Peds Exam 2 Hematologic D/os

Front 1

common condition in pediatrics
nutritional def no.1 cause

Back 1

iron def anemia

Front 2

causes of iron def anemia

Back 2

inadeq iron supply
impaired absorption
excessive demands
Hgn synthesis impairment
blood loss

Front 3

high risk groups for iron def anemia

Back 3

low income (WIC has dec frequency/rates)
ages 6m-24m
adolescents
vegetarian diets

Front 4

iron def anemia
S/S

Back 4

sympt r/t decreased O2 carrying capacity
pallor, poor muscle tone, infection prone, under/overweight
(edema, retarded growth)

Front 5

microcytic RBCs are typical finding (MCV)

Back 5

Fe def anemia

Front 6

iron def anemia
Dx

Back 6

H&P
labs (CBC, retics, stool for occult blood)

inf MCV >70, older MCV >70 + age/retics usually normal
p

Front 7

iron def anemia
management

Back 7

iron supplementatin (2 doses, btw meals)
meds can make stools tarry, constipation --> stool softener, use straw/syringe for liquid
educ--breast of Fe fort formula

Front 8

defect in the proteins that for the RBC membrane

causes cells to be inflexible, do not circulate well through spleen, prone to destruction

Back 8

hereditary spherocytosis

Front 9

hereditary spherocytosis S/S

Back 9

anemia, splenomegaly, jaundice

Front 10

often presents in first 24 hrs of life as severe hyperbilirubinemia

Back 10

hereditary spherocytosis

Front 11

last immature stage of a RBC

Back 11

reticulocyte

Front 12

hereditary spherocytosis Dx

Back 12

HgB, retics

Front 13

aplastic crisis

Back 13

pot comp of hereditary spherocytosis

sudden cessation of RBC production (Hgb, Hct drop rapidly)
us requires tranfusion, slower trans in peds, in ICU settin

Front 14

splenectomy

Back 14

pot comp of hereditary spherocytosis
fx is to stop splenic destruction of spherocytes
defect remains
5yrs+ for sx, fully immunized, proph penicillin

Front 15

most common inherited disorder

Back 15

sickle cell dz

Front 16

sickle cell life expectancy

Back 16

40 yr

Front 17

sickle cell
HgbSS

Back 17

sickle cell anemia, most severe form
two copies of sickle gene
children freq hospitalized

Front 18

sickle cell
HgbAS

Back 18

sickle cell trait
carrier, usually asymptomatic, one normal copy

Front 19

sickle cell patho

Back 19

O2 levels fall-->cells sickle-->sickled cells are trapped in small vessels-->deox & dec pH increase sickling-->blood viscosity increases leading to further sickling

(thrombosis, ischemia, infarction, acidosis)

Front 20

vasoocclusive crisis

Back 20

sickled cells lead to occlusions, causing pain in area, leading to cellular death

Front 21

this protects infants from effects of sickle cell

Back 21

fetal hemoglobin

FH does not sickle, ~6mos, fetal hemoglobin is replaced

Front 22

dactylitis

Back 22

hand-foot dz (seen w/ sickle cell)
seen 6m-2y, swollen hands and feet r/t infarction of short, tubular bone vessels

Front 23

functional asplenia

Back 23

seen w/ sickle cell
splenic tissue replaced w/ fibrous tissue, loss of fx
no filtering, no phagocytic response -->r/f infection

Front 24

conditions seen w/ SCD

Back 24

dactylitis
splenomegaly
weak bones, freq bone infections
freq infections
leg ulcers
infarct in brain, heart
heart failure (murm common)
priapism
pain

Front 25

baseline Hgb in SCD

Back 25

6-10

Front 26

SCD home education

Back 26

prophy penicillin from 3mos-5yr
fever (38.3/101) req prompt attn
vaccines important
prevent dehydration

Front 27

Rx to increase fetal hemoglobin

Back 27

hydroxyurea

Front 28

pain mgmt in SCD

Back 28

tylenol, tylenol-3, opioid (dilautid, morphine)

no demerol

Front 29

pooling of blood in spleen
r/t to occlusion of sickled cells

Back 29

splenic sequestration

Front 30

splenic sequestration S/S

Back 30

enlarged spleen
decreased blood volume-->prof anemia, CV collapse, shock

Front 31

aplastic crisis

Back 31

cessation/reduction in prod of RBCs secondary to viral illness (5thdz/parvo)

Front 32

S/S aplastic crisis

Back 32

fever, chills, n/v

Front 33

presents like pneumonia, caused by microinfarction in lungs (O2 cannot reach lung tissue)

Back 33

acute chest syndrome (sickle cell pneumonia)

Front 34

Acute Chest S/S

Back 34

white lung space on x-ray
fever followed by chest pain

Front 35

vasoocclusive crisis

Back 35

cycle of hypoxia and acidosis
most common comp of SCD
r/t infarctions caused by sickle cell clumping

Front 36

CVA

Back 36

secondary to infarct/occlusion of blood flow to or in brain --> dec O2 to brain
usually leads to major cog impairments

Front 37

chronic transfusion therapy (CTT)

Back 37

indicated for sickle cell pts w/ high HgbS
transfuses HgbA to dilute HgbS
q3wk, day long procedure

Front 38

thalassemia

Back 38

inherited blood disorder of Hgb production

Front 39

thalassemia major

Back 39

Cooley's anemia (Thal B)
severe anemia, incompatible w/ life without transfusion
q 3 wk transfusions

Front 40

enlarged head w/
prominent frontal & parietal bones
enlarged maxilla
bony changes r/t bone marrow expansion

Back 40

thalassemia, Cooley's/non-Cooley's (?)

Front 41

iron overload

Back 41

related to chronic blood transfusions
iron settles in vital organs
chelation therapy
Desferal sq infusion, Exjade oral

Front 42

VonWillebrand Dz

Back 42

affects both sexes
Factor VIII clotting defect an inability of plats to clump
leads to prolonged bleeding time

Front 43

DDAVP nasal spray

Back 43

tx for VolWillebrand Dz

Front 44

ITP

Back 44

acquired hemorrhagic d/o, usually follows 2 wks after viral infection
S/S thrombocytopenia, petechiae -->purpura, bruising, bleeding
usually full recovery

Front 45

CBC plats <20,000

Back 45

ITP

Front 46

may be misdiagnosed as leukemia

Back 46

ITP

Front 47

ITP tx

Back 47

IV Ig

Front 48

hemophilia

Back 48

x-linked recessive, affects males
Factor 8 or 9 deficiency
slow, persistent, prolonged bleeding

Front 49

hemophilia patho

Back 49

clot formation prevented due to factor def -->prolonged bleeding

Front 50

mild hemophilia

Back 50

5-50% CF

Front 51

moderate hemophilia

Back 51

1-5% CF

Front 52

severe hemophilia

Back 52

>1% CF (spontaneous bleeding)

Front 53

hemarthrosis

Back 53

bleeding into joint, seen in hemophila
esp knee, elbow, ankle-->swelling, pain, stiffness, loss of fx

Front 54

medical tx that causes vasoconstriction

Back 54

DDAVP (VonWillebrand, mild hemophilia)

Front 55

Henoch-Schonlein Purpura

Back 55

allergic vasculitis, usually follows URI
purpura on buttocks, lower ext
maculopapular rash
scalp and eyelid edema
risk for renal involvement

Front 56

HIV/AIDS

Back 56

virus invades CD4+lymphocytes adn replicates, CD4+produces virions, then becomes dysfunctional

Front 57

used as marker of HIV dz progression

Back 57

CD4+ counts

Front 58

pediatric CD4+ counts

Back 58

2000-3000

Front 59

vertical trans of HIV/AIDS

Back 59

mother to child, accounts for 90% of pediatric cases

Front 60

PCP prophylaxis

Back 60

Septra/Bactrim
q12h for 3 consec days for duration

Front 61

Severe Comb Immunideficiency Dz

Back 61

absence of humoral and cellular immunity
most common primary immunodeficiency
usually die w/in 2yr w/out tx
Tx: IV Ig, bone marrow transplant