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Flashcards in peds37 Deck (25):
1

kasabach-erritt syndrome

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

2

glanzmann's thomboasthenia

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

3

bernard-soulier syndrome

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

4

protein C

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

5

protein C def clinical features

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

6

treatment of protein C def

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

7

protein S def

hypercoagulability

8

antithrombin III def

hypercoagulability

9

factor V leiden def

hypercoagulability

10

kawasaki disease

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

11

mild neutropenia

1000-1500 cells

12

moderate neutropenia

500-1000 cells; infection involving skin and mucous membranes

13

severe neutropenia

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

14

most common cause of neutropenia in childhood

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

15

chronic benign neutropenia of childhood

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

16

clinical features of benign neutropenia of childhood

kids at incr risk for mild infections

17

labs in benign neutropenia of childhood

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

18

prognosis of benign neutropenia of childhood

most kids recover in months to a year

19

severe congenital agranulocytosis

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

20

cyclic neutropenia

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

21

chediak higashi syndrome

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

22

cartilage-hair hypoplasia syndrome

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

23

schwachman-diamond syndrome

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

24

hypersplenism can cause neutropenia how?

increased destruction

25

autoimmune neutropenia

anti-neutrophil antibodies are produced in response to infection