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14 Cards in this Set
- Front
- Back
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defen
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G6PD deficiency-X linked recessive( almost only men)
cant generate glutathione reductase and protect red cell from oxidant stress most common oxidant stress is infection other causes are dapsone quinidine sulfa drugs promoquine nitrofurantoin fava beans |
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what is most likely diagnosis
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look for AA or mediterr men with sudden anemia and jaundice who have a normal size of spleen,with an infection or are using the drugs previously listed
hemolys intravascular |
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where is hemolysis
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hemolys intravascular
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clinica
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acute intravasc hemolysis
with jandice 1.2 days afterexposure abdominal and back pain anemia ocasional renal failure |
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ds
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hemoglobinuria
jaundice heinz bodies increased RBC methhemoglobion can essay enzyme level |
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best initial test is for heinz and bite cells
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best initial test is for heinz and bite cells
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can essay enzyme level
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will be normal after hemolytic event
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most accurate test
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level of enzyme after 1-2 months after event
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pathology
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oxidation of hemoglobin produce meth hemoglobin with characterize heins bodies( denatured hemoglobin
it will lead to fragility of red blood cells and spleen will bite them |
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tx
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nothing will revers hemolysis
avoid stress |
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what will u see in intra vasc hemolysis
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high LDH
high bilirubin and reticulocyte count normal MCV low haptoglobin hemoglobinuria |
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most specific test
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level of enzyme
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types of G6PD
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2
mild- in AA( A variant) involving only old RBC an spare younger RBC. hemol episodes are usually triggered byinfection or drugs as antimalarian and sulfur containing more severe is in medeterrinian, youn RBc also involved when exposed to fava beans-->> severe hemolyss may need transfusion until drug is eliminated from the body |
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what will be om essay
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deficit of NADPH
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