VI

Question 1

most common malignancy in childhood

Answer 1

wilms tumor (nephroblastoma)

Question 2

associated syndromes with Wilms tumor

Answer 2

WAGR, wilsm, aniridia, genitourinary, retardation

Question 3

clinical presentation of wilms tumor

Answer 3

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

Question 4

treatment of wilms tumor

Answer 4

tumor excision, nephrectomy

chemo

Question 5

Dx wilms tumoR

Answer 5

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

Question 6

describe abdominal mass in neuroblastoma

Answer 6

crosses the membrane with systemic symptoms

Question 7

pyelonephritis

Answer 7

fever, chills, flank pain, pyuria and bacteriuria

Question 8

presentation of ALL

Answer 8

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

Question 9

how to confirm ALL

Answer 9

bone marrow biopsy

Question 10

why is haptoglobin low in sickle cell

Answer 10

binds to haptoglobin depleting circulating amounts

Question 11

What should sickle cell patients be vaccinated with

Answer 11

conjugate capsular polysaccharide

Question 12

common live vaccinations in kids

Answer 12

mmr and varicella

Question 13

common bacterial toxoid vaccines

Answer 13

tetanus and diphteria

Question 14

hemorrhage in first week of life

Answer 14

vitamin K deficiency

Question 15

IgA deficiency at risk for what transfusion reaction

Answer 15

anaphylaxis

Question 16

X linke agammaglobulinemia

Answer 16

lack B cells and all clases Ig

sinopulmonary infections and pseudomonas

Question 17

wiskott aldrich syndrome

Answer 17

X linked combined B and T cell disorder

recurrent otitis media, eczema, thrombocytopenia

Question 18

Diamond-blackfan syndrome

Answer 18

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

Question 19

macrocytic anemia without hypersegmentation of nucleus in neutrophils

Answer 19

diamond backfin syndrome

Question 20

what chemicals can cause idiopathic aplastic anemia

Answer 20

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

Question 21

fanconis anemia

Answer 21

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

Question 22

symptoms of anemia of prematurity

Answer 22

mildy tachycardia, increased apnea, poor weight gain

Question 23

Hct>65% in neonate

Answer 23

polycythemia

Question 24

what can cause increase EPO in neonate

Answer 24

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

Question 25

clinical presentation of polycythemia in neonate

Answer 25

``` ruddy skin, hypoglycemia respiratory distress cyanosis apnea irritability jitteriness abdominal distention ```

Question 26

Tx symptomatic polycythemia neonates

Answer 26

partial exchange transfusion

Question 27

ALL age group

Answer 27

2-10

Question 28

lymphoblasts on PBS in neonate with HSM and lymphadenopathy

Answer 28

ALL

Question 29

+TdT staining

Answer 29

ALL

Question 30

starry sky histologically

Answer 30

burkitt lymphoma

Question 31

auer rods

Answer 31

AML

Question 32

PAS + lymohocytes

Answer 32

could be ALL

Question 33

howell jolly bodies

Answer 33

hyposplenism

Question 34

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

Answer 34

acquired aplastic anemia

Question 35

RBC RDW in thalassemias

Answer 35

normal

Question 36

RBC RDW in iron deficiency

Answer 36

increased

Question 37

pathogens in wiskott aldrich

Answer 37

strep pneumo N mening H influenza many patients have eczema

Question 38

what causes thrombocytopenia in wiskott aldrich

Answer 38

decreased platelet production

Question 39

what is seen on genetic analysis in fanconi anemia

Answer 39

chromosomal breaks

Question 40

describe presentation of hereditary angioedema

Answer 40

rapid onset: - noninflammatory edema of the face, limbs genitalia - laryngeal edema of intestines - no urticaria

Question 41

what causes herediaty angioedema

Answer 41

C1 inhibitor deficiency

Question 42

what is elevated in c1 inhibitor deficiency

Answer 42

C2b and bradykinin

Question 43

What tpe of HS is Rh hemolytic anemia

Answer 43

II

Question 44

type I HS

Answer 44

IgE, mast cells, pruritis and urticaria

Question 45

cell mediated HS

Answer 45

IV ppd allergic contact dermatitis

Question 46

HS III

Answer 46

immune complex deposition like in serum sickness

Question 47

what does not work in chronic granulomatous disease

Answer 47

mutation causing loss of NADPH oxidase

Question 48

infections in chronic granulomatous disease

Answer 48

staph aureus | any catalase expressing organisms

Question 49

what killing method is dysfunctional inc chronic granulomatous disease

Answer 49

intracellular killing-- so find neutrophils with bacteria in them

Question 50

deficiency in SCID

Answer 50

adenosine deaminase deficiency | auto recessive

Question 51

msot common infections in SCID

Answer 51

pneumocystis jiroveci, candida, parainfluenza, and HSV

Question 52

agammaglobulinemia

Answer 52

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