Section: USMLE

Question 11 of 60

11) A 2-month-old child is evaluated for failure to thrive. During the examination, the child has a seizure. 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?