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178 Cards in this Set

  • Front
  • Back
Top Childhood Cancers -
What are they
1. Leukemia (ALL)
2. CNS tumors
3. Lymphoma
4. Neuroblastoma
Neuroblastoma -
What is it
Associated with?
Embryonal tumor of
neural crest cell origin
MC cancer in infants
more than 1/2 kids < 2 y/o
associations -
neurofibromatosis
Hirschsprung's
n-myc oncogene
Neuroblastoma -
Hx/PE
Can occur anywhere
Sx vary with location
nontender abdom mass
(may cross midline)
Horner's syndrome
HTN
cord compression
anemia
FTT
fever

site-specific mets can cause-
proptosis
periorbital bruising
subq tumor nodules
bone pain with pancytopenia
opsoclonus/myoclonus
"dancing eyes, dancing feet"
Neuroblastoma -
Dx
Abdom CT
24-hr urinary catecholamines

assess extent of disease -
CXR
bone scan
CBC
LFTs
BUN/Cr
coag panel
Neuroblastoma -
Tx
■ Excision - localized tumors
■ chemo includes -
cyclophosphamide
doxorubicin
■ adjunctive radiation -
if tumor spread beyond origin
■ prognosis improved for
kids with low-risk dis. -
< 1 y/o
no N-myc amplification
localized
Wilms' Tumor -
What is it
Associated with?
Embryonal cancer of kidney
MC renal tumor in kids
2-4 y/o
associated with -
family Hx
Beckwith-Wiedemann syndrome
WAGR
neurofibromatosis
Wilms' Tumor -
Hx/PE
Painless, palpable abdom mass
does not cross midline
n/v
fever
weight loss
hematuria
HTN
Wilms' Tumor -
Dx
Abdom CT or US -
intrarenal mass

check for metas -
CXR
chest CT
CBC
LFTs
BUN/Cr
Wilms' Tumor -
Tx
■ Transabdom nephrectomy
■ postsurgical chemo -
vincristine
dactinomycin
■ flank irradiation (for some)
■ prognosis good -
depends on staging & histo
RDS -
What is it
MCC of resp failure
in premies
surfactant deficiency =>
inc. surface tension
(poor lung compliance)
and alveolar collapse
(atelectasis)
surfactant made by
T2 pneumocytes
mainly ~35th week
dipalmitoyl phospha-
tidylcholine

risk factors -
maternal diabetes
males
2nd born of twins
RDS -
Hx/PE
Presents in 48-72 hrs of life
RR > 60/min
intercostal retractions
expiratory grunting
nasal flaring
cyanosis
progressive hypoxemia
RDS -
Dx
ABGs
CBC (to r/o infection)
BC (to r/o infection)
CXR -
bilat diffuse atelectasis
causing ground-glass
appearance with
visible air bronchograms
lecithin:sphingomyelin ratio
< 2
RDS -
Tx
CPAP or
intubation and mech vent
artificial surfactant
to prevent -
mom gets corticosteroids
monitor fetal lung maturity
(L:S ratio)
RDS -
Complications
Persistent PDA
bronchopulmonary dysplasia
retinopathy of prematurity
intraventricular hemorrhage
NEC
Intussusception -
What is it
MCC of bowel obstruction in
1st 2 yrs. of life
males > females
usu prox to ileocecal valve
cause - idiopathic

in older kids -
mass or intest. abnormality
triggers the telescoping:
adenovirus or rotavirus
parasites
CF
celiac disease
polyps
intestinal lymphoma
Meckel's diverticulum
Henoch-Schonlein purpura
Intussusception -
Hx/PE
Hx -
colicky abdom pain
in apparently healthy kids
n/v
too young to talk -
cry
draw knees up to chest
dyspnea with pain

advanced signs -
red "current jelly" stool
lethargy
fever

PE -
abdom tenderness
pos. stool guaiac
palpable "sausage-shaped"
RUQ abdom mass
Intussusception -
Dx
Abdom XR
abdom US
air contrast barium enema
CBC
Intussusception -
Tx
Correct vol & electrolytes
check CBC

air-contrast barium enema -
diagnostic and therapeutic

surgical reduction or
resection (if gangrenous)
Pyloric Stenosis -
What is it
Hypertrophy of
pyloric sphincter
1st-born males more affected
Pyloric Stenosis -
Hx/PE
1st 2 wks - 4 mos. of life
nonbilious emesis
=> projectile emesis after
each feeding
so, babies feed well init
=> malnutrition & dehydration

palpable olive-shaped,
mobile, NT epigastric mass
visible gastric peristalsis
Pyloric Stenosis -
Dx
Abdom US - diagnostic

barium studies -
string sign
pyloric beak

hypochloremic, hypokalemic
metabolic alkalosis
Pyloric Stenosis -
Tx
First -
hydration
correct acid-base &
electrolyte abnormalities
NG tube - possible
long. pyloromyotomy
Child Abuse -
What is it
Neglect
physical abuse
sexual abuse
emotional abuse
suspect -
if Hx doesn't match
physical findings
if there was a delay in
getting medical care
Child Abuse -
Hx/PE
Infants may have apnea,
seizures, FTT

Exam findings include -
. cutaneous -
ecchymoses of varying ages
patterned injuries
. skeletal -
. spiral fractures of femur
and humerus in kids < 3
= abuse unless prove else
. epiphyseal/metaphyseal
injuries -
can happen in infants from
pulling/twisting limbs
. rib injuries < 2 y/o
. sexual abuse -
STDs or genital trauma
Child Abuse -
Dx
R/o conditions that mimic

skeletal survey & bone scan -
can show fractures in various
stages of healing

if sexual abuse suspected -
test for gonorrhea, chlamydia
and HIV

to r/o shaken baby syn -
check for retinal hemorrhages
CT for subdural hemorrhages
MRI for white matter changes
Child Abuse -
Tx
Document injuries
notify child protective svcs
hospitalize if nec.
Epiglottitis -
What is it
Serious, rapidly progressive
infection of supraglottic

before immunization -
from H influ type B

now -
Streptococcus
nontypable H flu
viral agents
Epiglottitis -
Hx/PE
Sudden-onset high fever
dysphagia
drooling
muffled voice
soft stridor
cyanosis
"sniffing dog" position
"tripod" position
insist on sitting up in bed
untreated - life-threatening
Epiglottitis -
Dx
Clinical
DON'T EXAMINE THROAT unless
anesthesiologist present
definitive Dx -
direct fiberoptic visual
of cherry-red, swollen
epiglottis & arytenoids
lat XR - thumbprint sign
Epiglottitis -
Tx
Emergency
call anesthesiologist
transfer pt. to OR
endotrach intubation or trach
IV ABx -
ceftriaxone or
cefuroxime
Croup (Laryngotracheobronchitis) -
What is it
Inflammation of larynx and
upper airway,
mainly subglottic space
=> narrowing of airway
kids 3 mos. - 3 yrs.
MCC - parainfluenza virus 1
also -
PIV-2, PIV-3, RSV, rubeola,
influenza, adenovirus,
Mycoplasma pneumonia
Croup (Laryngotracheobronchitis) -
Hx/PE
Prodrome - URI Sxs 1-7 days
stridor - worse by agitation
fever - low grade
hoarseness
barking cough
Croup (Laryngotracheobronchitis) -
Dx
Clinical
XR - steeple sign
Croup (Laryngotracheobronchitis) -
Tx
Mild - cool mist

moderate -
oral corticosteroids

severe -
(resp. distress at rest)
admit
nebulized racemic epi
Bronchiolitis -
What is it
Acute inflammation of
smallest airways
acute viral bronchiolitis
MCC - RSV
infants & kids < 2 y/o
can progress to resp. failure

risk for severe RSV -
< 6 mos. old
premies
heart or lung dis.
immunodeficiency
Bronchiolitis -
Hx/PE
Hx -
low-grade fever
rhinorrhea
cough
apnea - young infants

PE -
tachypnea
wheezing
hyperresonance to percussion
Bronchiolitis -
Dx
CXR - hyperinflation of lungs
interstitial infiltrates
atelectasis

ELISA of nasal washings
for RSV -
hi sens & spec
Bronchiolitis -
Tx
Mild -
outpt.
fluids, nebulizers, O2 if nd

admit if -
marked resp distress
O2 saturation < 95%
toxic appearance
dehydration/poor oral feeding
premie (< 34 wks)
< 3 mos. old
underlying cardiopulmon dis.
unreliable parents

inpatients -
contact isolation
hydration
O2
ribavirin

RSV prophylaxis -
RespiGam or Synagis
high-risk pts. in winter
Otitis Media -
What is it
Risk Factors
Middle ear infection
MCC -
#1 - S. pneumoniae
#2 - H. flu
#3 - Moraxella catarrhalis
kids predisposed - eusta tube

risk factors -
viral URIs
trisomy 21
CF
immunodeficiency
smoke exposure
day-care attendance
bottle feeding
cleft palate
prior otitis media
Otitis Media -
Hx/PE
Fever
ear tugging
hearing loss
irritability

Erythema
bulging
decreased mobility of tym memb
loss of light reflex and
bony landmarks
tym memb may be perforated
Otitis Media -
Dx
Clinical
Otitis Media -
Tx
Amoxicillin - 10 days
Tx failure after 3 days -
switch to
amoxicillin-clavulanic acid,
ceftriaxone or
cefuroxime
Otitis Media -
Complications
Mastoiditis
meningitis
hearing loss
cholesteatoma
tympanosclerosis
chronic suppurative OM
Kawasaki Disease
(Mucocutaneous Lymph Node Syndrome) -
What is it
Multisystem acute vasculitis
usually kids < 5 y/o
esp. Asian
at risk for coronary artery
aneurysms => MI
Kawasaki Disease
(Mucocutaneous Lymph Node Syndrome) -
Hx/PE
"CRASH and Burn"
Conjunctivitis - b/l
Rash
Adenopathy - cervical lymph
Strawberry tongue
Hands and feet -
swollen, red, desquamation
fever > 40C for > 5 days
Kawasaki Disease
(Mucocutaneous Lymph Node Syndrome) -
Dx
Clinical
thrombocytosis - wk 2 or 3
inc. ESR
Kawasaki Disease
(Mucocutaneous Lymph Node Syndrome) -
Tx
High-dose aspirin
IVIG - to prevent aneurysms

corticosteroids
are contraindicated
(they may inc. aneurysms)
FTT -
What is it
Persistent weight
below 3rd to 5th percentile
or falling off growth curve
organic - medical condition
nonorganic - psychosocial
nonorganic is MC
risk factors -
chronic illness
poverty
low maternal age
chaotic envi
genetic dis. (CF)
inborn errors of metabolism
HIV
FTT -
Hx/PE
Low weight for age and height
minimal weight gain
or weight loss
plot on growth chart
ck for signs of systemic dis.
diet Hx
observe caregiver-child
interaction
FTT -
Dx
Calorie count
CBC
electrolytes
Cr
albumin
total protein
sweat chloride test
UA/UC
stool culture
O&P
assess bone age
FTT -
Tx
Tx depends on cause
supplement nutrition if
breastfeeding inadequate
admit if -
neglect
severe malnourishment
Atrial Septal Defect (ASD) -
What is it
Opening in atrial septum
lets blood flow bet. atria
L to R shunting due to
lower R pressure
blood flow to lungs inc.
Atrial Septal Defect (ASD) -
Hx/PE
Hx -
usu. presents in
late childhd or early adult
onset & severity dep. on size
large defect -
tire easy (DOE)
freq. resp. infections
FTT
=> CHF => cyanosis

PE -
RV heave
wide, fixed split S2
systolic ejection murmur -
upper left sternal border
Atrial Septal Defect (ASD) -
Dx
Echo with color flow Doppler -
diagnostic
ECG - rt-axis deviation
CXR - cardiomegaly and
inc. pul vascular markings
Atrial Septal Defect (ASD) -
Tx
. Small defects may close
spontan (no Tx needed)
. ABx prophylaxis
before dental procedures
. surgical closure -
infants with CHF
pts. > 2:1 pul to sys bld flow
correct early to prevent -
arrhythmia
RV dysfunction
Eisenmenger's syn
Ventric Septal Defect (VSD) -
What is it
MC congenital heart defect
More common in pts. with -
Apert's syn
Cri-du-chat
Trisomies 13 & 18
Ventric Septal Defect (VSD) -
Hx/PE
Hx -
Sxs dep. on degree of shunting
small def usu asymp at birth
large def -
CHF
freq resp infections
FTT

PE -
pansystolic murmur -
lower lt sternal border
loud pulmonic S2
in severe defects -
systolic thrill
cardiomegaly
crackles
Ventric Septal Defect (VSD) -
Dx
Echocardiogram - diagnostic
EKG -
may show RVH or LVH
normal with small VSDs
Ventric Septal Defect (VSD) -
Tx
. Most small defects close
spontan (no Tx needed)
. ABx prophylaxis before dental or pulmonary procedures
. surgical closure -
correct early to prevent -
Eisenmenger's syn, et al
. Tx CHF & resp infections
Patent Ductus Arteriosus (PDA)
What is it
Failure of DA to close
=> L-to-R shunt
(aorta to pulmonary artery)
risk factors -
high altitude (low O2)
1st trimester rubella in mom
premies
females (more common)
Patent Ductus Arteriosus (PDA)
Hx/PE
Hx -
typically asymp (small PDA)
slowed growth
recurrent lwr resp infections
lwr extremity clubbing
CHF Sxs

PE -
wide pulse pressure
continuous machine murmur
loud S2
bounding periph pulses
Patent Ductus Arteriosus (PDA)
Dx
Small -
often no signs of cardiomeg

Large -
echocardio - LA & LV enlarged
EKG - LVH
CXR - cardiomegaly

color-flow doppler- diagnostic
Patent Ductus Arteriosus (PDA)
Tx
Indomethacin
(unless need PDA for survival)
if indomethacin fails or
child > 6-8 mos. old -
surgical closure preferred
Coarctation of the Aorta -
What is it
Narrowing prox or distal to DA
=> inc. flow above,
dec. flow below coarctation
more common in males
Turner's
25% have bicuspid aortic valve
Coarctation of the Aorta -
Hx/PE
Hx -
often presents in childhood
with asymp HTN
headache
syncope
epistaxis
DOE
claudication

PE -
systolic BP higher in upr ext
may be gtr in right arm
femoral pulses weak or delayed
late systolic murmur in
left axilla
apical impulse forceful
advanced cases -
well-developed upper body
lwr ext wasting
Coarctation of the Aorta -
Dx
EKG - LVH
echocardiography
color-flow doppler
CXR - "3" sign
rib notching
aortography - diagnostic
(cardiac catheterization)
Coarctation of the Aorta -
Tx
Surgery or
balloon angioplasty
endocarditis prophylaxis
Transposition of the
Great Arteries -
What is it
Pulmon & sys circ in parallel
aorta connected to RV
pulmon artery connected to LV

incompatible with life unless
septal defect or PDA

risk factors -
babies of DM moms
Apert's syn
Down's
cri-du-chat
Trisomies 13 & 18
Transposition of the
Great Arteries -
Hx/PE
Critically ill
cyanosis immed after birth
tachypnea
prog. resp failure
CHF (some pts.)
Transposition of the
Great Arteries -
Dx
Echocardiography
CXR -
"egg-shaped sihouette"
"egg on a string"
"apple on a string"
Transposition of the
Great Arteries -
Tx
Prostaglandin E1 (PGE1) -
to keep PDA open
balloon atrial septostomy
arterial or atrial switch op
Tetralogy of Fallot -
What is it
VSD
pulmonary stenosis
RVH
overriding aorta
rt-to-lt shunting
=> early cyanosis
risk factors -
Down's
cri-du-chat
Trisomies 13 & 18
Tetralogy of Fallot -
Hx/PE
Hx -
cyanosis
dyspnea
fatigability
profound cyanosis = tet spell
squatting for relief
hypoxemia => FTT,
mental status changes

PE -
SEM at left sternal border
RV lift
single S2
CHF signs possible
Tetralogy of Fallot -
Dx
Echocardiography
catheterization

CXR -
boot-shaped heart
dec. pulmon vascular markings

EKG -
rt-axis deviation
RVH
Tetralogy of Fallot -
Tx
PGE1 - keep reopen PDA

for cyanotic spells -
O2
propanolol
knee-chest position
fluids
morphine

balloon atrial septostomy
before surgical correction
Cerebral Palsy (CP) –
What is it
Group of nonprogressive, nonhereditary neurological d/o
d/o in movement and posture
MC movement d/o in kids
MCC unknown -
prenatal, perinatal
and post insults

risk factors –
prematurity
perinatal asphyxia
intraut. growth retardation
early infection or trauma
brain malformation
neonatal cerebral hemorrh
Cerebral Palsy (CP) –
What are the categories
Spastic (pyramidal) -
spastic paresis of any limb
75% of cases
MR up to 90%

athetoid -
extrapyramidal, b. ganglia
uncontrollable jerking
writhing
worse with stress
disappears during sleep

ataxic -
cerebellum
hard to coordinate movement
wide-based gait

mixed
Cerebral Palsy (CP) –
Hx/PE
May be associated with -
seizure d/o
behavioral d/o
hearing or vision impaired
learning disabilities
speech deficits

hyperreflexia
Babinski
inc. tone/contractures
weakness
underdevelopment
toe walking
scissor gait
hip dislocations
scoliosis
Cerebral Palsy (CP) –
Dx
Clinical
r/o metabolic d/o,
cerebellar dysgenesis,
spinocerebellar degen
EKG (if seizures)
Cerebral Palsy (CP) –
Tx
Special ed
physical therapy
braces
surgical rel of contractures

for spasticity -
diazepam
dantrolene
baclofen

for severe contractures -
baclofen pumps
posterior rhizotomy
Febrile Seizures -
What is it
In kids 6 mos. - 6 y/o
no evidence of intracranial
infection or other cause
risk factors -
rapid rise in temp
Hx in close relative
Febrile Seizures -
Hx/PE
Most are simple seizures

simple -
high fever
fever onset within hrs of sz
generalized seizure
lasts < 15 min.
1 in a 24-hr period


complex -
low-grade fever
fever for several days before
seizure onset
seizure has focal features
can have postictal paresis
lasts > 15 min.
> 1 in a 24-hr period
Febrile Seizures -
Dx
Find source of infection
LP - if signs of CNS infection
No labs if presentation
consistent with febrile sz

Atypical presentation -
electrolytes
glucose
BC
UA
CBC with diff
EEG & MRI - complex seizures
Febrile Seizures -
Tx
Simple -
aggressive antipyretics
tx underlying illness

complex -
thorough neuro exam
chronic anticonvulsants may
be necessary
Febrile Seizures -
Complications
Febrile Sz will recur in 30%
no inc. risk of epilepsy,
developmental, intellectual
or growth abnorm

those with complex seizures -
10% risk of dev. epilepsy
Neonatal Jaundice -
What is it
What are the types
What is kernicterus
Inc. serum bilirubin
from inc. production or
dec. excretion
conjugated - always pathologic
uncon - patholog or physiolog

physiologic jaundice -
not present until 72 hrs
after birth
bilirubin peaks < 15 mg/dL
resolves by 1 wk in term
resolves by 2 wks in premies

pathologic jaundice -
present in 1st 24 hrs of life
bilirubin rises to > 15 mg/dL
persists past 1 wk in term
persists past 2 wks in premies

kernicterus -
unconjug hyperbilirubinemia
bilirubin deposits in pons,
basal ganglia, cerebellum
irreversible
can be fatal
risk factors -
premies
asphyxia
sepsis
Neonatal Jaundice -
Hx/PE
Hx -
child breastfed or formula?
intrauterine drugs
fam Hx of -
hemoglobinopathies
enzyme def.
RBC defects

Sxs -
abdom distention
delayed passage of meconium
light-colored stools
dark urine
low Apgar scores
weight loss
vomiting

kernicterus -
lethargy
poor feeding
high-pitched cry
hypertonicity
seizures

jaundice may be cephalopedal
check for signs of -
infection
congen malformations
cephalohematomas
bruising
pallor
petechiae
hepatomegaly
Neonatal Jaundice -
Dx
CBC
periph blood smear
blood type mom and baby
Coombs' test
bilirubin levels

direct hyperbilirubinemia -
LFTs
bile acids
BC
sweat test
tests for aminoacidopathies
& a1-antitrypsin deficiency

Sepsis w/u and ICU -
jaundice
febrile
hypotensive
and/or tachypneic
Neonatal Jaundice -
Tx
Tx underlying cause

unconjugated -
severe - exchange transfusion
if mild - phototherapy
start earlier for premies
(start at 10-15 mg/dL)
Down Syndrome -
What is it
Trisomy 21
MC chromosome d/o
#2 cause of congen MR
risk inc. with mom's age, but
80% of kids are born to
women < 35 y/o
flat facial profile
prominent epicanthal folds
simian crease
dec. levels of AFP
brushfield spots
duodenal atresia -
double bubble on US/XR
congen heart disease -
septum primum-type ASD due to
endocardial cushion defect
Alzheimer's > 35 y/o
inc. risk of ALL
meiotic nondisjunction
Edwards' Syndrome -
What is it
Trisomy 18 (election age=18)
severe MR
rocker bottom feet
low-set ears
micrognathia
congen heart dis
clenched hands
prominent occiput
death usu < 1 y/o
Patau's Syndrome -
What is it
Trisomy 13 (puberty=13)
severe MR
microphthalmia
microcephaly
cleft lip/palate
abnorm forebrain
polydactyly
congen heart disease
death usu < 1 y/o
Klinefelter's Syndrome -
What is it
XXY (male)
inactivated X (Barr body)
1 of MCC of male hypogonadism
testicular atrophy
eunuchoid body shape, long
long extremities
gynecomastia
female hair distribution
Turner' Syndrome -
What is it
XO (No Barr body)
short stature
ovarian dysgenesis
webbing of neck
cystic hygroma
coarctaton of aorta
MCC of primary amenorrhea
Double Y males -
What is it
XYY
phenotypically normal
very tall
severe acne
antisocial behavior
Phenylketonuria (PKU) -
What is it
Phenylalanine => tyrosine
In PKU, dec. phenylalanine
hydroxylase or
tetrahydrobiopterin cofactor
tyrosine becomes essential
phenylalanine builds up
=> excess phenylketones

phenylketones -
phenylacetate
phenyllactate
phenylpyruvate

MR
fair skin
eczema
musty body odor
screened for at birth
Tx - dec. phenylalanine
(in Nutrasweet) and
inc. tyrosine in diet
Fabry's Disease -
(lysosomal storage disease)
What is it
X-linked recessive
def. of a-galactosidase A
ceramide trihexoside accums
renal failure
Krabbe's Disease -
(lysosomal storage disease)
What is it
AR (autosomal recessive)
def. of B-galactosidase
galactocerebroside accums
in the brain
optic atrophy
spasticity
early death
Gaucher's Disease -
(lysosomal storage disease)
What is it
AR
def. of B-glucocerebrosidase
glucocerebroside accums in
brain, liver, spleen, bm
Gaucher's cells -
"crinkled paper" enlarged
cytoplasm
Type I - more common, compa-
tible with normal life span
Niemann-Pick Disease -
(lysosomal storage disease)
What is it
AR
def. of sphingomyelinase
sphingomyelin and cholesterol
build up in reticuloendo-
thelial & parenchymal cells
cherry-red spot on macula
death by age 3
Tay-Sachs Disease -
(lysosomal storage disease)
What is it
AR
absence of hexosaminidase A
GM2 ganglioside accums
cherry-red spot on macula
death by age 3
MC lysosomal storage disease
that causes MR
Metachromatic leukodystrophy -
(lysosomal storage disease)
What is it
AR
def. of arylsulfatase A
sulfatide accums in brain,
kidney, liver, periph n.
Hurler's Syndrome -
(lysosomal storage disease)
What is it
AR
def. of a-L-iduronidase
corneal clouding
MR
Hunter's Syndrome -
(lysosomal storage disease)
What is it
X-linked recessive
def. of iduronate sulfatase
mild form of Hurler's
no corneal clouding
mild MR
Fragile X Syndrome -
What is it
X-linked
3rd MCC of MR
FMR1 gene affected
anticipation
triple repeat of CGG
autism
large testes, jaw, ears
floppy/prolapsed mitral valve
APGAR Score -
Chart
Appearance -
skin color
0 = blue all over
1 = blue at extremities
2 = normal

Pulse -
0 = none
1 = < 100
2 = > 100

Grimace -
reflex irritability
0 = none
1 = grimace, feeble cry
2 = sneeze, cough, pull away

Activity -
muscle tone
0 = none
1 = some flexion
2 = active movement

Respiration -
0 = none
1 = weak or irreg
2 = strong
APGAR Score -
What do total scores mean
Score at 1 min. after birth
then at 5 min.

score 8-10 -
good cardiopulm adaptation

score 4-7 -
possible need for resus
observe
stimulate
possible need for vent support

score 0-3 -
resus immed
Erythema Infectiosum -
(Fifth Disease)
Cause
Characteristics
Cause -
parvovirus B19

prodrome none
fever absent or low-grade

"slapped cheek" erythematous
pruritic
maculopapular rash
goes to arms
spreads to trunk and legs
worse with fever and sun
Erythema Infectiosum -
(Fifth Disease)
Complications
Arthritis
hemolytic anemia -
aplastic crisis in sickle cell
encephalopathy
associated c hydrops fetalis
Measles -
Cause
Characteristics
Paramyxovirus

prodrome -
low-grade fever
conjunctivitis
coryza
cough

Koplik's spots -
buccal mucosa after 1-2 days
maculopap rash from ears down
Measles -
Complications
Giant cell pneumonia
otitis media
laryngotracheitis
rare -
subac scleros. panencephalitis
Rubella -
Cause
Characteristics
Rubella virus

prodrome -
asymp or
tender, generalized
lymphadenopathy

erythematous, tender,
maculopapular rash
slight fever
polyarthritis in adolescents
Rubella -
Complications
Encephalitis
thrombocytopenia
congen infections associated
with congen anomalies
Roseola Infantum -
Cause
Characteristics
HHV-6
prodrome -
acute onset of high fever
no other Sxs for 3-4 days
maculopap rash as fever breaks
starts on trunk
=> face and extremities
often lasts < 24 hrs
Roseola Infantum -
Complications
Rapid fever onset =>
febrile seizures
Rotavirus -
Characteristics
Primary cause of diarrhea
in kids < 2
fever and vomiting
then diarrhea
upper resp Sxs
lasts < 1 wk
infection confirmed by Elisa
oral rehydration sufficient
Varicella -
Cause
Characteristics
VZV

prodrome -
mild fever
anorexia
malaise
precedes rash by 24 hrs

generalized, pruritic,
"teardrop" vesicular rash
starts on trunk
spreads to periphery
lesions often at different
stages of healing

infectious from 24 hrs
before eruption til
lesions crust over
Varicella -
Complications
In immunocompromised kids -
progressive varicella with
meningoencephalitis
and hepatitis

congen infections =>
congen anomalies
Varicella Zoster -
Cause
Characteristics
Prodrome -
reactivation of Varicella
infection
starts as pain along affected
sensory n.

pruritic "teardrop"
vesicular rash
in dermatomal distribution

uncommon unless immunocomp
Varicella Zoster -
Complications
Encephalopathy
aseptic meningitis
pneumonitis
TTP
Guillain-Barre
cellulitis
arthritis
Hand-Foot-and-Mouth Disease -
Cause
Characteristics
Coxsackie A

prodrome -
fever
anorexia
oral pain

rash -
oral ulcers
maculopap vesicular rash on
hands, feet, buttocks
Hand-Foot-and-Mouth Disease -
Complications
None
(self-limited)
Tracheoesophageal Fistula -
What is it
Tract between trachea & esoph
associated with esoph atresia
& VACTERL anomalies -
vertebral
anal
cardiac
tracheal
esophagus
renal
limb
Tracheoesophageal Fistula -
Caused by
Presentation
Polyhydramnios in utero

inc. oral secretion
inability to feed
gagging
resp distress
Tracheoesophageal Fistula -
Dx
CXR after NGT
air in GI tract
bronchoscopy - to confirm
Tracheoesophageal Fistula -
Tx
Surgical repair
Congenital Diaph Hernia -
What is it
GI tract segment protrudes
thru diaph into thorax
90% are post. lt. Bochdalek
Congenital Diaph Hernia -
Presentation
Resp distress from -
pulmonary hypoplasia
pulmonary HTN
sunken abdomen
bowel sounds over
lt. hemithorax
Congenital Diaph Hernia -
Dx
US in utero
postnatal CXR - to confirm
Congenital Diaph Hernia -
Tx
Hi-freq ventilation or
extracorporeal membrane
oxygenation (ECMO)
(to manage pulmonary HTN)
surgical repair
Gastroschisis -
What is it
Herniation of intestine
thru abdom wall
next to umbilicus
(usually on right)
with no sac
Gastroschisis -
Caused by
Associated with
Polyhydramnios in utero
often premie
associated with -
GI stenoses
GI atresia
Gastroschisis -
Tx
Surgical emergency
single-stage closure possible
in only 10%
Omphalocele -
What is it
Herniation of abdom viscera
thru abdom wall
at umbilicus
into sac covered by
peritoneum and amniotic memb
Omphalocele -
Caused by
Associated with
Polyhydramnios in utero
often premie
associated with
other GI & cardiac defects
Omphalocele -
Tx
C-section -
to prevent sac rupture

if sac intact -
postpone surg correction
until pt. fully resuscitated

keep sac covered/stable
with petroleum & gauze

intermittent NG suction -
to prevent abdom distention
Duodenal Atresia -
What is it
Complete or partial failure
of duodenal lumen
to recanalize
during gestational wks 8-10
Duodenal Atresia -
Presentation
Caused by
Associated with
Bilious emesis within hrs
after 1st feeding

polyhydramnios in utero
associated with -
Down's
other cardiac/GI anomalies:
annular pancreas
malrotation
imperforate anus
Duodenal Atresia -
Dx
Double-bubble sign on XR
(prox to site of atresia)
Duodenal Atresia -
Tx
Surgical repair
Meckel's Diverticulum -
What is it
MC congen GI tract anomaly
vestigial remnant
of omphalomesenteric duct

rule of 2's -
2x's as many males
2 ft. from ileocecal valve
2% of people affected
2 types of mucosa -
gastric
pancreatic
Meckel's Diverticulum -
Presentation
MC presentation -
painless rectal bleeding

painful diverticulitis
intest. obstruction from -
intussusception or volvulus
Meckel's Diverticulum -
Dx
Meckel's scan -
for ectopic gastric mucosa
uses IV technetium
pertechnetate
Meckel's Diverticulum -
Tx
Surgery
Hirschsprung's Disease -
(Congen Aganglionic Megacolon)
What is it
Absence of autonomic
innervation of bowel wall
inadeq relaxation
and peristalsis
=> intest. obstruction
Hirschsprung's Disease -
(Congen Aganglionic Megacolon)
Presentation
Abdom distention
bilious vomiting
fail to pass meconium in
1st 24 hrs of life
Hirschsprung's Disease -
(Congen Aganglionic Megacolon)
Dx
Barium enema -
dilated prox segment
narrowed distal segment
rectal Bx - to confirm
Hirschsprung's Disease -
(Congen Aganglionic Megacolon)
Tx
Colostomy prior to
corrective surgery
Hypospadias -
What is it
Abnorm urethral opening
on ventral surface of penis
due to incomplete dev of
distal urethra
Hypospadias -
Presentation
Associated with
Chordee
associated with -
hernias
cryptorchidism
Hypospadias -
Tx
Circumcision contraindicated
surgical repair uses
preputial tissue
X-linked Agammaglobulinemia
(Bruton's Disease) -
What is it
B-cell def.
boys only
may present < 6 mos. of age
at risk for life-threatening
Pseudomonas infections
X-linked Agammaglobulinemia
(Bruton's Disease) -
Dx
No B cells
low levels of all Ab classes
X-linked Agammaglobulinemia
(Bruton's Disease) -
Tx
IVIG
prophylactic ABx
Common Variable Immunodeficiency -
What is it
Ig levels drop in
2nd-3rd decade of life
inc. risk of lymphoma
inc. risk of autoimmune dis.
Common Variable Immunodeficiency
Dx
Ig levels
antibody titers
Common Variable Immunodeficiency
Tx
IVIG
prophylactic ABx
IgA Deficiency -
What is it
MC immunodeficiency
usually asymptomatic
may have recurrent infections
DiGeorge Syndrome
(Thymic aplasia) -
What is it
CATCH-22
Cardiac defects
Abnormal facies
Thymic hypoplasia
Cleft palate
Hypocalcemia
22 - microdeletions in chrom22

tetany in first days of life
DiGeorge Syndrome
(Thymic aplasia) -
Dx
Absolute lymphocyte count,
mitogen stimulation response
and delayed hypersensitivity
skin testing
DiGeorge Syndrome
(Thymic aplasia) -
Tx
BMT - if severe
IVIG
thymus transplant
Ataxia-Telangiectasia -
What is it
DNA repair defect
oculocutaneous telangiectasias
progressive cerebellar ataxia
Ataxia-Telangiectasia
Tx
No effective Tx for CNS abnorm
neuro deterioration progresses
death by 30 y/o
Severe Combined
Immunodeficiency (SCID) -
What is it
Severe lack of B & T cells
freq. severe bact. infections
chronic Candidiasis
opportunistic organisms
Severe Combined
Immunodeficiency (SCID) -
Tx
BMT or stem cell transplant
IVIG
PCP prophlaxis until BMT
gene therapy may be
future option
Wiskott-Aldrich Syndrome -
What is it
X-linked recessive
T & B cell dysfunction
thrombocytopenia
small-sized platelets
eczema
high IgE
high IgA
low IgM

bloody diarrhea
bleeding gums
prolonged nosebleeds
Wiskott-Aldrich Syndrome -
Tx
Protective helmet
IVIG
aggressive ABx for infections
HLA-identical BMT
if no BMT -
rarely survive to adulthood
Chronic Granulomatous Disease-
(CGD)
What is it
X-linked or AR
deficient superoxide
production by PMNs & M0s

usual sites of infection -
skin
lungs (pneumonia)
lymph nodes
liver (abscesses, hepatitis)
bones (osteomyelitis)

swollen collections of
infected tissue obstruct
intestines (IBD) and
urinary tract (UTIs)
Chronic Granulomatous Disease-
(CGD)
Dx
Absolute neutrophil count
and adhesion

assays -
chemotaxic
phagocytic
bactericidal

diagnostic -
neg. nitroblue tetrazolium
dye reduction test (NBT)
Chronic Granulomatous Disease-
(CGD)
Tx
Daily TMP-SMX
judicious ABx use during
infections

IFN-g -
can dec. incidence of
serious infection
Chediak-Higashi -
What is it
AR
giant lysosomal granules dev.
in neutrophils
lysosomes can't fuse with
phagosomes =>
ingested bact. can't be lysed
oculocutaneous albinism
neuropathy
neutropenia
C1 Esterase (Inhib) Def. -
(Hereditary Angioedema)
What is it
C1 inhibitor -
acute phase protein
inhibits proteinases of:
complement pathway
clotting pathway
kinin generator pathway
fibrinolytic pathway

deficiency => her. angioedema
AD
can affect -
hands & feet - local edema
bowel - extreme abdom pain
mouth
airway - life-threat. edema

usually lasts 3 days
can be precip by trauma, virus
aggravated by stress
C1 Esterase (Inhib) Def. -
(Hereditary Angioedema)
Dx
Total hemolytic C' assay- CH50
if defect in one component -
no CH50 reduction
then det. which component
C1 Esterase (Inhib) Def. -
(Hereditary Angioedema)
Tx
Daily prophylactic danazol
purified C1 esterase and FFP -
prior to surgery
Terminal Complement Deficiency
(C5-C9) -
What is it
AR
recurrent N. meningitidis
& dissem. gonorrhea infections
rarely - systemic lupus
Terminal Complement Deficiency
(C5-C9) -
Tx
Meningococcal vaccine
appropriate ABx
Hyper IgM Syndrome -
What is it
MC - def. in CD40 ligand
in T Helper
=> can't class switch
normal or high IgM
low IgG, IgA, IgE
XL recessive (most common)
AR (others)
severe upr & lwr respiratory
diarrhea - Cryptosporidium
Hyper IgM Syndrome -
Tx
IVIG
PCP prophylaxis - TMP-SMX