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 Wilson's disease and A1AT disease  

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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?



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!


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


serum cerulloplasmin????


hi serum and urinary copper, low serum ceruloplasmin

HEPATIC copper content>250 ug/g dry wt


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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