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Step2CK > Pediatrics Uworld > Flashcards

Pediatrics Uworld Flashcards

1
Q

7 year old with precocious puberty and exam shows pelvic mass on right ovary

A

granulosa cell tumor

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2
Q

friedreich ataxia

A 
most common spinocerebellar ataxia
auto recessive
onest before 22 years old
gait ataxia, falling, dysarthria
skeletal deformities
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3
Q

most common cause death in friedreich ataxia

A

cardiomyopathy- concentric hypertrophic cardiomyopathy

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4
Q

Tx for acute abnormal uterine bleeding in hemodynamically stable individual

A

high dose estrogen therapy

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5
Q

when is beswetting normal

A

before age 5

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6
Q

hormones in turners

A

high FSH from lack ofnegative feeback

low estrogen

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7
Q

any sexually active female under age 24 shoul dbe screen for what

A

C trach

N gonn

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8
Q

Acute otitis media

A

middle ear effusion with acute eardrum inflammation

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9
Q

otitis media with effusion

A

middle ear effusion without acute inflammation

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10
Q

streak ovaries

A

Turners

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11
Q

cholesteatoma

A

retraction pocket in middle ear that fills with granulation tissue and skin debris
can cause hearing loss, CN palsies, vertigo and abscesses

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12
Q

Tx cholesteatoma

A

send to specialist and may need CT

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13
Q

primary amenorrhea and has uterus on US

A

do FSH
if increased- karyotype
if decrease- cranial MRI

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14
Q

Chronic granulomatous disease pathogens

A 
catalase +
Staph
Serratia
Burkholderia
Aspergillus
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15
Q

Dx chronic granulomatous disease

A

Dihydrorhodamine 123 test

nitroblue tetrazolium test

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16
Q

what happens in chronic granulomatous disease

A

no phagocytic oxidative burst so cannot kill intracellular pathogens

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17
Q

signs chronic granulomatous disease

A

pneumonia, cutaneous abscesses, suppurative adenitis

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18
Q

Turners are at greatest risk of developing what

A

osteoporosis

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19
Q

kartagener inheritance

A

auto recessive

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20
Q

adolescent with nasal bostruction, visual mass and frequen nosebleeds

A

juvenile angiofibroma

go to specialist

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21
Q

Hereditary angioedema

A

C1 inhibitor deficiency
noninflammatory edema of face, limbs and genitalia
laryngeal edema- life threatening
edema of intestines causing abdominal pain
no urticaria

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22
Q

C1 deficiency leads to elevated levels of what

A

C2b and bradykinin

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23
Q

maternal estrogen effects in newborns

A

breast hypertropy
swollen labia
leukorrhea
uterine withdrawal bleeding

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24
Q

why wait till puberty to remove undescended testes from patient with andorgen insensitivty syndrome

A

benefits from gonad-stimulated puberty outweigh small risk malignancy

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25
Q

best thing for long term survivial SCID

A

stem cell transplant

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26
Q

defect in SCID

A

failure T cell development

B cell dysfunction from absent T cells

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27
Q

inheritance SCID

A

x linked recessive or auto recessive

28
Q

signs of SCID

A

recurrent viral fungal and opportunistic infections
failure to thrive
chronic diarrhea

29
Q

CD19+

A

B cells

30
Q

CD3+

A

T cells

31
Q

turners child has lymphedema

A

congenital probably from abnomral development of lymphatic network
can cause cystic hygroma

32
Q

lymphedema

pitting or nonpitting

A

nonpitting

33
Q

defect in mccune albright

A

defect in g protein cAMP kinase

34
Q

triad in McCune albright

A

precocious puberty
pigmentations (cafe au lait)
polyostotic fibrous dysplasia

35
Q

X linked aggamglobulinemia signs

A

recurrent sinopulmonary and GI infections after age 6 months

no tonsils or lymph nodes

36
Q

Dx of agammaglobulinemia

A

dec Ig and B cells
normal T cells
no response to vaccines

37
Q

Tx for X linked agammaglobulinemia

A

Ig replacement

prophylactic antibiotics

38
Q

defect in agammaglobulinemia

A

defect in tyrosine kinase to prevent development mature B cells

39
Q

defect in hyper IgM

A

x linked defect in CD40L

40
Q

recurrent skin and mucosal bacterial infections in a kid with no pus formation

A

leukocyte adhesion deficiency

41
Q

causes acute otitis media in kid

A

strep pneumo
H flu
moraxella catarrhalis

42
Q

Tx acute otitis media

A

initial is amoxicilin

again is ugmentin

43
Q

when to do myringostomy with tympanostomy tubes

A

> 3 episodes in 6 mo

>4 episodes in 12 months

44
Q

myotonic dystrophy

A

auto dominant expansion CTG in DMPK gene on chrom 19q

later onset age 12-30

45
Q

absent dystrophin gene

A

duchenne MD

presents 2- 3 years old

46
Q

reduced dystrophin gene

A

beckers MD

presents 5-15 years

47
Q

weight and height of child at 1 year old

A

weight should triple and height should increased by 50%

48
Q

language development of a 2 year old

A

200 words

2 word short sentences

49
Q

how to confirm duchenne MD

A

genetic testing

dystrophin gene on Xp21

50
Q

complications for infants small for gestational age

A

hypoxia, perinatal asphyxia, meconium aspiration, hypothermia, hypoglycemia, polycythemia

51
Q

risk factors for infants small for gestational age

A

preeclampsia, malnutrition, placental insufficiency, multiparity and drug use

52
Q

mutation in marfans

A

fibrillin 1

53
Q

CBC and Ig levels in chronic granulomatous disease

A

normal leukocyte, platelet and B and T cell concentrations

54
Q

what antibiotic prophylaxis do you give to patients with chronic granulomatous disease

A

TMP SMX and itraconazole

55
Q

contraindications to rotavirus vaccine

A

anaphylaxis to vaccine ingredients
Hx intussusception
Hx uncorrected congenital malformations of GI tract like meckels
SCID

56
Q

which intussusceptions are not seen with contrast enemas

A

small bowel

need US to see target sign

57
Q

Intussuceptions assoc with HSP

A

tend to be ileo ileal and actually require surgery

58
Q

how to remove foreign body from vagina in little kid

A

calcium alginate swab or irrigate with warmed fluids after topical antibiotic applied

59
Q

Immunizations are given to gestational age or chronological

A

chronological

60
Q

weight has to be above what to give first Hep B vaccine

A

> 2 kg

61
Q

complications cryptorchidism

A

inguinal hernia
testicular torsion
subfertility
testicular cancer

62
Q

dx for prader wili

A

karyotype and methylatoin studies

FISH and microsatellite probes

63
Q

Kallmans syndrome

A

46 XX

failure of migration of GnRJ and olfactory eurons

64
Q

hormone levels in kallmans syndrome

A

decreased FSH and LH

65
Q

Klinefelter karyotype

A

47XXY

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