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Q549

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1. A 2-month-old child is evaluated for failure to thrive. As the pediatrician is examining the child, a convulsion occurs. Stat serum chemistries demonstrate severe hypoglycemia, hyperlipidemia, lactic acidosis, and ketosis. Physical examination is remarkable for hepatomegaly, a finding confirmed by CT scan, which also reveals renomegaly. Which of the following diseases best accounts for this presentation?
A.
B.
C.
D.
E.


 

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