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 Wilson's disease and A1AT disease  



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Author6 Posts
  #1

I had a practice questions that had dealt with wilsons's disease and while readingup a quick review of it, i read that it can also stain positive for PAS.(periodic acid-schiff). however i know that alpha 1 antitrypsin deficiency disease also is pos for PAS.

when i had anohte quesion that dealt with it i had a choice btwn the two, this time i picked wilsons, but i got this wrong as well. so which is it..positive pas for wilsons or A1AT disease?

thank,
mustafa





  #2

what i think i figured it out, im confusing wilson's disease with whipple disease with the PAS staining, bc whipple disease has positive PAS staining in the lamina propria.

thanks for looking!



  #3

smiling face..and how do we dx wilson's?


  #4

serum cerulloplasmin????


  #5

hi serum and urinary copper, low serum ceruloplasmin

HEPATIC copper content>250 ug/g dry wt



  #6

The presence of Kayser-Fleischer rings and ceruloplasmin levels of less than 20 mg/dL in a patient with neurologic signs or symptoms suggest the diagnosis of Wilson disease

If a patient is asymptomatic, exhibits isolated liver disease, and lacks corneal rings, the coexistence of a hepatic copper concentration of more than 250 mg/g of dry weight and a low serum ceruloplasmin level is sufficient to establish a diagnosis.






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