Scienceisgood
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Hey Guys;

I am trying to find the reference ranges required to diagnose Wilson's Disease with respect to Liver Function Tests but am having trouble...

Any help please?
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macpatgh-Sheldon
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(Original post by Scienceisgood)
Hey Guys;

I am trying to find the reference ranges required to diagnose Wilson's Disease with respect to Liver Function Tests but am having trouble...

Any help please?
Scienceisgood


Hi,

The affliction of the liver in Wilson's disease is due to the deficiency of ceruloplasmin (copper-binding alpha-globulin) that results in deposition of copper in some organs (including the liver), and can, in the case of the liver, result in hepatocellular failure. This problem of the liver failing will produce abnormal results of LFTs (Liver Function Tests), which are of a more general nature [i.e. can be abnormal in other causes of hepatic failure e.g. hepatitis C, or in alcoholic cirrhosis [fibrosis of the liver]], as well as specific abnormalities e.g. low ceruloplasmin levels (<200mg/L) (diagnostic if Kayser-Fleischer rings [greenish circular rings around the iris border in the eye see pic below) are also present); 24-hr urinary copper excretion in excess of 1.6 micromoles; hepatic Cu concentration < 40-50 micrograms/g dry weight usually excludes Wilson's disease.

LFTs include bilirubin levels [elevated in liver disease], GOT (glutamate-oxaloacetate transaminase [old name - new: ALT??]), but these are not specific to Wilson's d.

Name:  Fig 12-7-2-2 Kaiser-Fleischer ring.jpg
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Scienceisgood
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(Original post by macpatelgh)
Scienceisgood


Hi,

The affliction of the liver in Wilson's disease is due to the deficiency of ceruloplasmin (copper-binding alpha-globulin) that results in deposition of copper in some organs (including the liver), and can, in the case of the liver, result in hepatocellular failure. This problem of the liver failing will produce abnormal results of LFTs (Liver Function Tests), which are of a more general nature [i.e. can be abnormal in other causes of hepatic failure e.g. hepatitis C, or in alcoholic cirrhosis [fibrosis of the liver]], as well as specific abnormalities e.g. low ceruloplasmin levels (<200mg/L) (diagnostic if Kayser-Fleischer rings [greenish circular rings around the iris border in the eye see pic below) are also present); 24-hr urinary copper excretion in excess of 1.6 micromoles; hepatic Cu concentration < 40-50 micrograms/g dry weight usually excludes Wilson's disease.

LFTs include bilirubin levels [elevated in liver disease], GOT (glutamate-oxaloacetate transaminase [old name - new: ALT??]), but these are not specific to Wilson's d.

Name:  Fig 12-7-2-2 Kaiser-Fleischer ring.jpg
Views: 122
Size:  9.2 KB
Thanks, more or less what I could find but aside from the copper excretion, Bilirubin Levels and ALT, I couldn't find much. =(

Again, thanks. =)
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