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mcq - nimishkum
#1
A 10-year-old boy of Ashkenazi Jewish descent is seen in clinic for a regularly scheduled check-up. For the past month the child has complained of tiring easily and leg pain with physical exertion. On physical examination the child is somewhat ill-appearing with conjuctival pallor. The spleen is palpable 5 cm below the left costal margin. There is some nodularity apparent. There is some pain with weight bearing on the left leg. Laboratory results are significant for hemoglobin (Hb) 10.4 g/dL, platelets 75k/mcL, whole blood count (WBC) 2.4k/mcL and absolute neutrophil count 1.5k/mcL. X-rays of the left leg show an exaggerated flaring deformity of the left distal femur. A diagnostic work-up including bone marrow aspirate confirms the diagnosis most strongly suggested by the information provided. What treatment is indicated at this time?


A. Corticosteroid administration
B. Imiglucerase administration
C. Plasmapheresis
D. Platelet transfusion
E. Splenectomy
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#2
B...
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#3
I think its Gaucher's disease
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#4
femor bone, Jews, splenomegaly, what's that? lysosome disease? Gaucherr? what's the treatment? give that enzyme, but B is not enzyme. take D
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#5
B, Imiglucerase
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#6
B surely is an enzyme, but not THAT enzyme, never heard this enzyme name
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#7
b correct
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#8
Alglucerase and Imiglucerase are modified glucocerbrosidase
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