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USMLE Step Two > Pediatric Disorders > Flashcards

Pediatric Disorders Flashcards

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USMLE® Step 1 flashcards
1
Q

cutis marmorata

A

lacy, reticulated vascular pattern over most of body when baby is cooled, improves after first month, can be associated with some syndromes, non-concerning

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2
Q

milia

A

firm, white papules, inclusion cyst, on palate midline; epstein pearls, spontaneous resolution

caused by accumulation of sweat beneath eccrine sweat ducts

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3
Q

salmon patch (nevus simplex)

A

pale, pink vascular macules found in nuchal area, glabella, eyelids, usually symmetric, facial ones disappear, posterior ones persist

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4
Q

mongolian spots

A

blue or slate-gray macules, seen on presacral, back, posterior thighs, arrest melanocytes, usually fade over first few years

differential: child abuse

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5
Q

erythema toxicum

A

firm, yellow-white papules/pustules with erythematous base, peaks on second day of life, contain eosinophils, self-limited

differential: staphylococcal scalded skin syndrome

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6
Q

port wine stain (nevus flammeus)

A

vascular malformation, permanent defect, unilateral, most on head and neck, rule out sturge-weber syndrome

treat with pulsed laser

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7
Q

hemangioma

A

superficial: bright red, protuberant, appear in first 2 months, involution by 5-9 years of age
deeper: bluish hue, firm, less likely to regress, treat with steroids or pulsed laser if huge and interfering with function

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8
Q

neonatal acne

A

erythematous papules on face, caused by maternal androgens, no treatment

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9
Q

preauricular tags/pits

A

look for hearing loss and genitourinary anomalies

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10
Q

nevus sebaceous

A

alopecia of orange-colored, nodular skin, possible malignant degeneration, remove before adolescence

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11
Q

coloboma of iris

A

cleft at “6 o’ clock” position, associated with CHARGE syndrome

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12
Q

aniridia

A

hypoplasia of iris, defect usually through to retina, associated with wilms tumor

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13
Q

branchial clef cyst

A

mass arising laterally, infections common, requires surgical removal

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14
Q

thyroglossal duct cyst

A

mass arising midline, moves with swallowing

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15
Q

hypospadias

A

urethral opening on ventral surface (under surface), common with other anomalies, no cricumcision

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16
Q

epispadias

A

urethral opening on dorsal surface (top surface)

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17
Q

phenylketonuria diagnosis and management

A

phenylalanine hydroxylase accumulation
mental retardation, vomiting, growth retardation, hyperactive, purposeless movements, athetosis, seizures
fair hair, fair skin, blue eyes, tooth abnormalities, microcephaly
MR occurs gradually over first few months

treatment: low phenylalanine diet for life

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18
Q

classic galactosemia diagnosis and management

A

G-1-P uridylyltransferase deficiency, accumulation of G-1-P with injury to kidney, liver, and brain
jaundice, hepatomegaly, hypoglycemia, cataracts, seizures, MR
predisposition to e. coli sepsis
may begin prenatally

treatment: no lactose reverses growth failure and cataracts but not neurodevelopmental problems

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19
Q

symmetric IUGR

A

early, in utero insult

genetic syndromes, chromosomal abnormalities, congenital infections, teratogens, toxins

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20
Q

asymmetric IUGR (head bigger than body)

A

relatively late onset after fetal organ development, abnormal delivery of nutritional substances and oxygen to the fetus
uteroplacental insufficiency secondary to maternal diseases, risk of neurologic asphyxia

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21
Q

characteristics of post-term infants

A 
increased birth weight
absence of lanugo
decreased/absent vermix
desquamating, pale, loose skin
abundant hair, long nails
meconium staining
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22
Q

transient tachypnea of the newborn

A

slow absorption of fetal lung fluid, decreased pulmonary compliance
tachypnea after birth, common in term infant delivered by c-section or rapid second stage of labor
rapid improvemen within hours or days

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23
Q

meconium aspiration

A

meconium passed from hypoxia or fetal distress can be aspirated, causing respiratory distress and hypoxemia, overdistention of chest, patchy infiltrates, increased AP diameter

treatment: positive pressure ventilation, nitric oxide, ECMO

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24
Q

diaphragmaic hernia

A

failure of diaphragm to develop, abdominal contents enter chest causing pulmonary hypoplasia
bowel sounds easily heard in chest

treatment: immediate intubation, ECMO, followed by surgical correction

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25
Q

meconium ileus

A

associated with cystic fibrosis, hirschsprung disease

treatment: high gastrografin enema

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26
Q

necrotizing enteroclitis

A

transmural intestinal necrosis caused by prematurity, introduction of feeds

treatment: cessation of feeds, gut decompression, systemic antibiotics, surgical resection of necrotic bowel if necessary

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27
Q

duodenal atresia

A

bilious vomiting from first feeding sign, AXR shows double-bubble sign

treatment: surgical correction

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28
Q

imperforate anus

A

failure to pass stool after birth, no anal opening visible

treatment: surgical correction

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29
Q

breast-feeding jaundice

A

jaundice that occurs in the first days of life, baby does not nurse adequately, becomes dehydrated, lack of calories causes jaundice

treatment: rehydrate baby

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30
Q

breast-milk jaundice

A

jaundice occurs in second week of life, occurs due to glucuronidase present in some breast milk

treatment: stop breast feeding for 1-2 days, bilirubin should fall significantly, although it will rise again, it will not rise to previous level, baby may then be safely breast fed

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31
Q

toxoplasmosis diagnosis and management

A

jaundice, hepatosplenomegaly, thrombocytopenia, anemia, microcephaly, chroioretinitis, hydrocephalus, intracranial calcifications, seizures

treatment: maternal treatment during pregnancy with spiramycin, infants treated with sulfonamide or pyrimethamine for first 6 months of life

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32
Q

congenital rubella

A

blueberry muffin rash, PDA, peripheral pulmonary artery stenosis, cataracts, congenital hearing loss, hepatosplenomegaly, thrombocytopenia

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33
Q

babies lose weight in the first few days after birth, when is original birth weight regained by

A

2 weeks of age

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34
Q

when does birth weight double by

A

4 months

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35
Q

what does birth weight triple by

A

12 months

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36
Q

when does birth weight quadruple by

A

24 months

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37
Q

birth height increases by 50% by this time

A

1 year

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38
Q

birth height doubles by this time

A

4 years

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39
Q

birth height triples by this time

A

13 years

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40
Q

how much does head circumference grow by during 0-3 months

A

5 cm

41
Q

how much does head circumference grow by during 3-6 months

A

4 cm

42
Q

how much does head circumference grow by during 6-9 months

A

2 cm

43
Q

how much does head circumference grow by during 9-12 months

A

1 cm

44
Q

car seat guidelines

A

< 1 year and < 20 pounds: face backward in the middle seat

> 1 year AND > 20 pounds: may face forward

> 40 pounds: move into booster seat until seatbelt still functions properly (4’ 9”)

45
Q

how much calories are in breast milk and formula?

A

20 kcal/ounce for both

46
Q

what is the caloric requirements in babies younger than 6 months

A

100-120 kcal/kg/day

47
Q

what workup should be performed in infant with single umbilical artery

A

obtain renal sonogram to check for anomalies

48
Q

caput v. cephalohematoma

A

caput: diffuse swelling/edema of the scalp that crosses suture lines and resolves within a few days
cephalohematoma: does not cross suture lines, resolves within weeks-months, increases likelihood of jaundice, more common with vacuum deliveries

49
Q

harlequin color change

A

intense reddening of gravity dependent side, may be due to immature autonomic system, completely benign and will resolve in days-weeks

50
Q

macular stains (stork bites)

A

permanent vascular malformations on nape of neck, upper eyelids, and middle of forehead, benign but permanent

51
Q

SIDS

A

usually occurs 2-4 months of age
associated with low birth weight, prone sleeping position, sleeping on soft surface, overheating

prevention methods: supine position, pacifier use

52
Q

when does anterior fontanelle close

A

96% by 24 months
if delayed, consider down sydrome, rickets, achondroplasia, congenital hypothyroidism
if closes too early, monitor for craniosynostosis

53
Q

PFAPA syndrome

A

benign 4-5 day syndrome consisting of periodic fever, aphthous ulcers, pharyngitis, and adenitis, occurs monthly, usually affects preschool-aged children

treatment: glucocorticoides, maybe cimetidine

54
Q

pertussis

A

7-10 days incubation period
mild URI symptoms precede paroxysmal cough, inspiratory whoop, and post-tussive emesis (can go on for weeks)

treatment: erythromycin/azithromycin, TMP-SMX

55
Q

additional work-up in child diagnosed with UTI

A

voiding cystourethrogram and renal U/S in boys all ages or girls younger than 3, children with febrile or recurrent UTIs

56
Q

2-3 mm yellow pustules with red base arising in first 24-72 hours after birth, pustular contents reveal numerous eosinophils

A

erythema toxicum

57
Q

spider-webbing/marbling of the skin, benign condition

A

cutis marmorata

58
Q

intense redding of gravity dependent side and blanching of the nondependent side with a line of demarcation between the two, lasting seconds-minutes

A

harlequin color change

59
Q

due to accumulation of sweat beneath eccrine sweat ducts that are obstructed by keratin at the stratum corneum

A

milia

60
Q

what medication is used for measles

A

vitamin A, possibly ribavirin

61
Q

treatment for roseola infantum

A

anti-pyretics

62
Q

etiology of delayed closure of the anterior fontanelle

A

down syndrome, rickets, achnodroplasia, congenital hypothryoidism

63
Q

how would you expect weight to increase in the first 2 years of life

A

quadruple from birth weight

64
Q

when do children first exhibit stranger anxiety

A

6 months

65
Q

when is gender identity typically formed

A

2 years

66
Q

when can children begin to eat solid foods

A

4-6 months

67
Q

when can children drink cow’s milk

A

12 months

68
Q

what interventions have been shown to reduce the incidence of SIDS

A

supine sleeping, pacifier, fan on in the room, no smoking, no toys in bed

69
Q

when can a child convert from rear-facing to front-facing car seats

A

must be > 1 year AND > 20 pounds

70
Q

how many total doses of DTaP vaccine should a 6-year-old have received

A

5 doses

71
Q

what age is meningococcal vaccine indicated

A

after 11 years

72
Q

bruton’s agammaglobulinemia

A

X-linked, B cell deficiency, defective tyrosine kinase gene

low levels of all IgGs, recurrent bacterial infections after 6 months

73
Q

digeorge syndrome (thymic aplasia)

A

3rd and 4th pouches fail to develop, no thymus or parathyroid, no T cells, hypocalcemia and tetany
congenital defects in heart/great vessels
recurrent viral, fungal, and protozoal infections
associated with 22q11 deletion

74
Q

severe combined immunodeficiency

A

severe recurrent infections, chronic mucocutaneous candidiasis, chronic diarrhea, failure to thrive, no thymic shadow
do not give live vaccines

75
Q

chronic mucocutaneous candidiasis

A

T cell dysfunction, treat with anti-fungals

76
Q

wiskott-aldrich immunodeficiency

A

thrombocytopenia, purpura, eczema, recurrent pyogenic infections

77
Q

ataxia-telangiectasia

A

IgA deficiency
cerebellar ataxia, poor smooth pursuit of moving target with eyes, elevated AFP after 8 months, telangiectasias of face, increased risk of lymhomas and leukemias
radiation sensitivity

78
Q

glycogen storage disease: lactic acidosis, hyperlipidemia, hyperuricemia

A

von gierk’s disease

liver cannot perform gluconeogenesis

79
Q

glycogen storage disease: diaphragm weakness, respiratory failure

A

pompe’s disease (adult form)

80
Q

glycogen storage disease: increased glycogen in liver, severe fasting hypoglycemia

A

von gierk’s disease

liver cannot perform gluconeogenesis

81
Q

glycogen storage disease: hepatomegaly, hypoglycemia, hyperlipidemia (normal kidneys, lactate, and uric acid)

A

cori’s disease

82
Q

glycogen storage disease: painful muscle cramps, myoglobinuria with strenuous exercise

A

mcardle’s disease

83
Q

glycogen storage disease: severe hepatosplenomegaly, enlarged kidneys

A

von gierk’s disease

liver cannot perform gluconeogenesis

84
Q

congenital heart defect, low calcium, recurrent infections

A

digeorge syndrome

85
Q

chronic mucocutaneous candidiasis, chronic diarrhea, failure to thrive

A

SCID

86
Q

trombocytopenia, eczema, and recurrent infections

A

wiskott-aldrich

87
Q

poor smooth pursuit of eyes, elevated AFP after 8 months

A

ataxia-telangiectasia

88
Q

partial albinism, recurrent URIs, neurological disorders

A

chediak-higashi disease

89
Q

cleft lip/palate, life expectancy less than 1 year, polydactyly

A

trisomy 13

90
Q

high-pitched cat-like cry

A

cri-du-chat

91
Q

elfin facial features, cardiac defects

A

william’s syndrome

92
Q

congenital anomaly with obesity and over eating

A

prader-willi syndrome

93
Q

micrognathia, life expectancy less than 1 year, rocker-bottom feet

A

trisomy 18

94
Q

happy mood, inappropriate laughter, ataxic gait

A

angelman syndrome

95
Q

large ears, MR, macroorchidism

A

fragile X syndrome

96
Q

MR, simian crease, GI and cardiac defects

A

trisomy 21

97
Q

short stature, infertility, coarctation of aorta

A

turner syndrome

98
Q

when do infections typically begin in children with immune disorders

A

after the first 3 months of life

USMLE Step Two (17 decks)

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