Which disease results from a defect in neutrophils and is characterized by recurrent bacterial infections?

Which disease results from a defect in neutrophils and is characterized by recurrent bacterial infections?

• A. Chronic granulomatous disease
• B. Severe combined immunodeficiency
• C. Adenosine deaminase deficiency
• D. Wiskott-Aldrich syndrome

Answer: (A)

Chronic granulomatous disease (CGD) is characterized by a defect in the NADPH oxidase complex in neutrophils, which impairs the ability of these immune cells to produce reactive oxygen species (ROS) during the respiratory burst. This inability to adequately kill ingested bacteria means that individuals with CGD are particularly susceptible to recurrent bacterial and certain fungal infections. Patients often experience infections with catalase-positive organisms, such as Staphylococcus, Aspergillus, and others, which are particularly problematic for those with CGD since these pathogens can neutralize hydrogen peroxide, one of the few reactive species produced by neutrophils that are functional in CGD. The hallmark symptom of CGD is repeated infections, especially during childhood, which can lead to significant morbidity if not managed appropriately. The other options, while associated with immune system deficiencies, do not specifically relate to a defect in neutrophil function leading to recurrent bacterial infections in the same direct way as CGD.

Chronic granulomatous disease (CGD) is characterized by a defect in the NADPH oxidase complex in neutrophils, which impairs the ability of these immune cells to produce reactive oxygen species (ROS) during the respiratory burst. This inability to adequately kill ingested bacteria means that individuals with CGD are particularly susceptible to recurrent bacterial and certain fungal infections.

Patients often experience infections with catalase-positive organisms, such as Staphylococcus, Aspergillus, and others, which are particularly problematic for those with CGD since these pathogens can neutralize hydrogen peroxide, one of the few reactive species produced by neutrophils that are functional in CGD. The hallmark symptom of CGD is repeated infections, especially during childhood, which can lead to significant morbidity if not managed appropriately.

The other options, while associated with immune system deficiencies, do not specifically relate to a defect in neutrophil function leading to recurrent bacterial infections in the same direct way as CGD.