peds37

Question 1

kasabach-erritt syndrome

Answer 1

enlarging hemangioma, microangiopathic hemolytic anemia, thrombocytopenia, and consumptive coagulopathy

Question 2

glanzmann’s thomboasthenia

Answer 2

aut recess disorder characterized by decr ability of platelets to aggregate and form clot; def glycoprotein Iib/IIIa on platelet cell membrane

Question 3

bernard-soulier syndrome

Answer 3

aut recessive; decr platelet adhesion as a result of absence of plateelt membrane glycoproteins; large unusualy platelets seen on blood smear

Question 4

protein C

Answer 4

vit K dependent factor that is the most potent anticoag protein known

Question 5

protein C def clinical features

Answer 5

hypercoagulability; homozygotes at birth get pupura fulminans; hets present later with DVT or stroke

Question 6

treatment of protein C def

Answer 6

heparin, FFP, and warfarin; purified concentrates of protein C have been used

Question 7

protein S def

Answer 7

hypercoagulability

Question 8

antithrombin III def

Answer 8

hypercoagulability

Question 9

factor V leiden def

Answer 9

hypercoagulability

Question 10

kawasaki disease

Answer 10

autoimmune vasculitis in kids; most serious effect is on heart, where it can cause coronary artery aneurysms; more common in asians

Question 11

mild neutropenia

Answer 11

1000-1500 cells

Question 12

moderate neutropenia

Answer 12

500-1000 cells; infection involving skin and mucous membranes

Question 13

severe neutropenia

Answer 13

less than 500 cells; severe infections like pneumonia, sepsis, meningtiis; gram neg bacteria are typical organisms

Question 14

most common cause of neutropenia in childhood

Answer 14

infections that suppress bone marrow, marginate neutrophils, or exaust bone marrow reserves

Question 15

chronic benign neutropenia of childhood

Answer 15

common cause of neutropenia in kids less than 4 yo; noncyclic neutropenia is the only abnormality

Question 16

clinical features of benign neutropenia of childhood

Answer 16

kids at incr risk for mild infections

Question 17

labs in benign neutropenia of childhood

Answer 17

low absolute neutrophil count, with slightly low total WBC, bone marrow shows immature neutrophil precursurs (development of mature neutrophils is arrested)

Question 18

prognosis of benign neutropenia of childhood

Answer 18

most kids recover in months to a year

Question 19

severe congenital agranulocytosis

Answer 19

aka Kostmann syndrome; aut recessive; frequent life-threatening pyogenic bacterial infections; ANC is VERY low

Question 20

cyclic neutropenia

Answer 20

regular episodes of neutropenia with resultant infections; cycles last 21 days

Question 21

chediak higashi syndrome

Answer 21

aut recess; oculocutaneous albinism, neutropenia, blond or brown hair w silver streaks; large blue-gray granules in neutrophils

Question 22

cartilage-hair hypoplasia syndrome

Answer 22

aut reces; short staturs, immundef, fine hair, and neuropenia

Question 23

schwachman-diamond syndrome

Answer 23

exocrine pancreatic insuff, short stature caused by metaphyseal chondrodysplasia; and neutropenia; FTT and recurrent infections

Question 24

hypersplenism can cause neutropenia how?

Answer 24

increased destruction

Question 25

autoimmune neutropenia

Answer 25

anti-neutrophil antibodies are produced in response to infection