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Flashcards in Pediatrics Uworld Deck (65):
1

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

granulosa cell tumor

2

friedreich ataxia

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

3

most common cause death in friedreich ataxia

cardiomyopathy- concentric hypertrophic cardiomyopathy

4

Tx for acute abnormal uterine bleeding in hemodynamically stable individual

high dose estrogen therapy

5

when is beswetting normal

before age 5

6

hormones in turners

high FSH from lack ofnegative feeback
low estrogen

7

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

C trach
N gonn

8

Acute otitis media

middle ear effusion with acute eardrum inflammation

9

otitis media with effusion

middle ear effusion without acute inflammation

10

streak ovaries

Turners

11

cholesteatoma

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

12

Tx cholesteatoma

send to specialist and may need CT

13

primary amenorrhea and has uterus on US

do FSH
if increased- karyotype
if decrease- cranial MRI

14

Chronic granulomatous disease pathogens

catalase +
Staph
Serratia
Burkholderia
Aspergillus

15

Dx chronic granulomatous disease

Dihydrorhodamine 123 test
nitroblue tetrazolium test

16

what happens in chronic granulomatous disease

no phagocytic oxidative burst so cannot kill intracellular pathogens

17

signs chronic granulomatous disease

pneumonia, cutaneous abscesses, suppurative adenitis

18

Turners are at greatest risk of developing what

osteoporosis

19

kartagener inheritance

auto recessive

20

adolescent with nasal bostruction, visual mass and frequen nosebleeds

juvenile angiofibroma
go to specialist

21

Hereditary angioedema

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

22

C1 deficiency leads to elevated levels of what

C2b and bradykinin

23

maternal estrogen effects in newborns

breast hypertropy
swollen labia
leukorrhea
uterine withdrawal bleeding

24

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

benefits from gonad-stimulated puberty outweigh small risk malignancy

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