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

question 213

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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?


Definitions:

Subcultures

Smaller cultures that develop within a larger organizational culture and are based on differences in training, occupation, or departmental goals.

Beliefs

Acceptance that something exists or is true, especially without proof, or the held opinions or convictions of an individual or group.

Institutionalized Socialization

The process by which individuals learn and adapt to the norms and roles expected of them by an organization or society at large.

Individualized Socialization

A personalized approach to integrating a new member into a group or organization, taking into account their unique needs and abilities.

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