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Pediatrics Coagulation disorders ff89be57

The gold standard Lab test for diagnosis of PNH (Paroxysmal Nocturnal Hemoglobinuria) is?

A
Ham test
B
Haptoglobin
C
Flow cytometry
D
Sucrose Lysis test
High-Yield Explanation
1.)CD59 -It is also given the name ofMIRL(Membrane Inhibitor of Reactive Lysis). 2.)CD55 -It is given the name ofDAF(Decay Accelerating Factor) . It is responsible for breakdown ofC3. -Flow cytometry of RBC showing absence of CD55 and CD59 is gold standard diagnostic test for PNH. Other tests used are: Sucrose hemolysis test: patient's whole blood is mixed with isotonic sucrose solution , which promotes binding of complement to red blood cells, then incubated and examined for hemolysis; greater than 10percent hemolysis indicates PNH Acidified serum (Ham test): Acidification of serumactivates complement alternate pathway and causes haemolysis of red cells if they are abnormally sensitive to complement.

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