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shelf PEDS > VI > Flashcards

VI Flashcards

1
Q

most common malignancy in childhood

A

wilms tumor (nephroblastoma)

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2
Q

associated syndromes with Wilms tumor

A

WAGR, wilsm, aniridia, genitourinary, retardation

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3
Q

clinical presentation of wilms tumor

A

asymptomatic, firm, smooth, abdominal mass that does nto cross midline

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4
Q

treatment of wilms tumor

A

tumor excision, nephrectomy

chemo

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5
Q

Dx wilms tumoR

A

abdominal US then CT abdomen for chest to look for pulmonary mets

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6
Q

describe abdominal mass in neuroblastoma

A

crosses the membrane with systemic symptoms

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7
Q

pyelonephritis

A

fever, chills, flank pain, pyuria and bacteriuria

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8
Q

presentation of ALL

A

pallor and petechiae from bone marrow infiltration, anemia and thrombocytopenia
lymphadenopathy and HSM

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9
Q

how to confirm ALL

A

bone marrow biopsy

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10
Q

why is haptoglobin low in sickle cell

A

binds to haptoglobin depleting circulating amounts

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11
Q

What should sickle cell patients be vaccinated with

A

conjugate capsular polysaccharide

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12
Q

common live vaccinations in kids

A

mmr and varicella

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13
Q

common bacterial toxoid vaccines

A

tetanus and diphteria

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14
Q

hemorrhage in first week of life

A

vitamin K deficiency

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15
Q

IgA deficiency at risk for what transfusion reaction

A

anaphylaxis

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16
Q

X linke agammaglobulinemia

A

lack B cells and all clases Ig

sinopulmonary infections and pseudomonas

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17
Q

wiskott aldrich syndrome

A

X linked combined B and T cell disorder

recurrent otitis media, eczema, thrombocytopenia

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18
Q

Diamond-blackfan syndrome

A

congenital hypoplastic anemia pure red cell
macrocytic anemia, low reticulocyte count and congenital anomalies
webbed neck, short stature, cleft lip, shielded chest and triphalangeal thumbs

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19
Q

macrocytic anemia without hypersegmentation of nucleus in neutrophils

A

diamond backfin syndrome

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20
Q

what chemicals can cause idiopathic aplastic anemia

A

benzene, phenylbutazone, chloramphenicol, sulfonamides, viral hepatitis or ionizing radiation

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21
Q

fanconis anemia

A

auto recessive
progressive pancytopenia and macrocytosis
cafe au lait spots, microcephaly, microphthalmia, short stature, horseshoe kidneys and absent thumbs

22
Q

symptoms of anemia of prematurity

A

mildy tachycardia, increased apnea, poor weight gain

23
Q

Hct>65% in neonate

A

polycythemia

24
Q

what can cause increase EPO in neonate

A

intrauterine hypoxia from maternal DM, haternal HTN, smoking or IUGR

25
Q

clinical presentation of polycythemia in neonate

A 
ruddy skin, hypoglycemia
respiratory distress
cyanosis
apnea
irritability
jitteriness
abdominal distention
26
Q

Tx symptomatic polycythemia neonates

A

partial exchange transfusion

27
Q

ALL age group

A

2-10

28
Q

lymphoblasts on PBS in neonate with HSM and lymphadenopathy

A

ALL

29
Q

+TdT staining

A

ALL

30
Q

starry sky histologically

A

burkitt lymphoma

31
Q

auer rods

A

AML

32
Q

PAS + lymohocytes

A

could be ALL

33
Q

howell jolly bodies

A

hyposplenism

34
Q

bone marrow biopsy shoes hypocellular marrow with decreased megakaryocytes and precursors in cell ines

A

acquired aplastic anemia

35
Q

RBC RDW in thalassemias

A

normal

36
Q

RBC RDW in iron deficiency

A

increased

37
Q

pathogens in wiskott aldrich

A

strep pneumo
N mening
H influenza

many patients have eczema

38
Q

what causes thrombocytopenia in wiskott aldrich

A

decreased platelet production

39
Q

what is seen on genetic analysis in fanconi anemia

A

chromosomal breaks

40
Q

describe presentation of hereditary angioedema

A

rapid onset:

  • noninflammatory edema of the face, limbs genitalia
  • laryngeal edema of intestines
  • no urticaria
41
Q

what causes herediaty angioedema

A

C1 inhibitor deficiency

42
Q

what is elevated in c1 inhibitor deficiency

A

C2b and bradykinin

43
Q

What tpe of HS is Rh hemolytic anemia

A

II

44
Q

type I HS

A

IgE, mast cells, pruritis and urticaria

45
Q

cell mediated HS

A

IV
ppd
allergic contact dermatitis

46
Q

HS III

A

immune complex deposition like in serum sickness

47
Q

what does not work in chronic granulomatous disease

A

mutation causing loss of NADPH oxidase

48
Q

infections in chronic granulomatous disease

A

staph aureus

any catalase expressing organisms

49
Q

what killing method is dysfunctional inc chronic granulomatous disease

A

intracellular killing– so find neutrophils with bacteria in them

50
Q

deficiency in SCID

A

adenosine deaminase deficiency

auto recessive

51
Q

msot common infections in SCID

A

pneumocystis jiroveci, candida, parainfluenza, and HSV

52
Q

agammaglobulinemia

A

B cell maturation arrest
no ab
get sinusitis, otitis, penumonia

shelf PEDS (13 decks)

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