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  #1  
Old 10-11-2012
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Urine Sample Complement Mediated Hemoglobin in Urine!

A 4 yr old male child has been taken to the physician for evaluation of difficulty in swallowing since a week by his parents...his accompanying symptoms include unable to play with peers well, tiredness, orthopnea and observed dark urine from 2 days...his physical examination include his eyes look pale but with no scleral icterus. his blood examination not shown any kind of heinz bodies or schistocytes, with normocytic to microcytic hemolytic anemia..urine exam detects Hb loss in urine. his diagnosis is detected as complement mediated defect with no autoimmune etiology..what is the confirmatory test used for the disease to confirm?
a) FISH
b) Hb electrophoresis
c) sucrose lysis test
d) osmotic fragility test
e) flow cytometry
f) RFLP analysis
g) direct coombs test
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Old 10-11-2012
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Sucrose hemolysis test .. PNH ...
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Quote:
Originally Posted by alice View Post
Sucrose hemolysis test .. PNH ...
sucrose hemolysis test is not confirmatory ......
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  #4  
Old 10-11-2012
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Flow cytometry for PNH.

Sucrose lysis test is for screening.

what abt Ham test? is it for screening or confirmatry??
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Quote:
Originally Posted by Timmi View Post
Flow cytometry for PNH.

Sucrose lysis test is for screening.

what abt Ham test? is it for screening or confirmatry??
it's also confirmatory

thanks
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Old 10-11-2012
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I dint know that ! Thanks
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Old 10-11-2012
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e) flow cytometry search for CD 55 or CD 59. if not present confirmation of PNH
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Correct Answer E is right

the correct answer is E) flow cytometry

the child is suffering from PNH---complement mediated RBC lysis with fatigue due to hemolytic anemia,lightheadedness,tiredness,dysphagia and odynophagia in severe cases,dark colored urine due to intravascular hemolysis(hemoglbinuria,hemosiderinuria,decreased haptoglobin),orthopnea due to acidosis
due to defect in GPI anchor protein which anchors for DAF and MIRL which protect from complement destruction ....
triggering factor for complement activation------> acidosis(sleep cause shallow breathing leading to acidosis)--->early morning dark urine

diagnosis-----screening by sucrose lysis(by administering sucrose--activate complement and lyse rbc)

confirmation by acidified serum/HAM test(acidosis is triggering for activation of complement)..
nowadays flow cytometry is also using for confirmation instead----PNH has expression of CD55 and CD 59 ,which are detected by cytometry

flourescence induced tests have been developing too....but not yet mandatory for diagnosis
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Old 10-11-2012
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Two misnomers about it are 1. It is not an episodic hematuria and 2. It is not nocturnal.. There is microscopic hematuria all through out the day .. ( u world cleared it ! )
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screening test is sugar water test
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Quote:
Originally Posted by venky2600 View Post
the correct answer is E) flow cytometry

the child is suffering from PNH---complement mediated RBC lysis with fatigue due to hemolytic anemia,lightheadedness,tiredness,dysphagia and odynophagia in severe cases,dark colored urine due to intravascular hemolysis(hemoglbinuria,hemosiderinuria,decreased haptoglobin),orthopnea due to acidosis
due to defect in GPI anchor protein which anchors for DAF and MIRL which protect from complement destruction ....
triggering factor for complement activation------> acidosis(sleep cause shallow breathing leading to acidosis)--->early morning dark urine

diagnosis-----screening by sucrose lysis(by administering sucrose--activate complement and lyse rbc)

confirmation by acidified serum/HAM test(acidosis is triggering for activation of complement)..
nowadays flow cytometry is also using for confirmation instead----PNH has expression of CD55 and CD 59 ,which are detected by cytometry

flourescence induced tests have been developing too....but not yet mandatory for diagnosis
I think, PNH has DECREASED expression of CD 55 and CD59 (due to loss of GPI anchor protein.) in Flow cytometry.
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  #12  
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Quote:
Originally Posted by Janakpriya View Post
I think, PNH has DECREASED expression of CD 55 and CD59 (due to loss of GPI anchor protein.) in Flow cytometry.
ya,it's decreased expression....thanks
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