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  #1  
Old 11-04-2011
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Liver Andersens disease

A 6-month-old infant with hepatosplenomegaly is diagnosed with Andersenís disease. Which of the following would lead to cirrhosis?
  • A. Accumulation of abnormally folded glycoprotein in hepatocytes
  • B. Recurrent hypoglycemia
  • C. Absence of glycogen in hepatocytes
  • D. Foreign body reaction to abnormal glycogen in hepatocytes
  • E. Persistent lactic acidemia
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  #2  
Old 11-04-2011
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A= occurs in alpha1 antitrypsin deficiency
B= occurs in MaCardle's and many other diseases
C= don't think it may lead to cirrhosis
D= Glycogen is not a protein
E= i think it's is the answer based on guess work
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  #3  
Old 11-04-2011
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i guess E too
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  #4  
Old 03-14-2013
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A is the answer
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Old 03-14-2013
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I guess A also but not sure why.

pls inbox me the correct answer.
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  #6  
Old 03-14-2013
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A seems right but
why not D ?
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  #7  
Old 03-14-2013
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My first thought went to A but i have forgotten the reason, i remember seeing the disease somewhere in my reading
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Old 06-18-2013
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I agree

Quote:
Originally Posted by scopusmount View Post
A 6-month-old infant with hepatosplenomegaly is diagnosed with Andersenís disease. Which of the following would lead to cirrhosis?
  • A. Accumulation of abnormally folded glycoprotein in hepatocytes
  • B. Recurrent hypoglycemia
  • C. Absence of glycogen in hepatocytes
  • D. Foreign body reaction to abnormal glycogen in hepatocytes
  • E. Persistent lactic acidemia
i think it's D , but it should not be a foreign body reaction,,,,
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Old 06-18-2013
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I believe the answer is D.

In Andersen's Disease, the branching enzyme is deficient. This leads to formation abnormal glycogen (long chain without branches). Long chain glycogen is relatively less soluble and have a tendency to precipitate. As the result, the body immune attack the hepatocytes perceiving they're foreign. So one of main characteristic of this disease is cirrhosis and death by 2 - 5 years. Cirrhosis also occur in Hers Disease but it's more mild.
Hope that help,

Quote:
Originally Posted by scopusmount View Post
A 6-month-old infant with hepatosplenomegaly is diagnosed with Andersenís disease. Which of the following would lead to cirrhosis?
  • A. Accumulation of abnormally folded glycoprotein in hepatocytes
  • B. Recurrent hypoglycemia
  • C. Absence of glycogen in hepatocytes
  • D. Foreign body reaction to abnormal glycogen in hepatocytes
  • E. Persistent lactic acidemia
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