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Kaplan Qbank USMLE



Author4 Posts
  #1

what is the major consequence of the hereditary defect in production of active pyruvate kinase in liver and erythrocytes?

a.dec capacity to fix co2

b.dec gluconeogenesis from alanine

c.inc afinity of hemoglobin for oxygen

d.dec oxidation of pyruvate

e.dec prod of atp from glucose


  #2

e

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  #3

agree with you

  #4

Energy is derived from glucose (carbohydrate) metabolism and this currency is either ATP or NADPH. RBCs lack mitochondria, so there is no Kreb's cycle but they make a lot of energy through Glycolysis ( which yields ATP) and HMP shunt ( which yields NADPH). Any enzymatic defect in these steps leads naturally to hemolytic anemias amongst other things. Now, 2 most common enzyme defects are pyruvate kinase def - glycolysis - so the result will be a loss in its currency ie ..ATP... and G6PD def- HMP shunt pathway - so loss of NADPH. in both PK and NADPH there will be hemolysis but loss of ATP & NADPH respectively..other points of interest - PK is autosomal recessive while G6PD is sex linked recessive







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