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shelf PEDS > VII > Flashcards

VII Flashcards

1
Q

digeorge syndrome dysmorphogenesis of what structures

A

third or fourth pharyngeal pouches

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2
Q

immunizations should be given based on chronological or gestational age

A

chronological

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3
Q

what are the inactivated pediatric vaccines

A

polio

hep A

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4
Q

what are the inactivated toxin (toxoid) vaccines

A

diptheria, tetanus

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5
Q

what are the conjugate vaccines

A 
hep B
pertussis
H influenza type B
pneumococcal
meningococcal
hpv
influenza
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6
Q

what are the live attenuated vaccines

A 
rotavirus
measles
mumps
rubella
varicella
influenza
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7
Q

weight minimum for first hep B vaccine

A

> 2 kg

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8
Q

Dx confirmation SCID

A

absent lymph nodes and tonsils, lymphopenia, absent thymic shadow on CX, abnormal T,B and natural killer cell enumeration by flow cytometric analysis

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9
Q

X linked aggamaglobulinemia

A

also known as Brutons

male infant asymptomatic until 6-9 mo then recurrent S pneumo and H influenza

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10
Q

Dx confirmation brutons agammaglobulinemia

A

decreased serum concentrations IgG IgA, IgM and IgE

no decrease in circulating B cells

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11
Q

less than 2% CD19+ B cells in peripheral circulations

A

x linked agammaglobulinemia

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12
Q

immunologic findings of wiskott aldrich syndrome

A

low IgM
high IgA and IgE
poor Ab response to polysacharide Ag
reduced # T cells and platelets

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13
Q

Dx by nitro blue tetrazolium test

A

chronic granulomatous disease

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14
Q

Tx hyepr IgM

A

antibiotic prophylaxis and interval administration IV immunoglobulin

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15
Q

infections with leukocyte adhesion deficiency type 1

A

S aureus

gram negative

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16
Q

deficient expression of CD18

A

leukocyte adhesion deficiency

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17
Q

contraindications to diptheria/tetanus

A

anaphylxis to vaccine ingredients

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18
Q

contraindications to pertussis

A

anaphylaxis to vaccine ingredients
progressive neurologic disorder
encephalopathy within week of previous vaccine dose

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19
Q

defect in x agammaglobulinemia

A

defect in tyrosine kinase in B cells

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20
Q

lack of IgA increase risk of what protozoan

A

giardia

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21
Q

Chediak Higashi syndrome

A

decreased degranulation, chemotaxis and granulopoiesis

22
Q

signs of chediak higashi syndrome

A

mild coagulopathy, peripheral and cranial neuropathy, hepatosplenomegaly, pancytopenia, partial oculocutaneous albinism, frequent bacterial infections and progressive lymphoproliferative syndrome

23
Q

giant lysosomes in neutrophils

A

chediak higashi syndrome

24
Q

Tx chediak higashi syndrome

A

TMP SMX

25
Q

Hyper IgE

A

jobs syndrome: chronic pruritis dermatitis, recurrent staphylococcal infections, markedly elevated IgE levels, eosinophilia and coarse facial features

26
Q

gynecomastia in pubertal male

A

reassurance

resolve in few mo to 2 years w/o intervention

27
Q

what causes gynecomastia in pubertal male

A

increased testicular production of E

28
Q

elevated prolactin in male

A

breast enlargement with hypogonadism

29
Q

premature development secondary sexual characteristics ages

A

girls

30
Q

isolated premature adrenarche

A

increased adrenal androgen

31
Q

typical manifestations of increased adrenal androgen

A

body odor, oily hair, skin, acne, pubic hair, axillary hair

32
Q

laboratory findings in premature adrenarche

A

increased DHEA-S

testosterone low

33
Q

isolated premature adrenarche puts significant risk for

A

polycystic ovarian syndrome
type 2 DM
metabolic syndrome

34
Q

what causes gonadotropin-dependent precocious puberty

A

premature activation of hypothalamic pituitary gonadal axis

35
Q

clinical management of gonadotropin-dependent precocious puberty

A

adrenarche, telarche, pubarche or advanced age

36
Q

imaging for those with GDPP and elevated LH

A

brain MRI with contrast

37
Q

Tx idiopathic gonadotropin-dependent precocious puberty

A

GnRH agonist to prevent premature epiphyseal plate fusion

38
Q

enzyme deficiency in congenital adrenal hyperplasia

A

21-hydroxylase deficiency CYP21A2

39
Q

21 hydroxylase converts what

A

17 hydroxyprog to 11 deoxycortisol

40
Q

CAH in neonatal period

A

adrenal insufficiency and ambiguous genitalia

41
Q

late onset Congenital adrenal hyperplasia

A

androgen excess
premature adrenarch/pubarche
severe cystic acne resistant to treatment
accelerated linear growth and advanced bone age

42
Q

which onset congenital adrenal hyperplasia has salt wasting

A

classic congenital adrenal hyperplasia (neonatal)

43
Q

most common cause primary hypogonadism

A

klinefelter syndrome 47 XXY

44
Q

advanced bone age
coarse axillary
pubic hair
and severe cystic acne

A

late onset congenital adrenal hyperplasia

from 21-hydroxylase deficiency

45
Q

congenital hypothyroid

A

normal at birth

develop apathy, weakness, hypotonia, large tongue, sluggish movement, abdominal bloating and an umbilical hernia

46
Q

wernig hoffman syndrome

A

auto recessive

degeneration anterior horn cells and cranial nerve motor nuclei

47
Q

refeeding syndrome

A

starvation or anorexia patient
now has food, stimulate insulin,
now uptake K, phosphorus and Mg
can lead to arrhythmias

48
Q

steroid increased in 21-hydroxylase deficiency

A

17 hydroxyprog

49
Q

female effects of deficient 21 hydroxylase or congenitaladrenal hyperplasia

A

virilization

50
Q

differentiate central precocioub puberty and peripheral

A

central have elevated FSH and LH

peripheral have low FSH and LH

51
Q

most common cause of congenital hypothyroid

A

thyroid dysgenesis

shelf PEDS (13 decks)

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