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48 Cards in this Set

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microspherocytes
-small spherical RBCs
-high volume-to-surface ratio
-less deformable
-trapped & destroyed in spleen
-growth failure
-splenomegaly
-chronic transfusions
hereditary spherocytosis
hereditary spherocytosis: Tx
1.folic acid supplementation
2.splenectomy (after age 6)
when to do splenectomy in hereditary spherocytosis pts?
after age 6 (due to sepsis risk w/encapsulated bugs)
hereditary spherocytosis: dx confirmation
(+) osmotic fragility test
sickling phenomenon: exacerbated by...
1.hypoxia
2.acidosis
3.temperature changes
4.dehydration
T/F: sickling shortens RBC survival time --> chronic hemolytic anemia
true
sickle cell disease presents clinically at age x
4 months (HbF decreases, HbS increases)
common manifestations of anemia in sickle cell pts
1.pallor
2.jaundice
3.splenomegaly
4.systolic ejection murmur
5.delayed sexual development & growth
T/F: splenic sequestration can lead to hypovolemic shock
true
autoinfarction of spleen
1.microvascular obstruction
2.infarction
3.fiborsis
sickle cell disease pts w/fever - must be wary of...?
invasive bacterial infection
prophylaxis for sickle cell disease pts
penicillin starting at age 4 months until age 5
sickle cell disease: required vaccinations
1.HIB (2,4,6, 12-15 mos)
2.Prevnar (2,4,6, 12-15 mos)
3.PPV (age 2, 4-6 yrs)
sickle cell pt w/ams + seizure + focal paralysis. dx?
stroke (large cerebral vessel occlusion)
priapism in sickle cell pts occurs at ages x
age 6-20
sickle cell pts' hearts
enlarged hypertrophic --> CHF
sickle cell pts' GI
microvascular obstruction of intestines -> ileus -> rebound tenderness
purpose of hydroxyurea in sickle cell pts
1.reduce # & severity of vaso-occlusive crises
dactylitis:
what is it?
what age group?
-hand-foot syndrome
-symmetrical, painful swelling of dorsal surface of hands & feet
-etiology:avascular necrosis of metacarpal & metatarsal bones
-age group: 4-6 mos
painful crises in sickle cell: Tx
NSAIDS
narcotics
X made in liver, dependent on vitamin K
1.coagulation factors 2,7,9,10
2.antithrombotic factors C,S
causes of vitamin K deficiency
1.malabsorption (CF)
2.abx
3.meds (coumadin, phenobarbital, phenytoin)
most common disorder from vitamin K deficiency
hemorrhagic disease of the newborn
prophylaxis for hemorrhagic disease of the newborn
vitamin K 1mg IM
generalized ecchymosis + GI hemorrhage + bleeding fr/circumcision site & umbilical stump
hemorrhagic disease of newborn
(vitamin K deficiency)
hemorrhagic disease of newborn: Tx
1.FFP
2.prothrombin complex concentrate
hemorrhagic disease of newborn: confused w/...
1.liver disease
2.DIC
physiologic anemia of infancy: occurs at age x
2-3 mos (full-term)
6 wks (premie)
T/F: ESR elevated in anemia of chronic disease
true
hypochromic microcytic anemias: most common causes
1.iron deficiency
2.thalassemias
3.anemia of chronic disease
enhances nonheme iron absorption
ascorbic acid (vit. C)
adjusted reticulocyte count (ARC)
measured hct / expected hct x retic count
ARC < 2
ineffective erythropoiesis
ARC > 2
effective erythropoiesis (i.e. hemolysis, chronic blood loss)
pica: seen in...
1.lead poisoning
2.iron deficiency anemia
iron deficiency in premies seen as early as age x
3 months
baby w/severe anemia (hb < 3): symptoms
1.CHF
2.tachy
3.S3
4.cardiomegaly
5.hepatomegaly
iron deficiency anemia most common at ages x
6 mos - 2 years
mild/moderate anemia: tx
3-6 mg/kg/day elemental iron
iron deficiency tx results expected in...
1 month
most common RBC enzyme defect
G6PD deficiency
Heinz bodies = ?
globin precipitant seen in G6PD deficiency
G6PD: etiologies
1.sulfonamides
2.nitrofurantoin
3.primaquine
4.dimercaprol
5.fava beans
6.infection
protects RBCs from oxidative stress
reduced glutathione
G6PD deficiency results in...
decreased NADPH
G6PD: Tx
1.avoidance
2.supportive
3.IVF
4.pRBCs transfusion
5.urine alkalinization
bite/blister cells:
seen in...
occur b/c...
G6PD deficiency: macrophages take bite out of cells' Heinz bodies