Fa: Step 2 Ck. Pediatrics.

Front 1

What are the principal causes of non-cyanotic heart disease?

Back 1

"The 3 D's"

VSD
ASD
PDA

Front 2

What are the principal causes of cyanotic heart disease?

Back 2

"1, 2, 3, 4, 5"

1. Truncus arteriosus = 1 arterial vessel overriding ventricles
2. Transposition of the great vessels = 2 arteries switched
3. TRIcuspid atresia
4. TETRAlogy of Fallot
5. Total anomalous pulmonary venous return (5 words)

Front 3

What syndromes are frequently associated with VSD?

Back 3

Apert's
Down
fetal alcohol syndrome
TORCH
cri du chat
trisomies 13 and 18

Front 4

What are the TORCH infections?

Back 4

Toxoplasmosis
Other agents
Rubella
CMV
HSV

Front 5

Who should be surgically treated for VSD?

Back 5

symptomatic patients who fail medical management
children <1 years with signs of PAH
children with large VSDs that have not decreased in size over time

Front 6

What is the characteristic physical exam finding of atrial septal defect?

Back 6

a fixed, widely split S2

Front 7

What syndromes are associated with atrial septal defect?

Back 7

Holt-Oram (absent radii, ASD, 1° heart block)
Down
fetal alcohol syndrome

Front 8

What type of ASD is most common? When does it typically present?

Back 8

ostium secundum

ostium primum defects present in early childhood

ostium secundum defects present in late childhood or early adulthood

Front 9

What are the clinical presentations of ostium primum and ostium secundum, respectively?

Back 9

primum: early in life; murmur or fatigue with exertion

secundum: late childhood; symptom severity dependent on size of defect

Front 10

What are the physical exam findings of ASD?

Back 10

right ventricular heave
wide and fixed, split S2
systolic ejection murmur at LUSB (incr. flow across pulmonary valve)

may be a mid-diastolic rumble at LLSB

Front 11

What are the echo findings in ASD?

What are the EKG findings?

What are the CXR findings?

Back 11

Echo: Doppler shows blood flow between atria, paradoxical ventricular wall motion, dilated RV

EKG: right axis deviation, RVH, PR prolongation

CXR: cardiomegaly and increased pulmonary vascular markings

Front 12

What is an indication for surgical closure of an ASD?

What are possible complications of untreated severe ASD?

Back 12

>2:1 pulmonary to systemic blood flow

arrhythmias, RV dysfunction, Eisenmenger's

Front 13

What are risk factors for patent ductus arteriosus (PDA)?

Back 13

1st trimester rubella infection
prematurity
female gender

Front 14

What are the physical exams findings in PDA?

Back 14

continuous "machinery" murmur
loud S2
bounding peripheral pulses

Front 15

How is PDA diagnosed?

Back 15

Echocardiogram with Doppler demonstrating blood flow from aorta to pulmonary artery

Front 16

What is the treatment to close PDA?

What if the treatment fails?

When is this treatment contraindicated?

Back 16

indomethacin

surgical correction (also if age >6 mo)

in the setting of intraventricular hemorrhage

Front 17

What conditions necessitate persistence of a PDA?

What can be given to maintain a PDA?

Back 17

transposition of great vessels
tetralogy of Fallot
hypoplastic left heart

prostaglandin E1

Front 18

What is the most common location of aortic coarctation?

Back 18

just below left subclavian artery

Front 19

What conditions are associated with coarctation of the aorta (4)?

Back 19

bicuspid aortic valve (>2/3rds of patients)
Turner syndrome
berry aneurysms
male gender

Front 20

What are some common childhood presentations for coarctation of the aorta?

Back 20

asymptomatic hypertension
lower extrem. claudication
syncope
epistaxis
headache

Front 21

What are some physical exam findings in coarctation of the aorta?

Back 21

weak femoral pulses
radiofemoral delay
short systolic murmur in left axilla
forceful apical impulse

Front 22

In an infant with critical aortic coarctation, what pattern of cyanosis will be seen following closure of the ductus arteriosus?

Back 22

greater cyanosis in left upper and bilateral lower extremities (postductal circulation)

right upper extremity, head and neck will show less cyanosis (preductal circulation)

Front 23

What compensatory changes may be seen in older children with coarctation of the aorta (3)?

Back 23

LVH on ECG
"figure 3" sign on CXR
rib notching

Front 24

What is the most common cyanotic congenital heart lesion in the neonate?

What is the most common cyanotic congenital heart lesion in children?

Back 24

transposition of the great vessels

tetralogy of Fallot

Front 25

What are risk factors for transposition of the great vessels?

Back 25

maternal diabetes (common)
DiGeorge syndrome (rare)

Front 26

What mnemonic describes DiGeorge syndrome?

Back 26

CATCH-22

Cardiac abnormalities
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia
22q11 deletion

Front 27

What are the exam findings in transposition of the great vessels?

Back 27

tachypnea
progressive hypoxemia
extreme cyanosis

in addition, possibly:
single loud S2
signs of CHF

Front 28

What are the components of tetralogy of Fallot?

Back 28

pulmonary stenosis
RVH
overriding aorta
VSD

Front 29

Why does the cyanosis of tetralogy of Fallot sometimes decrease in the weeks following birth?

Back 29

right-sided pressures decrease, R-to-L shunting across VSD reverses

if pulmonary stenosis is severe, right-sided pressures may remain high and cyanosis may worsen

Front 30

What are risk factors for ToF?

Back 30

maternal PKU
DiGeorge syndrome

Front 31

What position can relieve the hypoxic symptoms of ToF?

Back 31

squatting (increases systemic vascular resistance and reverses R-to-L shunting through VSD)

Front 32

What are the exam findings in ToF?

Back 32

systolic ejection murmur at LUSB (due to RV outflow obstruction)
RV heave
single S2

Front 33

How is ToF diagnosed?

Back 33

echo + catheterization
CXR showing boot-shaped heart
ECG showing right axis deviation and RVH

Front 34

What is the treatment for ToF?

Back 34

severe cases require PGE1 to maintain PDA + urgent surgery

cyanotic spells treated with O2, propranolol, phenylephrine, knee-chest position, fluids, morphine

Front 35

What is a Blalock-Taussig shunt?

Back 35

creation of an artificial shunt (e.g. balloon atrial septostomy) for temporary relief of ToF symptoms

Front 36

Give the major gross motor developmental milestones:

2 mo
4 mo
6 mo
10 mo
12 mo
15 mo
18 mo
2 y
3 y
4 y
5 y

Back 36

2 mo - lifts head when prone
4 mo - rolls front to back
6 mo - sits unassisted
10 mo - crawls, pulls to stand
12 mo - cruises/walks alone
15 mo - walks backward
18 mo - runs, kicks ball
2 y - walks up/down steps; jumps
3 y - rides tricycle; alternating stair climb
4 y - hops
5 y - skips, walks backward long distances

Front 37

Give the major fine motor developmental milestones:

2 mo
4 mo
6 mo
10 mo
12 mo
15 mo
18 mo
2 y
3 y
4 y
5 y

Back 37

2 mo - tracks past midline
4 mo - grasps rattle
6 mo - transfers object; raking grasp
10 mo - 3-finger pincer grasp
12 mo - 2-finger pincer grasp
15 mo - uses cup
18 mo - builds tower of 2-4 blocks
2 y - builds tower of 6 blocks
3 y - copies a circle
4 y - copies a square
5 y - ties shoelaces, knows L v. R, prints letters

Front 38

Give the major language developmental milestones:

2 mo
4 mo
6 mo
10 mo
12 mo
15 mo
18 mo
2 y
3 y
4 y
5 y

Back 38

2 mo - alerts to sound, coos
4 mo - orients to voice, consonant sounds
6 mo - babbles
10 mo - "mama/dada" nonspecific
12 mo - "mama/dada" specific
15 mo - 4-6 words
18 mo - names common objects
2 y - two-word phrases
3 y - three-word sentences
4 y - knows colors, some numbers
5 y - five-word sentences

Front 39

Give the major social/cognitive developmental milestones:

2 mo
4 mo
6 mo
10 mo
12 mo
15 mo
18 mo
2 y
3 y
4 y
5 y

Back 39

2 mo - recognizes parent; social smile
4 mo - looks around, laughs
6 mo - stranger anxiety
10 mo - waves bye-bye. paddy cake
12 mo - separation anxiety
15 mo - temper tantrums
18 mo - starts toilet training
2 y - two-step commands, removes clothes
3 y - brushes teeth with help, washes/dries hands
4 y - cooperative play, board games
5 y - domestic role playing, dress-up

Front 40

What percent of birth weight do infants lose immediately following birth?

By what age should infants return to birth weight?

By what age should they have doubled their birth weight?

Back 40

5-10%

14 days

4-5 months

Front 41

How is failure to thrive (FTT) defined?

Back 41

Persistent weight less than 5th percentile for age

or

"falling off growth curve" i.e. crossing two major percentile lines on growth chart

Front 42

Give some organic and nonorganic causes of FTT

Back 42

Organic: due to underlying medical condition, e.g.
cystic fibrosis
congenital heart disease
celiac sprue
pyloric stenosis
chronic infection (e.g. HIV)
GERD

Nonorganic: primarily due to psychosocial factors including:
maternal depression
neglect
abuse

Front 43

What is the order of "falling off" the growth curves in infants with FTT?

Back 43

first fall off weight curve

then fall off height curve

finally, fall off head circumference curve

Front 44

Give the usual order of sexual development for girls.

Back 44

thelarche (breast buds; average age 10.5y)
pubarche (pubic hair)
menarche (average age 12.5y)

Front 45

Give the usual order of sexual development for boys.

Back 45

gonadarche (testicular enlargement; average age 11.5 years)
pubarche (pubic hair development)
adrenarche (axillary/facial hair, voice changes)
growth spurt

Front 46

Define the following:

1. delayed puberty
2. constitutional growth delay
3. pathologic puberty delay
4. precocious puberty

Back 46

1. no gonadarche in boys by 14; no thelarche/pubarche in girls by age 13

2. MCC of delayed puberty; consistent lag in growth curve, often with + family history; ultimately achieve target height

3. due to IBD, malnutrition, gonadal dysgenesis (Klinefelter, Turner), endocrine abnormalities (hypopituitary, hypothyroid, Kallmann, androgen insensitive, Prader-Willi)

4. any sign of sexual maturation in girls <8y or boys <9y

Front 47

What conditions are associated with Down syndrome (3)?

Back 47

duodenal atresia
Hirschsprung's disease
congenital heart disease (AV canal = ASD, VSD, mitral and tricuspid anomalies; due to endocardial cushion defect)

Front 48

Patients with Down syndrome are at increased which for developing what conditions (3)?

Back 48

ALL
hypothyroidism
early-onset Alzheimer's

Front 49

Give the characteristic findings for the following genetic syndromes:

1. Edwards (trisomy 18)
2. Patau (trisomy 13)

Back 49

1. rocker-bottom feet; micrognathia; may have congenital heart disease and horseshoe kidneys

2. severe mental retardation, microphthalmia, microcephaly, cleft lip/palate, holoprosencephaly, polydactyly

Front 50

What is the treatment for patients with Klinefelter syndrome?

Back 50

testosterone

this prevents gynecomastia and improves secondary sexual characteristics

Front 51

What is the most common cause of primary amenorrhea?

Back 51

Turner syndrome (45,XO), due to gonadal dysgenesis

these patients may also have horseshoe kidneys, aortic coarctation and/or bicuspid aortic valve

Front 52

What are features of "double Y" (47,XYY) males?

Back 52

phenotypically normal, but very tall with severe acne and antisocial behavior (incr. frequency in prison inmates)

Front 53

What is the biochemical abnormality in PKU?

Back 53

decreased phenylalanine hydroxylase or decreased tetrahydrobiopterin cofactor

Front 54

Which amino acid is essential in PKU?

Which must be avoided?

Back 54

tyrosine

phenylalanine

Front 55

What are the two most common genetic causes of mental retardation?

Back 55

1. Down syndrome
2. Fragile X syndrome

Front 56

What are the typical features of fragile X syndrome?

Back 56

macro-orchidism
long face with large jaw
large, everted ears
autism

as a triplet repeat expansion disorder, this syndrome may show anticipation

Front 57

What is the gene affected in cystic fibrosis, and what does it code for?

Back 57

Cystic Fibrosis Transmembrane Regulator (CFTR) gene on chromosome 7

codes for a chloride channel

Front 58

What bugs frequently cause pulmonary infections in CF patients?

Back 58

Pseudomonas
Staph. aureus

Front 59

What percent of CF patients present with a meconium ileus?

For what vitamin deficiencies are CF patients at risk?

Back 59

15%

fat-soluble vitamins (A, D, E, K)

Front 60

When do GI symptoms predominate in CF patients?

When do pulmonary symptoms predominate?

Back 60

infancy

early childhood and beyond

Front 61

What sweat chloride values are diagnostic for CF?

Back 61

>60 mEq/L for those <20y

>80 mEq/L for adults

Front 62

Give the mode of inheritance for the following lysosomal storage diseases:

1. Fabry's
2. Krabbe's
3. Gaucher's
4. Niemann-Pick
5. Tay-Sachs
6. Metachromatic leukodystrophy
7. Hurler's
8. Hunter's

Back 62

1. X-linked recessive
2. AR
3. AR
4. AR
5. AR
6. AR
7. AR
8. X-linked recessive

Front 63

What enzyme is deficient in Fabry's disease?

What are the consequences?

Back 63

alpha-galactosidase

accumulation of ceramide trihexosidase in heart, brain, kidneys; causes renal failure and increased risk of stroke and MI

Front 64

What enzyme is deficient in Krabbe's disease?

What are the consequences?

Back 64

galactosylceramide and galactoside

accumulation of galactocerebroside in brain; causes optic atrophy, spasticity, early death

Front 65

What enzyme is deficient in Gaucher's disease?

What are the consequences?

Back 65

glucocerebrosidase

accumulation of glucocerebroside in brain, liver, spleen, bone marrow (causes "wrinkled paper" cells); presents with hepatosplenomegaly, anemia, thrombocytopenia

Type 1 is more common form, does not affect brain and is compatible with normal lifespan

Front 66

What enzyme is deficient in Hurler's syndrome?

What are the consequences?

Back 66

alpha-L-iduronidase

corneal clouding and mental retardation

Front 67

What enzyme is deficient in Hunter's syndrome?

What are the consequences?

Back 67

iduronate sulfatase

a mild form of Hurler's with no corneal clouding, mild mental retardation

Front 68

What enzyme is deficient in Niemann-Pick disease?

What are the consequences?

Back 68

sphingomyelinase

accumulation of sphingomyelin cholesterol in reticuloendothelial cells and parenchymal cells and tissues; type A pts die by age 3

Front 69

What enzyme is deficient in Tay-Sachs disease?

What are the consequences?

Back 69

hexosaminidase

GM2 ganglioside accumulation; Sx appear at 3-6 mos of age with weakness, regressed development, exaggerated startle response; cherry-red spot on macula

Front 70

Give mnemonics for the following lysosomal storage diseases:

1. Tay-Sachs
2. Niemann-Pick
3. Hunter's

Back 70

1. Tay-SaX lacks heXosaminidase
2. "No-man picks" his nose with his sphinger
3. Hunters need to see (no corneal clouding, as seen in Hurler's) to aim for the X

Front 71

What is the MCC of bowel obstruction in the first two years of life?

Back 71

intussusception (males > females)

usually seen between 3mo-3y

Front 72

What are risk factors for childhood intussusception?

Back 72

conditions with potential lead points:
Meckel's diverticulum
intestinal lymphoma
Henoch-Schonlein purpura
parasites
polyps
adenovirus/rotavirus infection
celiac disease
CF

Front 73

What is the classic triad of intussusception?

Back 73

abdominal pain
vomiting
blood per rectum

triad affects 1/3 patients with intussusception

Front 74

What are late signs of intussusception?

Back 74

"currant jelly" stool
lethargy
fever

may progress to shock

Front 75

What are the exam findings in intussusception?

Back 75

abd. tenderness
+ stool guaiac
palpable "sausage-shaped" RUQ mass

Front 76

What ultrasound finding is characteristic of intussusception?

Back 76

a "target sign"

Front 77

What procedure is diagnostic and therapeutic for intussusception?

Back 77

air-contrast barium enema

Front 78

What conditions are associated with pyloric stenosis?

Back 78

firstborn male
tracheoesophageal fistula
maternal Hx of pyloric stenosis

Front 79

When do symptoms of pyloric stenosis commonly begin?

What is the treatment for pyloric stenosis?

Back 79

3 weeks, with progressive increase in forcefulness of nonbilious emesis

pyloromyotomy

Front 80

What imaging modality is preferred to Dx pyloric stenosis?

What do barium studies show?

Back 80

ultrasound

narrow pyloric channel ("string sign")

Front 81

What is the cause of Meckel's diverticulum?

Back 81

failure of omphalomesenteric (vitelline) duct to obliterate

Front 82

What is the "rule of 2's" for Meckel's diverticulum?

Back 82

most common in kids <2
males:females = 2:1
contains 2 types of tissue (pancreatic, gastric)
2 inches long
within 2 feet of ileocecal valve
occurs in 2% of the population

Front 83

What is the classic presentation of Meckel's diverticulum?

How is the condition diagnosed?

Back 83

painless rectal bleeding

Meckel scintigraphy scan (technetium-99m pertechnetate)

Front 84

What conditions are associated with Hirschsprung's disease?

Back 84

male gender
Down syndrome
Waardenburg's syndrome
MEN type 2

Front 85

How is Hirschsprung's disease diagnosed?

Back 85

barium enema showing narrowed distal colon

anorectal manometry will show failure of internal sphincter to relax; full-thickness rectal biopsy confirms Dx

Front 86

What are the consequences of malrotation with volvulus?

Back 86

abnormal bowel position (cecum in R hypochondrium)

formation of fibrous bands (Ladd's bands) that predispose to obstruction, constriction of blood flow

Front 87

What is the usual presentation of malrotation with volvulus?

Back 87

bilious emesis (often in neonatal period)
crampy abd pain
passage of blood or mucus in stool

Front 88

How is malrotation with volvulus diagnosed?

Back 88

upper GI series showing abnormal location of ligament of Treitz

Front 89

What is necrotizing enterocolitis (NEC)?

What is a major risk factor for NEC?

Back 89

condition in which portion of bowel undergoes necrosis; it is the most common GI emergency in neonates

prematurity (but occurs in term infants as well)

Front 90

What are the abdominal plain film findings in NEC?

How often should abdominal plain films be repeated?

Back 90

dilated bowel loops
pneumatosis intestinalis (intramural air bubbles representing gas produced by bacteria within bowel wall)

q6 hours

Front 91

What is the most appropriate initial treatment for NEC?

What are indications for surgery?

Back 91

NPO, orogastric tube, correction of dehydration and electrolyte abnormalities, TPN, IV abx

perforation or interval worsening of serial abdominal plain films

Front 92

What are potential complications of NEC?

Back 92

formation of intestinal strictures

short-bowel syndrome

Front 93

Give the major characteristics of B-cell immunodeficiency.

Back 93

most common immunodef; typically present >6 mo of age with recurrent sinopulmonary, GI, UTIs with encapsulated organisms (Klebs, Salmon, H. flu, S. pneumo, Neisseria)

Front 94

Give the major characteristics of T-cell immunodeficiency.

Back 94

present earlier than B-cell, around 1-3 months; opportunistic and low-grade fungal, viral, intracellular bacterial infxns (e.g. mycobacteria). Secondary B-cell dysfunction may be seen.

Front 95

Give the major characteristics of phagocyte immunodeficiency.

Back 95

mucous membrane infections, abscesses, poor wound healing; infections with catalase+ organisms, fungi, Gram neg. enteric organisms.

Front 96

Give the major characteristics of complement immunodeficiency.

Back 96

in children with congenital asplenia or splenic dysfunction (e.g. sickle cell disease); characterized by recurrent bacterial infections with encapsulated organisms.

Front 97

What is the treatment for B-cell immunodeficiency?

Back 97

IVIG (except IgA deficiency)

giving IVIG in IgA deficiency may cause the formation of anti-IgA antibodies

Front 98

What is the usual age of presentation of Kawasaki disease?

What are the three phases of Kawasaki disease?

Back 98

80% <5 years of age

acute, subacute, chronic

Front 99

Describe the acute phase of Kawasaki disease.

Back 99

lasts 1-2 weeks, presents with fever of 5+ days and at least four of the following:
b/l, nonexudative conjunctivitis
polymorphous truncal rash
painful, unilateral cervical lymphadenopathy (one node >1.5 cm)
diffuse mucous membrane erythema ("strawberry tongue")
erythema of palms and soles, indurative edema of hands and feet

Front 100

Describe the subacute phase of Kawasaki disease.

Back 100

begins after abatement of fever; lasts additional 2-3 weeks

findings of thrombocytosis and elevated ESR; coronary artery aneurysms in untreated kids

Front 101

Describe the chronic phase of Kawasaki disease.

Back 101

begins when all clinical Sx have disappeared and ends when ESR normalizes

untreated kids at risk of coronary artery expansion and MI

Front 102

Give the mnemonic for Kawasaki disease symptoms.

Back 102

CRASH and burn!

Conjunctivitis
Rash
Adenopathy
Strawberry tongue
Hands and feet (red, swollen, flaky)

burn (fever >40C for 5+ days)

Front 103

What is the treatment of Kawasaki disease?

Back 103

high-dose ASA (for inflammation, fever)
IVIG (to prevent aneurysms)

low-dose ASA continued for 6 weeks
corticosteroids for IVIG-refractory cases, but not routine treatment

Front 104

What are the three forms of juvenile idiopathic arthritis?

What are the general features of this disease?

Back 104

pauciarticular (oligoarthritis)
polyarthritis
systemic-onset (Still's disease)

arthritis with morning stiffness
gradual loss of motion
present for at least 6 weeks in patient <16 years old

Front 105

What are the laboratory results for ANA and RF in each form of JIA?

Back 105

systemic-onset: ANA-, RF-
pauciarticular: ANA+, RF-
polyarticular: ANA+, RF+ if severe

Front 106

Give the major features of the three forms of JIA.

Back 106

Pauciarticular: most common; four or fewer joints affected; uveitis common; no systemic Sx

Polyarticular: involves 5+ joints, symmetric; systemic Sx rare

Systemic-onset (Still's): may present with recurrent high fever, hepatosplenomegaly, salmon-colored macular rash

Front 107

What are treatment options for JIA?

Back 107

1st-line: NSAIDs and strengthening exercises

2nd-line: corticosteroids and immunosuppressants (methotrexate; anti-TNF agents e.g. etanercept)

corticosteroids indicated for carditis

Front 108

What are the most common bacterial causes of acute otitis media?

What are the most common viral causes?

Back 108

S. pneumoniae
nontypable H. influenza
Moraxella catarrhalis

influenza A
RSV
parainfluenza

Front 109

What is the treatment of acute otitis media?

What are potential complications?

Back 109

high-dose amoxicillin x10 days; amoxicillin/clavulanate for resistant cases

TM perforation, mastoiditis, meningitis, cholesteatomas, chronic OM

recurrent cases can cause hearing loss with speech, language delay

Front 110

Define bronchiolitis.

What are the most common causes?

Back 110

an acute inflammatory illness of the small airways that primarily affects infants and children <2 years, often in fall/winter months

RSV is most common cause
parainfluenza
influenza
metapneumovirus

Front 111

What are exam findings in bronchiolitis?

Back 111

tachypnea
wheezing
intercostal retractions
crackles
prolonged expiration
expiratory wheeze
hyperresonance to percussion

Front 112

How is bronchiolitis diagnosed?

Back 112

predominantly a clinical Dx!

CXR may be useful to r/o pneumonia and will show hyperinflation, interstitial infiltrates, atelectasis

nasopharyngeal aspirate for RSV has high sens/spec, but does not change management

Front 113

What is the treatment for bronchiolitis?

Back 113

primarily supportive; fluids and nebulizers

admit to hospital if:
marked respiratory distress
O2 sat <92%
toxic-appearing
dehydration/poor PO intake
Hx of prematurity
age <3 months
underlying cardiopulmonary disease

Front 114

What is the role of ribavirin in the treatment of bronchiolitis?

Back 114

sometimes used in high-risk infants with underlying cardio, pulmonary, or immune disease

Front 115

What agents are used for RSV prophylaxis?

What is the role of RSV prophylaxis in preventing bronchiolitis?

Back 115

injectable poly- or monoclonal antibodies against RSV

indicated only for high-risk patients <2 years (Hx of prematurity, chronic lung dz, congenital heart dz)

Front 116

What is croup?

What are the most common pathogens?

Back 116

acute, viral, inflammatory disease of the larynx; primarily within subglottic space

parainfluenza virus type 1 (most common), type 2, type 3
RSV
influenza
adenovirus

Front 117

What are the history and physical exam findings in croup?

Back 117

prodromal URI symptoms followed by low-grade fever, mild dyspnea, inspiratory stridor worse with agitation, hoarse voice, characteristic barking cough

Front 118

How is croup diagnosed?

Back 118

clinical Dx, often based on degree of stridor and respiratory distress

AP neck film may show classic "steeple sign" from subglottic narrowing, but this finding is neither sensitive nor specific

Front 119

What is the treatment of croup?

Back 119

mild: cool mist therapy, fluids

moderate: supplemental O2, oral and IM corticosteroids, nebulized racemic epinephrine

severe: hospitalization, racemic epinephrine

Front 120

What is epiglottitis?

What are the most common pathogens?

Back 120

a seriously, rapidly progressive infection of supraglottic structures

Strep. species, nontypable H. flu, viruses

Front 121

What is the typical presentation of epiglottitis?

Back 121

acute-onset high fever (39-40C)
dysphagia
drooling
muffled voice
inspiratory retractions
cyanosis
soft stridor

Front 122

What is the typical appearance of a patient with epiglottitis?

Back 122

seated with neck hyperextended ("sniffing dog") and leaning forward in "tripod" position

Front 123

How is epiglottitis diagnosed?

Back 123

clinical Dx; airway must be secured before examination and definitive diagnosis

definitive diagnosis by direct fiberoptic visualization of cherry-red, swollen epiglottis and arytenoids

Front 124

What is the "thumbprint sign?"

Back 124

the classic radiographic finding in epiglottitis; a swollen epiglottis on lateral x-ray that resembles a thumbprint

Front 125

What is the treatment for epiglottitis?

Back 125

endotracheal intubation or tracheostomy
IV antibiotics (ceftriaxone or cefuroxime)

Front 126

In what age group does bacterial meningitis most often occur?

In what age group does viral meningitis most often occur?

Back 126

children <3 years

children of all ages

Front 127

What are the most common bacterial causes of meningitis?

What are the most common viral causes?

Back 127

S. pneumoniae, N. meningitidis, E. coli

enteroviruses

Front 128

What is the treatment for meningitis?

Back 128

ceftriaxone, vancomycin, ampicillin

neonates should receive ampicillin + cefotaxime/gentamicin

acyclovir if concern for herpes encephalitis

Front 129

What is the classic presentation of pertussis?

Back 129

infant <6 months of age with post-tussive emesis and apnea

Front 130

How is pertussis diagnosed?

Back 130

elevated WBC with lymphocytosis (often >70%)
culture is the gold standard

Front 131

What is the treatment for pertussis?

Back 131

hospitalize infants <6 months of age
erythromycin x14 days to patients and close contacts (incl. day care)
patients should not return to day care or school until 5 days after treatment or 3 weeks after no treatment

Front 132

Back 132

varicella

Front 133

Back 133

coxsackie A (hand-foot-mouth disease)

Front 134

Back 134

measles

Front 135

Back 135

roseola infantum

Front 136

Back 136

rubella

Front 137

Back 137

erythema infectiosum (fifth disease)

Front 138

Back 138

varicella zoster

Front 139

What pathogen causes measles?

What is the prodrome of measles?

Back 139

paramyxovirus

cough
coryza
conjunctivitis

Front 140

What pathogen causes rubella?

What is the prodrome of rubella?

Back 140

rubella virus

generalized lymphadenopathy, asymptomatic or tender

Front 141

What pathogen causes roseola infantum?

What is the prodrome of roseola infantum?

Back 141

HHV-6

acute onset of high fever (>40C); no other symptoms for 3-4 days; rash often lasts <24h

Front 142

What pathogen causes varicella?

What is the prodrome of varicella?

Back 142

varicella-zoster virus (VZV)

mild fever, anorexia, malaise preceding rash by 24 hours

Front 143

What pathogen causes hand-foot-mouth disease?

What is the prodrome of hand-foot-mouth disease?

Back 143

coxsackie A virus

fever, anorexia, oral pain

Front 144

Describe APGAR scoring.

Back 144

Feature (0; 1; 2 pts) for total 10 pts

Appearance (blue; pink trunk; all pink)
Pulse (0, <100, >100)
Grimace (none, grimace, grimace and cough)
Activity (limp, some, active)
Respiratory effort (none, irregular, regular)

Front 145

Define kernicterus.

Back 145

complication of unconjugated hyperbilirubinemia that results from irreversible bilirubin deposition in basal ganglia, pons, and cerebellum; typically occurs at bilirubin levels of 25-30 mg/dL

Front 146

What are risk factors for kernicterus (3)?

Back 146

prematurity
asphyxia
sepsis

Front 147

Give the differential for neonatal conjugated (direct) hyperbilirubinemia.

Back 147

extrahepatic cholestasis
intrahepatic cholestasis
Dubin-Johnson syndrome
Rotor's syndrome
TORCH infections

Front 148

Give the differential for neonatal unconjugated (indirect) hyperbilirubinemia.

Back 148

physiologic jaundice
hemolysis
breastmilk jaundice
incr. enterohepatic circulation (e.g. GI obstruction)
disorders of bilirubin metabolism
sepsis
Crigler-Najjar syndrome
Gilbert syndrome

Front 149

What is the presentation of kernicterus (5)?

Back 149

lethargy
poor feeding
high-pitched cry
hypertonicity
seizures

Front 150

How is neonatal jaundice diagnosed?

Back 150

CBC with peripheral blood smear, blood typing of mother and infant; Coombs' test; bilirubin levels

ultrasound and/or HIDA scan can reveal cholestatic disease

for direct hyperbilirubinemia: LFTs, bile acids, blood cultures, sweat test, alpha1-antitrypsin deficiency

Front 151

How is unconjugated hyperbilirubinemia treated?

Back 151

phototherapy
exchange transfusion (if >20 mg/dL)

begin phototherapy earlier (~10 mg/dL) for preterm infants

Front 152

Distinguish between physiologic and pathologic jaundice:

1. onset
2. rate of bilirubin increase
3. peak bilirubin concentration
4. proportion of direct bilirubin
5. time for resolution

Back 152

physiologic (pathologic)

1. >72 hours (first 24 hours)
2. <5 mg/dL/day (>0.5 mg/dL/hour)
3. <14-15 mg/dL (>15 mg/dL)
4. <10% of total (>10% of total)
5. resolves by 1 wk in term, 2 wks in preterm (persists longer!)

Front 153

What is respiratory distress syndrome (RDS)?

Back 153

formerly known as "hyaline membrane disease," it is the MCC of respiratory failure in preterm infants, affecting >70% of infants born at 28-30 weeks' gestation

Front 154

What is the pathophysiology of RDS?

Back 154

surfactant deficiency leading to poor lung compliance, alveolar collapse, and atelectasis

Front 155

What are some risk factors for RDS (3)?

Back 155

maternal diabetes
male gender
second-born of twins

Front 156

What is the typical presentation of RDS?

Back 156

presents in first 48-72 hours of life with RR >60/min, progressive hypoxemia, cyanosis, nasal flaring, intercostal retractions, expiratory grunting

Front 157

Name the condition with the given radiographic findings:

1. prominent perihilar streaking in interlobular fissures
2. nonspecific patchy infiltrates
3. ground-glass appearance; diffuse atelectasis; air bronchograms
4. coarse, irregular infiltrates; hyperexpansion; pneumothoraces

Back 157

1. transient tachypnea of the newborn (due to retained amniotic fluid)
2. congenital pneumonia
3. respiratory distress syndrome (RDS)
4. meconium aspiration

Front 158

What is the treatment for RDS?

Back 158

CPAP or intubation/mechanical ventilation
artificial surfactant administration (decr. mortality)
corticosteroids for mothers at risk for delivery at <30 weeks

monitor fetal lung maturity via lecithin:sphingomyelin ratio and presence of phosphatidylglycerol in amniotic fluid

Front 159

What are the potential complications of RDS and its treatment?

Back 159

persistent PDA
bronchopulmonary dysplasia
retinopathy of prematurity
barotrauma from positive pressure vent.
intraventricular hemorrhage
necrotizing enterocolitis

Front 160

Define cerebral palsy.

Back 160

a range of nonhereditary, nonprogressive disorders of movement and posture; the most common movement disorder in children

Front 161

What is the cause of cerebral palsy?

Back 161

perinatal neurologic insult (minority)
idiopathic (majority)

Front 162

Describe the two general categories of cerebral palsy.

Back 162

pyramidal (spastic): spastic paresis of any or all limbs; 75% of cases; mental retardation present in 90% of cases

extrapyramidal (dyskinetic): includes ataxic, choreoathetoid, and dystonic subtypes

Front 163

What are risk factors for febrile seizures?

In what age group do they usually occur?

Back 163

rapid incr. in temperature; febrile seizures in a close relative

6 mo - 5 years

Front 164

Compare simple v. complex febrile seizures in the following characteristics:

1. duration
2. distribution
3. # seizures per 24h
4. time between fever onset and seizure

Back 164

Simple (Complex)

1. <15 min (>15 min)
2. generalized (focal)
3. one (multiple)
4. seizure within hours of high fever (possibly low grade fever for days before seizure)

Front 165

What is the treatment for febrile seizures?

Back 165

acetaminophen* (not ASA since Reye's risk)
treat underlying illness

for complex seizures, thorough neurologic exam including EEG and MRI; chronic anticonvulsants may be necessary

* antipyretic therapy does NOT decrease recurrence of febrile seizures

Front 166

What is the risk of recurrence of febrile seizures? Within what time period?

Back 166

<30%, most within one year

Front 167

What are the risk factors for development of epilepsy?

Back 167

complex febrile seizures (~10% risk)
+ family history of epilepsy
abnormal neurological exam or developmental delay

Front 168

What are the most common childhood malignancies?

Back 168

ALL
CNS tumors
lymphomas

Front 169

Name the most frequent leukemias in children.

With what conditions is childhood leukemia associated?

Back 169

97% are ALL or AML (ALL > AML)

trisomy 21
Fanconi's anemia
prior radiation
SCID
congenital bone marrow failure states

Front 170

What are common presenting Sx in pediatric leukemia?

Back 170

abrupt in onset:
anorexia
fatigue
bone pain
fever (from neutropenia)
anemia
ecchymoses
petechiae
hepatosplenomegaly

Front 171

What is a chloroma?

Back 171

a greenish soft-tissue tumor on the skin or spinal cord; may be presenting symptom of AML

Front 172

Describe the syndrome that commonly develops at the onset of chemotherapy.

Back 172

tumor lysis syndrome:

hyperkalemia
hyperphosphatemia
hyperuricemia

Front 173

Define neuroblastoma.

In what age group does this tumor occur?

Back 173

an embryonal tumor of neural crest origin

more than half of patients are <2 years (70% with distant metastases at presentation)

Front 174

What conditions are associated with neuroblastoma (3)?

Back 174

neurofibromatosis
Hirschsprung's disease
N-myc oncogene

Front 175

What are the most common sites of neuroblastoma?

Back 175

(in order of frequency)
abdominal
thoracic
cervical

Front 176

What are some presenting signs/symptoms of neuroblastoma?

Back 176

depends on the location of the tumor

abdomen: nontender mass, HTN
thorax: Horner's syndrome
paraspinal: cord compression

Front 177

What are signs of distant metastasis of neuroblastoma (5)?

Back 177

bone marrow suppression
proptosis
hepatomegaly
subcutaneous nodules
opsoclonus/myoclonus

Front 178

How is neuroblastoma diagnosed?

Back 178

CT scan; fine needle aspirate of tumor
elevated 24h urinary catecholamines (VMA, HVA)
bone scan, bone marrow aspirate
CBC, LFTs, coags, chemistry

Front 179

What is the histologic appearance of neuroblastoma cells?

Back 179

small, round, blue tumor cells with characteristic rosette pattern

Front 180

Back 180

neuroblastoma

Front 181

With what conditions is Wilms' tumor associated?

Back 181

Beckwith-Wiedemann syndrome (hemihypertrophy, macroglossia, visceromegaly)
neurofibromatosis
WAGR syndrome (Wilms', Aniridia, GU abnormalities, mental Retardation)

Front 182

What is the presentation of Wilms' tumor?

Back 182

similar to abdominal neuroblastomas, but 2-5 years of age: asymptomatic, nontender, smooth abdominal mass

if present, symptoms include abdominal pain, fever, HTN, microscopic or gross hematuria

Front 183

Contrast Ewing's sarcoma and osteosarcoma on the following characteristic:

origin

Back 183

Ewing's sarcoma: neuroectoderm; associated with 11:22 translocation

osteosarcoma: osteoblasts (mesenchyme)

Front 184

Contrast Ewing's sarcoma and osteosarcoma on the following characteristic:

epidemiology

Back 184

Ewing's sarcoma: Caucasian male adolescents

osteosarcoma: male adolescents

Front 185

Contrast Ewing's sarcoma and osteosarcoma on the following characteristic:

history/physical exam

Back 185

Ewing's sarcoma: local pain and swelling + systemic Sx (fever, anorexia, fatigue)

osteosarcoma: local pain and swelling

Front 186

Contrast Ewing's sarcoma and osteosarcoma on the following characteristic:

location

Back 186

Ewing's sarcoma: midshaft of long bones

osteosarcoma: metaphyses of long bones (distal femur, proximal tibia and humerus); metastases to lungs in 20%

Front 187

Contrast Ewing's sarcoma and osteosarcoma on the following characteristic:

diagnosis

Back 187

Ewing's sarcoma: leukocytosis, elevated ESR; lytic bone lesion with "onion skin" periosteal rxn on plain film

osteosarcoma: elevated alk phos; "sunburst" lytic bone lesions; chest CT to r/o metastasis

Front 188

Contrast Ewing's sarcoma and osteosarcoma on the following characteristic:

treatment

Back 188

Ewing's sarcoma: local excision, chemotherapy, radiation

osteosarcoma: local excision, chemotherapy

Front 189

At what age should solid foods be introduced to children?

At what age should cow's milk be introduced to children?

Back 189

6 mos

12 mos

Front 190

Until what age is strabismus normal?

Define amblyopia.

Back 190

up to 3 mos

suppression of retinal images in misaligned eye, leading to permanent vision loss; possible consequence of uncorrected strabismus after 3 mos of age

Front 191

What are contraindications to vaccination?

Back 191

severe allergy to vaccine component or prior dose (egg allergy = no MMR vaccine)
encephalopathy within 7 days of prior pertussis vaccine
avoid live vaccines in immunocompromised and pregnant patients

Front 192

What live vaccines may be given to patients with HIV (2)?

Back 192

MMR
varicella

Front 193

Who should receive pneumococcal polysaccharide vaccine (PPV)?

Back 193

high-risk groups:

sickle cell disease
splenectomy
immunodeficient

Front 194

What are the presenting symptoms of lead poisoning (7)?

Back 194

irritability
hyperactivity/apathy
anorexia
intermittent abdominal pain
constipation
intermittent vomiting
peripheral neuropathy (wrist/foot drop)

Front 195

At what blood lead level is acute encephalopathy seen?

What are the signs of acute encephalopathy?

Back 195

>70 mcg/dL

incr. ICP
vomiting
confusion
seizures
coma

Front 196

What do CBC and peripheral blood smear show in lead poisoning?

Back 196

microcytic, hypochromic anemia
basophilic stippling

Front 197

What is the treatment for lead poisoning?

Back 197

<45 mcg/dL + asymptomatic: remove sources of exposure, retest at 1-3 months
45-69 mcg/dL: chelation therapy of inpt EDTA or outpt oral succimer (DMSA)
>70 mcg/dL: chelation therapy of inpatient EDTA + BAL (IM dimercaprol)