A 4-Year-Old Boy Is Evaluated Due to 4 Days of Fever

question 213

Multiple Choice

A 4-year-old boy is evaluated due to 4 days of fever and leg pain.  Medical history includes recurrent skin abscesses and lymphadenitis.  A maternal uncle died of recurrent infections during childhood.  The patient is at the 10th percentile for height and weight.  Temperature is 38.3 C (100.9 F) .  Examination shows an ill-appearing patient with point tenderness over the right lower tibia.  There are healed incisions over the thighs and neck from prior drainage sites.  Imaging reveals evidence of tibial osteomyelitis.  Blood and bone cultures grow Serratia marcescens.  Evaluation of the patient's neutrophils by dihydrorhodamine flow cytometry shows an absence of fluorescence.  This patient's condition is most likely due to impaired activity of which of the following enzymes?

• A)Adenosine deaminase
• B)Cytochrome c oxidase
• C)Lysozyme
• D)Myeloperoxidase
• E)NADPH oxidase

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