A 2-year-old boy is brought to the emergency department due to 4 days of worsening cough and fevers. This evening, the patient developed increasingly rapid breathing. He has a history of 2 prior episodes of cervical lymphadenitis due to Staphylococcus aureus. Temperature is 39 C (102.2 F). Blood pressure is 100/72 mm Hg, pulse is 130/min, and respirations are 35/min. Prominent intercostal and supraclavicular retractions are present. There are diffuse crackles bilaterally, with decreased breath sounds at the lung bases. Heart sounds are normal. The patient is intubated. Laboratory results are as follows: Bronchioalveolar lavage fluid tests positive for Aspergillus fumigatus. Serum immunoglobulin levels are normal. Which of the following is the most likely underlying cause of this patient's presentation?

Question

A 2-year-old boy is brought to the emergency department due to 4 days of worsening cough and fevers.  This evening, the patient developed increasingly rapid breathing.  He has a history of 2 prior episodes of cervical lymphadenitis due to Staphylococcus aureus.  Temperature is 39 C (102.2 F).  Blood pressure is 100/72 mm Hg, pulse is 130/min, and respirations are 35/min.  Prominent intercostal and supraclavicular retractions are present.  There are diffuse crackles bilaterally, with decreased breath sounds at the lung bases.  Heart sounds are normal.  The patient is intubated.  Laboratory results are as follows:  Bronchioalveolar lavage fluid tests positive for Aspergillus fumigatus.  Serum immunoglobulin levels are normal.  Which of the following is the most likely underlying cause of this patient's presentation?

Quizplus · Accepted Answer

Eductional Objective: Chronic granulomatous disease should be suspected in a child with recurrent infections by catalase-positive organisms (eg, Staphylococcus aureus, Aspergillus) who has normal immunoglobulins and no leukopenia.  Pathogenesis involves the defective intracellular killing of phagocytized organisms within neutrophils due to an impaired respiratory burst.

A 2-Year-Old Boy Is Brought to the Emergency Department Due