HC
decreased W Decreased Ht Normal HC
decreased W Decreased Ht Decreased HC
IHe head circumference; Ht height; Wt weight!
Definition
Suggestve Abnormali
Cal ori c i nsuffi ci ency
Decreased i ntake
Structural dystrophies
Endocrine disorder
Intrauterine i nsult
Hypermetabol i c state
Increased losses
Constituti onal growth delay
Fami l i al short stature
Genetic abnormality
weight<3''percentile,orlallsacrosstwomajorpercentilecurves,or<80, olexpected
weightlorheightandage
inadequatecaloricintakemostcommonlactorinpoor weightgain
mayhaveothernutritionaldeliciencies e.g. protein,iron, vitaminD)
history
durationolproLlemandgrowthhistory
detaileddietaryandleedinghistory, appetite, LehaviourLelore andalterleeds,
LowelhaLits
pregnancy,Lirth,andpostpartumhistory,developmentalandmedicalhistory
includingmedications),socialandlamilyhistoryparentalheight, weight, growth
pattern)
assess4areasollunctioning. childstemperament,child-parentinteraction, leeding
Lehaviour andparentalpsychosocialstressors
physical exam
height Ht), weight Wt), headcircumlerenceHC),armspan,upper-to-lowerU/L)
segmentratio
assessmentolnutritionalstatus,dysmorphism,Tanner stage,evidenceolchronic
disease
oLservationolaleedingsessionandparent-childinteraction
signsolaLuseorneglect
investigations (as indicated by clinical presentation)
C5C, Lloodsmear,electrolytes,urea, ESR T4, TSH,urinalysis
Loneagex-ray leltwrist compared to standardized wristx-rays)
karyotypeinallshortgirlsandinshortLoyswhereappropriate
anyothertestsindicatedlromhistoryandphysicalexam.renalorliverlunction
tests, venousLloodgases, lerritin, immunogloLulins, sweat chloride, lecallat
Organic
I nabi l i ty to feed
Inadequate absorption
I nappropriate uti l ization of nutri ents
Increased energy requi rements
Fi gure 1. Causes of FTT
Organic FTT
inaLilitytoleed
Ca uses of FI
I
insullicientLreastmilkproduction
poor retentionCERD,vomiting)
Non-Organic
Mal nutri ti on
I nadequate nutri ti on
Poor feedi ng techni que
Errors in maki ng formula
CMS,neuromuscular, mechanicalproLlemswithswallowing,sucking
anorexiaassociatedwithchronicdisease)
inadequateaLsorptionseePediatric Gastroenterolog, P34)
malaLsorption. celiacdisease,cysticliLrosis CF),pancreaticinsulliciency
loss lromtheCItract. chronic diarrhea, vomiting
Pediatrics P9
- `
F
Energy Requirements
0 1 0 kg: 1 00 cal/kg/day
020 kg: 1 . 000 cal + 50 cal/kg/day
for each kg > 1 0
+20 kg: 1 . 500 cal + 20 cal/kg/day
for each kg > 20
F
Clinical signs of FI
SMALL KI D
Subcutaneous fat l oss
Muscl e atrophy
Al opeci a
Lethargy
Laggi ng behi nd normal
Kwashi orkor
Infecti on (recurrent)
Dermati ti s
P10 Pediatrics
Geographic and Demographic Vration in the
Prevalence dDeleight Canadian Childre
Obesi Research 11151:66873, 2003 May
Purpose: To deterine geographic and
demograplic variation in the prevalence of
overeight Canadian children.
Std: Assessment of trends in BMI using data
from the 1981 Canadian Fitness Surey and the
1996 National Longitudinal Survey of Children and
Youth.
Main Outomes: The prevalence of overeight and
obese clildren age 7 to 13 years, secar trends
from 1981 to 1996 by province, and provincial
varation after adjusting for socioeconomic and
demograplic characteristics.
Results: In 1996, 33% mboys and 26% mgirls were
classified as overeight, and 10% of boys and 9% of
girls were classified as obese. The odds ratio
associated with the 1981 to 1996 change in the
prevalence of overeight children was 3.24195%
CI, 2.83-3.701 for Canada as a whole. There are
clear regional differences, with those in Atlantic
Canada more likely to be overweight and Prairie
children less likely. These differences were not
sufficiently accounted for by differences in
socioeconomic circumstances.
Conclusions: The prevalence of clildhood obesity is
increasing in all areas mCanada, although more so
in Atlantic Canada.
Primary Care Pediatrics Toronto Notes 2010
inappropriateutilizationolnutrients
renalloss.e.g.tuLulardisorders
inLornerrorsolmetaLolism
endocrine.typeJ diaLetes,diaLetesinsipidus DI),hypopituitarism,congenital
hypothyroidism
increasedenergyrequirements
pulmonary disease. CF
cardiac disease
endocrine.hyperthyroidism,DI,hypopituitarism
malignancies
chronicinlections
inllammatory. systemic lupuserythematosusSLE)
decreasedgrowthpotential
specilicsyndromes, chromosomalaLnormalities,CHdeliciency
intrauterineinsults. letalalcohol syndrome FAS), TORCHinlections
treatment. cause-specilic
Non-Organic FTT
oltenduetomalnutrition,inadequatenutrition, poorleedingtechnique, errorsinmaking
lormula
thesechildrenmaypresentaspickyeaters,with poor emotionalsupportathomeorpoor
temperamentallitwithcaregiver
mayhavedelayedpsychomotor,language,andpersonal / socialdevelopment
emotionaldeprivation, poor parent-childinteraction,dyslunctionalhome
childaLuseand/orneglect
parentalpsychosocialstress,personalhistoryolsulleringaLuseorneglect
treatment.mostaremanagedasoutpatientswithmultidisciplinaryapproach
primarycarephysician, dietitian,psychologist,socialwork,childprotectionservices
Obesity
prevalenceolchildhood oLesityinCanadahastripledJ98J-J996)
Definition
weight>95''percentilelorageandheight, or5MI>30,orweight >20, greater than
expectedlorage andheight
causedLya chronically positiveenergy Lalance intakeexceedsexpenditure)
Risk Factors
geneticpredisposition.
ilJ parentisoLese 40,chanceoloLesechild
ilLothparents are oLese 80, chanceoloLese child
geneticheritaLilityaccountslor25-40, oljuvenileoLesity
Clinical Presentation
history.diet, activity lamilyheights and weights, growthcurves
Lodymassindex 5MI) tendsnottoLeusedLypediatricians
riortoadolescence
physicalexamination.maysuggestsecondarycause,e.g.Cusingsyndrome
organiccausesarerare 5,)
genetic.e.g.Prader-Willi, Carpenter,Turnersyndromes
endocrine. e.g. Cushingsyndrome,hypothyroidism
complications
childhoodoLesityisanunreliaLle predictor oladultoLesity
unless >J80,olidealLodyweight
however, /0, oloLese adolescents Lecome oLese adults
associationwith.hypertension,dyslipidemia, slipped capitallemoralepiphysis,
type2diaLetes,asthma,oLstructivesleepapnea
Loys.gynecomastia
girls. polycysticovariandisease, earlymenarche,irregularmenses
psychological.teasingdecreasedsell-esteem,unhealthycopingmechanisms,depression
management
encouragement andreassurance,engagementolentirelamily
diet. qualitative changes,donotencourageweightlossLutallowlorlineargrowthto
catchupwithweight,specialdietsusedLyadultsarenotencouraged
evidenceagainstverylowcalorie dietslor
readolescents
Lehaviourmodilication.increaseactivity, c
angeeatinghaLits / mealpatterns
insullicientevidencelororagainstexercise,lamilyprogramsloroLesechildren
education.multidisciplinaryapproach,dietitian, counselling
surgeryandpharmacotherapyarenotusedinchildren
Colic
ruleol3s.unexplainedparoxysmsolirritaLilityand crying lor>3hours /dayand
>3 days / weeklor>3weeks inanotherwisehealthy, well-ledLaLy
occurs in J0,olinlants
etiology. generally regardedasalaginthedevelopmentolnormal peristalticmovement
ingastrointestinaltract,othertheoriessuggestalackolsell-soothingmechanisms
otherreasonswhyLaLies cry.wet,hungerorgaspains,toohotorcold,overstimulated,
needtosuckorLeheld
timing.onsetJ0daysto3monthsolage,peak6-8weeks
childcries,pullsuplegsandpassesgassoonalterleeding
management
arentalreliel, restandreassurance
oldLaLy, soother, carride,music,vacuum, checkdiaper

medicationsOvol`drops,gripewater)olnoprovenLenelit
ilLreastleeding,eliminationolcows milkproteinlrommother sdietellectivein
verysmallpercentageolcases)
trycaseinhydrosylateslormulaMutramigen)
Milk Caries
decayolsuperiorlrontteeth andLack molars inlirst4yearsollile
oltenoccurinchildrenputtoLed withaLottleolmilkorjuice
canalsoLecausedLyLreast-leedingespeciallyprolongednightleeds)
prevention
noLottleatLedtime unlessplainwater)
usewateras thirstquenchersduringtheday, donotsweetenpacilier
cancleanteethwithsoltdampclothortoothLrushandwater
avoidlluoridatedtoothpasteuntilaLletospit>3years)Lecauseollluorosisrisk
stainsteeth)
CanadianDentalAssociationrecommendsassessmentLydentist6monthsalter
eruptionollirsttooth,orLyJ yearolage
Injury Prevention Counselling
injuriesaretheleadingcauseoldeathinchildren>Jyear olage
maincauses. motorvehicle crashes,Lurns, drowning, lalls,choking,inlanticide
Tabl e 1 0. I njury Prevention Counsel l i ng
(6 months
Do not leave infant alone on bed,
on change table or in tub
Keep crib rai l s up
Check water temp before bathi ng
Do not hol d hot l i qui d and i nfant
at the same ti me
Turn down hot water heater
Check mi l k temp before feedi ng
Have appropriate car seats -
required before allowed to leave
hospi tal
<9 kg: rear-faci ng
1 0-1 8 kg: front-faci ng
1 8-36. 4 kg: booster seat
61 2 months
I nstal l stai r barriers
Discourage use of wal kers
Avoid play areas wi th
sharp-edged tabl es and
corners
Cover electrical outlets
Unpl ug appl i ances when
not i n use
Keep smal l objects, pl asti c
bags, cleani ng products, and
medicati ons out of reach
Superi sed during feedi ng
always have Poison Control number by telephone
have smoke and carbon monoxide detectors in the house and check yearly
Poison Prevention
1 2 years
Never leave unattended
Keep pot handles turned to
back of stove
No nuts, raw carrots,
etc. due to choki ng hazard
No runni ng whi l e eati ng
25 years
Bicycle hel met
Never leave unsuperi sed at
home, driveway or pool
Teach bi ke safety, stranger safety,
and street safety
Swi mmi ng lessons, sunscreen,
toddler seats i n the car, fences
around pools, denti st by age 3
keepalltypes olmedicines,vitamins, andchemicals lockedupina secure container
potentiallydangerous.drugs,draincleaners,lurniturepolish, insecticides, cosmetics,nail
polishremover, automotive products
donotstoreanychemicalsinjuice,soltdrink, orwaterLottles
keepalcoholicLeveragesoutolachildsreach.aslittleas3ouncesolhardliquorcankill
a2-year-old
alwaysreadthelaLelLeloreadministeringmedicinetoensureitistherightdrugand
correctdose
alwayskeepthetelephonenumLerolPoisonControlnearyourphone
Sudden Infant Death Syndrome (5105)
Definition
suddenandunexpecteddeatholaninlant<J2monthsolageinwhichthe cause oldeath
cannotLeloundLyhistory, examinationora thoroug postmortemand death scene
investigation
Pediatrics PH
P12 Pediatrics Primary Care Pediatrics Toronto Notes 2010
E pidemiology
0. 5/ J,000 leading cause oldeathLetweenJ-J2monthsolage), M.F * 3. 2
morecommoninchildrenplacedinproneposition
inlullterminlants, peakincidenceis2-4months,95,olcases occurLy6months
increaseindeathsduringpeakrespiratorysyncytialvirus RSV)season
mostdeathsoccurLetweenmidnightand8AM
Risk Factors
morecommoninprematurity, ilsmokinginhousehold,minoritieshigherincidencein
aLoriginalsandAlricanAmericans), socially disadvantaged
riskolSIDSisincreased3-5timesinsiLlingsolinlantswhohavediedolSIDS
Prevention - "Back to Sleep, Front to Play"
place inlantonLack, MOTinprone positionwhensleeping
allowsupervisedplaytimedailyinproneposition
alarms /othermonitorsnotrecommended increaseanxietyanddonotprevent
lile-threateningevents
avoidoverheatingandoverdressing
appropriate inlantLeddinguselirmmattress,avoidlooseLedding, donotusecriL
Lumperpads)
nosmoking
paciliers appear tohavea protective ellect,donotreinsertillallsout
Circumcision
electiveproceduretoLe perlormedonlyinhealthy, staLleinlants
contraindicatedwhengenital aLnormalitiespresenthypospadias,etc. )or knownLleeding
disorder
usually perlormed lor socialorreligiousreasonsnotcoveredLyOHIP)
complications J,). localinlection,Lleeding,urethralinjury
medicalLenelits includepreventionolphimosis,slightly reduced incidence olUTI,
Lalanitis, cancer olthepenis
2recentRCTs (Lancet 369, FeL200/)suggestedthatroutinecircumcisionsignilicantly
reduced HIVtransmissionstudies conducted inhigh endemic areas, i. e. Alrica),
circumcisionalso appears toreduceHPVtransmission
routinecircumcisionisnotcurrentlyrecommendedLytheCPSorAAP
Toilet Training
90, olkids attainLowelcontrolLeloreLladdercontrol
generallylemalestrainearlierthanmales
25,Ly2yearsoldinMorthAmerica),98,Ly3yearsoldhavedaytimeLladdercontrol
signsoltoiletreadiness
amLulatingindependently, staLleonpotty, desiretoLeindependentortoplease
caregivers i.e. motivation),sullicientexpressiveandreceptivelanguageskills
2-stepcommandlevel),canstaydrylor severalhourslargeenoughLladder)
Approach to the Crying/Fussing Child
History
descriptionolinlantsLaselineleeding, sleeping, cryingpatterns
inlectioussymptoms lever,tachypnea,rhinorrhea,illcontacts
leedingintolerance gastroesophagealrelluxwithesophagitis
nausea,vomiting,diarrhea,constipation
trauma
recentimmunizationsvaccinereaction)ormedicationsdrugreactions),including
maternaldrugstakenduringpregnancy neonatalwithdrawalsyndrome), anddrugsthat
mayLetranslerred viaLreast milk
inconsistenthistory, patternolnumerousemergency department ED) visits,high-risk
socialsituationsallraiseconcernolaLuse
Physical E xamination
perlormathoroughhead-to-toeexamwiththechildcompletelyundressed
Toronto Notes 2010 Primary Care Pediatrics/Abnormal Child Behaviours
Tabl e 1 1 . The Physical Exami nati on of the Cryi ngussi ng Chi l d
Organ System
HEENT
Neurologic
Cardiovascular
Respirator
Abdomen
Geniournary
Rectal
Musculoskeletal
Examination Findings
Bul gi ng fontanel l e
Bl epharospasm, teari ng
Reti nal hemorrhage
Oropharngeal infections
Irritability or lethargy
Poor perfusion
Tachycardia
Tachypnea
Grunti ng
Mass, empty RLQ
Scrotal swel l i ng
Peni le/clitoral swel l i ng
Anal fissure
Hemoccult positive stool
Point tenderness or decreased movement
Possible Diagnosis
Meni ngi ti s
Corneal abrasion
Shaken baby syndrome
Thrush
Herpangi na
Meni ngi ti s
Sepsi s
Meni ngi ti s
Congestive Heart Fai l ure ( CHF)
Supraventri cul ar tachycardia
Pneumoni a
Respi rator disease
I ntussusception
I ncarcerated herni a
Hai r tourni quet
Consti pation or diarrhea
I ntussusception
Volvul us
Fracture
Osteomyel i ti s
Abnormal Child Behaviours
Elimination Disorders
ENURE SIS
Gi ngivostomatitis
Otitis media
Anomal ous coronar arter
Myocarditis
Tachycardia
CHF
Response to pai n
Testi cul ar torsion
Necroti zi ng Enterocol i ti s
Syphi l i s
Toe/fi nger hai r tourni quet
involuntaryurinaryincontinenceLydayand/ornight typicallyLy5-6yearsold)
wettingatleasttwiceaweekloratleast3consecutivemonthsorcausessignilicant
distress tothechild
treatmentshouldnotLeconsidereduntil6years olage,highrateolspontaneouscure
shouldLeevaluatedil>6yearsold,dysuria,changeingrosscolour,odour, stream,
secondaryordiurnal
Primary Nocturnal E nuresis
wetonlyatnightduringsleep,canLenormaluptoage6
prevalence.J0,ol6-year olds, 3,olJ2-year olds, J, olJ8-yearolds
developmentaldisorderormaturationallaginLladdercontrolwhileasleep
morecommoninLoys,lamilyhistorycommon
treatment.
timeandreassurance -20, resolvespontaneouslyeachyear),Lehaviour
modilicationlimitingnighttimelluids,voidingpriortosleep),engagingchildusing
rewards,Lladderretentionexercises, scheduled toileting
conditioning. wet alarmwakeschilduponvoiding/0, successrate)
medicationsconsideredsecondlinetherapy). DDAVPLynasalsprayororal
taLletshighrelapserate,costly),oxyLutyninDitropan),imipramineTolranil)
rarelyused,lethalil overdose, cholinergicsideellects)
Secondary E nuresis
developsalter childhassustainedperiodolLladdercontrol6monthsormore)
nonspecilicregressioninthelaceolstressoranxietye.g.LirtholsiLling,signilicantloss,
lamilydiscord)
mayalsoLesecondarytourinarytractinlectionUTI),diaLetesmellitusDM),diaLetes
insipidusDI),neurogenicLladder,cereLralpalsyCP),sicklecelldisease,seizures,pinworms
mayoccurilengrossedinotheractivities
treatmentdependsoncause
Diurnal E nuresis
daytime wetting60-80, also wetatnight)
timid,shy temperament proLlems
mayresultlrompsychosocialstressors,ruleoutstructuralanomalies e.g.ectopic
ureteralsite,neurogenicLladder),UTI,constipation,CMSdisorders,mostcommoncauseis
micturitiondelerral
treatment.dependsoncause,Lehaviouralscheduledtoileting, douLlevoiding,good
Lowelprogram),pharmacotherapy
Pediatrics P13
P14 Pediatrics Abnoral Child Behaviours Toronto Notes 2010
ENCOPRE SIS
lecalincontinenceinachild>4yearsold,atleastoncepermonthlor3months
prevalence.J-J. 5,olschool-agedchildrenrareinadolescence),M.F * 6. Jinschool-aged
children
usuallyassociatedwithchronicconstipation
mustexcludemedicalcauses e.g. Hirschsprungdisease,hypothyroidism,hypercalcemia,
spinalcordlesions,anorectalmallormations)
Retentive E ncopresis
causes
physical.anallissurepainlulstooling)
emotional.disturLedparent-childrelationship,coercivetoilettraining,socialstressors
history
childwithholdsLowelmovement,developsconstipation,leadingtolecalimpaction
andseepageolsoltorliquidstooloverllowincontinence)
crosseslegsorstandsontoesto resisturgetodelecate
distressedLysymptoms,soilingolclothes
toilettrainingcoerciveorlackinginmotivation
mayshowoppositionalLehaviour
physicalexam
digitalrectalexam.largelecalmassinrectalvault
anallissuresresultlrompassageolhardstools)
treatment
completeclean-outolLowel
enemasandsuppositories
maintenanceolregularLowelmovements-complianceiscrucial
stoolsoltenerse.g.Colace,Lactulose,Lansoyl`,mineraloilregularly)
dietmodilicationseePediatric Gastroenterolog, P34)
toiletscheduleandpositivereinlorcement
assessmentandguidanceregardingpsychosocialstressors
Lehaviouralmodilication
complications.continuingcycle,toxicmegacolonrequires>3-J2monthstotreat),Lowel
perloration
Sleep Disturbances
Daily Sleep Requirement
<6months J6hours
6months J4.5hours
J2months J3. 5hours
2years J3hours
Nap Patterns
2/ dayatJyear
J / dayat2years. 2-3hours
0.5/dayat5years. J. /hours
4years
6years
J2years
J8years
Types of Sleep Disturbances
insullicientsleep quantity
dillicultylallingasleep
e.g.LimitSettingSleepDisorder
preschoolandolderchildren
Ledtimeresistance
JJ.5hours
9.5hours
8.5hours
8hours
duetocaregiversinaLilitytosetconsistentLedtimerulesandroutines
oltenexacerLatedLychildsoppositionalLehaviours
poorsleepquality
lrequentarousals
e.g. SleepOnsetAssociationDisorder
inlantsandtoddlers
childlearnstolallasleeponlyundercertainconditionsorassociationswith
parent,withlighton,inlrontoltv)
childlosesaLilitytosellsoothe
duringthenormalLrielarousalperiodsolsleepq90-J20min),childcannotlall
LackasleepLecausesameconditionsarenotpresent
parasomnias
episodicnocturnalLehaviours
olteninvolvescognitivedisorientationandautonomic /skeletalmuscledisturLance
e.g. sleepwalking,sleepterrors,nightmares
Management of Sleep Disturbances
setstrictLedtimesandwind-down routines
donotsendchildtoLedhungry
always sleep inLed, darkandquietandcomlortaLleroom, withoutassociations
Toronto Notes 2010 Abnoral Child Behaviours/Child Abuse and Neglect
donotuseLedroomlortimeouts
systematicignoringandgradualextinctionlorsleeponsetassociationdisorder
positivereinlorcementlorlimitsettingsleepdisorder
Nightmares
prevalence. commoninLoys, 4-/years old
associatedwithREMsleep anytimeduringnight)
uponawakening,childisalertandclearlyrecalls lrighteningdream
mayLeassociatedwithdaytimestress/anxiety
treatment. reassurance
Night Terrors
prevalence.J5,olchildrenhaveoccasionalepisodes
aLruptsittingup,eyesopen, screaming
panicandsignsolautonomicarousal
occursinearlyhoursolsleep,non-REM,stage4olsleep
no memoryolevent,parentsunaLletocalmchild
stress/anxietycanaggravatethem
course.remitsspontaneouslyatpuLerty
treatment.reassurance
Breath-Holding Spells
occur in0. J-5, olhealthy children6months-4yearsolage
spellsusually start duringlirstyearollile
2types
cyanoticmorecommon),usuallyassociatedwithanger/lrustration
pallid,usuallyassociatedwithpain/surprise
childisprovokedusuallyLyanger,injuryorlear)
startstocryandthenLecomessilent
spellresolves spontaneouslyorthechildmayloseconsciousness,rarelyprogressesto
seizures
treatment
Lehavioural helpchildcontrolresponsetolrustrationandavoiddrawingattention
tospell
avoidLeingtoopermissiveinlearolprecipitatingaspell
Child Abuse and Neglect
Definition
anactolcommissionaLuse physical, sexual, orpsychological)oromissionneglect)Lya
caregiverthatharms achild
Legal Duty to Report
uponreasonaLlegroundstosuspectaLuseand/orneglect, physicians arerequiredLylaw
to contacttheChildrensAidSociety CAS) personallytodiscloseallinlormation
dutyto report overridespatientconlidentiality,physicianisprotectedagainstliaLility
ongoingdutyto report. ilthereareadditionalreasonaLlegroundstosuspectaLuseand/or
neglect,alurtherreporttotheCASmustLemade
Risk Factors
environmentallactors
social isolation
poverty
domesticviolence
caregiverlactors
parentswereaLusedaschildren
psychiatricillness
suLstanceaLuse
singleparentlamily
poorsocialandvocationalskills,Lelowaverage intelligence
childlactors
dilliculttemperament
disaLility, specialneeds e.g.developmentaldelay)
premature
Presentation of Physical Abuse
historyinconsistentwithphysicallindings, orhistorynotreproduciLle
delayinseekingmedicalattention
injuriesolvariedages,recurrentormultipleinjuries
distinctivemarks.LeltLuckle,cigaretteLurns,handprints
patternsolinjury.Lruisesonthelace,aLdomen,Luttocks,genitalia,upperLack,posterior
riLlractures,immersionLurnsi.e.hotwater)
Pediatrics P15
"If no crui si ng, no brui si ng."
Presentation of Neglect
Fai l ure to thrive, devel opmental del ay
I nadequate or di rty clothi ng, poor
hygi ene
Chi l d exhi bits poor attachment to
parents, no stranger anxiety
P16 Pediatrics Child Abuse and NeglectAdolescent Medicine Toronto Notes 2010
alteredmentalstatus.headinjury,poisoning
physicallindingsnotconsistentwithanyunderlyingmedicalcondition
shakenLaLysyndrome
violentshakingolinlantresultinginintracranialhemorrhages,retinalhemorrhages,
and posteriorriLlractures
diagnosisconlirmedLyheadCTorMRI,ophthalmologicexam,skeletalsurvey/Lone
scan
head traumaistheleadingcauseoldeathinchildmaltreatment
Sexual Abuse
prevalence.J in4lemales,J in J0 males
peakagesat2-6andJ2-J6years
mostperpetratorsaremaleandknowntochild
indecreasingorder.lamilymemLer,non-relativeknowntovictim,stranger
presentation
disclosure. diagnosisusuallydependsonchildtellingsomeone
psychosocial. specilicorgeneralizedlears,depression,nightmares,socialwithdrawal,
lackoltrust,lowsell-esteem,schoollailure,sexuallyaggressiveLehaviour, advanced
sexualknowledge,sexualpreoccupationorplay
physicalsigns.recurrentUTIs,pregnancy,STIs,vaginitis,vaginalLleeding,pain,
genitalinjury,enuresis
investigationsdependonpresentation,age,sex,andmaturityolchild
sexualassaultexaminationkitwithin24hoursilprepuLertal,within/2hoursilpuLertal
ruleoutSTI,UTI,pregnancyconsiderSTIprophylaxisormorningalterpill)
ruleoutotherinjuries vaginal /anal /oralpenetration,lractures,headtrauma)
Management of Child Abuse and Neglect
history
lromchildandeachcaregiverseparately ilpossiLle)
physicalexam
headtotoedonotlorce)
emotionalstate
development
documentand/orphotographallinjuries. type,location,size,shape,colour, pattern
Leawareolredherrings e.g.MongolianLluespotsvs.Lruises)
investigations
Lloodteststoruleoutmedicalcausese. g.thromLocytopeniaorcoagulopathy)
STIwork-up
skeletalsurvey/Lonescan
CT/ MRI
ophthalmologyexam
reportallsuspicionstoChildrensAidSociety,requestemergencyvisitilimminentriskto
childoranysiLlingsinthehome
acutemedicalcare.hospitalizeilindicatedorilconcernsaLoutlurtherorongoingaLuse
arrangeconsultationtosocialworkandappropriatelollow-up
mayneedtodischargechilddirectlytoCASortoresponsiLleguardianunderCASsupervision
Adolescent Medicine
Normal Sexual Development
puLertyoccurswiththematurationolthehypothalamicpituitarygonadalaxis
increasesinthepulsatilereleaseolgonadotropinhormone CnRH) increasedrelease
olLHandFSH maturationolgonadsandreleaseolsexsteroids secondarysexual
characteristics
alsorequiresadrenalproductionolandrogens
Females
occursLetweenage/-J3yearsmaystartearly as6yearsinAlrican-Americangirls)
usualsequence
thelarche. LreastLuddingLreastasymmetrymayoccurasoneLreastmaygrow
lasterthantheother,LecomeslessnoticeaLleasmaturationcontinues)
adrenarche.axillaryhair,Lodyodour,mildacne
growthspurt
menarche. meanage J3years, occurs2yearsalterLreast development andindicates
thatgrowthspurtisalmostcompleteTannerStage4)
earlypuLertyiscommonandoltenconstitutional,latepuLertyisrare
Toronto Notes 2010 Adolescent Medicine
Males
occursLetweenage9-J4yearsstarts2yearslaterthaningirls)
usualsequence
testicularenlargement
penileenlargement.occursatTannerStage4
adrenarche. axillaryandlacialhair,Lodyodour,mildacne
growthspurt. occurslaterinLoysTannerStage4)
earlypuLertyisuncommonneedtoruleoutorganicdisease)LutlatepuLertyiscommon
andoltenconstitutional
Tabl e 1 2. Tanner Stagi ng ( Sexual Maturity Rati ngl
FEMALE MALE
Stage Breast Pubic Hair Genitalia Pubic Hair
Bud Sparse l abi al hai r Scrotal/testes enlargement Sparse hair at base of peni s
3 Bud enl arges Hair over pubi s I ncrease i n l ength of peni s Hai r over pubi s
4 Areola + papi l l a Coarse adul t hai r Further i ncrease i n length Coarse adult hai r
secondary mound and breadth of peni s
Areola recedes Extends to medi al thi gh Adult si ze and shape Extends to medi al thi gh
adul t si ze and shape
Normal Variation In Puberty
Premature Thelarche
isolatedLreasttissuedevelopmentingirls6monthsto2years
requirescarelulhistoryandphysicaltoensurenootherestrogenellectsorothersignsol
puLertye.g.growthspurt)
mayLeduetoincreasedsensitivitytoestrogen
requiresoLservationandperiodicexaminationsevery6-J2monthstoensurenolurther
signsolpuLerty
Physiologic L euk orrhea
occursinthe6monthspriortomenarche, scantmucoid,cleartomilkyvaginaldischarge,
notassociatedwithpruritisorloulodour
duetostimulationolendometrialglandsLyestrogen
Irregular Menstruation
mensesmayLeirregularindurationandlengtholcycle
onaverageittakes J8monthstogothroughthelirst J2periods
LirthcontrolpillsshouldLeavoidedastreatment
Premature Adrenarche
usuallydevelopsinLoys andgirlsLeloretheageol6,Lenignsell-limitingcondition
adrenalproductionolDHEAS precursor olandrostenedione, testosteroneandestrogen)
reachespuLertallevelsatanearlierage
puLicandaxillaryhair,Lodyodour,mildacne
determinewhetherothersignsolpuLertyarepresentgirls thelarche,Loys testicular
enlargement)
excludeandrogensecretingtumoursinvestigations.DHEASlevels,androstenedione,
testosterone,Loneage)
Gynecomastia
transientdevelopmentolLreasttissueinLoys
commonsell-limitedconditionseenin50,olmaleadolescentsduringpuLerty
mustdistinguishtrueLreasttissuelromlat.J-3cmround,moLile,sometimestender,lirm
massunderareola
dischargelromnippleorlixedmassshouldLeinvestigated
Other Adolescent Medicine Topics
SuLstanceALuse see Ps_chiatr_
EatingDisorders see Ps_chiatr_
Depression/ Suicide see Ps_chiat_
SexuallyTransmittedInlections seeC_necolo_
Pediatrics P17
- `
P
Adolescent Psychosocial Assessment
HEADSS
Home
Educati on/Employment/Eating
Acti vi ti es
Drugs
Sexual i ty/Sui ci de and depressi on
Salety
PIS Pediatrics
-`
+
Fetal ci rcul ati on i s desi gned so that
oxygenated bl ood i s preferenti al l y
del ivered to the brai n and myocardi um.
Cardiolog Toronto Notes 2010
Cardiology
Heart Murmurs
50-80, olchildrenhave audiLleheartmurmurs atsome pointintheirchildhood
mostchildhoodmurmursarelunctionale.g. innocent)withoutassociatedstructural
aLnormalitiesandhavenormalECCandradiologiclindings
ingeneral, murmurs canLecomeaudiLle oraccentuatedinhighoutputstates,e.g.lever,
anemia
Tabl e 1 3. Diferentiating I nnocent and Pathol ogi cal Heart Murmurs
Innocent Pathological
Hi stor and Physi cal
Ti mi ng
Asymptomatic
Systol i c ejection murmur ( SEM)
<3/6
Symptoms and si gns of cardiac disease , Jexercise i ntol erance)
Al l di astol i c, pansystolic, or conti nuous (except venous hum)
Grade ' 3/6 (pal pabl e thri l l )
Spl i tti ng
Extra sounds/Clicks
Change of Position
Physiologic S2
None
May have fi xed spl i t or si ngl e S2
May be prese nt
Murmur varies Unchanged
Tabl e 1 4. Five I nnocent Heart Murmurs
Type Descripton Age
Peripheral Pul moni c Stenosi s Neonates, l ow pi tched, radiates neonates, usual ly
to axi l l a and back di sappears by 36 mos
Sti l l 's Murmur Vi brator, lower l eft sternal 36 yrs
border (LLSB) or apex, SEM
Venous Hum Infracl avi cul ar hum, conti nuous, 36 yrs
R >L
Pul monary Ejection Soft, blowi ng, upper left 8 1 4 yrs
sternal border (ULSB), SEM
Supraclavi cul ar Areri al Brui t Low i ntensity, above cl avi cl es any age
Congenital Heart Disease (CHD)
PRENATAL CIRCULATION
Before Birth
letallungsLypassedLyllowthroughletalshunts.
shuntingdeoxygenatedLlood
Diferental Diagnosis
Patent Ductus Arteriosus (PDA)
Pul monar stenosi s
Subaori c stenosis
Small ventri cul ar septal defect (VSD)
PDA
Atrial septal defect (ASD)
Pul monar stenosi s
Aortic stenosi s
Bi cuspi d aortic valve
ductusarteriosus.connectionLetweenpulmonaryarteryandaorta
shuntingoxygenatedLlood
loramenovale.connectionLetweenR andLatria
ductusvenosus.connectingLetweenumLilicalveinandIVC
circula tion.
placentaoxygenatedLlood)>umLilicalvein >ductusvenosus >IVC>Ratrium >
oxygenatedLloodshuntedthrough loramen ovale>Latrium >Lventricle >aorta >
Lrain/myocardium/upperextremities
deoxygenatedLloodreturnsviaSVCtoRatrium >J / 3 olLloodenteringRatrium
doesnotllowthroughloramenovaleandllowstotheRventricle >pulmonary
arteries >ductusarteriosus >aorta >systemiccirculation >placentalorreoxygenation
At Birth
withlirstLreath,lungsopenupand pulmonary resistancedecreasesallowingpulmonic
Lloodllow
withseparationollowresistanceplacenta,systemiccirculationLecomesahighresistance
system
withclosureoltheletalshuntsandchangesinpulmonic / systemicresistance,inlant
circulationassumesnormaladultllow
increasingpulmonicllowincreasesleltatrialpressuresleadingtoloramenovaleclosure
increasedoxygenconcentrationinLloodalterlirstLreathleadstodecreased
prostaglandinsleadingtoclosureoltheductusarteriosus
astheumLilicalcordisclamped,theumLilicalveincloses,systemicvascularresistance
increasesandtheductusvenosuscloses
Toronto Notes 2010 Cardiolog
E mbryologic Development
mostcriticalperiodolletalheart developmentisLetween3-8weeksgestation
singlehearttuLegrowsrapidlylorcingittoLendLackuponitsellandLegintoassumethe
shapeola4chamLeredheart
insultsatthistimearemostlikelytoleadtoCHD
E pidemiology
8/ J,000liveLirths canpresentwithheart murmur, heart lailure,orcyanosis
ventricularseptaldelectisthemostcommonlesion
Tabl e 1 5. Ri sk Factors for Common CHD
I NFANT FACTORSGENETI C CONDI TI ONS MATERNAL FACTORS
Abnonalit Dominant cardiac defect Abnonalit (% rsk) Dominant cardiac defect
Prematurity PDA Prior chi l d wi th CHD (24% risk)
CHARGE association TOF AVSD, ASD, VSD Torch esp. rubel l a ( 35%) PDA, PS
Di George Aortic arch anomal i es Diabetes Mel l i tus ( 23%) TGA, coarctati on, VSD
Down syndrome AVSD, VSU ASD, TOF PKU ( 2550%) TOF
EhlersDanlos Mitral prolapse, di lated aortic root SLE (2040%) Complete heart block
Kartagener's Dextrocardia Al cohol ( 2530%) ASD, VSD
Marfan Mitral prolapse, aortic dissection or
i nsuffi ci ency, di lated aortic root
Noonan Pul monar stenosis, ASD Medi cati ons: Phenytoi n VSD, ASD, PS, AS, coarctation
Osteogenesi s I mperfecta Aortic i ncompetence Medi cati ons: Valproate Coarctation, HLHS, AS, VSD
Turner Coarctati on, bi cuspi d aortic valve Medi cati ons: Reti noi c aci d Aorti c arch abnormal i ti es
VSD ; ventrcular septal defect: ASD ; atri al septal defect: PDA ; patent ductus areriosus: TOF ; tetralogy of Fallot: TGA ; transposition of great areres:
PS ; pulmonary stenosis: AS ; aoric stenosis: HLHS ; hypoplastic left hear syndrome: AVSD ; atrioventricular septal defect
Investigations
Echo, ECC, CXR
CYANOTIC VS. ACYANOTIC CONGENITAL HEART DISEASE
cyanosis.LluemucousmemLranes,nailLeds,and skinsecondaryto anaLsolute
concentrationoldeoxygenatedhemogloLinolatleast3 g/dL
cyanotic heart disease: ie.R>Lshunt)LloodLypassesthelungs >nooxygenationoccurs
>highlevelsoldeoxygenatedhemogloLinentersthesystemiccirculation >cyanosis
acyanotic heart disease: ie. L > Rshunt,oLstructionoccurringLeyondlungs)Llood
passesthroughpulmoniccirculation >oxygenationtakesplace >lowlevelsol
deoxygenatedLloodinsystemiccirculation >nocyanosis
Pediatrics P19
'
Characteristic Chest X-Ray Findings

in Congenital Heart Disease
BootShaped Heart - Tetral ogy of
Fal lot, Tri cuspi d Atresi a
EggShaped Hear - Transposi ti on of
Great Areri es
"Snowman" Hear - Total
Anomal ous Pul monary Venous Return
LNcN|1L dc1b| bcbc
A. Patent Ductus Arteri osus B. Transposi ti on of Great

Arteries
Fi gure 2. Common Congenital Heart Diseases
cy1aL c
L ~ R shunt
ASD
VSD
PDA
Atrioventri cul ar
Septal defect
( endocardi al
cushi on defect)
I
Obstructive
Coarctati on
Aort i c stenosi s
Pul moni c stenosi s
Uy1aL c al esi cnsj
I
R ~ L shunt
TOF
Ebstei n' s anomal y
Other
I
TGA
Hypopl asti c l eft
heart syndrome
Truncus arteri osus
Total anomal ous
pul monary venous
drai nage
Tri cuspi d atresi a
P20 Pediatrics
-`
Moderate-to-large VSD
Si ze ofVSD i s i nversel y rel ated to
i ntensity ofmurmur.
-`
Physical Exam for PDA

(in term infant)
Heavy "machi nery" murmur
High pul se rate
Wide pul se pressure
Hyperactive precordi um
Bi g boundi ng pul se
Acyanotic Congenital Heart Disease
1. LEFT TO RIGHT SHUNT LE SIONS
extraLloodisdisplacedthroughacommunicationlromthelelttotherightsideolthe
heart increasedpulmonaryLloodllow increasedpulmonary pressures
shuntvolumedependentuponthreelactors.sizeoldelect,pressuregradientLetween
chamLersorvessels,peripheraloutllowresistance
untreatedshuntscanresultinpulmonaryvasculardisease,rightventricularhypertension
andhypertrophyRVH),andeventuallyR Lshunts
Atrial Septal Defect (AS D)
threetypes. ostium primum commoninDown syndrome), ostiumsecundummost
commontype,50-/0,),sinusvenosusdelectlocatedatentryolsuperiorvenacava
mtorightatrium)
epidemiology.6-8,olcongenitalheartlesions
naturalhistory. 80-J00,spontaneousclosurerateilASD diameter<8mm
ilremains patent,congestiveheart lailureCHF)andpulmonaryhypertensioncandevelop
inadultlile
history.oltenasymptomaticinchildhood
physicalexam.grade2-3/ 6pulmonicoutllowmurmurSEM),amid-diastolicrumLleat
theleltlowersternalLorder,andawidelysplitandlixedS2
investigations
ECC. rightaxisdeviationRAD),mildRVH,rightLundleLranchLlock R555)
CXR.increasedpulmonaryvasculature
treatment.electivesurgicalorcatheterclosureLetween2-5yearsolage
Ventricular Septal Defect (VSD)
mostcommoncongenitalheartdelect30-50, ol CHD)
Smal l VSD (majority)
history.asymptomatic,normalgrowthanddevelopment
physicalexam.earlysystolictoholosystolicmurmur,LestheardatleltlowersternalLorder
LLS5)
investigations. ECC and CXRarenormal
treatment.mostclosespontaneously
Moderate-to-Iarge VSD
naturalhistory. secondarypulmonaryhypertension,CHFLy2monthsolage
history.delayedgrowthanddevelopment, decreasedexercisetolerance,recurrentURTIsor
asthma episodes, CHF
physicalexam.holosystolicmurmuratLLS5withthrill,mid-diastolicrumLleatapex,size
olVSDisinverselyrelatedtointensityolmurmur
investigations.
ECC. leltventricularhypertrophyLVH),leltatrialhypertrophyLAH),RVH
CXR.increasedpulmonaryvasculature,cardiomegaly, CHF
treatment.treatmentolCHFandsurgicalclosureLyJyearolage
Patent Ductus Arteriosus (PDA)
patentvesselLetweendescendingaortaandleltpulmonaryartery
epidemiology
lunctionalclosurewithinlirst J5hoursollile,anatomicalclosurewithinlirstdaysol
lile
5-J0,olallcongenitalheartdelects
delayedclosureolductusiscommoninprematureinlantsJ / 3olinlants<J/50grams),
thisisdillerentlromPDAinterminlants
naturalhistory.spontaneous closure commonin prematureinlants,lesscommonin
terminlants
history.mayLeasymptomaticorhave apneic orLradycardicspells,poorleeding,
accessorymuscleuse
physicalexam.tachycardia,Loundingpulses,hyperactiveprecordium,widepulse
pressure,continuousmachinerymurmur,Lestheardatleltinlraclaviculararea
investigations
ECC. mayshowLAH,LVH,5VH
CXR.normaltomildlyenlargedheart,increasedpulmonaryvasculature,prominent
pulmonaryartery
diagnosisLyechocardiography
treatment
indomethacinIndocid) PCEJantagonistPCEJmaintainsductusarteriosus
patency)inprematureinlantsilnecessary
catheterorsurgicalclosureilPDAiscontriLutingtorespiratorycompromise,poor
growthorpersistsLeyond3rdmonthollile
E ndocardial Cushion Defect (Atrioventricular [AVj Canal)
spectrumlrom endocardialcushionVSD andostiumprimumASDtocompleteAVcanal
withcommonAV valve
commonlyassociatedwithDownsyndrome
treatment
naturalhistorydependsonsizeoldelectandvalvularinvolvement,andshouldLe
repairedLyage6monthstopreventdevelopmentolpulmonaryhypertension
completeAV canalrequiresearlycompletesurgicalrepair, preleraLlyLelore3months
olage
2. OBSTRUCTIVE LE SIONS
present withpallor, decreasedurineoutput, coolextremitiesandpoorpulses, shockor
suddencollapse
Coarctation of the Aora
narrowingolaortaalmostalwaysattheleveloltheductusarteriosus
commonlyassociatedwithLicuspidaorticvalve50,),Turnersyndrome35,)
lewhavehigh5Pi ninlancyJ60-200mmHgsystolic)Lutthisdecreasesascollateralsdevelop
ilsevere,presentswithshockintheneonatalperiodwhentheductuscloses
history.oltenasymptomatic
physicalexam.upperextremitysystolicpressuresolJ40-J45mmHg,decreasedLlood
pressureandweak/aLsentpulsesinlowerextremities,radial-lemoraldelay, aLsentor
systolicmurmurwithlatepeakatapex,leltaxilla,andleltLack
investigations.ECC-RVHearlyininlancy,LVHlaterinchildhood
prognosisandtreatment
ilassociatedwithotherlesions e.g. PDA,VSD)cancauseCHF
complications.hypertension
management.giveprostaglandinstokeepductusarteriosuspatentlorstaLilization,
Lalloonarterioplastyorsurgicalcorrectioninsymptomaticneonate
Aortic Stenosis
valvular/5,),suLvalvular20,),supravalvularandidiopathic hypertrophicsuLaortic
stenosisIHSS)5,)
history.oltenasymptomaticLutmayLeassociatedwithCHF,exertionalchest pain,
syncope orsuddendeath
physicalexam.SEMatupperrightsternalLorderURS5)withaorticejectionclickattheapex
treatment
surgicalrepairilinlantwithcriticalaorticstenosisorolderchildwithsymptomsor
peakgradient>50mmHg
exerciserestrictionrequired
Pulmonary Stenosis
valvular90,),suLvalvular,orsupravalvular
usuallypartolothercongenitalheartlesionse. g.TetralogyolFallot)orinassociationwith
othersyndromes e.g.congenitalruLella,Moonansyndrome)
criticalpulmonicstenosis.inadequatepulmonaryLloodllow,dependentonductuslor
oxygenation,progressivehypoxiaandcyanosis
history.spectrumlromasymptomatictoCHF
physicalexam.widesplitS2onexpiration,SEMatULS5,pulmonaryejectionclick
investigations
ECC. RVH
CXR.dilatedpost-stenoticpulmonaryartery
treatment.surgicalrepairilcriticallyillorseverePS,orilpresenceolsymptomsinolder
inlants/children
Cyanotic Congenital Heart Disease
systemicvenousreturnre-enterssystemiccirculationdirectly
mostprominentleatureiscyanosisOsat</5,)
dillerentiateLetweencardiacandothercausesolcyanosiswithhyperoxictest
oLtainpreductal,rightradialA5Cinroomair,repeatA5Calterthechildinspires
J00,oxygen
ilPaOimprovestogreaterthan J50mmHg,cyanosislesslikelycardiacinorigin
survivaldependsonmixingviashuntse.g.ASD,VSD,PDA)
shouldnotuseO
lortheselesions
Pediatrics P21
P22 Pediatrics
Tetralogy of Fallot
1 . Ventri cul ar septal delect (VSD)
2. Ri ght ventri cul ar outllow tract
obstructi on (RVDTD)
3. Aori c root "overri di ng" VSD
4. Ri ght ventri cul ar hypertrophy
See Fi gure 2
- `
Hypoplastc LHS
Hypoplastc LV
Narrow mi tral /aorti c val ves
Small Ascendi ng Aorta
Contracted Aora
1. RIGHT TO LEFT SHUNT LE SIONS
Tetralogy of Fallot
J0,olallCHD, most commoncyanotic heart delectdiagnosedLeyond inlancy
emLryologically, asingle delectwithhypoplasiaoltheconuscausing.
VSD
rightventricleRV)outllowtractoLstructionRVOTO)
overridingaorta
RVH
degreeolRVOTOdirectlydeterminesthe directionanddegreeolshunt andtherelorethe
extentolclinicalcyanosisanddegreeolRVH
inlantsmayinitiallyhaveaL Rshunt andthereloreare not cyanoticLut the RVOTOis
progressive,resultinginincreasingR Lshuntingwith hypoxemiaandcyanosis
history.hypoxic tet spells
primarypathophysiologyishypoxia,leadingtoincreasedpulmonaryvascular
resistancePVR)and decreasedsystemicresistance,occurringinexertionalstates
e.g.crying,exercise)
paroxysmolrapidanddeepLreathing,irritaLilityandcrying
hyperpnea, increasingcyanosisoltenleadingtodeepsleepanddecreasedintensityol
murmurdecreasedllowacross RVOTO)
peakincidenceat2-4monthsolage
ilsevere mayleadtoseizures, loss olconsciousness,deathrare)
management. L_knee-chestposition,lluidLolus,morphinesullate,propanolol
physicalexam.singleloudS2duetoseverepulmonarystenosisi.e.RVOTO)
investigations
ECC. RAD,RVH
CXR.LootshapedheartsmallPA, RVH),decreasedpulmonaryvasculature,right
aorticarchin20,)
treatment. surgical repair withinlirsttwoyears ollile,orearlierilmarked cyanosis, tet
spells,orsevereRVoutllowtractoLstruction
E bstein' s Anomaly
congenitaldelectolthetricuspidvalveinwhichtheseptalandposteriorlealletsare
mallormedanddisplacedintotheRVleadingtovariaLledegreesolRVdyslunction,TS,
TRorlunctionalpulmonaryatresiailRVunaLleto open pulmonic valves
RAmassivelyenlarged,interatrialcommunicationPFO)oltenexistsallowingR L
shunting
TRandaccessoryconductionpathways WPW)are oltenpresent oltenassociatedwith
arrhythmia
cause. unknown,associatedwithmaternallithiumandLenzodiazepineuseinJ` trimester
treatment
innewLorns,considerclosureoltricuspidvalve+aortopulmonaryshunt,or
transplantation
inolderchildren,tricuspidvalverepairorvalvereplacement+ASDclosure
2. OTHE R CYANOTIC CONGENITAL HEART DISEASE S
Transposition of the Great Areries (TGA)
3-5, olall congenitalcardiaclesions,mostcommoncyanoticCHD inneonate
parallelpulmonaryandsystemiccirculations
systemic.Lody RA RV aorta Lody
pulmonary.lungs LA LV pulmonaryartery lungs
physicalexam
nomurmurilnoVSD
newLornpresentswithprogressivecyanosisunresponsivetooxygentherapyasthe
ductusarteriosusclosesandmixingLetweenthetwocirculationsdiminishes,severe
hypoxemia,acidosis,anddeathcanoccurrapidly
ilVSDpresent,cyanosisisnotprominentandinlantpresentswithCHFalteralew
weeksollile
investigations
ECC. RAD,RVH
CXR.egg-shapedheartwithnarrowmediastinumeggonastring)
treatment
prostaglandinEJ ProstinVR`)inlusiontokeepductusopenuntil septostomy or
surgery arterialswitchprocedure)
inlantswithoutVSDmustLerepairedwithin2weekstoavoidweakLVmuscle
Hypoplastic Lef Heart Syndrome
J-3, olallcongenitalcardiac lesions
aspectrumolhypoplasiaolleltventricle,atreticmitraland/oraorticvalves,small
ascendingaorta,coarctationoltheaortawithresultantsystemichypoperlusion
mostcommoncauseoldeathlromcongenitalheartdiseaseinlirstmonthollile
systemiccirculationisdependentonductuspatency,uponclosureoltheductus,inlant
presentswithcirculatoryshockandmetaLolicacidosis
treatment
intuLateandcorrect metaLolicacidosis
IVinlusionolPCEJ tokeepductusopen
surgicalcorrectionoverallsurvival50,tolatechildhood)orhearttransplant
Total Anomalous Pulmonary Venous Connection
onlyJ-2,ol CHD
characterizedLyallolthepulmonaryveinsdrainingintotheright-sidedcirculation
supracardiac SVCorinnominatevein,inlracardiac hepatic/portalveinorIVC,
intracardiac coronarysinusorRA)
no directoxygenatedpulmonaryvenousreturntoleltatrium
oltenassociatedwithoLstructionatconnectionsites
anASDmustLepresenttoallowLloodtoshuntintotheLAandsystemic circulation
treatment. surgicalrepairilseverecyanosisorCHFrelatedtopulmonaryvenous
oLstruction
Truncus Arteriosus
asinglegreat vessel arisinglromtheheartwhichgivesrisetotheaorta,PA,andcoronary
arteries
thetruncalvalveoverliesalargeVSD
treatment.surgicalrepairwithinlirst6monthsolliletopreventdevelopmentol
pulmonaryvasculardisease
Congestive Heart Failure (CHF)
seeCardiolo_
E tiology
congenitalheartdiseaseCHD)
arteriovenousmallormationsAVMs)
cardiomyopathy
arrhythmias
acutehypertension
anemia
corpulmonale
myocarditis
Symptoms
inlant.leedingdilliculties,easylatiguaLility, exertionaldyspnea,diaphoresiswhen
sleepingoreating,respiratorydistress, lethargy, cyanosis,FTT
child.decreasedexercise tolerance,latigue,decreasedappetite,lailuretothrive,respiratory
distress,lrequentURTIsorasthma episodes
orthopnea,paroxysmalnocturnaldyspnea,pedal / dependantedemaarealluncommonin
children
Physical Findings
lourkey leatures. tachycardia,tachypnea,cardiomegaly, hepatomegaly
lailuretothriveFTT)
respiratorydistress,galloprhythm,wheezing,crackles,cyanosis,cluLLingwithCHD)
alterationsinperipheralpulses,lourlimLLloodpressuresinsomeCHDs)
dysmorphicleaturesassociatedwithcongenitalsyndromes
CXR cardiomegaly,pulmonaryvenouscongestion
Management
correctionolunderlyingcause
general. sittingup,O sodiumandwaterrestriction,increasedcaloricintake
pharmacologic.diuretics, digoxin,alterloadreducers
Infective Endocarditis
seeInlectiousDiseases
/0, Streptococcus, 20, Staphylococcus (S. aureus, S. epidermidis)
serialpositiveculturesareneededlordelinitivediagnosis,Lutrelyonclinicalsuspicion
andotherinvestigationsilinitiallynegative i.e.useEchotolooklorvegetations)
J0-J5,olcasesareculturenegative,thisisarisklactorlorpoorprognosis
Oslersnodes,]anewayslesions,splinterhemorrhagesarelatelindingsinchildren
antiLioticprophylaxisisnecessarylorallpatientswith.
cyanoticcongenitalheartdiseaseincludingTetralogyolFallot,TCA,ELsteins
anomaly, totalanomalouspulmonaryvenousreturn)
rheumaticvalvelesions exceptilnovalvedyslunction)
prostheticheartvalves
palliativeshuntsandconduits
Pediatrics P23
- ` *
w
4 Key Features of CHF
2Tachy's and 2 Megaly's
Tachycardi a
Tachypnea
Cardi omegal y
Hepatomegal y
L
Pharmacologic Management of CHF
3 D's
Di ureti cs
Di goxi n
Alterl oad Decreasers
P24 Pediatrics
-`
+
Pediatric vs Adult ECG
Pedi atri c ECG li ndi ngs that may be
normal :
o HR >1 00 bpm
Shorer PR, Oi nterval s and QRS
durati on
Inleri or and l ateral smal l Q waves
RV l arger than LV i n neonates, so
normal to have:
Ri ght axi s devi ati on
Large precordi al R waves
Upri ght T waves
Cardiolog/evelopment Toronto Notes 2010
previous endocarditis
pacemakerleads
S5Eprophylaxis.amoxicillin50mg/ kg30to60minutesLeloreprocedure.Ilallergic
topenicillin,thenuseclindamycin20mg/ kg
highrisk patients lorCI / CUproceduresmayreceive2dosesamp+gentIV30minLelore
procedureand6hourslater)
Dysrhythmias
seeCardiolo_
canLetransientorpermanent,congenitalstructurallynormaloraLnormal)oracquired
toxin,inlection,inlarction)
Sinus Arrhythmia
phasicvariationswithrespiration
presentinalmostallnormalchildren
Premature Atrial Contractions (PACs)
may Le normalvariantor can Le causedLyelectrolytedisturLances,hyperthyroidism,
cardiacsurgery, digitalistoxicity
Premature Ventricular Contractions (PVCs)
commoninadolescents
Lenignilsingle,unilorm,disappearwithexercise,andno associatedstructurallesions
ilnotLenign,maydegenerateintomoreseveredysrhythmias
Supraventricular Tachycardia (SVT)
mostlrequent sustained dysrhythmia inchildren
notlile-threateningLutcanleadtosymptoms
causedLyre-entryviaaccessoryconnectionatrioventricularAV)nodemostcommonsite)
characterizedLyarateolgreater than2J0Lpm
treatment
staLlealert,normal5P)
vagalmaneuvres
adenosine
synchronizedcardioversion
unstaLledecreasedLOC,decreased5P)
immediatesynchronizedcardioversion
Complete Hear Block
congenitalheartLlockcanLecausedLymaternalRhoorLaantiLody e.g.inmotherswith
lupus)
oltendiagnosedinutero mayleadtodevelopmentolletalhydrops
clinicalsymptomsrelatedtolevelolLlockthelowertheLlock,thegreaterthesymptoms
olinadequatecardiacoutput)
symptomaticpatientsneedapacemaker
Development
Developmental Delay
developmentaldelayisdelinedasperlormancesignilicantlyLelowaverage inagivenarea
E pidemiology
5-J0,olchildrenhaveneurodevelopmentaldelay
mayhaveisolateddelays inspecilicareas motor,speech/ language),orgloLaldelays
E tiology
genetic /chromosomaldisorderstrisomy2J, Fragile X)
intrapartumasphyxia
CMSaLnormalitiesmeningitis / encephalitis,TORCHinlections,structural)
metaLolicdisordersinLornerrorsolmetaLolism,hypothyroidism)
environmentalpsychosocialneglect,leadexposure,antenataldrugoralcoholexposure)
Toronto Notes 2010 Development
Approach
detailedhistory
intrauterineexposures,perinatalevents
lamilyhistory, consanguinity
detaileddevelopmentalmilestones-rateolacquisition,regressionolskills
associatedproLlems leeding,seizures,Lehaviour,sleep)
socialhistory
physicalexamination
dysmorphology, hepatosplenomegaly,neurocutaneousmarkers,growthparameters,
detailedneurologicalexamination
ancillarytesting
neurodevelopmemalassessment,hearingtest, psychosocialevaluation,occupational
therapyand/orphysiotherapyassessments,geneticsconsultation
laLoratorytesting

target testingbasedonhistoryandphysicalexam
chromosomes,FISH,neuroimaging,metaLolictesting,neuroelectophysiologictesting
Intellectual Disability
MMMMMMMMMMMMMMMMMMM
E pidemiology
J, olgeneralpopulation,M.F - J. 5. J
higherratesolsensorydelicits,motorimpairment,Lehavioural / emotionaldisorders,
seizures,psychiatricillness
E tiology
genetic. Downsyndrome, FragileX,PKU, otherchromosomaldisorders,developmental
LrainaLnormality,inLornerrorsolmetaLolism
prenatal.ruLella,letalalcoholsyndrome,prenatalexposuretoheroin, cocaine,TORCH
inlections,HIV, maternalDM,toxemia,maternalmalnutrition,Lirthtrauma/ hypoxia
perinatal.prematurity, lowLirthweight, cereLralischemia,intracranialhemorrhage,
maternaldeprivation
childhood.intracranialinlection,headtrauma,FTT,leadpoisoning
psychosociallactors.mildMRassociatedwithlowsocioeconomicstatusSES),limited
parentaleducation,parentalneglect,teenpregnancylamilyinstaLility
Diagnosis
Lelow averagegeneralintellectualluctioningasdelinedLyanIQolapproximately/0or
Lelow 2standarddeviations Lelow themean)AM
delicitsinadaptivelunctioninginatleasttwool.
communication,sell-care,home-living,socialskills,sell-direction,academicskills,
work,leisure,health,salety
onsetLelore J8yearsolage
Tabl e 1 6. Classification of I ntellectual Di sabi lity
Severit % Cases I n
Mi l d 85% 5070
Moderte , 0% 3549
Sever
Prfound
Treatment
34%
' 2%
2034
<20
mainoLjective.enhanceadaptivel:mctioninglevel
therapy
emphasizecommunity-Lasedtreatmentvs.institutionalizationandearlyintervention
individual /lamilytherapy, Lehaviour modilicationtodecrease
aggressive /distractingLehaviours),multidisciplinaryrehaLilitation,medicationslor
associatedconditions
education.lileskills,vocationaltraining,communicationskills,lamilyeducation
psychosocialsupportolparentsandrespitecare
Pediatrics P25
P26 Pediatrics Development Toronto Notes 2010
Language Delay
Diferential Diagnosis
hearingimpairment
spectrumolimpairment slighttoproloundloss
languagedevelopmentmayseemnormallorupto6monthsincludingcooingand
LaLLling)Lutmayregressdueto lack olleedLack
risklactorslorsensorineuralhearingloss>Jrisklactorwarrantsinlantscreening,il
newLornscreeningnotavailaLleinjurisdictionlorallnewLorns).
geneticsyndromes/lamilyhistory
congenitalTORCH)inlections
craniolacial aLnormalties
<J,500gLirthweight
hyperLlmLmema/kerncterus
asphyxia/ lowAPCARscores
Lacterialmeningitis,viralencephalitis
toevaluatehearinglossinchildren.
<6monthsold. auditoryLrainstemresponse A5R). tympanometry
impedancetesting),evokedpotentials
>6-8monthsold.Lehaviouraudiometry
>3-4yearsold. puretoneaudiometry
cognitivedisaLility
gloLaldevelopmentaldelay,mentalretardation
Lothreceptiveandexpressivelanguagecomponentsallected
childoltenhasinterestincommunication
pervasivedevelopmentaldisorder PDD),includingautism
poorsocialinteractionandlanguageimpairment,stereotypicalLehaviours
selectivemutism
achildhoodanxietydisorderwithonsetage5-6
onlyspeaksincertainsituations,usuallyathome
healthychildrenwithnohearingimpairment
oltenaLove-averageintelligence
Landau-Klellersyndromeacquiredepilepticaphasia)
presentsinlatepreschooltoearlyschoolageyears,mayLesimilartoautism
childLeginstodeveloplanguagenormally,thensuddenregressionollanguage
childhas severe aphasiawithEECchanges,oltenhasovert seizure activity
mechanicalproLlems
cleltpalate
cranialnervepalsy
socialdeprivation
Management
EMTanddentalrelerralilmechanicalcause
speechtherapyindisordersollluency,receptiveorexpressivelanguage
psychiatricconsultationinselectivemutism,PDD
Learning Disorders
Definition
a specilic andpersistentlailuretoacquireacademicskillsdespiteconventionalinstruction,
adequateintelligence,andsocioculturalopportunity
asignilicantdiscrepancyLetweenachildsintellectualaLilityandhisorheracademic
perlormance
E pidemiology
prevalence.2-J0,
psychiatriccomorLidity* J0-25,olindividualswithdysthymia,conductdisorderCD),
majordepressivedisorderMDD),oppositionaldeliantdisorderODD),attentiondelicit
hyperactivitydisorder ADHD)
Diagnosis
categorizedLy
individualscoresonachievementtestsinreading,mathematicsorwrittenexpression
WISCIII,WRAT)signilicantlyLelow>2SD)thatexpectedlorage,education,andIQ
interlereswithacademicachievementorADLsthatrequirereading, mathematics,or
writingskills
types.reading, mathematics,disordersolwrittenexpression
ruleoutoccultseizuredisorder,sensoryimpairments
Complications
lowsell-esteem,poorsocialskills
40,schooldrop-outrate
Toronto Notes 2010 DevelopmentEndocrinolog
Fetal Alcohol Spectrum Disorder (FASD)
thisdisorderrelerstothelullrangeolproLlemsresultinglromtheuseolalcoholduring
pregnancy,includingFetalAlcohol Syndrome FAS)and FetalAlcohol Ellects FAE)
Fetal Alcohol Syndrome
prevalenceol FAS. J in300
notknownhowmuchalcoholisharmlulduringpregnancy
criterialorDiagnosisolFetalAlcohol Syndrome
a)growthdeliciency lowLirthweightand/orlengthatLirththatcontinuesthrough
childhood
L)aLnormalcraniolacialleatures smallhead,shortpalpeLrallissures,longsmooth
philtrum,thinupperlip
c)centralnervoussystemdyslunction microcephalyand/orneuroLehavioural
dyslunctione.g.hyperactivity,linemotorproLlems,attentiondelicits,learning
disaLilities,cognitivedisaLilities)
d)strongevidenceolmaternaldrinkingduringpregnancy
cardiacandrenaldelects,hypospadiasmayoccur
FASDisoneolthemostcommoncausesolmentalretardationintheworld
Fetal Alcohol Spectrum Disorder
prevalence olFASD.J in200
childLorntoamother whowasknowntoLedrinkingheavilyduringpregnancy
childhassomeLutnotallolphysicalcharacteristicsolFAS,oltenmisseddiagnosisas
leaturesaresuLtle
mostchildrenwithFASDhavenormalstatureanddonothavemicrocephaly, however,
theymayshowmilder lorms olthelacial leatures present inFAS
ilpresent,shortstatureandmicrocephalywillpersistthroughadolescence, while lacial
leatureswillLecomemoresuLtlewithage
Endocrinology
Diabetes Mellitus (DM)
seeEndocrinolo_
TYPE 1 DIABE TE S (Insulin- Dependent DM)
E pidemiology
insulindependent, mostcommontypeinchildhood
prevalence.J in400-500childrenunder J8yearsolage
canpresentatanyage,Limodalpeaks5-/yearsandpuLerty
classicpresentation.polyuria,polydipsia,aLdominalpain,weightloss,andlatigue
25, presentwith diaLetic ketoacidosisDKA)
E tiology
geneticpredispositionandenvironmentaltrigger
autoimmunedestructionolLeta-cellsolthepancreasantiLodiesdirectedtowards
glutamicaciddecarLoxylasehaveLeenidentilied)
anon-immunevariationhasLeendescriLed
diseasesolpancreasi.e.cysticliLrosis)andlongtermcorticosteroids
Management of Uncomplicated Diabetes
mealplan,exercise, education, psychosocialsupport
insulininjections2-3timesperday,Lloodglucosemonitoring
youngchildrenmoresusceptiLletoCMSdamagewithhypoglycemiawithlewerLenelits
lromtightcontrol,hencetargetglucoserangehigherat6-J2mmol / LJJ0-220mg/dL)
increasinglytightercontrolinolderchildren,4-8mmol / L/0-J40mg/dL)
continuoussuLcutaneousinsulininlusionCSII)pump
containsacartridgelullolshort-actinginsulinLisproTM) orasyringeconnectedtoa
catheterthatisinsertedintothesuLcutaneoustissue
continuouslydeliverspredeterminedLasalratestomeetnonprandialinsulin
requirements
Lolusinlusiontocovermealtimeorsnacktimeinsulinrequirements
requiresasmuchormoreLloodglucosemonitoringwhencomparedtoinjections
ketonemonitoring- patients mustLehighlymotivated
allowslortighterglycemiccontrol
riskolDKAwithoperatorormechanicallailurecatheterocclusion,Latterylailure,
depletedinsulin)
Pediatrics P27
- `
No "sale" l evel ofal cohol consumpti on

duri ng pregnancy has been establ i shed.
M
Diagnostic Criteria for Diabetes
Mellitus
Symptoms (pol yuri a. pol ydi psi a. wei ght
l oss) + random gl ucose ; 1 1 . 1 mmol/L
(200 mg/dL)
OR
Fasti ng gl ucose ; 7. 0 mmol/L
( 1 26 mg/dL)
OR
2hr gl ucose duri ng OGTT ; 1 1 . 1 mmol /L
( 200 mg/dL)
OGToral glucose tolerance test
II a chi l d presents with pol yuri a and

polydi psi a. di p uri ne lor gl ucose and
ketones.
P28 Pediatrics
- `
F
Blood Glucose Targets by Age
Age Pe-eal blood HbA1C target
range glucose target
<6 5, 6- 1 0, 0 ( 1 00-l S01 7, 5-S, 5%
6-1 2 5, 0-1 0, 0 (90-1 S01 <S%
>1 2 5, 0-7, 2 (90-1 301 <7, 5%
Endocrinolog
Complications of Type 1 Diabetes
hypoglycemia
Toronto Notes 2010
cause.missed/delayedmeals,excessinsulin,increasedexercise,illness
complications.seizures,coma
musthaveglucagonkitlorquickinjections
inlantsmaynotshowclassic catecholaminergicsignswithhypoglycemia
hyperglycemia
cause.inlection,stress,diet-to-insulinmismatch,eatingdisorder
complications.riskolDKA,long-termend-organdamage
DKA
cause.new-onsetdiaLetes,missedinsulindoses,inlection
medicalemergency.mostcommoncauseoldeathinchildrenwithdiaLetes
attriLutedtocereLraledema)
long-termcomplicationsretinopathy, nephropathy, neuropathy)
usuallynotseeninchildhoodoltenLegins5yearsalterpresentationor3-5years
alterpuLerty)
metaLolicsyndrome
otherauto-immuneconditions e.g. celiacdisease,hypothyroidism)
TYPE 2 DIABE TE S
thesetendtoLeoLeseand present withglycosuriawithout ketonuria, aLsent ormild
polydipsiaandpolyuria,littleweightloss
especiallyprevalentamongMorthAmericanALoriginals,Alricans,Asians,Hispanics
onset >J0andinmiddletolatepuLerty
maypresentinDKAorHOMKstates
Treatment
illchildwith ketosis insulin
wellchildwithoutketosis diet,exercise,oralagents
metlorminisusedinchildrenLecauseit doesnotcausehypoglycemia
Diabetes Insipidus (DI)
DIistheinaLilityolthekidneystoconcentrateurine
central.
duetodecreasedADHproductionlromtheLraingenetic,duetotrauma,surgery,
radiation,neoplasm,meningitis)
presents withpolyuria, polydipsia, enuresis
nephrogenic.
renalunresponsivenesstoADHgenetic,drug-induced)
X-linkedrecessiveconditionthatallectsmalesinearlyinlancy
polyuria,FTT,hyperpyrexia,vomiting,hypernatremicdehydration
Diagnosis
symptoms.polyuria,enuresis, nocturia, polydipsia,dehydration
laLs.diluteurineSC<J. 0J0),hypernatremia,elevatedserumosmolality,lowurineosmolality
waterdeprivationtest,centralcauseil>50,changeinurineosmolalityalterADH
administration
Management
central.DDAVPintranasally SC,orPO
nephrogenic.low-solutediet,thiazidediuretics
Syndrome of Inappropri ate Antidiuretic
Hormone (SIADH)
etiology.intracranial,malignancy pulmonary disease,psychiatricdisease,drugs
commoninhospitalizedpatients associatedwithpost-oppainandnausea)
symptoms. asymptomatic,oliguria,volumeexpansion,orhyponatremicsymptoms
nausea,vomiting,H/A,seizure,coma)
laLs. hyponatremia,urineOsm>plasmaOsm,urineMa>20mmol / L
management.lluidrestriction,3,MaCllorsymptomatichyponatremia
Hypercalcemia/Hypocalcemia/Rickets
seeEndocrinolo_E38
Toronto Notes 2010 Endocrinolog
Hypothyroidism
seeEndocrinolo_
Congenital Hypothyroidism
incidence.J in4000 Lirths, F.M=2. J
usuallycausedLymallormationolthethyroidglandagenesisorectopic)alsomaternal
lactors iodinedeliciency,prenatalexposuretoantithyroidmedicationsorradioiodine
diagnosisthroughnewLornscreeningolTSHorT4
usuallyasymptomaticinneonatalperiodLecausematernal T4crossestheplacentaLutmay
havethelollowingsymptoms.
prolongedjaundice
constipation
sluggish,hoarsecry,letharg,poorleeding
macroglossia,coarselacialleatures,largelontanelles,umLilicalhernia
prognosis
excellentiltreatmentstartedwithinJ-2monthsolLirth
iltreatmentstartedalter3-6monthsolagemayresultinpermanentdevelopmental
delayand/ormentalretardationmildtoprolound)
management.thyroxinereplacement
Acquired Hypothyroidism
most commonlyHashimotosthyroiditis autoimmunedestructionolthethyroid)
signsandsymptomssimilartohypothyroidisminadults,Lutalso.
delayedLoneage,declineingrowthvelocity,shortstature,goiter
sexualpseudoprecocity.earlysexualdevelopmentwithshortstatureanddelayed
Loneage
doesnotcausepermanentdevelopmentaldelay
treatedwithL-thyroxine J0g/ kg/ day
Hyperthyroidism
seeEndocrinolo_
Congenital Hyperthyroidism
resultslromtransplacentalpassageolmaternalthyroid stimulatingantiLodiesmother
withCravesdisease)
clinicalmanilestationsintheneonatemayLemaskedLytransplacentalmaternal
antithyroidmedication
presentation.tachycardiawithcongestiveheartlailure,irritaLility, craniosynostosis,poor
leeding,lailuretothrive,heartmurmur,goitre
spontaneousresolutionLy2-3monthsollileasantiLodiescleared
management.propylthiouraciluntilantiLodiescleared,symptomatictreatment
Graves' Disease
peak incidence inadolescence,F. M* 5. J
mayexhiLitclassicsignsandsymptomsolhyperthyroidism,Lutalsopersonalitychanges,
schooldilliculty mood instaLility
managementsimilartoadults.anti-thyroiddrugspropylthiouracil,methimazole),
radioiodinereservedlorolderteens,surgicalthyroidectomy
childrenwithasolitarythyroidnodulerequirepromptevaluationas30-40,have
carcinoma,theremainderhaveanadenoma,aLscess,cyst,ormultinodulargoiter
J uvenile Graves' Disease
gradualonset over 6-J2months oldilluse goiter, ophthalmopathy, dermopathy
increasedappetite,weightlossormaintenance,latigue,myopathy,sweating,menstrual
irregularities
treatmentispropylthiouracilandpropranolol
thyroidectomyissometimesperlormed
Ambiguous Genitalia
E tiology
malepseudohermaphroditeXY)
inLornerroroltestosteroneLiosynthesisorLeydigcellhypoplasia
5-alpha-reductasedeliciency,androgenreceptordeliciencyorinsensitivity
luteinizinghormoneLH) / hCCunresponsiveness
smallphallus, hypospadias, undescendedtesticles
Pediatrics P29
Thyroi d neopl asms that devel op in

chi l dhood have a hi gher rate of
mal i gnancy; be suspi ci ous of rapi d and
pai nl ess enl argement of the thyroi d
gl and.
P30 Pediatrics
- `
P
The salt-wasting form of CAH i s a
medi cal emergency - babi es can di e of
vomi ti ng. dehydrati on and shock at 2-4
weeks of age; order gl ucose and
el ectrol ytes; repl ace fl ui ds and
el ectrol ytes. manage hypogl ycemi a.
and start the chi l d on l i fel ong
hydrocorti sone.
Endocrinolog Toronto Notes 2010
lemalepseudohermaphroditeXX)
virilizingcongenitaladrenalhyperplasiaCAH)mostcommon)
maternalsource.virilizingovarianoradrenaltumours,untreatedmaternalCAH,
placentalaromatasedeliciency
virilizationolexternalgenitalia clitoralhypertrophy, laLioscrotallusion
mixedpattern
truehermaphrodite
LothovarianlolliclesandseminileroustuLulesinthesamepatientwitha46XX
karyotype
increasedriskolmalignanttranslormationolgonadtissue
mixedgonadaldysgenesis
Investigations
history.pregnancyhormonesandmedications),lamilyhistory
physicalexam.palpationolgonads,rectalexamexceptinnewLorns)
investigations
karyotype
electrolytesandreninevidenceolsalt-wastinginCAH)
measurementolJ/-OH-progesteronemustwaituntilday3ollile),androgens,
lolliclestimulatinghormone FSH)andluteinizinghormone LH)
aLdominalU/Stolookloruterus,testicles,ovaries
depends onthe degree andthespecilicdeliciency
inlantsmaypresentwithFTT,shock, salt-wastingadrenalcrisisduetolackol
aldosterone),hyperpigmentationgenital, areola), clitoralhypertrophy, lusedlaLia
hypertensionisrareusuallyseenintheJJ-hydroxylasevariant)
adultonsetJJ-hydroxylasevariant)moreinsidious,maypresentashirsutism
lemale.amLiguousgenitaliatocompletevirilization,amenorrhea
male.precociouspuLerty, withearlyadrenarche,dehydration
acceleratedlinearLonegrowthinearlyyears,Lutprematureepiphysealclosuredueto
hightestosterone,resultingindecreasedadultheight
possiLleAddisonianpictureadrenalinsulliciency)iladrenaloutputolcortisolseverely
compromised
Congenital Adrenal Hyperplasia (CAH)
occursinJ/J5000liveLirthsandi sthemostcommoncauseolamLiguousgenitalia
autosomal recessive conditioncausingpartialortotalenzymedelect
2J-hydroxylasedeliciencycauses95,olCAHcases,thiscausesdecreasedcortisoland
aldosteronewithshuntingtowardoverproductionolandrogens
cortisoldeliciencyleadsto elevatedACTH,whichcausesadrenalhyperplasia
clinicalpresentationdependsonthespecilicdeliciencyandthecause maypresentwith
shock,hyperkalemiailnotsuspected
highACTH,increasedJ/-OHprogesterone,increasedtestosterone,DHEAS,urinary
J/-ketosteroids,advancedLoneage
Late-Onset 21-Hydroxylase Deficiency
allelicvariantolclassic 2J-hydroxylasedeliciency mildenzymaticdelect
girlspresentwithamenorrhea
LoyspresentwithprecociouspuLertywithearlyadrenarche,dehydration
acceleratedlineargrowthinearlypuLertyLutearlylusionolepiphysesleadingto
decreasedadultheight
diagnosis
increasedplasma J/-OH-progesteronealterACTHstimulationtest
treatment
dexamethasone, spironolactone anti-androgen)
mineralocorticoidreplacementisnotneeded
Salt-Wasting 21- Hydroxylase Deficiency CAH(2/ 3 of cases)
inlants presentwithshock,FTT, low Ma,high K low Cl, low glucose,highACTH
adrenalinsulliciency
hyperpigmentationolgenitalsandareola
Simple Viriliz ing 21-Hydroxylase Deficiency
virilizationingirls
11- Hydroxylase Deficiency
sexualamLiguityinlemales
mayhaveinsidiousonset,maypresentwithhirsuitism,occasionallyhypertension
17 -Hydroxylase Deficiency
sexualamLiguityinmales
Investigations
lowMa,highK, lowcortisol,highACTHilLothglucocorticoidandmineralocorticoid
deliciency
increasedserumJ/-OH-progesterone suLstrate lor2J-hydroxylase)
increasedtestosterone,DHEAS,urinaryJ/-ketosteroids
advancedLoneage
Treatment
glucocorticoidreplacementtolowerACTH
insalt-wastingtypemineralcorticoidsgivenaswell
spironolactoneisusedinlate-onset2J-hydroxylasedeliciencyasanti-androgen
surgerytocorrect amLiguousgenitalia
Tabl e 1 7. Cl i ni cal Features of CAH Based on Enzyme Defect
Enzyme Defect Sexual Ambiguit Postnatal Virlization Sal Wasing Hyerension
Female Male
2 1 hydroxylase
salt-wasting + +
si mpl e vi ri l i zi ng + +
late onset +
1 1 -hydroxylase + + +
1 7 -hydroxylase + +
Precocious Puberty
secondarysexualdevelopment<8yearsingirls,<9yearsinLoys
incidence.JinJ0,000
morecommoninlemales,moreworrisomeinmaleshigherincidenceolpathology)
indicationslormedicalinterventiontodelayprogressionolpuLertyaremalesex,age<6,
Loneageadvancingmorequicklythanheightage,andpsychologicalissues
CnRHagonistssuchasleuprolidearemostellectiveatdelayingcentralprecociouspuLerty
medicationsusedinperipheralprecociouspuLertyincludeketoconazoletoLlocksteroid
roduction),5-oreductaseLlockers linasteride),steroidreceptorLlockers
spironolactone),aromataseLlockerstestolactone,anastrozole)
True (Central) Precocious Pubery
hypergonadotropichypergonadism, hormonelevelsasinnormalpuLerty
prematureactivationolthehypothalamic-pituitary-gonadalaxis
muchmorecommoninlemalesthanmales 9. J
dillerentialdiagnosis
idiopathicorconstitutionalmostcommon, especiallylemales)
CMSdisturLances.tumours,hamartomas,post-meningitis,increasedICPradiotherapy
neuroliLromatosisMF),primaryseverehypothyroidism
Pseudo (Peripheral) Precocious Puberty
hypogonadotropichypergonadism
adrenaldisorders.CAH,adrenalneoplasm
testicular/ovariantumour
gonadotropin secretingtumour.hepatoLlastoma,intracranialteratoma, germinoma
exogenoussteroid administration
McCune-AlLrightsyndrome.endocrinedyslunctionresultinginprecociouspuLerty,
cal-au-laitspots, and liLrousdysplasiaol skeletalsystem
Investigations
history.symptomsolpuLerty, lamilyhistoryolpuLerty onset,medicalillness
physicalexam.growthvelocity, Tannerstaging,neurologicalexam
estradiol,testosterone,LH,FSH,TSH,CnRHtest
Loneageoltenadvanced)
considerCTorMRIolhead,U/Soladrenals,pelvis
Treatment
CnRHanalogs,CnRHagonistLupron`) negativeleedLacktodownregulate CnRH
receptors
medroxyprogesterone slowsLreastandgenitaldevelopment
treatunderlyingcause
Heterosexual Precocious Pubery
development olsecondarysexualcharacteristicsoppositetogenotypicsex
e.g.virilizingtumourovarian,adrenal),CAH,exogenousandrogenexposure
Pediatrics P31
- '
A chi l d with proven central precoci ous

puberty shoul d recei ve an MRI of the
brai n.
P32 Pediatrics
'
'
Short Stature DDx
ABCDEFG
Al one (negl ected i nfant)
Bone dyspl asi as ( ri ckets. scol i osi s.
mucopolysacchari doses)
Chromosomal (Turner. Down)
Delayed growth
Endocri ne (low growth hormone.
Cushi ng. hypothyroi d)
Fami l i al
GI mal absorpti on (cel i ac. Crohn)
P
4 Duestions to Ask when Evafuating
Short Stature
1 . Was there I UGR?
2. I s the growth proporti onate?
3. I s the growth vel ocity normal ?
4. I s bone age del ayed?
` *
Cal culate mi d-parental hei ght (predi cted

adult hei ght) 8 cm for 2 SD ranges
Check the mi d-parental hei ght for
percenti le of adults
Boy * [father hei ght (cm) + mother
hei ght (cm) + 13 cmJ/2
Gi rl * [father hei ght (cm) + mother
hei ght (cm) 13 cmJ!2
Endocrinolog Toronto Notes 2010
Delayed Puberty
seeC_ecolo_
aLsenceolpuLertaldevelopmentLyageJ3ingirlsandageJ4inLoys
morecommoninmales,moresuggestiveolpathologyinlemales
Central Causes
delay inactivationolhypothalamic-pituitary-gonadalaxis
hypogonadotropichypogonadism
constitutionalLoneagedelayed) mostcommon>90,)
Peripheral Causes
hypergonadotropichypogonadisme.g.primarygonadallailure)
genetic e.g. Turnersyndrome,Kleineleltersyndrome)
gonadaldamage inlection,radiation,trauma
gonadaldysgenesis
hormonaldelect androgeninsensitivity, 5-alpha-reductasedeliciency
Investigations
history.weight loss, short stature, lamilyhistory olpuLerty onset,medicalillness
physicalexam.growthvelocityminimum4cm/ year),Tannerstaging,neurologicalexam,
completephysicalexam
hormonelevels.estradiol,testosterone,LH,FSH,TSH,CnRH,testLoneage
considerCTorMRIolhead,ultrasoundoladrenals,pelvis
karyotypeingirls<3'percentileinheightruleoutTurnersyndrome)
Management
identilyandtreatunderlyingcause
hormonalreplacement. cyclicestradiolandprogesteronelorlemales,testosteronelormales
Short Stature
Tabl e 1 8. Short Stature
NORMAL GROWH VELOCIT
(non-pathological shor stature)
Constiutional Growth Delay
Delayed puberty
May have fami l y hi stor of delayed puberty
May require short-term therapy wi th androgensestrogens
Delayed bone age
Often mi d-parental hei ght i s normal
Familial
Normal bone age
Treatment not i ndi cated
Fami l y Hx of shor stature
DECREASED GROWH VELOCIT
(pathological shor stature)
Prmordial (hei ght, wei ght, and HC are affected)
Chromosomal (e. g. Turner, Down syndrome, dysmorphi c features)
Skeletal dyspl asi as
Intrauterine growth restriction ( I UGR) (teratogen, pl acental
i nsufficiency, i nfecti on)
Endocrine (hei ght affected more than wei ght) - "short and fat"
GH deficiency (slow growth velocity, decreased bone age, delayed
pubery)
Hypothyroi di sm
Hypercorisol i sm ( Cushi ng syndrome) (exogenous and endogenous)
Hypopi tuitarism
Chronic disease (wei ght affected more than hei ght) - "short and ski nny"
Cyanotic congeni tal heart di sease
Cel i ac di sease, i nfl ammatory bowel di sease, cystic fibrosis
Chroni c infections
Chroni c renal fai l ure (often hei ght more affected)
Psychosocial neglect (psychosocial dwarfism)
Usual ly decreased hei ght and wei ght (decreased head circumference
i f severe)
specialgrowthchartsavailaLlelorTurnersyndrome,achondroplasia,DownsyndromeDS),
thesechildrengrowalongpercentilesspecilictotheirconditionandgrowthvelocityis
oltennormal
childrenareusuallyinapercentileLetweentheirparentsheight
decreasedgrowthvelocitymayLemoreworrisomethanactualheight
Assessment of Shor Stature
height<3''percentile,heightcrosses2majorpercentilelines,lowgrowthvelocity
25''percentile)
history.perinatalhistorygrowthpattern,medicalhistory, parentalheightandageol
puLertalgrowthspurt
physicalexam.growthvelocityover6monthperiod),sexualdevelopment
growthhormoneCH)deliciencyaccountslorasmallminorityolchildrenwithshortstature
Investigations
Loneageanteroposteriorx-rayollelthandandwrist)
karyotypeingirlstoruleoutTurnersyndromeorildysmorphicleaturespresent
othertestsasindicatedLyhistoryandphysicalexam
Management
depends onseverityolproLlemasperceivedLyparents/child
no treatmentlornon-pathologicalshortstature
CHtherapyilrequirementsmetseeGrowth Hormone Defciency)
Growth Hormone (GH) Deficiency
CHimportantlorchondrocyte prolilerationandICF-Jrelease
CHhaslittleellectonletalgrowthmaternalICF-J,uterinelactorsmoreimportant)
ICF-JactsatlongLones,liver,negativeleedLack
E tiology
congenitalCHdeliciency
idiopathic
emLryologicCMSmallormation.associatedmidlaceanomalies,neurologicdelects,
micropenisinmalesandhypoglycemia
perinatalasphyxia
raremutations
acquiredCHdeliciency
tumours e.g. craniopharyngioma),trauma,cranialinlection,irradiation,post-surgical
inlantile leatures andlatdistriLutionshort andchuLLy),delayedpuLerty,hypoglycemia,
high-pitchedvoice
Investigations
TestinglorCHDeliciency StimulationTesting)onlyperlormedwhen.
height<3''percentile
decreasedgrowthvelocity
midlinecraniolacialanomalies
episodes olhypoglycemia
delayedLoneage,puLerty
physiologicincreaseinCHwith.arginine,clonidine,insulin,dopamine,orpropranolol
positivetestillailuretoraiseCH>8-J0ng/mLpost-stimulation
Treatment
CHTherapyilthelollowingcriteriaaremet.
CHshowntoLedelicientLy2dillerentstimulationtests
patientisshort,insullicentgrowthvelocity, <3rdpercentile
Loneagex-raysshowunlusedepiphyses
Turnersyndrome,Moonansyndrome,chronicrenallailure
Tall Stature
constitutionaltallstatureisadvancedheightandLoneageduringchildhoodLutnot
necessarily associatedwithtalladultheight oLesitycancontriLutetothisLycausingLone
agetoadvancemorerapidly)
E tiology
constitutional /lamilial. mostcommon, advancedLoneage /physicaldevelopmentin
childhoodLutnormalonceadulthoodreached
endocrine.hypophysealpituitary)gigantism,precociouspuLerty, thyrotoxicosis,
5eckwith-Wiedemannsyndrome
genetic.Marlan,Kleineleltersyndromes,Sotossyndrome,homocystinuria
Investigations
history andphysicalexamination. dillerentiate lamiliallromothercauses
calculatemid-parentalheigtpredictedadultheight)
looklorassociatedaLnormalitiese.g.hyperextensiLlejoints,longlingersinMarlansyndrome)
Treatment
dependsonetiology
treatmentonlyrequiredinpituitarygigantism
estrogenusedinlemalestocauseepiphyseallusionrarelyindicated)
Pediatrics P33
*P
Upper to Lower (U/L) Segment Ratio
is . . .
I ncreased i n achondropl asi a. shor l i mb
syndromes. hypothyroi d. storage
di seases.
Decreased in Marfan. Kl i nefelter.
Kal l man. testosterone defi ci ency.
P34 Pediatrics
- `
*
Vomiting: forcef ul expul si on of
stomach contents through the mouth
Regurgitation: the return of parl y
di gested food from the stomach to the
mouth

Pl ori c stenosis 3 P's
Pal pabl e mass
Peri stal si s vi si bl e
Projecti l e vomi ti ng (2-4 weeks after
bi rh)
Gastroenterolog Toronto Notes 2010
Gastroenterology
Vomiting
investigationsLasedonhistoryandphysicalexam)
Lloodyemesis.investigatelorcausesoluppergastrointestinalCI)Lleed
Liliousemesis.ruleoutoLstructionupperCIseries,U/S)
evaluatelorgastroesophagealrellux24-houresophagealpHproLe)
C5C,electrolytes,5UM,creatinine,ESRvenousLloodgases,amylase,lipase
urine,Llood,stoolC&S
aLdominalx-ray,U/S,contrastradiology, endoscopy
considerheadimaging
management
rehydrationseePediatric Nephrology section)
Vomiting in the Newborn Period
Tracheoesophageal Fistula (TEFl

incidence. J. 3,000-J.4,500
clinicalpresentationvarywithtypeollistula)
mayhavehistoryolmaternalpolyhydramnios
maypresentalterseveralmonths,ilno associated esophagealatresia,withvomiting,
coughing,andgagging
cyanosis withleeds, respiratorydistress, recurrent pneumonia
lrothyLuLLles olmucusinmouthandnosethatreturnaltersuctioning
associatedanomaliesin50,.VACTERLassociationseePediatric Genetics,
Dysmorphisms, and Metabolism, P44)
x-ray.anatomicaLnormalities,MCtuLecurledinpouch
management
investigatelorothercongenitalanomalies
earlyrepairLysurgicalligationtopreventlungdamageandmaintainnutritionand
growth
complications
pneumonia,sepsis,reactiveairwaysdisease
lollowingrepair.esophagealstenosisandstricturesatrepairsite,gastroesophageal
relluxandpoorswallowingie.dysphagia,regurgitation)
Duodenal Atresia
incidence.J. J0 000, 50,areLornprematurely
clinicalleatures
Lile-stainedvomitingilatresiadistaltoLileduct
noaLdominaldistention
dehydration
associatedwithDownsyndrome,prematurity
oltenhavehistoryolmaternalpolyhydramnios
aLdominalx-ray.air-lluidlevelsonuprightlilm,douLleLuLLlesigndilatedstomach
andduodenum)
dillerentialdiagnosis. annularpancreas,aLerrantmesentericvessels,pyloricstenosis
treatment
decompressionwithMCtuLe
correctionolmetaLolicaLnormalities
surgicalcorrection
Pyloric Stenosis
incidence. J. 500, M.F * 4. J,onsetcommoninlirst-Lornmales, positiveFHx
clinicalleatures
non-Liliousprojectilevomitingthatoccursalterleeding
usuallystartsat2-4weeksolage
inlanthungryandalert,willre-leed
constipation,FTT,wasting
dehydration,mayleadtoprolongedjaundice
gastricperistalsis goeslromleltupperquadrant LUQ)to epigastrium
olivesign. olive-shapedmassatmarginolrightrectusaLdominusmuscle
hypochloremicmetaLolicalkalosis
diagnosis.clinical,aLdominalU/Sandx-ray
treatment.surgicalpyloromyotomy)
Toronto Notes 2010 Gastroenterolog
Malrotation of the Intestine
incidence. J. 500
80,experiencesymptomsinlirsttwomonthsollile
3presentations
recurrentvomitingLiliousintermittently)
FTTwithvomiting
suddenonsetaLdominalpainandthenshockilvomitingwithLiliousmaterial,
malrotationwithvolvulusuntilprovenotherwise)
clinicalleatures
distendedaLdomen
vomitingduetovolvulusandLandsacrossduodenum
diagnosis.aLdominalU/SandupperCIseries
treatment.MCtuLedecompressionandsurgery
complications.volvulusisasurgicalemergencyasitcanresultinLowelperloration,
peritonitis,andbowelnecrosis
Vomiting After the Newborn Period
Infectious and Inflammatory

CIcauses.gastroenteritis,peritonitis,appendicitis,hepatitis,ulcers,pancreatitis,
cholecystitis
non-CIcauses.urinarytractinlectionUTI),pyelonephritis,nephrolithiasis,otitismedia,
laLyrinthitis,meningitis,pneumonia
Anatomic
CItractoLstruction
intussusception,volvulus
loreignLodye. g.Lezoar)
Gastroesophageal Reflux
extremely commonininlancyupto50,). thrivingLaLyrequiresnoinvestigation
vomitingtypicallysoonalterleeding,non-Lilious,rarelycontainsLlood,smallvolume J oz)
investigationsrequiredil.FTT,recurrentcough,pneumoniaorLronchospasm, CILlood
loss,symptomspersistalterJ8months
24-hourpHproLe, UCIseries toruleoutanatomicalcause,upperendoscopyand
esophagealLiopsylorsuspectedesophagitis
management
conservative.thickenedleeds,lrequentandsmallerleeds,elevateLedto45
medical.
short-termparenteralleedingtoenhanceweightgain
ranitidine,omeprazole.todecreasegastricacidity,decreaseesophagealirritation
domperidone.toimprovegastricemptyingandCImotility
surgical.indicatedlorlailureolmedicaltherapyMissenlundoplication)
complications.esophagitis,strictures,5arrett sesophagus,FTT,aspiration
Central Nervous System
increased intracranialpressureICP)e.g.hydrocephalus,neoplasm)
drugs/toxins
migraine,cyclicvomiting
Other
metaLolic /endocrine.DKA, inLornerrorsolmetaLolism, liverlailure
poisons /drugs.lead,digoxin,erythromycin,theophylline
psychogenic. ruminationsyndrome,anorexia/ Lulimia
loodallergy
overleeding
pregnancy
Acute Diarrhea
Tabl e 1 9. Causes of Acute Diarrhea
Infectious
Viral
Rotavirus
Noralk
Enteric Adenovirus
Bacterial
Salmonela
Campy/obacter
Shigela
Pathogeni c |coli
Yerinia
C. dificile
Parasitic
Giaria lamblia
Entmoeba histo/ytica
Non-Infectous
Anti bi oti ci nduced
Nonspeci fi c: associ ated wi th systemi c infection
Hi rschsprung' s disease
Toxi n i ngestion
Primary di sacchari de deficiency
Pediatrics P35
`
O
Di arrhea i s defi ned as an i ncrease in
frequency and/or decreased
consi stency of stool s compared to
normal .
Normal stool vol ume:
Infants: 5 - 1 0 g/kg
Chi l dren: 200 g/day
P36 Pediatrics
I ndications for Medical Evaluation of
Acute Diarrhea
Age < 6 months
Fever
Vi si ble blood in stool
Frequent, substanti al vol ume of
di arrhea
Si gns of dehydrati on
Change i n mental status
NNWhhc0mmh Z00J,bZ| 11 I | I I
P
Chroni c di arrhea: three or more l oose,
watery stool s per day, l asti ng > 14 days.
4F's Diet for Chronic Diarrhea

Adequate Fi bre
Normal Fl ui d i ntake
35-40% Fat
Di scourage excess Fruit j ui ce
VIRAL INFECTION
mostcommoncauseolgastroenteritisinCanadaandworldwide
rotavirusesarethemostcommonagent,oltenseeninwintermonthsintemperate
climates
astrovirusesarethesecondmostimportantetiologicalagent,commoninLothdeveloping
anddevelopednations
Morwalkvirusisthethirdmostcommonagent,ittypicallyallectsolderchildrenandadults
clinicalleatures
associatedwithURTIs
resolvesin3-/days
slightlever, malaise, vomiting,vagueaLdominal pain
BACTE RIAL INFECTION
clinicalleatures.severeabdominalpain,highlever,bloodydiarrhea
risklactors.travel,poorlycookedmeat,poorlyrelrigeratedloods
Investigations
history andphysicalexaminationcriticaltodeterminedegree oldehydrationseePediatric
Nephrology, TaLle4J)
rectalexamlorlecalconsistencyandlormicroscopyleukocytessuggestiveolinvading
pathogen)
stoollorcultureandsensitivityC&S),ovaandparasites O&P),electronmicroscopylor
viruses
ilsevere. routineLloodwork,Lloodandurinecultures
Treatment
preventionandtreatmentoldehydrationismostimportantseeDehydration, P/3)
oralrehydrationtherapywithlrequentsmallvolumesolpediatricrehydrationsolutions,
IVmayLerequired,seeFluid c Electrolyte Therapy section,P/3
earlyreleedingadvisaLle,startwithsmallamountsoleasilydigestedcarLohydrates,
postponedairyandliLrousvegetaLles
antiLiotictherapywhenindicated,antidiarrhealmedicationsnotindicated
notilyPuLlicHealthauthoritiesilappropriate
Chronic Diarrhea
Investigations
perlormserialheights,weights,growthpercentiles
ilchildisgrowingwellandthriving,minimalworkupisrequired
investigationsdependingonsuspecteddiagnosis
stool.consistency,pH,reducingsuLstances,microscopy,occultLlood, O&PC&S,
C. diffcile toxin,3-daylecallat
urinalysis
C5C,dillerential,ESR,smear, electrolytes, totalprotein,alLumin
aLsorptiveandnutritional status. alLumin, carotene, Ca,POMg,Fe,lerritin,lolate,
lat-soluLlevitamins,PTT,IMR
sweatchloride,thyroidlunctiontests,urinevanillylmandellicacidVMA)and
homovanillic acid HVA), HIVtest, lead levels
CXR,upperCIseriesandlollow-through
specializedtests.endoscopy, smallLowelLiopsy
Tabl e 20, Causes of Chroni c Di arrhea
03 months
GI infection
Disaccharidase deficiency
Cow's mi l k intolerance
Cystic fibrosis
3 months-3 years
Toddl er's di arrhea
GI i nfecti on
Cel i ac disease
3 years-1 8 years
GI infection
Cel i ac disease
I BD
Uncommon causes
Consti pation wi th overflow di arrhea
Drug i nduced
UTI
Short bowel syndrome
Chronic Diarrhea Without Failure to Thrive
Infectious
Lacterial.Campylobaeter, Salmonella
antiLiotic-induced.C. diffcile colitis
parasitic. Giardia lamblia
post-inlectious.secondarylactasedeliciency
Toronto Notes 2010
Toddler' s Diarrhea
epidemiology
Gastroenterolog
most commoncauseolchronicdiarrhea duringinlancy
onsetLetween6-36 monthsolage,ceasesspontaneouslyLetween2-4years
clinicalpresentation
diagnosisolexclusioninthrivingchildnoweightloss / FTT,nolluidorelectrolyte
aLnormalities)
diethistory.toomuchjuiceoverwhelmssmallLowelresultingindisaccharide
malaLsorption
stoolmaycontainundigestedloodparticles,4-6Lowelmovements5Ms)perday
excoriateddiaperrash
management
reassurance, sell-limiting
lourFsadequateIiLre,normalIluidintake,35-40,Iat,discourageexcessIruitjuice)
Lactase Deficiency (Lactose Intolerance)
clinicalleatures
chronic,waterydiarrhea
aLdominalpain,Lloatingassociatedwithdairyintake
primarylactoseintolerance. crampy aLdominalpainwithloosestoololder children,
usuallyolEastAsianandAlricandescent)
secondarylactoseintolerance.olderinlant,persistentdiarrheapostviral / Lacterial
inlection,celiacdisease,orI5D)
diagnosis
trialollactose-lree diet
waterystool,acidpH,positivereducingsugars
positiveLreathhydrogentestil>6years
management
lactose-lreediet,soylormula
lactase-containingtaLlets /capsules /drops e.g. Lacteeze, Lactaid)
Irritable Bowel Syndrome
diagnosisolexclusioninolderchild/adolescent,mayLesimilartorecurrentaLdominalpain
management
encouragehighliLrediet
reassurance
medicationscAMPinhiLitors)lorrelractorycases
Chronic Diarrhea With Failure to Thrive
1. INTE STINAL CAUSE S
Celiac Disease
J. 250- J. J00incidence
alsoknownasgluten-sensitiveenteropathy,causedLyareactiontogliadenagluten
protein)
T-cell-mediatedinllammation damagetoenterocytes
delectinmucosa.immune-mediatedinllammationanddestructionolaLsorptivevilli
clinicalleatures
presentsatanyage,usually6-24monthswiththeintroductionolgluteninthediet
FTTwithpoorappetite,irritaLility, apathy
anorexia,nausea,vomiting,edema,anemia,aLdominalpain
wastedmuscles,distendedaLdomen, llatLuttocks,cluLLingollingers
rickets
non-CImanilestations.dermatitisherpetilormis,dentalenamelhypoplasia,
osteopenia/osteoporosis,shortstature,delayedpuLerty
associatedwithotherauto-immunedisorders
diagnosis
anti-transglutaminase tTC),antigliadin,antiendomysialantiLodiesanti-EMA),low
D-xyloseaLsorption
latmalaLsorptionstudies
smallLowelLiopsygoldstandard).totalvillousatrophywithresolutionaltertrialol
gluten-lreediet
treatment
gluten-lreedietlorlile
complications iluntreated
smallLowellymphoma
malnutrition,FTT
Pediatrics P37
Cel i ac di sease di et must avoi d gl uten

present i n "BROW foods
Barl ey
Rye
Oats
Wheat
- `
Cel i ac di sease i s associ ated with an

i ncreased preval ence of IgA defi ci ency,
Si nce tG i s an IgAdetecti ng test, you
must order an accompanyi ng I gA level !
P38 Pediatrics
- `
Constipation
Decreased stool frequency
3 stools/week) and/or stool fl ui dity.
- `
As many as 20% of chi l dren <5 years

of age experi ence consti pati on.
Milk Protein Allergy
immune-mediatedmucosalinjury
upto50, olchildrenintoleranttocowsmilkmayLeintoleranttosoyproteinaswell
oltenhistoryolatopy
2scenarios
enterocolitis.vomiting,diarrhea,anemia,hematochezia
enteropathy.chronicdiarrhea,hypoalLuminemia
treatment.caseinhydrosylatelormuladairy-lreee.g.Mutramigen,Pregestimil)
Inflammatory Bowel Disease (IBD) seeCastroenterolo_)
incidence.J5-30. J00 000,increasinginMorthAmerica,mostlyolderchildrenandteenagers
Other
specilicenzyme deliciencies
liverdisease,Liliaryatresia
alpha-Leta-lipoproteinemia
shortguttoxicorimmunologicreaction
Llindloopsyndrome
giardiasis
2. PANCREATIC INSUFFICIENCY
Cystic Fibrosis (CF) seePediatric Respirolog section,P9J)
Schwachman-Diamond Syndrome
incidence. J. 20,000,autosomalrecessive
pancreaticinsulliciency, cyclicneutropenia,andanemia
skeletal aLnormalities metaphysealdysostosisleadingtoshortstature)
recurrentpyogenicinlections
distinguishedlromCFLynormalsweat chloridetest,characteristicmetaphyseallesions,
lattypancreasonCT
3. OTHE R
dietsrichinsorLitol,lructosepoorlyaLsorLedcarLohydrates)
metaLolic/endocrine
thyrotoxicosis,Addisonsdisease, galactosemia
immunedelects
IgAdeliciency,hypogammagloLulinemia,severecomLined
immunodeliciencySCID),AIDS
neoplastic
pheochromocytoma,lymphomaolsmallLowel,carcinoidtumours,secretorytumours
loodallergy
laxativeaLuse
Constipation
Functional Constipation
99, olcases olconstipation
lackolliLreindietorchangeindiet,poorlluidintake
inlants.oltenwhenintroducingcowsmilkalterLreastmilkLecauseincreasedlatand
soluteamounts,lowerwatercontent
toddlers/olderchildren.canoccurduringtoilettraining,orduetopainondelecation,stool
withholding
complications
painretentioncycle.anallissuresandpain withholdpassingstool chronic
dilatationand overllowincontinence
treatment
adequatelluidintakeil<6months, J50ml / kg/day)
adequatedietaryliLre,mineraloil,gentlelaxatives occasionally chronicusenot
recommended)
appropriatetoilettrainingtechnique
Hirschsprung' s Disease (congenital aganglionic megacolon)
lailureolnormalinnervationolthedistalcolonLytheganglioncellsolthemyentericplexus
colonremainscontractedandimpairslecalmovement
incidence. M.F- 3. J,J in5,000liveLirths
clinicalleatures
typicallyonlyrectosigmoidinvolvementLutmayextendtoentirecolon
nomeconiumwithinlirst24hours
palpaLlestoolonaLdominalexamwithemptyrectumondigitalrectalexam
intermittentdiarrhea,5Monlywithrectalstimulation
constipation,aLdominaldistention,vomiting, lailuretothrive
Toronto Notes 2010 Gastroenterolog
complications
enterocolitis.mayLelatal,peakincidence2-3monthsolage
toxicmegacolonandperloration
diagnosis
aLdominalx-ray cannotseegasinrectum
Lariumenema.proximaldilatationduetolunctionaloLstruction,emptyrectum
manometricstudies.showslailureolanalsphincterrelaxation,mayhavelalse
positives
rectalLiopsy.delinitivediagnosisaLsentganglioncells)
treatment
nonsurgicalilshortsegment.increaseliLreandlluidintake,mineraloil
surgical.colostomyandre-anastomosis
Other Organic Disorders Causing Constipation
endocrine. hypothyroidism,diaLetesmellitus DM),hypercalcemia
neurologic.spinaLilida
anatomic.LoweloLstruction,anusimperlorate,atresia,stenosis)
drugs.lead,chemotherapy,opioids
Acute Abdominal Pain
Tabl e 21 . Differential Di agnosis for Acute Abdomi nal Pain
Gastrointestnal
Gastroenteritis
I ncarcerated herni a, volvul us, i ntussuscepti on
Appendi ci ti s
Malrotation
Mesenteric adeni ti s
Cholecystitis
Meckel's diverti cul i ti s
Assessment
Other Systems
UTI
HenochSchonl ei n Purpura ( HSP)
Si ckle cel l cri si s
Pneumoni a
DK
Nephrol i thi asi s
Gynecol ogi cal (ectopic pregnancy, PI D, endometriosis, menstruati on)
descriptionolpainlocation,radiation,duration,constantvs.colicky, relativitytomeals)
associatedsymptoms.nausea,vomiting, diarrhea, lever
physicalexamination.peritonealsigns,Lowelsounds,rectalexam
laLs. C5C,dillerential,urinalysistoruleouturinarytractinlectionUTI)
Appendicitis
seeCeneralSurger_
mostcommoncauseolacuteaLdomenalter5yearsolage
clinicalleatures
lowgradelever,anorexia
nausea,vomitingalteronsetolpain)
aLdominalpain, peritonealsigns
generalizedperitonitisisacommonpresentationininlants /youngchildren
treatment. surgical
complications.perloration,aLscess
Intussusception
90,idiopathic, childrenwithCForC]tuLeatsignilicantlyincreasedrisk
50,Letween3-J2months,/5,Lelore2yearsolage
telescopingolsegmentolLowelintodistalsegment ischemiaandnecrosis
usualsite. ileocecaljunction,jejunuminchildrenwithC]tuLes
leadpointoltelescopingsegmentmayLeswollenPeyer spatches, Meckels diverticulum,
polyp, malignancy,Henoch-SchnleinPurpura
clinicalleatures
classictriad
aLdominalpain
palpaLlesausage-shapedmass. uppertomid-aLdomen
redcurrantj ellystoolsonlyinJ0-J5,olpatients)
suddenonsetolrecurrent,paroxysmal,severeperiumLilicalpainwithpain-lree
intervals
latervomitingandrectalLleeding
shockanddehydration
diagnosisandtreatment
U/S
airenemadiagnostic,canLetherapeuticreduceintussusceptionin/5,olcases)
reductionunderhydrostaticpressure
surgeryrarelyneeded
Pediatrics P39
Curent Recommended Treatent dChildhood
Constipation are Not Evidenc Based: A
stematc Literature Review on te Efect d
Laxative Treatment and Dietar Measures
Arch Dis Child 2009; 94:117131
Study: Systematic review of studies assessing the
effect of medical and dietary treatment of functional
constipation.
Patients: 37 included publications. a total of 1912
children with constipation.
Interenton: PEG vs. placebo. vs. lactulose. and vs.
other laxatives; Lactose vs. other laxatives; Senna;
Mineral oil; Fibre; etc.
Outome: Defecation frequency.
Results: No clirically sigrilcant difference beteen
laxatives, and beteen fibre vs. placebo; although
most studies failed to compare laxatives to placebo.
There was greater treatment success with PEG as
compared to all other laxatives.
-
Intussuscepton - Classic Triad

Abdomi nal pai n
Pal pabl e mass
Red current jel l y stool s
P40 Pediatrics
F
Rule of 3's
3 epi sodes ofsevere pai n
Chi l d >3 years ol d
Over 3 month peri od
Red Flags for Organic Etiology of
Age < 5 years ol d
Fever
Local izes pai n away lrom mi dl i ne
Anemi a
Rectal bleedi ng
Rash
Pai n awakens chi l d at ni ght
Travel hi story
Promi nent vomi ti ng, di arrhea
Wei ght l oss or lai l ure to gai n wei ght
Joi nt pai n
J0-J5,olchildren
delinitionruleol3s=3episodesolpainsevereenoughtoallectactivities,occurringina
child>3years olageoveraperiodol3months
distinguishorganiclromnonorganic
Organic 10%)
gastrointestinal
constipationcausevs.ellect),inlectious
I5D,esophagitis, pepticulcerdisease, lactoseintolerance
anatomicanomalies,masses
pancreatic,hepatoLiliary
genitourinarycauses
recurrenturinarytractinlections,nephrolithiasis,chronicPID,mittelschmerz
neoplastic
Functional/ Recurrent Abdominal Pain (RAP) (90%)
schoolage, peak8-J0years
prevalence.J0,olschoolchildren,F>M
characteristics
vague,crampyperiumLilicalorepigastricpain,vividimagerytodescriLepain,
clusteringolepisodes
seldomawakenschildlromsleep
aggravatedLyexercise,alleviatedLyrest
psychologicallactorsrelatedtoonsetand/ormaintenanceolpain,schoolavoidance
psychiatriccomorLidity.anxiety, somatolorm,mood,learningdisorders,sexualaLuse,
eatingdisorders,eliminationdisorders
diagnosisLyexclusionolorganicdisorders
investigationsasindicated
C5C,ESR,urinalysis, stoolslorO&PC&S, occultLlood
treatment
continuetoattendschool
manageanyemotionalorlamilyproLlems, counselling
trialolhigliLrediet,trialollactose-lreediet
reassurance
prognosis
painresolvesin30-50, olkidswithin2-6weeksoldiagnosis
30-50, olkidswithRAPhavelunctionalpainasadultse.g.irritaLleLowelsyndrome)
Abdominal Mass
Tabl e 22, Differential Di agnosi s for Abdomi nal Mass
Benign Malignant
Renal Hydronephrosis Nephroblastoma (Wi l ms)
Polycystic ki dney di sease ( PCKD) Renal cel l carci noma ( RCC)
Hamartoma
Adrenal Neuroblastoma
Ovaran Ovarian cysts Ovarian tumours
Other Splenomegal y Lymphoma
Pyloric stenosi s Rhabdomyosarcoma
Abdomi nal herni a Tetroperitoneal
Teratoma
Fecal i mpaction
50, olaLdominalmassesinthenewLornarerenalinorigin
Upper Gastrointestinal Bleeding
seeCastroenterolo_
Toronto Notes 2010 Gastroenterolog/Genetics, Dsmorphisms and Metabolism
Lower Gastrointestinal Bleeding
seeCastroenterolo_
E TIOLOGY
Acute
inlectious
Lacterial, parasitic, antiLiotic-induced C. diffcile)
necrotizingentercolitisinpreterminlants
anatomic
malrotation/volvulus,intussusception
Meckel sdiverticulitis
anallissures,hemorroids
vascular/ hematologic
Henoch-SchnleinPurpuraHSP)
hemolyticuremicsyndromeHUS)
coagulopathy
Chronic
anallissuresmostcommon)
colitis
inllammatoryLoweldiseaseI5D)
allergicmilkprotein)
structural
polyps mostarehamartomas)
neoplasmsrare)
coagulopathy
Assessment
hemodynamicstatus,evidenceolFTT,lever
analandrectalexam
tags,lissures,anallistulas,polyps
loreignLody
Llood
stoolappearance
MCaspirate
lowerCILleedma
presentasmelenailitinvolves thesmallLowel)orhematochezia
stoolculturesC. diffcile)
urinalysisandmicroscopy
C5C,smear,dillerential,platelets,ESR,electrolytes,urea,creatinine,IMR,PTT, alLumin,
ironstudies, amoeLatiters
radiologicinvestigations
aLdominalx-rayAXR)toruleoutoLstruction
Treatment
acutestaLilization.A5Cs, volumeandLloodreplacement,Lowelrest MPO, MCtuLe)
oncestaLle,endoscopyandsurgeryasindicated
Genetics, Dysmorphisms and Metabolism
minoranomaly- anunusualanatomicleature thatisolno seriousmedicalorcosmetic
consequencetothepatients
majoranomaly
J
mallormation resultslromanintrinsicallyaLnormaldevelopmentalprocess
e.g.polydactyly)
disruption resultslromtheextrinsicLreakdownol,oraninterlerencewith,an
originally normaldevelopmentalprocesse.g.amnioticLanddisruption
sequence)
delormation resultslrommechanicallorcese.g.potterdelormationsequence)
Mendelian Inheritance
disorderscausedLymutationoloneorLothcopiesolagene,inheritedinoneolseveral
patterns
autosomal encodedLygenesononeol22pairsolautosomes
autosomaldominantAD)- disorderisexpressedinaheterozygote
inheritanceis `vertical`,Lothmalesandlemalesareallectedandcantransmit
thetrait), e.g. neuroliLromatosis typeI
autosomalrecessive AR)* disorderis manilestonlyinhomozygotes
inheritanceis `horizontal`,diseasenotloundinmultiplegenerations,parentsol
anallectedchildareusuallynormal),e.g.cysticliLrosis
Pediatrics P41
- `
+P
Definitions
Association * nonrandom
concurrence of i ndependent
malformati ons whose eti ol ogy i s
unknown ( e. g. VACTERL associ ati on)
Sequence pattern of multi pl e
anomal i es derived from a si ngl e known
or presumed prior anomal y or
mechani cal factor ( e. g.
ol i gohydramni os sequence)
Syndrome * recognized pattern of
devel opmental ly i ndependent
malformati ons havi ng one known
eti ol ogy ( e. g. Down syndrome)
P42 Pediatrics Genetics, Dysmorphisms and Metabolism Toronto Notes 2010
X-linked encodedLyageneontheXchromosome
maleshaveasingleXchromosomeandareallected,lemaleshavetwoX
chromosomes,andrecessiveX-linkeddisordersarerarelyexpressedinlemales,
ie.DuchenneMuscularDystrophyDMD)
Approach to the Dysmorphic Child
2-3, olinlantsareLornwithaseriouscongenital delect, J, olnewLornshavea
monogeneticdisease,0. 5,haveachromosomaldisorderandJ-3,haveamultilactorial
illness
geneticdisordersarethemostcommoncauseolinlantdeathindevelopedcountries
diagnosisolsyndromesisLasedonpatternoldysmorphicleaturesandorganinvolvement
History
prenatal / oLstetricalhistory seeOLstetrics)
complete3generationlamilypedigree.consanguinity,stillLirths,neonataldeaths,specilic
illnesses,intellectualdisaLility, multiplemiscarriages,ethnicity
growthparametersandpattern.headcircumlerence HC), heigt Ht), andweight Wt)
skull. contour andsymmetry
hair.textureandpattern
neck. looklorredundantnuchalskin/ weLLedneck
lacialgestalt.comparewithsiLlingsandparents
ears. structure,size,placementandrotation
eyesandadnexa.distanceapart,orientation,eyeLrowsandeyelashes,anyloldsorcreases,
coloLoma,lundus
nose. nasalLridge,nostrils
philtrum.lengthandshape
mouth.lips,palate,tongueandteeth
chin.sizeandposition
thorax.shape,size,andnipplespacing
handsandleet.creases,structuree.g.overlappinglingers / toes),andnails
limLs.proportionsandamputations
spine.scoliosis, kyphosis
genitalia.amLiguous
skin.hairtults, sacraldimples /sinus
Investigations
asklorserialphotographsilchildisolder,lamilypictures
x-raysilLonyaLnormalitiesorilsuspectacongenitalinlection
cytogenetic /chromosomestudies skinliLroLlasts
Liochemistry.specilicenzymeassays
geneticproLesnowavailaLlee.g.FragileX, microdeletion22,intheluture,microarray
techniques
prenatalcounsellingandrecurrence riskassessment
skinliLroLlastsilmosaicismsuspected
Genetic Syndromes
Tabl e 23. Common Genetic Syndromes
Disease
Incidence
CraniunVrain
Eyes
Ears
Facial features
Trsomy 21
Down syndrome
1 : 600800 bi rhs
Trsomy 1 8
Edwards syndrome
1 : 6000 live bi rths
Most common abnormality of autosomal chromosomes Femal e: mal e " 3: 1
Ri ses with advanced maternal age from 1 : 2000
at age 20 to 1 : 20 by age 45
Mi l d microcephaly, fl at occi put, 3rd fontanel le,
brachycephal y
Upsl anti ng pal pebral fi ssures, i nner epi canthal folds,
speckled iris ( Brushfi el d spots), refractive errors
(myopi a), acqui red cataracts, nystagmus and strabi smus
Lowset, smal l , overfol ded upper hel ix, frequent ADM,
heari ng loss
Protrudi ng tongue, large cheeks, low flat nasal bridge,
small nose
Mi crocephal y, promi nent occi put
Microopthal mi a, hypotel ori sm, i ri s coloboma,
reti nal anomal i es
Lowset, malformed
Cleft l i p/palate
Smal l mouth, mi crognathi a
Trisomy 1 3
Patau syndrome
1 : 1 0 000 live bi rths
Mi crocephaly, sl opi ng forehead, occi pital
scal p defect, hol oprosencephal y
Mi croopthal mi a, corneal abnormal iti es
Lowset, malformed
6080% cleft lip and palate
Toronto Notes 2010 Genetics, Dysmorphisms and Metabolism Pediatrics P43
Tabl e 23. Common Genetic Syndromes ocnt|nuedj
Trsomy 21 Trisomy 1 8 Trsomy 1 3
SkeletaVMSK
Ca rdiac defect
GI
GU
CNS
Other features
Short stature
Excess nuchal ski n
Joi nt hyperflexi bi l i ty (80%) i ncl udi ng dysplastic hi ps,
vertebral anomal ies, atl antoaxi al i nstabi l ity
40%, parti cul arly AVSD
DuodenaVesophageaVanal atresia, TE fistul a,
Hi rschsprung disease, chroni c consti pati on
Cryptorchi di sm, rarel y fertile
Hypotoni a at bi rth
Low In, devel opmental del ay, heari ng probl ems
Onset of Al zhei mer's di sease i n 40's
Si mi an (transverse pal mar) crease, cl i nodactyly and
absent middle phal anx of the 5th fi nger
1 % lifetime ri sk of l eukmi a
Polycythemi a
Hypothyroi di sm
Short stature
Cl enched fist with overl appi ng di gits,
hypopl asti c nai l s, cl i nodactyly, polydactyly
60% (VSD, PDA, ASD)
Herni a
Polycystic kidneys, crptorchi di sm
Hypertonia
Small for gestati onal age (SGA)
Rocker-bottom feet
PrognosisManagement Prognosis: long-term
Management:
44% di e i n 1 st month
1 0% surive past 1 year (profound i ntel l ectual
di sabi l i ty i n surivors) Recommend chromosomal anal ysi s
Echo, thyroi d test, atlanto-occi pi tal x-ray at 2 and
1 2 years (controversi al ), heari ng test, ophthal mology
assessment
Early interention programs help chi ldren reach ful l
potenti al
Tabl e 24. Most Common Sex Chromosome Di sorders
Genotype
Incidence
Phenotpe
Turner Syndrome
45X (most common)
Mosai c: 46XX wi th p del eti on,
45XO/47XXX
1 : 4000 live female bi rths
Ri sk not increased wi th advanced
maternal age
Noonan Syndrome
46XX and 46XY
Autosomal domi nant with
variable expression
Hi gher transmi ssi on of affected
maternal gene
1 : 2000 mal e and femal e live bi rths
Certai n phenotypi c features si mi l ar
to females wi th Turner syndrome
Kinefelter Syndrome
47 M(most common)
48) 49XXX
1 : 1 000 live male bi rths
Increased ri sk wi th advanced
maternal age
Tal l , sl i m, underei ght
No features prepuberty
Severe growth retardation
Polydactyly, cl enched hand
80% (VSD, PDA, ASD)
Polycystic kidneys
Hypo- or hypertoni a
Sei zures, deafness
Severe developmental delay
Si ngl e umbi l i cal arter
Mi dl i ne anomal i es: scalp, pi tui tar, palate,
heart, umbi l i cus, anus
33% di e i n 1 st month, 50% by 2nd month,
90% by 1 year from FTI
Profound i ntel l ectual di sabi l ity i n surivors
Fragile XSyndrome
X-l i nked
Geneti c anti ci pation
CGG tri nucl eotide repeat on X
chromosome confers easy breakage
of chromosome
1 : 3600 mal es
1 : 6000 femal es
Most common geneti c cause of
i ntel l ectual di sabi l ity i n boys
Overgrowth: Short stature, short webbed neck,
low posterior hair l i ne, wide
carrying angle
Broad chest, wi del y spaced ni ppl es
Lymphedema of hands andor feet,
cystic hygroma in newborn wi th
polyhydrami ni nos, l ung hypopl asi a
Short stature, webbed neck,
triangul ar facies, hypertelorism, low
set ears, epi canthal folds, ptosis
Postpuberty: male may suffer from
developmental delay, long l i mbs,
gynecomastia, l ack of faci al hai r
Promi nent jaw, forehead, and nasal
bridge
Long, thi n face wi th large protuberant
ears, macroorchi di sm
Coarctati on of aorta, bi cuspi d aortic
valve
Pectus excavatu m
Ri ght-si ded congeni tal heart
disease, pul monar stenosi s
Renal and cardiovascular abnormal iti es,
i ncreased ri sk of HTN
l 0hBehaviour
Gonad hReproductve
Function
Less severe spectrum wi th mosaic
Mi l dl y deficient to normal
i ntel l i gence
Streak ovaries with defi ci ent
fol l i cles, inferti l i ty, primary
amenorrhea, i mpai red
development of secondar
sexual characteristics
Diagnosisrognosil Normal life expectancy i f no
Management compl i cati ons
Increased ri sk of X-l i nked
diseases
Management:
Echo, ECG to screen for cardiac
malformation
GH therapy for short stature
Estrogen replacement at ti me of
puberty for devel opment of
secondar sexual characteristics
(same as mal es)
Moderate i ntel lectual di sabi l ity
in 25% of patients
Delayed puberty
Management:
Affected males may requi re
testosterone replacement therapy
at puberty
Echo, ECG
Mi l d i ntel lectual di sabi l ity
Behavi oural or psychiatric
disorders - anxi ety, shyness,
aggressive behavi our, anti soci al
acts
Infertility due to hypogonadi sm
hypospermia
Management:
Testosterone i n adol escence
Hyperextensi bi l ity, hi gh arched pal ate
Compl i cati ons:
Sei zures, scol iosis, mi tral valve
prolapse
Mi l d to moderate i ntel l ectual di sabi l ity,
20% of affected mal es have normal l Q
ADHD andor auti sm
Femal e carriers may show i ntel l ectual
i mpai rment
Di agnosi s:
Cytogeneti c studi es: regi on on Xq
whi ch fai l s to condense duri ng
mi tosi s, fragi le X marker
Mol ecul ar testi ng: overampl i fi cati on
of the tri nucl eotide repeat, length of
segment is proporti onal to severity of
cl i ni cal phenotype (geneti c
anti ci pati on)
P44 Pediatrics
Tabl e 25. Other Genetic Syndromes
DiGeorge Synd rome
Genote Mi crodel eti ons of 22q1 1
PraderWiIIi Syndrome
Lack of paternally i mprinted genes on
chromosome 1 5q 1 1
Due to del eti on of paternal chromosome 1 5q1 1
or two maternal chromosome 1 5s (maternal
uni parental di somy)
Toronto Notes 2010
Angelman Syndrome
Lack of maternal l y i mprinted genes on
chromosome 1 5q1 1
Incidence Second most common geneti c di agnosi s
(next to Down syndrome)
1 : 1 5000
Clinical Features "CATCH 22"
Cyanoti c CHD (may account for up to 5% of al l cases of CHD)
"H30": hypotoni a and weakness, hypogonadi sm,
obsessive hyperphagi a, obesity
Severe i ntel l ectual di sabi l ity, seizures,
tremul ousness, hypotonia
Anomal i es: craniofacial anomal i es typi cal ly micrognathia and Short stature, al mondshaped eyes, small hands
and feet wi th taperi ng of fingers
Mi dface hypoplasia, fai r hai r,
uncontrol l abl e l aughter low set ears
Thymi c hypopl asi a "i mmunodeficiency" recurent infections Development delay (vari abl e)
Cognitive i mpai rment Hypopi gmentati on, DM I I
Hypoparathyroi di sm, hypocal cemi a
22q1 1 microdeleti ons
- `
*P
Causes of I ncreased CK
Prol onged exerci se ( 5 1 Ox i ncrease)
Trauma
Rheumatoi d arthri ti s
DMD ( i ncrease from bi rth)
Muscular Dystrophy (MD)
agroupolinheriteddiseasescharacterizedLyprogressiveskeletaland cardiac muscle
degeneration
Duchenne Muscular Dystrophy (DMD)
J.4,000males,J / 3 spontaneousmutations,2/ 3X-linkedrecessive
missingstructuralproteindystrophin muscleliLrelragility liLreLreakdown
necrosisandregeneration
clinicalleatures
proximalmuscleweaknessLyage3,Cower ssigni.e.childuseshandsto climLup
thelegstomovelromasittingtoastandingposition),waddlinggait,toewalking
common
hypertrophyolcallmusclesandwastingolthighmuscles
decreasedrellexes
dystrophinisexpressedintheLrain, andLoyswithDMDmayhavedelayedmotor
andcognitivedevelopment,thisisnotprogressive
cardiomyopathy
moderateintellectualcompromise
diagnosis
lamilyhistory pedigreeanalysis)
increasedCK50-J00xnormal)andlactatedehydrogenase
muscleLiopsy electromyography EMC)
complications
patientusuallywheelchair-LoundLyJ2yearsolage
earlyllexioncontractures, scoliosis, developosteopeniaolimmoLility, increasedrisk
ollracture
deathduetopneumonia/respiratorylailureorCHFin2nd-3rddecade
treatment
supportive i.e.physiotherapy, wheelchairs,Lraces),preventoLesity
surgicallorscoliosis)
useolsteroidse.g.prednisoneordellazacort)
genetherapytrialsunderway
Beck er' s Muscular Dystrophy
X-linkedrecessive, duetoamutationindystrophingenewithsome proteinproduction
sym
tomssimilartoDuchenneLutonsetislaterinchildhoodandprogressionisslower
deat
[
duetorespiratorylailurein4thdecade
Associations
Associationsareanon-randomoccurrenceolmultiplemallormationswithunknown
etiology, whilesyndromesareapatternolanomalies thathaveaknownetiology
twocommonassociationsareVACTERLandCHARCE
VACTERLshouldLesuspectedwhenachildisloundtohavetracheo-esophageallistula.
V VerteLraldysgenesis/0,)
A Analatresiaimperlorateanus) listula80,)
C Cardiacanomalies VSD53,)
T-E TracheoEsophageallistula esophagealatresia/0,)
R Renalanomalies53,)
L LimLanomalies radialdysplasia,pre-axialpolydactyly, syndactyly 65,)
mayalsopresentwithasingleumLilicalarteryorFTT
prognosis.mayhavenormalhealthandmentaldevelopmentwithaggressive
treatmentolaLnormalities
Toronto Notes 2010
CHARGE
C ColoLoma
H congenitalHeartdisease
A choanalAtresia
R mentalRetardation
G GUanomalies
E Earanomalies
etiologythoughttoLeduetoaLnormaldevelopmentratherthanageneticmechanism
Metabolic Disease
aninheriteddisorderolintermediarymetaLolism,oltenautosomalrecessive
inlantsandolderchildrenmaypresentwithlailuretothriveFTT)ordevelopmentaldelay
currentlyinCanadaandtheUnitedStates,newLornsmayLetestedlorCAH,congenital
hypothyroidism, galactosemia, sicklecelldisease incertainethnicgroups), PKU,maple
syrupurinedisease,homocystinuria,andLiotinidasedeliciency
typesoldisorders
proteins.PKU,tyrosinemia,organicaciddisorders,ureacycledelects
carLohydrates.galactosemia,glycogenstoragediseases
lats.lattyacidoxidationdelects
organelledisorders.congenitaldisordersolglycosylation,mucopolyaccharidosis
Clinical Manifestations
vomitingandacidosisalterleedinginitiationaminoacidorcarLohydrate metaLolicdisorder)
hepatosplenomegalymetaLolitesaccumulateintheliver)
neurologics
ndrome.acuteandchronicencephalopathy, intellectualdisaLility,
megalencep alymucopolysaccharidedisorders)
severe acidosisaminoaciduria),hyperammonemiaureacycleandorganicaciddisorders)
growthretardation,seizures,coma,hypoglycemia
autonomicmanilestationse.g. pallor,sweating,tremor)
Physical E xam
odour.Lurntsugar, sweaty leet,musty,ammonia-like
skin.hypo / hy
erpigmentation,rash,xanthomas
hair. alopecia,irsutism,aLnormalarchitecture,laircolouring
eyes.corneaclouding,crystals),lens cataracts, dislocation), retinamacularcherryred
spot,pigmentretinopathy,opticatrophy)
Initial Investigations
electrolytes,A5Cs calculateaniongap,ruleoutacidosis)
C5Cwithdillerentialandsmear
Lloodglucosehypoglycemiaseenwithorganicacidemia,lattyacidoxidationdelectsand
glycogenstoragediseases)
lactate,ammoniumhyperammonemiawithureacycledelects),plasmaCaandMg
routineurinalysis.ketonuriamustLeinvestigated
others.urate,urinenitroprusside,aminoacidscreen,CSFglycine,lreelattyacids
3-Leta-hydroxyLutyrateratio> inlattyacidoxidationdelect)
storagediseases.urinemucopolysaccharideandoligosaccharidescreen
Phenylketonuria (PKU)
incidence.JinJ0,000
screenedinallnewLorns
etiology.deliciencyolphenylalaninehydroxylasepreventsconversionolphenylalanineto
tyrosineleadingtoLuildupoltoxicmetaLolites
motherswhohavePKUmayhaveinlantswithcongenitalaLnormalities
presentation
LaLyisnormalatLirththendevelopsamustyodour, eczema,hypertonia,tremors,
andmentalretardation
hypopigmentationduetolowtyrosinelairhair,Llueeyes)
treatment
PKUscreeningatLirth
dietaryrestrictionolphenylalaninestartingwithinthelirstJ0daysollile
durationoldietaryrestrictioncontroversial lilelon
oruntilendolpuLerty, should
Leresumed duringpregnancytomaintainnormalp
enylalaninelevels
Galactosemia
incidence.J in60,000,autosomalrecessivedisease
mostcommonlyduetodeliciencyolgalactose-J-phosphateuridyltransleraseleadingtoan
inaLility toprocesslactose/galactose
increasedriskolsepsis
ilthediagnosisisnotmadeatLirth,liverandLraindamagemayLecomeirreversiLle
Pediatrics P45
- '
P
Metabol i c di sease must be rul ed out in
any newborn who becomes acutel y i l l
after a peri od of normal behavi our and
devel opment or wi th a fami l y hi story of
early infant death.
P46 Pediatrics
Normal Hg Values by Age
Age Hg Range (giL)
Newborn 1 37-201
2 weeks 1 30-200
3 months 95- 1 45
6 months- 6 years 1 05- 1 40
7- 1 2 years 1 1 0- 1 60
Adul t lema I e 1 20- 1 60
Adul t mal e 1 40- 1 BO
~ `
VOVi n chi l dhood vari es with age

Rule ofthumb: lower normal l i mi t of
VOV * 70 + age(yrs) unti l BO II ( adul t
standard) .
-`
+F
Iron deli ci ency i s rare i n chi l den <6
months i n the absence o f bl ood l oss or
prematuri ty.
-`
+P
Ferri ti n i s an acute phase reactant,
therefore, normal or hi gh ferritin does
not excl ude i ron defi ci ency anemi a
duri ng an i nfecti on.
Genetics, Dysmorphisms and Metabolism/ematolog Toronto Notes 2010
leatures.neonatewhoingestslactose /galactoseexhiLitssignsolliverandrenallailure,
jaundice,FTTandcataracts
treatment
eliminationolgalactoselromthediet ie. dairyLreastmilk)
mostinlantsareleda soy-Laseddiet
Hematology
Physiologic Anemia
highhemogloLin>J/0g/ L)andreticulocytecountatLirth asa result olrelativelyhypoxic
environmentinutero
alterLirth,levelsstarttolallduetoshorterletalR5Clilespan, decreasedR5Cproduction
duringlirst6-8weeksollilevirtuallynoerythro
oiesisduetonewOrichenvironment)
andincreasingLloodvolumesecondarytogrowt
[
lowestlevels aLout J00 g/ Lat8-J2weeksageearlierandmoreexaggeratedinpremature
inlants),levelsrisespontaneouslywithactivationolerythropoiesis
notreatmentusuallyrequired
Iron Deficiency Anemia
most commoncause olchildhoodanemia
lullterminlantsexhaustironreservesLy6monthsage
preterminlantshavelower reserves exhaustLy2-3monthsolage
commondiagnosisLetween6months-3yearsandJJ-J/years. periods olrapidgrowthand
increasedironrequirements,adolescentsalsohavepoor dietand menstruallosses
cancauseirreversiLleellectsondevelopmentiluntreatedLehaviouralandintellectual
deliciencies)ininlancy
presentation.usuallyasymptomaticuntil marked anemia, pallor, latigue, pica eating
non-loodmaterials), tachycardia,systolicmurmur
complications.angularcheilitis, glossitis,koilonychia
E tiology
dietaryrisklactors
milkLaLy LaLy9-24monthsold)receivinglargevolumes>20ozperday)ol
cowsmilkusuallyLyLottleleadingtopoorintakeoliron-richloods
lormulawithoutiron
delayedintroductionolironlortiliedinlantcereal
Lloodloss
iatrogenic.repeatedLloodsamplingespeciallyinhospitalizedneonates)
truecowsmilkallergy.occultLleedingandprotein-losingenteropathysecondaryto
CIinllammation
Investigations
C5C. lowMCVandMCH,reticulocytecountnormal orhigaLsolutenumLerlow),
normalW5C
MentzerindexMCV/ R5C)canhelpdistinguishLetweentwomostcommoncausesol
microcyticanemia.irondeliciencyanemialromthalassemia.Ilratio<J3suggestiveol
thal,>J3suggestiveirondeliciency
Lloodsmear.hypochromic, microcyticR5Cs,pencilshapedcells,poikylocytosis
ironstudies.lowlerritin,lowiron,highTI5C
initialtheraphy.trialoliron
Prevention
Lreast-ledlull-terminlants. alter6months,giveiron-lortiliedcereals andiron-richloods
non-Lreast-ledinlants.giveiron-lortiliedlormulalromLirth
prematureinlants.giveironsupplementslromJmonthtoJyearolage
Management
encourage diverse,Lalanceddiet,limithomogenizedmilkto J6-20oz/day
oralirontherapy 6mg/ kg/ dayelementaliron,divided5IDtoTID,lor3monthsto
repleteironstores
increasedreticulocytecountin2-3dayspeaksday5-/)
increasedhemogloLinin4-30days
repletionolironstoresinJ-3months
re-checkhemogloLinlevelsalterJmontholtreatment
poorresponsetooralirontherapy.non-compliance,ongoingLloodloss,incorrect
diagnosis,insullicientdurationoltherapy,highgastricpHantaciduse)
Toronto Notes 2010 Hematolog
Anemia of Chronic Disease
mostoltennormocytic,normochromicmicrocytic,hypochromicmayoccurwithchronic
inlection/malignancy)
multi-lactoral inorigin
chronicinllammatory statesincludingjuvenileidiopathicarthritis ]IA), chronicinlections,
chronicrenallailure,andmalignancies
ironstoresarevariaLleand lerritinlevelsareunreliaLleacutephasereactant)therelore
LonemarrowassessmentmayLenecessarylordiagnosis
anemiaolchronicrenallailurepredominantlycausedLydecreasedEPOproduction
treatmentwitherythropoietin
Hemog lobinopath ies
SICKLE CE LL DISEASE
seeHematolo_
identilicationolspecilicgenotypesimportantduetodillerencesinlrequency,typeand
severityolclinicalcomplicationsmostsevere.SS,lesssevere.SC,S-Letathalassemia,
rare.SD). Alsoimportantlorgeneticcounselling.
Pathophysiology
causedLyageneticdelectinLeta-gloLingenes
HgS. singleamino acidreplacement glutamic acid valine)
redLloodcellssickleunderconditionsolstresslowpO
,dehydration,lever,acidosis)
acuteintravascularsicklingresults ininlarctionoltissue capillaryocclusionand
thromLosis spleen, lungs, Lones, Lrain, digits)
hemolysiscauseschronic,well-compensatednormochromicnormocyucanemiaH60-90g/L)
increasedincidencei npeopleolAlricanandMediterraneanheritage
greatestcauseolmortalityisinlection
Presentation
newLorns lromhigh-risklamiliesundergo screeningmayLepartolprovincialnewLorn
screeningprogram
clinicaldiseasepresentsalter5-6monthsolagealterlallinletalHL
anemia,lever,jaundice, splenomegaly, crisis dactylitisisoltenthelirstpresentation)
sicklecelltrait.asymptomaticmayhavemicroscopichematuria)
Types of Crises
vaso-occlusivecrisis
duetooLstructionolLloodvesselsLyrigid,sickledcells tissuehypoxia cell
death,presentsasleverandpaininanyorgan,mostcommonlyinlongLonesolarms
andlegs,chest,aLdomen,CMSstroke),dactylitisinyoungchildren),priapism
acutechestcrisis.lever,chestpain,progressiverespiratorydistress,increasedW5C
count,pulmonaryinliltrates
aplasticcrisis depressionolerythropoiesis decreasedreticulocytecountto<J,,
decreasedHL),generallyassociatedwithinlectionespeciallyparvovirus5J9)
acutesplenicsequestration suddenmassivepoolingolredcellsinspleen,splenomegaly,
tenderspleen,acutelallin hemogloLin,shock,increasedreticulocytecount
Functional Asplenia
splenicdyslunctionusuallyLy5yearsolagesecondarytoautoinlarction
susceptiLle toinlectionLyencapsulatedorganisms especially S. pneumoniae)
requiresprophylacticantiLiotics,pneumococcal / meningococcal /H. injuenzae typeL
vaccination, andimmediate evaluationolanylever
Other Manifestations
increasedincidenceolosteomyelitisespeciallydueto Salmonella)
longtermcomplications.growthdelay,LonyaLnormalities e.g.avascularnecrosisAVM)
ollemoralhead, gallstones,retinopathrestrictivelungdiseasescreenwithPFTs),
cardiomyopathy screenwithecho)
Management
acutecrises
admitlorsupportiveandsymptomatictreatment
lluids J. 5xmaintenance,Jxmaintenanceonlyilinchestcrisis), analgesiamorphine,
multi-modal),antiLiotics e.g. 3rdgen.cephalosporins),incentivespirometryto
decreaseriskolchestcrisis
straighttranslusionslorsymptomatic /signilicantanemia e.g. aplasticcrisis),
evolvingchestcrisis
R5Cexchangetranslusionlorimpendingstroke,severechestcrisis,persistentpriapism
O
ilrespiratorydistressorchestcrisiswithincentivespirometry)
culturesandC5CilleLrile,reticulocytecounts,CXRorLPilindicated
Pediatrics P47
` *
8% of Afri can Ameri cans carry the HbS

trai t, 0. 2% have the di sease.
Heterozygotes (trai t) are rel ati vel y
mal ari a resi stant.
P48 Pediatrics
-
-P
G6PD defi ci ency protects agai nst
parasi ti sm of RBCs ( i . e. mal ari a) .
Hematolog Toronto Notes 2010
chronic
earlyaggressivetreatmentolinlections,prophylacticantiLioticsdailyoralpenicillin)
pneumococcal, meningococcal,hepatitis5,HiL,andinlluenzavaccines
lolatesupplementationillolatedelicient
hydroxyureaillrequentcrises raisesHLFlevel)
transcranialdopplerultrasoundtoassess riskolstroke
chronictranslusionprogramilhistoryolstrokeoraLnormaltranscranialdoppler
geneticcounsellingandeducation
annualophthalmologicexamalterJ0yearsold)
relerraltohematology
HE REDITARY SPHE ROCYTOSIS
redcellmemLraneproteinaLnormality, causesaspheringol R5CswhichareremovedLy
thespleen
genetics
autosomaldominant positivelamilyhistoryin/5,)
highspontaneousmutationratenolamilyhistoryin25,)
widerangeolclinicalseveritylromwell-compensated,mildhemolyticanemiatosevere
hemolyticanemiawithgrowthlailure,splenomegaly,gallstones,neonataljaundiceand
chronictranslusionrequirementsininlancy
diagnosis.spherocytes circular R5Cs)onLloodsmear,osmoticlragilitytest
management
translusion,splenectomyasindicated
geneticcounselling
GLUCOSE 6 PHOSPHATE DEHYDROGENASE (G6PD) DEFICIENCY
X-linkedrecessive,themostcommonenzymedeliciencyworldwide
higherprevalenceinMediterraneans,AlricanAmericans,Asians
enzyme-delicientR5CunaLletodelendagainstoxidative stress inlection,drugs) lorm
HeinzLodies HLprecipitateswithinR5Cs) phagocytosedLysplenicmacrophages
creatingLitecells R5CsthatappeartohaveLitestakenoutolthem,alsoknownas
cookiecells)
presentswithacutehemolyticanemiahemogloLinuria,decreasedhaptogloLin,increased
LDH,andelevatedindirectLiliruLin)withjaundice,palloranddarkurinerarelycauses
chronicanemia)
diagnosis.C6PDenzymeassay
management.supportive,hydration,translusion,phototherapy
prevention.avoidknownoxidantse.g.lavaLeans,antimalarialsprimiquine),sullonamides)
Bleeding Disorders
seeHematolo_
Coagulation Defects
characterizedLyLleedingintojoints hemarthroses)andmuscles
largespreadingecchymosesandhematomas
Platelet Abnormalities
characterizedLypetechiae,purpura,Lruises,mucocutaneousLleedingi.e.epistaxis,
gingivalLleeding),menorrhagia,prolongedLleedinglromsuperlicialcuts
Tabl e 26. Classificati on of Bleeding Disorders
Site of Pathophysiology
Blood Vessels
Platelet
Coagulation Pathway
Mechanism
Vascul i ti s
Decreased production
Increased destruction
Increased consumpti on
Dysfuncti onal
Vitami n K deficiency
Factor VI I I deficiency
Factor IX deficiency
Abnormal vWF
Eamples
HenochSchinl ei n purpura
Drugs, marrow i nfi l trati on, l eUkemi a/l ymphoma
I mmune thrombocytopeni c purpura, infection, drugs
DI G, gi ant hemangi oma, hyperspl eni sm
von Wi l l ebrand di sease, drugs (ASA), uremi a
Hemorrhagic disease of the newborn
Hemophi l i a A
Hemophi l i a B
von Wi l l ebrand di sease
Immune Thrombocytopenic Purpura (lTP)
mostcommoncauseolthromLocytopeniainchildhood
peakage.2-6years,M=F
incidence5inJ00,000childrenperyear
Toronto Notes 2010 Hematolog/Infectious Diseases
causedLyantiLodiesthatLindtoplateletmemLranes splenicuptakeFc-receptor
mediated) destructionolplatelets
presentationandcourse
50,present J-3weeksalterviralillnessURTI,chickenpox)
suddenonsetolpetechiae,purpura,epistaxisinanotherwisewellchild
nolymphadenopathy, nohepatosplenomegaly
laLs. thromLocytopeniawithnormalR5C,W5C
ilatypical presentation>J celllineaLnormal,hepatosplenomegaly),doLonemarrow
toruleoutleukemia
dillerentialdiagnosis. leukemia,drug-inducedthromLocytopenia, HIVinlection
viral), SLE
highestriskolLleedinginlirst J-2 weeks
management
sell-limitedinchildren,spontaneous recoveryin>80,olcases
usuallychoosetotreatLecausespontaneousrecoverytakesalewmonths,andriskol
Lleedingespeciallyintracranialhemorrhagewithplatelets<20)
IVICororalprednisonemainstaysoltreatment),IV anti-DilLloodgroupKpositive)
splenectomyonlylorlile-threateningLleeding)
avoidASA/ MSAIDS
nocontactsports
reassurance.verylowriskolserioushemorrage3,),andCMShemorragerare 0. 5,)
Hemophilia
seeHematolo_
von Willebrand's Disease
seeHematolo_
Tabl e 27. Evaluati on of Abnormal Bruisi ngBl eedi ng
BT M M VI I I : C vF Platelets Fibrnogen
Hemophi l i a A N N J + N N N
Hemophi l i a B N N J N N N N
von Wi l l ebrand's J N N or J + + N N
DI C N or J J J + N + +
Vit. K deficiency N J J N N N N
Thrombocytopeni a J N N N N + N
BT Bleeding Time, VIII:C Factor VIII Coagant Activity, vWF von Willebrand's Factor, DIC Disseminated Intravascar Coagulation
Infectious Diseases
Tabl e 28. Anti biotic Treatment of Pediatri c Bacteri al I nfecti ons
Infection
MeningitsSepsis
Neonatal
(birth up to 6 weeks)
6 weeks3 months
>3 mos
Otiis Media
1 st l i ne
2nd l i ne
Treatment Fai l ure
Strep Pharngiis
UTI
Pathogens
GBS, Eco/i, Listra,
Other: Gramnegative baci l l i
Same pathogens as above and below
S. pneumococcus, N. meningitdis,
H. infuenae type b ( > 5 yrs)
S pneumoniae, H. infuenae,
M catarha/is, S pvogenes
Treatment
Ampi ci l l i n + ami noglycoside (gentami ci n or tobramyci n) (sepsi s)
Ampi ci l l i n + cefotaxi me Z vancomyci n ( meni ngi ti s)
Ampi ci l l i n + cefotaxi me Z cloxaci l l i n i f ri sk of S aurus (sepsi s)
Ampi ci l l i n + cefotaxi me Z vancomyci n ( meni ngi ti s)
Cefotaxi me + vancomyci n (sepsi s)
Ceftriaxone + vancomyci n ( meni ngi ti s)
amoxi ci l l i n
Hi gh dose amoxi ci l l i n or clavul i n
Hi gh dose clavul i n or cefuroxime or ceftriaxone
Group A betahemolytic Steptcoccus Peni ci l l i nlamoxi ci l l i n or erthromycin ( peni ci l l i n allergy)
E coli, Kebsiela, Prtus, H. infuenae, Cephalexi n, cefixime ( uncompl icated)
Pseudomonas, S saprphycus, or
Entrcoccus, GBS IV ampi ci l l i n and gentami ci n (i f compl icated)
Ampi ci l l i n and getami ci n (neonates)
Pediatrics P49
Coricosteroids versus Intravenous Immune
Globulin for the Treatet dAcute Immune
Trombocyopenic Purpura in Children: A
Systematc Reiew and Meta.analysis d
Randomized Contolled Trials
J Piar 2005 Oct;1 47141: 521 -7
Study: Meta-analysis of 1 0 RCTs from 1 985-2003.
RCTs compared corticosteroids and IVIG in the
treatment of pediatric ITP and had to include patient
platelet counts.
Patients: 586 clildren 3 months to 1 8 years mage
who were presenting for the first time with prmary
acute ITP with no other underlng condition.
Interenton: Coricosteroids and IVIG at any dose.
Coricosteroid treatments included
methpredrisolone 1 0-30 !!d and prednisone
2-4 !wd. IVIG dosing ranged from 0.5-1 !kWd.
Treatment durations ranged from 1 -5 days.
Main Outome: Primary outcome was platelet
levels > 20,000/mm' 120xl O'ILI at 48 hours after
treatment. IThis oucome was chosen because
intracrarial hemorhage rarely occurs at platelet
above 201. Secondary outcomes included incidence
of i CH.
Results: The relative risk I RRI of reaching a platelet
count >20,000/mm' at 48 hours was 0.74 195%11
0. 65-0. 851 for corticosteroids versus IVIG lat any
dosel, with a NNT of 4.55 195%CI 3.23-7.691.
Subgroup analyses by dosing favoured IVIG in 6/1 0
dose comparisons. Oriy 3/586 clildren developed
ICH - %were treated with coricosteroids and one
with lVIG.
Summar: Children treated with coricosteroids are
less likely to have a platelet count > 20,OOlmm'
than clildren treated with IVIG after 48 hours of
therapy. However, optimal dosing of IVIG is unclear,
and impact of IVIG versus corticosteroids on ICH
and mortality are unclear.
-'
P
Extensive brui si ng i n the absence of l ab
abnormal i ti es: consi der chi l d abuse.
-'
7
Rochester Criteria " devel oped to
i denti fy i nfants " 60 days of age wi th
fever Irectal temperature " J8Ojat
low ri sk of seri ous bacteri al i nfecti on
Clinically
WBC count
Bands
Urinalysis
Well
51 5 x l O'IL
<1 . 5 x l O'IL
1 0 WBCHPF
Stool l diarrheal 5 WBC/HPF
Past Health Bom >37 wk
Home with/before mom
No hospitaliztions
No prior antibiotics use
No treated unexplained
hyperbi li rubi nemi a
No chronic di sease
P50 Pediatrics
Febrle Infants at Lo Risk for Serious Bacteral
Infection - An Appraisal dthe Rochester
Crtera and Implicatons for Management
Febrile Infant Colabotve Stdy Graup. Pdancs.
1 994; 94131; 390396
Purpose: To test the hypothesis that infants urJikely
to have serous bacteral infection ISBII can be
corectly identified using the Rochester critera.
Study Characterstics: Prospective study with
1 057 infants.
Paricipants: Febrile infants less than 60 days old.
Interenton: Application of Rochester criteria.
Main Outomes: Cture of speciments of blood,
cerebrospinal fld and urine for bacteria.
Results: Of the 1 057 febrile infants that were
involved, 931 were welllooking and 437 met the
remairing low rsk criteria. The negative predictive
value of the low rsk critera was 98.9% 195% CI.
97.2%-99.6%1 for SBI.
Conclusions: low rsk Rochester crteria are useful
in identifing infants at decreased rsk of SBI and
antibiotic use may be delayed in these patients.
`
>
Observation opti on onl y appropri ate i f
fol l owup can be ensured i f persi stent
symptoms.
A Review dAntibiotcs for AOM i n Children
Comparng any Antbiotic Against Obseration
Indicates tat Antbiotc Use has No Signifcant
Impact on Pain at l!hours and No Significant
Efect on Hearng
Cochrne 2004
In older children >6 years, it is approprate to
consider a "wait and see prescription", with
instrctions for reevaluation if the child has not
improved significantly within 72 hours.
In ctildren <2 years with bilateral AOM or AOM
with otorrhea, antibiotics are recommended by a
recent metaanalysis. IRovers MM, Glasou
Appelman Cl, et ai, Antibiotics for acute otitis
media: a metaanalysis with individual patient data.
Lancet 2006; 368: 1 4291 435. 1
Infectious Diseases Toronto Notes 2010
Tabl e 28. Anti bi otic Treatment of Pediatri c Bacteri al I nfecti ons ocnt|nued)
Infection Pathogens
Pneumonia (Communit Acquired, Bacteral)
Neonatal GBS, Gramnegative baci l l i ,|col),
btrchomats, S aurus, Listera
1 3 mos S pneumoniae, b trchomats,
5.penssis, S aurus, H. infuenzae
3 mosS yrs S pneumoniae, S aureus,
H. infuenzae, Mvcoplasma pneumoniae
>S years As above
Fever
, admit, ful l SWU'
anti bi oti cs pendi ng resul ts
or I F
, age 2890 days
, nontoxic and
' admi t,
ful l SWU' and
Treatment
, Ampi ci l l i n + gentami ci n, add erythromycin i f Chl amydi a suspected
, Cefuroxime 7 macrolide (erythromyci n)
or
, Ampi ci l l i n 7 macrolide
, Ampi ci l l i n/amoxi ci l l i n or cl avul i n or cefuroxime
, Macrolide (1 st l i ne)
or cefuroxime
or ampi ci l l i n/amoxi ci l l i n
or clavul i n
, rel i abl e F/U2 and
, l ow ri sk3 criteria
treat (I V anti bi oti cs) , uri ne R&M ' uri ne R&M
1
, SWU+Abx
, may consi der treati ng as outpatient4
NOES:
, CBC ' obserati on
I \
' F/U2 in 24 hours

WBC > 1 S
_
W
_
B
_
C
_
<
_
1 S
--
, blood C&S
, uri ne C&S
, acetami nophen
, amoxi ci l l i n 60 mg/kg/day
, bl ood C&S
, obserati on
, acetami nophen
, F/U2 i n 24 hours
1. Full Septic Workup SWUI . blood C&S. CBC and differential. urine R&M. C&S. LP CXR if respiratory SSx. stool C&S if GI SSx
2. Follovup is crucial if adequate FlU is not assured. a more aggressive diagnostic and terapeutic approach may be indicated
3. LowRisk RocheserjCriteria
4. Consi derble praOice variation exists i n terms of empirical Abx treatment
5. Imporant Principles the younger the child. the greater the difficulty to clinically assess the degree o illness
Fi gure 3. Approach to the Febri l e Chi l d
E pidemiology
peak incidence3mo-3yrs
E tiology
Lacterial/0,) - S. pneumoniae 25-40,),non-typaLleH. infuenzae J0-30,),M. catarrhalis
5-J5,), CroupAStreptococcus 3,), S. aureus
viral20,) - commonlyRSVinlluenza,parainlluenza,adenovirus
Definitions
certain diagnosis: J) recent,usuallyaLrupt, onsetolsignsandsym
tomsolmiddleear
inllammationandellusion2)presenceolmiddleearellusionMEE)3)middleear
inllammationMEI)
uncertain diagnosis: doesnotmeetallthreecriteria
severe illness: moderatetosevereotalgiaorlever>39C
non-severe il lness: mildotalgiaandlever<39C
Toronto Notes 2010 Infectious Diseases
History
otalgia, tuggingatears, otorrhea,decreasedhearing
irritaLility,lever,URI symptoms
nausea,vomiting,diarrhea
risklactors.Lottleleeding,passivesmoking,daycare,lowSES,cleltlip,Downsyndrome,
previous /recurrentAOM,lamilyhistoryolrecurrentOM,prematuritymalegender,
siLlings inhousehold
riskoldrugresistance.previousantiLiotictherapyinpastmonth,historyolAOM,daycare
attendance,ageJ8-24months,recenthospitalization
recurrentAOM 3episodesin6monthsor4 episodesinJyear
leLrile
MEEindicatedLyanyolthelollowingonotoscopy.lossollandmarks,Lulgingtympanic
memLraneTM),opaqueerythematousTM,yellowlluidLehindTM,decreasedTM
moLilityair-lluidlevelsLehindtheTM,otorrheailTMperlorated)
MEIindicatedLyeither.distincterythemaoltheTMordistinctotalgiadiscomlortclearly
releraLletothe ears)thatresultsininterlerencewithnormalactivityorsleep)
Management
80,olAOMinchildrensell-resolvewithout antiLiotictherapy,therelorecontroversy
existssurroundingantiLioticuseinAOMmanagement
antiLiotics
non-severe.amoxicillin40-50mg/kg/daydividedtid
severe.amoxicillin80-90mg/ kg/ dayusuallyreservedlorlailureolstandarddose)
treatmentlailure48-/2hralterinitialantiLioticuseinsevereAOM.celtriaxone,
celuroxime,oramoxicillin80-90mg/ kg/dayclavulin6.4mg/ kg/ day
analgesics.acetaminophenoriLuprolenlorpainmanagement
seeOtolar_ngolo_
Tabl e 29. Treatment of AOM
Age Cerain Diagnosis
< 6 mos Antibacterial therapy x 1 ad
6 mos to 2 yrs Anti bacteri al therapy x 1 ad
; 2 yrs Antibacterial therapy i f severe i l l ness x 5d;
obserati on opti on if nonsevere i l l ness
Meningitis
Uncerain Diagnosis
Antibacterial therapy x 1 ad
Severe i l l ness: anti bacteri al therapy x 1 ad
Nonsevere i l l ness: obserati on opti on
Obserati on opti on
peakage.6-J2months,90,occursin<5yearsold
Risk Factors
immunocompromisede.g.HIV, asplenia, prematurity)
neuroanatomicaldelects e.g. dermalsinus,neurosurgery)
parameningealinlectione.g.sinusitis,mastoiditis,orLitalcellulitis)
environmentale.g. day-carecentres,householdcontact)
E tiology
0-3months. Croup5 Strep., L. cali, L. monocytogenes, otherCram-negatives,viral HSV
enteroviruses)
3months-3years. S. pneumoniae, N. meningitidis, H. inJuenzae, T5,viralenteroviruses,
herpesvirus6,HSV)
3-J8years. S. pneumoniae, N. meningitidis, H. inJuenzae, viralenteroviruses,adenoviruses,
herpesviruses)
Pathophysiology
URTI compromisein integrityolmucosa Lloodstreaminvasion lromrespiratory
tract hematogenousseedingolmeninges meningealandCMSinllammation
toxic
URTI prodrome
lever,lethargy, irritaLility, photophoLia,nausea/vomiting,headache,still/soreneck
youngerinlants.maynotdemonstratelocalizingsigns,mayhavenon-specilicsymptoms
poorleeding,irritaLility, lethargy),Lulginglontanelle,increasingheadcircumlerence
signsolmeningismus
5rudzinskissign.rellexllexionolhipsandkneesuponactivellexionoltheneck
Kernigssign.rellexcontractionandpaininhamstringsuponextensionollegthatis
llexedatthehip
opisthotonos. spasminwhichheadandheelsareLentLackwardandLodyLowed
lorward
nuchalrigidity
Pediatrics P51
Te Concept and Practice da WaitandSee
Approach to Acut Oitis Media
Cure Opno i Pdarcs, 2008, 20:72-78
Purpose: To summarize recent AOM trials
comparing antibiotics versus a "wait and see
prescrption".
Study Characterstics: Guidelines forated
based on evidence from three recent clirical trals.
Recommendations:
Treat immediatelv wth antibiotics when:
Age <6 months
III appearance Ishock, unresponsive!
Recurrent AOM
Suspicion of another concurrent bacterial
illness le.g. pneumonia!
Recent treatment wth antibiotics Iwthin last 7
days!
Pertorated TM lincluding tympanostomy tubes!
Immunocompromised
Craniofacial abnormalities
Poor access to meclcal care
-'
Observation opti on onl y appropri ate i f

fol l owup can be ensured i f persi stent
symptoms. Consi der a safety net
anti bi oti c prescri pti on.
5'
Signs of Meningismus
BONK on the head
Brudzi nski 's si gn
Opi sthotonos
Nuchal ri gi dity
Kerni g' s si gn
P52 Pediatrics Infectious Diseases Toronto Notes 2010
signsolincreasedICP.headache,diplopia,ptosis,CMVIpalsy, Lradycardiawith
hypertension,apnea,papilledemaisucommon
seizurein 20-30, olpatientswithLacterialmeningitis
petechialrashmeningococcemia).associatedwithpoor prognosis
Diagnosis
lumLarpuncture LP)lorcereLrospinallluidCSF)
raisedopeningpressurenorms.recumLentandrelaxed,lessllexedposition
<J60mmH
O,llexedlateraldecuLitusposition- J00-280mmH
O)
cloudyinLacterialinlection
CSFexamination.W5C,protein,glucose,Cramstain,C&S,latexagglutinationtests il
partiallytreatedLacterialmeningitis),Ziehl-MeilsonstainilT5suspected)
viralversusLacterialmeningitisseeTaLle30)
Llood work.C5C,Lloodculturespositivein90,cases),Lloodglucose,electrolytesto
monitorlorSIADH)
Tabl e 30. CSF Fi ndi ngs of Meni ngitis
Component Nonal Child Normal Newborn Bacteral Meningitis Viral Meningiis Herpes Meningits
WBC V/L) 06 030 > 1 000 1 00500 1 0 1 000
Neutrphi l s ,X| 0 2-3 >50 <40' <50
Gl ucose ( mgdL) 40-80 32- 1 2 1 <30 >30 >30
Prtei n (mgdL) 20-30 1 9- 1 49 > 1 00 50-1 00 >75
RBC/ 0-2 0-2 0- 1 0 0-2 1 0-50
* lymphocytes predominate [Modified from Smith A, 1 1 9931 Pds in Review 1 4 : 1 1 - 1 8 and Ahmed A. et al, 1 1 9961 Ped Inf Dis J 1 5 : 298-3031
Complications
mortality.neonate J5-20,,children<J-8,,pneumococcus >meningococcus >HiL
morLidity.upto50, mayhaveneuroLehaviouralmorLidity, severeneurodevelopmental
sequelaeinJ0-20,
acute
SIADH hyponatremia Lrainedema seizures
suLduralellusion/empyema
LrainaLscess, disseminatedinlectionosteomyelitis,septicarthritis,aLscess)
shock/DIC
chronic
hearingloss
intellectualdisaLility, learningdisorders
neurologicaldelicit,seizuredisorder
hydrocephalus
Treatment
isolationwithappropriateinlectioncontrolproceduresuntil24hralterculture-sensitive
antiLiotictherapy
Lacterial.empiricantiLiotics,seeTaLle28
viral.supportive,acyclovirlorHSVmeningitis
most cases olviralmeningitiscanLesenthome exceptHIV)
ilneonatal,usehigdoseampicillinaspartolregimenuntilC5SandListeria ruledout
monitor. glucose, acid-Laseandvolumestatus
anticonvulsantsmayLeneededtotreatseizures
prophylaxis
H. infuenzae typeLvaccine routine
meningococcalvaccine ilasplenism, complementdeliciency,loroutLreaks, routine
insomeprovinces
pneumococcalvaccine ilimmuocompromised,asplenLm,routiwinsomeprovinces
5CC vaccine ilLorninT5-endemicarea
antiLioticprophylaxislorcontacts andindexcase
H. infuenzae rilampin
N. meningitidis rilampin,celtriaxoneorciprolloxacin
reporttopuLlichealth.acutemeningitis Lacterial,viral,other)
HIV Infection
seeInlectiousDiseases, ID28
E pidemiology
riskolverticaltransmission20-30,Lornto :mtreated HIVinlected women riskdecreases
lrom25,to<J, withappropriateantiretroviraltreatmentduringpregnancy)
transmission
inlantsandchildren.transplacentalmostcommon),maternalLlood,Lreastmilk
adolescents.sexualintercourse,needlesIVdruguseandtattoos),Lloodproducts
Risk Factors
HIVpositivemother
IVillicitdruguseIVDU)
motheriswithHIVpositivepartner
unprotectedsex
receiptolLloodproductsrare)
sexualaLuse
Incubation
timelromcontractinginlectiontodevelopingsymptomsisusually<2years,LutcanLe
severalyears
Clinical Features of AIDS in Infants and Children
signsandsymptomsoccuroltenwithinthelirstyear, mostwithintwoyears olage
encephalo
athy
recurrent/persistentthrush
chronicinterstitialpneumonitis relatively common),Pneumocystis jiroveci pneumonia
P]P)inlectionlormerlyPCP)
hepatomegaly
FTT,opportunisticinlections,lymphadenopathy
HIV Testing
shouldLe olleredtoallwomenasearlyaspossiLleinpregnancyrequiresconsent)
J'step.screeninglorHIVALwithELISA
ilpositivedo2^'step.conlirmatorytestlorALusingWesternLlotorimmunolluorescence
sensitivityandspecilicityol99,)
maternal HIVantiLodiescanpersistupto J8monthscanresultinalalse positive
HIVtest),typicallytestevery6monthslrom0-J8monthsilasymptomatic
ilLreastleedingrepeattest3monthsalterstoppingLreastleeding
othertests.viralnucleicacidLyPCR,viralculture,viralantigen
ilsexuallyactive,must re-test 6monthsalter J'test ilnegative)
Management
adequate nutritionLreastleedingcontraindicatedindevelopedcountries)
prompttreatmentolinlections
prophylaxis
TMP/ SMXlorP]P
azithromycinlormycoLacteriumaviumcomplexMAC)
nystatin,ketoconazole,acyclovirilindicated
IVIC
immunizations
allroutineimmunizationsincludingMMRand varicellailwell)
avoidOPV5CCandyellowlever
pneumococcal,inlluenza,andvaricellavaccines
suppressionolHIV
HAARThighlyactiveanti-retroviraltherapy)
HIVpositivepregnantwomenshouldLeolleredantiretroviraltherapyalongwith
resourceslorlormulaleedingtodecreaseperinataltransmission.
electiveC-sectionilnotontherapy, orilsignilicantviralload
Pharngitis and Tonsillitis
E tiology
viraladenoviruses,enteroviruses,E5Vvirus)- 80,
LacterialCroupAStreptococcus) - 20,
others.lungal(Candida), Kawasakis,retropharyngeal / peritonsillaraLscess,epiglottitis,
Lacterialtracheitis
cannotLereliaLlydistinguishedonclinicalleaturesalone
Clinical Features
relertoTaLle3J
exudativetonsillitis.CAS, adenovirus, E5Vdiphtheria
soltpalatepetechiae.CAS,E5V
Tabl e 31 . Cl i ni cal Features of Pharyngitis
Age
Onset
Fever
Clinical Featu res
Viral
<3
Gradual
Lowgrade
Sore throat
Rhi norrhea/cough
Conjunctivitis
Hoarseness
Rash
Bacterial
>3
Abrupt
>3BoC
Sore throat
NO rhi nitis! cough
Nausea
Abdomi nal di scomfor
HEENT fi ndi ngs: red pharnx, tender ceri cal nodes, tonsi l l ar exudates pal atal petechi ae
Pediatrics P53
P54 Pediatrics
R
Mci saac Criteria
Hot LCE
Fever >J8O
Lymphadenopathy- anterior. tender.
cervi cal
Age J I 1
No Cough
Erythematous. exudati ve tonsi l s
K
Scarlet Fever
4 S and 4 P
Sore throat
Strawberry tongue
Sandpaper rash
Peri oral Spari ng
Non-Pruri ti c
Non-Pai nful
Peel i ng
Streptococcal (GAS) Pharyngitis
Clinical Features
CroupAStreptococcus CAS) inlection
mostcommonly school aged,uncommoninchildren<3yrs
McIsaacCriteria.nocough,tender anterior cervicallymphadenopathy, erythematous
tonsilswithexudate,lever>38C,age3-J4
score0-J.noculture,noantiLiotic,2-3.culture,treatilpositive,4. antiLiotics
Management
>2yearsold,cultureLeloretreatmentordorapid Strep antigentest
rapidstreptestonly/0-90,sensitivepickup20,olcarriersolCAS),cultureil
negativethroatswaLlorcultureisgoldstandard,sensitivity90-95,)
symptomatic
ilJsymptom,nocultureorantiLiotics
il>Jsymptom,culture antiLiotics
penicillinVoramoxil 40mg/ kg/ dayPOdividedLidxJ0days
erythromycin40mg/ kg/ dayPOdividedtidx J0daysilallergictopenicillin
acetaminophenlordiscomlort
canpreventrheumaticleveriltreatedwithin9-J0days
antiLioticsdonotaltertheriskolpost-streptococcalglomerulonephritis
tonsillectomylorproven,recurrent streptococcaltonsillitis
complications
iluntreated,canleadto
suppurativecomplications. otitis media, sinusitis, cervicaladenitis, pneumonia,
mastoiditis
directextension.retropharyngeal/peritonsillaraLscess
scarletlever,rheumaticlever
hematogenousspread.Lone /j ointinlection,meningitis,S5E
acuteglomerulonephritisirrespectiveolantiLiotictreatment)
invasiveCASdisease.illnessassociatedwithisolationolCASlromnormallysterile
sitesLlood,CSF,orpleurallluid)
treatmentolinvasiveCASdisease
admit
IVclindamycin40mg/kgdividedinto3-4dosesIVpenicillin25000O00000U/kg/day
dividedinto6doses
otherillnessescausedLystrep.impetigo,cellulitis,Lacteremia,vaginitis,toxicshock
syndrome
streptococcaltoxicshock.illness associated withisolationolCASlromnormallysterile
sitesLlood,CSF,orpleurallluid)hypotension,renalimpairment,coagulopathy, liver
impairment,RDS,rash,solttissuenecrosisnecrotizinglasciitis,myositis,organgrene)
SCARLE T FEVE R
erythrogenic strainolCroupAStreptococcus
acuteonsetollever,sorethroat, strawLerrytongue
24-48hoursalterpharyngitis,rashLeginsinthegroin,axillae,neck,antecuLitallossa
within24hours,sandpaperrashLecomesgeneralizedwithperioralsparing,
non-pruritic,non-painlul
rashladesalter3-4days,mayLelollowedLypeeling
treatment. penicillin, amoxicillinorerythromycinilpenicillinallergic)xJ0 days
RHEUMATIC FEVE R
]onesCriteriarevised)
requires2majorORJ majorand2minorPLUSevidenceolprecedingstrepinlection
historyolscarletlever,groupAstreptococcalpharyngitisculture, rapidAgdetection
testonlyuselulilpositive),anti-streptolysinL titersASOT))
majorcriteria.SPACE"
SuLcutaneousnodules,pea-sized,lirm,non-tendernodulestypicallyon
extensorsurlaces
Pancarditisinvolvingpericardium,myocardium,endocardium
Arthritismigratory).verytender,red,warm,swollenjoints,allectsmostly
largejoints
ChoreaSydenhams).mayLecharacterizedLyclumsiness,dillicultywith
handwriting
Erythemamarginatum.LeginsaspinkmaculesontrunkwithcentralLlanching,
non-pruritic
minorcriteria
previoushistoryolrheumaticleverorrheumaticheartdisease
polyarthralgia
lever
elevatedESRorC-reactiveproteinorleukocytosis
prolongedPRinterval
treatment
penicillinorerythromycinloracutecoursexJ0days
ASAlorarthritis
prednisoneilseverecarditis
secondaryprophylaxiswithdailypenicillinorerythromycin,coursedependson.
withoutcarditis. 5yearsoruntil2Jyearsold,whicheverislonger
withcarditisLutnoresidualheart disease novalvulardisease). J0years orlonger
carditisandresidualheart disease persistant valvulardisease).atleast J0yearssince
lastepisode,sometimeslilelongprophylaxis
complications
acute.myocarditis,conductionsystemsinustachycardia,atrialliLrillation),
valvulitisacuteMR),pericarditis
chronic.rheumaticvalvularheartdisease mitraland/oraorticinsulliciency/
stenosis,increasedriskolinlectious endocarditis thromLoemLolicphenomenon
onsetolsymptomsusuallyalterJ0-20yearlatencylromacutecarditisolrheumatic
lever
Infectious Mononucleosis
thegreatimitator.systemicviral inlectionthat allects manyorgansystems
Epstein-5arrvirusE5V). amemLerolherpesviridae
incuLation.J-2months
spreadthroughsalivakissingdisease),sexualactivity
Clinical Features
prodrome.2-3daysolmalaise,anorexia
inlantsandyoungchildren.oltenasymptomaticormilddisease
olderchildrenandyoungadults.maydeveloptypicalinlectiousmononucleosissyndrome
lever,tonsillarexudate,generalizedlymphadenopathy, pharyngitis
hepatosplenomegaly
rashrashmorelrequentwithpatients treatedwithamoxicillin/ampicillin)
any -itis includingarthritis,hepatitis,nephritis,myocarditis)
chroniclatigue
resolvesover2-3weeksalthoughlatiguemaypersistlor several months
administrationolamoxicillinresultsinrashin >90, olcases
Complications
asepticmeningitis,encephalitis,Cuillain-5arr,splenic rupture, agranulocytosis,
myocarditisrare)
Diagnosis
heterophilantiLody testMonospottest)85, sensitive inadultsand older children,
only 50, sensitive <4yrsolage
lalsepositiveresults withHI,SLE, lymphoma, ruLella, parvovirus
E5Vtitres
C5Cdillerential.atypicallymphocytes,lymphocytosis,Downeycells, anemia,
thromLocytopenia
Treatment
throatculturetoruleoutstreptococcalpharyngitis
supportive careLedrest,lluids,salinegargleslorsorethroat,acetaminophen)
ilairwayoLstructedsecondarytonodeand/ortonsillarenlargement,admittohospital,
steroids
patientswithsplenicenlargementoltennotapparentclinicallysoallpatientsshouldavoid
contactsportslor6-8weeks
acyclovirnotuselul
Pertussis
Bordetella pertussis, whoopingcoug, J00-daycoug
incuLation.6-20days,inlectivity.J weekLeloreparoxysmsto3weeksalter
increaseinnumLerolreportedcasessinceearlyJ990s
spread.highlycontagious,viaairdropletsreleasedduringintensecouging
greatestincidenceamongchildren<Jyearandadolescents
prodromalcatarrhalstage
J-2weeks,mostcontagious
coryza,mildcough
Pediatrics P55
P56 Pediatrics
K
Complications of vv
2HAPE
2ndary bacteri al i nlecti on
Hepati ti s
Cerebel l ar Ataxia
Pneumoni a
Encephal i ti s
paroxysmalstage
2-4weeks
paroxysmsolcough,sometimeslollowedLyinspiratorywhoopwhoopmayLe
aLsentinchildren<6monthsoradults)
inlantsmaypresentwithapnea
vomitingwithcoughingspells
onsetolattacksprecipitatedLyyawningsneezing,eating,physicalexertion
canhaveseveresymptomslor6weeks,coughlor6months
pressureellect suLconjunctivalhemorrhage, rectalprolapse,hernias,epistaxis
convalescentstage
J-2weeks,noninlectious
occasional paroxysms olcough,Lutdecreasedlrequencyandseverity, lastsupto6
months
Di agno si s
clinical. URTI symptomslollowedLyparoxysmsolcoughinanaleLrilechild
lymphocytosis
PCR olnasopharyngealswaLoraspirate
Compli cati ons
otitismedia
respiratorycomplications
sinusitis,secondarypneumonia,atelectasis,pneumomediastinum,pneumothorax,
interstitialorsuLcutaneousemphysemasecondarytorupturedalveoli
neurologicalcomplications
seizures,encephalopathy J. J00,000),intracranialhemorrhage
Treatment
supportivecare
hospitalizeilparoxysmsolcoughareassociatedwithcyanosisand/orapnea, giveO
)
erythromycin40mg/ kg/ dayx J0daysstartedwithin3weeks alteronsetolcough
isolateuntil5daysoltreatment
treatmentwilldecreaseinlectivityLutnotchange course olillness
shortens period olcommunicaLility
antiLioticprophylaxis.erythromycinlorallhousehold contacts
prevention.acellularpertussisvaccine Pentacel)ininlantsandchildren,andpertussis
LoosterAdacel`)inadolescentsandadults
Varicella (Chickenpox)
varicella-zostervirus(VZV)
incuLation.J0-2Jdays,inlectivity.J-2dayspre-rashuntilvesicleshavecrustedover
transmissionrateis86,inhouseholdcontacts,viarespiratorysecretionsairLorne)and
vesicularlluid
primaryinlectionwithvirususually results inlile-longimmunity. >95, olyoungadults
withvaricellaareimmune
maternal inlectioninlirst orearly secondtrimester 2, risk)cancausecongenital
varicellasyndromelowLirthweight,CNS aLnormalities, digit / limLaLnormalities,
cutaneousscarring,eyedelects)
maternalinlection5daysLeloreto2daysalterdeliverycanleadtoseverevaricellaol
neonate
J-3dayprodrome.leverandrespiratorysymptoms
characteristicpolymorphousrash
verypruritic
cropsolredmaculeswhichquicklyLecomevesiclessurroundedLyerythema
dewdrop onerythematousLase
vesiclesLurstandlesionscrustover
ontrunk,lace,scalp,conjunctivae,vagina,oralmucosa,palmsandsoles
newcropsusuallystoplormingalter5-/days
Compli cati ons
secondary bacterial infection mostcommon)
inlectionwithstaph,CAS
presentsasimpetigo,aLscesses,cellulitis,necrotizinglasciitis,sepsis
cereLellarataxia,pneumonia,hepatitis,encephalitis
immunocompromisedpatients.varicellamayLelile-threatening
neonatesLorntomotherswho develop varicellalrom5daysLeloreto2daysalterdelivery
areconsideredhighrisk
mustadminister varicella-zosterimmunegloLulin(VZIG) andlollowlorsignsol
inlection/sepsis,considerstartingacyclovir
viruslatentinsensorygangliaand reappearsasherpeszosterin68/ J00,000individuals
incidenceisincreasedinimmunocompromisedpatients
Treatment
supportive hydration, acetaminophen, antipruritics,AVOID salicylates)
properhygiene,discouragescratching
acyclovirlorseveredisease,immunocompromisedpatients,neonates
avoidcontactwithothersuntillesionsaredryandcrustedandnonewonesareappearing
Prophylaxis and Prevention
immunization seeRoutine Immunization, P5)
VZIG lorpost-exposureinhighrisksusceptiLlepatientwithin96hoursolexposure)
Roseola
humanherpesvirus6
incuLation.5-J5days,inlectivityandspread.unknown
typically allects children<3years
highlever>39C)lasting3-5days,cough,respiratorysymptoms,nasalcongestion
pharynx,tonsilsandtympanicmemLranesareerythematous
cervical,posteriorcervicallymphadenopathy,LulginganteriorlontanellenCMSinvolvement)
leverceasesLelorerashappears
pinknon-pruriticmaculesandmaculopapules
maculescoalesceanddisappearinJ-2days
Treatment
supportiveacetaminophen)
Complications
leLrile seizures
encephalitis
Measles
morLillivirus
incuLation.J0-J4days,inlectivity.4dayspre-rash,spreadLyairLorneroute
prodrome.13 C's" cough, coryza, conjunctivitis, lever, eyelidedema
Koplikspots J-2daysLeloreandalterrash).smallwhitepapulesonredLaseonLuccal
mucosa
maculopapularrashspreadsoverlaceandhairlinespreadinginadescendinglashionover
theLodyover3days
Diagnosis
clinicalexaminationandpositiveserologylormeaslesIgM
Treatment
supportiveandsymptomaticappropriatetreatmentolsecondaryLacterialinlection)
prophylacticimmunogloLulintopreventdiseaseiladministeredwithin6daysolexposure
vitaminA supplementationinselectedchildren
Complications
secondaryLacterialinlectionlaryngotracheoLronchitis, otitis media, sinusitis),
Lronchopneumonia,croup
encephalitis J. J,000).ataxia,vomiting,seizures,coma
suLacutesclerosingpanencephalitis J. J00,000). slowmeaslesvirusinlectionolLrain
manilestingyearslater,characterizedLyprogressivecereLraldeteriorationwithmyoclonic
jerks,latalwithin6-J2months
Mumps
paramyxovirus
incuLation. J2-25days,inlectivity./dayspre-parotitisto/dayspost-parotitis, spreadLy
droplets
diagnosis.urineorsalivalorviralserology
lever,headache,parotitisLilateral,pushesearloLesupandout), myalgia,malaise
30-40,olcasesaresuLclinicalwithminimalsymptoms
Pediatrics P57
Absolute Contraindicaton
Do not use ASA i n chi l dren wi th
chi ckenpox due to the ri sk of Reye
syndrome (see Pa8j .
PS8 Pediatrics Infectious Diseases Toronto Notes 2010
Treatment
supportive
Complications
meningoencephalomyelitis. over J0,olpatientwithparotitis
orchitis,epididymitis,inlertility
pancreatitis.maysee elevatedserumamylasewithoutsymptoms
other.ocularcomplications,thyroiditis,hearingimpairment,myocarditis,arthritis,
thromLocytopenia,cereLellarataxia,glomerulonephritis
Rubella
ruLivirus
incuLation. J4-2J days
inlectivity. /dayspre-rashto5dayspost-rash, spreadLydroplets
diagnosis.serologylorruLellaIgM,maynotLedetected4-5daysalterrashonset
prodromeolnonspecilicrespiratorysymptomsandsuLoccipitaladenopathy
rash
maculopapular,initiallyonlace,thenspreadingtoentireLody
pruritic,disappearingLylourthday
congenitalruLellasyndromeCRS)
motherinlectedinlirst4monthsolpregnancyhighestrisk)
inlectioninutero,lailureolruLellavaccineis<5,andrarelyresultsinCRS
cataracts / congenitalglaucoma,congenitalheartdisease,hearing
impairmentcommon),purpura LlueLerrymullinLaLy),hepatosplenomegaly,
jaundice,microcephaly, developmentaldelay, radiolucentLonedisease
prevention.routinechildhoodimmunization,assureimmunityolwomenol
childLearingagewithvaccination
Treatment
symptomatic
Prognosis
excellent prognosisin patients withacquireddisease
irreversiLledelectsincongenitallyinlectedpatients
Complications
arthritis /arthralgia.polyarticularlingers,wrists,knees), lasts daystoweeks
encephalitis
Reye Syndrome
acutehepatic encephalopathyandnoninllammatorylattyinliltrationolliverandkidney
mitochondrialinjuryolunknownetiologyresults inreductionolhepaticmitochondrial
enzymes,diagnosisLyliverLiopsy
associatedwithaspiriningestionLychildrenwithvaricellaorinlluenzainlection
40, mortality
vomiting
hyperventilation,tachycardia,decereLrateposturing
respiratorylailure
agitated delirium, coma, death
Treatment
shouldLetailoredLasedonseverity olpresentation
IV glucosetocounteractellectsolglycogendepletion)
lluidrestriction,mannitolilcereLraledema)
prevention.avoidaspirinwithviralillness
Erythema Infectiosum
parvovirus5J9, lilthdisease
incuLation.4-J4days,inlectivity.priortoonsetolrash
initial/-J0days. llu-likeillnesswithlever
dayJ0-J/.rashappearsimmueresponse)
raisedmaculopapularlesionsoncheeksslappedcheekappearance),lorehead,
chin,circumoralsparing
warm,nontender,mayLepruritic,mayalsoappearonextensorsurlaces,trunk,neck,
Luttocks
daystoweeks.rashlades,mayreappearwithlocalirritationheat,s:mlight)
Treatment
supportive
Lloodtranslusionslorsomewithaplasticcrisis
Complications
arthritisJ0/,painandstilmessinperipheraljoints),vasculitis
inlectionduringpregnancymayleadtoletalhydrops,letalloss
aplasticcrisis.reticulocytopeniaoccurslorJ weekduringillness,unnoticedinnormal
individuals,Lutsevereanemiainpatientswithchronichemolyticanemia
Urinary Tract Infection (UTI)
Definition
urinespecimenwith>J0`colonies /mlolasingleorganism
E pidemiology and E tiology
3-5, olgirls, J/ olLoys
<Jyr.morecommoninLoys
>JyrF.M* J0. J
E. coli 80-90/),Klebsiella, Proteus especiallyLoys),S. saprophyticus, Enterococcus, and
Pseudomonas
risklactors.lemale,vesicoureteralrelluxVUR),diaLetes,immunocompromised,urinary
stasis neurogenicLladder),voidingdysl:mction,wipinglromLacktolront
Symptoms and Diagnosis
cystitis. dysuria,urgency,lrequency, suprapuLic pain,incontinence,malodorousurine
pyelonephritis. aLdominalorllankpain,lever, malaise,nausea,vomitingmaypresentas
non-specilicillnessinnewLorn)
sterilespecimenrequired.suprapuLicaspiration,transurethralcatheter,cleancatch
dipsticklornitrates,leukocytes,andLlood,urineC&Sdelinitivediagnosis)
ilsystemicallyill.C5C,electrolytes,Cr,5UM,Lloodcultures
Radiologic E valuation
U/Stoassesslorrenalgrowth,hydronephrosis,orstructuralanomaliesandvoiding
cystourethrography VCUC)toassesslorVURlorallchildren<2yrspresentingwith
leLrileUTI
dimercaptosuccinicacid DMSA)ilVCUCaLnormalorhistoryolpyelonephritistoassess
lorrenalscarring
nuclearstudiestolollowVURorassessluction
Treatment
encouragelluidintakepromotesurinaryllow)
:mcomplicatedUTI.oralcephalexin,orceliximex/d
complicatedUTI acutely ill,<2-3months, vomiting,immunocompromised).admitlor
hydration,IVampicillinandaminoglycoside
prophylaxis. TMP-SMXlorallkidswithrellux,awaitinginvestigationsand/or>3UTIs/yr,
Trimethoprimaloneil<2mo
lollow-up
ilnoclinicalresponsewithin48hrre-cultureurine
Complications
longtermmorLidity. localrenal scarringmayleadtohypertensionand end-stage renal
disease
Pediatrics P59
- '
+F
Bagged speci men not useful for rul i ng
i n UTI ( hi gh fal se + >85%) but useful
for rul ing out UTI ( hi gh sensi ti vi ty).
- '
Sensitivity and Specificity of Urine

Dip in Children
Leukocytes
Ni trates
Both
Sensitivity
62%
50%
46%
Specificity
70%
92%
94%
'Wlter, Bal. The urne dipstick test usef to rle
out infections. A metaanalysis of the accuracy.
BMC Ural. 2004; 4: 4.
Prophylaxis Afer First Febrle Urnar Tract
Infection in Children? A Multicenter, Randomized
Controlled, Noninferority Trial
Pcales 2008;122:1 0641 071
Study: Randomized, controlled, openlabel, 2 armed,
noninferiority trial .
Patients: 338 patients aged 2 mo to <7 years who
had a Irst episode mfebrle UTI .
Intelenon: No prophylaxis with prophylaxis
Outome: Recurrence rate of febrle UTI and rate of
renal scarrng.
Results: No sigrilcant difference in recurrence rate
or on the rate of renal scarring between the
prophylaxis and no prophylaxis group.
P60 Pediatrics Neonatolog Toronto Notes 2010
Neonatology
Normal Baby at Term
RR. 40-60Lreaths / min
HR.J20-J60Leats / minwhenawake
s5P. 50-80 mmHg,d5P. 30-40 mmHg
weight. 2,500-4,500
glucose. >2.6 mmol / L45mg/ dL)
Gestational Age (GA) and Size
~~~~~~~~~~~~~
Definitions
classilicationLygestationalageCA)
pre-term.<3/weeks
term.3/-42weeks
post-term.>42weeks
classilicationLyLirthweight
smalllorgestationalage SCA). 2SD <meanweightlorCAor<3'percentile
appropriatelorgestationalage ACA). within2SDolmeanweightlorCA
largelorgestationalage LCA).2SD>meanweightlorCAor>9/''percentile
CAcanLedeterminedalterLirthusingDuLowitz/ 5allardscores.
assessmentatdeliveryolphysicalmaturitye.g.plantarcreases,lanugo,ear
maturation)andneuromuscular-maturitye.g.posture,armrecoil)translatesintoa
score lrom-J0to50
higherscoremeansgreatermaturityincreasedCA)
-J0* 20weeks,50* 44weeks
ideal- 35-40whichcorrespondstoCA38-40weeks
onlyaccurate2weeks
Tabl e 32. Abnormalities of Gestational Age and Size
Features
Pre-term Infant
<37 weeks
Post-term Infants
>42 weeks
Wizened looking, leather ski n
Meconi um stai ni ng
SGA Infant
< 3'd percenti l e
Asymmetric (headspari ng) :
late onset, growth arrest
Symmetric: early onset,
lower growth
LGA Infant
> 97th percenti l e
Causes
Most common cause unknown
Maternal disease e.g. preecl ampsi a
Drugs/EtOH, smoki ng
Chromosomal
Mul ti pl e pregnancy
Pl acental i nsuffi ci ency
Extri nsi c causes: pl acental
i nsufficiency, poor nutri ti on,
hypertensi on, mUl ti pl e pregnancies,
drugs, EtOH, smoki ng
Intri nsi c causes: potenti al infections
(TORCH) , congenital abnormal i ti es,
syndromal, i di opathi c
Maternal diabetes
Racial or fami l i al factors
Certai n syndromes
Routine Neonatal Care
Perlormedindeliverysuite
Problems
Respi rator di stress syndrome, apnea of prematuity,
chroni c l ung disease, bronchopul monar dyspl asi a
Feedi ng di ffi culties, necrotizing enterocol i ti s ( NEC)
Hypocalcemi a, hypoglycemi a, hypothermia
Anemia, jaundi ce
Reti nopathy of prematurity
Intracrani aVintraventri cul ar hemorrhage
Patent ductus arteriosus ( PDA)
Hypoxia, meconi um aspi rati on
Hypoglycemi a
Perinatal hypoxia
Hypoglycemi a, hypocal cemi a, hypothermia
Hyperiscosity ( polycythemi a), jaundice
Hypomotility
Patent ductus arteriosus ( PDA)
Bi rth trauma, peri natal hypoxi a, meconi um aspi rati on,
respirator distress syndrome, transi ent tachypnea of
newborn (TIN), persistent pul monary hypertensi on
( PPHN)
Jaundi ce, hypoglycemi a, hypocalcemi a, polycythemi a
J. erythromycinointment appliedtoLotheyeslorophthalmianeonatorumgonorrhea,
chlamydia)prophylaxis
2. vitaminKIM toavoidhemorrhagicdiseaseolnewLorn
3. screeningtests
variesacrossCanadaandUnitedStatesseeMMSCRCweLsites)
newtandemmassspectrometryMS/ MS)candetect25inLornerrorsolmetaLolism
IEM)inasingleprocesslromheelprick
J00, sensitivityand83-99,specilicitydependingonIEM
Toronto Notes 2010 Neonatolog
inOntario,newLornscreeningtests lor
endocrinedisorderscongenitaladrenalhyperplasia,congenitalhypothyroidism)
cysticliLrosis
hemogloLinopathiesHLSS,HLSc,s5thal)
inLornerrorsolmetaLolism22intotal)
3categories.lattyacidoxidationdelects,aminoacidopathies,organicacid
delects
others.Liotinidasedeliciencyandgalactosemia
4. ilmotherKnegative.sendcordLloodlorLloodgroup,anddirectantigloLulintest
5.ilindicated.C6PDdeliciencytesting
6.ilmotherhepatitis5surlaceantigenpositive.H5Igandstarthepatitis5vaccineseries
Approach to the Depressed Newborn
adepressednewLornlacksoneormoreolthelollowingcharacteristicsloranormal
newLorn
pulse >J00Lpm
crieswhenstimulated
activelymovesallextremities
hasagoodstrongcry
Letween5-J0,olnewLornLaLiesrequireassistancewithLreathingalterdelivery
Tabl e 33. Eti ol ogy of Respi ratory Depression i n the Newborn
Etiology
Respirator problems
Anemia (severe)
Maternal causes
Congenial malformationsirh injur
Shoc!CyanosisCongenital hear disease
Other
Diagnosis
vitalsigns
takeagood maternalhistory
Examples
Respi rator Di stress Syndrome/Hyal i ne Membrane Disease
eNS depression
Meconi um aspi rati on
Pneumoni a
Pneumothorax
Erythroblastosis fetal i s
Secondar hydrops fetal i s
Drugsanesthesia
Diabetes mel l i tus
Pregnancyi nduced hypertensi on
Nuchal cord
Hypothermia
Hypoglycemi a
Infection
includeprenatalcare,illnesses,useoldrugs,laLour,previoushighriskpregnancies,
inlectionsduringpregnancy,currentinlections,durationolrupturedmemLranes,
LloodtypeandK status,amnioticlluidstatus, gestationalage,meconium,Apgar
scores
clinicallindingsoLservelorsignsolrespiratorydistress.cyanosis,tachypnea,retractions,
andgrunting)
laLoratoryresults C5C, A5C, pH,Lloodtype)
transilluminationorchestx-rayilsuspectingpneumothoraxordiaphragmatichernia)
Management
A5Cs
suctionilmeconiumpresentandinlantisdepressed
applytactilestimulationdonot'spank )
provideair/oxygenandassistedventilationilapneicorHR<J00Lpm
monitoroxygensaturationandheartrateilHR<60Lpm,startchestcompressions )
treattheunderlyingcause
counselandprovideexplanationandsupporttolamily
Pediatrics P61
P62 Pediatrics
R
Apgar Score
"How Ready I s Thi s Chi l d?"
See Table 34
'
Chronic Non-bacterial Perinatl
Infections
TORCHES
Toxopl asmosi s
Others
Rubel l a vi rus
Cytomegal ovi rus/Chi ckenpox
Coxsackievi rus
Herpes si mpl ex vi rus, HI V, Hepati ti s 8
Erythema infecti osum (parvovi rus 81 9)/
Epstei n-8arr vi rus
Syphi l i s
Neonatolog Toronto Notes 2010
Neonatal Resuscitation
assessApgarsatJ and5minutes
il</at5minutes thenreassessq5min,until>/
donot waittoassignApgarscoreLeloreinitiatingresuscitation
Tabl e 34, Apgar Score
Sign 0 2
Heart Rate Absent < l Oa/min > l Oa/mi n
Respi rator Effort Absent Slow, irregul ar Good, cri ng
Irri tabi l i ty No response Gri mace Cough/cr
Tone Li mp Some flexion of exremities Active motion
Col our Bl ue, pal e Body pi nk, extremities bl ue Compl etel y pi nk
(acnocyanosi s)
Initial Resuscitation
anticipation know maternalhistory, historyolpregnancy,laLour,anddelivery, prepare
equipment
steps totakelorallinlantsLeloreA5Cs)
providewarmth.warm radiantheater, warmtowels),drythenewLorn removewet
towels)
positionandclearairway.snilling position
stimulateinlantilneeded).ruLLackgentlyorllicksolesolleetEXCEPTil
meconium present inwhichcase suctionFIRST)
assessLreathing,heartrateandcolour
Airway
ilmeconiumispresentand
LaLyisvigorous strongrespiratoryellort, good muscletone,HR>J00). suction
mouthandnosealterdeliveryolhead
LaLyisnotvigorous.lreellowO,intuLateandsuctiontrachea
ilnomeconiumispresent,removesecretionsLywipingmouthandnosewith
towelorgentlesuctionolmouththennose
Breathing
ilHR<J00orapneic, applypositivepressureventilationPPV)
PPVatrateol40-60/min,J00,OwithenougpressuretoseevisiLlechestexpansion
Circulation
ilHR<60alter30secolellectiveventilation,startchestcompressions60orless,
compress)
chestcompressionsatlowerJ / 3 olthesternumatJ / 3 oltheAPdepthatarateolJ20
events /min3compressions.J ventilation- 90compressions /min.30Lreaths/min)
Tabl e 35. Drugs Used in Neonatal Resuscitation
Drug Name
epi nephri ne
(adrenal i n)
naloxone
(Narcan'M )
fl ui d bol us
( NS, whol e blood,
Ri nger's lactate)
Schedule
0, 1 0, 3 mVkgdose of 1 : 1 0,000
sol uti on IV 0, 3 - 1 ,0 ml
i ntratracheal q35 mi n prn
0. 1 mglkg of a 0.4 mglmL
sol uti on , " 0. 25 mUkgldose)
IVIM/C
1 0 mUkg/dose over 5 1 0 mi n
Indications
HR < 60 and not ri si ng
Newborn wi th respirator
depression and maternal
narcotic use 4 hours before
del iver
Evidence of hypovolemi a
Comment
Side effects: tachycardia, hypertensi on,
cardiac arrhythmi as
Do not use for chroni c opi ate exposure may
cause withdrawal symptoms i ncl udi ng
hypertensi on, irritabi l i ty, poor feedi ng
Action of narcotic outlasts action of nal oxone
Sepsis in the Neonate
Tabl e 36. Sepsis Considerations in the Neonate
Eary Onset (0- days)
Verti cal transmi ssi on, 95% present wi thi n 24 hr
Ri sk factors:
Maternal infecti on: UTI, GBS positive, previous chi l d wi th
GBS sepsi s or meni ngi ti s
Maternal fever/leukocytosischori oamni oni ti s
Prolonged rupture of membranes (> 1 8 hrs)
Preterm l abour
Pathogens: GBS, L coli, listra
Late Onset (5-28 days)
Acqui red after bi rth
Most common i n preterm i nfants i n NICU (due to coagulase
negative staph)
Al so i n healthy, ful l term
Same pathogens pl us:
Strptococcus, Meningococcus, Stphylococcus
Signs of Sepsis
no reliaLle aLsolute indicatoroloccultLacteremiaininlants<3months,mostconsistent
resulthasLeenW5C<5
temperatureinstaLilityhypo/ hyperthermia)
respiratorydistress,cyanosis,apnea
tachycardia/ Lradycardia
lethargirritaLility
poorleeding,vomiting,aLdominaldistention,diarrhea
hypotonia,seizures,conlusion,lethargcoma
jaundice,hepatomegaly, petechiae,purpura
Cyanosis
Approach to Neonatal Cyanosis
2majortypes
peripheral cyanosis
canLenormaltransientlyLutmayindicate sepsis, temperatureinstaLility
central cyanosis
2majorcategories
deoxygenatedhemogloLin
respiratory
upperchoanalatresia,macroglossia,airwayhypoplasia,laryngeal
weL/cyst,loreignLody)
lowerpneumonia,meconiumaspirationsyndromeMAS),
pneumothorax,diaphragmatichernia,AV listula,pulmonary
hypoplasia)
cardiovascularcongenitalheartdisease,PPHM)
neurologic
CMSasphyxia,hemorrhage,seizure,narcotics / sedatives)
neuromuscularmyastheniagravis,Lotulism)
hematologicpolycythemia)
sepsis
aLnormalhemogloLinmethemogloLinemia,carLoxyhemogloLinemia)
note: whilecarLoxyhemogloLinemiasecondarytocarLonmonoxidepoisoning)resultsin
impairedLindingoloxygentohemogloLin,itdoesnotdiscolourtheLloodandmay
therelorenotregisteronpulse-oximetryandcyanosismaynotLeevidentclinically. In
addition, withmethemoLloLinemiapulseoximetrytypically readshigherthanthetrue
leveloloxygemogloLin. Thisisduetothelactthat methemogloLin alterstheaLsorptionol
redlightatthetwowavelengthsthatpulseoximetryusesto predictoxygensaturation.
Management
A5Cs
elevatedCOsuggestsrespiratorycause
hyperoxictesttoruleoutCH).getLaselinepO
inroomair,thenpO
onJ00, O
xJ0-J5min
pO<J50mmHg.suggestscongenitalheartdiseaseseePediatric Cardiolog, PJ8)
pO>J50mmHg.suggestsrespiratoryairway, chest,lungs),LrainorLloodproLlems
CXR- looklorrespiratoryaLnormalitiesrespiratorytractmallormations,evidenceol
shunting,pulmonaryinliltrates)andcardiacaLnormalitiescardiomegaly, aLnormalitiesol
thegreatvessels).
Persistent Pulmonary Hypertension of the
Newborn (PPHN)
incidenceJ. 9/ J000liveLirths
presentwithinJ2hoursolLirthwithseverehypoxemia/cyanosisLutrelativelymild
respiratorydistress
Pathophysiology
duetopersistenceolletalcirculationasa result olpersistentelevationolpulmonary
vascularresistance
RtoLshuntthroughPDA,loramenovale,intrapulmonarychannels decreased
pulmonaryLloodllowandhypoxemia lurtherpulmonaryvasoconstriction
Risk Factors
asphyxia, MAS,RDS, sepsis, structuralaLnormalitiese.g.diaphragmatichernia,
pulmonaryhypoplasia) secondary PPHM)
primaryPPHMoccursinaLsenceolrisklactors
Pediatrics P63
Investigations
measure pre- andpost-ductaloxygenlevels
echocardiogramrevealsincreasedpulmonaryarterypressureandaR Lshunt,also
usedtoruleoutothercardiacdelects
Treatment
maintaingood oxygenationSaO>95,) inatriskinlants
Ogivenearlyandtaperedslowly, minimize stress andhypoxia, alkalinization,inotropes
tomakesystemicpressuregreaterthanpulmonarypressure)
mechanicalventilation,highlrequencyoscillationHFO)
nitricoxide
extracorporealmemLraneoxygenationECMO)usedinsomecentres
Apnea
periodicLreathingisanormal respiratorypatternseeninnewLorns duringsleep inwhich
periodsolrapidrespirationarealternatedwithapneicepisodeslasting5-J0seconds
apnea.aLsenceolrespiratorygasllowlormorethanJ5-20secondsorlessilassociated
withLradycardiaorcyanosis) 3types
central.nochestwallmovement
oLstructive.chestwallmovementcontinues
mixed. comLinationolcentraland oLstructive apnea
interminlants,apneaalwaysrequireslullwork-up
apnea<24hrs stronglyassociatedwithsepsis
apnea>24hrs
CMS
apneaolprematurity.comLinationolCMSprematurityandoLstructiveapnea,
resolvesLy36weeksCA,diagnosisolexclusion
seizures
intracranialhemorrhageICH)
hypoxicinjury
inlectious.sepsis,meningitis,necrotizingenterocolitisMEC)
CI. gastroesophagealrelluxdiseaseCERD),aspirationwithleeding
metaLolic.hypoglycemia, hyponatremia,hypocalcemia
cardiovascular.lowandhighLloodpressure,anemia,hypovolemia,PDA,heartlailure
drugs. morphine
Management
O, continuouspositiveairwaypressureCPAP), mechanicalventilation
tactilestimulation
correctunderlyingcause
medications methylxanthinescalleine)stimulatetheCMSanddiaphragmandareused
lorapneaolprematuritynotinterminlants)
Respiratory Distress in the Newborn
tachypnea.RR>60/min,tachycardia.HR>J60/min
grunting,intercostalindrawing, nasalllaring
duskiness,centralcyanosis
decreasedairentry,cracklesonauscultation
Diferential Diagnosis of Respiratory Distress
pulmonary
respiratorydistresssyndromeRDS)
transienttachypneaolthenewLornTTM)
meconiumaspirationsyndromeMAS)
pleuralellusions,pneumothorax
congenitallungmallormations
inlectious
sepsis
pneumoniaC5S+others)
cardiac
congenitalheartdiseasecyanotic,acyanotic)
persistentpulmonaryhypertensionolthenewLorn PPHM)
hematologic
Lloodloss
polycythemia
anatomic
tracheoesophageallistula
congenital diaphragmatichernia
upperairwayoLstructionseeOtolar_n_olo_)
choanalatresia
Pierre-RoLinsequenceretrognathiaand/ormicrognathiapluscleltpalate,and
glossoptosis)
laryngealmalacia)
trachealmalacia,vascularring)
mucousplug
cleltpalate
metaLolic
hypoglycemia
inLornerrorsolmetaLolismaminoacidemia,organicacidemia,ureacycle
disturLance,galactosemia, Jlacticacidosis)
neurologic
CMSdamagetrauma,hemorrhage)
drugwithdrawalsyndromes
Investigations
CXR,A5CorcapillaryLloodgas,C5C,Lloodglucose
Lloodcultures
ECHO,ECCilindicated
Respiratory Distress Syndrome (RDS)
alsoknownashyalinememLranedisease
Pathophysiology
surlactantdeliciency poorlungcomplianceduetohighalveolarsurlace tensionand
atelectasis decreasedsurlace arealorgasexchange hypoxia+acidosis
respiratorydistress
surlactantdecreasesalveolarsurlacetension,improveslungcomplianceandmaintains
lunctionalresidualcapacity
thereisusuallysullicientsurlactantproductionLy36weeks
Risk Factors
prematurity
lowLirthweight
rareatterm
maternaldiaLetes.insulininhiLitsthe cortisolsurgenecessarylorsurlactantsynthesis
C-sectionwithoutlaLour
asphyxia,acidosis,sepsis, meconiumaspiration
males >lemales
hypothermia
secondLorntwin
signsolrespiratorydistress tachypnea, tachycardia,grunting,intercostalindrawing,nasal
llaring,cyanosis,lungcrackles)
onsetwithinlirstlewhoursollile,worsensovernext24-/2hours
inlantsmaydeveloprespiratorylailureandrequireventilation
CXR.decreasedaerationandlungvolumes,reticulonodularpatternthroughoutlunglields
withairLronchograms,atelectasis,mayresemLlepneumonia,ilseverecanseewhite-out
Prevention
steroidtherapy e.g.Celestone)lormothersJ2mgq24hx2doses)whoareatriskol
pretermLirth
monitorlecithin.sphingomyelinL/S)ratiowithamniocentesis,L/S>2.J indicateslung
maturity
prophylacticsurlactantoltengiventohigriskinlants 28weeks)atLirth
Treatment
supportive
OassistedventilationCPAPorintuLationandmechanicalventilation )
administerlluids cautiously toavoid pulmonary edema
endotrachealsurlactantadministration
Prognosis
insevere prematurityand/orprolongedventilation, increasedriskolLronchopulmonary
dysplasia5PD)/chroniclungdisease
Complications
Lronchopulmonarydysplasia
pulmonaryairleakspneumothorax)
Pediatrics P65
-'
P
RDS i s the most common cause of
respi ratory di stress in the preterm
i nfant.
-'
"Ground gl ass" appearance of l ungs is

pathognomoni c of RDS.
Transient Tachypnea of the Newborn (TTN)
alsoknownas wetlugsyndromeandrespiratorydistress syndrometypeII

Pathophysiology
delayedresorptionolletallunglluid accumulationollluidinperiLronchiallymphatics
andvascularspaces tachypnea
Risk Factors
lulltermornear-terminlant
no laLour/shortlaLour hypothesizedlack olcatecholaminerelease)
C-sectionlungsarenotcompressedduringpassagethroughpelviclloor)
diaLeticmother/gestationalweight>4500g
maternalasthma
malesex
tachypneawithinthelirstlewhours ollile,mildretractions,grunting,nasalllaring,
withoutsignsolsevere respiratorydistress
usuallyresolvesin24-/2hours
CXR.lluidinlissures,increasedvascularityslightcardiomegaly
Treatment
supportive.O
,carelullluidadministration,mayuseCPAP
Prognosis
lullrecoveryexpectedwithin2-5days
inthepastitwasgenerallyLelievedthatTTMwasasell-limitingconditionwhich,once
resolved,hadnolong-termsequelae.CurrentresearchsuggeststhatchildrenwithTTM
mayLeatincreasedriskoldevelopingwheezingsyndromessuchasasthma)inchildhood
Meconium Aspiration Syndrome (MAS)
J0-J5,olallimantsaremeconiumstainedatLirth,-5,olmeconiumstainedimantsgetMAS
usuallyassociatedwithletaldistressinutero,orpost-terminlant
respiratorydistresswithinhoursolLirth
smallairwayoLstruction,chemicalpneumonitis tachypnea,LarrelchestwithaudiLle
crackles
CXR.hyperinllation,streakyatelectasis,patchyandcoarseinliltrates
J0-20,havepneumothorax
Complications
hypoxemia,hypercapnea,acidosis,PPHM,pneumothorax,pneumomediastinum,
pneumonia,sepsis,respiratorylailure, death
Treatment
supportivecare,assistedventilationimportanttomaintainadequateoxygenation)
ventilatedinlantsoltenrequiresedation
mayLenelitlromsurlactantreplacementsurlactantlunctionisinhiLitedLypresenceol
meconium)
inhalednitricoxide,extracorporealmemLraneoxygenationatsomecentres
Prevention
inutero. carelulmonitoring
alterdeliveryolthehead. suctionoro/nasopharynx
atLirth. intuLateandsuctionLelowcordsilinlantisdepressed
note.presenceolmeconiumstainingaloneisMOTanindicationlortrachealsuctioning
Iltheinlantisvigorous, intuLationandsuctioningollower airwayisunncessary
Pneumonia
seePediatric Respirolog, P89
considerininlantswithprolongedorprematureruptureolmemLranesPROM),maternal
lever,orilmotherC5Spositive
suspectilinlantexhiLitstemperatureinstaLility,W5Cloworlelt-shilted
symptomsmayLenon-specilic
CXR.hazylungdistinctinliltratesmayLedilliculttodillerentiatelromRDS)
Diaphragmatic Hernia
ilresuscitationrequiredatLirth,DOMOTmask LagLecause airwillenterstomachand
lurther compresslungs,inlant requiresendotrachealintuLation
respiratorydistress,cyanosis
scaphoidaLdomenandLarrel-shapedchest
allectedsidedulltopercussionandLreathsoundsaLsent,mayhearLowelsoundsinstead
heartsoundsshiltedtocontralateralside
asymmetricchestmovements,tracheadeviatedawaylromallectedside
resultantpulmonaryhypoplasiaonallectedandcontralateralside
maypresentoutsideolneonatalperiod
oltenassociatedwithotheranomaliescardiovascular,CMSlesions)
CXR.portionolCItractinthoraxusuallyleltside),displacedmediastinum
Treatment
surgery
Chronic Lung Disease (CLD)
alsoknownas5PD Lronchopulmonarydysplasia)
mostlrequentlyassociatedwithverypretermLirth
maydevelopalterprolongedintuLation/ ventilationwithhighpressuresandhighO
concentrationoltenalter ventilationlorRDS)
delinedasO
requirement at28days/36wksCAandaLnormalCXRlindingslung
o
acilication,thencystswithsitesoloverdistentionandatelectasis, appearsspongy)
cronicrespiratorylailuremayleadtopulmonaryhypertension, poorgrowth,and
right-sidedheartlailure
Treatment
no goodtreatments
gradualweanlromventilator, optimizenutrition
dexamethasonemayhelpdecreaseinllammationandencourageweaning,howeveruseol
dexamethsoneisassociatedwithincreasedriskoladverseneurodevelopmentaloutcomeso
indicationslorusearelimited
Prognosis
patients with5PDcontinuetohavesignilicantimpairmentanddeteriorationinlung
lunctionlateintoadolescence
studiesshowaninverserelationahipLetweenFEVatschoolageanddurationol
supplementaloxygen
somelungaLnormalitiesmaypersistintoadulthoodincluding.airwayoLstruction,airway
hyperreactivity, andemphysema
associatedwithincreasedriskoladverseneurodevopmentaloutcome
Hypoglycemia
glucose <2.6mmol / L40mg/ dL)
E tiology
decreasedcarLohydratestores premature,IUCR)
inlantoladiaLeticmother IDM). maternalhyperglycemia letalhyperglycemiaand
hyperinsulinism hypoglycemiainthenewLorninlantLecauseolhighinsulinlevels
sepsis
endocrine.hyperinsulinismduetoisletcellhyperplasiae.g.5eckwith-Wiedemann
syndrome), panhypopituitarism
inLornerrorsolmetaLolism. lattyacidoxidationdelects,galactosemia
Clinical Findings
signsoltennon-specilic and suLtle. lethargy, poor leeding, irritaLility tremors, apnea,
cyanosis,seizures
Management
identilyandmonitorinlantsatrisk, pre-leedLloodglucosechecks)
LeginoralleedswithinlirstlewhoursolLirth
ilhypoglycemic,provideglucoseIVDJ0,DJ2. 5)
ilpersistenthypoglycemia past day3),hypoglycemiaunresponsivetoIVglucose, and/or
no predisposingcauselorhypoglycemia,sendthelollowingcriticalLloodworkduring
anepisodeolhypoglycemia
insulin
cortisol
growthhormoneCH)
Leta-hydroxyLutyrate
lactate
Pediatrics P67
P68 Pediatrics
.
*P
Jaundi ce i s very common 60% of
term newborns devel op vi si bl e
j aundi ce.
. `
*F
Jaundi ce i n the fi rst 24 hours i s always
pathologi cal .
Neonatolog Toronto Notes 2010
ammonia
lreelattyacidsFFAs)
A5C
treathyperinsulinismwithglucagonanddiazoxide
Jaundice
jaundice visiLleatserumLiliruLinlevelsol85-J20umol / L5-6mg/dL)
lookatsclera, mucousmemLranes,palmarcreases,tipolnose,lrenulum
jaundice more severe/prolongedduetoincreasedretentionolLiliruLininthecirculation)
with.
prematurity
acidosis
hypoalbuminemia
dehydration
note.scleralicterusyellowdiscolourationolthewhiteoltheeyes)iscausedLyLiliruLin
inthescleralconjunctivanottheslcera itsell)
UNCONJUGATED
Pathol ogi c -I Ogi C
Hem!lytic Non-Hemolytic

hematoma ( cephal ohematoma)
pol ycythemi a
Intrinsic Extri nsi c sepsi s
membrane i mmune hypothyroi di sm
spherocytosi s ABO i ncompatabi l ity Gi l bert syndrome
el l i ptocytosi s Rh i ncompatabi l ity Cri gl er- Najjar
enzyme Kel l y, Duffy, etc.
G6PD defici ency non-i mmune
PK defi ci ency spl enomegal y
hemogl obi n sepsi s
O thal assemi a AV mal formati on
Fi gure 4. Approach to Neonatal Hyperbi l i rubi nema
PHYSIOLOGIC JAUNDICE
E pidemiology
CONJUGATED
Hepatic Post-Hepatic
infecti ous bi l iary atresia
sepsi s chol edochal
hep B, TORCH cyst
metabol i c
gal actosemi a
tyrosi nemi a
a-l -anti trypsi n defi ci ency
hypothyroi di sm
CF
drugs
TP
i di opathi c neonatal hepati ti s
terminlants.onset2-3daysollile,resolutionLy/daysollile
prematureinlants.higherpeakandlongerduration
Pathophysiology
increasedhematocritanddecreasedR5Clilespan
immatureglucuronyltransleraseenzymesystemslowconjugationolLiliruLin)
increasedenterohepaticcirculation
Tabl e 37. Risk Factors
Materal Factors Perinatal Factors
Ethni c group (e. g. Asi an, native Ameri can)
Compl i cati ons duri ng pregnancy ( i nfant of
di abetic mother, Rh or ABO i ncompati bi l ity)
Bi rth trauma (cephalohematoma,
ecchymoses)
Prematurity
Breast feedi ng
Tabl e 38. Causes of Neonatal Jaundi ce by Age
<24 hours
ALWAYS PATHOLOGI C
Hemolytic
Rh or ABO
i ncompati bi l ity
Sepsi s
GBS
Congeni tal infection
(TORCH)
24-72 hour
Physi ol ogi c, polycythemi a
Dehydration
(breast feedi ng jaundi ce)
Hemol ysi s
G6PD deficiency
Pyruvate ki nase
deficiency
Spherocytosi s
Brui si ng, hemorrhage,
hematoma
Sepsi s/congenital infection
Breast Feeding J aundice
common
72-96 hours
Physi ol ogi c Z breast
feedi ng jaundice
Sepsi s
Neonatal Factors
Infection
Genetic factors
Polycythemi a
Drugs
Difficulty establ i shi ng breastfeedi ng
Prolonged (>1 week)
Breast mi l k jaundice
Prolonged physiologic jaundice i n preterm
Hypothyroi di sm
Neonatal hepatitis
Conjugati on dysfunction
e.g. Gi lbert syndrome, Crigler-Nalar
syndrome
Inborn errors of metabol i sm
e. g. galactosemia
Bi l i ar tract obstruction
e.g. bi l i ary atresia
duetolackolmilkproductionandsuLsequentdehydration,leadingtoexaggerated
physiologicjaundice
Toronto Notes 2010
Breast Milk J aundice
rareJin200Lreast-ledinlants)
inhiLitorolglucuronyl translerase loundinLreastmilk
onset/daysollile, peakat2ndto3rdweekollile
PATHOLOGIC JAUNDICE
mustLeinvestigatedil.
jaundiceat<24hoursolage
Neonatolog
serumunconjugatedLiliruLinrisesrapidlyorisexcessivelorpatientsageand
weigt >85 (t mol / Lperdayor>220(| mol / LLelore4daysolage)
conjugatedLiliruLin>35 (t mol / L2.0mg/dL)
persistentjaundicelastingLeyondJ-2weeksolage
investigations
unconugatedhyperLiliruLinemia.
emolyticwork-up.C5C,Lloodgroup motherandinlant),peripheralLlood
smear,CoomLstest,LiliruLinconjugated,unconjugated)
ilLaLyisunwellorhaslever,septicwork-up.C5Cdillerential,Lloodand
urineculturesLP, CXR
other.TSH,C6PDscreeninmales)
conjugatedhyperLiliruLinemia.considerliverenzymesAST,ALT),coagulation
studiesPT,PTT),serumalLumin,ammonia,TSH,TORCHscreen, septicwork-up,
galactosemiascreenerythrocytegalactose-J-phosphateuridyltransleraselevels),
metaLolicscreen,aLdominalU/S,HIDAscan,sweatchloride
TREATMENT OF UNCONJUGATED HYPE RBILIRUBINEMIA
topreventkernicterus seeLelow)
LreastleedingdoesnotneedtoLediscontinued,ensureadequateleedsandhydration
getlactationconsultantsupport,mothertopumpalterleeds
treatunderlyingcauses e.g. sepsis)
phototherapy
insoluLleunconjugatedLiliruLinisconvertedtoexcretaLlelormviaphotoisomerization
serumLiliruLinshouldLemonitoredduringandimmediatelyaltertherapyriskol
reLoundLecausephotoisomerizationreversiLlewhenphototherapydiscontinued)
contraindicatedinconjugatedhyperLiliruLinemia.resultsinLronzedLaLy
sideellects.hypernatremicdehydration,eyedamage,skinrash,diarrhea
usepuLlishedguidelineslorinitiationolphototherapy
exchangetranslusion
preventstoxicellectsolLiliruLinLyremovallromLody
indications. dependingonlevelandrateolriseolLiliruLin
sideellects.inlections,translusionreactions
mostcommonlyperlormedlorhemolyticdiseaseandC6PD
KE RNICTE RUS
E tiology
unconjugatedLiliruLinconcentrationsexceedalLuminLindingcapacityandLiliruLinenters
andisdepositedintheLrainresultinginpermanentdamageoltenLasalgangliaorLrainstem)
incidenceincreasesasserumLiliruLinlevelsincreaseaLove340(| mol / LJ9. 8mg/dL)
canoccuratlowerlevelsinpresenceolsepsis,meningitis,hemolysis,hypoxia,
hypothermia,hypoglycemiaandprematurity
upto J5,olinlantshavenooLviousneurologicsymptoms
acutelorm
lirstJ-2days.lethargy, hypotonia,poorleeding,high-pitchedcry,emesis,seizures
middleollirstweek.hypertonia,opisthotonicposturing,lever,Lulginglontanelle,
pulmonaryhemorrhage
chroniclormlirstyearandLeyond)
hypotonia,delayedmotorskills,extrapyramidalaLnormalitieschoreoathetoid
cereLralpalsy),gazepalsy, MR,sensorineuralhearingloss
Prevention
exchangetranslusion
Complications
sensorineuraldealness, choreoathetoidcereLralpalsy CP), gazepalsy, mentalretardation
BILIARY ATRE SIA
atresiaoltheextrahepaticLileducts
cholestasisandincreasedconjugatedLiliruLinalterthelirstweekollile
incidence.J / J0,000-J5,000liveLirths
darkurine,palestool,jaundicepersistinglor>2weeks), aLdominaldistention,hepatomegaly
Diagnosis
HIDAscan
liverLiopsy
Pediatrics P69
- '
+
"Bronzed" Baby in Infants with
Conjugated Hyperbilirubinemia
Phototherapy results i n the producti on
and accumul ati on of a toxi c metabol ite
whi ch al so i mparts a bronze hue on the
baby's ski n.
Treatment
surgicaldrainageprocedure
hepatoportoenterostomyKasaiproceduremostsuccesslulilLelore8weeksolage)
usuallyultimatelyrequireslivertransplantation
VitaminsA,D,E,andKdietshouldLeenrichedwithmedium-chaintriglyceridesto
ensureadequatelatingestion
Bleeding Disorders in Neonates
oozinglromtheumLilical stump, excessiveLleedinglromperipheralvenipuncture /heel
sticksites / IVsites,largecaputsuccedaneum,cephalohematomasinaLsenceolsignilicant
Lirth trauma), and prolonged Lleeding lollowingcircumcision.
Approach to Bleeding Disorders in Neonates
4 major categories
J.increased platelet destruction
maternal ITPSLE
neonatalalloimmunethromLocytopenia MAIT)
inlection
DIC
drugs
extensivelocalizedthromLosis
2. decreased platelet production/function:
Lonemarrowreplacement
pancytopenia
Fanconianemia
trisomyJ3andJ8
3. metabolic:
congenitalthyrotoxicosis
inLornerrorolmetaLolism
4. coagulation factor deficiencies/presence of inhibitors seeHematolo_)
haemophiliaA
haemophilia5
hemorrhagicdiseaseolthenewLorn
NEONATAL ALLOIMMUNE THROMBOCYTOPENIA(NAIT)
Pathophysiology
plateletequivalentolK diseaseolthenewLorn
occurswhenmotherisnegativelorhumanplateletantigenHPA)andletusispositive
developmentolmaternalIgCantiLodiesagainstHPAantigensonletalplatelets
E pidemiology
J /4000-5000liveLirths
Clinical Features
clinicalleatures.petechiae,purpura, thromLocytopeniainotherwisehealthyneonate
severe MAITcanleadto intracranialLleeding
Diagnosis
matenalandpaternal platelettypingandidentilicationolplateletalloantiLodies
Treatment
IVICtomotherprenatally, starts insecondtrimester,treatneonatewithIVIC
andtranslusionolinlantwithwashedmaternalplateletsordonorHPAnegativeplatelets
AUTOIMMUNE THROMBOCYTOPENIA
Pathophy siology
causedyantiplateletantiLodieslrommaternalITPorSLE
passivetranslerolantiLodies acrossplacenta
similar presentationto MAITLutLleedingusuallylesssevere
Treatment
steroidstomotherxJ0-J4da
)
spriortodelivery,orIVICto motherLelore
deliveryortoinlantalterdelvery
caution.translusionolinlantwithmaternalorrandomdonorplateletswillresultin
destructionolplatelets
HEMORRHAGIC DISEASE OF THE NEWBORN
causedLy vitaminKdeliciency
lactorsII,VII,IX,XarevitaminK-dependent,thereloreLothPTandPTTareaLnormal
E tiology and Clinical Presentation
neonates atriskolvitaminKdeliciencyLecause.
vitaminKpoorlytranslerredacrosstheplacenta
insullicientLacterialcolonizationolcolonatLirthsynthesizevitK)
dietaryintakeolvitaminKinadequateinLreastledinlants
2types
J.earlyvitaminKdeliciencyLleedingVKD5)
causedLymaternalingestionoloralanticoagulants,anticonvulsants,or
antituLerculosisagents
presents withICHwithinthelirst24hoursollile
2. classicalVKD5
occursininlantswhodidnotreceivevitKatLirthandareLreastleeding
presents LetweendaysJ and/
Prevention
vitaminKIMadministrationatLirthtoallnewLorns
Necrotizing Enterocolitis (NEe)
intestinal inllammationassociatedwithlocalordilluseulcerationandnecrosis
primarilyallectingterminalileumandcolon
allects J-5, olpretermnewLornsadmittedtoMICU
Pathophysiology
postulatedmechanismolLowelischemia mucosaldamage,andenteralleeding
providingasuLstratelorLacterial growthandmucosalinvasion,leadingto Lowelnecrosis
organgreneandperloration
Risk Factors
prematurity immaturedelenses)
asphyxia,shockpoorLowelperlusion)
hyperosmolarleeds
enteralleedingwithlormulaLreastmilkcanLeprotective)
sepsis
distendedaLdomen
increasedamountolgastricaspirate /vomituswithLilestaining
lrankoroccultLlood instool
leedingintolerance
diminishedLowelsounds
signsolLowelperlorationsepsis,shock, peritonitis,DIC)
Investigations
aLdominalx-ray. intramuralairtraintracks),lreeair,lixedloops,thickenedLowelwall,
portalvenousgas
PneumonitisIntestinalis gasintheLowelwallseenonaLdominalx-ray). Inneonatal
period,thisismostcommonlyassociatedwithnecrotizingenterocolitis.
C5C,A5C,Lloodculture
highorlowW5C,lowplatelets, hyponatremia, acidosis, hypoxia,hypercapnea
Treatment
MOmnimumJweek),vigorousIlluidresuscitation,MCdecompression,supportivemerapy
TPM
antiLioticsusuallyampicillin,gentamicinmetronidazoleilriskolperlorationx/-J0days)
serialaLdominalx-raysdetectearlyperloration
peritonealdrain/ surgeryilperloration
surgicalresectionolnecroticLowelandsurgerylorcomplicationse.g.perloration,strictures)
Intraventricular Hemorrhage (l VH)
intracranialhemorrhageoriginatingintheperiventricularsuLependymalgerminalmatrixCM)
incidenceandseverityinversely proportionaltoCA
Risk Factors
extremeprematurityneedlorvigorousresuscitationatLirth, pneumothorax,ventilated
preterminlants,suddenincreaseinarterialLloodpressurewithvolumeexpansion,
hypotensiveevent,hypertension,RDS,lluctuatingcereLralLloodllow,coagulopathy
Pediatrics P7
manyinlantswithIVHareasymptomatic
suLtlesigns.apnea,Lradycardia,changesintoneoractivity, alteredlevelolconsciousness
catastrophicpresentation.Lulginglontanelle,dropinhematocrit,acidosis,seizures,hypotension
Classification
Papile classilication
gradeI. CMhemorrhage
gradeII.IVHwithoutventriculardilatation
gradeIII.IVHwithventriculardilatation
gradeIV.CMhemorrhageorIVHwithparenchymalextension
parenchymalhemorrhagemayalsooccurintheaLsenceolintraventricularhemorrhage
50, olIVHoccurswithin8hoursolLirth,90,occursLyday3
routineheadultrasoundscreeningolallpreterminlants<32weeksgestationthroughout
MICUstay
Management of Acute Hemorrhage
supportivecaretomaintainLloodvolumeandacid-Lasestatus
avoidlluctuationsinLloodpressureandcereLralLloodllow
lollow-upwithserialimaging
Prognosis
outcomedepends onCrade olIVH
short-term outcomeslorsevereIVH.mortality, posthemorrhagichydrocephalusPHH),
posthemorrhagicinlarction
possiLlelong-termmajorneurologicalsequelae. cereLralpalsy, cognitivedelicits,motor
delicits,visualandhearingimpairment
gradesJand2hemorrhageshavearelativelylavouraLleprognosis
greatestmorLidityandmortalityisseenwithCrade4hemorrhageandPHHrequiring
ventriculoperitonealshuntplacement
prematureLaLiesarealsoatriskolPVLperi ventricularleukomalacia) radiologically
seenascystsorischemicareasintheperiventricularwhitematter,alsoputtingthematrisk
oladverseneurologicaloutcome
Retinopathy of Prematurity (ROP)
interruptioninthegrowtholdevelopingretinalvessels
Pathophysiology
earlyvasoconstrictionandoLliterationolthecapillaryLed repairresponse
neovascularisation
retinaldetachmentoccursinasmallpercentage
Risk Factors
associationwithperiodolhighoxygenconcentrations isnownotsoclear
extremeprematurityisthemostsignilicantrisklactor
KLisclassiliedLystage I-VwithVLeingmostsevere)
seeO_hthalmolo_
Assessment
ophthalmoscopicexamination
inlantswithLirthweight<500gor<30weeksCA.startingat4-6weeksol
chronologicageorat32weekscorrectedage whicheveris later)withexams
q2-3weeksuntilretinalmaturitywithnodiseaseordiseaseisregressing
inlantswithKLorveryimmaturevessels. examsqJ-2weeks
Management
laserphotocoagulationlorsevere prethresholdandthresholdKL
lollow-upeyeexaminationslormyopia,straLismus,amLlyopia,glaucoma,andlatedetachment
Prognosis
stageIand II. 90, spontaneousregression
stageIII+. -50, spontaneousregression,withtreatment,incidenceolpoorvisualoutcome
reducesLy-50,
Toronto Notes 2010 Neonatolog/Nephrolog
Common Neonatal Skin Conditions
Tabl e 39. Common Neonatal Ski n Conditions
Neonatal Ski n Conditions
Vasomotor Response
( Cuti s Marmorata, acrocyanosi s)
Vernix Caseosa
Sl ategrey nevus of chi l dhood
(,Mongol i an spots')
Capi l l ar Hemangi oma
Erythema Toxi cum
Mi l i a
Pustul ar mel anosi s
Angi omatous l esi ons ( Sal mon patch)
Neonatal Acne
Description
Transi ent mottl i ng when exposed to cold; usual l y normal , parti cularly in prems
Soft creamy white layer covering baby at bi rth
Bl ui sh grey macul es over l ower back and buttocks (may look l i ke brui ses); common i n dark
ski nned infants
Rai sed red l esi on which increases in size after birth and involutes; 50% resolved by 5 yrs,
90% by 9 yrs
Erythematous vesi cul opustul ar rash, l esi ons di sappear and reappear in mi nutes to hours,
resolves by 2 weeks
Lesi ons 1 2 mm firm white pearly papules on nasal bridge, cheeks, and pal ate; selfresolving
Brown macular base wi th dr vesicles, seen more commonly in African Ameri can infants
Transitor macul ar capi l l ar hemangi omas of the eyel i ds and neck
("Angel Kiss" and "Stork Bi te"); usual ly di sappears wi th age
Selfresolvi ng
Nephrology
Dehydration
Tabl e 40. Assessment of Dehydration
Point of Assessment
Vol ume deficit
Osmol ar disturbance
Acidbase disturbance
Potassi um
Method
Hi stor, physi cal exami nation
Serum Na
Blood pH, pCOz, bicarbonate
Serum K
Renal functi on BUN, creati ni ne, uri ne speci fi c gravity/osmol al ity, uri ne sedi ment
Tabl e 41 . Assessment of Severity of Dehydratati on
Mild Moderate Severe
Pul se ( HR) Normal , ful l Rapi d Rapi d, weak
Bl ood Pressure ( BP) Normal Normallow Shock - decreased BP
Uri ne Output ( UO) Decreased Markedly decreased Anuri a
Oral Mucosa Sl i ghtl y dr Dr Parched
Anterior Fontanel l e Normal Sunken Markedly sunken
Eyes Normal Sunken Markedly sunken
Ski n Turgor Normal Decreased Tenti ng
Capi l l ar Refi l l Normal 3 sec) Normal to increased Increased ( >3 sec)
% los of Pre-Illness Body Weight
<2 years 5% 1 0% 1 5%
>2 years 3% 6% 9%
Fluid and Electrolyte Therapy
Principles of Treatment
provisionolmaintenancedailylluidandelectrolyterequirements seeTaLle42)
PLUSreplacementoldelicitlluidsandelectrolytesseeTaLle43)
PLUSreplacementolongoinglossesconsiderurineoutput,Lowelmovements /diarrhea,
lever)
Pediatrics P73
'
Assessment of Severity of Dehydraton
C BASE H20
Capi l lary refi l l
BP
Anteri or fontanel l e
Ski n turgor
Eyes sunken
HR
Oral mucosa
Output of uri ne
P74 Pediatrics
K
Causes of Coloured Urine with
Negative Dipsti ck
URI NE BLEAD
Urates
Ri lampi n
I huprolen
Ni trolurantoi n
Exogenous (Iood col ouri ng)
Beets
Lead
Nephrolog
Tabl e 42. Mai ntenance Fl ui d and Electrolyte Requi rements
Body Weight 1 00:50:20 Rule 4:2:1 Rule
1 1 0 kg
1 1 20 kg
>20 kg
( 24hour mai ntenance fl ui ds)
1 00 cckg/day
( hourly rate of mai ntenance fl ui ds)
4 cckg/hr
Electrolyte Requirement
Na:
K:
CI :
50 cc/kg/day
20 cckg/day
3 mEqkgday
2 mEqkgday
3 mEqkgday
2 cckg/hr
1 cckg/hr
Tabl e 43. Correction of Fl ui d and El ectrolyte Deficits
Dehydration 1 5% 1 0% Rate
Toronto Notes 2010
Isotonic (80%) Na 45 mmoVkg Na 8 1 0 mmoVkg 1 /2 total repl acement over 1 st 8 hours,
K 45 mmoVkg then 1 /2 over 1 6 hours
Hypotonic2 (5%) Na 56 mmoVkg Na 1 0 1 2 mmoVkg If Na > 1 05, correct as above
(Na < 1 30 mmoVL) K 3 mmoVkg K 5 mmol/kg If Na < 1 05, correct by 20 mmoVL maxi mum
over 0. 54 hours wi th hypertonic sal i ne
Hyperonic ( 1 5%) Na 24 mmoVkg Na 24 mmoVkg Correct over 4872 hours
(Na > 1 50 mmoVL) K 24 mmol/kg K 24 mmol/kg Do not al low serum Na to drop faster than
1 0 1 5 mmollday3
Note: 'For all types dehydration, H20 for 5% dehydration ; 50mlkg; for 1 0% dehydration ; 1 00 mlkg
'To calculate exact deficit: INaj deficit ; ( [ Najtarget - INajactual) x body weight (kg) x total body water ( L)
'To lower serum Na by a predictable amount. remember: 4 mlkg of free H20 lowers serum Na by 1 mmoVL
Common IV Fluids
lirstmonthollile.D5W/ 0. 2MS+ 20mEqKCl / LonlyaddKClilvoidingwell)
children.D5W/ 0. 9MS+20mEqKCl / LorD5W/ 0.45MS+20mEqKCl / L
MS.as Lolust orestorecirculationindehydratedchildren
Tabl e 44. Common Manifestations of Renal Di sease
Neonate
Fl ank Mass
Hematuri a
Anuria/ol i guri a
Child and Adolescent
Cola/redcoloured uri ne
Gross Hematuria
Edema
Hypertensi on
Pol yuri a
Ol i guri a
Ungency
Hematuria
Dyspl asi a, polycystic di sease, hydronephrosis, tumour
Asphyxi a, malformation, trauma, renal vei n thrombosis
Agenesi s, obstruction, asphyxia
Hemogl obi nuri a ( hemol ysi s)
Myogl obi nuri a (rhabdomyolysis)
Hematuri a (e.g. glomerul onephri ti s), pi gmenturi a
Glomerul onephritis, beni gn hematuri a, trauma, cystitis, tumour, stones
Nephrotic syndrome, nephritis, acute/chroni c renal fai l ure (al so consi der cardiac or liver di sease)
Acute gl omerul onephri ti s, renal fai l ure, dyspl asi a (al so consi der coarctati on of aorta, drugs,
endocri ne causes)
OM, central and nephrogeni c diabetes i nsi pi dus, hypercal cemi a, pol yuri c renal fai l ure
Dehydration, acute tubul ar necrosis (ATN), interstitial nephri ti s
Uri nary tract infection (UTI), vagi ni ti s
delinition.liveormoreR5C/ hpl,inthreeconsecutivecentrilugedurinesamples
0. 5-2, prevalenceolasymptomaticmicroscopichematuriainschool-agedchildren
historyolprioracuteinlectionupperrespiratory, skinorCI)
lamilyhistory.dialysis,transplant,SLE,lamilialhematuria
physicalexam.5Pedema,rashes,arthritis
E tiology
nephrologic
glomerulardisease
recurrentgrosshematuria.IgAnephropathy,Lenignlamilialhematuria,Alport
syndrome
post-streptococcalCM,lupusnephritis,HSPHUS,Coodpasturediseaserarein
childhood)
tuLulointersitial.ATM,interstitialnephritis,pyelonephritis,hypercalciuria
inlection.Lacterial,T5,viral,UTI,pyelonephritis
Toronto Notes 2010 Nephrolog
hematologic.coagulopathies,thromLocytopenia, sickle celldiseaseortrait,renalvein
thromLosis
nephrolithiasis
anatomicaLnormalities.congenital,trauma,polycystickidneys,vascularaLnormalities,
tumoursWilms)
other.exercise,drugs
Approach to Hematuria
4categoriesolhematuria
J. CrossHematuriavisiLleLloodinurine) mustconlirmpresenceolR5Cs
UTI
perinealirritationpinworm,anallissure)
coagulopathy
trauma
nephrolithiasis
urolithiasis / hypercalciuria
tumour
glomerular
IgAnephropathy
glomeularLasementmemLranedisorders
Alportsyndrome
Lenignlamilialhematuria
postinlectiousglomerulonephritis
Henoch-Schonleinpurpuranephritis
SLEnephritis
urinalysisU/A)
renalsource
cola/tea-colouredurine
casts,proteinuria,dysmorphicR5Cs
associatedsymptomsandsignse.g.edema,azotemia,hypertension)
post-renalsource
Lrightredurine,initialandterminalstreamhematuria,clots
normalR5Cmorphology, <2+proteinuria,nocasts
verylargerenalLleedingcanlooklikealowerurinarytractLleed
2. SymptomaticMicroscopicHematuria
UTI
acuteglomerulonephritis
lamilialhematuria
Henoch-SchonleinpurpuraHSP)
SLE
HTM
hypercalciuria
nephrolithiasis
tumour
3. AsymptomaticMicroscopicIsolatedHematuria
Lenignlamilialhematuriain2/ 3olcases
dipsticksensitive,Luthighlalsepositiverate.
5,olschool-agedchildrenpositiveonasingleurinedipstick
withrepeattesting<0.5, willhavepersistenthematuria
repeat2-3samplesover2-3weeksandmonitorlorclinicalsymptoms
4. AsymptomaticMicroscopicHematuria
delinition.5-J0R5C/HPFolcentrilugedurine
dipsticks areverysensitive,Luthaveahighlalsepositiverate
5,olschool-agedchildrenhavealalsepositiveonasingletestLut
<J, onrepeatedtesting
mild/earlyacuteglomerulonephritis
Lenignlamilialhematuriain2/ 3olcasesnoassociatedproteinuria)
tumour
Investigations
C5C,urinedipandculture, creatinine, 5UM,24-hrurinecollectionlorcreatinineand
protein, Ca,serumC3andC4level
ilsuspectedpostinlectiousglomerulonephritis.antistreptolysinL titer,AMA,throatswaL
ilaLovedonotyieldadiagnosis,considerU/S Dopplertoruleoutstructuralanomalies
treatunderlyingcauseilapplicaLle
supportivetreatmente.g.anthihypertensives,lluidrestriction,dietarymodilications)
relerraltopediatricnephrologist
maywarrantrenalLiopsydependingonlindings
Pediatrics P75
- '
P
False Positive Dipsti ck (positive
dipstick. but no RBCs): myogl obi nuri a
due to rhabdomylosi s. hemogl obi nuri a
due to intravascul ar hemol ysi s or
coagul ati on.
P76 Pediatrics Nephrolog Toronto Notes 2010
Proteinuria
asmallamountolproteinisloundintheurineolhealthy children<4mg/ m
/h or
<J00mg/m
/d
delinition
qualitative.J+indilute,2+inconcentratedurinespecilic
gravity >J. 0J5)
quantitative.>4mg/m/ hontimedurine>40mg/ m
/ hisnephroticrange)
urinedipstickistheleastaccuratelalsepositivesilurinepH>8orSC>J.025)
protein/creatinineratioonspoturineismoreaccuratenormal<0.5il6mo-2yrs,
<0.2ilover2yrs)
24-hourproteinisthemostaccuratenormal<J00mg/m
/day)
microalLuminuriaassessesriskolprogressiveglomeronephropathyindiaLetesnormal
<30mgalLumin/gramcreatinineonlirstmorningvoid)
progressiveproteinuriaistheLestpredictorolrenaldisease
transientproteinuria.duetolever>38.3C/ J0JF),dehydration,exercise,seizures,stress
persistentproteinuria>Jondipstick)
orthostaticmorecommoninadolescents usuallyLenign).elevatedprotein
excretionwhenuprightandnormalwhenrecumLent,rarelyexceedsJ g/m
/d
glomerulare.g.nephroticsyndrome, glomerulonephritis)
tuLulointerstitiale.g. Fanconisyndrome,ATM)
structuralaLnormalitiesolurinarytracte.g. hydronephrosis)
Abnormal uri ne di psti ck protei n in an
afebri l e chi ld
1 :1 +
sU. Zmg protei n

============= per mg creati ni ne
Obtai n fi rst A. M. voi d for uri ne total
protei ncreati ni ne ratio and uri nalysi s
wi th mi croscopi c exam
and normal U/A
I >0. Zmg protei n per mg
creati ni ne or abnormal U/ A
|bh1hbhbVLb1| N
Hi story ( drugs, fami ly hi story)
Physi cal exam ( i ncl udi ng BP)
Serum chemi stri es i . e. creati ni ne, BUN,
electrolytes, cholesterol , and al bumi n
Also consi der (when appropriate)
Renal ultrasonography
Serum C3/C4 complement, ANA
Hepati ti s B and C serology
HIV testi ng
Trace
Repeat di psti ck on first A. M. voi d
I
In one year
Consul t wi th a pedi atri c nephrol ogi st
who wi l l consi der a ki dney bi opsy
and defi ne appropriate therapy
based on the fi ndi ngs
Fi gure 5. Evaluati on of Persistent Protei nuria i n Chi ldren/Adolescents
Hogg R, J, et ai, 120001 Paediatrics Vol. 105: 124249,
Hemolytic Uremic Syndrome (HUS)
E pidemiology
most commoncauseolacuterenallailureinchildren
morecommonlrom6monthsto4yearsolage
Pathophysiology
L. coli OJ5/.H/verotoxinhamLurgerdisease)orshigatoxin
toxinLinds,invadesanddestroyscolonicepithelialcells,causingLloodydiarrhea
toxinentersthesystemiccirculation,attachesandinjuresendothelialcellsespecially
inkidney)causingareleaseolendothelialproducts e.g. vonWilleLrandlactor,
plateletaggregatinglactor)
lormplatelet/liLrinthromLiinmultipleorgansystemse.g.renal,pancreas,Lrain)
resultinginthromLocytopenia
R5CsarelorcedthroughoccludedvesselsresultinginlragmentedR5C schistocytes)
andremovedLythereticuloendothelialsystemhemolyticanemia)
other,rarelormsolHUSinchildhoodareduetoLacteria e.g. 5. pneumoniae), viruses,
lamilialinheritance,ordrugs
triad.acuterenallailure,thromLocytopenia,microangiopathichemolyticanemia
initialpresentationolaLdominal painanddiarrhea,lollowedLyLloodydiarrhea
Toronto Notes 2010 Nephrolog
within5-/daysthepatientLeginstoshowsignsolanemia,thromLocytopeniaandrenal
insulliciency
history. weakness, lethargy, oliguria
physicalexam.pallor,jaundice hemolysis),edema,petechiae,hypertension
Investigations
C5C,platelets,Lloodsmear,urinalysis,5UM, creatinine, stoolcultureand
shigellatoxinassay
Treatment
supportivetreatment,nutritionalsupport,monitorelectrolytes,dialysisilelectrolyte
aLnormalitycannotLecorrected,lluidoverload,or5UM>J00mg/dL,PR5Clor
symptomaticanemia
steroidsnothelplul,antiLioticsnotindicated
Prognosis
5-J0,mortality, J0-30,kidneydamage
Nephritic Syndrome
acute, suLacuteorchronic
hematuriawithR5Ccasts, proteinuria 50mg/ kg/ day,notnephrotic-range),
azotemia,hypertension
renallailureoliguria)
POST STREPTOCCOCAL GLOME RULONEPHRITIS
Pediatrics P7
Risk Factors
mostcommoninchildren,aged4to8yearold,M>F

occursJ-3weekslollowinggroupALeta-hemolyticstreptococcalinlectionolskinorthroat
Nephritic Syndrome
Pathophysiology
antigen-antiLodymediatedcomplementactivation
dilluse,prolilerativeglomerulonephritis
Diagnosis
elevatedserum antiLody titres againststrep antigens ASOT,antiDMAse5)
Prognosis
95,olchildrenrecovercompletelywithin J-2 weeks
5-J0,havepersistenthematuria
Management
symptomatictreatment.lluidrestriction,antihypertensives,diuretics
inseverecases. hemodialysisorperitonealdialysismayLenecessary
eradicationolinlectionpenicillinorerythromycin)
Tabl e 45. Major Causes of Acute Gl omerul onephritis
Renal
Systemic
Decreased C3
Post-i nfecti ous GMN
Membranoprol i ferative
Type I (50-80%)
Type I I ( >80%)
SLE
SBE
Shunt nephri ti s
Croglobul i nemi a
Normal C3
IgA Nephropathy
Idi opathi c rapi dly progressive GMN
Anti - GBM disease
Polyarteritis nodosa
Wegener's granul omatosis
Goodpasture's syndrome
Henoch-Schonl ei n purpura ( HSP)
Nephrotic Syndrome
severe proteinuria>50mg/kg/dayor>40mg/m
/hr)
hypoalLuminemia<20g/L 2. 0g/dL)
edemausuallylirstsign,initiallyseelacialswellingespeciallyperiorLital,andpretiLial
edema)
hyperlipidemia>5. J/mmol / L200mg/dL)
secondarylindings.hypocalcemia,hyperkalemia,hyponatremia,hypercoagulaLility
decreasedPTT)
PHAROH
Protei nuri a 50 mg/kgld)
Hematuri a
Azotemi a
RBC casts
Ol i guri a
Hyperensi on
K'
PALE
Protei nuri a ( >50 mg/kg/d)
HypoAl bumi nemi a 20gll)
Hyperli pi demi a
Edema
P78 Pediatrics Nephrolog Toronto Notes 2010
E tiology
Primary Nephrotic Syndrome
minimalchangediseaseMCD) /6,)
peakoccurrenceLetween2-6yearsolage,morecommoni nLoysthangirls2. J)
oltentreatedempiricallywithsteroidswithoutkidneyLiopsy, 90,steroidresponsive
memLranousglomerulonephritis8,)
localsegmentalglomerularsclerosisFSCS)/,)
memLranoprolilerativeglomerulonephritis5,)
Secondary Nephrotic Syndrome
vasculitis
inlectionse.
.hepatitis5&C, syphilis, HIV)

medicationse.g.captopril,penicillamine,MSAIDs,anticonvulsants)
malignancy
hereditarye.g.sickle celldisease,Alportsyndrome)
metaLolic,inllammatory e.g. lupusnephropathy, rheumatoidarthritis)
Complications
riskolinlections e.g.spontaneousperitonitis, cellulitis, sepsis)
hypercoagulaLilityduetodecreasedintravascularvolumeandantithromLinIIIdepletion
pulmonaryemLolism,renalveinthromLosis)
sideellectsoldrugsdiuretics,steroids,immunosuppressants)
hypotension,shock,renallailure
Investigations
toruleoutsecondarycauses olMS.serumcomplementlevels, 5UM, Cr, serumchemistries,
AMA,antistreptolysinL titre,incertaincasesHIVHep5/ Candsyphilistiters
considerkidneyLiopsyil
HTMhigherriskolFSCS),steroidresistant,lrequentrelapses>2relapsesin6month
period),lowserumcomplement,severelydecreasedrenallunction
presentationLelorelirstyearollilehighlikelihoodolcongenitalnephroticsyndrome)
presentationalter J0yearsolagetoruleoutmoreseriousrenalpathologythanMCD
Management
saltandwater restriction, diureticmayLerequired
optimalnutrition,includinghigh-qualityprotein
dailyweightstoassesstherapeuticprogress
varicellaantiLodytitreilnotimmune
pneumococcalvaccinealterremissionavoidlivevaccines)
initialtreatmentolMCD
oralprednisoneorequivalent)60mg/ m
/dayindivideddosesmax.dose
80mg/ day)lorupto J2weeks
anegativetuLerculinskintestshouldLeperlormedLelorestartingsteroidmedications
ameasuraLledecreaseinproteinexcretionmaytakeatleast/to J0dayslollowing
initiationoltreatment,andproteinuriaclearsLythirdweekoloralprednisone
upto2/ 3olpatientsexperiencerelapses
ilunresponsivetosteroids,lrequentrelapsesorsteroid-resistantproteinuriacontinues
Leyond3months)
considerrenalLiopsyortreatwith cytotoxic agenti.e.cyclophosphamideor
chloramLucil),immunomodulatingagentssuchaslevamisoleandcyclosporineA,
andhigh-dosepulsecorticosteroidwithguidanceolapediatricnephrologist
Hypertension in Childhood
E tiology
considerwhitecoathypertensionlorallages
Tabl e 46. Eti ology of Chi ldhood Hypertensi on by Age Group
System <1 year 1 6 years 71 2 year
Cardiovascular Coarctati on of the aorta Neuroblastoma
Coarctati on of aora
Endocrne Wi 1 m's tumour Endocrine causes (hyperhyroi d,
Metabolic hyperparathyroid, Cushi ng,
pri mar hyperaldosteroni sm)
Renal Renal arer/vei n thrombosis Renal arery stenosis Renal parenchymal disease
Congenital renal disease Renal parenchymal abnormal iti es of renal
disease vasculature
Respiratory Bronchopul monar dyspl asi a Essenti al hypertensi on
Hypercal cemi a
>1 3 years
Essenti al hypertensi on
Endocrine cause
Renal parenchymal
disease
Toronto Notes 2010 Nephrolog/Neurolog
Tabl e 47. 95th Percentile Bl ood Pressures ( mmHgl
Age (Yearsl
1 2
1 7
Female
50t Percentle for 75t Percentle for
Height Height
1 04/58 1 05/59
1 1 1 m
1 23/80
1 29/84
1 1 2/73
1 24/81
1 30/85
50th Percentle for
Height
1 02157
1 1 4m
1 23/81
1 36/87
Male
75t Percentle for
Height
1 04/58
1 1 5n5
1 25/82
1 38/88
Adapted from "Update on te |J/Task Force Report on High Blood Pressure i Children and Adolescent workin group report from te Natonal High Blood
Pressure Educaton Program",
Investigations
laLs
urinedipsticklorLloodandproteinsuggestsrenaldisease)
urinecatecholaminesandtheirmetaLolitesmaysuggestpheochromocytoma)
electrolytes,creatinine,catecholamines,renin,aldosterone
imaging
echocardiography
aLdominalU/S
dopplerstudies,angiography, orradionuclideimagingolrenalarteries
Management
weightreduction, reductioninsaltintake,exercise
lirstlineantihypertensivesarethiazidediuretics,Lutnoneoltheantihypertensiveshave
Leenlormallystudiedinchildren
relerraltospecialist
medicationsusedinhypertensiveemergencies.niledipine,hydralazine,laLetalol,sodium
nitroprusside
assessmentandmanagementolendorgandamage e.g. retinopathy, LVH)
Neurology
Seizure Disorders
seeMeurolo_
Diferential Diagnosis of Seiz ures in Children
LenignleLrile seizure mostcommon)
hypoxicischemicencephalopathyasphyxia)
intracranialhemorrhage,trauma
metaLoliccauses e.g.hypoglycemia,hypocalcemia,hyponatremia)
CMSinlection
idiopathicepilepsyandepilepticsyndromes
neurocutaneoussyndromes
CMS tumour
arteriovenousmallormation
ingestions /drugwithdrawal
ruleoutconditionsthatmimicseizure.
Lreathholding
nightterror
Lenignparoxysmalvertigo
narcolepsy
pseudoseizure
syncope
tic
hypoglycemia
TIA
Investigations
C5C,electrolytes,calcium, magnesium, glucose
toxicologyscreenilindicated
EEC,CT,LPilindicated
EECmayLeindicatedlorlirst-timenon-leLrileseizure
EEC/CTnotindicatedlorLenignleLrileseizuresrecurrencerisk,determineseizure
type,orepilepticsyndrome
Pediatrics P7
` *
P
Heart probl ems such as l ong O
syndrome and hyperrophi c
cardi omyopathy are often
mi sdi agnosed as epi l epsy, I ncl ude
cardi ac causes of syncope i n your
di fferenti al di agnosi s, parti cul arl y when
the epi sodes occur duri ng physi cal
activity,
P80 Pediatrics
KETOGENIC DI E
Seizures Decreased Rapidly Afer Testing
PreliminaryStdies dthe Ketogenic Die
Freeman JM, Iny DR Arc Pd Adol Med 1999;
153191: 946-949
Low protein, low carb, hgh fat diet resting in
ketosis. Used to treat seilre rlsorders.
Shown to decrease seizure frequency b >50% in
Lennox-Gastaut.
Neurolog Toronto Notes 2010
CHILDHOODEPILEPTIC SYNDROME S
Infantile Spasms
onset4-8months
Lriel,repeatedsymmetriccontractions olneck,trunk,extremities llexionand
extension)lastingJ0-30seconds
occurinclusters,oltenassociatedwithdevelopmentaldelay
20,unknownetiology,mayhavegoodresponsetotreatment
80,duetometaLolicordevelopmentalaLnormalities,encephalopathies,orare
associatedwithneurocutaneoussyndromes,thesehavepoor responsetotreatment
candevelopintoWestsyndromeinlantilespasms, psychomotordevelopmental
arrest,andhyperarrythmia)orLennoxCastaut
typicalEEC. hypsarrhythmiahighvoltageslowwaves,spikesandpolyspikes,
Lackgrounddisorganization)
treatment.ACTH,vigabatrin,benzodiazepenes
Lennox- Gastaut
onsetcommonly3-5years olage
characterizedLytriadolJ)multipleseizuretypes,2)dillusecognitivedyslunction
and3)slowgeneralizedspikeandslowwaveEEC
seenwithunderlyingencephalopathyandLrainmallormations
treatment.valproicacid,Lenzodiazepinesandketogenicdiet,however, response
oltenpoor
J uvenile Myoclonic E pilepsy (J anz )
adolescentonset J2-J6years olage),autosomaldominantwithvariaLlepenetrance
myoclonusparticularlyinmorning,lrequentlypresentsasgeneralizedtonic-clonic
seizures
typicalEEC. 3.5-6Hzirregularspikeandwave,increasedwithphoticstimulation
requireslilelongtreatment valproicacid),prognosisexcellent
Childhood Absence E pilepsy
multipleaLsenceseizures per daythatmaygeneralizeinadolescenceorresolve
spontaneously
peakageolonset6-/,F>M,stronggeneticpredisposition
eachseizureislessthan30seconds,no post-ictalstate,mayhavemultipleseizures
perday
typicalEEC. 3/secspikeandwave
treatment.valproicacidorethosuximide
Benign Focal E pilepsy of Childhood with Rolandic/Centrotemporal Spik es
onset peaks at5-J0years olage, J6, olallnon-leLrileseizures
localmotorseizuresinvolvingtongue,mouth,lace,upperextremityusuallyoccuring
insleep-waketransitionstates
remainsconsciousLutaphasicpost-ictally
remitsspontaneouslyinadolescence,nosequelae
typicalEEC. repetitivespikesincentrotemporalareawithnormalLackground
treatment.lrequentseizurescontrolledLycarLamazepine,no medicationilinlrequent
seizures
Treatment
anticonvulsantsolteninitiatedil>2unprovokedaleLrile seizures within6-J2months
J. initiate. treatmentwithdrugappropriatetoseizure type
2. optimize. startwithonedrugandincrease dosageuntilseizures controlled
3. ilnoellect,switchovertoanotherLeloreaddingasecondanticonvulsant
4. continue ticonvulsant treatmentuntilpatient lreeolseizureslor2yearsormore,then
weanmedicationsover4-6months
ketogenicdiethiglatdiet) usedinpatientswhodonotrespondtopolytherapyorwho
donotwishtotakemedicationvalproicacidcontraindicatedinconjunctionwith
ketogenicdietLecause mayincreasehepatotoxicity)
educationlorpatientandparents
privilegesandprecautionsindailylile e.g.Luddysystem,showersinsteadolLaths)
legaloLligationtoreporttoMinistryolTransportationilpatientwishestodrive,Ministry
willdetermineildriver slicenseispermitted
Generaliz ed and Partial Seiz ures
seeMeurolo_,M8
Toronto Notes 2010 Neurolog
Benign Febrile Seizures
E pidemiology
most commoncauseolseizure inchildren
3-5,olallchildren,M>F
age6months-6years
thoughttoLeassociatedwithinitialrapid riseintemperature
noneurologicaLnormalitiesordevelopmentaldelayLeloreoralterseizure
no evidenceolCMSinlection/inllammationLeloreoralterseizure
nohistoryolnon-leLrileseizures
duration<J5minutes95,<5minutes)
generalizedtonic-clonic,symmetric
doesnotrecurina24-hourperiod
Complex Febrile Seiz ure
any oneolthelollowingleatures
localonset,localleaturesduringtheseizure,orneurologicaldelicitalter
duration >J5minutes
recurrent seizures >Jin24-hourperiod)
previousneurologicalimpairment
theseseizuresarenottheLenign,simpletype,andrequirelurtherinvestigations
Risk Factors for Recurrence
33,chanceolrecurrence, /5,recurwithinJyear
50,chanceolrecurrenceil<Jyearolage
28,chanceolrecurrenceil>Jyearolage
lamilyhistoryolleLrileseizuresorepilepsy
risklactorsincludedevelopmentalorneurologicalaLnormalitiesolchildpriortoseizures,
lamilyhistoryolnon-leLrileseizures,andanatypicalinitialseizure,multiplesimpleleLrile
seizures
Work up
history.determinelocusollever,descriptionolseizure,meds,traumahistory,
development,lamilyhistory
exam.LOC,signsolmeningitis,neurologicalexam
septicwork-upincludingLPilsuspectingmeningitisilchild<J2months,strongly
considerdoinganLP,ilchildisJ2-J8months, considerincludinganLP,ilchild
>J8months,doLPilmeningealsigns)
EECnotwarrantedunless complexleLrileseizureoraLnormalneurologiclindings
ilsimpleleLrileseizure,investigationsunnecessaryexceptlordetermininglocusollever
Management
counselandreassurepatientandparents leLrileseizuresdonotcauseLraindamage, very
smallriskoldeveloping epilepsy,9,inchildwithmultiplerisklactors,2,inchildwith
leLrile simple seizurescomparedto J, ingeneralpopulation)
antipyreticse.g.acetaminophen)andlluidslorcomlortneitherpreventseizure)
prophylaxisnotrecommended
ilhighrisklorrecurrentorprolongedseizures,haverectalorsuLlingual lorazepamat
homedangerollorazepamisthatitmayhidesignsolaCMSinlection)
treatunderlyingcauseollever,e.g.otitismedia)
Recurrent Headache
seeMeurolo_
Assessment
ilunremarkaLlehistory,andneurologicalandgeneralphysicalexamisnegative,likely
diagnosisismigraineortension-typeheadache
oLtainCTorMRIilhistoryorphysicalrevealsredllags
inquireaLoutleveloldisaLility, academicperlormance,alter-schoolactivities
primaryheadache.tension, migraine, cluster
secondaryheadache. seeMeurolo_
MIGRAINE
4-5,olschoolagedchildren
revalenceF.M- 2.J alterpuLerty
eterogeneousautosomaldominantinheritancewithincompletepenetrancemajorityol
patientshaveapositivelamilyhistory)
Pediatrics PSI
-'
If a febri l e sei zure l asts > 1 5 mi nutes.

suspect meni ngi ti s or a toxi n.
Randomized. Controlle Tral dIbuprofen Syrp
Administred During Febrile Illnesses to Prevent
Febrle Seizre Recurrences
van Stjvenberg M. et al. Piatrc. 1 021 51 : E5 1 .
1 998 No
Purpose: To assess the effcacy mintermitent
antipyretic treatment in the prevention of febrle
seizure recurrences.
Stdy: Double blind RCT with 220 children and 2
year followup.
Paricipants: Children age 1 4 1th febrle seizre
within the last month. and at least one risk factor for
febrile seizure recurrence: either fanily history of
febrile seizures. previous recurrent febrile seizures.
temperature <40. 0 C at the iritial seizure. or multiple
type iritial seizure.
Interventions: Ibuprofen 5 mglkg every 6 hours
durng fever Irectal T >38.4 CI or placebo.
Main Outomes: First recurrence of febrle seizure.
Results: On an intentiontotreat analysis. the 2year
recurrence probabilities were 32% in the ibuprofen
group and 39% in the placebo group. Ith a non
sigrificant risk reduction of 0. 9 195% CI. 0. 61 . 51.
Conclusions: There is no evidence to support
intermittent antipyretic therapy in preventing febrile
seizures.
Headache - Red Flags
New headache
Worst headache of thei r l i ves
Acute onset
Focal neurol ogi cal defi ci ts
Consti tuti onal symptoms
Worse i n morni ng
Worse wi th bendi ng over. coughi ng.
strai ni ng
Change i n level of consci ousness
P82 Pediatrics
- '
Causes of hypotoni a that respond to

rapi d treatment: hypokal emi a,
hypermagnesemi a, aci demi a, toxi ns,
drugs, hypogl ycemi a, sei zure, infecti on,
i ntracrani al bleedi ng, hydrocephal us.
Neurolog Toronto Notes 2010
Types
commonwithoutaura) mostcommoninchildren,associatedwithintensenauseaandvomiung
classic withaura)
complicated.e.g.Lasilar,ophthalmoplegic,conlusional,hemiplegic
Clinical Features
ininlancy,symptomsinclude spells olirritaLility, sleepiness,pallor,andvomiting
inayoungchild,symptomsincludeperiodicheadacheswithnauseaandvomiting,
relievedLyrest
usuallyunilateralthroLLingheadachesinkidswithphotophoLiaorphonophoLia
Prognosis and Treatment
over 50, olchildrenundergo spontaneous prolonged remissionalterJ0yearsolage
earlyanalgesiaiLuprolen)andrestinquiet,darkroom
non-pharmacologicaltreatmentandprophylaxis.avoidtriggerspoorsleep,stress,cheese,
chocolate,calleine),LioleedLacktechniques,exercise
pharmacologicalprophylaxis.Leta-Llockerspropranolol),antihistamines,antidepressants
e.g.amitryptiline),calcium-channelLlockers,anticonvulsants e.g. divalproexsodium)
children>J2yearscanusesumatriptannasalspray, othertryptans
TENSIONHEADACHE S
usually consists olLilateralpressingtightnessanywhereonthecraniumorsuLoccipital
region,usuallylrontal,hurtingorachingquality,non-throLLing
lasts30minutestodays, waxesandwanes,mayLuildinintensityduringtheday
nonausea/ vomiting,notaggravatedLyroutinephysicalactivity
mostchildrenhaveinsigtintotheoriginolheadache. poorsell-image,learolschool
lailure
redllags. suddenmoodchanges, disturLed sleep,latigue, withdrawallromsocial
activities,chronicsystemicsignse.g.weightloss,lever,anorexia,localneurologicalsigns)
treatment
reassuranceandexplanationaLouthowstressmaycauseaheadache
ruleoutrelractory errorsineyesightascauseolheadache
mildanalgesiaMSAIDs,acetaminophen)
supportivecounselling
ORGANIC HEADACHE S
organicetiologyoltensuggestedwithoccipitalheadacheandredllagsLelow
withincreasedICP
etiology.Lraintumours,hydrocephalus,meningitis,encephalitis,cereLralaLscess,
pseudotumourcereLri,suLduralhematoma
characteristics.dilluseearlymorningheadaches,earlymorningvomiting,headache
worsenedLyincreasedICPcough,sneeze,Valsalva),asICPincreases,headacheis
constantandchildislethargicandirritaLle
withoutincreasedICP
etiology.cereLralarteriovenousmallormationAVM),aneurysm,collagenvascular
diseases,suLarachnoidhemorrhage,stroke
Hypotonia
decreasedresistancetomovement lloppyLaLy
properassessmentoltonerequiresaccuratedeterminationolgestationalage
evaluate
spontaneousposturespontaneousmovement,movementagainstgravity,lrog-leg
position)importantinevaluationolmuscleweakness
j ointmoLilityhyperextensiLility)
muscleLulk,presenceollasiculations
posturalmaneuvers
tractionresponse pulltosit,looklorllexionolarmstocounteracttractionandheadlag
axillarysuspension suspendinlantLyholdingataxillaandlilting,hypotonicLaLies
willslipthroughgraspLecauseollowshouldergirdletone
ventralsuspension inlantisproneandsupportedundertheaLdomenLyonehand,
inlantshouldLeaLletoholdupextremities,inverted U posturingdemonstrates
hypotonia,i.e.LaLywilldrapeselloverexaminersarms
investigationswilldependonhistoryandphysicalexam
ruleoutsystemicdisorders
Lloodglucose
enhancedCTolLrain
peripheralCK,EMC,muscleLiopsy
chromosomeanalysis, genetictesting
treatment.counselparentsonprognosisandgeneticimplications,relerpatientslor
specializedcare,relerlorrehaLilitation,OT,PT,assessleedingaLility
central
chromosomale.g.Downsyndrome,Prader-Willi, FragileX)
metaLolice.g.hypoglycemia,kernicterus)
Toronto Notes 2010 Neurolog
perinatalproLlemse.g.asphyxia, ICH)
endocrinee.g.hypothyroidism,hypopituitarism)
inlections e.g.TORCH)
CMSmallormations
dysmorphicsyndromes
peripheral
motorneurone.g.spinalmuscularatrophy, polio)
peripheralnervee.g.Charcot-Marie-Toothsyndrome)
neuromuscularjunctione.g.myastheniagravis)
muscleliLrese.g.mitochondrialmyopathy,musculardystrophy,myotonicdystrophy)
Cerebral Palsy (CP)
asymptomcomplex,notadisease
nonprogressivecentralmotorimpairmentsyndromeduetoinsulttooranomalyolthe
immatureCMS,extentolintellectualimpairmentvaries,presentationoltheimpairment
changeswithage
incidence. J. 5-2.5. J,000liveLirthsdevelopingcountries)
lileexpectancyisdependentonthedegreeolmoLilityandintellectualimpairment,noton
severity olCMSlesion
E tiology
oltenoLscure,nodeliniteetiology identiliedinJ / 3olcases
onlyJ0, relatedtointrapartumasphyxia
J0, duetopostnatalinsultinlections,asphyxia,prematuritywithintraventricular
hemorrhageandtrauma)
associationwithlowLirthweightLaLies
Tabl e 48. Types of Cerebral Palsy
Type
Spastic
Athetoid
Dyskinetic
Ataxc
% of Total CP Characteristcs
7080% Truncal hypotoni a i n 1 st year
1 0 1 5%
<5%
Increased tone, increased reflexes, clonus
Affects one l i mb (monopl egi a), one si de of body
(hemi pl egi a), both legs (di pl egi a), both arms and
l egs (quadri pl egi a)
Athetosis ( i nvoluntary writhi ng movements)
Z chorea ( i nvol untar jerk movements)
Can involve face, tongue (results i n dysarthria)
Poor coordi nati on, poor bal ance (wide based gai t)
Can have i ntenti on tremor
Area of Brain Involved
UMN of pyrami dal tract
Di plegi a associated with periventricular
l eukomal aci a (PL) i n premature babies
Quadri pl egi a associated wi th HIE (asphyxi a),
associated wi th hi gher i ncidence of MR
Basal gangl i a ( may be associated wi th
kernicterus)
Cerebel l um
Atonic <5% Marked hypotonia, hyperreflexia, severe cognitive del ay
Mixed 1 0 1 5% More than one of the above motor patterns
Other Signs
swallowing incoordination aspiration
microcephaly25,)
seizures
mentalretardation,learningdisaLilities
delayinmotormilestones
Investigations
mayincludemetaLolics,chromosomestudies, serology, neuroimaging, EMC, EEC
ilseizures),ophthalmologyaudiology
Treatment
maximizepotentialthroughmultidisciplinaryservices,importantlorlamilytoLe
connectedwithvarioussupportsystems
orthopaedicmanagement e.g.dislocations,contractures,rhizotomy)
managementolsymptoms.spasticityLaclolen,Lotox),constipationstoolsolteners)
Neurocutaneous Syndromes
characterizedLytendencytolormtumoursolCMS,PMS,visceraandskin
Neurofibromatosis Type I (NF- 1)
autosomaldominantLut50, aretheresultolnewmutations
alsoknownasvonRecklinghausendisease
incidenceJ. 3000,mutationinMFJgeneonJ/qJJ. 2codeslorneuroliLrominprotein)
learningdisorders,aLnormalspeechdevelopment,andseizuresarecommon
Pediatrics P83
- '
I n neurocutaneous syndromes, the

younger the chi l d at presentati on, the
more l i kel y they are to devel op mental
retardati on.
P84 Pediatrics Neurolog Toronto Notes 2010
diagnosis olMF-J requires 2ormoreol
>6cal-au-laitspots>5mmilprepuLertal,>J.5cmilpostpuLertal)
>2neuroliLromasolanytypeoroneplexilormneuroliLroma
>2Lischnodules hamartomasoltheiris)
opticglioma
lrecklingintheaxillaryoringuinalregion
adistinctiveLonylesione.g. sphenoid dysplasia, corticalthinningollongLones)
alirstdegreerelativewithconlirmedMF-J
Neurofibromatosis Type II (NF-2)
autosomaldominant
incidenceJ. 33000
characterizedLypredispositiontolormintracranial,spinaltumours
diagnosedwheneitherLilateralvestiLularschwannomaslound,oralirst-degreerelative
withMF-2andeitheraneuroliLroma,meningioma,glioma,orschwannoma
also associatedwithposteriorsuLcapsularcataracts
treatmentconsistsolmonitoringlortumourdevelopmentandsurgery
Sturge-Weber Syndrome
port-winenevussyndromeinVJ distriLutionwithassociatedangiomatousmallormations
olLraincausingcontralateralhemiparesisandhemiatrophy, alsoassociatedwithseizure,
glaucomaandmentalretardation
Tuberous Sclerosis
autosomaldominantinheritance, 50, newmutations
adenomaseLaceum angiokeratomasonlace,olteninmalardistriLution), Shagreenpatch
isolated raisedplaqueoverlowerLack,Luttocks), ashleal hypopigmentationseenwith
WoodslampUVlight)
cardiacrhaLdomyomas, kidneyangiomyolipoma,mentalretardationandseizures
cereLralcortextuLersareasolcereLraldysplasia),suLependymalnodulesSEM)may
evolveintogiantcellastrocytomasmaycauseoLstructivehydrocephalus)
calcilicationswithintheSEMareseenonCT,MRIespeciallyaroundtheloramenol
Munroe),thesemayoLstructtheloramenandcausehydrocephalus
Acute Disseminated Encephalomyelitis (ADEM)
E pidemiology
medianageolonset5-8years
malepredominanceF.M 0. 6. J)
annualincidenceinMorthAmericai sestimatedt oLe0.4perJ00,000children<20yrsolage
Pathophysiology
AcuteDisseminatedEncephalomyelitisADEM)isanimmune-mediatedinllammatory
disorderolthecentralnervoussystem,seeninchildren
characterizedLya widespreaddemyelinationthatpredominantlyallectingthewhite
matteroltheLrainandspinalcordsimilartoMultipleSclerosis)
usuallyprecededLyaviralinlectionorvaccination,andistherelorecommonlycategorized
aseitherpost-vaccinationorpost-inlectious
aLsenceolclearprecedenteventhasLeenreportedin26, olpatients
oltenoccurs 2daysto4weeks alteraclinically evident inlectionorvaccination
headache,nausea,vomiting
pyrexia/malaise
rapidonsetencephalopathy
multilocaldelicits
seizures
pyramidalsyndrome
cereLellarataxia
Lrainsteminvolvement
clinicalcourseisrapidlyprogressiveandusuallydevelopsoverhourstomaximumdelicits
withindays
Investigations
J. LumLarpuncture CSFshowingvariaLlepleocytosisandoligoclonalLanding
2. MRI large, multilocal, poorly marginatedregionsoldemyelinationallectingLilateral
suLcorticalwhitematter,anddeepgreymatterthalamus,Lasalganglia),lesionsshow
completeorpartialresolutiononlollow-up,withaLsenceolnewclinicallysilentlesions
Treatment
highdosecorticosteroidsandsupportivemeasures
Prognosis
lavouraLle,thoughsomeresidualdelicitsoltenexist
Toronto Notes 2010 Oncolog
Oncology
cancerissecondmostcommoncauseoldeathinchildrenalterJyearolageinjuriesarelirst)
causeisrarelyknown,Lutincreasedriskwith
chromosomalsyndromes
priormalignancy
neurocutaneoussyndromes
immunodeliciencysyndromes
lamilyhistory
exposuretoradiation,chemicals,Liologicagents
leukemiasarethemostcommontypeolpediatricmalignancy40,),lollowedLyLrain
tumours20,),andlymphomasJ5,)
some malignancies aremoreprevalentincertainagegroups
newLorns.neuroLlastoma,otheremLryonaltumourse.g.Wilms
tumour
nephroLlastoma),retinoLlastoma
inlancyandchildhood.leukemia,neuroLlastoma,Wilmstumour,retinoLlastoma
adolescence.lymphoma,gonadaltumours,Lonetumours
uniquetreatmentconsiderationsLecauseradiation,chemotherapyandsurgerycanimpact
growthanddevelopment,endocrinelunctionandlertility
mostchildrendosurvive treatmentshaveledtoremarkaLleimprovementsinoverall
survivalandcurerateslormanypediatriccancers
Leukemia
seeHematolo_
E pidemiology
meanageoldiagnosis2-5yearsLutcanoccur atanyage
heterogenousgroupoldiseases.
acutelymphoLlasticleukemiaALL)/5,)
acutemyeloLlasticleukemiaAML)J0,)
chronicmyelogenousleukemiaCML)5,)
uncleartypeJ0,)
childrenwithDownsyndromeareJ5timesmorelikelytodevelopleukemia
E tiology
mostlyunknown,retrovirus HTLV)mayLeassociatedwithT-cellleukemia
inliltrationolleukemiccells intoLonemarrow resultsinLonepain,andsuLsequentLone
marrowlailureanemia,neutropenia,thromLocytopenia,purpura,petechiae)
inliltrationintotissuesresultsin.lymphadenopathyhepatosplenomegalyCMSmanilestations
lever,latigue,weightloss
Prognosis
80-90, 5-year event-lreesurvivallorALL, 50, lorAML
Lymphoma
see Hematolo_
Hodgk in' s Lymphoma
incidenceisLimodal-peaksatageJ5-34and50
similartoadultHodgkins
mostcommonpresentationispersistent,painless,lirm,cervicalorsupraclavicular
lymphadenopathy
canpresentaspersistentcoughsecondarytomediastinalmass)orlesscommonlyas
splenomegaly, axillaryoringuinallymphadenopathy
constitutionalsymptoms 5symptoms)in30, olchildren
Non-Hodgk in' s Lymphoma
incidencepeaks /-JJ years
generallycategorizedintolymphoLlastic,largecell,and5urkitts / 5urkitts-likelymphoma
rapidlygrowingtumourwithdistantmetastaseswhichdillerslromadultnon-Hodgkin
lymphoma
signsand symptoms relatedto diseasesite, mostcommonlyaLdomen,chestmediastinal
mass),headandneckregion
E tiology
mostlyunknown,E5VassociatedwithAlrican5urkittlymphoma
Pediatrics P85
, ' *
' B Symptoms' = fever, ni ght sweats,

unexpl ai ned wei ght l oss
P86 Pediatrics Oncolog Toronto Notes 2010
Treatment
aggressivemultidrugchemotherapywithradiationandsurgerytodeLulklargetumourmasses
80-90,5-yearsurvivalinHodgkins,50-/5,innon-Hodgkins
Brain Tumours
seeMeurosurger_
classiliedLylocationandhistology
location.60,inlratentorialcereLellum,midLrain, Lrainstem)versussupratentorial
histology.glialcereLellarastrocytomasmostcommon),primitiveneuroectodermal
medulloLlastoma),neuronal,pineal
inlratentorial.increasedICPoLstructionol4thventricle),vomiting,morningheadache,
increasedheadcircumlerence,VInervepalsy, upward-gazingeyes,ataxia,cranialnerve
palsies
supratentorial. localdelicits,seizures,longtractsigns,visuallielddelects
evaluation
history, physicalexamincludingcompleteneurologicalexam
CTand/orMRIolhead
Wilms' Tumour (Nephroblastoma)
usuallydiagnosedLetween2and5years olage
mostcommonprimaryrenalneoplasmolchildhood
M=F
5-J0, olcasesLothkidneysareallectedsimultaneouslyorinsequence)
hydronephrosis,polycystickidneydisease, renalcellcarcinoma,neuroLlastoma
80,presentwithasymptomatic,unilateralaLdominalmass
mayalsopresentwithhypertension,grosshematuria,aLdominalpain,vomiting
mayhavepulmonary metastases attimeolprimarydiagnosisrespiratorysymptoms)
Associated Congenital Abnormalities
WACRsyndrome Wilms tumour, Aniridia, Genital anomalies,mentalRetardation)with
JJpJ3deletion
5eckwith-Wiedemannsyndrome
characterizedLyenlargementolLodyorgans,hemihypertrophy, renalmedullary
cysts,andadrenalcytomegaly
alsoatincreasedrisklordevelopinghepatoLlastoma,adrenocorticaltumours,
rhaLdomyosarcomas, andpancreatictumours
Denys-Drashsyndrome
characterizedLygonadaldysgenesisandnephropathyleadingtorenallailure
Management
nephrectomy
staging,chemotherapy,radiation
Prognosis
90,long-termsurvival
Neuroblastoma
E pidemiology
most common canceroccurringinlirstyearollile
neuralcrestcelltumourarisinglromsympathetictissuesneuroLlasts)
adrenalmedulla45,)
sympatheticchain25,retroperitoneal,20,posteriormediastinal,4,pelvis,4,neck)
canoriginate lromanysiteinsympatheticnervous system, presentingasmassinneck,
chestoraLdominal mass most commonsiteisadrenalgland)
signsand symptomsoldiseasevarywithlocationoltumour
thoracic.dyspnea,Hornerssyndrome
aLdomen. palpaLle mass
spinalcordcompression
metastasesarecommonatpresentation>50,presentwithadvancedstagedisease)
usuallytoLoneorLonemarrowpresentsasLonepain,limp)
Toronto Notes 2010 Oncolog
canalso present withperiorLitalecchymoses,aLdominalpain,emesis,lever, weight
loss, anorexia, hepatomegaly, LlueLerrymullin skinnodules
paraneoplastic.hypertension,palpitations,sweatinglromexcessivecatecholamines),
diarrhea,FTTlromVIPsecretion),opsomyoclonus
Investigations
C5C,electrolytes,LFTs,renallunctiontests, LDH,Ca,Mg, serumlerritin
urineVMA,HVAlevels
CTscanolchest,aLdomenandpelvis,Lonescan
Lonemarrowanalysis neuroLlastomacellsinrosettes
tissueLiopsy
Good Prognostic Factors
ageand stage areimportantdeterminantsolLetter outcome
<Jyearold
stageI,II,IV-SdiseaseSdesignatesaSpecialclassilicationonlypertainingto
inlants)
primary site. posteriormediastinumandneck
lowserumlerritin
specilichistology
tumourcellmarkers
aneuploidy
aLsentM-myconcogene amplilication
Management
depends onprognosticlactorsandmayincludeanyolthelollowingaloneorin
comLination. surgery radiation, chemotherapy, Lone marrow transplantation
prognosisisoltenpoorasitisloundatanadvancedstage
Rhabdomyosarcoma
thirdmost commonextracranialsolidtumourolchildren alterneuroLlastomaandWilms
tumour)
no clearpredisposingrisklactors
commonsitesoloriginarestructuresoltheheadandneck,CUtractandextremities
presentation.lirm,painlessmass
metastasestolung,LonemarrowandLones
evaluation.MRIorCTscanolprimarysite,CTchest,Lonescan,LilateralLonemarrow
aspiratesandLiopsies
treatment.multidrugchemotherapyandsurgery
Generalized Lymphadenopathy
leatures olmalignantlymphadenopathyLAD).lirm,discrete,non-tender,enlarging,no
associatederythema, warmth, lluctuance,suspicisousmass / imaginglindings, ` 5`
symptoms
inlection.
viral URTI,E5V,CMVAdenovirus,HIV
Lacterial S. aureus, CAS,anaeroLes,Mycobacterium, catscratchdisease
other. lungal,protozoan, rickettsia
auto-immune.rheumatoidarthritis,SLE,serumsickness
malignancy.lymphoma,leukemia,metastaticsolidtumours
storagediseases.Miemann-Pick,Cauchers
other. sarcoidosis,Kawasakidisease,histiocytoses
Investigations
generalizedLAD
C5C&dillerential,Lloodculture
uricacid,LDH
AMA,RF,ESR
E5V/ CMV/ HIVserology
toxoplasmatitre
lungalserology
CXR
PPD
Liopsy
regionalLAD
periodoloLservationinanasymptomaticchild
trialoloralantiLiotics
Liopsy especiallyilpersistent>6weeksand/or `5` symptoms)
Pediatrics P87
Most common cause of acute bi l ateral

cervi cal LD i s vi ral i l l ness.
PSS Pediatrics Respirolog Toronto Notes 2010
Respirology
Approach to Dyspnea
seeTaLleJ AverageVitalsatVariousAges, P3
F0l m00aty bat0lac l00t
I
Upper Airay Lower airay Pleura CHF 1 I CP
Forei gn body Tracheitis Pleural efusion Cardiac tamponade Ascites
Croup Bronchi olitis Empyema Pulmonary embol us Scol i osi s
Larngeal edema Pneumoni a Pneumothorax
Epiglottitis Atelectasis
Retropharngeal Asthma
abscess
Fi gure 6. Approach to Dyspnea i n Chi ldhood
Upper Respiratory Tract Diseases
seeOtolarngolo_
diseaseaLove thethoracicinletcharacterizedLyinspiratorystridor,hoarseness,and
suprasternalretractions
dillerentialdiagnosisolstridor
croup
Lacterialtracheitis
epiglottitis
loreignLodyaspiration
suLgloticstenosis-congenitaloriatrogenic
laryngamalacia/ tracheomalacia collapseorepiglottiscartilageoninspiration
Tabl e 49. Common Upper Respi ratory Tract I nfecti ons
Anatomy
Epidemiology
Etiology
Clinical
presentation
Investigations
Treatment
Croup (Laryngotracheobronchiis)
Subgl ottic l arngi ti s
Common
6 mo4 yrs
Peak i nci dence: fal l and early winter
Parai nfl uenza (75%)
I nfl uenza A and B
RSV
Adenovirus
Hoarse voice
Barki ng cough
Stridor
Worse at ni ght
Cl i ni cal di agnosi s
CXR i n atypi cal presentati on:
"steeple si gn" from subglottic
narrowing
Humi di fi ed O2
Dexamethasone: PO 1 dose
Racemi c epi nephri nes: nebul ized,
1 3 doses, q 1 2 hours
Intubati on i f unresponsi ve to treatment
Bacteral tracheits
Subglottic tracheitis
Rare
Al l age groups
S aurus
H infuenzae
al phahemolytic strep
Pneumococcus
Moraela catnalis
Si mi l ar symptoms as croup but
more rapi d deterioration wi th
hi gh fever
Toxi c appearance
Does not respond to croup
treatments
Endoscopy: defi ni ti ve di agnosi s
Start therapy for croup
Usual l y requires i ntubati on
Anti bi oti cs
Epiglotits
Supraglottic laryngi ti s
Rare
Usual ly older (26 yrs)
H. infuenzae
betahemolytic strep
Toxi c appearance
Rapid progression
Severe airay obstruction
Drool i ng
Stridor
Tri poi d posi ti on
Sternal recession
anxi ous
Avoid exami ni ng the throat to prevent
furher respi ratory exacerbation
Intubati on
Anti bi toi cs
Prevented wi th Hi b vacci ne
Lower Respiratory Tract Diseases
oLstructionolairways Lelowthoracicinlet,producesmoreexpiratorysounds
classicsymptom.wheezing
Toronto Notes 2010 Respirolog
Diferential Diagnosis of Wheez ing
common
asthma.recurrentwheezingepisodes,identiliaLletriggers
Lronchiolitis.lirstepisodeolwheezing
recurrentaspiration. oltenneurologicalimpairment
pneumonia.lever,cough,malaise
uncommon
loreignLody.acutewheezingandcoughing
cysticliLrosis. prolongedwheezing,unresponsiveto therapy
Lronchopulmonarydysplasia.oltendevelopsalterprolongedventilationinthenewLorn
rare
congestiveheartlailure
mediastinalmass
LronchiolitisoLliterans
tracheoLronchialanomalies
Pneumonia
inllammationolpulmonarytissue,associatedwithconsolidationolalveolarspaces
incidenceisgreatestinlirstyearollile
viralcauseismore commoninchildren<5years old
viral
cough, wheeze, stridor
CXR dilluse,streakyinliltratesLilaterally
Lacterial
cough,lever,chills,dyspnea
CXR loLarconsolidation,possiLlypleuralellusion
E tiology and Management
supportivetherapy.hydration, antipyretics,humidiliedO
Tabl e 50. Common Causes and Treatment of Pneumoni a at Di fferent Ages
Age Bacterial Viral Atyical bacteria Treatment
Neonates
' 3 months
3 months
5 years
>5 years
GBS
Lcol
Listeria
S aurus
H. infuenae
S. peumoniae
5.perussis
S peumoniae
S aurus
H. infuenae
GAS
S peumoniae
H. infuenae
S aurus
Bronchiolitis
CMV
Herpes vi rus
Enterovirus
CM\ RSV
I nfl uenza vi rus
Parai nfl uenza vi rus
RSV
Adenovirus
I nfl uenza vi rus
I nfl uenza vi rus
Vari cel l a
Adenovirus
Mvcoplasma hominis ampi ci l l i n + gentami ci n / tobramycin
Uraplasma uralycum (add erthromycin i f suspect Chlamvdia)
Chlamvdia trcomats cefuroxime OR ampi ci l l i n 7
Ureaplasma erthromycin OR clarithromycin
M. pneumoniae, TB ampi ci l l i n OR cefuroxime
Mi l d: PO amoxi ci l l i n
Mvcoplasma pneumoniae erthromycin OR clarithromycin
(most common) ( 1 st l i ne)
Chlamvdia pneumoniae OR ampi ci l l i n
TB OR cefuroxime
Legionela pneumophila
delinedas thelirstepisodeolwheezingassociatedwithURTIandsignsolrespiratorydistress
E pidemiology
common, allects 50, olchildreninlirst 2yearsollile
peakincidenceat6months, winterorearly spring
occursinchildrenpronetoairwayreactivity, increasedincidenceolasthmainlaterlile
E tiology
respiratorysyncytialvirus RSV)>50,)
parainlluenza,inlluenza,rhinovirus,adenovirus,rarelyM. pneumoniae
prodromeolURTIwithcoughandlever
leedingdilliculties, irritaLility
wheezing, respiratorydistress,tachypnea,tachycardia,retractions, poorairentrylasting
lor5-6days
Pediatrics P89
P90 Pediatrics Respirolog Toronto Notes 2010
childrenwithchroniclugdisease,severeCHDandimmunodeliciencyhaveamoresevere
courseoltheillness
Investigations
CXRonlyneededinsevere disease, poor responsetotherapy, chronicepisode)
airtrapping,periLronchialthickening,atelectasis,increasedlinearmarkings
nasopharyngealswaL
directdetectionolviralantigenimmunolluorescence)
WCusuallynormal
Treatment
milddistress
supportive.oralorIVhydration,antipyreticslorlever
humidiliedOmaintainO
sat>92,)
inaledbronchodilatorVentolin)0.03ccm3mlMSbymask,q20min, andthen
qJhour stopilno response
moderatetoseveredistress
asaLove- rarely, intuLationandventilation
ipratropium Atrovent)and steroids arenotellective
considerreLetolRiLavirin`)inhighriskgroups.Lronchopulmonarydysplasia,
CHD,congenitallungdisease,immunodelicient
monthlyRSV-IgorpalivizumaLmonoclonalantiLodyagainsttheF-glycoproteinolRSV)
mayollersomeprotectionagainstseverediseaseinhighriskgroups
caselatalityrate<J,
antiLioticshavenotherapeuticvalueunlesstherei ssecondaryLacterialpneumonia
indicationslorhospitalization
hypoxia.O
saturation<92,oninitialpresentation
ersistentrestingtachypnea>60/ minuteandretractionsalterseveralsalLutamol
entolin)masks
pasthistoryolchroniclungdisease,hemodynamicallysignilicantcardiacdisease,
neuromuscularproLlem,immunocompromised
younginlants<6monthsoldunlessextremelymild)
signilicantleedingproLlems
socialproLleme.g.inadequatecareathome)
Asthma
seeRes_irolo_
characterizedLyairwayhyperreactivity,Lronchospasmandinllammation,reversiLlesmall
airwayoLstruction
verycommon,presentsmostolteninearlychildhood
associatedwithotheratopicdiseasessuchasallergicrhinitisoratopicdermatitis
episodicLouts ol
wheezing
dyspnea
cough.atnight,earlymorning,withactivity, withcoldexposure
tachypnea
tachycardia
post-tussive emesis
physicalexammayrevealhyper-resonantchest,prolongedexpiration,wheeze
Triggers
URviralorMycoplasma)
weathercoldexposure,humiditychanges)
allergens pets), irritantscigarettesmoke)
exercise,emotionalstress
drugsaspirin,Leta-Llockers)
Classification
mildasthma
occasionalattacksolwheezingorcoughing 2 perweek)
symptomsrespondquicklytoinhaledLronchodilator
moderateasthma
morelrequentepisodeswithsymptomspersistingandchroniccough
decreasedexercisetolerance
severeasthma
dailyandnocturnalsymptoms
lrequentERvisitsandhospitalizations
Toronto Notes 2010
Management
acute
OtokeepO
saturation>92,
lluidsildehydrated
Respirolog
Leta2-agonists. salLutamolVentolin)0.03cc / kgin3ccMSq20minutesLymask
untilimprovement,thenmaskshourlyilnecessary
ipratropiumLromideAtrovent`)ilsevere.J ccaddedtoeachollirst3salLutamol
masks
steroids.prednisone2mg/ kginER,thenJmg/ kgPOODx4days)or
dexamethasone 0.3mg/ kg/ dayPO)
inseveredisease,givesteroidsimmediatelysinceonsetolactionisslow4hours)
indicationslorhospitalization
pre-treatmentOsaturation<92,
pasthistoryollile-threateningasthmaICUadmission)
unaLletostaLilizewithq4hmasks
concernoverenvironmentalissuesorlamilysaLilityto cope
chronic
education, emotional support, avoidanceolenvironmentalallergensorirritants,
developmentolanactionplan
exerciseprograme.g.swimming)
monitoringolrespiratorylunctionwithpeakllowmeter improvescompliance and
allowsmodilicationolmedication)
PFTslorchildren>6years
patientswithmoderateorsevereasthmawillneedregularprophylaxisinadditionto
Lronchodilatorse.g.dailyinhaledsteroids,long-actingLeta-agonists,
anticholinergics,sodiumcromoglycate,theophylline,leukotrienereceptorantagonist)
CanadianPaediatricAsthmaConsensusCuidelineslorassessingadequatecontrolol
childhoodasthma.
J.daytimesymptoms<4days / wk
2. nighttimesymptoms<Jnight / wk
3. normalphysical activity
4. nowork/schoolaLsenteeism
5. needlorLetaagonist<4doses /wk
Cystic Fibrosis (CF)
seeRes_irolo_
autosomalrecessive,CFTRgeneloundonchromosome/F508 mutationin/0,, over 800
dillerentmutationsidentilied)
Jin3,000liveLirths,mostlyCaucasians
mutationintransmemLraneconductanceregulatorolchloride causescellstoLe
impermeaLletoClwhichincreasesthe reaLsorptionolMa
leadstorelativedehydrationolairwaysecretions,resultinginimpairedmucociliary
transportandairwayoLstruction
neonatal
meconiumileus
prolongedjaundice
antenatalLowelperloration
inlancy
pancreaticinsulliciency withsteatorrheaandFTTdespitevoraciousappetite)
childhood
anemia,hypoproteinemia,hyponatremia
heatintolerance
wheezingorchroniccough
recurrentchestinlections(S. aureus, P. aeruginosa, H. injuenzae)
hemoptysis
nasalpolypsassociatedwithmilderdisease)
distalintestinaloLstructionsyndrome,rectalprolapse
cluLLingollingers
older patients
chronicoLstructive pulmonarydisease COPD)
inlertility
Investigations
sweatchloridetestx2>60mEq/L)
lalsepositivetests.malnutrition,celiacdisease,adrenalinsulliciency,anorexia
nervosa,hypothyroidism,nephrogenicdiaLetesinsipidus,nephroticsyndrome
lalsenegativetests. peripheraledema,cloxacillin,glycogenstoragedisease,
hypoparathyroidism, atopicdermatitis,Klineleltersyndrome,
hypogammagloLulinemia
Pediatrics P91
F '
CF Presenting Signs
CF PANCREAS
Chroni c cough and wheezi ng
Fai l ure to thri ve
Pancreati c i nsuffi ci ency (symptoms of
mal absorpti on l i ke steatorrhea)
Al kal osi s and hypotoni c dehydrati on
Neonatal i ntesti nal obstructi on
(meconi um i l eus)/Nasal pol yps
Cl ubbi ng of fi ngers/Chest radi ograph
wi th characteri sti c changes
Rectal prol apse
Electrolyte elevati on i n sweat, salty ski n
Absence or congeni tal atresi a of
vas deferens
Sputum wi th Staphor eadomouas
( mucoi d)
P92 Pediatrics Respirolog/Rheumatolog Toronto Notes 2010
pancreaticdyslunction determinedLy3-daylecallatcollection
genetics uselulwheresweatchloridetestisequivocal
Treatment
nutritionalcounselling
highcaloriediet
pancreaticenzymereplacements
latsoluLlevitaminsupplements
managementolchestdisease
physiotherapy,posturaldrainage
exercise
Lronchodilators
aerosolizedDMAase
antiLiotics.dependsonsputumC&S e.g. cephalosporin,cloxacillin, ciprolloxacin,
inhaledtoLramycin)
lungtransplantation
geneticcounselling
Complications
respiratorylailure
pneumothoraxpoorprognosticsign)
corpulmonalelate)
pancreaticliLrosiswithdiaLetesmellitus
gallstones
cirrhosiswithportalhypertension
inlertility
earlydeathcurrentmediansurvivalis30years)
Rheumatology
Evaluation of Limb Pain
Tabl e 51 . Diferential Di agnosi s of Li mb Pai n
Cause <3 year 31 0 year
Trauma x x
Infectious
Septi c arthritis x x
Osteomyel i ti s x x
Inflammatory
Transi ent synovitis x x
JIA x x
Seronegative spondyloarthropathy
SLE
Dermatomyositis
HSP x
AnatomicOrhopaedic
LeggCalvePerthes disease x
Sl i pped capi tal femoral epi physi s
OsgoodSchlatter di sease
Neoplastc
Leukemi a x x
Neuroblastoma x x
Bone tumour x
Hematologic
Hemophi l i a ( hemarthosi s) x x
Si ckle cel l anemi a x x
Pain Syndromes
Growi ng pai ns x
Fi bromyal gi a
Reflex sympathetic dystrophy
Investigations
C5C,dillerential,Lloodsmear,ESR
x-raysolpainluljoints / limLs
>1 0 years
x
x
x
x
x
x
x
x
x
x
x
x
x
x
x
x
x
x
asindicated.LloodC&S,AMA,RF,PTT, sickle cell prep, viralserology, immunogloLulins,
complement,urinalysis,synovialanalysisandculture,T5test,ASOT,slitlamp
Toronto Notes 2010 Rheumatolog
Septic Arthritis
medical emergency
hematogenousosteomyeliticspreadseenmostcommonlyinneonatesandinlants
clinicalpresentation. acutemonoarthritiswitherythema,warmth,swelling,intensepainon
passivemovementpainmayLesoseverethatitcausespseudoparalysisolinvolvedlimL),
leverandchills
delinitivetest.j ointaspirateandculture
management.properantiLioticselectionrequiresknowledgeollikelyLacterialpathogenat
variousages
Growing Pains
age2-J2years,M=F
diagnosis
intermittent,wellLetweenepisodes
poorlylocalizedpaininthelegs
usuallyLilateral
occursineveningorawakenschildatnight
respondstoreassurance, massage oranalgesics
resolves completelyinthemorning
no associatedsystemicsymptoms e.g. lever)
possiLlelamilyhistoryolgrowingpains
normalphysicalexamination
laLinvestigationsnotnecessaryiltypicalpresentation
Transient Synovitis
age3-J0years, M>F
Lenign,selllimited disorder,usuallyoccursalterupper respiratorytract inlection,
pharyngitis,Lronchitis, otitismedia
aleLrile orlow-gradelever,paintypically occurs inhips, knees,painlullimpLutstill
capaLleolamLulating
symptoms resolveover/-J0days
Investigations
ESR,W5Cwithinnormal limits
x-rayistypicallynormal
U/Smayshowjointellusion
mustexcludeseptic arthritis, osteomyelitis,AVM, slippedcapitallemoral epiphysis SCFE)
Treatment
symptomaticandanti-inllammatorymedications
Juvenile Idiopathic Arthritis (JIA)
lormerly knownas]uvenileRheumatoidArthritisgRA)
aheterogenousgroupolconditions characterizedLypersistentarthritisinchildrenunder
J6years
diagnosis.arthritisin Jjointslasting>6weeksinchild<J6yearswithexclusionolother
causesolarthritis
classilicationdelinedLyleatures/numLeroljointsallectedinthelirst6monthsolonset
Systemic Arhritis (Still' s disease)
highspikinglever 38C)lor atleast2weeks
extra-articularleatures.erythematoussalmon-colouredmaculopapularrash,
lymphadenopathy, hepatosplenomegaly,leukocytosis,thromLocytosis,anemia,serositis
pericarditis,pleuritis)
onsetatanyage,M=F
arthritismayoccurweekstomonthslater
highESR,CRPW5C,plateletcount
Oligoaricular Arthritis (arhritis of 1- 4 j oints in the first 6 months)
persistent allectsno more than4jointsduringthediseasecourse
extended allectsmorethan4jointsalterthelirstsixmonths
onset J-3yearsolage,F>M
typically allectslargejoints knees >ankles,elLows,wrists,hip involvementunusual
AMApositive-80,,rheumatoidlactor RF)negative
Pediatrics P93
-'
Microorganisms Involved in Septc

ArhritsOsteomyelis
Age Pathogens
Neonate G BS, S. evrevs,OMJ
Infant S|re. q.,S|e|.s.,
( 1 3 months I H, |n|venree
Pathogens as per neonate
Chi ld S.evrevs, S.nemcn|ee,
GAS
Adolescent
Si ckle cell
di sease
As above; also
.gcncrr|ceee
As above; also
Se|mcne|e
Puncture As above; also
wound of foot semcmcnes
GBS - group B Strep
GNB - Gramnegative bacilli
GA -group A Strep
P94 Pediatrics Rheumatolog Toronto Notes 2010
screeningeyeexamslorasymptomaticanterior uveitis occursin-20,)
complications.kneellexioncontracture,quadricepsatrophy, leg-lengthdiscrepancy,
growthdisturLances
Polyaricular Arthritis (arhritis of 5 or more j oints)
RFnegative
olteninvolveslargeandsmalljointsolhandsandleet,temporomandiLularjoint,
cervicalspine
patients, especiallythosewhoareAMApositive, arepronetochronicuveitis
RFpositive
similartotheaggressivelormoladultrheumatoidarthritis
severe,rapidlydestructive,symmetricalarthritisollargeandsmalljoints
mayhaverheumatoidnodulesatpressurepointselLows,knees)
unremittingdisease, persistsintoadulthood
E nthesitis- Related Arthritis
arthritisorenthesitisorLothwithatleasttwool
sacroiliactendernessand/orinllammatoryspinalpain
HLA52/positive
lamilyhistoryolconlirmedHLA52/-associateddiseaseinaJ'or2^'degree relative
symptomatic acute)anterioruveitis
onsetolarthritisinaLoy>8years
Psoriatic Arhritis
arthritisand psoriasisorarthritisandatleasttwool
dactylitis,nailaLnormalities,orlamilyhistoryolpsoriasisinaJ'degree relative
Unclassified
arthritisolunknowncause thatpersistslor6 weeks andeitherdoesnotlullillcriterialor
anycategoryorlullillscriterialormorethanonecategory
Management
childrenmaycomplainverylittleaLouttheirpainanddisaLility
exercisetomaintainrangeolmotionROM)andmusclestrength
multidisciplinaryapproachwithOT/ PT, socialwork,orthopaedics,ophthalmology,
rheumatology
lirstlinedrugtherapy. MSAIDs,intra-articularcorticosteroids
2ndline
DMARDs methotrexate,sullasalazine
othercorticosteroids intra-articular,systemiclorsystemiconsetol]IA, topicaleye
dropsloruveitis
Liologicagents etanerceptEnLrel`).LindsTMFandLlocksitsinteractionwithcell
surlacereceptors
Systemic Lupus Erythematosus (SLE)
seeRheumatolo_
autoimmuneillnessallectingmultipleorgansystems
incidenceJ. J000,morecommonlyage>J0, F.M* J0. J
Reactive Arthritis
arthritislollowsLacterialinlectionespeciallywithSalmonella, Shigella, Yersinia,
Campylobaeter, Chlamydia, andStreptococcus post-streptococcalreactivearthritis)
prognosis.
typicallyresolves
mayprogresstochronicillnessorReiter ssyndromeurethritis,conjunctivitis)
Lyme Arthritis
seeInlectiousDiseases
causedLyspirocheteBorrelia burgdorferi
incidencehigestamong5-J0yearolds
arthritisLeginsmonthsalterinitialinlectionlateLymedisease)
typicallyinvolveslargejoints,especiallykneesallectedin>90, olcases)
large,expandingerythematousmaculewithlever- erythemamigransolLymearthritis
management.doxycyclineoramoxicillinlor30days,donottreatchildren<8yearsold
withdoxycyclineasitmaycausepermanentdiscolourationolteeth
Toronto Notes 2010 Rheumatolog
Vasculitides
HENOCH SCH
O
NLEIN PURPURA (HSP)
mostcommonvasculitisol childhood
vasculitisolsmallvessels
peakincidence4-J0years,M.F- 2. J
recurrenceinaLoutonethirdolpatients
oltenhavehistoryolURTI J-3weeksLeloreonsetolsymptoms
skin.palpaLle,non-thromLocytopenicpurpurainlowerextremitiesandLuttocks,edema,
scrotalswelling
joints.arthritis/arthralgiainvolvinglargejoints
CI.abdommalpam,CIbleedmg,mtussusception
renal.IgAnephropathyhematuria,proteinuria,hypertension,renallailurein<5,
Management
symptomatic,corticosteroidsmayrelieveaLdominalpain
monitorlorrenaldisease,maydeveloplate
immunosuppressivetherapy lorsevere renaldisease
Prognosis
sell-limiteddiseasein90,
Kawasaki Disease
acutevasculitis olunknownetiology
mainlyallectingmedium-sizearteries
mostcommoncauseolacquiredheartdiseaseinchildren
peakage<5years,EastAsians >5lacks >Caucasians
Diagnostic Criteria
leverpersisting5daysormoreAMD
4olthelollowingleatures.
J.Lilateralnonpurulentconjunctivitis
2. redlissuredlips,strawLerrytongue,erythemaoloropharynx
3. changesoltheperipheralextremities
acutephase. erythema,edemaolhandsandleet
suLacutephase.peelinglromtipsollingersandtoes
4. polymorphousrash
5. cervicallymphadenopathy>J. 5cmindiameter
exclusionolotherdiseases e.g. scarletlever,measles)
atypicalKawasakidisease.lessthan5ol6diagnosticleaturesLutcoronaryartery
involvement
Associated Features
acutephase aslongasleverpersists,aLout J0days)
mostoldiagnosticcriteriapresent
irritaLilityasepticmeningitis,myocarditis,pericarditis,CHF
diarrhea,gallLladderhydrops,pancreatitis,urethritis,arthritis
suLacutephaseresolutionollever,peelingolskin,elevatedESRandplatelets,usually
days JJ-2J)
arthritis
Leau`slinesseenonnailgrowth
convalescentphaselastsuntilESRandplateletsnormalize,>2Jdays)
coronaryarteryaneurysms,aneurysmrupture,myocardialinlarctionMI),CHF
Management
highanti-inllammatory)dose olASAwhileleLrile
lowanti-platelet)doseolASAinsuLacutephaseuntilplateletsnormalizeorlongeril
coronaryarteryinvolvement
IVimmunogloLulin2g/ kg)reducesriskolcoronaryaneurysmlormation
Laseline2D-echoandlollowupperiodic2D-echocardiogramsusuallyat6weeks)
Complications
coronary artery vasculitiswithaneurysmlormationoccurs in20-25,oluntreated children,
<5,ilreceive IVICwithinJ0daysollever
50,olaneurysmsregresswithin2years
anticoagulationlormultipleorlargecoronaryaneurysms
risklactorslorcoronarydisease.male,age<Jor>9years, lever >J0days
Pediatrics P95
Diagnostic Criteria for

Kawasaki Disease
Warm CREAM
Fever " 5 days
Conj uncti vi ti s
Rash
Edema/Erythema (hands and feet)
Adenopathy
Mucosal i nvol vement
P96 Pediatrics Rheumatolog/Common Medications Toronto Notes 2010
Juvenile Dermatomyositis
Pathophysiology
activationol5 andT cells smallvesselvasculitisolskeletalmuscle withcharacteristic
skinmanilestations
E pidemiology
morecommoningirlsthanLoys
typicalageolonset4-J0yrs
Clinical Characteristics
insidiousprogressivelatigue,malaise
muscleweakness
primarilyallectingproximalmuscleshipsandshoulders)
dillicultyclimLingstairsandgettingupollolthelloorgower s)
low-gradelevers
rash
onlaceandacrosscheeks
shawlsignrashonshoulders /Lack)
heliotropediscolourationoleyelids
gottronpapulesscalyredplaquesonknucklesandextensorsurlaces)
periungualerythemaanddilatednailloldcapillaries
upperairwayinvolvementmaycausesvoicechangenasalvoice)anddysphagia
smallj ointarthritis
Treatment
systemic corticosteroids,andmethotrexatearethemainstaysoltreatment
mayalsoconsidercyclophosphamide, IVIC,andhydroxychloroquine
Prognosis
variaLle note. unlikeinadults,]DMisnotassociatedwithmalignancyinchildren)
Common Medications
Tabl e 52. Commonly Used Medications in Pediatrics
Drug Name Dosing Schedule Indicatons Comments
acetami nophen (Tyl enol ) 1 0 1 5 mglkgdose PO/PR q45h prn Anal gesi c, anti pyretic
amoxici l l i n (Amoxi l ) 80-90 mg/kg/day PO di vi ded q8h Otitis medi a (severe)
dexamethasone 0.5 mg/kg IV x 1 OR 1 mglkg PO x 1 Croup
0. 3 mg/kg/day PO Acute asthma
fl uti casone ( Fl ovent) moderate dose 250-500 Jgday di vi ded bid Asthma
high dose > 500 Jgday di vi ded bi d
i buprofen (Advi l ) 5- 1 0 mg/kg/dose PO q5-8h Anal gesi c, anti pyretic Cauti ous use in pati ents
wi th liver i mpai rment,
hi stor of GI bl eedi ng or
ul cers
iron 5 mg/kg/day el emental iron bi dti d Anemi a Si de effects: dark stool,
consti pati on, dark uri ne
predisone 1 -2 mg/kg/day PO x 5 days Asthma
3-4 mg/kg/day PO ITP
50 mgm2/day PO Nephrotic syndrome
sal butamol (Ventol i n) 0. 01 -0. 03 mL/kg/dose i n 3 mL normal sal i ne Acute asthma
vi a nebul izer ql /2-4h prn
1 00-200 Jg/dose prn, max frequency q4h Mai ntenance treatment
for asthma
From Lau, E. 120081 Te 2JJ-2JJJFormularv - Te Hospital for Sick Children
Toronto Notes 2010 References
References
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20082009 Drug Handbook and Formulary. In: Lau E, ed. Toronto: The Hospital for Si ck Children Department of Pharmacy; 2008.
Publicly Funded Immunization Schedules for Ontario January 2009.
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Nelson essentials of pediatrics. 5th ed. Philadelphia, PA. : Elsevier Saunders; 2006.
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American Diabetes Association. Type 2 Diabetes in Children and Adolescents. Pediatrics. 2000; 1 051 31 : 671 68 1 .
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http.//www. cdaadc.ca

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p

Safet of MMR Vaccine

oldLaLy, soother, carride,music,vacuum, checkdiaper

Characteristic Chest X-Ray Findings

A. Patent Ductus Arteri osus B. Transposi ti on of Great

Physical Exam for PDA

No "sale" l evel ofal cohol consumpti on

II a chi l d presents with pol yuri a and

Thyroi d neopl asms that devel op in

A chi l d with proven central precoci ous

Cal culate mi d-parental hei ght (predi cted

Tracheoesophageal Fistula (TEFl

Infectious and Inflammatory

4F's Diet for Chronic Diarrhea

Cel i ac di sease di et must avoi d gl uten

Cel i ac di sease i s associ ated with an

As many as 20% of chi l dren <5 years

Intussuscepton - Classic Triad

VOVi n chi l dhood vari es with age

8% of Afri can Ameri cans carry the HbS

Observation opti on onl y appropri ate i f

Sensitivity and Specificity of Urine

"Ground gl ass" appearance of l ungs is

alsoknownas wetlugsyndromeandrespiratorydistress syndrometypeII

sU. Zmg protei n

.hepatitis5&C, syphilis, HIV)

revalenceF.M- 2.J alterpuLerty

If a febri l e sei zure l asts > 1 5 mi nutes.

Causes of hypotoni a that respond to

I n neurocutaneous syndromes, the

' B Symptoms' = fever, ni ght sweats,

Most common cause of acute bi l ateral

Microorganisms Involved in Septc

Diagnostic Criteria for

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