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259 Cards in this Set

  • Front
  • Back
Lifts head / chest when prone
2 months
Tracks past midline
2 months
Alerts to sounds; coos
2 months
Recognizes parent; social smile
2 months
Rolls front to back; back to front
5 months
Grasps rattle
4-5 months
Orients to voice; makes consonant sounds; razzes
4-5 months
Enjoys looking around; laughs
4-5 months
Sits unassisted
6 months
Transfers objects; raking grasp
6 months
Babbles
6 months
Stranger anxiety
6 months
Crawls; pulls to stand
9 - 10 months
uses three finger pincer grasp
9 - 10 months
says "mama/dada"
9-10 months
waves bye-bye; plays pat-a-cake
9-10 months
cruises
11 months
walks alone
12 months
uses two finger pincer grasp
12 months
says "mama/dada"
12 months
imitates action
12 months
separation anxiety
12 months
walks backwards
15 months
uses cup
15 months
uses 4 - 6 words
15 months
temper tantrums
15 months
runs
18 months
kicks a ball
18 months
builds tower of 2-4 objects
18 months
names common objects
18 months
may start toilet training
18 months
walks up and down steps w/ help
2 years
jumps
2 years
builds tower of six cubes
2 years
uses two-word phrases
2 years
follows two step commands
2 years
removes clothes
2 years
rides tricycle
3 years
climbs stairs w/ alternating feet
3 years
copies a circle
3 years
uses utensils
3 years
uses three word sentences
3 years
brushes teeth with help
3 years
washes / dries hands
3 years
hops
4 years
copies square
4 years
knows colors and some numbers
4 years
cooperative play
4 years
plays board games
4 years
skips
5 years
walks backward for long distances
5 years
ties shoe laces
5 years
knows left and right
5 years
prints letters
5 years
uses five word sentences
5 years
domestic role playing
5 years
plays dress-up
5 years
Ventricular septal defect
harsh holosystolic murmur
most close spontaneously
Surgery for symptomatic, children < 1 w/ pulmonary hypertension
Atrial septal defect
fixed splitting of S2,
often with right ventricular hypertrophy from L to R shunt
CXR: cardiomegaly and increased pulmonary vascular markings
Tx: most close spontaneously
Patent ductus arteriosus
continuous "machinery" murmur, loud s2, bouding peripheral pulses

Tx give indomethacin to close PDA unless needed for survival; surgery if indomethacin fails
When do you need the PDA opened
Tetralogy of Fallot
Transposition of the great Vessels
Hypoplastic Left Heart
Coarctation of the aorta
asymptomatic hypertension with BP higher in upper extremities; difference in BP between left and right arm

Tx: open w/ PGE1
Transposition of the Great Vessel
cyanosis immediately after birth. Single loud S2.
CXR w/ absence of main pulmonary artery, and increased pulmonary vascular markings.

Tx: 1. IV PGE1 to open PDA
2. Surgery
Tetrology of Fallot
Pulmonary Stenosis
Right ventricular hypertrophy
Overriding aorta
Ventricular septal defect

Most common cyanotic congenital heart disease in children. Seen w/ Tet spells

Tx:
1. PGE1
2. Temporary palliation w/ shunt before surgical correction
X-linked agammaglobulinemia
B-cell deficiency only in boys

1. low quantitative Immunoglobulin levels
2. absent B cells, high T cells

Tx: IVIG and prophylactic antibiotics
Common variable immunodeficiency
Immunoglobulin level drops in 20s and 30s; usually a combined B and T cell defect

Increased pyogenic uper and lower respiratory infections

Tx: IVIG
IgA deficiency
Mild, may develop recurrent respiratory or GI infections

Do NOT give immunoglobulins
Thympic aplasia (DiGeorge)
Increased infections with fungi and P. jiroveci

Tx: bone marrow transplantation and IVIG for antibody deficiency; PCP prophylaxis
Ataxia Telangiectasia
DNA repair defect

Oculocutaneous telangiectasias
Cerebellar ataxias
Increased risk of skin cancers

No specific Treatment
Severe combined immunodeficiency
Adenosine Deaminase Deficiency

Lacks T and B cells.
frequent bacterial infections; chronic candidiasis,

Tx: bone marow or stem cell transplant, and IVIG
Needs PCP prophylaxis
Wiskott Aldrich Syndrome
X-linked disorder w/ less severe T and B cell dysfunction

Eczema, increased IgE and IgA, decreased IgM, thrombocytopenia

Increased risk of atopic disorders


Tx is IVIG and abx
Chronic granulomatous disease
deficient superoxide production by PMN's and macrophages

Dx: nitroblue tetrazolium test
Tx: daily TMP-SMX
Leukocyte adhesion defect
A defect in chemotaxis of leukocytes
Recurrent skin, mucosal, and pulmonary infections

Delayed separation of umbilical cord

Increased incidence of S. pyogenes, S. aureus, and Pseudomonas

Tx: Bone marrow transplant
Chediak-Higashi syndrome
Defect in neutrophil chemotaxis/microtubule polymerization

Blond, light colored
Increased incidence of infections w/ S. pyogenes, S. aureus, and Pseudomonas.

Tx: Bone marrow transplant
R/o that mimic child abuse
bleeding disorders
mongolion spots (burises)
osteogenesis imperfecta (fractures)
bullous impetigo (cigarette burns)
coining (alternative tx in certain cultures)
Child abuse management
1. skeletal survey / bone scan - various stages of healing
2. test: gonorrhea, syphilis, chlamydia, HIV if sexual abuse
3. Shaken baby syndrome - retinal hemorrhages, CT contrast for subdral
Child abuse tx
1. document
2. notify CPS
3. hospitalize
ventricular septal defect
most common congenital heart defect
harsh holocystolic infectionn
narrow s2 w/ increased p1
mid-diastolic apical rumble reflecting increase flow across mitral valve
ventricular septal defect
echocardiogram
vsd tx
most resolve spotaneously

surgical repair for children < 1 yo pulmonary hypertension, and older children w/ large VSDs that have not decreased in size over time
atrial septal defect
wide and fixed split s2
systolic ejection murmur at upper left sternal border (from increased flow across pulmonic valve)
atrial septal defect dx
echocardiogram
cxr: cardiomegaly and increased pulmonary markings
atrial septal defect tx
most close spontaneously

surgery if infants w/ CHF and patients w/ mroe than a 2:1 ratio of pulmonary to systemic flow
patent ductus arteriosus
risk factor: maternal rubella, prematurity, female

continuous machinery murmur at sternal border
patent ductus arteriosus dx
color flow doppler from aorta to pulmonary artery
patent ductus arteriosus tx
indomethacin unless pda needed for survival

surgery if indomethacin fails or child > 6-8 months of age
when pda needed for survival
transposition of great vessels
tetralogy of fallot
hypoplastic left heart
Coarctation of aorta
asymptomatic hypertension, low extremity claudication, syncope, epistaxis, and headache
coarctation of aorta dx
echocardiography
cxr: 3 sign, rib notching
coarctation of aorta tx
1 pge1 to keep open
2. surgery
transposition of great vessels
most common cyanotic congenital heart lesion in newborns

risk factors: diabetic mothers, DiGeorge syndrome

cyanosis an dcritical illness immediately after birth

single loud s2
Transposition of the Great Vessels dx
1. echocardiography
2. cxr: narrow heart base, egg-shaped silhouette
Transposition of the Great Vessels Tx
1. IV PGE1 to maintain or open the PDA
2. surgery not feasible within first few days of life, or if PDA can't be maintained w/ prostaglandin, perform balloon atrial septostomy to create an enlarged ASD
3. Surgical correction
Tetralogy of Fallot
pulmonary stenosis
right ventricular hypertrophy
overriding aorta
ventricular septal defect

cyanosis often not present at birth, but develops over first two years of life; degree of cyanosis reflexts extent of pulmonary stenosis

children oftten squat for relief (increase SVR) during hypoxemic episodes (tet spells)

systolic ejection murmur at L upper sternal border
Tetralogy of fallot dx
1. echocadiography and catheterization
2. cxr: boot shaped heart w/ decreased pulmonary vascular markings
3. ecg: right axis deviation due to righ ventricular hypertrophy
tetralogy of fallot tx
1. pge1 to keep pda open
2. treat hypercyanotic tet spells w/ oxygen, propanolol, phenylephrine, knee-chest position, fluids, morphine
3. temporary palliation through creation of artificial shunt (e.g., balloon atrial septostomy) before definitive surgical correction (Blalock-Taussig) shunt
girl sexual development
1. thelarche - breast bud development
2. pubarche - pubic hair development
3. growth spurt
4. menarche - first menstrual period; average 12.5 years
boy sexual development
1. gonadarche - testicular enlargement
2. pubarche
3. adrenarche - axillary hair, facial hair, vocal changes
male delayed puberty
no testicular enlargement in boys by age 14
female delayed puberty
no breast development or pubic hair in girls by age 13
Down syndrome
trisomy 21 (most common) or robertsonian translocation (higher risk of recurrence)

mental retardation, flat facial profile, epicanthal folds, simian crease

associated w/ duodenal atresia, Hirschsprung's disease, and gongenital heart disease (ASD, VSD, endocardial cushing defects)

associated w/ ALL, hypothyroidism and early-onset Alzheimer's
Edward Syndrome
Trisomy 18

Rocker bottom feet
micrognathia
low set ears
congenital heart disease
horseshoe kidneys
Patau
Trisomy 13

polydactyly
microphthalmia
microcephaly
holoprosoncephaly
Klinefelter
45 xxy

inactivated x chromosome (Barr body)
one of the most common causes of hypogonadism in males
testicular atrophy, tall stature, long extremities, gynecomastia

Tx: testosterone
Turner syndrome
45XO

gonadyl dysgenesis
no barr body
short stature, shield chest, widely spaced nipples, webbed neck, coarctation of aorta, lymphedema of hands and feet, horseshoe kidneys
double y males
47 xyy

increased frequency among inmates of penal institutions
very tall and severe acne and antisocial behavior
phenylketonuria
decreased phenylalanine hydroxylase or decreased tetrahydrobiopterin cofactor

tyrosine becomes essential adn phenylalanine builds up excess ketones

mental retardation, fair skin, eczema, musty or mousy urine odor

blond hair, blue eyes, increased risk of heart disease

Tx: decrease phenylalanine and increase tyrosine in diet
Fragile X
X-linked efect in FMR1 gene, triplet repeat disorder

second most common cause of mental retardation

macro-orchidism, long face w/ large jaw, large, everted ears, autism
Cystic Fibrosis
autosomal recessive disorder in CFTR gene on Chr 7

failure to thrive, chronic sinopulmonary disease

recurrent pulmonary infections, meconium ileus, at risk for fat soluble vitamin deficiency
Cystic Fibrosis diagnosis
Sweat chloride test > 60 mEq/L if less than 20 years old

> 80 mEq/L in adults
Fabry's disease
x-linked recessive
deficiency of alpha-galactosidase A

accumulation of ceramide trihexoside in heart, brain, and kidneys

renal failure, increased risk of stroke and MI
Krabbe's disease
autosomal recessive

absence of galactosylceramide and galactoside

accumulation of galactocerebroside in brain

optic atrophy, spasticity, early death
Gaucher's disease
autosomal recessive

deficiency of glucocerebrosidase

accumulationglucocerebroside in brain, liver, spleen, and bone marrow

hepatosplenomegaly, anemia, and thrombocytopenia
"crinkled paper" enlarged cytoplasm

type 1 is more common and compatible w/ normal life span and does not affect brain
Nieman Pick disease
autosomal recessive

Nieman Pick PICKS his SPHINGER

deficiency of sphingomyelinase leads to buildup of sphingomyelin cholesterol in reticuloendothelial and parenchymal cells and tissue

Patients die by age of three
Tay-Sachs disease
Tay SaX lack heXosaminidase

absence of hexosaminidase leads to accumulation of GM2 ganglioside

Infants noraml until 3-6 months of age, then weakness begins and development slows and regresses

cherry-red spot on macula

death by 3
Metachromatic leukodystrophy
autosomal recessive

deficiency of arylsulfatase A leads to accumulation of sulfatide in brain, kidney, liver, and peripheral nerves
Hurler syndrome
autosomal recessive

deficiency of alpha L idurinidase

corneal clouding and mental retardation
Hunter's syndrome
x-linked recessive

deficiency of iduronidase sulfatase

milder form of no corneal clouding and mild mental retardation

Hunters need to see (no corneal clouding) to aim for X
Cystic Fibrosis Treatment
1. Pulmonary: chest physical therapy, bronchodilators, steroids, antibiotics, DNase
2. pancreatic enzymes, fat soluble vitamins for malabsorption
3. maintain high calorie highprotein diet
4. if disease severe but can tolerate surgery, can try for lung or pancreas transplants
Intussusception
abrupt onset, colicky pain in healthy children, often accompanied by flexed knees and vomiting

triad: abdominal pain, vomiting, blood per rectum

currant jelly stools, lethargy, fever, sausage shaped RUQ mass

Associated w/ Meckel's diverticulum, intestinal lymphoma (> 6 yo), Henoch-Schonlein purpura, parasites, polyps, adenovirus or rotavirus infection, celiac disease, CF
Intussusception managment
1. correct volume or electrolyte abnormalities
2. w/ high suspicion, air contrast barium enema performed WITHOUT DELAY (both diagnostic and therapeutic)

surgical reduction and resection if peritoneal signs
Pyloric stenosis
hypochloremic hypokalemic metabolic acidosis

nonbilious projectile emesis, olive shaped mass
Pyloric stenosis dx
abdominal ultrasound imaging of choice
Pyloric stenosis Tx
1. correct dehydration and acid-base electrolyte abnormalities
2. surgical correction w/ pyloromyotomy
Meckel's diverticulum
failure of omphalomesenteric (or vitelline) duct to obliterate

Rule of 2's
under 2 yo common
2x in males
2 types of tissue (pancreatic and gastric)
2 inches long
within 2 ft of ileocecal valve
2% of population

painless rectal bleeding
Meckel's diverticulum dx
mecek scintigraphy scan (technetium-99m pertechnate) is diagnostic
Meckel's diverticulum tx
excision of diverticulum with adjacent ileal segments
Hirschsprung's Disease
lack of ganglion cells in distal colon

neonate failure to pass meconium within 48 hrs of birth,
abdominal disension and explosive discharge of stool following rectal exam
Hirschsprung's Disease Dx
imaging study of choice: barium enema
Definitive diagnosis: full-thickness rectal biopsy
Hirschsprung's Disease Tx
two stage surgical repair
1. diverting colostomy at time of dx
2. definitive pull thru procedure connecting remaining colon to rectum (several weeks later)
Malrotation w/ volvulus
abnormal positioning of small intestine and formation of fibrous bands (Ladd's bands)

newborn w/ bilious emesis, abdominal pain, distension, and passage of blood or mucus in stool
Malrotation w/ volvulus dx
study of choice: upper GI - shows abnormal location of ligament of Tretiz
Malrotation w/ volvulus Tx
1. NG tube for decompression
2. IV fluid hydration
3. surgical repair (emergent when volvulus present)
Necrotizing Enterocolitis
common in premature infants

delayed gastric emptying, abdominal distension, rapidly progress to perforation, peritonitis

hyponatremic metabolic acidosis

radiographics may show dilated loops, pneumatosis intestinalis
Necrotizing Enterocolitis tx
1. NPO, orograstic tube, correct electrolyte abnormalities, TPN and IV antibiotics
2. surgery: perforation
X-linked agammaglobulinemia
B cell deficiency in boys only

life threatening: encapsulated organisms
X-linked agammaglobulinemia dx
quantitative immunoglobulin levels

if low, confirm w/ subsets
absent b cells, t cells often high

absent tonsils and lymphoid another clue
X-linked agammaglobulinemia tx
prophylactic abx and IVIG
Common variable immunodeficiency
immunoglobulins drop in 20s and 30s

usually a combined B and T cell defect

increased pyogenic upper and lower infections; increased risk of lymphoma nd autoimmune disease
Common variable immunodeficiency dx
quantitative immunoglobulin levels

confirm w/ b and t cell subsets
Common variable immunodeficiency tx
IVIG
IgA deficiency
mild, most common immunodeficiency

usually asymptomatic, may develop recurrent respiratory and GI symptoms

common presentation is anaphylactic transfusion reaction due to anti-IgA antibodies
IgA deficiency dx
quantitative IgA levels
IgA deficiency tx
treat ifnections

do NOT give immunoglobulins -- anaphylaxis with antibody development
Thymic aplasia
tetany secondary to hypocalcemia
think DiGeorge

high risk of fungi an dPCP infections
Thymic aplasia dx
absolute lymphocytic count
mitogen stimulation response
delayed hypersensitivity skin testing
Thymic aplasia tx
bone marrow transplantation and IVIG for antibody deficiency

pcp prophlyaxis

alternative:thymus transplant
Ataxia-telangiectasia
oculocutaneous telangiectaisas, cerebellar ataxia,hyperpigmentatin an dincreased risk of melanoma due to DNA repair defect

increased incidence of non-Hodgkin's lymphoma, leukemia, and gastric carcinoma
Ataxia-telangiectasia tx
no specific treatment
may require IVIG depending on severity of Ig deficiency
Severe Combined Immunodeficiency
severe lack of B and T cells
adenosine deaminase deficiency

severe frequent bacterial infections, chronic candidiasis and opportunistic infections
SCID tx
bone marrow transplant or stem cell transplant and IVIG

Needs PCP prophylaxis
Wiskott Aldrich Syndrome
X linked; less severe B cell and T cell dysfxn

eczema, thrombocytopenia, increased IgE and IgA, decreased IgM

bleeding eczema, recurrent otitis media

increased risk of lymphoma/leukemias, infections from encapslated organisms, atopic disorders
Wiskott Aldrich Syndrome tx
supportive w/ IVIG and abx

rarely survive to adulthood
Chronic Granulomatous Disease
X linked or autosomal recessive

deficient superoxide production by PMNs and macrophages

Anemia, lymphadenopathy, hyepergammaglobulinemia

Chronic skin, pulmonary, GI, and UTI's, osteomyelitis and hepatitis

Infected organisms catalase +,
Chronic Granulomatous Disease Dx
Diagnostic: nitroblue tetrazolium test
Chronic Granulomatous Disease Tx
Treat w/ daily TMP-SMX
Leukocyte adhesion deficiency
defect in chemotaxis of leukocytes

delayed separation of umbilical cord

recurrent skin, mucosal, and pulmonary infections

No pus w/ minimal inflammation in wounds
Leukocyte adhesion deficiency tx
Bone marrow transplant is curative
Chediak-Higashi syndrome
autosomal recessive

defectin neutrophil chemotaxis/microtubule polymerization

oculocutaneous albinism, neuropathy, neutropenia

increased incidence of overwhelming infections w/ S. pyogenes, S. aureus, and Pseudomonas
Chediak Higashi Syndrome dx
giant granules in neutrophils
Chediak Higashi Syndrome Tx
Bone marrow transplant
C1 esterase deficiency
autosomal dominant
hereditary angioedema provoked by stress/trauma

can lead to life-threatening airway edema

Tx: Purified C1 esterase and FFP can beu sed prior to surgery
Terminal complement deficiency C5 - C9
Inability to form membrane attack complex

recurrent meningococcal or gonococcal infections

Tx: Get appropriate meingococcal vaccine and appropriate antibiotics
Kawasaki Disease
CRASH and BURN
Conjnctivitis
Rash
Adenopathy
Strawberry tongue
Hands and feet swollen and red
BURN fever for several days
Kawasaki Disease Tx
High dose aspirin to prevent aneurysms
IVIG for inflammation and fever
Juvenile Idiopathic Arthritis
morning stiffness, gradual loss of motion for at least 6 weeks in patient < 16 yo
JIA dx
1. polyarticular, usually ANA+ and RF - , uveitis common

2. Polyarthritis ANA +

3. Still's dz (systemic onset) - presents w/ fever, hepatomegaly, and salmon colored macular rash; usually RF - and ANA -
JIA tx
1. NSAIDs and strengthening exercises
2. steroids
Acute otitis media pathogens
commonly S. pneumo, H. influenza and M. catarrhalis
Acute otitis media dx
otoscopic exam: erythematous tympanic membrane w/ decreased motility
Acute otitis media tx
High dose amoxicillin x 10 days
Bronchiolitis most common cause
RSV in fall/winter months
Bronchiolitis dx
clinical diagnosis

CXR if need to r/o pneumonia
Nasopharyngeal PCR confirmatory, but doesn't affect management
Bronchiolitis Tx
1. Supportive
2. Hospitalize w/ marked distress, O2 < 92%, toxic appearance, < 3 months old, underlying cardiopulmonary disease

Treat inpatient w/ contact isolation
- hydration
- oxygen
- can try aerosolized albuterol if it helps
Croup
Laryngotracheobronchitis
Parainfluenza virus
3 mo to 3 years
barking cough
Croup dx
clinical impression by stridor and barking cough
AP neck film: steeple sign from subglottic narrowing
Croup tx
mild: outpt cool mist therapy and fluids

moderate: supplemental O2, oral and IM steroids, nebulized epinephrine

severe: hospitalie and give nebulized racemic epinephrine
Epiglottitis
rapidly progressing supraglottic infection of supraglottic structures
3-7 years old
caused by H. influenza

muffled voice, drooling, neck hyperextended and chin protruding (sniffing dog position) and leaning forward in tripod position to maximize airway entry
Epiglottitis dx
clinical impression

1. secure airway before definitive diagnosis; do not eamine throat unless anesthesiologist or otolaryngologist present
2. definitive diagnosis: direct fiberoptic visualization of cherry red, swollen, epiglottitis and arytenoids

3. lateral x ray: thumb-print sign
Epiglottitis tx
keep patient calm, call anesthesia, transfer to OR

treat w/ endotracheal intubation or tracheostomy, and IV ceftriaxone or cefuroxime
Retropharyngeal abscess
6 mo - 6 yo

muffled hot potato voice, trismus,drooling cervical lympadenopathy

caused by group A strep, S. aureus, bacterioides

Prefers supine w/ neck extended
Retropharyngeal abscess dx
lateral neck x-ray: soft tissue plane under 50% of width
Retropharyngeal Abscess tx
aspiration or I&D of abscess

abx
Peritonsillar abscess
> 10 years age

muffled hot ptoato voice, trismus, drooling

group A strep, S. aureus, S. pneumonia; anaerobes
Peritonsillar abscess dx
clinical
Peritonsillar abscess tx
I&D +/- tonsillectomy; antibiotics
Meningitis workup
1. Head CT to rule out increased ICP
2. LP: cell count, gram stain, and culture
Meningitis tx
Empiric abx (ceftraixone, vacomycin, ampicillin) until bacterial meningitis ruled out

Neonates: ampicillin, cefotaxime, gentamicin

If concern for herpes encephalitis, then acyclovir
Pertussis
Bordatella pertussis - gram negative bacillus
Whooping cough

inspiratory whooop and post0tussive emesis

classic presentation is infant < 6 mo age w/ post-tussive emesis and apnea
Pertussis dx
elevated WBC
gold standard: culture
Pertussis tx
hospitalize patients < 6 mo age

erythromycin x 4 days to patients and close contacts

should not return to school/day care until five days after abx administered or three weeks have elapsed in no treatment initiated
Erythema infectiosum
Fifth disease
Parvovirus B19

Slapped erythematous cheecks
rash starts on arms and spreads to trunk and legs

rash worsens w/ fever an dsun exposure

can get arthritis, hemolytic anemia and aplastic crisis
Measles
Paramyxovirus

Cough, Coryza (head cold), Conjunctivits

Koplik spots - small red spots w/ central gray specks on buccal mcosa

erythematous rash spreads from head to feet

appears ill, unlike rubella
Rubella
tender lymphadenopathy

erythematous maculopapular rash that starts on face and spreads distaly

Unlike measles, only low grade fever an ddoes not appear ill
Roseola infantum
HHV-6 acute onset of fever w/ no other symptoms for 3-4 days

febrile seizures may occur w/ fever

maculopapular rash appears as fever breaks on trunk and spreads to face and extremiities
Varicella
VZV

mild fever, anorexia, and malaise
generalied pruritic tear drop vesicular periphery; lesions at different stages of healing
Varicella Zoster
VZV

reactivation of varicella infection; w/ pain along affected sensory nerve

pruritic tear drop vesicular rash in dermatomal distribution
Hand-foot-and-mouth disease
Coxsackie A

Prodrome: fever, anorexia, oral pain
Oral ulcers, maculopapular vesicular rash on hands and feet, and sometimes on buttocks
Conjugated hyperbilirubinemia differential
biliary atresia
choledochal cysts
intrahepatic cholestasis
Dubin-Johnson syndrome (black liver)
Rotor syndrome (black urine)
TORCH
unconjugated hyperbilirubinemia differential
physiologic jaundice
hemolysis
breast milk jaundice
GI obstruction leading to increased enterohepatic circulation
Crigler-Najjar
Gilbert
Kernicterus
uncojungated hyperbilirubinemia

lethargy, poor feeding,high pitched cry, hypertonicity, seizures
Treatment of unconjugatted hyperbilirubinemia
mild elevation: phototherapy
severe elevation > 20 mg/dL: exchange transfusion
Physiologic Jaundice
not present until 72 hrs after birth

bilirubin increase is less than 5 mg/dL/day

bilirubin peaks at less than 14-15 mg/dL

direct bilirubin is < 10% total

resolves by one week in term infants, two weeks in preterm
Pathological Jaundice
first 24 hrs of life
bilirubin increase > 0.5 mg/dL/hr
bilirubin peak >15 mg/dL
direct bilirubin > 10% total

persists beyond one week in term infants and two weeks in preterm
Tracheoesophageal fistula
VACTERL
vertebral, anal, cardiac, tracheal, esophagageal, renal limb anomalies

polyhydramnios in utero, increased oral secretions, inability to feed, gagging, aspiration pneumonia, respiratory distress
Tracheoesophageal fistula dx
CXR: Ng tube coiled in esophagus

Confirmation: bronchoscopy
Tracheoesophageal fistula tx
surgery
Congenital diaphragmatic hernia
GI tract segments protrude thru diaphragm into thorax; most thru posterior left (Bochdalek)

respitary distress, sunk abdomen
Congenital diaphragmatic hernia dx
ultrasound in utero

postnatal: cxr
Congenital diaphragmatic hernia tx
1. high frequency ventilation or ECMO to manage pulmonary hypertension
2. surgery
Gastroschisis
herniation of intestine thru abdomianl wall next to umbilicus w/ NO SAC

presentation: polyhydramnios, premature,
Gastroschisis tx
two stage surgery
Omphalocele
Herniation of abdominal viscera thru wall at umbilicus into sac

presentation: polyhydramnios, often premature, associated w/ other GI and cardiac defects; seen w/ Beckwth-Wiedemann syndrome and trisomies
Omphalocele Tx
C-section to prevent sac rupture

If sac intact, postpone surgery until fully resucitated

Keep sac covered/stable w/ petroleum and gauze

Intermittent NG suction to prevent distension
Duodenal atresia
Complete or partial failure of duodenal lumen to recanalize during gestational weeks 8-10

polyhydramnios, bilious emesis within hrs after first feeding; associated w/ Down syndrome
Duodenal atresia dx
abdominal XR: double bubble
Duodenal atresia tx
surgery
ARDS
respiratory failure in preterm infants due to surfactant deficiency

first 48-72 hrs of life, RR > 60/min, progressive hypoxemia, cyanosis, nasal flaring, intercostal retractions, expiratory grunt
ARDS diagnosis
1. ABG's, CBC, blood cultures
2. CXR: ground glass apperance, air bronchograms
transient tachypnea of newborn CXR
retained amionitic fluid

prominent perihilar streaking in interlobular fissures
Meconium aspiration CXR
coarse, irregular infiltrates; hyperexpansion and pneumothoraces
Congenital pneumonia CXR
non-specific pathy infiltrates; neutropenia, tracheal aspirate, gram stain
ARDS Treatment
1. CPAP
2. Artificial surfactant
3. < 30 weeks at risk for preterm delivery, give corticosteroids
4. > 30 weeks, monitor fetal lung maturity w/ L/S ratio
L/S ratio for need to administer glucocorticoids
L/S < 2:1
Complication of ARDS
bronchopulmonary dysplasia
Simple febrile seizure
< 15 minute duration, generalized seizure with one seizure in 24 hr period. high fever within hrs is typical
Complex febrile seizure
> 15 min or focal seizure, or multiple seizures in 24 hr period
Treatment of febrile seizure
if simple, treat fever

if complex, thorough neuro evaluation
Electrolyte profile of tumor lysis syndrome
hyperkalemia, hyperphosphatemia, hyperuricemia
hypocalcemia
Neuroblastoma
nontender abdominal mass that may cross midline
may have anemia, failure to thrive, and fever
Neuroblastoma Dx
1. CT: fine needle aspiration of tumor w/ round blue tumor cells in rosette pattern
2. urine VMA and HVA elevated
Neuroblastoma Tx
1. local excision
2. post-surgical chemotherapy and/or radiation
Wilm's Tumor
renal tumor associated w/ aniridia and hemihypertrophy

associated w/ Beckwith-Wiedemann syndrome and WAGR (Wilm's, Aniridia, Genitourinary abnormalities, mental Retardation)
Wilm's Tumor Dx
1. CBC, BUN, creatinine, UA
2. Abdomianl Ultrasound
3. CT of chest and abdomen to detect metastasis
Ewing's sarcoma
Chr 11:22
male adolescents
local swelling an dpain, with fever anorexia and fatigue

midshaft of long bones
Ewing's sarcoma dx
leukocytosis
elevated ESR
lytic bone lesiosn w/ "onion skin"
Ewing's sarcoma tx
1. local excision
2. chemothreapy
3. radiation
Osteosarcoma
Osteoblasts
male adolescents
local pain and swelling
NO FEVER ANOREXIA OR FATIGUE
METAPHYSIS of bone (distal femur, proximal tibia, proximal humerus)

20% metastasis to lungs
Osteosarcoma dx
increased alkaline phosphatase

Sunburst lytic lesions

Chest CT to r/o pulmonary metastasis
Osteosarcoma tx
Local excision, chemotherapy
What's the limit temperature of baby bath water?
< 38.8 C or < 120 F
How to decrease SIDS risk
baby sleeps on back
Osteosarcoma
Osteoblasts
male adolescents
local pain and swelling
NO FEVER ANOREXIA OR FATIGUE
METAPHYSIS of bone (distal femur, proximal tibia, proximal humerus)

20% metastasis to lungs
Osteosarcoma dx
increased alkaline phosphatase

Sunburst lytic lesions

Chest CT to r/o pulmonary metastasis
Osteosarcoma tx
Local excision, chemotherapy
What's the limit temperature of baby bath water?
< 38.8 C or < 120 F
How to decrease SIDS risk
baby sleeps on back
car safety seat
rear facing, back of car

when > 1 yo an d> 20 pounds, seats can face forward
when can gradually give solid foods
6 months
don't give cow's milk prior to ?
12 mo
Leukocoria differential
retinobalstoma
congenital cataracts
retinopathy of prematurity
Contraindications to vaccines
severe allergy
encephalopathy within 7 days prior to pertussis invaccination

egg allergy, no MMR or influenza vaccine

immunocompromized, avoid live vaccines (oral polio, varicella, MMR)

pregnant, avoid live vacines EXCEPT MMR and varicella
What are NOT contraindicatios to vaccination
mild fever
current abx therapy
prematurity
What children get pneumococcal polysaccharide vaccine?
sickle cell dz
splenectomy
immunodeficient
Lead poisoining
irritability, hyperactivity, apathy, anorexia abdominal pain, vomiting, peripheral neuropathy (foot or wrist drop)
What level of lead can impaired neurodevelopment occur at
10 ug/dL
Lead poisoning Tx < 45 ug/dL
remove sources of lead exposure

retest at 1-3 months
Lead poisoning Tx 45 - 69 ug/dL
chelation w/ inpatient EDTA or outpatient oral succimer (DMSA)
Lead poisoning Tx 70 ug/dL or greater
chelation therapy of inpatient EDTA + BAL (IM dimercaprol)