A 6-month-old boy is brought to the emergency department by his mother because of recent onset of vomiting, irritability, and jaundice. The infant was born at term and had been healthy until the onset of these symptoms. All of his vaccinations are up-to-date. He had been breast-fed exclusively until 1 week ago, when cereals and fruit juices were introduced into his diet. Further evaluation reveals hepatomegaly and abnormal liver function tests. Serum glucose is 30 mg/dL. Diagnostic testing confirms aldolase B deficiency. Which of the following should be removed from this patient's diet?

Question

A 6-month-old boy is brought to the emergency department by his mother because of recent onset of vomiting, irritability, and jaundice.  The infant was born at term and had been healthy until the onset of these symptoms.  All of his vaccinations are up-to-date.  He had been breast-fed exclusively until 1 week ago, when cereals and fruit juices were introduced into his diet.  Further evaluation reveals hepatomegaly and abnormal liver function tests. Serum glucose is 30 mg/dL.  Diagnostic testing confirms aldolase B deficiency.  Which of the following should be removed from this patient's diet?

Quizplus · Accepted Answer

Eductional Objective: Aldolase B deficiency, or hereditary fructose intolerance, leads to accumulation of the toxic metabolite fructose-1-phosphate.  Patients have hypoglycemia and vomiting when fructose or sucrose is consumed.  Treatment involves strict removal of both carbohydrates from the diet.

A 6-Month-Old Boy Is Brought to the Emergency Department by His