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