Justiz-Vaillant, A.A.; Williams-Persad, A.F.; Arozarena-Fundora, R.; Akpaka, P.E.; Gopaul, D. Chronic Granulomatous Disease (CGD): Pathogens and Management. Preprints2023, 2023061557. https://doi.org/10.20944/preprints202306.1557.v1
APA Style
Justiz-Vaillant, A.A., Williams-Persad, A.F., Arozarena-Fundora, R., Akpaka, P.E., & Gopaul, D. (2023). Chronic Granulomatous Disease (CGD): Pathogens and Management. Preprints. https://doi.org/10.20944/preprints202306.1557.v1
Chicago/Turabian Style
Justiz-Vaillant, A.A., Patrick Eberechi Akpaka and Darren Gopaul. 2023 "Chronic Granulomatous Disease (CGD): Pathogens and Management" Preprints. https://doi.org/10.20944/preprints202306.1557.v1
Abstract
Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by a defect in the phagocytic function of the innate immune system owing to mutations in genes encoding the five subunits of the nicotinamide adenine dinucleotide (NADPH) oxidase enzyme complex. This review aimed to provide a comprehensive approach to the pathogens associated with chronic granulomatous disease (CGD) and its management. The topics covered included common pathogens infecting CGD patients, granulomas, and CGD-related infectious diseases, including invasive aspergillosis, Mulch pneumonitis, and liver abscesses. In addition, we also covered the differential diagnosis of CGD and its management. Patients with CGD, often children, have recurrent life-threatening infections and may develop infectious or inflammatory complications. The most common microorganisms observed in the patients with CGD are Staphylococcus aureus, Aspergillus spp, Candida spp, Nocardia spp, Burkholderia spp, Serratia spp, and Salmonella spp. Triple-dose antimicrobial prophylaxis with empiric regimens is recommended. Therefore, therapeutics against this disease must be optimised, considering the lack of curative therapies and the long-term side effects of prophylactic regimens. Consequently, allogeneic haematopoietic stem cell transplantation from a human leukocyte antigen-identical donor has been proven to cure CGD.
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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