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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
best thing for long term survivial SCID
stem cell transplant
26
defect in SCID
failure T cell development | B cell dysfunction from absent T cells
27
inheritance SCID
x linked recessive or auto recessive
28
signs of SCID
recurrent viral fungal and opportunistic infections failure to thrive chronic diarrhea
29
CD19+
B cells
30
CD3+
T cells
31
turners child has lymphedema
congenital probably from abnomral development of lymphatic network can cause cystic hygroma
32
lymphedema | pitting or nonpitting
nonpitting
33
defect in mccune albright
defect in g protein cAMP kinase
34
triad in McCune albright
precocious puberty pigmentations (cafe au lait) polyostotic fibrous dysplasia
35
X linked aggamglobulinemia signs
recurrent sinopulmonary and GI infections after age 6 months | no tonsils or lymph nodes
36
Dx of agammaglobulinemia
dec Ig and B cells normal T cells no response to vaccines
37
Tx for X linked agammaglobulinemia
Ig replacement | prophylactic antibiotics
38
defect in agammaglobulinemia
defect in tyrosine kinase to prevent development mature B cells
39
defect in hyper IgM
x linked defect in CD40L
40
recurrent skin and mucosal bacterial infections in a kid with no pus formation
leukocyte adhesion deficiency
41
causes acute otitis media in kid
strep pneumo H flu moraxella catarrhalis
42
Tx acute otitis media
initial is amoxicilin | again is ugmentin
43
when to do myringostomy with tympanostomy tubes
>3 episodes in 6 mo | >4 episodes in 12 months
44
myotonic dystrophy
auto dominant expansion CTG in DMPK gene on chrom 19q | later onset age 12-30
45
absent dystrophin gene
duchenne MD | presents 2- 3 years old
46
reduced dystrophin gene
beckers MD | presents 5-15 years
47
weight and height of child at 1 year old
weight should triple and height should increased by 50%
48
language development of a 2 year old
200 words | 2 word short sentences
49
how to confirm duchenne MD
genetic testing | dystrophin gene on Xp21
50
complications for infants small for gestational age
hypoxia, perinatal asphyxia, meconium aspiration, hypothermia, hypoglycemia, polycythemia
51
risk factors for infants small for gestational age
preeclampsia, malnutrition, placental insufficiency, multiparity and drug use
52
mutation in marfans
fibrillin 1
53
CBC and Ig levels in chronic granulomatous disease
normal leukocyte, platelet and B and T cell concentrations
54
what antibiotic prophylaxis do you give to patients with chronic granulomatous disease
TMP SMX and itraconazole
55
contraindications to rotavirus vaccine
anaphylaxis to vaccine ingredients Hx intussusception Hx uncorrected congenital malformations of GI tract like meckels SCID
56
which intussusceptions are not seen with contrast enemas
small bowel | need US to see target sign
57
Intussuceptions assoc with HSP
tend to be ileo ileal and actually require surgery
58
how to remove foreign body from vagina in little kid
calcium alginate swab or irrigate with warmed fluids after topical antibiotic applied
59
Immunizations are given to gestational age or chronological
chronological
60
weight has to be above what to give first Hep B vaccine
>2 kg
61
complications cryptorchidism
inguinal hernia testicular torsion subfertility testicular cancer
62
dx for prader wili
karyotype and methylatoin studies | FISH and microsatellite probes
63
Kallmans syndrome
46 XX | failure of migration of GnRJ and olfactory eurons
64
hormone levels in kallmans syndrome
decreased FSH and LH
65
Klinefelter karyotype
47XXY

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