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Pediatric Rounds Review Questions

An 18-Month-Old Child with a Previously Undiagnosed Fatty Acid Oxidation Disorder

Mark A. Hostetler, MD, MPH

Dr. Hostetler is an Assistant Professor; Chief of the Section of Emergency Medicine, Department of Pediatrics; and Medical Director of the Pediatric Emergency Department, University of Chicago Children’s Hospital, Chicago, IL.

The questions below are based on the June 2003 Pediatric Rounds article, “An 18-Month-Old Child with a Previously Undiagnosed Fatty Acid Oxidation Disorder.”

Choose the single best answer for each question.

1. Which of the following is the appropriate treatment for symptomatic hypoglycemia in a 12-month-old child?
  1. 2 mL/kg of 50% dextrose solution
  2. 2 mL/kg of 25% dextrose solution
  3. 2 mL/kg of 5% dextrose solution
  4. 20 mL/kg of normal saline solution
Click here to compare your answer.


2. Which of the following is a cause of hypoglycemia in children?

  1. Acetaminophen ingestion
  2. Alcohol ingestion
  3. Closed head injury
  4. New-onset diabetes
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3. Which of the following best describes the constellation of metabolic disturbances associated with fatty acid oxidation disorders?
  1. Ketoacidosis with a high anion gap
  2. Hypoketotic hypoglycemia with normal to moderately elevated ammonia level
  3. Hypoketotic hypoglycemia with markedly elevated ammonia level
  4. Normoglycemia with markedly elevated ammonia level
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4. Which of the following is the most common of the fatty acid oxidation disorders?

  1. Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency
  2. Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency
  3. Carnitine palmitoyltransferase (CPT) I deficiency
  4. Carnitine palmitoyltransferase (CPT) II deficiency
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